MRCP PART 2 WRITTEN APRIL 2011 (recall of questions)

Discussion in 'MRCP Forum' started by Guest, Apr 7, 2011.

  1. sarahpart2

    sarahpart2 Guest

    will appear early next yr , no clinical experience yet
  2. sarahpart2

    sarahpart2 Guest

    dR albarwari we are waiting for more notes please
  3. Kumar Mongar

    Kumar Mongar Guest

    Hi all friends who passed the exam, please give your advice & learning tactics! I failed this time. I'll try for Nov exam. Thank you very much for Dr Albawari for his contribution.
    Who will sit for Nov exam. Please keep in touch for discussion.
  4. Guest

    Guest Guest

    thank alot for dr. albarwari ....really u r great man ....God bless u .... we keep praying for you ...u did hard work ....

    only we need more posting tpoic like nurology ..... resp...if u have time for that ...any way ...we are ashame to reguest another ....bec all work in bussy duty ...thanks again for your helping other.
  5. Dr.AY

    Dr.AY Guest


    thanks a lot for dr. albarwari... U R REALLY GREAT.. I AM UR SECRET FAN...
  6. sarah part 2
    when u have finshed part 2 already why r u waiting for the notes
  7. kumar mongar

    am planning for november

    what r u r plans

    what r u doing which site now
  8. Dr albarwari

    Dr albarwari Guest

    Dear Dr.AY Kumar Mongar
    Thanks for nice words

    But if you do not mind please ,,,do not praise any body,,,,I am not a great man as every body know himself better than other "I swear by ALLAH this is my feelings" I am just one of you and no difference from anyone of you ,,,and if I could be helpful,,this is just a grace and generosity of ALLAH who has blessed and honored me, even if not with ALLAH help, I was not passed this should make me to thank ALLAH

    If my notes are helpful I want to ask every body who will get benefit and share with others to pray for me not only for passing PACES as it is an exam like any other Exam in our life but really I want to pass the final EXAM when I stand between ALLAH hands I swear this is my feelings and what I beleive

    Dear all who failed the last Exam or new for the exam,,,beleive me you will pass the exam if you prepare well and trust ALLAH and have Determination and Insistence for passing the Exam INSHALLAH


    • use mefenamic acid for perimenstrual prophylaxis of menstrually related migraines, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation.
    • Metoclopramide has been shown to have efficacy for both the pain and nausea associated with migraine. safely used in pregnancy
    • Topiramate is second line prophlaxis for migraine after propranolol
    • Oral contraceptive pills are contraindicated in women with migraine with aura, especially if the aura involves more than just simple visual aura, if there are additional stroke risk factors, or if the aura begins after the initiation of oral contraception
    Tension-type headache
    • is distinguished from migraine by the fact that patients with tension headache are not disabled and can carry out activities of daily living in a normal, expedient manner.

    Medication overuse headache
    • headache for more than 15 days per month and the use of acute headache medication on more than 10 days per month.

    Trigeminal neuralgia.
    • affects the right side of the face five times more frequently than the left.
    • The number of attacks may vary from one a day up to a hundred per day, and patients are typically asymptomatic in between attacks
    • Trigeminal neuralgia is a disease of middle-to-late age female
    • We should evaluate for secondary causes of Trigeminal neuralgia if it
    o occur in a young patient
    o associated with any physical signs as reduced corneal reflex and reduced sensation in the mandibular and maxillary division of the trigeminal
    • secondary causes of Trigeminal neuralgia
    o multiple sclerosis,
    o posterior fossa tumors, and
    o vascular or aneurysmal compression of the trigeminal nerve.
    • Usual treatment for trigeminal neuralgia would be a titrated dose of carbamazepine, with addition of gabapentin or amitriptyline if needed.
    • The disorder may cease spontaneously after 6-12 months.
    The trigeminal autonomic cephalalgias

    Disease Duration Frequency Treatment
    Cluster headache 1 hour 1-3/day

    prefer to be mobile, because resting causes worsening of the pain
    1. verapamil
    2. Prednisone is the most appropriate treatment for episodic cluster headache.
    Paroxysmal hemicrania 15 minutes 11/day indomethacin
    SUNCT syndrome 60 seconds 30-200/day lamotrigine
    Migrain > 3hours Resting in a dark, quiet room results in improvement.

    Thunderclap headache
    • is head pain that begins suddenly and is severe at onset.
    • TCH might be the first sign of
    o subarachnoid haemorrhage,
    o unruptured intracranial aneurysm,
    o cerebral venous sinus thrombosis,
    o cervical artery dissection,
    o acute hypertensive crisis,
    o spontaneous intracranial hypotension,
    o ischaemic stroke,
    o retroclival haematoma,
    o pituitary apoplexy,
    o third ventricle colloid cyst, and
    o intracranial infection.
    • CT of the head without enhancement is the most sensitive test for subarachnoid hemorrhage.
    • A lumbar puncture is necessary if CT scan results are normal.
    • If results of both the CT scan and lumbar puncture are normal, neurovascular imaging is indicated in these patients and should include magnetic resonance or CT angiography (or venography).

    Vertebral artery dissection
    • Typically presents with
    o neck or head pain,
    o Horner's syndrome,
    o dysarthria,
    o dysphagia,
    o decreased pain and temperature sensation,
    o dysmetria,
    o ataxia, and
    o vertigo.
    • Magnetic resonance angiography is a sensitive diagnostic test for vertebral artery dissection as a cause of stroke.
    • If MR angiography negative and suspicion is still high, 4 vessel cerebral angiography can be arranged.
    • Where there is no evidence of sub-arachnoid haemorrhage, anti-coagulation is the management of choice to prevent thromboembolic sequelae as a result of the dissection
    Subclavian steal syndrome
    • is associated with retrograde flow in the vertebral artery due to a proximal subclavian artery stenosis.
    • Neurological symptoms are precipitated by vigorous exercise with the arm above the head, such as painting a wall, they are in fact related to reduced blood flow.
    • The diagnosis is often confused with transient ischaemic attacks or epilepsy.
    • Duplex ultrasound and magnetic resonance angiography are the investigations of choice.
    • Endarterectomy and stenting are common surgical methods involved in relieving symptoms associated with this condition.
    Subarachnoid hemorrhage
    • CT brain imaging within the first 48 hours is approximately 95% sensitive at identifying SAH.
    • However, a normal CT scan does not exclude SAH and a lumbar puncture should be performed if the CT scan is negative and SAH is still suspected.
    • Since bilirubin is undetectable in the cerebrospinal fluid (CSF) until 12 hours after the onset of symptoms, a lumbar puncture should be delayed until after this time unless meningitis is suspected.
    • Lumbar puncture is most sensitive in the initial period (12 hours after the onset of symptoms and up to 14 days later).
    • Bilirubin may be detected in CSF by spectrophotometry
    • visual inspection for the yellow discoloration (xanthochromia).from 12 hours up to 2 weeks
    • Evidence clearly indicates that visual inspection is not a reliable method.
    • CSF spectrophotometry detects the presence of both oxyhaemoglobin and bilirubin because either one or both of these pigments may contribute to xanthochromia following SAH. CSF supernatant is scanned
    • CSF may be of this
    Appearance Clear Clear
    Opening pressure 17 cm H20 (NR 4 - 18)
    CSF white cell count 0 cells/mL (NR <5)
    CSF red cell count 4 cells/mL (NR <5)
    CSF protein 0.40 g/L (NR 0.15 - 0.45)
    Xanthochromia screen Negative by visual inspection
    • CT angiography and four vessel angiography would be the next management step for those with a confirmed diagnosis of SAH.
    • An MRI brain scan is inferior to CT imaging in SAH.
    • MR angiography is less sensitive
    • Focal neurologic symptoms 3 to 7 days after a subarachnoid hemorrhage may be due to vasospasm with cerebral ischemia.
    • patient should receive adequate analgesia, balancing pain relief against interfering with Glasgow coma scale measurement
    • If the diagnosis is not confirmed, oral nimodipine is not recommended
    Use of steroid for all intracranial bleeds remains unproven, and in the absence of major shift cannot be recommended.

    CSF Examination in Subarachnoid hemorrhage
    • The gold standard method is by CSF spectrophotometry, which must be requested at the time of sending the CSF to the lab,
    • the CSF has to be spun down immediately (thus removing any ‘acute’ red cells that are contaminants from the LP itself).
    • The CSF can then be analysed by spectrophotometry for evidence of prior bleeding.
    • If sending CSF to the lab overnight, it can be spun down, then stored for analysis the next morning.
    • When blood enters the CSF by SAH or by traumatic tap it will haemolyse to form oxyhaemoglobin. This is seen as an absorption peak on the spectrophotometry.
    • The conversion of oxyhaemoglobin to bilirubin occurs only in the presence of haemoxygenase, which is found only in macrophages, the arachnoid membrane and the choroid plexus.
    • Bilirubin causes a second absorption peak on the downward slope, the oxyhaemoglobin peak. This bilirubin peak on spectrophotometry cannot be caused by a traumatic tap and indicates a SAH (unless a traumatic tap has been preceded by a previous traumatic tap more than 12 h before, or the serum bilirubin or CSF protein is elevated).

    Spectrophotometry can detect both oxyhaemoglobin and bilirubin. Oxyhaemoglobin produces an absorption peak at 413–415 nm, bilirubin when detected alone produces a peak at 450–460 nm.

    Extra cranial vertebral artery dissection
    • stroke in young people (less than 40 years old).
    • The onset of severe occipital pain usually caused by trivial trauma
    • About 85% of patients develop focal neurological signs due to ischaemia of the brain stem or cerebellum.
    o vertigo, dysarthria, ataxia, nausea and vomiting nystagmus
    o intention tremor
    o Tone, power, reflexes and sensation are all may be normal normal.
    • The commonest neurological manifestations are lateral medullary dysfunction
    • Infarction of the cervical spinal cord
    o occlusion of the anterior spinal artery
    o Weakness in shoulders Abduction
    o Knee flexors were 2/5 on both sides.
    o All limbs were flaccid and the tendon reflexes were universally absent.
    o Both plantars were upgoing.
    o Pinprick was impaired over both shoulders;
    o but joint position sense and vibration were intact for all the limbs
    • Ehlers–Danlos syndrome and fibromuscular dysplasia are risk factors.
    • Axial MRI images show a double lumen in the dissected vessel.
    • Treatment is anticoagulation
    Extracranial carotid artery
    • Neck pain
    • haematoma in the neck as a mass
    • Hemiparesis
    • Examination findings may include the following:
    o Ipsilateral partial Horner syndrome (32-82% of patients in various series)
    o Ipsilateral cranial nerve palsies, particularly cranial nerves 9, 10, 11, 12 more often than the"5, 6, 7 nerves" in up to 12% of patients
    o Audible bruit (up to 20% of patients)
    • Diagnosis
    o Gold standard for diagnosis of carotid dissection is contrast arteriography.
    o Other, non-interventional methods are more commonly performed when it is suspected
     duplex scanning and
     magnetic resonance angiography
     Fine computed tomography neck ‘cuts’ can also make the diagnosis in cases where there is significant haematoma.
    • Carotid dissections more rarely involve intracranial vessels and the neck pain in this case makes it likely to be extracranial.
    • The main complications are completed stroke, usually from thrombus formation associated with the damage to the endothelium. It is for this reason that anticoagulation is often advised if not otherwise contraindicated

    Occlusion of anterior inferior cerebellar artery
    • Ipsilateral
    o lateral inferior pontine infarction.
     8th nerve vertigo, vomiting, horizontal and vertical nystagmus, tinnitus.
     7th nerve A lower motor neuron facial weakness
     parapontine reticular formation,,,,paresis of conjugate lateral gaze
    o cerebellar ataxia
    o Horner’s syndrome (descending sympathetic) and
    o rarely deafness (cochlear nerve or nucleus)
    • Contralateral
    o impaired pain and temperature over the half of the body "spinothalamic tract" involvement.

