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Neurology

A broad-based ataxic gait occurs characteristically with:

Available marks are shown in brackets

1 ) proximal myopathy [0]

2 ) basal ganglia lesion [0]

3 ) right-sided cerebral infarction [0]

4 ) phenytoin toxicity [100]

5 ) cerebellar vermis lesion [0]

Comments:Broad based gait is associated with cerebellar syndrome. However, lesions of cerebellar vermis causetruncal ataxia and tendency to fall backwards. Right-sided cerebral infarction is associated with a

hemiplegic gait. Basal ganglia disease causes extrapyramidal signs with parkinsonism (festinant gait,marche a petit pas). Proximal myopathy causes a waddling gait.

A 3 day old neonate is noted by ward staff to have had a seizure. Which of the following statementsconcerning neonatal convulsions is correct?


1 ) An abnormal EEG indicates a high risk of later developing epilepsy [0]

2 )A blood glucose level of 1.7mmol/L in a term infant on Day 1 is invariably associated withconvulsions or apnoea.

[0]

3 )Convulsions occurring in the first 24 hours in a baby with an Apgar score of 8 at 5 minutescannot be due to intrapartum asphyxia.

[0]

4 ) If phenobarbitone is prescribed daily estimations of serum bilirubin are needed because ofrisks of kernicterus.

[0]

5 ) Maternal narcotic addiction is a well-recognised cause. [100]

Comments:Maternal narcotic addiction is associated with a neonatal drug withdrawal syndrome. Convulsionsoccurring in the first 24 hours may be the result of placental insufficiency and cerebral palsy.Hypoglycaemia may be associated with seizures, but not invariably. Hepatitis and jaundice are rare sideeffects , CNS depression is more common with phenobarbitone. An abnormal EEG does not correlatewith later risk of epilepsy.

The incidence of Down syndrome in children born to women aged less than 30 years is approximately


1 ) 1:500 [0]

2 ) 1:1000 [100]

3 ) 1:5000 [0]

4 ) 1:10000 [0]

5 ) 1:15000 [0]

Comments:The exact figure is 1 in 1200 births. Maternal age also affects incidence of hydrocephalus, anencephalyand achondroplasia.


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An 18-year-old female presented with bilateral ptosis and tiredness towards afternoons. She had a shorttensilon (edrophonium test which was positive). A diagnosis of myasthenia Gravis was made and shewas stated on pyridostigmine. She now relapses and is given edrophonium intravenously. However her

condition deteriorates and her forced expiratory volume falls to 1.0. She is transferred to the highdependency units. An initial CT scan and chest x-ray were normal.

What should be the next management step?


1 ) Azathioprine [0]

2 ) Emergency thymectomy [0]

3 ) Intravenous methylprednisolone [0]

4 ) Neostigmine [0]

5 ) Plasmaphoresis [100]

Comments:The diagnosis is Myasthenia crisis. The treatment of choice is either iv immunoglobulins or plasmaexchange. Transfer to ITU is essential because patients may deteriorate rapidly needing intubation andventilation.

A 15 year old boy presents with tremor of both hands. Over the previous months he has developed a milddysarthria. He has a history of behavioural problems, of a depressive/psychotic nature. The most likelydiagnosis is:


1 ) Alzheimer's disease [0]

2 ) Huntington's disease [0]

3 ) Neuroacanthocytosis [0]

4 ) variant Creutzfeldt-Jakob disease [0]

5 ) Wilson's disease [100]

Comments:

Wilson's disease is a rare disorder of copper metabolism which is inherited as an autosomal recessivedisease. It is associated with extrapyramidal features (tremor, parkinsonism, dystonia), dysarthria,psychiatric features, cirrhosis and a deposit of brownish-green pigment around the margin of the cornea(Kayser-Fleischer ring).Variant Creutzfeldt-Jakob disease is characterised by myooclonus and rapidonset dementia.

A newborn is diagnosed with an intracranial haemorrhage. Which of the following is typically associatedwith this diagnosis?


1 ) Prematurity. [100]

2 ) Infants of diabetic mothers. [0]

3 ) Congenital toxoplasmosis. [0]

4 ) Haemophilia. [0]

5 ) Hyaline membrane disease. [0]


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Comments:Prematurity is associated with intraventricular haemorrhages and is thought ot be a consequence ofvessel fragility. Intracranial haemorrhage is not usually a presenting feature of Haemophilia.

