Answers:

 

Q1: Answer D

The sudden occurrence of a partial Brown-Squard syndrome in this young lady should always prompt a search for multiple sclerosis. A compressing metastatic neoplastic tumor (like from a breast cancer) may give a similar picture. Notice the sensory level on lateral aspect of the right arm (C6); an MRI of the cervical cord with gadolinium is the key imaging investigation here.

 

Q2: Answer D

Severe cervical spondylosis is a well-known risk factor for brain stem stroke due to vertebro-basilar ischemia. Although there is no history of hypertension or diabetes or hyperlipidemia, a carotid Doppler study is worthy to assess the status of the carotids. Aspirin should be given to all patients with ischemic stroke unless contraindicated; this patient's clinical picture clearly indicates that the patient can receive this form of therapy; however, his history is present for about 24 hours, which excludes him from receiving thrombolytics. Always look for and control risk factors like smoking, diabetes, hypertension…etc.

 

Q3: Answer C

This woman's new generalized seizure should always prompt a search for hematoma expansion or the development of another hematoma that causes cortical irritation. Needless to say, hypo or hypernateremia should not be forgotten. Also, look for other causes like severe hypoxemia, medication side effects and interactions…etc.

 

Q4: Answer E

Remember that up to 90% of the pyramidal tract decussates in the lower medulla to the contralateral side. However, in a minority of normal population this decussation could be very minor or even absent as in this patient. In those people, a lesion in this tract above the lower medulla will not give a contalateral pyramidal signs, but instead these will be ipsilateral. This patient had a cortical irritating tumor, which causes focal fits at the same side of the tumor. Although other options might be true in real practice, this congenital anomaly should not be forgotten.

 

Q5: Answer C

He is likely to have been diagnosed with schizophrenia and been given a conventional neuroleptic, after which he developed neuroleptic malignant syndrome. Notice that this syndrome develops over few days and it is not an sudden one.

 

Q6: Answer A

In Friedreick's ataxia, there is no impairment or only mild impairment in cognition; a marked cognitive impairment should cast a doubt on the diagnosis. An absent family history does not refute the diagnosis; however, its presence is an excellent clue.

 

 

 

 

 

 

Q7: Answer E

The picture is of an acute left sided papillitis, which has a broad differential diagnosis and causes. The pace and severity of the clinical picture, physical findings, and careful investigations and follow up are very important. Vasculitides, infectious agents, sarcoidosis…etc all can produce this picture, and multiple sclerosis (MS) although unlikely it is worthy to exclude it. The visual loss in giant cell arteritis is irreversible, and a rapid spontaneous improvement goes with MS. Foster Kennedy gives a slowly progressive optic atrophy in one side and papilloedema in the other side.

 

 

 

Q8: Answer C

Notice that the overall picture is of a cluster headache-like, but the picture is atypical given the 2 years of non-remission and occurrence during day and night, and there are features of hypogonadism. All of them might indicate an intracranial space-occupying lesion in and around the sella. Actually, MRI of this patient revealed a large sellar mass with supra-sellar extension and impingement upon the left carotid artery, which explains both the hyperprolactinemia and the dysautonomia affecting the left side of the face (eye redness and nasal discharge) by affecting the sympathetic fibers around the left carotid artery. It was a prolactimona by histopathological examination after successful surgical debulking.

 

 

 

Q9: Answer A

These signs indicate that CNS involvement is present, which can be documented by CSF studies (after correcting the platelet count if severely depressed). The duration of steroids is too short to cause such a picture of obesity. Vincristine causes peripheral sensory neuropathy, not a cranial motor one. Those ALL patients with CNS involvement need intrathecal chemotherapy and cranio-spinal irradiation as the prognosis is unfortunately poor.

 

 

 

 Q10: Answer C

This patient is immune suppressed because of cyclosporine. Given the presence of impaired consciousness, fever, and neck stiffness, meningitis (or meningoencephalitis) is the most likely diagnosis. We should stop cyclosporine, and start broad-spectrum antibiotics and do CSF examination. Blood cultures can be done, and adding another immune suppressant (azathioprine) would be a silly step.