    Benign intracranial hypertension
    • Headache, which occurs in almost all (92–94%) cases. worse in the morning, generalized in character and throbbing
    • 10% of cases are free of headaches.
    • nausea and vomiting.
    • worse by coughing and sneezing.
    • pain may also be experienced in the neck and shoulders
    • pulsatile tinnitus
    • abducens nerve palsy "double vision"
    • rarely, the oculomotor nerve and trochlear nerve facial nerve
    • Bilateral papilledema, "transient visual obscurations", episodes of difficulty seeing that occur in both eyes but not necessarily at the same time.
    • Long-term untreated papilledema leads to visual loss, leads to optic atrophy
    • Diagnosis
    o Neuroimaging Should be done first CT or MRI "MRI brain is better than CT brain to look for cerebral venous thrombosis", rules out mass lesions. In IIH these scans may be normal, although small or slit-like ventricles and "empty sella sign" (flattening of the pituitary gland due to increased pressure) may be seen. An MR venogram to exclude the possibility of cerebral venous sinus thrombosis.
    o Lumbar puncture is performed to measure the opening pressure,Occasionally, the pressure measurement may be normal despite very suggestive symptoms. This may be due to the fact that CSF pressure may fluctuate over the course of the day. If the suspicion remains high, it may be necessary to perform more long-term monitoring of the ICP by a pressure catheter

    Ischemic stroke
    • The preferred treatment of intravenous rtPA,within 3 hours from stroke onset "even patient has AF"
    o Blood pressure lowered and stabilized (systolic <185 mm Hg, diastolic <110 mm Hg)
    o In a patient with ischemic stroke treated with recombinant tissue plasminogen activator (rtPA), systolic blood pressure should be kept below 180/105 mm Hg for 24 hours after rtPA treatment; intravenous labetalol or nicardipine can best achieve this goal.
    o rtPA is not contraindicated if warfarin taken if INR less than or equal to 1.7
    o No seizure with postictal residual neurologic deficits
    o Platelet count of 100,000/“µL (100 × 109/L) or greater and plasma glucose level of 50 mg/dL (2.8 mmol/L) or greater
    • To maximise benefit thrombolytic therapy should not be given if
    • The neurological deficit is mild
    • The time from onset is more than three hours
    • There is evidence of major stroke on CT and
    • The patient has uncontrolled hyperglycaemia more than 25 mmol/L
    • Other contraindications include
    • PT should be less than 15s
    • Platelet count less than 100
    • BP more than 185/110 mmHg.
    • Aspirin is indicated for acute ischemic stroke in patients who are not eligible for rtPA,
    • Aspirin (160 to 325 mg/d) administered within 48 hours of stroke onset results in a small but significant reduction in the risk for recurrent stroke during the first 2 weeks after the stroke and improves outcome at 6 months.
    • For patients with acute stroke who receive thrombolysis, aspirin should be withheld in the emergency department and for 24 hours after rtPA administration.
    • Although long-term anticoagulation is an effective treatment for prevention of cardioembolic stroke in patients with atrial fibrillation, acute anticoagulation with heparin has not been shown to be beneficial in patients with acute ischemic stroke.
    • Intra-arterial administration of rtPA is indicated for selected patients with acute stroke who have major intracranial artery occlusion within 6 hours of symptom onset and who are not otherwise candidates for intravenous administration of rtPA.
    • In a patient with stroke, dysphagia screening "water swallow test" should be performed before food, oral medication, or liquids are administered. a patient with stroke should be given nothing by mouth (kept NPO) until a swallowing assessment is conducted.
    • Elevated blood pressure is common at the time of initial stroke presentation, even among patients without chronic hypertension.
    • Rapid lowering of blood pressure may further impair cerebral blood flow and worsen the ischemic injury.
    • Elevated blood pressure often will resolve spontaneously or improve gradually during the first few days after a stroke.
    • The threshold for acute blood pressure lowering in patients with acute stroke who are eligible for thrombolysis is 185/110 mm Hg. In such a setting, preferred agents include intravenous infusions of labetalol or nicardipine.
    • The PROGRESS trial showed that BP reduction (using perindopril and indapamide) after "not in acute stage" ischaemic stroke reduces the risk of further events even in patients with ‘normal’ blood pressure.
    • The Heart Protection Study showed that treatment with simvastatin 40 mg reduces the risk of stroke and other vascular events in high-risk patients (previous ischaemic stroke, coronary or peripheral vascular disease, diabetes) even if the cholesterol level is ‘normal’ <3.5mmol/l
    • Carotid endarterectomy is the gold standard of surgical therapies for patients with Internal "not External" carotid stenosis 50-70% , it cannot be performed in
    o stenosis that is difficult to access surgically (above the C2 level),
    o medical conditions that greatly increase the risk of surgery, or
    o other specific conditions, such as
     radiation-induced stenosis or
     restenosis after carotid endarterectomy.
    • Recent NICE guidance suggests that in people with acute non-disabling stroke or TIA, who have symptomatic carotid stenosis of 50-99% , should be considered for endarterectomy
    • Combination of carotid angioplasty and stenting is alternative to Carotid endarterectomy in above conditions

    Cerebral venous sinus thrombosis
    • signs and symptoms of intracranial hypertension, such as
    o headache,
    o Bilateral papilledema
    o visual problems; homonymous hemianopsia,
    o focal neurologic findings as unilateral hemiparesis
    o seizures , fluent aphasia
    • mental status changes, stupor, and coma.
    • Major risk factors
    o thrombophilia,
    o pregnancy,
    o oral contraceptive use,
    o Dehydration
    o malignancy,
    o sepsis, and
    o head trauma.
    • The ‘absent delta sign’ on CT scan implies a filling defect in one of the venous sinuses, although often in this condition the CT scan is normal.
    • Magnetic resonance venography can readily detect obstruction of the venous sinuses by a thrombus and the damage to the brain caused by the resultant increased pressure
    • Treatment anticoagulation + Rehydration if dehydrated
    Chiari malformation as a cause of syncope
    • central nervous system syncope due to intermittent obstructive hydrocephalus.
    • This presents with sudden pressure headache which builds up over a few seconds before losing consciousness.
    Signs and symptoms of subdural hematoma
    • A history of recent head injury
    • Loss of consciousness or fluctuating levels of consciousness
    • Irritability
    • Seizures
    • Pain
    • Numbness
    • Headache (either constant or fluctuating)
    • Dizziness
    • Disorientation
    • Amnesia
    • Weakness or lethargy
    • Nausea or vomiting
    • Loss of appetite
    • Personality changes
    • Inability to speak or slurred speech
    • Ataxia, or difficulty walking
    • Altered breathing patterns
    • Hearing loss or ringing ringing
    • Blurred Vision
    • Deviated gaze, or abnormal movement of the eyes
    • Neurologic examination may demonstrate
    o mental status changes,
    o hemiparesis,
    o papilledema,
    o hyperreflexia or reflex asymmetry,
    o hemianopsia, or
    o third or sixth cranial nerve dysfunction.
    Hereditary haemorrhagic telangiectasia
    • Presents with epistaxis, and
    • Arteriovenous malformations
    o brain "stroke"
    o lung "hemoptysis"
    Do not confuse HHT with Wegener's granulomatosis which also present with epistaxis, hemoptysis but NO stroke as Neurological involovement is occasionally sensory neuropathy (10%) and rarely mononeuritis multiplex
    Nystagmus Nystagmus duration Imbalance Vomiting Hearing Tinnitus
    Peripheral Severe Nystagmus
    Horizontal, torsional Never vertical inhibited by fixation does not change direction with gaze to either side Usually< 1 minute
    fades after a few days
    Mild to moderate able to walk
    DixHallpike Latency of symptoms 15 seconds May be severe

    Central Mild Nystagmus
    vertical, horizontal, or torsional not inhibited by fixation of eyes may change direction with gaze Usually>1 minute
    may last weeks to months Severe unable to stand
    DixHallpikeNo Latency of symptoms Variable Rare
    NO Tinnitus

    Disease Type Duration Auditory symptoms
    Benign paroxysmal positional vertigo Peripheral
    Repositioning procedure Epley manoeuvre Medications not necessary
    Vestibular neuronitis Peripheral Days
    No Vestibular suppressant medication

    Labyrinthitis Peripheral Days Yes
    Meniere’s disease Peripheral Hours Sensitivity to noises
    Yes Sense of pressure in the ear low-salt diet and diuretics

    Acoustic neuroma
    Peripheral months
    Perilymphatic fistula
    Peripheral Seconds
    Hallpike manoeuvre
    • is the standard clinical test for benign positional vertigo.
    • The finding of classic rotatory nystagmus with latency and limited duration is considered pathognomonic.
    • A negative test result is meaningless except to indicate that active canalithiasis is not present at that moment.
    • If no nystagmus is observed, the procedure is then repeated on the other side.
    • Long-term medical therapy is not the treatment of choice as
    • Epley manoeuvre “Canalith repositioningâ€, carries a high chance of relieving symptoms. Most practitioners claim symptom relief in 97% of patients after two treatments.

    Down-beat jerk nystagmus
    • is a rarity caused by lesions around the foramen magnum (e.g. meningioma,cerebellar ectopia).

