Emrcp CNS 38-62

35

A 41-year-old man presents to his GP with a two week history headaches concentrated around the left side of his face. He describes having about two episodes a day each lasting around 30 minutes. What is the likely diagnosis?ia

A.A Migraineia

B.A Cluster headacheia

C.A Trigeminal neuralgiaia

D.A Tension headacheia

E.A Meningiomaia

Next question

Episodic eye pain, lacrimation, nasal stuffiness occurring daily - cluster headache

Cluster headache sqweqwesf erwrewfsdfs adasd dheOverview

more common in men (5:1) and smokershe

he earaer aeraer asdsadas eerw dssdfsselledsFeatures

pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hourshe clusters typically last 4-12 weekshe intense pain around one eye (recurrent attacks affect 'always' affect same side)he accompanied by redness, lacrimation, lid swellinghe nasal stuffinesshe miosis and ptosis in a minorityhe

he earaer aeraer asdsadas eerw dssdfsselledsManagement

prophylaxis: lithiumhe acute: 100% oxygen, sumatriptanhe

A 55-year-old man is referred to the neurology clinic due to a resting tremor and an abnormal gait characterised by short, shuffling steps. Which one of the following would features would point towards a diagnosis of Parkinson's disease rather than parkinsonism of another cause?ia

A.A Asymmetrical tremoria

B.A Bradykinesiaia

C.A Impairment of vertical gazeia

D.A Confusionia

http://www.emrcp.com/question.php?q=9

36

E.A Poor response to levodopa therapyia

Next question

Asymmetrical symptoms suggests idiopathic Parkinson's

Parkinson's disease: features sqweqwesf erwrewfsdfs adasd dheParkinson's disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra.. This results in a classic triad of features: bradykinesia, tremor and rigidity. The symptoms of Parkinson's disease are characteristically asymmetricalhe earaer aeraer asdsadas eerw dssdfsselledsBradykinesia

poverty of movement also seen: mask-like facieshe difficulty in initiating movementhe

he earaer aeraer asdsadas eerw dssdfsselledsTremor

most marked at rest, 3-5 Hzhe typically 'pill-rolling'he

he earaer aeraer asdsadas eerw dssdfsselledsRigidity

lead pipehe cogwheel: due to superimposed tremorhe

he earaer aeraer asdsadas eerw dssdfsselledsOther characteristic features

flexed posturehe short, shuffling stepshe micrographiahe drooling of salivahe depression, dementiahe

A 64-year-old man presents with a eight-month history of generalised weakness. On examination he has fasciculation and weakness in both arms with absent reflexes. Examination of the lower limbs reveal increased tone and exaggerated reflexes. Sensation was normal and there were no cerebellar signs. What is the most likely diagnosis?ia

A.A Lead poisoningia

B.A Motor neuron diseaseia

C.A Vitamin B12 deficiencyia

D.A Syringomyeliaia


37

E.A Multiple sclerosisia

Next question

'Fasciculations' - think motor neuron disease

Motor neuron disease: features sqweqwesf erwrewfsdfs adasd dheMotor neuron disease is a neurological condition of unknown cause which presents with both upper and lower motor neuron signs. It rarely presents before 40 years. Three distinct patterns of disease are recognised: amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsyhe earaer aeraer asdsadas eerw dssdfsselledsFor the MRCP, there are a number of clues which point towards a diagnosis of motor neuron disease:

fasciculationhe absence of sensory signs/symptoms*he lower motor neuron signs in arms and upper motor neuron signs in legshe

he earaer aeraer asdsadas eerw dssdfsselledsOther features

doesn't affect external ocular muscleshe no cerebellar signshe abdominal reflexes are usually preserved and sphincter dysfunction if present is a late featurehe

he earaer aeraer asdsadas eerw dssdfsselledsThe diagnosis of motor neuron disease is clinical, but nerve conduction studies will show normal motor conduction and can help exclude a neuropathy. Electromyography shows a reduced number of action potentials with an increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathyhe earaer aeraer asdsadas eerw dssdfsselleds*vague sensory symptoms may occur early in the disease (e.g. limb pain) but 'never' sensory signs

A 19-year-old female presents to her GP complaining of visual disturbance. Examination reveals a bitemporal hemianopia with predominately the lower quadrants being affected. What is the most likely lesion?ia

