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A 76-year-old man is admitted with a right hemiparesis. CT scan shows an ischaemic stroke and aspirin 300mg is commenced. In terms of further management in the acute phase, which one of the following values should not be corrected?ia
A.A BP 210/110ia B.A Blood glucose 9.4 mmol/lia C.A Oxygen saturation 94%ia D.A Temp 38.3Cia E.A Blood glucose 2.5 mmol/lia
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Elevated blood pressure should not be treated in the acute phase following a stroke unless complications develop. Other physiological parameters should be kept within normal limits - an aggressive approach with respect to this has been shown to improve outcome Stroke: management sqweqwesf erwrewfsdfs adasd dhe The Royal College of Physicians published guidelines on the diagnosis and management of patients following a stroke in 2004 he earaer aeraer asdsadas eerw dssdfsselleds Selected points relating to the management of transient ischaemic attacks (TIA) include: if a diagnosis of TIA is likely patients should be prescribed aspirin 300mg daily or an alternative antiplatelet immediatelyhe if there has been more than one TIA in a week then patients should be investigated in hospital immediatelyhe
he earaer aeraer asdsadas eerw dssdfsselleds Selected points relating to the management of acute stroke include: blood glucose, hydration, oxygen saturation and temperature should be maintained within normal limitshe blood pressure should not be lowered in the acute phase unless there are complications e.g. hypertensive encephalopathyhe aspirin 300mg orally or rectally should be given as soon as possible if a haemorrhagic stroke has been excludedhe anticoagulation should not be started until an haemorrhagic stroke has been excluded and usually not until 14 days have passed since the strokehe if the cholesterol is > 3.5 mmol/l patients should be commence on a statinhe
A 29-year-old man presents to his GP complaining of visual disturbance. Examination reveals a right superior homonymous quadrantanopia. Where is the lesion most likely to be?ia
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A.A Optic chiasmia B.A Left temporal lobeia C.A Right temporal lobeia D.A Left optic nerveia E.A Left parietal lobeia
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Visual field defects: left homonymous hemianopia means visual field defect to the left, i.e. lesion of right optic tract homonymous quadrantanopias: PITS (Parietal-Inferior, Temporal-Superior) incongruous defects = optic tract lesion; congruous defects= optic radiation lesion or occipital cortex
Visual field defects sqweqwesf erwrewfsdfs adasd dhe Greater detail is required in the MRCP when assessing visual field defects - quadrantanopias are described as are incongruous and congruous defects he earaer aeraer asdsadas eerw dssdfsselleds The 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 dssdfsselleds Homonymous 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 dssdfsselleds Homonymous quadrantanopias superior: lesion of temporal lobehe inferior: lesion of parietal lobehe mnemonic = PITS (Parietal-Inferior, Temporal-Superior)he
he earaer aeraer asdsadas eerw dssdfsselleds Bitemporal hemianopia lesion of optic chiasmhe upper quadrant defect > lower quadrant defect = inferior chiasmal compression, commonly a pituitary tumourhe
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lower quadrant defect > upper quadrant defect = superior chiasmal compression, commonly a craniopharyngiomahe
For patients with Parkinson's disease, which one of the following drugs is most useful in the management of tremor?ia
A.A Apomorphineia B.A Cabergolineia C.A Selegilineia D.A Amantadineia E.A Benzhexolia
Next question Parkinson's disease: management sqweqwesf erwrewfsdfs adasd dhe Currently accepted practice in the management of patients is to delay treatment until the onset of disabling symptoms and then to introduce a dopamine receptor agonist. If patient is elderly, levodopa is sometimes used as an initial treatment he earaer aeraer asdsadas eerw dssdfsselleds Dopamine receptor agonists bromocriptine, apomorphinehe newer agents: ropinirole, cabergoline, pergolidehe