A couple bring their 7 year old daughter to clinic concerned about her development. She has a history of

delayed speech, ataxia with a broad based gait and also has been diagnosed with epilepsy. She has astrange affect characterised by inappropriate laughter. Her karyotype is normal. What is the most likeldiagnosis in this patient?


1 ) Alice in Wonderland syndrome [0]

2 ) Angelman syndrome [100]

3 ) Asperger's syndrome [0]

4 ) Prader-Willi syndrome [0]

5 ) Turner's syndrome [0]

Comments:Angelman syndrome(happy puppet) is caused by a deletion of q12 in the maternal copy of chromosome

15. A similar deletion in the paternal copy of chromosome 15 causes Prader-Willi syndrome (mentalretardation and hyperphagia). Asperger's syndrome (autistic psychopathy) is characterized by severeand sustained impairment in social interaction, development of restricted and repetitive patterns ofbehavior, interests, and activities. The happy behaviour is not a feature.

A lesion of the Frontal lobe causes:


1 ) Apraxia [0]

2 ) Broca's (expressive) aphasia [100]

3 ) Cortical blindness [0]

4 ) Homonymous hemianopia [0]

5 ) Visuospatial neglect [0]

Comments:Lesions of the frontal lobe include difficulties with task sequencing and executive skills. Expressiveaphasia (receptive aphasias a temporal lobe lesion), primitive reflexes, perseveration (repeatedly askingthe same question or performing the same task), anosmia and changes in personality. Lesions of theparietal lobe include apraxias, neglect, astereognosis (unable to recognise an object by feeling it) andvisual field defects (typically homonymous inferior quadrantanopia). They may also cause alcalculia(inability to perform mental arithmetic). Lesions of the temporal lobe cause visual field defects (typicallyhomonymous superior quadrantanopia), Wernike's (receptive) aphasia, auditory agnosia, and memoryimpairment. Occipital lobe lesions include cortical blindness (blindness due to damage to the visualcortex and may present as Anton syndrome where there is blindness but the patient is unaware or deniesblindness), homonymous hemianopia, and visual agnosia (seeing but not percieving objects - it isdifferent to neglect since in agnosia the objects are seen and followed but cannot be named).

Stiff neck in a young child may be present in the following conditions except:


1 ) Acute poliomyelitis [0]

2 ) Retropharyngeal abscess [0]

3 ) Rubeola [100]

4 ) Still's disease [0]
http://people.zeelandnet.nl/fhof/angelman/asi.htm#whatisAS


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5 ) Tuberculosis [0]

Comments:Rubeola (Measles) can lead to many different complications: croup, bronchitis, bronchiolitis, pneumonitis,conjunctivitis, myocarditis, hepatitis, encephalitis. Measles can also make the body more susceptible toear infections or bacterial pneumonia. The involvement of the cervical spine in the arthritis of Still'sdisease may cause neck stiffness. TB may cause tuberculous meningitis, or Potts disease, both of which

may cause neck stiffness. Retropharyngeal abscess is a recognised cause of neck stiffness with anextended neck.

Causes of a small pupil include:


1 ) Carbon Monoxide Poisoning [0]

2 ) Ethylene Glycol Poisoning [0]

3 ) Holme's Adie pupil [0]

4 ) Pontine haemorrhage [100]

5 ) Third Nerve Palsy [0]

Comments:Causes of small pupils include Horner's syndrome, old age, pontine haemorrhage, Argyll Robertsonpupil, drugs and poisons (opiates, organophosphates). Causes of dilated pupils include Holme's Adie(myotonic) pupil, third nerve palsy, drugs and poisons (atropine, CO, ethylene glycol).

Which of the following is true regarding sensory neural hearing loss?


1 )The incidence is twice as high in babies admitted to neonatal intensive care unitscompared with the normal population.

