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    Neurology

    A 52 year old banker has recurrent episodes of short lived, severe pains in his jaw on the left. Thesecause him to screw his face up. e has seen a dentist who feels that the most likely cause for hisproblem is trigeminal neuralgia. e seeks your advice. !hich of the following are not true of thiscondition"#ptionsA. $t affects women more commonly than men%. $t may respond to phenytoin treatment&. Around '() of cases are bilateral*. $t can be a manifestation of multiple sclerosis+. #nset is most commonly after the age of 5(

    &Theme Trigeminal NeuralgiaTrigeminal Neuralgia-ore common after the age of 5(-ore common in women than menaro/ysmal attacks lasting seconds 0 affect any of the three divisions of the trigeminal nerveTriggered by touching the face, chewing or speakingTreated with carbama1epine or phenytoin, may respond to surgical microvascular decompressiom*ifferentiate fromostherpetic neuralgia, structural lesion , cluster headache, migraine, tempero3mandibular joint pain0 by length 4 location of attacks.

    A 25 year old woman with a previous history of optic neuritis is admitted with sudden onset ofdiplopia. !hich of the following would favour a diagnosis of -ultiple clerosis"#ptionsA. 6aised +6%. 7ymphocytic pleocytosis in the &8

    &. revious history of episodic gait disturbance*. eriventricular high signal lesions on T2 weighted -6$+. eripheral neuropathy

    &Theme #ptic neuritis and --ultiple clerosis 9-: may be mimicked by a range of inflammatory conditions including 7+,sarcoidosis, 7yme disease and jogren;s syndrome. +vidence of a remitting relapsing nature of thecondition would be the most suggestive of the available choices. A raised +6, peripheral neuropathyand lymphocytic pleocytosis 9although seen in -: raise the possibility of another diagnosis.eriventricular high signal lesions on T2 weighted scans are characteristic of - but may be seen inother conditions.

    A cold> the previous week, but felt that this hadcleared up. he is unable to walk because of the di11yness which is constant and severe. There istinnitus in her left ear, and some sensori3neural hearing loss, on e/amination. There is no nystagmus,and the rest of the e/amination is normal. !hat is the most likely diagnosis 3#ptionsA. estibular neuronitis%. %enign positional vertigo&. 7eft cerebello3pontine angle lesion*. estibular chwannoma+. Acute labyrinthitis

    +Theme ertigoThe acute onset, constant course and history of preceeding viral infection make acute labyrinthitis themost likely cause. This is thought to be an acute, infective process affecting the middle ear. $t istreatable with bed rest and anti3emetics.estibular neuronitis tends to run a more chronic relapsing course. A vestibular schwannoma9acoustic neuroma: is a cerebello3pontine angle lesion. These tend to be more chronic inpresentation, and often involve other cranial nerves. er vertigo is clearly not positional?

    A '@ year3old man complains of gradual onset of blurred vision in his right eye. #n direct uestioninghe admits to having some difficulty in his job as a wine critic. e has no other neurologicalsymptoms. e has a history of treated hypertension and impaired glucose tolerance. e has been onramipril for the past B years. There is no family history of note. e smokes 5 cigars a day and drinksin moderation. +/amination reveals a right sided relative afferent pupillary defect, right sided opticatrophy and right sided central scotoma with an upper uadrantic visual field defect in the left eye.7ower cranial nerves and the peripheral nervous system are normal. !here is the lesion 3#ptionsA. 6ight optic nerve%. 6ight anterior optic chiasm&. #ptic chiasm*. %oth retinae

    +. #ccipital lobe

    %Theme isual fields7esions of the anterior chiasm 9junction of optic nerve and chiasm: tend to produce an ipsilateralscotoma and a contralateral superior uadrantanopia. The contralateral defect is due to interruptionof the crossing nasal fibres 9!illebrandCs Dnee:.A lesion of the nerve would produce total blindness.&hiasmal lesions produce bitemporal hemianopia.#ccipital lobe lesions may produce homonymous hemianopia.