he earaer aeraer asdsadas eerw dssdfsselleds Levodopa usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopaminehe reduced effectiveness with time (usually by 2 years)he unwanted effects: dyskinesia, 'on-off' effect he no use in neuroleptic induced parkinsonismhe
he earaer aeraer asdsadas eerw dssdfsselleds Selegiline MAO-B inhibitorhe reduces dopamine metabolismhe
he earaer aeraer asdsadas eerw dssdfsselleds Antimuscarinics useful for tremorhe reduces inhibition of excitatory cholingeric neuronshe e.g. procyclidine, benzotropine, benzhexolhe
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he earaer aeraer asdsadas eerw dssdfsselleds Amantadine prevents reuptake of dopaminehe
A 27-year-old man presents to A&E with 2 day history of severe headache and pyrexia (38.9C). A CT scan shows petechial haemorrhages in the temporal and inferior frontal lobes. What is the most likely diagnosis?ia
A.A Brain abscessia B.A Meningococcal meningitisia C.A Cerebral malariaia D.A Herpes simplex encephalitisia E.A New variant CJDia
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Herpes simplex encephalitis sqweqwesf erwrewfsdfs adasd dhe Herpes simplex (HSV) encephalitis is a common topic in the MRCP. The virus characteristically affects the temporal lobes - questions may give the result of imaging or describe temporal lobe signs e.g. aphasia he earaer aeraer asdsadas eerw dssdfsselleds Features fever, headache, psychiatric symptoms, seizures, vomitinghe focal features e.g. aphasiahe peripheral lesions (e.g. cold sores) have no relation to presence of HSV encephalitishe
he earaer aeraer asdsadas eerw dssdfsselleds Pathophysiology HSV-1 responsible for 95% of cases in adultshe typically affects temporal and inferior frontal lobeshe
he earaer aeraer asdsadas eerw dssdfsselleds Investigation CSF: lymphocytosis, elevated proteinhe PCR for HSVhe CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patientshe MRI is betterhe EEG pattern: lateralised periodic discharges at 2 Hzhe
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he earaer aeraer asdsadas eerw dssdfsselleds Treatment aciclovirhe
he earaer aeraer asdsadas eerw dssdfsselleds Prognosis treated 25%he untreated 80%he A 27-year-old woman presents to A&E with a one-day history of headache and feeling generally unwell. She is pyrexial at 38.7C and there is no rash. he earaer aeraer asdsadas eerw dssdfsselleds
Serum glucose 5.1 mmol/l he earaer aeraer asdsadas eerw dssdfsselleds Lumbar puncture reveals: he earaer aeraer asdsadas eerw dssdfsselleds
White cells 250 / mm (85% polymorphs) he earaer aeraer asdsadas eerw dssdfsselleds What is the most likely diagnosis?ia
A.A Guillain-Barre syndromeia B.A Viral meningitisia C.A Bacterial meningitisia D.A Cerebral malariaia E.A Tuberculous meningitisia Meningitis: CSF analysis sqweqwesf erwrewfsdfs adasd dhe The table below summarises the characteristic CSF findings in meningitis: he earaer aeraer asdsadas eerw dssdfsselleds Bacterial Appearance Cloudy Viral Clear/cloudy Tuberculous Fibrin web
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Glucose Protein
he earaer aeraer asdsadas eerw dssdfsselleds The Ziehl-Neelsen stain is only 20% sensitive in the detection of tuberculous meningitis and therefore PCR is sometimes used (sensitivity = 75%) he earaer aeraer asdsadas eerw dssdfsselleds *mumps is unusual in being associated with a low glucose level in a proportion of cases
A 33-year-old man presents to his GP complaining of visual disturbance. Examination reveals a bitemporal hemianopia with predominately the upper quadrants being affected. What is the most likely lesion?ia
A.A Craniopharyngiomaia B.A Brainstem lesionia C.A Pituitary macroadenomaia D.A Frontal lobe lesionia E.A Right occipital lesionia
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Bitemporal hemianopia 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