A.A Brainstem lesionia

B.A Craniopharyngiomaia

C.A Frontal lobe lesionia

D.A Pituitary macroadenomaia

E.A Right occipital lesionia

Next question

Bitemporal hemianopia



38

lesion of optic chiasm upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary

tumour lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a

craniopharyngioma

Visual field defects sqweqwesf erwrewfsdfs adasd dheGreater detail is required in the MRCP when assessing visual field defects - quadrantanopias are described as are incongruous and congruous defectshe earaer aeraer asdsadas eerw dssdfsselledsThe main points for the exam are:

left homonymous hemianopia means visual field defect to the left, i.e. lesion of right optic tracthe homonymous quadrantanopias: PITS (Parietal-Inferior, Temporal-Superior)he incongruous defects = optic tract lesion; congruous defects= optic radiation lesion or occipital cortexhe

he earaer aeraer asdsadas eerw dssdfsselledsHomonymous hemianopia

incongruous defects: lesion of optic tracthe congruous defects: lesion of optic radiation or occipital cortexhe macula sparing: lesion of occipital cortexhe

he earaer aeraer asdsadas eerw dssdfsselledsHomonymous quadrantanopias

superior: lesion of temporal lobehe inferior: lesion of parietal lobehe mnemonic = PITS (Parietal-Inferior, Temporal-Superior)he

he earaer aeraer asdsadas eerw dssdfsselledsBitemporal hemianopia

lesion of optic chiasmhe upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary

tumourhe lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a

craniopharyngiomahe

Which one of the following features is most suggestive of Lewy body dementiaia

A.A Disinhibitionia

B.A Emotional labilityia

C.A Sensitivity to neurolepticsia

D.A Urinary incontinenceia

39

E.A Paucity of extrapyramidal signsia

Next question

Lewy body dementia sqweqwesf erwrewfsdfs adasd dheLewy body dementia is an increasingly recognised cause of dementia, accounting for up to 20% of cases. The characteristic pathological feature is cytoplasmic neuronal inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areashe earaer aeraer asdsadas eerw dssdfsselledsThe relationship between Parkinson's disease and Lewy body dementia is complicated, particularly as dementia is often seen in Parkinson's disease. Also, up to 40% of patients with Alzheimer's have Lewy bodieshe earaer aeraer asdsadas eerw dssdfsselledsNeuroleptics should be avoided in Lewy body dementia as patients are extremely sensitive and may develop irreversible parkinsonism. Questions may give a history of a patient who has deteriorated following the introduction of an antipsychotic agenthe earaer aeraer asdsadas eerw dssdfsselledsFeatures

progressive cognitive impairmenthe Parkinsonismhe visual hallucinations (other features such as delusions and non-visual hallucinations may also be seen)he

he earaer aeraer asdsadas eerw dssdfsselledsPrognosis

mean survival similar to Alzheimer's, rapid progression with death within 2 yearshe

Which types of motor neuron disease carries the worst prognosis?ia

A.A Relapsing-remittingia

B.A Bulbar palsyia

C.A Progressive muscular atrophyia

D.A Spinocerebellar ataxiaia

E.A Amyotrophic lateral sclerosisia

Next question

Motor neuron disease: types sqweqwesf erwrewfsdfs adasd dheMotor neuron disease is a neurological condition of unknown cause which presents with both upper and lower motor neuron signs. It rarely presents before 40 years and three distinct patterns of disease are recognised: amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy. In some patients however, there is a combination of clinical patternshe earaer aeraer asdsadas eerw dssdfsselledsAmyotrophic lateral sclerosis (50% of patients)

typically LMN signs in arms and UMN signs in legshe



40

in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutasehe

he earaer aeraer asdsadas eerw dssdfsselledsProgressive muscular atrophy (25% of patients)

anterior horn cell lesionhe affects distal muscles before proximalhe carries best prognosishe

he earaer aeraer asdsadas eerw dssdfsselledsBulbar palsy

palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nucleihe

carries worst prognosishe

A 25-year-old female is found to have a left hemiparesis following a deep vein thrombosis. An ECG shows RBBB with right axis deviation. What is the most likely underlying diagnosis?ia

A.A Ventricular septal defectia

B.A Patent ductus arteriosusia

C.A Ostium primum atrial septal defectia

D.A Ostium secundum atrial septal defectia

E.A Tetralogy of Fallotia

Next question

The ostium secundum in this patient has allowed passage of an embolus from the right-sided circulation to the left causing a stroke