    A 52 year old labourer has a two week history of increasing weakness. e has found it increasinglydifficult to climb stairs and get up out of his chair. e smokes 2( cigarettes a day and drinks around Bpints of lager a day. e has no previous medical history and takes paracetamol for headaches.!hich of the following features would make a peripheral neuropathy much more likely than amyopathy as a cause of his weakness3#ptions

    A. redominantly pro/imal pattern of weakness%. 7oss of refle/es&. 8ibrillations*. ain in the limbs+. $nvolvement of the cranial nerves

    %Theme Neurological e/amination7oss of refle/es8eature Neuropathy -yopathy*istribution *istal ro/imalain ossible ossible6efle/es 7ost 6etainedAtrophy resent Esually absent+-F 8ibrillations mall motor units&D Normal $ncreased&ranial nerves Esually spares ometimes involved-uscle biopsy Froup atrophy Necrosis

    A 22 year old trainee policeman has been increasingly confused over a three day period. e has ageneralised headache. e has become disorientated in time and place, with speech disturbance.e has a temperature of B@&, pulse GG( bpm. There is mild neck stiffness but no focal neurologicalabnormalities. ystemic e/amination is normal. A lumbar puncture is performed, it shows a protein of2g4l, glucose ' 9serumH

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    *Theme Acute confusionThe clinical picture of confusion associated with fever is suggestive of a central nervous systeminfection. The main differential is between a bacterial, viral or fungal infection. Alternatively, there area number of rarer aseptic causes of meningitis.$n this case, however, there are abnormalities of the lumbar puncture. The lack of neutrophils andnormal glucose would make a bacterial meningitis very unlikely. $n view of the history and theelevated protein in the cerebrospinal fluid, the most likely diagnosis would be a herpes simple/encephalitis. There is no history given of immunocompromise, that would make you think of T% orbrucellosis.

    A B2 year old psychologist is seen in the emergency department. he awoke that morning with asevere left sided headache, and blurring of her vision in the left eye. he was nauseous and di11y.

    #f note in her past medical history, she had had an episode of optic neuritis B years beforehand, inthe right eye, which had resolved spontaneously. he drank around thirty units of alcohol a week andused cannabis recreationally. he was married since the age of 2B.#n e/amination, there was a left afferent pupillary defect with weakness of the facial muscles on theright. Tone and refle/es were brisk on the right with power of B45 in the arm and the leg. he wasapyre/ial. -6$ of the brain revealed a large left temperoparietal mass, which was incompletely ringenhancing. There were one or two small white matter lesions also visible.!hat is the most likely diagnosis 3#ptionsA. &erebral abcess%. 7ymphoma&. -etastatic lesion*. -ultiple sclerosis+. -alignant glioma

    *Theme %rain mass

    A large mass like this may be due to infection, malignancy or inflammation.The possibilty of infection with an atypical agent would rise in the conte/t of immunocompromise.This also applies for the risk of cerebral lymphoma. %ut there are no risk factors for $, here. Atypical abcess would usually be associated with a fever and complete ring enhancement.A secondary malignancy would be unlikely in someone this young, but this should be considered, asshould a primary malignancy, like a glioma.$n view of the appearance of the lesion on -6$, the associated white matter changes and theprevious optic neuritis, the most likely cause would be a demyelinating lesion, which may present inthis fashion. This should be confirmed with visual evoked potentials and oligoclonal band analysis.

    A B( year old man presents with hand tremor. #n e/amination he has a fine postural tremor in bothhands. !hich of the following would disprove the likely diagnosis"#ptionsA. +/acerbation with alcohol%. -ild intention tremor&. Titubation*. 8amily history+. $mprovement with beta blockers

    ATheme TremorThis is benign essential tremor. $t may occur at any age and has an autosomal dominant inheritance.$t is predominantly postural although there may be a mild intention component. There may be headinvolvement 9titubation:. $mprovement with alcohol and beta blockers is characteristic. Treatment canbe difficult, and significant disability may result.

    A 25 year old woman presents to clinic with a < month history of right hand tremor. The freuency isappro/imately

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    A. Gst degree relatives being affected with similar problems%. Tremor restricted to the arms&. A response of the tremor to ben1odia1epines*. Enilaterality+. Tremor that involves his voice

    *Theme Tremor*ifferentiating arkinson;s disease and essential tremorAge 0 arkinson;s tends to come on later in l ife.7ocation 0 +ssential tremor is more generalised and symmetrical. $t often affects the head and voice.8irst degree relatives 0 -ore commonly affected in essential tremorAssociated neurological signs 0 rigidity, bradykinesia in arkinson;s, usually none in essential tremor.

    6esponse 0 arkinson;s will respond to 73*opa, anticholinergics and dopamine agonists. +ssentialtremor is often responsive to alcohol, ben1odia1epines and beta3blockers!riting 0 arkinson;s sufferers often demonstrate micrographia, while essential tremor often involvestremulous writing.