An upper quadrant defect implies inferior chiasmal compression making a pituitary macroadenoma the most likely diagnosis Visual field defects sqweqwesf erwrewfsdfs adasd dhe Greater detail is required in the MRCP when assessing visual field defects - quadrantanopias are described as are incongruous and congruous defects he earaer aeraer asdsadas eerw dssdfsselleds The main points for the exam are: left homonymous hemianopia means visual field defect to the left, i.e. lesion of right optic tracthe
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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 dssdfsselleds Homonymous 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 dssdfsselleds Homonymous quadrantanopias superior: lesion of temporal lobehe inferior: lesion of parietal lobehe mnemonic = PITS (Parietal-Inferior, Temporal-Superior)he
he earaer aeraer asdsadas eerw dssdfsselleds Bitemporal 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
A.A Relapsing-remitting diseaseia B.A Amyotrophic lateral sclerosisia C.A Secondary progressive diseaseia D.A Progressive-relapsing diseaseia E.A Primary progressive diseaseia
Next question Multiple sclerosis sqweqwesf erwrewfsdfs adasd dhe Multiple sclerosis is chronic disorder characterised by demyelination in the central nervous system. he earaer aeraer asdsadas eerw dssdfsselleds Genetics monozygotic twin concordance = 30%he dizygotic twin concordance = 2%he
he earaer aeraer asdsadas eerw dssdfsselleds A variety of subtypes have been identified:
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he earaer aeraer asdsadas eerw dssdfsselleds Relapsing-remitting disease most common form, 70-80% of patients initiallyhe acute attacks (e.g. last 1-2 months) followed by periods of remissionhe
he earaer aeraer asdsadas eerw dssdfsselleds Secondary progressive disease describes relapsing-remitting patients who have deteriorated and have developed neurological signs and symptoms between relapseshe
he earaer aeraer asdsadas eerw dssdfsselleds Primary progressive disease accounts for 10% of patientshe progressive deterioration from onsethe more common in older peoplehe
he earaer aeraer asdsadas eerw dssdfsselleds Progressive-relapsing disease primary progressive patients with superimposed relapsehe
A 21-year-old female is seen in the first seizure clinic in the outpatient department. A decision is made not to start her on anti-epileptic medication. What restrictions on driving should she be informed about?ia
A.A No restrictions but inform DVLAia B.A No restrictions, no need to inform DVLA if not on medicationia C.A Cannot drive for 1 month from date of seizureia D.A Cannot drive for 6 months from date of seizureia E.A Cannot drive for 1 year from date of seizureia
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DVLA: neurological disorders sqweqwesf erwrewfsdfs adasd dhe The guidelines below relate to car/motorcycle use unless specifically stated. For obvious reasons, the rules relating to drivers of heavy goods vehicles tend to be much stricter he earaer aeraer asdsadas eerw dssdfsselleds Specific rules first seizure - 1 year off drivinghe
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stroke - 1 month off drivinghe multiple TIAs over short period of times - 3 months off drivinghe craniotomy - 1 year off driving* he narcolepsy/cataplexy: cease driving on diagnosis, can restart once 'satisfactory control of symptoms'he
he earaer aeraer asdsadas eerw dssdfsselleds *if the tumour is a benign meningioma and there is no seizure history, licence can be reconsidered 6 months after surgery if remains seizure free
A 65-year-old man is referred to the neurology outpatient clinic due to a resting tremor of his right hand. A diagnosis of Parkinson's disease is made. He is otherwise well and is not currently disabled by his symptoms. What is the most appropriate treatment?ia
A.A Selegilineia B.A No treatmentia C.A New generation dopamine receptor agonist e.g. ropiniroleia D.A Conventional dopamine receptor agonist e.g. bromocriptineia E.A Antimuscarinicsia
Next question Parkinson's disease: management sqweqwesf erwrewfsdfs adasd dhe Currently accepted practice in the management of patients is to delay treatment until the onset of disabling symptoms and then to introduce a dopamine receptor agonist. If patient is elderly, levodopa is sometimes used as an initial treatment he earaer aeraer asdsadas eerw dssdfsselleds Dopamine receptor agonists bromocriptine, apomorphinehe newer agents: ropinirole, cabergoline, pergolidehe