 

Q11: Answer E

She has two sites of involvements: spinal cord and optic. Both are of white matter signs. The pace is somewhat rapid, and although she did not report an improvement, B12 deficiency and heredito-familial degenerative conditions are highly unlikely. Instead, one should exclude multiple sclerosis by having this clinical picture and should be differentiated from anti-phospholipid syndrome, SLE, vasculitis...etc. Therefore, Brain imaging would be a reasonable step here.

 

 

Q12: Answer A

This is most likely an embolic stroke to the main stem of the left middle cerebral artery. The infracted area is large with mass effect and midline shift. So, measures to reduce the raised intracranial pressure should be instituted without any delay like high concentration O2, mannitol infusion...etc; while anticoagulation is definitely indicated, it is should be omitted for the time being as there is a very high risk of hemorrhagic transformation in this large friable infarcted area, and starting it after 1-2 weeks is reasonable. Her atrial fibrillation is rapid and she needs measures to slow it like giving digoxin, and an echo study is needed to evaluate her heart.   A DC shock is not indicated as we don’t know the duration of the AF; besides, given the presence of mitral stenosis, there is a very high risk of recurrence and emboli showering in the presence of no anticoagulation coverage.

 

Q13: Answer E

Given the presence of such a long history, positive family history, stork-like legs and palpable nerves in the context of little disability and no other system affection, Charcot Marie Tooth disease is the most likely diagnosis.

 

Q14: Answer D

This is Broca's aphasia in a right-handed man; the posterior part of the lower left frontal gyrus is the culprit site.

 

Q15: Answer E

Weight gain, not weight loss, is a side effect. Alopecia is seen with valproic acid, while hirsutism is seen with phenytoin.

 

Q16: Answer C

Kayser-Fleischer ring is due to deposition of copper in the Descement's membrane of the cornea, which is reversible upon successful therapy with copper chelators.

 

 

Q17: Answer D

CIDP, not GBS, can be a cause. Notice that CMT type I is a demyelinating disease that produces palpable nerves; type II is an axonal one, with no such finding. Other cause: leprosy.

 

 

Q18: Answer C

Notice that both Carbamazepine and phenytoin produce worsening in cases of petit mal (and myoclonic) epilepsy and should be avoided. Valproic acid and Ethosuximide are first line agents for this epilepsy, while lamotrigine and clonazepam are second line agents.

Valproic acid is preferred because of its wide spectrum against many types of epilepsy, which might coexist with petit mal (like myoclonic jerks and GTC seizures).

 

 

 

 

 

Q19: Answer E

This is papilloedema! Notice that papillodema is not seen in multiple sclerosis (MS). Papillitis is seen in MS, which is seen as a red optic nerve head swelling with SEVERE reduction in the visual acuity.

 

Q20: Answer C

The clinical picture is suggestive of pyogenic meningitis in a previously healthy man with no history of head trauma; so staring iv ceftriaxone and vancomycin is the next step while arranging for lumbar puncture. Brain CT scan is not indicated as the history and findings are not focal. MRI is not used in critically ill and confused patients in the emergency departments; brain CT is the first line imaging modality. He has no risk factors for HIV, and ordering this test without a prior consent is not accepted; besides, the result will not change our lines of management initially. 

 

Q21: Answer E

Dysautonomias are seen in up to 65% of cases and might produce a sudden cardio-respiratory death. Some degree of asymmetry in findings may be seen in up to 9% of cases; however, marked asymmetry should cast a doubt on the diagnosis. Even in the best centers, the mortality rate is around 4-5% of cases. The CSF proteins level (as is the nerve conduction study) might be totally normal in the 1^st week of illness; hence normal studies early in the illness are not against the diagnosis.  Some patients, around 6%, may experience relapses and which might be associated with certain HLA haplotypes

 

 

Q22: Answer C

Mesial temporal or hippocampal atrophy is a common cause of temporal lobe epilepsy (TLE). Notice the prodromal features that are followed by the ictus (for which the patient recollects nothing). Carbamazepine and phenytoin are effective in many patients and should be the first line if no contraindication is present. Refractory TLE patients are the best candidates for surgical treatment for complex focal epilepsies in general. The EEG may show lateralized spikes in one or both temporal lobes.