    • MRI is superior to CT for detection of epileptogenic lesions.
    • CT is the imaging modality of choice for new-onset seizures only when there is a suspicion of an acute cerebral hemorrhage or when a contraindication to MRI is present
    • Continuous EEG monitoring is indicated for patients who remain unresponsive after resolution of clinical status epilepticus to distinguish nonconvulsive status epilepticus from a postictal state.
    • Risk of recurrence after a first unprovoked seizure
    o partial seizure
    o Todd paralysis,
    o status epilepticus on presentation,
    o age greater than 65 years,
    o abnormal findings on neurologic examination.
    • Juvenile myoclonic epilepsy,,, Lifelong drug treatment is usually necessary to avoid relapses in patients who achieve seizure-free status on medication.
    • Juvenile myoclonic epilepsy linked to chromosome 6
    • Lamotrigine is an alternate option to carbamazepine if a woman is considering starting a family, as the risk of teratogenicity is less compared to carbamazepine
    • Levetiracetam, gabapentin, and pregabalin
    o are the preferred choices in patients with significant liver disease
    o are renally excreted, the dosage may need to be lowered in the presence of renal insufficiency.
    o are preferred for those patients patients who have undergone organ transplantation because of the lack of significant drug interactions
    Video EEG is the gold standard investigation used to differentiate non-epileptic attacks from epileptiform events

    Epilepsy in elderly
    • Elderly patients may be particularly sensitive to the cognitive, motor, and coordination side effects of phenytoin, even if the serum phenytoin level is in the therapeutic range.
    • Gabapentin, lamotrigine, and carbamazepine are equally effective at controlling partial onset seizures in the elderly, but gabapentin and lamotrigine are better tolerated.
    Discontinuing antiepileptics
    • Those with no structural brain lesion,
    • no epileptiform or focal abnormalities on EEG,
    • a sustained seizure-free period "2 years"
    • no abnormalities on neurologic examination.
    Factors that have been shown to increase the risk of seizure recurrence on discontinuation of therapy include
    • older age,
    • use of multiple anticonvulsants
    • Seizures while on therapy
    • history of myoclonic or tonic clonic seizure,
    • previous abnormal imaging
    • previous abnormal EEG.
    Partial seizure with secondary generalization.
    • This type of seizure is characterized by sensory, motor, autonomic, or psychic (déjà vu, depersonalization) phenomena with or without altered awareness at onset followed by generalized tonic-clonic activity

    Nonconvulsive status epilepticus
    • a range of conditions in which electrographic seizure activity is prolonged and results in nonconvulsive clinical symptoms.
    • Arrange 24 hour EEG monitoring
    • intravenous lorazepam
    Pseudoseizure (non-epileptic attack).
    • Violent shaking, resistance to passive eye opening and normal vital signs
    • rapid post-ictal recovery,
    • attacks in public places and lack of injury.
    • Urinary incontinence can occur
    • tongue biting is rare.
    • Suddenly becoming anxious, breathing fast then suddenly dropping to the floor
    • Past medical history of anxiety and depression
    • Simple observation is the appropriate management in this case.
    • Pseudoseizures are commonly misdiagnosed as true seizures and treated inappropriately with anti-epileptic drugs.

    MERRF (myoclonic epilepsy and red ragged fibres).
    • generalised myoclonus (60%),
    • ataxia,
    • cognitive decline
    • encephalopathy (EEG findings of generalised slow waves)
    • epilepsy.
    • short stature (10%),
    • optic atrophy (20%),
    • sensorineural hearing loss
    • impaired glucose tolerance.
    • CSF lactate increased
    Multiple sclerosis
    • If facial nerve palsy occur it is of UMNL not LMNL,,,so Bells palsy is against MS
    • Oligoclonal bands in CSF may be negative in multiple sclerosis and may be positive in other diseases,,,It is important to remember that if they are detected it needs to be established whether or not they are present in the serum, as they may reflect systemic and not central nervous system inflammation. It is for this reason that a serum sample must be taken when cerebrospinal fluid (CSF) is sent for oligoclonal band analysis
    • Lhermitte's sign, an “electric shockâ€â€“like sensation down the neck, back, or extremities occurring with neck flexion, is a helpful clue to a cervical spinal cord disorder.occure in MS and other diseases as Cervical spondylotic myelopathy
    • Primary progressive multiple sclerosis (MS); no treatments have been shown to affect the disease course. Therefore, Physical therapy for evaluation and treatment of spasticity and back pain are most appropriate
    • Beta interferons, glatiramer acetate, and natalizumab, are only appropriate for relapsing-remitting disease.
    • Three or more relapses per year preclude any continuation of the interferons or glatiramer acetate injections (as the objective behind using them is to reduce relapse frequency).
    • Mitoxantrone therapy is of modest benefit in slowing progression of secondary progressive or severe relapsing–remitting multiple sclerosis.
    • The primary concern about mitoxantrone therapy is the risk for cardiotoxicity.
    • Women taking immunomodulatory treatment for multiple sclerosis should use effective contraception, or if they want to become pregnant, stop therapy several months before attempting to conceive.
    • Relapses resolve more rapidly with intravenous methylprednisolone therapy than oral prednisolone
    • Plasma exchange therapy in attacks of multiple sclerosis that are unresponsive to corticosteroids
    • Amantadine reduce MS-related fatigue.

    • Neuromyelitis optica
    o Transverse myelitis " severe paraparesis".
    o optic neuritis.
    o often associated with serum autoantibodies or other autoimmune diseases, and has a predilection for the optic nerves and spinal cord with relative sparing of the brain
    o can be distinguished from MS by the NMO-IgG autoantibody test
    Criteria for commencing beta-interferon therapy.
    • more than two separate episodes within the last two years
    • more than 18-years-old, and
    • Can walk more than 100metres
    Contraindications to beta-interferon are
    • History of severe clinical depression
    • Uncontrolled epilepsy
    • Hepatic dysfunction and
    • Myelosupression.
    Prognosis of multiple sclerosis
    • Benign
    o Onset with optic neuritis;
    o one year or more duration of first remission;
    o onset before the age of 40 years.
    o There was no association with sex.
    • Poor
    o Onset with pyramidal symptoms
    o extent of white matter lesions on MRI and subsequent clinical deterioration is well documented.
    Optic neuritis
    • In acute ON, the optic disc may appear normal since two thirds of cases of ON are retrobulbar. However, the optic nerve may become pale over time.
    • In case of ON without any clinical signs and symptoms of a systemic disease ,,, blood tests and CSF examination are of little benefit.
    • MRI can be used to assess inflammatory changes in the optic nerves and helps exclude structural lesions.
    • Intravenous methylprednisolone therapy followed by an oral prednisone taper speeds recovery of visual acuity in optic neuritis.
    Acute disseminated encephalomyelitis is usually readily distinguishable from multiple sclerosis (MS) by the presence of certain clinical features, including the following:
    o History of preceding infectious illness or immunization
    o Association with constitutional symptoms and signs such as fever
    o Prominence of cortical signs such as mental status changes and seizures
    o Comparative rarity of posterior column abnormalities, which are common in MS
    Benign fasciculations
    • are relatively common in patients who have undergone a period of very strenuous exercise, and are also seen after an alcohol binge.
    • the prognosis of this condition is entirely benign.
    • Benzodiazepines are generally ineffective in the management of benign fasciculations, but carbamazepine and phenytoin are effective

    Parkinson's disease
    • The presence of these suggest alternative condition other than Parkinson disease
    o symmetric signs and symptoms (tremor and rigidity)
    o postural tremor
    o early falls,
    o rapid progression,
    o poor or waning levodopa response,
    o dementia,
    o early autonomic failure
    o ataxia.
    • Pramipexole is a non-ergoline dopamine agonist indicated for treating early-stage Parkinson's disease and restless legs syndrome
    • A potential adverse effect of dopamine agonist therapy is the development of compulsive behaviors, such as pathologic gambling, shopping, and hypersexuality.

    Corticobasal degeneration
    • is a cause of adult-onset parkinsonism,‘Parkinson-plus’
    • usually unilateral initially but can progress to affect both sides.
    • rigidity and bradykinesia,
    • there may also be myoclonus,
    • apraxia or a
    • dystonia of a limb.
    • A particularly characteristic feature of CBD is the development of an ‘alien limb’ phenomenon, where a limb, usually an arm, appears to move on its own.
    • Cortical sensory loss is also seen
    • there is sometimes a supranuclear gaze palsy.
    • cognitive impairment that may include a progressive non-fluent aphasia. difficulties with speech ,,had some word-finding difficulties and his speech was at times effortful with the occasional wrong word
    • There is no curative treatment

    Disease Falls Autonomic Ataxia Tremor Other
    Parkinson NO NO NO Common Asymmetrical stiffness
    Multiple system atrophy Early Common postural hypotension impotence Common cerebellar NO Shy-Drager syndrome increased tone brisk reflexes Cerebellar sign intention tremor ataxic dysarthria. bradykinesia
    Progressive supranuclear palsy Early
    Repeated abrupt falling without any evidence of ataxia NO
    NO postural hypotension
    NO NO
    MRI may show midbrain atrophy Impaired down gaze limited upgaze Blepharospasm and involuntary eye closure Pseudobulbar palsy slurred speech, impaired swallowing. symmetrical stiffness Initially there are vague changes in personality
    Lewy bodies Dementia yes Less "postural hypotension" NO NO Visual hallucination Cognitive impairment Neurolyptic sensitivity

    Multiple system atrophy
    • Neuroimaging may be normal in MSA.
    • Hyperintensity of the lateral margin of the putamen in T2-weighted MRI is a characteristic
    • MRI can help to exclude cerebrovascular diseases.
    • Fluoride F 18 fluordeoxyglucose dopa positron emission tomography (PET) imaging can be used to differentiate between MSA and Parkinson’s disease, with reduced putaminal F 18 fluorodeoxyglucose in the MSA.
    • Autonomic function testing is useful in evaluation of the distribution and severity of parasympathetic and sympathetic function.
    • Sphincter electromyography is useful for demonstrating hyperreflexia of detrusor in MSA

    Dementia with Lewy bodies
    • fluctuating encephalopathy,
    • parkinsonism,
    • visual hallucinations
    • Deterioration with haloperidole,,,, haloperidole also deteriorate Parkinson disease
    • Affected patients have a central cholinergic deficiency. Cholinergic augmentation with a centrally acting anticholinesterase agent such as donepezil, galantamine, or rivastigmine is likely to alleviate the inattention, hallucinations, and fluctuating encephalopathy.
    • In patients with predominant parkinsonian features, dopamine agonists can be used, although improvement is usually short-lived
    an acetylcholinesterase inhibitor, may cause mild peripheral cholinergic side effects, including increased vagal tone, with bradycardia, and occasionally atrioventricular block.

    Creutzfeldt–Jakob disease
    • Rapidly progression of dementia to memory loss,
    • personality changes and hallucinations
    • with myoclonus
    • motor signs Limb weakness and rigidity
    • dysphasia,
    • Cerebellar signs ataxia Nystagmus
    • rigid posture,
    • seizures
    • EEG showing periodic sharp waves in two-thirds of patients
    • A positive CSF 14-3-3 protein
    • MRI. Normal or can show a characteristic signal change in the putamen and caudate. MRI is vital in excluding other diagnoses, in comparison to the findings in variant CJD in which increased signal in the pulvinar may be seen
    Variant Creutzfeld–Jakob disease
    • Presents with behavioural and psychiatric disturbances
    • Rapid cognitive decline in a young person with
    • myoclonic jerks
    • cerebellar ataxia
    • Cerebrospinal fluid (CSF) may reveal elevated 14-3-3 proteins.
    • All cases to date are homozygous for methionine at PRNP codon 129.
    • This disease can be diagnosed by detection of characteristic PrP immunostaining and PrPpc on tonsil biopsy demonstrated by western blot.
    • The tonsil biopsy obviates the need for brain biopsy.
    Hashimoto’s encephalopathy
    • subacute diffuse encephalopathy
    • neuropsychiatric signs (confusion, apathy, memory deficit),
    • myoclonic encephalopathy,
    • movement disorder (akineto-rigid extrapyramidal signs), and
    • seizures.
    • positive antithyroid autoantibodies comparable with Hashimoto’s thyroiditis, typically with a euthyroid state.
    • EEG is abnormal in most,
    • other etiologies such as infectious, metabolic, toxic, vascular, neoplastic, and paraneoplastic causes need to be considered.
    • Women appear to be more affected than men and the condition is usually steroid responsive.