[0]

2 ) The risk is increased in children who have had rubella. [0]

3 ) Approximately 1 per 1000 children will have greater than 40db hearing loss. [100]

4 ) The risk is increased in Noonan's Syndrome. [0]

5 ) The risk is increased in Down's Syndrome. [0]

Comments:

Sensory neural hearing loss is caused by lesions in the cochlea or the auditory nerve or centralconnections. It may be unilateral or bilateral. Language acquisition and secondary educational difficultiesfollow, with social isolation, and an increased risk of mental health problems. The approximate incidenceis 1 per 1000 children. Risk factors include:

NICU admission: low birth weight, less than 32 weeks gestation, prolonged ventilation,prolonged jaundice, ototoxic drugs, hypoxic ischaemic encephalopathy, neonatal meningitis.

Congenital infection (rubella, CMV).

Dysmorphic syndromes (affecting head and neck). Family history of a close relative needing a hearing aid below the age of 5 years.

Infections: acute bacterial or TB meningitis, mumps (latter usually unilateral).

If all risk factors are considered, only around 50% of cases could be identified by testing between 5 and10% of all babies. Conductive hearing loss is related to middle ear pathology. This is commoner inDown's Syndrome, cleft palate, Turner's Syndrome, and facial malformation syndromes.Copyright 2002 Dr Colin Melville


5/12

A 14 year old girl was found unconscious at the roadside by a passer-by, who called an ambulance.

On examination she was unkempt, had slurred speech and an ataxic gait. Her core temperature was34.5C, but there were no other specific abnormalities to find.BM was 2.9 mmol/l.

What is the most likely diagnosis?


1 ) Brain tumour [0]

2 ) Infections [0]

3 ) Ingestions [100]

Comments:

The picture suggests acute alcohol ingestion. This is often accompanied by hypothermia andhypoglycaemia.

It is important to exclude other ingestions and to look for coexisting social problems.

Gaucher's Disease is associated with the deficiency of :


1 ) Hexosaminidase A [0]

2 ) Sphingomyelinase [0]

3 ) Arylsulphatase-A [0]

4 ) B- Glucosidase [100]

5 ) Iduronidase [0]

Comments:Hexosaminidase A deficiency is associated Tay-Sachs disease. Sphingomyelinase deficiency isassociated with Niemann-Pick disease. Arylsulphatase-A deficiency is associated with metachromicleucodystrophy.Iduronidase deficiency is associated with Hurlers syndrome.

A 32 year old scientist presents to Casualty with a right facial weakness. He has recently returned from aconference in the USA. There is no history of systemic illness, but on examination he has mild neckstiffness and a painful right wrist and knee, with a right facial palsy. Investigations were as follows: WCC7 x 109 /l Hb 12 g/l Plat 190 x 109 /l Clotting normal ESR 32 Na 138 mmol/l K 4.0 mmol/l Urea 6.9 mmol/lCreatinine 76 umol/l Calcium and LFTs normal CXR Normal. CT head Normal. CSF: Protein 1.2g WCC67 (97% lymphocytes) No organisms seen. What is the most likely diagnosis?


1 ) Behcet's disease [0]2 ) HIV associated neuropathy [0]

3 ) Lyme disease [100]

4 ) Sarcoidosis [0]

5 ) Tuberculous meningitis [0]

Comments:All of the answers are possible causes of a facial palsy. The high protein and lymphocytosis of the CSFimply an acute or sub acute infective process. Neurosarcoidosis is thus unlikely. The main clinical


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features of Behcets are not present orogenital ulceration, iritis and pathergy. HIV is usually associatedwith a peripheral sensory neuropathy. TB meningitis is likely to lead to a generalised systemic illness.The fact that the patient has recently been to America and has a unilateral facial palsy makes Lymedisease the most likely diagnosis. Lyme disease is spread by the bite of ticks of the genus Ixodes that areinfected with Borrelia burgdorferi.

A 17-year-old man has been diagnosed with schizophrenia 4 weeks ago. He was started on haloperidol.Two weeks later he was found confused and drowsy. On examination he was pyrexial (40.7 C), rigid withblood pressure of 200/100. Which of the following treatment will you initiate?