Atrial septal defects sqweqwesf erwrewfsdfs adasd dheAtrial septal defects (ASDs) are the most likely congenital heart defect to be found in adulthood. They carry a significant mortality, with 50% of patients being dead at 50 years. Two types of ASDs are recognised, ostium secundum and ostium primum. Ostium secundum are the most commonhe earaer aeraer asdsadas eerw dssdfsselledsFeatures

ejection systolic murmur, fixed splitting of S2he embolism may pass from venous system to left side of heart causing a strokehe

he earaer aeraer asdsadas eerw dssdfsselledsOstium secundum (70% of ASDs)

associated with Holt-Oram syndrome (tri-phalangeal thumbs)he ECG: RBBB with RADhe


41

he earaer aeraer asdsadas eerw dssdfsselledsOstium primum

present earlier than ostium secundum defectshe associated with abnormal AV valveshe ECG: RBBB with LAD, prolonged PR intervalhe

A 76-year-old man is brought by his daughter to the local A&E department. She is concerned about his increasing forgetfulness and recent onset of urinary incontinence. Neurological exam reveals a shuffling gait. What is the most likely diagnosis?ia

A.A Lewy body dementiaia

B.A Motor neuron diseaseia

C.A Normal pressure hydrocephalusia

D.A Creutzfeldt-Jacob diseaseia

E.A Pick's diseaseia

Next question

Urinary incontinence + gait abnormality + dementia = normal pressure hydrocephalus

Normal pressure hydrocephalus sqweqwesf erwrewfsdfs adasd dheNormal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitishe earaer aeraer asdsadas eerw dssdfsselledsA classical triad of features is seen

urinary incontinencehe dementiahe gait abnormality (may be similar to Parkinson's disease)he

he earaer aeraer asdsadas eerw dssdfsselledsImaging

hydrocephalus with an enlarged fourth ventriclehe


ventriculoperitoneal shuntinghe

Internuclear ophthalmoplegia is most commonly associated with:ia


42

A.A Neurofibromatosisia

B.A Meningitisia

C.A Multiple sclerosisia

D.A Motor neuron diseaseia

E.A Myasthenia gravisia

Next question

Internuclear ophthalmoplegia sqweqwesf erwrewfsdfs adasd dheOverview

a cause of horizontal disconjugate eye movementhe due to a lesion in the medial longitudinal fasciculus, which connects the IIIrd, IVth and VIth cranial

nucleihe

he earaer aeraer asdsadas eerw dssdfsselledsFeatures

impaired adduction of the eye on the same side as the lesionhe horizontal nystagmus of the abducting eye on the contralateral sidehe

he earaer aeraer asdsadas eerw dssdfsselledsCauses

multiple sclerosishe vascular diseasehe

Which one of the following is not a feature of essential tremor?ia

A.A Improved by alcoholia

B.A Autosomal recessive inheritanceia

C.A Improved by propranololia

D.A Titubationia

E.A Worse on intentional movementia

Next question

Essential tremor is an AD condition that is made worse by intentional movement, made better by alcohol and propranolol

Tremor sqweqwesf erwrewfsdfs adasd dheResting tremor



43

parkinsonismhe if neuroleptic induced then usually bilateralhe

he earaer aeraer asdsadas eerw dssdfsselledsPostural tremor (e.g. worse if arms outstretched)

essential tremor (usually bilateral)he hepatic encephalopathyhe CO2 retentionhe anxietyhe thyrotoxicosishe

he earaer aeraer asdsadas eerw dssdfsselledsIntention tremor

cerebellar diseasehe

he earaer aeraer asdsadas eerw dssdfsselledsEssential tremor

worse on intentional movementhe improved by alcoholhe also most common cause of titubation (head tremor)he strong family history, autosomal dominanthe management: propranolol; primidone is sometimes usedhe

A 54-year-old man with small cell lung cancer complains of muscle weakness. Each one of the following are features of Lambert-Eaton syndrome, except:ia

A.A Proximal muscles more commonly affectedia

B.A Hyporeflexiaia

C.A Dry mouthia

D.A Repeated muscle contractions lead to decreased muscle strengthia

E.A Impotenceia

Next question

In myasthenia gravis repeated muscle contractions lead to reduced muscle strength. The opposite is however classically seen in the related disorder Lambert-Eaton syndrome

Lambert-Eaton syndrome sqweqwesf erwrewfsdfs adasd dheLambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer, and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous systemhe earaer aeraer asdsadas eerw dssdfsselledsFeatures