 

 

Q23: Answer C

A meningioma is an extra-axial tumor that is benign in a substantial number of victims. The presence of underlying brain edema indicates that the tumor may be malignant, or the presence of so-called secretary meningioma, or an atypical benign meningioma; hence massive brain edema is seen uncommonly. The tumor may stop growing, but never regresses. The commonest sites are over the cerebral convexities and along the falx cerebri; the infra-tentorial fossa and the spine are uncommon sites. Intra cranial meningiomas are seen more commonly in females with a history of breast cancer, and together with cranial irradiation in the past, are considered a risk factor for meningioma development. Multiple meningiomas should always prompt a search for a diagnosis of neurofibromatosis type II.

   

 

 

 

 

Q24: Answer B

Aphasia is a supratentorial sign, that almost always indicates a cortical lesion in the dominant hemisphere; thalamic aphasia is rare and has an excellent prognosis. Certain brainstem lesions might result in anarthria, which can be easily confused with aphasia.

 

 

 

 

Q25: Answer A

The idiopathic variety is the commonest type; other causes: intracranial venous sinus thrombosis, medications (tetracycline, vitamin A), Addison's disease and Cushing's syndrome. Headache free variety is well documented, and unfortunately those patients presents lately with visual loss. The visual loss is due to secondary (not primary) optic atrophy to a long-standing papilloedema. Although steroids can be a cause (long-term treatments, particularly upon withdrawal), they are the agent of choice in severe elevation of the ICP. Surprisingly, optic nerve sheath fenestration, although done in one eye, it protects both eyes.    

 

Q26: Answer B

CNS involvement in leukemias always portends a grave prognosis, especially when present at the time of the diagnosis. Headache, backache, confusion, cranial palsies, and root signs all might be clues to leukemic meningitis. A single CSF sample may fail to show the blast cells, and repeated samples are required to increase the diagnostic yield. Neck stiffness in leukemias could be due to leukemic meningitis, infectious meningitis, or subarachnoid hemorrhage (due to low platelets). The patient should be put on a certain treatment protocol that entails intrathecal chemotherapy (methotrexate, cytosar and hydrocortisone) and craniospinal irradiation.   

 

Q27: Answer D

Nystagmus is an infratentorial sign, and thus is due to posterior circulation ischemia in stroke patients. Hemiplegia could be due to anterior or posterior circulation strokes.

Seizures are seen in 5-15% of stroke patients because of cortical irritation (mainly in embolic or hemorrhagic strokes).

 

Q28: D

The concept of dominant and non-dominant hemisphere is important in localization in neurology. All aphasias are due to dominant hemispheric lesions. Hemiplegia is seen in many sites damaged by a pathology (like the cortex, internal capsule, basis pontis…etc). Asteriognosis is a cortical sign, but can be seen in both dominant and non-dominant parietal hemispheric lesions.  Constructional apraxia is due to non-dominant parietal lesions. Urinary incontinence is seen in lesions involving both mesial frontal lobes as in normal pressure hydrocephalus.

 

Q29: Answer B

Most likely, that clarithromycine intake had resulted in enzyme inhibition and carbamazepine toxicity manifesting as ataxia and diplopia.

 

Q30: Answer D

Motor neuron disease has no autonomic neuropathy as a feature. Other causes: fatal familial insomnia, vasculitides, Fabrey's disease, and multiple system atrophy.

 

Q31: Answer B

Most likely, he has TB meningitis, and anti TB medications are better to be started while awaiting the CSF culture for TB bacilli. The history is too long for a viral etiology, and the intra cranial pressure is high (normal 5-18 cm H2O). The hydrocephalus in TB meningitis could be both communicating and non-communicating. Even with treatment, many are left with considerable degree of neurological disability. 

 

Q32: Answer C

This man has pseudobulbar palsy. Option C is a cause of bulbar palsy.

 

Q33: Answer D

Notice that the presence of cerebellar signs, ocular palsies, marked dementia, Parkinsonian features, sensory signs and symptoms, and sphincter disturbances, all are against the diagnosis. The combination of ALS, Dementia, and Parkinsonian features is a very rare distinct clinical syndrome, and should not make you change your answer in the MRCP examination regarding the classical MND features.