1 ) phenytoin [0]

2 ) diazepam [0]

3 ) cefuroxime [0]

4 ) acyclovir [0]

5 ) dantrolene [100]

Comments:Neuroleptic malignant syndromeis the most likely diagnosis. Its major features are: rigidity, altered

mental state, autonomic dysfunction, fever, and high creatinine kinase. It is usually caused by potentneuroleptics. The treatment of choice is dantrolene and bromocriptine. Withdrawal of neuroleptictreatment is mandatory. Rhabdomyolysis and acute renal failure are potential complications.

A 15 year old girl presents with a two week history of headaches and double vision. She had also noticedan episode where her vision dimmed after sneezing. On examination her BMI was 32.4 kg/m

2, she had

bilateral optic disc swelling and a partial left sixth cranial nerve palsy was present.



1 ) benign intracranial hypertension [100]

2 ) Graves' ophthalmopathy [0]3 ) multiple sclerosis [0]

4 ) pituitary tumour [0]

5 ) sagittal vein thrombosis [0]

Comments:

This patient has the features of benign intracranial hypertensionwhich maybe associated with reductionof vision with manoeuvres that raise intracranial pressure and is associated with sixth nerve palsies.There are no features to suggest Graves' ophthalmopathy nor multiple sclerosis.

A pituitary tumour would be likely to produce visual field constriction not mentioned in this history. Asagittal vein thrombosis is a potential differential diagnosis here but is less likely than BIH.

BENIGN INTRACRANIAL HYPERTENSION (PSEUDOTUMOURCEREBRI):
http://www.onexamination.com/site/Paedia.asp?id=129


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most common in obese women of childbearing age. FEATURES: normal CT/MRI brain, normal CSF apartfrom increased pressure, headaches (90%), sixth nerve palsy (30%), papilloedema, visual field loss !!DRUGS ASSOCIATED WITH BIH: tetracyclines, o/c pill, excessive vitamin A, glucocorticoids andmineralocorticoids, and amiodarone. TREATMENT: prevention of visual field loss and relief of headache.(ref. Arch Neurol. 1989;46:1049)

Causes of dilated pupils include:


1 ) Argyll Robertson pupil [0]

2 ) Ethylene glycol poisoning [100]

3 ) Myotonic dystrophy [0]

4 ) Organophosphate poisoning [0]

5 ) Pontine haemorrhage [0]

Comments:

Causes of dilated pupils include Holme's Adie (myotonic) pupil, third nerve palsy, drugs and poisons

(atropine, CO, ethylene glycol). Causes of small pupils include Horner's syndrome, old age, pontinehaemorrhage, Argyll Robertson pupil, drugs and poisons (opiates, organophosphates).

Which vertebral level and corresponding structure is correct?


1 ) C4 and bifurcation of the carotid artery [100]

2 ) T2 and manubriosternal joint [0]

3 ) T10 and opening for vena cava in diaphragm [0]

4 ) T12 and oesophageal opening in the diaphragm [0]

5 ) T8 and aortic opening in the diaphragm [0]

Comments:The carotid artery bifurcates at C4. The manubriosternsal joint(angle of Louis) lies at the level of the T4/5intervertebral disk. The caval opening in the diaphragm lies at T8. The Oesoghageal opening of thediaphragm lies at T10. The aortic opening is at T12.

Compartment syndrome:-


1 ) Only occurs following fractures. [0]

2 ) Loss of distal pulse is an early sign. [0]

3 ) The presence of pain is unhelpful in diagnosis. [0]

4 ) Can be treated using a minimally invasive technique. [0]

5 ) Passive stretch of affected muscles exacerbates pain. [100]

Comments:Compartment syndrome can occur in the absence of a fracture e.g. crush injuries. Loss of peripheralpulses is a late sign indicating that the pressure within the compartment has exceeded arterial bloodpressure. Pain is the earliest and most reliable symptom of the onset of compartment syndrome.Treatment involves decompression of the affected compartment(s) including the skin. Passive stretch ofthe muscles traversing the compartment increases pain.


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Which visual field defect is most likely to occur with multiple sclerosis?


1 ) bitemporal hemianopia [0]

2 ) central scotoma [100]

3 ) homonymous hemianopia [0]

4 ) increased blind spot [0]

5 ) tunnel vision [0]

Comments:

Central scotoma likely with retrobulbar neuritis and optic atrophy. Tunnel vision occurs in glaucoma,retinitis pigmentosa and retinal panphotocoagulation. Increased blind spot occurs with papilloedema,which may lead to optic atrophy. Optic chiasma compression causes bitemporal heminopia.