    Frontotemporal dementia
    • The age of onset is normally between 45 and 60 years
    • a positive family history in up to 50%
    • There are three cardinal syndromes:
    o Frontal variant frontotemporal dementia which presents mainly with behavioural disturbance (decreased motivation, impulsiveness, disinhibition and loss of social skills are all seen);
    o Semantic dementia which presents with a fluent aphasia and difficulty naming objects due to a loss of semantic knowledge
    o Progressive non-fluent aphasia which presents with speech production difficulties.
    • Overlap between the syndromes is seen.
    • Patients often develop a sweet tooth or other changes in appetite.
    • Problems with planning and judgment may cause them to lose their job.
    • There is currently no treatment for the underlying condition.
    Radiation-induced leukoencephalopathy
    • is a subcortical process affecting white matter and
    • characterized by the triad of
    o gait apraxia,
    o dementia, and
    o urinary incontinence.
    • may occur months to years after radiation and
    • is more common after whole-brain compared with focal brain irradiation.

    • Huntington's disease,
    • Wilson's disease
    • drugs (neuroleptic agents, levodopa, dopamine agonists, anticholinergic agents),
    • hypothyroidism
    • infections (Sydenham's chorea),
    • systemic lupus erythematosus
    • antiphospholipid
    • This is a serologic blood test used to detect antibodies against antideoxyribonuclease B ,,is produced by group A streptococci.
    • This test is used to document a previous streptococcal infection in those who are suspected of having post-streptococcal disease (for example acute glomerulonephritis, rheumatic fever).

    Anti-basal ganglia antibodies
    • appears to be a potentially useful diagnostic marker in post-streptococcal neurological disorders
    Westphal variant of Huntington’s disease
    • presentation in youth
    • with associated Parkinsonian symptoms.
    • a sudden decline in academic performance, changes in handwriting,
    • behavioral problems,
    • seizures
    • brief, "shock-like" spasms of certain muscles or muscle groups (myoclonus); progressively impaired control of voluntary movement (ataxia); and dystonia
    • The family history of suicide is of relevance
    • may be associated with behavioural features of the disease
    • HD is inherited in an autosomal dominant pattern with complete penetrance.
    • CT scan of brain show Gross atrophy of the caudate nucleus and putamen on both sides is the characteristic
    • The gene for HD is on the short arm of chromosome 4, which contains CAG repeats (coding for glutamine).
    Wilson disease and Neuropsychiatric complications
    • Most patients have cirrhosis.
    • The most common presenting neurologic feature is asymmetric tremor
    o parkinsonian
    o pseudosclerotic group had tremor resembling multiple sclerosis.
    o Dystonic,,,hypertonicity associated with abnormal limb movements.
    o chorea associated with dystonia.
    • Psychiatric features include
    o Depression, poor concentration and "personality change"
    o emotional lability, to cry very easily
    o disinhibition, and
    o self-injurious behavior
    o Behavioral
    o Affective
    o Schizophrenic like
    o Cognitive
    • Kayser-Fleischer rings are observed in up to 90% of individuals with symptomatic Wilson disease and are almost invariably present in those with neurologic manifestations.
    Restless legs syndrome
    • consists of abnormal sensations in the legs and restlessness relieved by movement.
    • check serum iron levels. Oral iron therapy can alleviate symptoms and is recommended if serum ferritin levels are lower than 45 to 50 ng/mL (45 to 50 μg/L).
    • treated with dopamine agonists,
    • unilateral involuntary flinging movements of the proximal upper limbs.
    • The lesion is in the contralateral subthalamic nucleus of Luys.
    • In the elderly the usual cause is a vascular event such as a stroke,
    • whereas in younger patients the aetiology may be infectious or inflammatory.
    • Hemiballismus patients require treatment both for the underlying aetiology and for the movements themselves.
    • anti-ballismus therapy.
    o Dopamine receptor blocking agents have been shown to suppress choreic and ballistic movements.
    o neuroleptic drugs such as haloperidol are started at low doses and titrated as tolerated until the movements are controlled.
    o Catecholamine-depleting agents such as tetrabenazine may be considered when long-term therapy is required.
    Tourette’s syndrome
    • multiple tics, including sniffing, snorting, involuntary vocalisations. Some patients display repetitive and annoying motor behaviour.
    • An attention deficit hyperactivity disorder and obsessive–compulsive traits are associated
    • Intelligence does not deteriorate.
    • Treatment Dopamine receptor blocker"D2" like haloperidol is an effective treatment
    • Streptococcal infection has been implicated

    Essential tremor
    • First-line medications used to treat essential tremor include propranolol, primidone, gabapentin, and topiramate
    • Clonazepam is considered a second-line
    Friedrich’s ataxia
    • autosomal recessive
    • mentation is normal.
    • progressive spinocerebellar ataxia
    • areflexia
    • loss of vibration sense
    • bilateral extensor plantars indicate pyramidal tract involvement.
    • dysarthric speech,
    • bilateral optic atrophy.
    • Progressive muscular wasting and weakness
    • Sensorineural deafness,
    • oculomotor disturbance in the form of jerky pursuit,
    • retinitis pigmentosa.
    • cardiomyopathy,
    • diabetes mellitus,
    • pes cavus,
    • equinovarus deformity and scoliosis
    • Nerve conduction study reveals either
    o low amplitude or absent sensory nerve action potentials.
    o Motor nerve conduction velocities are usually normal.
    Alzheimer dementia
    • Speech dysfunction
    • visuospatial skills difficulties.
    • The presenilin-1 mutation is specific for early-onset familial Alzheimer dementia. autosomal dominant mutation
    • Frontotemporal dementia and Alzheimer dementia are sometimes difficult to distinguish on clinical grounds alone
    • Rivastigmine is licensed for the treatment of both mild to moderate dementia of Alzheimer type, and mild to moderate dementia associated with Parkinson’s disease.
    NICE guidance for treatment of Alzheimer's disease (AD).
    • Treatment with drugs for dementia should be initiated in mild to moderate AD whose MMSE is between 10-20 and supervised only by a specialist experienced in the management of dementia.
    • Repeating the cognitive assessment every six months assesses benefit.
    • Up to half the patients given these drugs will show a slower rate of cognitive decline.
    • The drug should be discontinued in those thought not to be responding.
    • In those who are responding, drugs should be stopped once the MMSE score is less than 10.
    • Memantine is not recomended as a treatment option for patients with moderately severe to severe AD except as part of a clinical trial.
    • memantine for moderately severe to severe Alzheimer’s disease. NICE Guide

    Pick's disease
    • Frontal lobe dementia
    • progressive changes of personality and behaviour.
    • Aggressiveness
    • Apathy and depression,, sometimes laughing or crying inappropriately.
    • Appetite had increased,,, developed a ‘sweet-tooth’ and ate little else except boiled sweets
    • disinhibited and was easily distractible with marked perseveration.
    • deficiencies in attention and calculation and naming.
    • 40–50% of cases have an affected family member.
    • A subgroup of patients with frontotemporal dementia have symptoms and signs of motor neurone disease.
    o impaired swallow,
    o speech has a nasal quality
    o an impaired swallow with tongue fasciculations
    o proximal limb weakness difficulty getting out of his chair and fasciculations
    • Memory and speech are usually intact.
    • Visuospatial function was preserved Performance in an MMSE is often surprisingly high, and more formal neuropsychological testing may be required to establish the typical cognitive deficits
    • Neuroimaging usually shows a unilateral atrophy of the frontal lobe.
    • There is no effective treatment at present.

    Binswanger's disease
    • "Subcortical arteriosclerotic encephalopathy"
    • is a form of multi-infarct dementia caused by damage to the white brain matter.
    • The average age of onset is between the fourth and seventh decades of life, and 80% of patients have a history of hypertension.
    • This disease is characterized by loss of memory and intellectual function and by changes in mood
    • Patients also show progressive motor, cognitive, mood, and behavioral changes over a period of 5-10 years.
    • early-onset urinary incontinence
    • gait disturbances
    • Patients may be apathetic or abulic.
    Tumour at the foramen of Monro
    • Headache
    • Nausea
    • Deterioration in his mental capacity
    • marked perseveration.
    • Ataxic gait
    • Bilateral papilloedema.
    • Ocular movements were full and the rest of the cranial nerves were normal.
    • bilateral palmomental reflexes,
    • tone, power and reflexes were all intact.
    • There is obstruction between the lateral ventricles and third ventricle, A CT scan of the brain showed dilated lateral ventricles with normal-sized third and fourth ventricles.

    Normal pressure hydrocephalus
    • The gait "Parkinsonism"
    o Apraxia falls
    o bradykinetic,
    o broad based,
    o shuffling.
    • The urinary symptoms
    o urinary frequency,
    o urgency, or
    o frank incontinence. occurs late in the disease process compared to Binswanger's disease where incontinence is a relatively early feature.
    • The dementia
    o prominent memory loss
    o Deterioration in concentration and immediate memory
    o Frontal and subcortical deficits
     marked perseveration
     deficiencies in executive function and naming
     bilateral palmomental reflexes
     forgetfulness,
     decreased attention, inertia, and bradyphrenia.
    • CT scan of the brain showed dilatation of all the ventricles.
    • The presence of cortical signs such as aphasia or agnosia should raise suspicion for an alternate pathology such as Alzheimer disease or vascular dementia.
    Fronto-temporal dementia
    • gait ataxia is extremely unusual in fronto-temporal dementia

    MELAS Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke ,,,Early symptoms may include
    • muscle weakness and pain,
    • Recurrent headaches, migraine , fever and confusion
    • loss of appetite, vomiting, and
    • seizures.
    • extrapyramidal movements,
    • autonomic dysfunction
    • Most affected individuals experience stroke-like episodes beginning before age 40.
    • Hemiparesis reflexes are brisk and the plantar response was extensor.
    • Vision loss
    • dementia
    • Glucose intolerance Diabetes
    • short stature,
    • proximal myopathy, progressive weakness in legs proximal muscle wasting of the thighs and arms
    • MRI scan brain shows multiple cortical lesions and have predominance for the occipital lobes.
    • May start with attacks of migraine with aura or
    • Subcortical transient ischemic attacks or
    • Strokes and upgoing planter
    • Mood disorders between 35 to 55 years of age.
    • The disease progresses to subcortical dementia associated with pseudobulbar palsy
    • Depression
    • urinary incontinence.
    • Leukoencephalopathy on MRI.
    • DNA testing for the notch-3 gene mutation confirms the diagnosis
    • CADASIL can be differentiated from MELAS by
    o the absence of extra neurological features,
    o normal lactate
    o rareness of cortical lesions on MRI imaging.
    Whipple’s disease
    • caused by a Gram-positive bacillus, which resides predominantly in the gut.
    • Hyperpigmentation and lymphadenopathy are usual manifestations.
    • Neurological manifestations include
    o slowly progressive memory loss and
    o supranuclear ophthalmoplegia where superior gaze is restricted Bell’s phenomenon is elicitable.
    o Nystagmus,
    o ataxia,
    o myoclonus and
    o seizures and
    o highly characteristic oculomasticatory movements complete the clinical picture.
    • MRI of the brain may or may not reveal enhancing focal lesions.
    • The diagnosis is made mainly by PAS staining of
    o an intestinal (jejunal) biopsy
    o PAS-positive material in the CSF.
    • Treatment consists of a course of penicillin and streptomycin followed by ceftriaxone or trimethoprim sulphamethoxazole for 1 year.