44

repeated muscle contractions lead to increased muscle strength* (in contrast to myasthenia gravis)he limb girdle weakness (affects lower limbs first)he hyporeflexiahe autonomic symptoms: dry mouth, impotence, difficultly micturatinghe ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)he

he earaer aeraer asdsadas eerw dssdfsselledsEMG

incremental response to repetitive electrical stimulationhe


guanidine is sometimes usedhe plasma exchange may be beneficialhe

he earaer aeraer asdsadas eerw dssdfsselleds*in reality this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease

A 34-year-old man from West Africa is admitted due to confusion associated with left-sided weakness and ataxia. He is known to be HIV positive but is not on anti-retroviral treatment. The following results are obtained:he earaer aeraer asdsadas eerw dssdfsselleds

CD4 43 u/l

CT head Low attenuation diffusely. No mass effect or enhancement

he earaer aeraer asdsadas eerw dssdfsselledsWhat is the most likely diagnosis?ia

A.A Toxoplasmosisia

B.A Tuberculosisia

C.A Progressive multifocal leukoencephalopathyia

D.A Cryptococcusia

E.A Cerebral lymphomaia

Next question

HIV: neurocomplications sqweqwesf erwrewfsdfs adasd dheGeneralised neurological diseasehe earaer aeraer asdsadas eerw dssdfsselledsEncephalitis

may be due to CMV or HIV itselfhe HSV encephalitis but is relatively rare in the context of HIVhe


45

CT: oedematous brainhe

he earaer aeraer asdsadas eerw dssdfsselledsCryptococcus

most common fungal infection of CNShe headache, fever, malaise, nausea/vomiting, seizures, focal neurological deficithe CSF: high opening pressure, India ink test positivehe CT: meningeal enhancement, cerebral oedemahe meningitis is typical presentation but may occasionally cause a space occupting lesionhe

he earaer aeraer asdsadas eerw dssdfsselledsProgressive multifocal leukoencephalopathy (PML)

widespread demyelinationhe due to infection of oligodendrocytes by human papovirus (JC virus)he symptoms, subacute onset : behavioural changes, speech, motor, visual impairmenthe CT: single or multiple lesions, no mass effect, don't usually enhance. MRI is better - high-signal

demyelinating white matter lesions are seenhe

he earaer aeraer asdsadas eerw dssdfsselledsAIDS dementia complex

caused by HIV virus itselfhe symptoms: behavioural changes, motor impairmenthe CT: cortical and subcortical atrophyhe

he earaer aeraer asdsadas eerw dssdfsselledsFocal neurological lesionshe earaer aeraer asdsadas eerw dssdfsselledsToxoplasmosis

constitutional symptoms, headache, confusion, drowsinesshe CT: usually multiple ring enhancing lesions, mass effect may be seenhe management: sulfadiazine and pyrimethaminehe

he earaer aeraer asdsadas eerw dssdfsselledsTuberculosis

single enhancing lesionhe

A 33-year-old female with multiple sclerosis complains that her vision becomes blurred during a hot bath. What is this an example of?ia

A.A Uhthoff's phenomenonia

B.A Oppenheim's signia

C.A Werdnig-Hoffman's signia

D.A Lambert's signia

46

E.A Lhermitte's signia

Next question

This is Uhthoff's phenomenon. Lhermitte's sign describes paraesthesiae in the limbs on neck flexionhe earaer aeraer asdsadas eerw dssdfsselledsOppenheim's sign is seen when scratching of the inner side of leg leads to extension of the toes. It is a sign of cerebral irritation and is not related to multiple sclerosishe earaer aeraer asdsadas eerw dssdfsselledsWerdnig-Hoffman's disease is also known as spinal muscular atrophy

Multiple sclerosis: features sqweqwesf erwrewfsdfs adasd dheVisual

optic neuritis: common presenting featurehe optic atrophyhe Uhthoff's phenomenon: worsening of vision following rise in body temperaturehe internuclear ophthalmoplegiahe

he earaer aeraer asdsadas eerw dssdfsselledsSensory

pins/needleshe numbnesshe trigeminal neuralgiahe Lhermitte's syndrome: paraesthesiae in limbs on neck flexionhe

he earaer aeraer asdsadas eerw dssdfsselledsMotor

spastic weaknesshe

he earaer aeraer asdsadas eerw dssdfsselledsCerebellar

ataxiahe tremorhe

he earaer aeraer asdsadas eerw dssdfsselledsOthers

urinary incontinencehe sexual dysfunctionhe intellectual deteriorationhe

Which one of the following features is most associated with temporal lobe lesions?ia