 

Q34: Answer C

Notice that this woman has primary optic atrophy. Any longstanding raised intracranial pressure states with papilloedema (for example from a space-occupying lesion) can produce secondary optic atrophy.

 

 

Q35: Answer D

Aphasia is cortical sign; all others are can be caused by white matter demyelination. Noticed that the dementia that is seen in advanced MS is a sub-cortical type.

 

Q36: Answer B

Oligodendogliomas are primary brain tumors that are usually supratentorially in location, less infiltrative that astrocytomas, and hence most of them are amenable to complete surgical excision, and usually responds well to systemic chemotherapy. Tumor calcification is usually seen in 30% of cases.

 

Q37: Answer C

Of all gliomas, glioblastoma multiforme is the most aggressive one, and infiltrating the adjacent areas diffusely making complete surgical excision impossible (debulking surgery is used to decrease its size, obtain a tissue diagnosis, and reduce the intracranial pressure). Gliomatosis cerebri is said to be present when there is diffuse infiltration without a clear cut mass or enhancement area on brain MRI. Needless to say, the prognosis is very poor. Metastasis outside the CNS is rare and is usually seen in those who are operated.

 

Q38: Answer C

Donepezil is a central inhibitor of acetyl cholinesterase, used in early cases of Alzheimer's disease. About 10-20% of patients will show a modest improvement in their cognition. The medication has a long half-life, and is given once per day. Tacarine (central inhibitor of acetyl cholinesterase) is hepatotoxic; donepezil is not. It can cause bradycardia, and thus contraindicated in those with advanced heart blocks. 

Q39: Answer A

NPH is a cause of potentially reversible dementia. It is uncommon cause of dementia (Alzheimer's disease comprises up to 50% of cases of dementia in old people). It can be idiopathic, or secondary to head trauma, subarachnoid hemorrhage, or meningitis. Brain MRI will show dilated ventricles (without prominent sulci) with upward bulging of the corpus callosum and widening of the Sylvius aqueduct, in the absence of a mass lesion. The definitive diagnosis is by radionuclide cisternography. Post-shunting, 30-50% of patients will show improvement in their gait and cognition.      

 

Q40: answer C

Huntington's disease (HD) is an autosomal dominant disease, due to trinucleotide (CAG) repeat expansion at the HD gene. Both seizures and Parkinsonian features are more common in juvenile cases, which are fortunately rare. Those who presents with chorea in the context of a positive family history, should be differentiated from benign familial chorea, which has a different prognosis.

 

Q41: Answer E

Benztropine (like all anticholinergics) is effective at treating tremor, not rigidity; and in those who are above the age of 60-65 years, can produce confusional state. Amantidine can be used in early cases, either alone or with anticholinergics, has a mild short-lived effect that rapidly wears off. L-dopa is the prodrug of dopamine. There are no D6 receptors (only D1-5). Tolcapone (and entacapone) is a COMT inhibitor, and thus enhances the effect of L-dopa therapy.

 

Q42: Answer A

Hematomyelia is a hematoma in the substance of the cord, can be due to AVMs, coagulation defects, and vasculitis. Decompression into the subarachnoid space can occur. When an AVM is the cause, spinal angiography is done to delineate the AVM together with evacuation of the hematoma; otherwise, the treatment is symptomatic.

 

Q43: Answer A

Tabes dorsalis is a form of tertiary syphilis. Rombergism is seen in 50% of cases only due to loss of kinesthesia (and hence vibration sense should be impaired also). Loss of bladder sensation causes urinary retention. Extensor planters are seen in Taboparesis or general paresis of insane, not in pure tabes dorsalis. The severe lancinating pains may cause severe abdominal pain (tabetic abdominal crises) that brings the patient as an acute abdomen (but no surgical cause can be found, so-called medical acute abdomen).