    Wernicke encephalopathy
    • The typical clinical triad of ataxia, areflexia, and ophthalmoplegia "lateral gaze nystagmus' is seen in only 19%
    • Nystagmus "Horizontal"
    • ophthalmoplegia, especially of the lateral rectus muscles impaired horizontal eye movements with preserved pupillary responses
    • absence of any upper motor neurone focal signs peripherally.
    • gait ataxia,
    • confusion
    • Stupor and coma
    • hypotension and
    • hypothermia
    • Conditions associated with Wernicke encephalopathy include
    o AIDS,
    o alcohol abuse,
    o cancer,
    o hyperemesis gravidarum,
    o prolonged total parenteral nutrition,
    o postsurgical status (particularly gastric bypass), and
    o glucose loading (in a predisposed patient).
    • The diagnosis remains a clinical one in patients with one or more of the classic features of mental status change, gait impairment, and ocular signs.
    • Measurements of serum thiamine level and erythrocyte transketolase activity lack specificity
    • treatment with intravenous administration of thiamine
    Wernicke's encephalopathy

    Korsakoff's amnestic syndrome
    • Recent onset of memory difficulties,
    • mild impairment of attention and concentration
    • lack of ability to reason
    • Confabulation It consists of the creation of false memories, perceptions, or beliefs about the self or the environment
    Peripheral neuropathy
    • A history of burning or lancinating distal extremity pain and examination findings showing only sensory loss suggest a small-fiber peripheral neuropathy, which is most frequently associated with diabetes mellitus and impaired glucose tolerance.
    Carpal tunnel syndrome
    • Mild to moderate symptoms a trial of conservative therapy using wrist splints is indicated.
    • Patients with mild to moderate symptoms who do not respond to wrist splinting should be considered for corticosteroid injection.The initial response rate to corticosteroid injections at or near the wrist is 70%. The duration of response is quite variable, however, and patients often require repeat injections or, ultimately, carpal tunnel surgery.
    • Gabapentin and other medications used to treat neuropathic pain are typically not indicated in carpal tunnel syndrome.
    • Carpal tunnel surgery should be considered in patients who have not responded to conservative therapy and in patients with moderate to severe signs or symptoms
    Multifocal motor neuropathy (MMN)
    • is an autoimmune neuropathy associated in 80% of cases with an elevated anti-GM1 ganglioside antibody
    • slowly progressive, asymmetric motor, predominantly distal weakness developing over years in isolated limb.
    • Fasciculation
    • Depressed reflexes
    • Weakness usually starts in a distribution of a single peripheral nerve with unilateral wrist drop, foot drop, or grip weakness
    • without sensory involvement.
    • Cranial nerves are rarely affected.
    • It is an important differential diagnosis of motor neurone disease as it is potentially treatable.
    • Treatment with IV immunoglobulin may be very effective in some patients. Cyclophosphamide is also used on a long term basis.
    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
    • chronically progressive or relapsing symmetric sensorimotor disorder duration of symptoms to be greater than 8 weeks
    • weakness, numbness, tingling, pain and difficulty in walking.
    • loss of deep tendon reflexes
    • There may be atrophy of muscles,
    • fasciculations (twitching) and
    • loss of sensation.
    • The patient may present with a single cranial nerve or peripheral nerve dysfunction.
    • Autonomic system dysfunction can occur; orthostatic dizziness, problems with bowel and bladder functions, and cardiac problems.
    • CIDP is clinically similar to Guillain-Barre syndrome except that it follows a chronic progressive course.
    • CSF Show increase protein
    • EMG and NCS
    1. a reduction in nerve conduction velocities b;
    2. the presence of conduction block or abnormal temporal dispersion in at least one motor nerve;
    3. prolonged distal latencies in at least two nerves;
    4. absent F waves or prolonged minimum F wave latencies in at least two motor nerves.
    • Sural nerve biopsy
    • First-line treatment for CIDP is normally either
    o oral prednisolone or
    o intermittent intravenous immunoglobulins:
    o evidence suggests they are of equal efficacy in the short term, and they have not been compared in the long term.
    Fabry disease
    • Small vessel stroke
    • Painful peripheral neuropathy
    • Renal disease
    • Skin stigmata and
    • Myocardial infarction
    Refsum’s disease
    • autosomal recessive
    • sensorimotor peripheral neuropathy.
    • defective alpha oxidation of phytanic acid
    • sensorineural deafness,
    • anosmia,
    • cerebellar ataxia
    • pes cavus.
    • Retinitis pigmentosa cause Night blindness and visual problems
    • Cardiac conduction abnormalities and cardiomyopathies
    • Epiphyseal dysplasia causes shortening of the fourth toe.
    • Serum phytanic acid levels are elevated.
    • Treatment is by dietary restriction of foods containing phytanic acid.
    • Phytanic acid is present in a wide variety of foods including dairy products, fish, beef and lamb

    Vitamin B12 deficiency
    • Neurologic signs of vitamin B12 deficiency may manifest in the absence of hematologic signs of vitamin B12 deficienc
    • The neuropsychiatric manifestations could present without the classical spinal cord and peripheral nerve involvement.
    o Irritability, recently sold his car at an unusually cheap price.
    o become hostile if made to realise his mistakes.
    o awake at night and sleep most of the day.
    o recent memory was impaired.
    o bilateral pale optic discs.
    o Gait was ataxic and mildly spastic
    • SACD has been described in operating room personnel who are exposed chronically to nitrous oxide. Such chronic exposure to the gas leads to the interference with methionine synthetase, a methylcobalamin-dependent enzyme.
    • In SACD, the serum level of B12 is less than 100 pg/ml and increased levels of methyl malonic acid (normal range 73–271 nmol/l) and homocysteine (normal range 5.4–16.2 nmol/l) are more reliable indicators of intracellular cobalamin deficiency.
    • A two-stage Schilling test is another reliable indicator of B12 deficiency but it recently been supplanted by measurement of antibodies to intrinsic factor and parietal cells.
    Treat metastatic epidural spinal cord compression.
    • intravenous corticosteroids followed by
    • decompressive surgery and
    • radiation therapy.
    • There is no role for chemotherapy in the immediate treatment of epidural spinal cord compression.
    Spinal cord compression
    • An urgent MRI spine is essential. The whole spine should be imaged because multiple bone metastases are possible.
    • Immediate high-dose steroids: this has been shown to improve overall outcome by reducing mass effect.
    • An urgent neurosurgical review should also be sought.
    • Surgery is the best option for a single site of metastases
    • Radiotherapy for multiple sites.
    • Further imaging by radio-isotope bone scan will be required to determine whether there are any metastases at other bony sites.
    • Pamidronate is only used to correct hypercalcaemia from malignancies and bone pain secondary to osteoporosis.
    • Chemotherapy would be indicated in chemosensitive tumours such as lymphoma.

    Autonomic dysreflexia
    • is a syndrome of massive imbalanced reflex sympathetic discharge occurring in patients with spinal cord injury (SCI) above the splanchnic sympathetic outflow
    • Hypertension An antihypertensive agent with rapid onset and short duration (nifedipine) is administered for elevated blood pressure.
    • sweating
    • erythema (more likely in upper extremities)
    • headaches and blurred vision.
    • Mortality is rare with AD, but morbidity such as stroke, retinal hemorrhage and pulmonary edema if left untreated can be quite severe.

    Spinal artery infarction
    • symptoms evolve over minutes.
    • acute onset of thoracic back pain,
    • Usually lower limb affected not upper limbs
    • CSF normal
    • Spinal shock
    o flaccid tone bilaterally, paraplegia, with areflexia and absent plantar responses
    o dissociated sensory loss ,,loss of pain and temperature sensation below the level of injury, with relative sparing of position and vibratory sensation
    o bladder

    Central cord syndrome, i.e. syringomyelia,
    • LMN weakness in the arms greater than UMN weakness in the legs, and
    • dissociated pain and temperature loss in the arms.

    Neuralgic amyotrophy "Brachial neuritis'
    • It usually proceeded by viral upper respiratory tract infection.
    • Pain around the shoulder is the presenting symptom that is usually very severe.
    • As the pain starts resolving, weakness begins and usually affects the muscles innervated by the upper brachial plexus (C5-6).
    o biceps and supinator jerk are both depressed
    o Weakness of shoulder abduction and elbow flexion
    o Sensation was reduced to all modalities over the deltoid
    • Treatment is conservative. It is usually a self-limiting condition (improvement over weeks to months).
    Anterior cord syndrome secondary to cervical disc herniation at C5/6
    • In the upper limb there is
    o lower motor neuron (LMN) signs at the level of C5/C6 as evidenced by absent biceps/supinator jerks and weak elbow flexion, and
    o UMN signs below this level (brisk triceps jerk, positive Hoffman's sign, weakness of handgrip, wrist extension, elbow extension).
    o Spinothalamic disruption affecting C6-T1 bilaterally reduced pain and temperature sensation affecting the thumb, middle, ring, little finger and medial forearm bilaterally.
    o Intact dorsal colomn Vibration and light touch were intact
    • In the lower limb there is
    o upper motor neuron (UMN) weakness and
    o evidence of spinothalamic disruption
    C5 radiculopathy secondary to disc protrusion
    • Acute onset pain at the base of the neck and shooting pain affecting the lateral aspect of his arm to the elbow
    • weakness of biceps and deltoid muscles "weakness of shoulder abduction"
    • depression of the biceps and supinator reflexes, which are supplied by C5/C6.
    • triceps reflex appeared intact.
    • diminished sensation over the deltoid, over the upper lateral aspect of the arm
    • some nuchal rigidity on testing neck movements.
    • Lateral disc protrusion
    o Lateral flexion to the left appeared to bring on the left sided shooting pain.
    o Lower limb examination was entirely normal.
    • central disc protrusion
    o leading to cord compression and spastic leg weakness.
    Axillary nerve injury.
    • This nerve arises from the posterior cord of the brachial plexus and supplies the teres minor and deltoid muscles.
    • It may be involved in
    o dislocations of the shoulder joint
    o fractures of the neck of the humerus,
    o serum and vaccine induced neuropathies.
    • diagnosis
    o paralysis of abduction of the arm between 15o and 90o,
    o wasting of the deltoid muscle and
    o slight impairment of sensation over the outer aspect of the shoulder.
    • Treatment is conservative
    Ulnar neuropathy
    • wasting of the dorsal interossei of the right hand,
    • clawing of the right little finger and partial clawing of the ring finger.
    • Tone and reflexes both appeared normal,
    • weakness of adduction and abduction of the fingers
    • Sensory impairment over the palmar and dorsal aspects of the little finger, with the medial aspect of the ring finger not extending beyond the wrist.