A.A Wernicke's aphasiaia


47

B.A Inferior homonymous quadrantanopiaia

C.A Gerstmann's syndromeia

D.A Cortical blindnessia

E.A Perserverationia

Next question

Brain anatomy sqweqwesf erwrewfsdfs adasd dheThe following neurological disorders/features may allow localisation of a brain lesion:he earaer aeraer asdsadas eerw dssdfsselledsParietal lobe lesions

sensory inattentionhe apraxiashe astereognosis (tactile agnosia) he inferior homonymous quadrantanopiahe Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left

disorientationhe

he earaer aeraer asdsadas eerw dssdfsselledsOccipital lobe lesions

homonymous hemianopiahe cortical blindnesshe visual agnosiahe

he earaer aeraer asdsadas eerw dssdfsselledsTemporal lobe lesion

Wernicke's aphasiahe superior homonymous quadrantanopiahe auditory agnosiahe

he earaer aeraer asdsadas eerw dssdfsselledsFrontal lobes lesions

expressive (Broca's) aphasiahe disinhibitionhe perserverationhe anosmiahe

A 14-year-old male is noted to have optic atrophy on fundoscopy. Neurological exam reveals dysarthric speech and nystagmus. Knee and ankle jerks are absent but there is an extensor plantar response. What is the likely diagnosis?ia

A.A Leber's optic atrophyia

B.A Ataxic telangiectasiaia


48

C.A Friedreich's ataxiaia

D.A Subacute combined degeneration of the cordia

E.A Multiple sclerosisia

Next question

Friedreich's ataxia sqweqwesf erwrewfsdfs adasd dheFriedreich's ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich's ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipationhe earaer aeraer asdsadas eerw dssdfsselledsThe typical age of onset is 10-15 years old he earaer aeraer asdsadas eerw dssdfsselledsNeurological features

absent ankle jerks/extensor plantarshe cerebellar ataxiahe optic atrophyhe spinocerebellar tract degenerationhe


hypertrophic obstructive cardiomyopathy (90%, most common cause of death)he diabetes mellitus (10-20%)he high-arched palatehe

A 29-year-old female presents to her GP complaining of weakness in her arms, leading to increasing difficulties at work. On examination she has a bilateral ptosis and loss of the red-reflex in both eyes. Urine testing also reveals glycosuria. What is the most likely diagnosis?ia

A.A Myotonic dystrophyia

B.A Homocystinuriaia

C.A Multiple sclerosisia

D.A Myasthenia gravisia

E.A HIVia

Next question

Myotonic dystrophy sqweqwesf erwrewfsdfs adasd dheInherited myopathy with features developing at around 20-30 years old. Affects skeletal, cardiac and smooth musclehe earaer aeraer asdsadas eerw dssdfsselledsGenetics



49

autosomal dominanthe a trinucleotide repeat disorderhe CTG repeat at the end of the DPMK gene on chromosome 19he

he earaer aeraer asdsadas eerw dssdfsselledsFacial features

myotonic facies (long, 'haggard' appearance)he frontal baldinghe bilateral ptosishe cataractshe dysarthriahe


myotonia (tonic spasm of muscle)he weakness of arms and legs (distal initially)he mild mental impairmenthe diabetes mellitushe testicular atrophyhe cardiomyopathyhe dysphagiahe

Which one of the following features would suggest neurofibromatosis type 2?ia

A.A Peripheral neurofibromasia

B.A Lisch nodulesia

C.A Bilateral acoustic neuromasia

D.A Axillary frecklesia

E.A Café-au-lait spotsia

Next question

Neurofibromatosis sqweqwesf erwrewfsdfs adasd dheOverview

two types, NF1 and NF2he both autosomal dominanthe

he earaer aeraer asdsadas eerw dssdfsselledsNF1

also known as von Recklinghausen's syndromehe caused by gene mutation on chromosome 17 which codes for neurofibrominhe affects 1 in 4,000he