he earaer aeraer asdsadas eerw dssdfsselleds Levodopa usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopaminehe reduced effectiveness with time (usually by 2 years)he unwanted effects: dyskinesia, 'on-off' effect he no use in neuroleptic induced parkinsonismhe
he earaer aeraer asdsadas eerw dssdfsselleds Selegiline MAO-B inhibitorhe reduces dopamine metabolismhe
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he earaer aeraer asdsadas eerw dssdfsselleds Antimuscarinics useful for tremorhe reduces inhibition of excitatory cholingeric neuronshe e.g. procyclidine, benzotropine, benzhexolhe
Each one of the following is associated with normal pressure hydrocephalus, except:ia
A.A Papilloedemaia B.A Dementiaia C.A Urinary incontinenceia D.A Gait abnormalityia E.A Enlarged fourth ventricleia
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Normal pressure hydrocephalus sqweqwesf erwrewfsdfs adasd dhe Normal 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 meningitis he earaer aeraer asdsadas eerw dssdfsselleds A classical triad of features is seen urinary incontinencehe dementiahe gait abnormality (may be similar to Parkinson's disease)he
he earaer aeraer asdsadas eerw dssdfsselleds Imaging hydrocephalus with an enlarged fourth ventriclehe
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A 47-year-old man with a known history of schizophrenia is admitted to A&E due to confusion. A bottle of procyclidine tablets are found in his pocket. On examination the temperature is 38.1C with a blood pressure of 155/100 mmHg. Neurological examination reveals a GCS of 13/15 but assessment of his peripheral nervous system is difficult due to generalised increased muscle tone. What is the most likely diagnosis?ia
A.A Neuroleptic malignant syndromeia B.A Procyclidine overdoseia C.A Catatonic schizophreniaia D.A Clozapine induced agranulocytosisia E.A Quetiapine induced rhabdomyolysisia
Next question Neuroleptic malignant syndrome sqweqwesf erwrewfsdfs adasd dhe Neuroleptic malignant syndrome is a rare but dangerous condition seen in patients taking antipsychotic medication. It carries a mortality of up to 10% and can also occur with atypical antipsychotics he earaer aeraer asdsadas eerw dssdfsselleds Features more common in young male patientshe onset usually in first 10 days of treatment or after increasing dosehe pyrexiahe rigidityhe tachycardiahe
he earaer aeraer asdsadas eerw dssdfsselleds A raised creatine kinase is present in most cases. A leukocytosis may also be seen he earaer aeraer asdsadas eerw dssdfsselleds Management stop antipsychotiche IV fluids to prevent renal failurehe dantrolene may be useful in selected caseshe bromocriptine, dopamine agonist, may also be usedhe
A.A Beta-blockeria B.A Alpha-blocker and a partial 5-HT2 agonistia C.A Specific 5-HT1 agonistia D.A 5-HT2 antagonistia E.A Tricyclic antidepressantia
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Migraine: drugs sqweqwesf erwrewfsdfs adasd dhe It should be noted that 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis he earaer aeraer asdsadas eerw dssdfsselleds Acute treatment he earaer aeraer asdsadas eerw dssdfsselleds Standard analgesia may be poorly absorbedhe often combined with anti-emetic e.g. metoclopramide to relieve associated nauseahe
he earaer aeraer asdsadas eerw dssdfsselleds Sumatriptan specific 5-HT1 agonist - opposes vasodilationhe very effectivehe
he earaer aeraer asdsadas eerw dssdfsselleds Ergotamine alpha-blocker and a partial 5-HT1 agonisthe often induces nausea and vomitinghe
he earaer aeraer asdsadas eerw dssdfsselleds he earaer aeraer asdsadas eerw dssdfsselleds Prophylaxis he earaer aeraer asdsadas eerw dssdfsselleds Prophylaxis should be given if patients are experiencing two or more attacks per month. Modern treatment is effect about 60% of patients he earaer aeraer asdsadas eerw dssdfsselleds 5-HT2 antagonists e.g. pizotifen, methysergidehe
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e.g. amitriptylinehe