    L2, L3 root Lesion:
    – weak hip flexors and quadriceps
    – knee jerks affected
    – radicular pain/paraesthesia over anterior aspect of the thigh, groin and testicle

    L4 root Lesion
    – weak quadriceps, tibialis anterior and posterior
    – knee jerks affected
    – radicular pain/paraesthesia over the anteromedial aspect of the leg

    L5 root Lesion
    – weak hamstrings, peroneus longus, extensors of all toes
    – no tendon reflexes affected
    – radicular pain/paraesthesia over the buttock, posterolateral thigh, anterolateral leg and dorsum of the foot
    S1 sacral root:
    – weak plantar flexors, extensor digitorum brevis, peroneus longus, hamstrings
    – ankle jerks affected
    – radicular pain/paraesthesia over the buttock, back of thigh, calf, lateral border of the foot

    Diabetic Lumbosacral Plexopathy
    • Asymmetrical pain in the hip, buttock, or thigh.
    • Proximal weakness in quadriceps, hip adductors, and iliopsoas muscles is characteristic.
    • Patients may have underlying distal symmetrical polyneuropathy (DSPN).
    • Significant recent weight loss frequently is reported.
    • Proximal lower limb muscle weakness and wasting are characteristic. The patient has particular difficulty getting up from a squatting position.
    • Minimal sensory loss is observed.
    • The knee-jerk reflex is absent, with commonly preserved ankle jerks; however, ankle jerks also may be absent with underlying distal symmetrical polyneuropathy.

    Upper lumbar plexus lesion
    • weakness of hip flexion
    • weakness of adduction of the thigh and extension of the leg
    • with anaesthesia over the anterior thigh and leg.
    Lower plexus lesions
    • weaken the posterior thigh and foot muscles.
    Lesions affecting the entire plexus
    • will affect all muscle groups causing weakness or paralysis of the leg, areflexia and anaesthesia from the toes, to involve the perianal area.

    Sciatic nerve palsy (L4,5,S1,S2,S3)
    • Supplies the hamstrings "back of thigh" and all muscles below the knee
    • weakness of knee flexion " wasting of the hamstrings"
    • No weakness of knee Extension "Knee reflex is normal which is supplied by femoral nerve for quadriceps muscles"
    • Foot droop "failure of ankle dorsiflexion"
    • Loss of ankle reflex
    • Sensory examination reveals reduced sensation to all modalities over the whole of the right lower leg and back of thigh.
    • involvement of thigh adduction, flexion and anaesthesia over the anterior thigh would go against a sciatic nerve palsy
    • Causes
    o trauma or fracture to the hip or pelvis.
    o ill-placed intramuscular injections
    o following hip surgery.
    o Other causes include
     neurofibromas or
     nerve necrosis secondary to infection or diabetes.
    Femoral neuropathy (L2-4)
    • hip weakness, knee extension and anaesthesia over the anterior thigh,
    • but would not explain weakness of the foot.
    In Polymyositis Deep tendon reflexes may be impaired or absent if weakness is severe
    Guillain-Barre syndrome
    • Back pain occurred at rest radiating to both legs
    • Fever should not be present, and if it is, another cause should be suspected.
    • Ascending symmetrical progressive weakness and numbness affecting initially the legs and extending to involve the upper limb.
    • with or without numbness or tingling.
    • bulbar weakness, dysphagia
    • Facial weakness is also commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant
    • Areflexia bilaterally
    • Sensory loss, if present, usually takes the form of loss of position sense
    • Sensory loss to all modalities bilaterally may occur
    • loss of autonomic function is, manifesting as
    o wide fluctuations in blood pressure,
    o resting tachycardia
    o Bladder dysfunction
    • CSF findings
    o Often normal at the onset of symptoms in GBS
    o May be elevated protein level
    o CSF white cell count is usually normal,,,
    o A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.
    o the major importance of a lumbar puncture is to exclude other diagnoses due to infections or neoplasms
    • Nerve conduction studies help in the diagnosis but again can be normal early on in the disease
    • EMG may demonstrate evidence of denervation
    • anti-GM1-ganglioside antibodies: found in 30% and generally reflect worse prognosis
    • The most important aspect of initial management is
    o monitoring of respiratory function
    o monitoring of autonomic function
    • Treatment
    o initially IV immunoglobulin
    o bedside four hourly monitoring of her forced vital capacity.
    o Plasma exchange could be considered if the patient had significant respiratory compromise
    o prophylactic anticoagulation and stockings
    o monitoring for cardiac arrhythmias
    o steroids are of no benefit
    Disease Age presentation Reflex Other Investigation
    Lambert-Eaton proximal upper and lower limb weakness Absent Autonomic neuropathy

    NO ptosis, NO diplopia 1. Antibodies to voltage-gated calcium channel receptors
    2. electrodiagnostic studies
    Myasthenia gravis Normal Normal sensory 1. EMG
    Inclusion body myositis Distal upper and proximal lower limb weakness Normal 1. Muscle biopsy
    2. creatine kinase can be normal
    Guillain-Barré syndrome. Weakness of distal lower extremity, with stocking-glove sensory loss Absent sensory loss 1. EMG demyelinating
    2. CSF

    Pupillary abnormalities do not occur in myasthenia gravis.

    Lambert-Eaton myasthenic syndrome
    • proximal upper and lower limb weakness,
    • Autonomic symptoms occur in up to 75% of patients and include
    o dry mouth,
    o constipation,
    o blurred vision,
    o sluggish pupillary reflexes
    o postural hypotension
    o impaired sweating.
    o erectile dysfunction),
    • proximal muscle weakness (legs greater than arms)
    • absent reflexes that return after brief
  9. Dr albarwari

    Dr albarwari Guest


    Lambert-Eaton myasthenic syndrome
    • proximal upper and lower limb weakness,
    • Autonomic symptoms occur in up to 75% of patients and include
    o dry mouth,
    o constipation,
    o blurred vision,
    o sluggish pupillary reflexes
    o postural hypotension
    o impaired sweating.
    o erectile dysfunction),
    • proximal muscle weakness (legs greater than arms)
    • absent reflexes that return after brief exercise
    • The diagnosis of Lambert-Eaton myasthenic syndrome precedes the clinical diagnosis of cancer in up to 50% of affected patients
    • Nerve conduction test Decremental response at lower rates of stimulation after repetitive nerve stimulation and incremental response at higher rates of stimulation
    • Tensilon test may be +ve
    • Voltage-gated calcium channel antibodies are seen in almost 100% of LEMS patients with cancer and in more than 90% of individuals without cancer
    • Management 3,4-diaminopyridine

    Myasthenia gravis crisis
    • Acetylcholine antibodies may be negative
    • dysphagia requiring nasogastric feeding and/or
    • severe respiratory muscle weakness necessitating ventilation.
    • treated with plasmapheresis or IV immunoglobulin.
    Seronegative myasthenia gravis
    • Patients without anti-AChR antibodies
    • have antibodies against muscle-specific kinase (MuSK). MuSK
    • are predominantly female, and respiratory and bulbar muscles are frequently involved.
    • Late onset myasthenia gravis is associated with thymic atrophy as opposed to thymic hyperplasia.
    • The most important factor influencing management in this patient is whether there is impending respiratory muscle weakness.
    • the patient should be initially commenced on pyridostigmine 60 mg QDS.
    • If not responding, prednisolone can be cautiously added in small doses to start with, normally 10-20 mg/day.
    • High dose prednisolone can lead to paradoxical worsening of myasthenia gravis.
    Miller–Fisher syndrome
    • Ophthalmoplegia
    • Ataxia
    • Areflexia
    • Flexor plantar responses
    • no disturbance of consciousness.
    • anti-GQ1b antibodies are often positive.
    • History of viral upper respiratory tract infection

    Bickerstaff’s brainstem encephalitis
    • Ophthalmoplegia
    • Ataxia
    • Hyperreflexia
    • Extensor plantar responses
    • Disturbance of consciousness.
    • anti-GQ1b antibodies are often positive.
    • History of viral upper respiratory tract infection
    • Botulism can be
    o food-borne or
    o 3 Days after wound infection or
    o from intravenous drug injection.
    • A descending flaccid paralysis affecting cranial nerves and then spreads towards the limbs
    o Diplopia
    o Ptosis
    o Facial weakness
    o Dysarthria
    o Dysphagia
    • Reflexes are depressed or absent OR normal
    • sensation is normal
    • Autonomic features particularly
    o Vomiting
    o Abdominal pain
    o paralytic ileus
    o bradycardia
    o Dry mouth
    o fixed dilated pupils
    • CSF is normal in botulism.
    • Diagnosis is by isolating the toxin in the blood, urine or stool.
    • It is a neuromuscular junction disorder and therefore nerve conduction studies and EMG are normal.
    • Botulism can cause a false positive tensilon test result
    • Repetitive nerve stimulation shows incremental responses, which is diagnostic of botulism.
    • Large doses of botulinum antitoxin can be given intravenously, but are ineffective once the toxin has bound to an acetylcholine receptor site.
    • The drug guanidine hydrochloride helps to reduce neuromuscular blockade
    • Treatment
    o Early administration of IV antitoxin may help reverse paralysis.
    o NO role for Intravenous (iv) immunoglobulin
    o should be managed in an ITU setting.
     Supportive therapy with intubation and ventilation,
     respiratory physiotherapy,
     nasogastric tube placement and
     catheterisation
    o Recovery occurs on average between 30 and 100 days after infection;
    o severe cases may require ventilation for many months.

    Lyme disease
    spare the extraocular muscles.

    Myotonia dystrophica
    • Trinucleotide repeats on chromosome 19.
    • Weakness of the muscles of the face temporalis, masseters and sternocleidomastoids), neck, and distal extremities are typically involved.
    • Both hands and wrists were wasted and weak,,, Bilateral foot drop was noticed. Tendon reflexes were hypoactive
    • They are prone to recurrent jaw dislocation.
    • With progressive disease the proximal muscles, bulbar muscles and even the diaphragm are involved.
    • Impotence
    • Respiratory failure
    • Progressive dementia,
    • abnormal immunoglobulins with the risk of increased infections
    • Myotonia is the hallmark and is characterised by inability to quickly relax a contracted muscle.
    • Diagnosis is normally based on
    o clinical features with a
    o characteristic EMG of myotonic discharges.
    o Creatine kinase is generally normal and
    o muscle biopsy is non-specific.
    • The disabling grip myotonia can be treated with phenytoin but should be used carefully as may have cardiac conduction defects.