50

he earaer aeraer asdsadas eerw dssdfsselledsNF2

caused by gene mutation on chromosome 22he affects 1 in 100,000he

he earaer aeraer asdsadas eerw dssdfsselledsNF1 features

café-au-lait spots (>= 6)he axillary/groin freckleshe peripheral neurofibromashe iris: Lisch nodules in > 90%he

he earaer aeraer asdsadas eerw dssdfsselledsNF2 features

bilateral acoustic neuromashe

A 67-year-old man presents to his GP concerned about increasing clumsiness. Examination reveals an ataxic gait and increased upper limb tone with cog-wheel rigidity. Blood pressure is 135/80 lying and 95/70 standing. What is the most likely diagnosis?ia

A.A Motor neuron diseaseia

B.A Progressive supranuclear palsyia

C.A Parkinson's diseaseia

D.A Multiple sclerosisia

E.A Multiple system atrophyia

Next question

Whilst postural hypotension may be seen in Parkinson's disease the cerebellar signs point towards a diagnosis of multiple system atrophy

Multiple system atrophy sqweqwesf erwrewfsdfs adasd dheShy-Drager syndrome is a type of multiple system atrophyhe earaer aeraer asdsadas eerw dssdfsselledsFeatures

Parkinsonismhe autonomic disturbance (atonic bladder, postural hypotension)he cerebellar signshe

A 54-year-old man presents to his GP concerned about leg weakness. On examination he is noted to have increased tone in both legs, brisk reflexes and weakness in both lower limbs. Examination of his upper limbs is normal. Which one of the following is least likely to produce these symptoms?ia


51

A.A HIVia

B.A Sarcoidosisia

C.A Cervical spondylosisia

D.A Multiple sclerosisia

E.A Parasagittal meningiomaia

Next question

Spastic paraparesis sqweqwesf erwrewfsdfs adasd dheSpastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbshe earaer aeraer asdsadas eerw dssdfsselledsCauses

demyelination e.g. multiple sclerosishe cord compression: trauma, tumourhe parasagittal meningiomahe tropical spastic paraparesishe transverse myelitis e.g. HIVhe syringomyeliahe cervical spondylosishe hereditary spastic paraplegiahe

A 19-year-old man is admitted following a generalised seizure. He has no past medical history of note but on examination it is noted that he has three patches of hypopigmented skin and fibromata under two of his finger nails. What is the most likely diagnosis?ia

A.A Neurofibromatosisia

B.A Systemic lupus erythematousia

C.A Vitiligoia

D.A Solvent abuseia

E.A Tuberous sclerosisia

Next question

This man has a neurocutaneous syndrome which raises the possibility of neurofibromatosis or tuberous sclerosis. Given the areas of hypopigmentation and subungual fibromas the most likely diagnosis is tuberous sclerosis

Tuberous sclerosis sqweqwesf erwrewfsdfs adasd dheTuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. As is neurofibromatosis, the majority of features seen are neuro-cutaneoushe earaer aeraer asdsadas eerw dssdfsselledsCutaneous features



52

depigmented 'ash-leaf' spots which fluoresce under UV lighthe roughened patches of skin over lumbar spine (Shagreen patches)he adenoma sebaceum: butterfly distribution over nosehe fibromata beneath nails (subungual fibromata)he café-au-lait spots* may be seenhe

he earaer aeraer asdsadas eerw dssdfsselledsNeurological features

developmental delayhe epilepsy (infantile spasms or partial)he intellectual impairmenthe

he earaer aeraer asdsadas eerw dssdfsselledsAlso

retinal hamartomas: dense white areas on retina (phakomata)he rhabdomyomas of the hearthe gliomatous changes can occur in the brain lesionshe polycystic kidneys, renal angiomyolipomatahe

he earaer aeraer asdsadas eerw dssdfsselleds*these of course are more commonly associated with neurofibromatosis. However a 1998 study of 106 children with TS found café-au-lait spots in 28% of patients

Which of the following factors indicate a poor prognosis in multiple sclerosis?ia

A.A Relapsing-remitting diseaseia

B.A Presence of sensory symptomsia

C.A Young age of onsetia

D.A Male sexia

E.A Long interval between first two relapsesia

Next question

Multiple sclerosis: prognostic features sqweqwesf erwrewfsdfs adasd dheGood prognosis features

female sexhe young age of onsethe relapsing-remitting diseasehe sensory symptomshe long interval between first two relapseshe

he earaer aeraer asdsadas eerw dssdfsselledsi.e. the typical patient carries a better prognosis than an atypical presentation