    A 25 year old man is diagnosed with relapsing3remitting multiple sclerosis following B attacks of opticneuritis and ata/ia over a 2 year period. e is now severely disabled, and cannot mobilise without theaid of a wheelchair. Although previously continent, he has had increasing problems with urinaryurgency and freuency and G episode of retention. !hich of the following is not an appropriatemanagement strategy3#ptionsA. &lean intermittent self catheterisation%. Trial of o/ybutinin&. $nstitution of a bowel regime*. Trial of steroids+. +/clusion of a urinary tract infection

    *Theme -ultiple sclerosis%ladder problems are common in multiple sclerosis. They may involve urgency and freuencyalongside episodes of retention. $ndeed, retention with overflow can occur. $t is important to rule outassociated constipation, and la/ative use is important.$nfections of the urinary tract occur in where there are voiding difficulties. &oncurrentimmunosupression can cause major problems when this happens.*epending on the results of urodynamics and post3mictruition residual measurement, the use of anti3cholinergics and4or catheterisation may be appropriate. An indwelling supra3pubic catheter is apossible option for some patients. .%ladder and bowel management are very important to patients, but are often forgotten or notdiscussed.

    Neurology -6&G 9part two:

    A 25 year old woman with a previous history of optic neuritis is admitted with sudden onset ofdiplopia. !hich of the following would favour a diagnosis of -ultiple clerosis"#ptionsA. 6aised +6%. 7ymphocytic pleocytosis in the &8&. revious history of episodic gait disturbance*. eriventricular high signal lesions on T2 weighted -6$+. eripheral neuropathy

    &Theme #ptic neuritis and --ultiple clerosis 9-: may be mimicked by a range of inflammatory conditions including 7+,sarcoidosis, 7yme disease and jogren;s syndrome. +vidence of a remitting relapsing nature of thecondition would be the most suggestive of the available choices. A raised +6, peripheral neuropathyand lymphocytic pleocytosis 9although seen in -: raise the possibility of another diagnosis.eriventricular high signal lesions on T2 weighted scans are characteristic of - but may be seen inother conditions.

    A G< year old boy is falling behind in school. e has become withdrawn and depressed. e has begunfalling over and has difficulty performing simple tasks. #n e/amination, he is of normal height, buildand general appearance. e has slurred speech and has difficulty with finger3nose co3ordination. ehas ata/ia, with a broad3based gait. Tone, power and refle/es are all normal, as is his sensorye/amination. ou notice that he occasionally adopts bi1arre and prolonged postures where he e/tendshis arms or legs and, sometimes, his neck, pulling his head back. e appears to be droolingsignificantly. !hat is the diagnosis"#ptionsA. -yotonia *ystrophica%. &erebellar tumour

    &. Ouvenile arkinsonism*. !ilson;s disease+. Ata/ia3Telangiectasia

    *Theme &erebellar disease in childrenThe basic elements are3&erebellar disease 9ata/ia, co3ordination problems, speech:3&ognitive $nvolvement 9low mood poor school performance:3*ystonia 9posturing:.A cerebellar tumour does not produce secondary dystonia or 9generally: cognitive disturbance.-yotonia *ystrophia involves the musculature rather than co3ordination Ouvenoile arkinsonism cangive secondary dystonia, but not the other features. Ata/ia Telangiectasia would not involve dystoniaand would usually have presented before this point.This makes !ilson;s disease the best answer. *iagnosis would be confirmed by a 2' hour urinarycopper level, slit lamp e/amination 9for Dayser38leischer 6ings: or demonstration of copper in theliver if this was involved.

    A 25 year old woman with a previous history of optic neuritis is admitted with sudden onset ofdiplopia. !hich of the following would most favour a diagnosis of multiple sclerosis"#ptionsA. 6aised +6%. 7ymphocytic pleocytosis in the &8&. revious history of episodic gait disturbance*. eriventricular high signal lesions on T2 weighted -6$+. eripheral neuropathy

    &Theme *iagnosing multiple sclerosis- may be mimicked by a range of inflammatory conditions including 7+, sarcoidosis, 7yme diseaseand jogren;s syndrome. +vidence of a remitting relapsing nature of the condition would be the mostsuggestive of the available choices. A raised +6, peripheral neuropathy and lymphocytic pleocytosis9although seen in -: raise the possibility of another diagnosis. eriventricular high signal lesions onT2 weighted scans are characteristic of - but may be seen in other conditions.

    The 7ambert3+aton myasthenic syndrome3#ptionsA. *oes not respond to treatment with cholinesterase inhibitors%. &ommonly causes distal rather than pro/imal weakness&. $s associated with antibodies to post3synaptic voltage gated &a channels*. $s commonly associated with non3small cell lung cancer+. -ay be associated with postural hypotension

    +Theme 7ambert3+aton myasthenic3myopathic3 syndrome 97+-:The clinical features are pro/imal weakness, loss of tendon refle/es with post3tetanic potentiation,and autonomic dysfunction. i/ty per cent of cases are paraneoplastic. Antibodies to pre3synapticvoltage3gated &a channels are foundK the underlying malignancy 9as in most paraneoplasticneurological syndromes: is usually small cell lung carcinoma.