A.A Oxygen saturationsia B.A PEFRia C.A Flow volume loopia D.A Arterial blood gasesia E.A FVCia
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Guillain-Barre syndrome: management sqweqwesf erwrewfsdfs adasd dhe Guillain-Barre syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni) he earaer aeraer asdsadas eerw dssdfsselleds Management plasma exchangehe IV immunoglobulinshe steroids and immunosuppressants have not been shown to be beneficialhe FVC regularly to monitor respiratory functionhe
he earaer aeraer asdsadas eerw dssdfsselleds Prognosis 20% suffer permanent disability, 5% diehe
Which one of the following is most characteristically associated with a very high protein level in the cerebrospinal fluid?ia
A.A Myasthenia gravisia B.A Multiple sclerosisia C.A New variant CJDia D.A Motor neuron diseaseia E.A Guillain-Barre syndromeia
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Next question Cerebrospinal fluid: raised protein sqweqwesf erwrewfsdfs adasd dhe Normal values of cerebrospinal fluid (CSF) are as follows: pressure = 60-150 mm (patient recumbent)he protein = 0.2-0.4 g/lhe glucose = > 2/3 blood glucosehe cells: red cells = 0, white cells < 5/mmhe
he earaer aeraer asdsadas eerw dssdfsselleds The following conditions are associated with raised protein levels Guillain-Barre syndromehe TB/fungal/bacterial meningitishe spinal block (Froin's syndrome)he viral encephalitishe
What is the first line medication in the treatment of adults with generalised seizures?ia
A.A Gabapentinia B.A Lamotrigineia C.A Sodium valproateia D.A Carbamazepineia E.A Phenytoinia
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Epilepsy medication: first-line generalised seizure: sodium valproate partial seizure: carbamazepine
Epilepsy: treatment sqweqwesf erwrewfsdfs adasd dhe Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present: the patient has a neurological deficithe brain imaging shows a structural abnormalityhe the EEG shows unequivocal epileptic activity he the patient or their family or carers consider the risk of having a further seizure unacceptable he
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he earaer aeraer asdsadas eerw dssdfsselleds Sodium valproate is considered the first line treatment for patients with generalised seizures with carbmazepine used for partial seizures he earaer aeraer asdsadas eerw dssdfsselleds Tonic-clonic seizures sodium valproatehe second line: lamotrigine, carbamazepinehe
he earaer aeraer asdsadas eerw dssdfsselleds Absence seizures (Petit mal) sodium valproate or ethosuximidehe sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsyhe
he earaer aeraer asdsadas eerw dssdfsselleds Myoclonic seizures sodium valproatehe second line: clonazepam, lamotriginehe
he earaer aeraer asdsadas eerw dssdfsselleds Partial seizures carbamazepinehe second line: sodium valproate, lamotriginehe
Which one of the following features is most associated with frontal lobe lesions?ia
A.A Wernicke's aphasiaia B.A Gerstmann's syndromeia C.A Perserverationia D.A Cortical blindnessia E.A Superior homonymous quadrantanopiaia
Next question Brain anatomy sqweqwesf erwrewfsdfs adasd dhe The following neurological disorders/features may allow localisation of a brain lesion: he earaer aeraer asdsadas eerw dssdfsselleds Parietal lobe lesions sensory inattentionhe apraxiashe astereognosis (tactile agnosia) he
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inferior homonymous quadrantanopiahe Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientationhe
he earaer aeraer asdsadas eerw dssdfsselleds Occipital lobe lesions homonymous hemianopiahe cortical blindnesshe visual agnosiahe
he earaer aeraer asdsadas eerw dssdfsselleds Temporal lobe lesion Wernicke's aphasiahe superior homonymous quadrantanopiahe auditory agnosiahe
he earaer aeraer asdsadas eerw dssdfsselleds Frontal lobes lesions expressive (Broca's) aphasiahe disinhibitionhe perserverationhe anosmiahe
Which of the following features is least likely to be found in a patient with tuberose sclerosis?ia
A.A Adenoma sebaceumia B.A Caf?au-lait spotsia C.A Retinal hamartomasia D.A 'Ash-leaf' spotsia E.A Lisch nodulesia