 

Q44: Answer D

The idea of giving Beta INFs in RRMS is to decrease the severity and frequency of relapses (by one third), so the occurrence of 1 relapse after 1 year of therapy is not a reason to stop this form of therapy; instead, 3 or more attacks within 1 year that necessitate steroid pulses, is an indication of treatment failure, and the Beta INF should be stopped. Options a, b, and c are true.

 

 

 

 

 

Q45: Answer E

Levitiracetam is an anti-epileptic agent. Also avoid cocaine, quinolones, and chlopromazine.

 

Q46: Answer C

The precise mode of action of Riluzole is unknown, but it is supposed to have an anti-glutamate action, and thus interfering with excito-toxic mechanisms. It is given orally and has been found to produce a modest improvement in survival figure. It is hepato-toxic, and can cause weight loss.

 

Q47: Answer B

Dapsone (usually when given in the long term, as in the treatment of leprosy, in which the patient shows a new motor worsening after a period of improvement) can cause a pure motor peripheral neuropathy.

 

Q48: Answer B

The distinction between demylinating and axonal neuropathy is very important regarding the possible etiology and prognosis. There is no definite clinical test for this discrimination, but a nerve conduction study can answer us. Vitamin B12 deficiency is a cause of subacute/chronic primary peripheral axonopathy.

 

Q49: Answer D

The anticholinesterases have a symptomatic benefit only, and do not affect the course of illness. Thymectomy has no place in: neonatal myasthenia, congenital myashtenic syndromes, patients above the age of 65 years, myasthenia duration of more than 7 years, and pure ocular myasthenias. Iv Ig is used in myasthenic (not cholinergic) crisis. Although prednisolone is effective in almost all cases, it can produce a dramatic worsening at the start of treatment. Cyclosporine has a modest effect as an immune suppressive agent in MG.

 

Q50: Answer D

Drug induced myopathy is usually under-diagnosed in clinical practice. The list is long, but paracetamol is not implicated. Zidovudine can cause myopathy with ragged red fibers due to mitochondrial toxicity. The risk of statine-induced myopathy (or myositis) is greatly increased when these agents are used with cyclosporine or gemfibrozil.

 

Q51: Answer C

IBM is the commonest cause of inflammatory myositis in those above the age of 50 years. Although the EMG may show evidence of denervation, there is no degeneration of anterior horn cells. It is unfortunately resistant to immune suppressive therapy (occasionally, many cases are diagnosed as polymyositis, which comes into light after failure of immune suppressive therapy). The prognosis is poor. Some cases show familial clustering. The presence of rimmed vacuoles on biopsy specimen is highly suggestive of IBM, and there are many other distinctive features on histopathological examination, like deposition of amyloid, presence of eosinophilic cytoplasmic inclusions…etc.

 

 

 

Q52: Answer D

Only facioscapulohumeral muscular dystrophy (MD) is not associated with cardiac involvement (although it may be associated with sensory neural deafness and retinal detachment, and labile hypertension). Limb girdle MD uncommonly associated with cardiac involvement.

 

Q53: Answer B

Notice that botulism is a disease of motor end plate due to interference of acetylcholine release, and affect acetylcholine release at autonomic ganglia causing various dysautonomias.

 

Q54: Answer E

Machado-Joseph's disease is a spinocerebellar ataxia type 3, due to CAG repeat expansion. 

 

Q55: Answer C

In 30% of cases, no obvious source of infection can be found, and the surrounding edema is vasogenic. LP is both dangerous and unnecessary. After successful treatment, around 50% are left with seizures. 

 

Q56: Answer A

About 5-10% of PCR-proven cases will have a normal brain MRI (and 33% will have a normal brain CT scan). The virus can not be cultured from the CSF. The negative predicative value of a negative CSF PCR assay for HSV DNA is 98%, and acyclovir should be started without delay when there is a clinical suspicion of HSV encephalitis, as it decreases both morbidity and mortality figures.

Q57: Answer E

Positive CSF protein 14-3-3 in the context of rapidly progressive dementia and myoclonus is highly suggestive (but not diagnostic) of CJD (it can be also positive in HSV encephalitis but negative in Alzheimer's disease). Any fever, high ESR, peripheral blood neutrophilic leukocytosis, or neutrophilic CSF pleocytosis should cast a doubt on the diagnosis.