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    A 5( year3old man presents with a history of severe daily unilateral headaches for the past weekwhich wake him up at night. +/amination is normal e/cept for a right ornerCs syndrome. &T head isnormal. !hat is the diagnosis3#ptionsA. -igraine%. &arotid dissection&. &luster headache*. Trigeminal neuralgia+. Tension headache

    &Theme eadache and ornerCs syndrome

    Enilateral, severe, throbbing headache associated with lacrimation and occurring daily 9often at thesame time: for several days on end is usually cluster headache. #ccasionally ornerCs syndrome maybe seen.

    A '' year old advertising e/ecutive is admitted with worsening difficulty in swallowing. is wife hasrecently returned from holiday in Freece.#n e/amination, there are bilateral stressed> or tired. +ach episode lasts for around G5 minutes and resolvesspontaneously. Neurological and physical e/amination, including slit lamp, were entirely normal. #ntapping the right foot for over a minute, the foot would e/tend and invert. !hat therapeutic trialwould you instigate"#ptionsA. A trial of levodopa%. A trial of amitryptilline&. A trial of carbama1epine*. A trial of chlorproma1ine+. A trial of lora1epam

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    ATheme &ramping of the footThis patient has a localised dystonia which is a prolonged contracture of a particular muscle group.There are no other disorders that are present. !riterCs cramp, torticolis and blepharospasm are alltypes of adult onset focal dystonias. *ystonias like this are defined as primary in order todifferentiate them from the large number of conditions that produce dystonia as a secondary feature.

    A sub group of primary dystonias are dramatically dopa3responsive. Therefore, of the optionsavailable, a trial of levodopa would be the most appropriate option.Amitryptilline and carbama1epine are used in the treatment of neuropathic pain. &hlorproma1ine isan old antipsychotic while lora1epam is a short acting sedating be1odia1epine.

    A B( year old builder has acute onset of 6ight orbital pain at work. The ne/t day he notices that his

    right eye appears odd. #n e/amination, he has a mild right ptosis and the right pupil is 2mm smallerthan the left, but both react normally to light. isual acuity, fields and eye movements are normal.The site of injury is to which of the following structures"#ptionsA. #ptic Tract%. 7eft #ccipital corte/&. #culomotor Nerve*. TG Nerve 6oot+. uperior &ervical Fanglion

    +Theme Enilateral ptosisThis patient has a unilateral ornerCs syndrome. A ornerCs syndrome involves ptosis, pupillaryconstriction 9but reaction to light: and a loss of sweating on the affected side. The l esion is in thesuperior cervical ganglion as this is the major synpathetic outflow to the head. Normal e/tra3ocularmovements and fields rule out the other causes.Any lesion to the sympathetic chain on the ipsilateral side may produce a ornerCs.

    A '< year old man presents with painless bilateral arm weakness and abdominal pain. #ne/amination he has bilateral wrist drop. !hich one of the following substances is the -#T likelycause of his symptoms"#ptionsA. 7ead%. -ercury&. #rganophosphate*. Arsenic+. Thallium

    ATheme oisons7ead neuropathy3chronic ingestion of lead produces similar condition to Acute $ntermittent orphyria.$n adults causes colic, anaemia and peripheral neuropathy. %ilateral wrist drop is classic 9i.e.,. rare:presentation. -ercury3neurospyschiatric manifestations 9mad hatter?: and movement disorders.#rganophosphate3immediate anticholinesterase effect 9headache, vomiting, abdo cramps, salivation,miosis: delayed distal polyneuropathy

    Arsenic3symptoms of encephalopathy or peripheral neuropathy.Thallium3rapidly progressive painful polyneuropathy, ophthalmoplegia and optic atrophy.

    A 5' year3old accountant presents to casualty at 2 a.m. with a B day history of being woken up in theearly morning with severe headache which lasts G32 hours. The headache is Pboring; in character,involves the eye and frontal region on the left side, and is associated with eye3watering. e says it isthe worst pain that he has ever had, and he is unable to sleep until it subsides. e denies any visualsymptoms. #n e/amination he appears in pain. There is no neck stiffness. The left conjunctiva isinjected and there is a left orner;s syndrome. !hat is the diagnosis #ptionsA. -igraine%. &luster headache&. aro/ysmal hemicrania*. Trigeminal neuralgia

    +. Transient ischaemic attack

    %Theme eadacheEnilateral headaches that occur periodically, mostly at night, with episodes lasting for

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    pseudodementia, unlike those with Al1heimer;s disease, often complain about their memory. There issome retention of insight. $n pseudodementia both the short term and long term memory appear tobe eually affected in the early stage of the disease, unlike Al1heimer;s disease where only the shortterm memory is initially involved. $n pseudodementia improvement in cognitive testing usually occursfollowing treatment with antidepressants.