Next question Lisch nodules are seen in neurofibromatosis Tuberous sclerosis sqweqwesf erwrewfsdfs adasd dhe Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. As is neurofibromatosis, the majority of features seen are neuro-cutaneous he earaer aeraer asdsadas eerw dssdfsselleds Cutaneous features depigmented 'ash-leaf' spots which fluoresce under UV lighthe roughened patches of skin over lumbar spine (Shagreen patches)he
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adenoma sebaceum: butterfly distribution over nosehe fibromata beneath nails (subungual fibromata)he caf?au-lait spots* may be seenhe
he earaer aeraer asdsadas eerw dssdfsselleds Neurological features developmental delayhe epilepsy (infantile spasms or partial)he intellectual impairmenthe
he earaer aeraer asdsadas eerw dssdfsselleds Also 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 caf?au-lait spots in 28% of patients An obese 24-year-old female presents with headache and blurred vision to her GP. Examination reveals bilateral blurring of the optic discs but is otherwise unremarkable with no other neurological signs. Blood pressure is 130/74 and she is apyrexial. What is the most likely underlying diagnosis?ia
A.A Multiple sclerosisia B.A Meningococcal meningitisia C.A Brain abscessia D.A Normal pressure hydrocephalusia E.A Idiopathic intracranial hypertensionia
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Obese, young female with headaches / blurred vision think idiopathic intracranial hypertension
The combination of a young, obese female with papilloedema but otherwise normal neurology makes idiopathic intracranial hypertension the most likely diagnosis Idiopathic intracranial hypertension sqweqwesf erwrewfsdfs adasd dhe Idiopathic intracranial hypertension (also known as pseudotumour cerebri and formerly benign intracranial hypertension) is a condition classically seen in young, overweight females. he earaer aeraer asdsadas eerw dssdfsselleds Features
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headachehe blurred visionhe papilloedema (usually present)he enlarged blind spothe sixth nerve palsy may be presenthe
he earaer aeraer asdsadas eerw dssdfsselleds Risk factors obesityhe female sexhe pregnancyhe drugs: oral contraceptive pill, steroids, tetracycline, vitamin A he
he earaer aeraer asdsadas eerw dssdfsselleds Management weight losshe diuretics e.g. acetazolamidehe repeated lumbar puncturehe
A 45-year-old man is noted to have a peripheral motor neuropathy on examination. He has a long history of recurrent abdominal pain. What is the most likely diagnosis?ia
A.A Acute intermittent porphyriaia B.A Myelomaia C.A Huntingdon's diseaseia D.A Lawrence-Moon-Biedl syndromeia E.A Friedreich's ataxiaia
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In the MRCP, neurological signs combined with abdominal pain is acute intermittent porphyria or lead poisoning until proven otherwise
Acute intermittent porphyria sqweqwesf erwrewfsdfs adasd dhe Acute intermittent porphyria (AIP) is a rare autosomal dominant condition caused by a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem. The results in the toxic accumulation of delta aminolaevulinic acid and porphobilinogen. It characteristically presents with abdominal and neuropsychiatric symptoms in 20-40 year olds. AIP is more common in females (5:1) he earaer aeraer asdsadas eerw dssdfsselleds Features abdominal: abdominal pain, vomitinghe neurological: motor neuropathyhe
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psychiatric: e.g. depressionhe hypertension and tachycardia commonhe
he earaer aeraer asdsadas eerw dssdfsselleds Diagnosis classically urine turns deep red on standinghe raised urinary porphobilinogen (elevated between attacks and to a greater extent during acute attacks)he assay of red cells for porphobilinogen deaminasehe raised serum levels of delta aminolaevulinic acid and porphobilinogenhe
Which one of the following infections is most strongly associated with the development of Guillain-Barre syndromeia
A.A Shigellaia B.A Salmonellaia C.A E. coli H7:0157ia D.A Herpes simplexia E.A Campylobacter jejuniia