A 16 year old girl presented with a three week history of headache and horizontal diplopia on far rightlateral gaze. On two separate occasions she noted dimmed vision whilst bending forwards. Over the lastyear she had gained 12 kilograms in weight. On examination, her weight was 95 kg, and height 162cms.Neurological examination revealed bilateral papilloedema and a partial right sixth cranial nerve palsy.What is the most likely diagnosis?


1 ) Benign intracranial hypertension. [100]

2 ) Multiple sclerosis. [0]

3 ) Pituitary tumour [0]

4 ) Superior sagittal vein thrombosis. [0]

5 ) Thyroid eye disease. [0]

Comments:This patient is markedly obese with a BMI of 36 and the history suggestive of BIH. Vision may be affectedwith enlargement of the blind spot and the visual obscuration with movements that provoke a rise in ICP(eg bending) is typical of BIH. Dysthyroid eye disease would not present like this and is more commonly

associated with Hyperthyroidism. The papilloedema would argue against MS. A bitemporal hemianopiaor a visual field defect would be expected with a pituitary tumour. Venous sinus thrombosis is a possibilitybut would be expected to produce deteriorating symptoms.

A 4 year old boy presents with dizziness and vomiting. He has been unwell over the past few days withfever, right ear pain and discharge. Full term normal delivery with no neonatal complications.Immunisations up to date. There is no FH/SH of note.

On examination he has a fever of 38.6C, RR 20/min and HR 100/min. Chest is clear. He has a purulentdischarge from his right ear. He has nystagmus, and walks with a broad-based gait.




2 ) Infections [100]

3 ) Ingestions [0]

Comments:

The history suggests an acute otitis media with developing ataxia and vomiting. This suggests extensionof the infection into the vestibular apparatus. From there it can extend into the brain. IV antibiotics and


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surgical drainage may be required.

A 5 year old girl presents with ataxia. She keeps falling to the left during a ballet lesson. The symptomspersist, and parents take her to casualty. 41/40 gestation 2.9kg, with no neonatal problems.

On examination she is alert with temperature of 36.9C (tympanic), RR of 20/min and HR of 95/min. She

has obvious nystagmus, dysdiadochokinesis and falls to the left when asked to walk.




2 ) Infections [0]

3 ) Ingestions [0]

Comments:

The history is of subacute cerebellar abnormality in the absence of fever. The most likely diagnosis is a

posterior fossa tumour. This can be confirmed by CT or MRI scan. The latter is preferable because thebony posterior fossa interferes with CT images.

Cerebellar Mnemonic

What signs and symptoms do you get with cerebellar disease?

'VANISHED'

V = Vertigo

A = Ataxia

N = Nystagmus

I = Ipsilateral pendular knee jerk/ Intention tremor

S = Skewed deviation of eye

H = Hypotonia

E = Explosive speech (staccato)

D =Dysdiadochokinesis/ dysarthria/ dysmetria


10/12

Which of the following statements regarding hiccup is true?


1 ) Is caused by a tonic relaxation of the diaphragm. [0]

2 ) May be caused by local irritation to the vagus nerve. [0]

3 ) Can reliably be treated with theophylline. [0]

4 ) May be caused by a posterior fossa tumour. [100]

5 ) May be caused by a foreign body in the nose. [0]

Comments:

Hiccup is caused by frequent or rhythmic clonic contraction of the diaphragm. When prolonged, othercauses should be considered including:

CNS disease: Posterior fossa tumour, brain injury, encephalitis.

Phrenic nerve or diaphragm irritation: Tumour, pleurisy, pneumonia, intrathoracic adenopathy,pericarditis, gastro-oesophageal reflux, oesophagitis.

Systemic causes: Alcohol intoxication, uraemia.

Other: Foreign body or insect in the ear. In infants it may be associated with apnoea orhyperventilation.

Folk remedies include aerophagia, breath holding, pharyngeal stimulation, distraction. Haloperidol,metaclopramide and several anaesthetic agents are also said to work.

Which of the following statements regarding Phenylketonuria is correct?