    A B< year old man is still unable to return to work si/ months after the death of his partner. All thesymptoms described below are consistent with normal grief reactions e/cept 3#ptionsA. &omplaints regarding e/haustion or lack of strength%. 8eelings of increased emotional distance&. re3occupation with guilt and personal responsibility*. $ncreased activity without feelings of loss

    +. 8eelings of irri tability, hostility and anger

    *Theme FriefAcute grief is a definite syndrome with psychological and somatic symptoms. $t may presentimmediately after a crisis or onset maybe delayed. The clinical presentation includes sensations ofsomatic distress, feelings of diminished strength or physical e/haustion, feelings of milddepersonalisation or de3realisation, recurrent dreams or ruminations about the death itself or thedeceased person, feelings of guilt and personal responsibility for the loss, diminished desire for inter3personal contact, mood lability and irritability. athological grief reactions may present as over3activity without a sense of loss, acuisition of physical symptoms belonging to the deceased, onset ofany medical condition, conspicuous alteration in social adjustment, e/treme deterioration in moodand character, and frank depression or psychosis.

    A B( year old man is admitted following a suicide attempt. !hich of the following does not increasethe risk of a successful attempt 3#ptions

    A. +pilepsy%. -&. chi1ophrenia*. %eing married+. ersonality disorder

    *Theme 6isk factors for suicide6isk factors for suicide include chronic illnesses or pain, alcoholism, depression, unemployment andaccess to the means. ome factors are protective, such as being in a close relationship andreligiosity. Around 2() of those who attempt self harm will, eventually, comit suicide.

    A BJ3year3old &aucasian man presents to &asualty after having turned up for his work on his day offand behaved unusually. At interview his speech is rapid and slurred. e is aroused, animated andover3familiar with clang3associations and tangentiality. e states that there are voices in his head,that the T and radio talk directly to him, that his father has been a secret agent and his motherassassinated. e states that he has saved a girl;s life and complains of a four3month headache. e istoo distractible for cognitive stage e/amination and no further history is available. hysical

    e/amination 0 no abnormalities are elicited.%lood investigations reveal a neutrophilia of =.J 92.23J.5: and a lymphopenia of (.'J 9G.23'.(:.#verall the white cell count was GG.G 9B.@3GG.(:. 6%&, T, -& and platelet counts are all withinnormal parameters as were biochemistry results.&ollateral history reveals that his father had been a diplomat and had possibly performed for theintelligent services. is mother had died in a car crash. e had not saved a girl;s life as claimed. Thepatient claimed to have had an accident after being sideswiped on a motorway, while driving apowerful motorbike at great speed. is family stated that it was a farm accident on a moped. Thepatient has recently lost his flat after accumulating substantial debts for non3payment of hismortgage. is supervisor reports that he has had difficulties at work for the last two years and hasreuired e/tra support. e has been moved repeatedly from positions of responsibility to lesser onesand has been over3familiar with customers via the loudspeaker system and has had to be removedfrom cash related tasks as he had been found to be less than careful in his duty.The following morning the patient had two tonic3clonic sei1ures, was pyre/ial and had increased tone

    in the right side of his body.A lumbar puncture shows an elevated &8 protein of (.@5 9.G23.

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    #ptionsA. yphilis serology%. Neurological e/amination&. ++F*. &T4-6$ scan of the brain+. *etailed mental state e/amination

    &Theme $nvestigation of dementia++F is non3specific and only shows slowed wave activities in dementia. $t has a role in the differentialdiagnosis of difficult cases but is not routinely reuested as part of the initial investigations.

    A =< year old nursing home resident is becoming withdrawn and forgettful. All of the following are

    correct in relation to dementia disorders e/cept #ptionsA. *ementia with 7ewy bodies account for about 2() of all dementia patients%. oint prevalence of dementia in those in their eighties is 53= )&. Anticholinesterase inhibitors may be useful in patients with vascular dementia*. The -ini3-ental tate +/amination is not a sensitive test for frontotemporal dementia+. The Lmirror signM may be seen in patients with severe Al1heimer;s disease

    %Theme *ementiaoint prevalence of dementia in all those over the age of