    Kearns–Sayre syndrome
    • mitochondrial diseases
    • starts before the age of 20 years.
    • progressive external ophthalmoplegia,
    • ptosis,
    • retinitis pigmentosa, increased difficulty seeing at night
    • cardiac conduction defects and a
    • cerebellar syndrome. ataxic gait
    • sensorineural hearing loss
    • endocrine pathology

    Chronic thyroid myopathy (Basedow paraplegia)
    • is a progressive weakness and wasting of skeletal muscles occurring in conjunction with overt or masked hyperthyroidism.
    • The classic signs of hyperthyroidism may or may not be present.
    • This type of muscle disease presents in middle-aged males more often than females.
    • Muscles of the pelvic girdle and thigh are weakened more often, though all muscles are variably affected.
    • Tendon reflexes are brisk.
    • Serum concentration of muscle enzymes is not elevated.
    • EMG reveals polyphasic potentials.
    • With successful treatment of hyperthyroidism, muscle bulk and power return to normal.
    McArdle’s syndrome
    • a type-V glycogen-storage disorder
    • This disorder is characterised by muscle phosphorylase deficiency.
    • Symptoms include exercise-induced muscle pain and stiffness with contractures that subside at rest.
    • Myoglobinuria can occur.
    • In normal patients there is a sustained increase in serum lactate after exertion, but this does not occur in patients with McArdle's.
    • The diagnosis is confirmed by muscle biopsy, which demonstrates subsarcolemmal glycogen deposition and central migration of muscle nuclei.

    Critical illness myopathy.
    • Critical illness refers to the syndrome of sepsis and multiple organ failure.
    • in the intensive care unit,
    o either in patients who have difficulty weaning from mechanical ventilation (up to 30% of patients) or
    o in patients who have developed severe limb weakness during or after recovery from critical illness.
    • Creatine phosphokinase (CPK) is elevated
    • EMG to exclude other conditions, such as Guillain-Barré syndrome, and confirm the diagnosis of critical illness myopathy or neuropathy.
    • EMG reveals myopathic motor unit potentials and fibrillations.
    • Muscle biopsy reveals loss of myosin filaments.
    • Most patients recover after several weeks.
    • Physical and occupational therapy is ideally initiated immediately on recognition of this syndrome.
    • It is important to note that this type of myopathy has not been seen after high-dose corticosteroid treatment for neurological diseases like multiple sclerosis
    Listeria meningoencephalitis
    • Affects mainly older patients (more than 50 years old)
    • immunosuppressed (diabetes, excess alcohol, oncology patients).
    • It produces a mixed picture of
    o meningitis and
    o brain stem encephalitis. fifth, sixth and seventh cranial nerve palsies with bulbar dysfunction
    • The treatment of choice is ampicillin intravenously. Delayed treatment carries a high mortality.

    CSF leucocytosis may occur in
    • Lyme disease
    • Sarcoidosis
    • Behcet's

    Partially treated bacterial meningitis.
    CSF Finding
    Opening pressure 210 mmH20 (NR 50 - 180mm H20)
    CSF protein 0.98 g/L (NR 0.15 - 0.45 g/L)
    CSF white cell count 140 per ml (NR ≤5 cells per ml)
    CSF white cell differential 90% lymphocytes (NR 60 - 70%)
    CSF red cell count 4 per ml (NR 3.8 - 5.6 x 1012/L )
    CSF glucose 3.6 mmol/L (NR 3.3 - 4.4 mmo/L)

    Subdural empyema
    • from an infection in the frontal or ethmoid sinuses, less commonly from middle ear infection.
    • Streptococci (viridans and non-haemolytic) are the most common organisms, followed by Staphylococcus aureus, E.coli, proteus and pseudomonas.
    • Focal lesions may occur as partial seizure "continuous twitching of the hand and fingers".

    Lumbar puncture may reveal:
    Opening pressure 20 cm H2O (5–18)
    Protein 0.85 g/l (0.15–0.45)
    WCC 120 cells/ml (90% lymphocytes)(≤5)
    RCC 3 cells/ml(≤5)
    Glucose 3.4 mmol/l (3.3–4.4)

    • MRI is more dependable at demonstrating a collection of pus and meningeal enhancement than CT scan, and should be arranged urgently.
    • In the meantime, high-dose intravenous (iv) antibiotics and iv lorazepam should be given to treat infection and simple partial seizures.
    TB meningitis
    • Subacute onset with confusion;
    • in a homeless person;alcoholic
    • Brainstem signs
    • associated secondary spinal meningitis;
    • characteristic CSF findings especially the
    o very high protein and
    o very low glucose.
    • Low blood sodium of 119 mmol/l is suggestive of SIADH (secretion of inappropriate antidiuretic hormone).
    • Spinal arachnoiditis
    o weakness and pain of lower limbs
    o retention of urine.
    o spastic paraparesis with sensory levels of different segments eg, T4, T6, T8, L3 defect while T5, T7, L1, L2 Normal
    o confirmed on myelogram (candle guttering appearance) or on MRI
    • an empirical treatment with four antituberculous (eg rifampicin, INH, pyrazinamide and ethambutol) drugs is usually started when diagnosis is suspected on clinical grounds.

    Limbic encephalitis
    • paraneoplastic or
    • autoimmune disease.
    • It presents with
    o a subacute onset of memory impairment,
    o disorientation
    o +/– seizures and
    o psychiatric disturbances (hallucinations, agitation and sleep disturbance).
    o Hypothalamic disease (hyperthermia, weight gain, endocrine dysfunction, hypersomnia)
    • MRI show Medial temporal lobe changes
    • CSF shows
    o raised protein
    o oligoclonal bands
    o 50% have raised WBC.
    • A recently described subgroup of patients has
    o antibodies to voltage-gated potassium channels.
    o 80% have resistant hyponatraemia.
    o only 10% had oligoclonal bands.
    o This group has shown an improvement with immunosuppressive treatment (IVIg, plasma exchange, steroids) – both clinically and with a decrease in the titre of voltage-gated potassium channel antibodies.

    • are often found incidentally during neuroimaging studies.
    • may be asymptomatic or have subtle signs and symptoms.
    • Seizure is the most common presentation and is reported in greater than 50% of symptomatic patients.
    • Meningiomas characteristically appear on CT scans and MRIs
    • Treatment . Older adults with small, asymptomatic tumors can be managed conservatively with observation and serial neuroimaging studies.
    • Surgical resection is the treatment of choice for younger patients with and without symptoms and for symptomatic older adults and is curative when complete.
    • Radiation therapy or stereotactic radiosurgery may benefit patients with unresectable or rare aggressive meningiomas

    Parasagittal meningiomas
    • may produce seizures may be partial "as rhythmic jerking of the limb" or be entirely asymptomatic,
    • Lesions that reach sufficient size may cause spastic paraparesis
    • Upgoing planter bilaterally
    • incontinence.
    • Diagnosis MRI of brain

    Brain metastases
    • are 10 times more common than primary brain tumors,
    • occur in 20% to 40% of adults with cancer.
    • The tumors most likely to metastasize are
    o lung and
    o breast carcinoma and
    o melanoma.
    • Presenting signs and symptoms ,,,local mass effect or increased intracranial pressure; common symptoms include seizure, headache, behavioral changes
    • Contrast-enhanced MRI is the diagnostic modality of choice when brain metastasis is suspected.
    o MRI has a higher sensitivity and specificity than CT scanning
    o It is safer because MRI contrast does not cause nephrotoxicity, and allergies are extremely rare.
    o normal findings on a CT scan do not exclude the presence of a brain tumor, so a follow-up MRI would be required.

    Meningeal carcinomatosis
    • is seen in 5% of cases of adenocarcinoma of the breast, lung, gastrointestinal tract, melanoma, lymphoma etc.
    • The clinical presentation is variable
    o headache,
    o sciatic pain,
    o cauda equina syndrome,
    o multiple cranial nerve palsies,
    o confusional state,
    o seizures,
    o focal neurological deficits and
    o poly-radiculoneuropathy.
    • The diagnosis is established by
    o MRI brain with gadolinium
    o CSF examinations
     identifying tumour cells in CSF using
    o cytospin,
    o millipore filtering and
    o flow cytometry.
     It is important to note that several CSF examinations are usually required using large quantities of CSF,,so if CSF –ve it can be repeated
     CSF opening pressure is increased and
     lymphocytic pleocytosis up to 100 cells/mm3,
     elevation of protein, and
     low glucose are common findings.
    • Prognosis The median duration of survival is 6 months.

    Foster-Kennedy syndrome
    • headaches
    • loss of smell.
    • olfactory meningioma with optic nerve involvement.
    • optic atrophy on one side with
    • contralateral papilloedema
    • due to frontal tumour or tumour within the olfactory bulb compressing the ipsilateral optic nerve and causing raised intracranial pressure.
    Cerebellar pontine angle syndrome
    • Involvement of cranial nerve V, VI, VII, and VIII.
    • cerebellar dysfunction

    Conus medullaris lesion = UMN+LMN
    Cauda equina lesion is a LMN
    Conus Medullaris Syndrome Cauda Equina Syndrome
    Presentation Sudden and bilateral Gradual and unilateral
    Reflexes Knee jerks preserved but ankle jerks affected Both ankle and knee jerks affected
    Radicular pain Less severe More severe
    Low back pain More Less
    Sensory symptoms and signs Numbness tends to be more localized to perianal area; symmetrical and bilateral; sensory dissociation occurs Numbness tends to be more localized to saddle area; asymmetrical, may be unilateral; no sensory dissociation; loss of sensation in specific dermatomes in lower extremities with numbness and paresthesia; possible numbness in pubic area, including glans penis or clitoris
    Motor strength Typically symmetric, hyperreflexic distal paresis of lower limbs that is less marked; fasciculations may be present Asymmetric areflexic paraplegia that is more marked; fasciculations rare; atrophy more common
    Impotence Frequent Less frequent; erectile dysfunction that includes inability to have erection, inability to maintain erection, lack of sensation in pubic area (including glans penis or clitoris), and inability to ejaculate
    Sphincter dysfunction Urinary retention and atonic anal sphincter cause overflow urinary incontinence and fecal incontinence; tend to present early in course of disease Urinary retention; tends to present late in course of disease
    NO incontinence
    Pontine myelinolysis
    may occur in Rapid correcting HypoNatremia acute para- or quadraparesis, dysphagia, dysarthria, diplopia, loss of consciousness, and other neurological symptoms associated with brainstem damage. The patient may experience locked-in syndrome

    Serotonin syndrome as in Moclobemide
    Serotonin toxicity leads to
    • Changes in mental status:
    o Confusion
    o Agitation
    o Hypomania (the ‘happy drunk’ state)
    o drowsiness,
    o myoclonus,
    o Anxiety
    o Coma
    • Systemic changes:
    o Tachycardia
    o Hypertension
    o Nausea
    o Salivation
    o Rhabdomyolysis
    o DIC + Acute renal failure
    o Pyrexia
    • Motor complications:
    o Myoclonus
    o Hyper-reflexia
    o Seizures
    o Inco-ordination
    o Tremor.
    Treatment is supportive and involves
    • removing the precipitating drugs,
    • controlling agitation,
    • administering serotonin antagonists (cyproheptadine or methysergide) and
    • controlling hyperthermia.
    Treatment for hyperthermia includes
    • reducing muscle over-activity via sedation with a benzodiazepine.
    • More severe cases may require muscular paralysis with vecuronium, intubation, and artificial ventilation. Succinylcholine is not recommended for muscular paralysis as it may increase the risk of cardiac dysrhythmia from hyperkalemia associated with rhabdomyolysis.
    • Antipyretic agents are not recommended as the increase in body temperature is due to muscular activity not a hypothalamic temperature set point abnormality

    Selective serotonin reuptake inhibitor (SSRI) discontinuation syndrome
    which typically occurs following abrupt withdrawal of SSRIs.
    Symptoms can occur within days and can last months following withdrawal and then disappear.
    • Psychiatric (anxiety, insomnia, mood lability, vivid dreams)
    • Gastrointestinal (nausea, vomiting) and
    • Neurological (dizziness, headache, paraesthesia, dystonia, tremor)

    Neuroleptic malignant syndrome
    • Causes
    o anti-psychotic neuroleptic medication
    o patients with Parkinson's disease (PD) who suddenly stop their L-dopa treatment The mortality is 15-30% if it is not recognised and treated properly.
    • Presentation
    o confusion,
    o hyperthermia,
    o muscle rigidity "meningism"
    o autonomic dysfunction (dysphagia).
    o unstable blood pressure and profuse sweating
    • Serum creatine phosphokinase may rise up to 60 000 units/l and may lead to myoglobinuria and Rhabdomyolysis + acute renal failure
    • Treatment should be started early when consciousness is first altered and temperature is rising.
    • Bromocriptine 5 mg tid (up to 20 mg tid) will terminate the condition.
    • Intravenous dantrolene should be given if the patient is unable to swallow.