1 ) Serum tyrosine levels are typically low [0]

2 ) Urinary phenylalanine metabolites are typically high [100]

3 ) Mental retardation does not occur if the patient adheres to a phenylalanine free diet [0]

4 ) Is classically due to deficiency of tyrosine hydroxylase [0]5 ) Inheritance is X-linked recessive [0]

Comments:Phenylketonuria (PKU) is the result of hyperphenylalaninaemia. It is autosomal recessive, with aprevalence of 1:10-20,000 live births. Phenylalanine is an essential amino acid. Dietary phenylalanine isnot utilised for protein synthesis, but is normally degraded via the tyrosine pathway. Failure of this resultsin other metabolites that cause brain damage. Deficiency of phenylalanine hydroxylase (Chromosome12) or of the cofactor tetrahydrobiopterin (Genes on Chromosome 10 and 4) causes accumulation ofphenylalanine in body fluids. The affected infant is usually normal at birth, and although bloodphenylalanine levels may rise as early as 4 hours after birth, mental retardation develops gradually, andmay not be apparent for a few months. About 25% if infants have seizures, but over 50% have anabnormal EEG. Microcephaly, prominent maxilla, growth retardation and wide-spaced teeth are found inuntreated children. Even with dietary treatment, some degree of cognitive impairment is seen and can

vary from gross impairment or changes detected on cognitive tests. Cerebral white matter changes areseen in older patients and may reflect a combination of late diagnosis and dietary indiscretion. Diagnosisof classic PKU requires raised Phe levels, normal plasma tyrosine levels, increased urinary Phemetabolites and normal cofactor (tetrahydrobiopterin) concentrations


11/12

An 18 year old man presented with a history of a sudden onset of a frontal headache and photophobia.He had neck stiffness and a temperature of 38C.

Which one of the following findings would suggest a diagnosis of subarachnoid haemorrhage rather thanbacterial meningitis?

Available marks are shown in brackets1 ) a blood neutrophil leucocytosis [0]

2 ) a family history of polycystic renal disease [100]

3 ) a fluctuating conscious level [0]

4 ) a history of diabetes mellitus [0]

5 ) a history of opiate abuse [0]

Comments:

Fluctuating level of consiousness can occur in both meningitis and subarachnoid

haemorrhage (SAH). Hypertension is a risk factor for SAH, but not diabetes. Opiate

abuse does not increase the risk for SAH. Cerebral aneurysm are associated withpolystic kidney disease.

An 18 year-old man is referred with a 6 month history of daily headache which is mostly frontal in locationand occasionally associated with nausea. He has been taking Paracetamol 3g/day, Aspirin 300 mg tdsand Codeine 40mg tds, which has only a temporary effect. He has a 2 year history of depression, treatedwith Paroxetine. No abnormalities were found on examination. What is the most likely diagnosis?


1 ) Analgesic misuse headache [100]

2 ) Cerebral tumor [0]

3 ) Cluster headache [0]

4 ) Headache due to depression [0]

5 ) Migraine [0]

Comments:

The two commonest causes of chronic daily headache are tension type headache and analgesic misuseheadache. The latter is the most likely diagnosis for this patients symptoms. It usually occurs as a resultof chronic use of analgesics such as codeine phosphate and paracetamol. Typically the headache isrelieved with the analgesics just to return in the next hours. The treatment of choice is the slow reductionand withdrawal of analgesics.

A child presents with delayed walking. Which of the following would lead you to suspect a diagnosis ofDuchenne muscular dystrophy.

Available marks are shown in brackets1 ) Calf muscle wasting [0]

2 ) A history of recurrent falls [100]

3 ) Poor feeding since birth [0]

4 ) Urinary incontinence [0]

5 ) Abnormal nerve conduction studies [0]

Comments:


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This form of muscular dystrophy usually manifests in toddlers and early childhood when motor skills aredeveloped such as running and jumping. Pseudohypertrophy of the calf muscles is characteristic. Thefirst symptoms may be delayed walking and frequent falls. Poor feeding is not a feature. Urinaryincontinence is associated with spinal cord and upper motor neurone lesions and not with musculardystrophy. Motor and sensory nerve conduction velocities are normal, but EMG shows myopathicchanges.

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