53

A 23-year-old male is admitted with the sudden onset of painful headache. A subarachnoid haemorrhage is suspected but the CT scan is normal. At what time should a lumbar puncture be done?ia

A.A Immediatelyia

B.A 2 hours post-headacheia

C.A 4 hours post-headacheia

D.A 12 hours post-headacheia

E.A 24 hours post-headacheia

Next question

Subarachnoid haemorrhage sqweqwesf erwrewfsdfs adasd dheCauses

85% are due to rupture of berry aneurysms (conditions associated with berry aneurysms include adult polycystic kidney disease, Ehlers-Danlos syndrome and coarctation of the aorta)he

AV malformationshe traumahe tumourshe

he earaer aeraer asdsadas eerw dssdfsselledsInvestigations

CT: negative in 5%he LP: done after 12 hrs (allowing time for xanthochromia to develop)he

he earaer aeraer asdsadas eerw dssdfsselledsComplications

rebleeding (in 30%)he obstructive hydrocephalus (due to blood in ventricles)he vasospasm leading to cerebral ischaemiahe


neurosurgical opinion: no clear evidence over early surgical intervention against delayed interventionhe nimodipine (e.g. 60mg / 4 hrly, if BP allows) has been shown to reduce the severity of neurological deficits

but doesn't reduce rebleeding*he

he earaer aeraer asdsadas eerw dssdfsselleds*the way nimodipine works in subarachnoid haemorrhage is not fully understood. It has been previously postulated that it reduces cerebral vasopasm (hence maintaining cerebral perfusion) but this has not been demonstrated in studies

Which one of the following factors most strongly suggests true seizures rather than pseudoseizures?ia


54

A.A Tongue bitingia

B.A Gradual onsetia

C.A Female sexia

D.A Family member with epilepsyia

E.A Pelvic thrustingia

Next question

Pseudoseizures sqweqwesf erwrewfsdfs adasd dheFactors favouring pseudoseizures

pelvic thrustinghe family member with epilepsyhe more common in femaleshe crying after seizurehe don’t occur when alonehe gradual onsethe

he earaer aeraer asdsadas eerw dssdfsselledsFactors favouring true epileptic seizures

tongue bitinghe raised serum prolactinhe

he earaer aeraer asdsadas eerw dssdfsselledsVideo telemetry is useful for differentiating

Which one of the following is least associated with the development of chorea?ia

A.A Ataxic telangiectasiaia

B.A SLEia

C.A Wilson's diseaseia

D.A Oral contraceptive pillia

E.A Infective endocarditisia

Next question

Chorea can be a very rare manifestation of infective endocarditis, following embolisation to the basal ganglia. It is however the least likely of the above five options

Chorea sqweqwesf erwrewfsdfs adasd dheChorea describes rapid, jerky movements which often move from one part of the body to another. Slower, sinuous



55

movement of the limbs is termed athetosis. Chorea is caused by damage to the basal ganglia, especially the caudate nucleushe earaer aeraer asdsadas eerw dssdfsselledsCauses of chorea

Huntingdon's disease, Wilson's disease, ataxic telangiectasiahe SLE, anti-phospholipid syndromehe rheumatic fever: Sydenham's choreahe drugs: oral contraceptive pill, L-dopa, antipsychoticshe neuroacanthocytosishe chorea gravidarumhe thyrotoxicosishe polycythaemia rubra verahe carbon monoxide poisoninghe

Which one of the following antibodies are associated with painful sensory neuropathy in patients with small cell lung cancer?ia

A.A Anti-Riia

B.A Anti-Laia

C.A Anti-Roia

D.A Anti-Huia

E.A Anti-Yoia

Next question

Paraneoplastic syndromes affecting nervous system sqweqwesf erwrewfsdfs adasd dheLambert-Eaton myasthenic syndrome

associated with small cell lung cancer (also breast and ovarian)he antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous systemhe can also occur independently as autoimmune disorderhe

he earaer aeraer asdsadas eerw dssdfsselledsAnti-Hu

associated with small cell lung cancerhe sensory neuropathy - may be painfulhe cerebellar syndromehe encephalomyelitishe

he earaer aeraer asdsadas eerw dssdfsselledsAnti-Yo

associated with ovarian and breast cancerhe cerebellar syndromehe


56

he earaer aeraer asdsadas eerw dssdfsselledsAnti-Ri

associated with small cell lung cancerhe retinal degenerationhe

Documents

Emrcp CNS 38-62