    Upper brachial plexus injury C5, 6, 7
    o idiopathic brachial neuritis,
    o irradiation
    o trauma (arm jerked downward)
    lower brachial plexopathy.C8, T1
    o infiltration by malignant tumor,
    o cervical rib
    o trauma (arm jerked upward)
    o C8/T1 root involvement associated with increasing shoulder pain and an ipsilateral Horner's syndrome

    • cranial nerves are present 50-70% of cases. facial nerve, optic nerve involvement.
    • Seizures (mostly of the tonic-clonic/"grand mal" type)
    • Meningitis headache and nuchal rigidity (
    • granulomas
    • hydrocephalus
    • spinal cord is rare,
    • cauda equina symptoms
    • hypopituitarism: amenorrhoea, diabetes insipidus hypothyroidism or hypocortisolism (deficiency of cortisol).
    Mental and other
    • depression and psychosis.
    • Peripheral neuropathy
    Weber without lateralization Weber lateralizes left Weber lateralizes right
    Rinne both ears AC>BC Normal Sensorineural loss in right Sensorineural loss in left
    Rinne left BC>AC Conductive loss in left Combined loss : conductive and sensorineural loss in left
    Rinne right BC>AC Combined loss : conductive and sensorineural loss in right Conductive loss in right

    Progressive multifocal leukoencephalopathy
    • infection of oligodendrocytes with JC virus, which is a papovavirus found latent in most healthy adults.
    • With CD4 counts of less than 100 this virus becomes active leading to progressive neurological deterioration.
    • Causes
    o neoplasm or
    o a chronic immunodeficiency state.
    o It may also occur in patients receiving immunosuppressive drugs.
    o Natalizumab for multiple sclerosis was shown to have led to this disease
    • Behavioural changes in clear consciousness.
    • There is absence of fever or depressed conscious level, which would be more in keeping with lymphoma, HIV encephalopathy or abscess.
    • Personality changes and intellectual impairment are the initial features that evolve over several days or weeks followed by
    o focal neurological signs
     Hemiparesis,
     ataxia and
     Dysphasia
     cortical blindness, and visual field defects,
     seizures.
    o This is followed by coma and death over 3 to 6 months.
    • A brain biopsy would be the definitive diagnostic test showing asymmetric foci of demyelination and intranuclear inclusions containing the JC virus.
    • There is no effective treatment, but progression can be slowed by initiation of antiretroviral therapy

    Sub-acute sclerosing panencephalitis.
    • It is recognised to be the result of chronic measles infection. a history of measles very early in life followed by 6–8 years of asymptomatic period.
    • Intellectual deterioration "decline in proficiency in school" is followed by Diffuse myoclonic jerks "shock like contractions of shoulder muscles " in association with Focal and generalised seizures and Visual deterioration due to choiroidoretinitis. This is followed by Pyramidal signs, rigidity Finally the patient lies decorticated followed by death.
    • The course of the illness is 1 to 3 years. No effective treatment is available.

    Cerebral malaria
    • symmetrical encephalopathy
    • no focal signs.
    • Three negative blood films are required to exclude this diagnosis.
    • should be considered, even though the patient is taking prophylaxis

    Permanent vegetative state (PVS)
    • is defined as a state of 'wakefulness without awareness'.
    • The patient breathes spontaneously without mechanical support, is haemodynamically stable and has cycles of eye closure and opening that resemble a normal sleeping pattern but the patient is inattentive and unaware of his/her surroundings.
    • Patients may have spontaneous movements (moaning, grunting, teeth grinding, roving eye movements) and may also smile, laugh and cry without any apparent reason.
    • Although there may be eye movement, the eyes do not track a moving object.
    • Patients may respond to painful stimuli and
    • may have myoclonus in response to startling stimuli.
    • Primitive reflexes may be present.
    • Posture may become decorticate and
    • plantar responses are commonly extensor.
    • The condition typically occurs when there is irreversible damage to the cerebral hemispheres but
    • the brain stem remains intact.
    Causes include
    • Head injury
    • Hypoxic injury (cardiac arrest, carbon monoxide poisoning)
    • Stroke
    • Hypoglycaemia
    • Intracranial infection
    • End-stage degenerative brain disease (for example, Alzheimer's).

    Locked-in syndrome
    • patients are aware of themselves and their environment but
    • are unable to respond due to loss of motor and speech function.
    The cause is usually either
    (1) an upper motor neuron lesion of the descending corticospinal tracts in the brainstem (often the pons) below the level of the oculomotor nerve nuclei, (for example, infarction, haemorrhage, tumour, demyelination, head injury, central pontine myelinolysis after hyponatraemia) or
    (2) widespread lower motor neuron disease (for example, polyneuropathy such as Guillain-Barre syndrome).
    Clinically, the patient is unable to speak or move but patients may be able to open their eyes and may blink in an effort to communicate.

    Locked-in syndrome
    • Usually results in quadriplegia and the inability to speak
    • lesions in the Ventral pons "not dorsal"
    • central pontine myelinolysis
    • Basilar artery "not vertebral" occlusion may cause it
    Basilar artery territory cerebrovascular disease
    • is typically associated with a poor prognosis.
    • 40–60% of patients present with motor deficits,
    • 30–60% with dysarthria and speech involvement,
    • 50–75% with nausea, vertigo and vomiting
    • 40% with headaches
    • 20–30% with visual disturbances. This includes
    o abducens nerve palsy,
    o conjugate gaze palsy,
    o internuclear ophthalmoplegia and
    o ocular bobbing.
    • Around 70% of patients presenting with basilar artery territory stroke are hypertensive.
    • Management includes vigorous control of hypertension and antiplatelet agents.

    Phenytoin toxicity typically gives rise to a
    • Cerebellar-like syndrome.
    • Nystagmus is present even in mild toxicity.
    • cause abnormal liver function test
    • Nystagmus is not a feature of sodium valproate toxicity but can cause nausea and ataxia.

    Gradenigo's syndrome "petrous osteitis"
    • 1st 5 + 6
    • Retroorbital pain due to pain in the area supplied by the ophthalmic branch of the trigeminal nerve
    • Ipsilateral paralysis of the abducens nerve
    • otitis media.
    • Other symptoms can include photophobia, excessive lacrimation, fever
    Trigeminal nerve and Abducence palsy 5 + 6
    • The involvement of all the three divisions of the trigeminal nerve suggests that the lesion has either involved the Gasserian ganglion or its roots and since the neighbouring 6th nerve is also affected the latter localisation at the petrous temporal bone is more likely.
    • Lesions like
    o meningiomas,
    o schwannomas,
    o metastasis, and
    o nasopharyngeal carcinoma invading the base of the skull,
    o granulomatous infections or
    o carcinomatous meningitis would be the likely causes.
    Tolosa-Hunt syndrome
    • 3+4+1st 5+6
    • severe and unilateral headaches
    • extraocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves,
    • pain around the sides and back of the eye

    Glomus jugulare tumour
    • Female ,,present between 40 and 70 years of age
    • A combination of 9th, 10th, 11th nerve palsies "jugular foramen syndrome"
    • The patient has also developed pulsatile tinnitus
    • Hearing loss may be conductive or sensorineural; with the latter there may be vertigo
    • Less commonly, the tumours can enlarge sufficiently to cause affect cranial nerves VII and XII.
    • Intracranial extension can produce headache, hydrocephalus and raised intracranial pressure.
    • Otoscopic examination may reveal a characteristic pulsatile reddish-blue mass behind the tympanic membrane.
    • Surgical resection is the treatment of choice, although embolisation and radiotherapy have been used.
    Jugular foramen syndrome
    • dysphonia/hoarseness
    • soft palate dropping
    • deviation of the uvula towards the normal side
    • dysphagia
    • loss of sensory function from the posterior 1/3 of the tongue
    • decrease in the parotid gland secretion
    • loss of gag reflex
    • sternocleidomastoid and trapezius muscles paresis
    • Glomus tumors (most frequently)
    • Meningiomas
    • Schwannomas (Acoustic neuroma)
    • Metastatic tumors located at the cerebellopontine angle
    Cerebellopontine angle lesion 5,6,7,8th palsy
    • Causes acoustic neuroma, glioblastoma, lipoma, vascular malformations, meningiomas, cholesteatomas and hemangiomas.
    • 8th palsy 'buzzing noise' in ear sensorineural deafness Weber's test reveals lateralisation of the sound to the normal ear. Rinne's test reveals air conduction better than bone conduction in both ears,
    Foramen magnum syndrome
    • combination of lower cranial nerve dysfunction
    • associated with long tract signs.
    • There may be an accompanying occipital headache and neck stiffness and,
    • on occasion, obstruction of the cerebrospinal fluid (CSF) drainage can lead to papilloedema.
  10. Dr albarwari

    Dr albarwari Guest

    Great Thanks to aippg forum this forum is amazing wonderful
  11. Drcool

    Drcool Guest

    What about paces everyone?
  12. dr groon , dr albarwari, dr oman can u plz answer this
    and give ur explanations

    A 68. y.o. hispanic lady with advanced cryptogenic cirrhosis came to er with diarrhea, found to have high urea, creatinine, acidotic and differential was ATN vs.Hepatorenal, within 24 hours, pt was anuric. Surgeon placed a right int jugular quinton dialysis catheter, (see cxr) dialysis was started. WIthin 15 min , patient went into rapid afib at 130s and BP dropped. Previoulsy sinus rhythm with preserved LV function. Dialysis stopped and digoxin was given. What are the possible causes of the cardiac arryhtmia
  13. RNA

    RNA Guest

  14. Guest

    Guest Guest

    cause of dysrhythmia>>>>>> cath related????????
  15. sarahpart2

    sarahpart2 Guest

    ny1 got HIV notes please
  16. Dr Hopeful

    Dr Hopeful Guest

    Sarah I thought you passed ???

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