 

Q58: Answer C

When the ICP is recorded invasively through ventriculostomy, the CSF can be drained to decrease the pressure. The head of bed should be elevated. Steroids can be used in edemas associated with brain tumors or abscesses (they are useless in edema associated with ischemic stroke or hemorrhagic stroke). Plasma osmolality should be kept BELOW 320 mosmol/Kg when using mannitol.

 

Q59: Answer B

CPM results from too rapid correction of hyponatremia or hypo-osmolal states. Partial forms are not uncommon, and may present as dysarthria, confusion, or even gaze palsies; thus, the absence of quadriparesis is not against the diagnosis. The usual picture is an acute onset of quadriparesis and pseudobulbar palsy in a chronic alcoholic man who received an aggressive correction of chronic hyponatremia.

 

 

 

 

Q60: Answer E

Option E is an "inclusion criteria". You should be familiar with both inclusion and exclusion criteria for receiving thrombolytic therapy in this setting.

 

Q61: Answer E

Hypothyroidism is a cause of slowly progressive ataxia in long standing cases. 

 

Q62: Answer B

Kearns-Sayre syndrome is a sporadic, not inherited cause of SCA.

 

Q63: Answer C

Enteroviruses are the commonest agents responsible for viral meningitis. All other infectious agents are considered to be uncommon causes. 

 

Q64: Answer D

Not every viral encephalitic process portends a gloomy prognosis. La Crosse (California) virus, Venezuelan equine encephalitis virus, and Ebstein Barr virus have a good prognosis in general. EEE (Eastern Equine viral Encephalitis) has a case fatality rate of 50-75%, and at lest 80% of survivors are left with severe neurological deficits.

 

Q65: Answer D

Status epilepticus is a medical emergency with high mortality rate, the commonest precipitating event is sudden withdrawal (self or iatrogenic) of anti-epileptics. Lactic acidosis is very common but does not warrant any treatment per se. Phenobarbitone infusion should be used as a 3^rd step (after lorazepam and phenytoin). Hyperthermia, not hypothermia is seen.

 

Q66: Answer C

Ischemic stroke in young people (less than 45 years old) should prompt a thorough search for an underlying cause, like atrial fibrillation, SBE, meningovascular syphilis, paradoxical embolism through a patent foramen ovale…etc.

 

Q67: Answer A

In the absence of chronic hypertension, or in patients younger than 40 years of age, spontaneous "hypertensive" hemorrhage is unlikely. Instead, we should look for other causes like AVM, coagulopathy, drug abuse, head trauma, hemorrhage in tumors…etc. Congophilic angiopathy is seen in old people. 

 

Q68: Answer D

This is a new onset of focal motor seizures in an adult man. Intracranial space occupying lesions should always be excluded. Brain CT scan may miss many lesions; hence, MRI is superior to it. After head traumas, some patients may develop early and or late onset of focal epilepsy. A migraine like headache with focal seizures should always prompt a search for APS (anti-phospholipid syndrome) or AVM. Carbamazepine is the drug of choice in focal seizures.

   

Q69: Answer A

Aspirin has been shown to decrease stroke related morbidity and mortality, and early stroke recurrence. Heparin is indicated in persistent cardiogenic source of embolization. Heparin is associated with increased risk of CNS (or systemic) hemorrhage which statistically outweighs any benefit. The combination of aspirin and clopidogrel in the acute setting is still controversial.

 

 

Q70: Answer D

Ocular and facial muscles in polymyositis are usually spared; while the anterior neck muscles are commonly involved (causing neck drop). Skin rash defines dermatomyositis. Respiratory muscles involvement is fortunately uncommon but dangerous and mainly seen in acute cases.

 

Q71: Answer C

Cerebellar masses in children are usually malignant brain tumors, and the pilocytic variety of low-grade astrocytomas has an excellent prognosis and complete cure can be achieved if removed completely. In general, infratentorial tumors tend to raise the intracranial pressure early, unlike the supratentorial ones. Neck stiffness, with negative Kerning's sign, indicates an infratentorial mass lesion. The most fearful event is secondary brainstem compression and herniation syndromes.   

 

 

End

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