Next question Campylobacter jejuni is strongly associated with the development of Guillain-Barre syndrome Guillain-Barre syndrome sqweqwesf erwrewfsdfs adasd dhe Guillain-Barre syndrome describes an immune mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni) he earaer aeraer asdsadas eerw dssdfsselleds Pathogenesis cross reaction of antibodies with gangliosides in the peripheral nervous systemhe correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstratedhe anti-GM1 antibodies in 25% of patientshe
he earaer aeraer asdsadas eerw dssdfsselleds Miller-Fisher syndrome variant of Guillain-Barre syndromehe associated with areflexia, ataxia, ophthalmoplegiahe anti-GQ1b antibodies are present in 90% of caseshe
A patient is referred by her GP due to a third nerve palsy associated with a headache. On examination meningism is present. Which one of the following diagnoses needs to be urgently excluded?ia
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A.A Weber's syndromeia B.A Internal carotid artery aneurysmia C.A Multiple sclerosisia D.A Posterior communicating artery aneurysmia E.A Anterior communicating artery aneurysmia
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Given the combination of a headache and third nerve palsy it is important to exclude a posterior communicating artery aneurysm Third nerve palsy sqweqwesf erwrewfsdfs adasd dhe Features eye is deviated 'down and out'he ptosishe pupil may be dilated (sometimes called a 'surgical' third nerve palsy)he
he earaer aeraer asdsadas eerw dssdfsselleds Causes diabetes mellitushe vasculitis e.g. temporal arteritis, SLEhe false localizing sign due to uncal herniation through tentorium if raised ICPhe posterior communicating artery aneurysm (pupil dilated)he cavernous sinus thrombosishe Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokeshe other possible causes: amyloid, multiple sclerosishe
A 34-year-old female presents with collapse and vomiting preceded by an occipital headache of acute onset. On examination she was conscious and alert with photophobia but no neck stiffness. CT brain is reported as normal. Which one of the following investigations would yield the diagnosis?ia
A.A CT brain with contrastia B.A Repeat CT brain in 24hia C.A CSF examinationia D.A Cerebral angiographyia E.A MRI brainia
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Next question Subarachnoid haemorrhage sqweqwesf erwrewfsdfs adasd dhe Causes 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 dssdfsselleds Investigations CT: negative in 5%he LP: done after 12 hrs (allowing time for xanthochromia to develop)he
he earaer aeraer asdsadas eerw dssdfsselleds Complications rebleeding (in 30%)he obstructive hydrocephalus (due to blood in ventricles)he vasospasm leading to cerebral ischaemiahe
he earaer aeraer asdsadas eerw dssdfsselleds Management 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 A 45-year-old man presents to A&E following the sudden onset of pain in his right eye whilst hammering a nail into the wall. The pain is described as severe with occasional exacerbations. On examination he has a mild right ptosis and small right pupil. What is the most likely diagnosis?ia
A.A Trigeminal neuralgiaia B.A Glaucomaia C.A Carotid artery dissectionia D.A Cluster headacheia E.A Migraineia
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Next question This patient has Horner's syndrome caused by a carotid artery dissection. This may be caused by relatively benign trauma to the neck such as hyperextension whilst doing DIY Horner's syndrome sqweqwesf erwrewfsdfs adasd dhe Features miosis (small pupil)he ptosishe enophthalmos (sunken eye) he anhydrosis (loss of sweating one side)he
he earaer aeraer asdsadas eerw dssdfsselleds Distinguishing between causes heterochromia (difference in iris colour) is seen in congenital Horner'she anhydrosis: see belowhe
he earaer aeraer asdsadas eerw dssdfsselleds Anhydrosis if affects head, arm and trunk suggests central lesionhe if affects just face suggests pre-ganglionic lesionhe if absent suggests post-ganglionic lesionhe
he earaer aeraer asdsadas eerw dssdfsselleds Central causes (from origin of sympathetic supply) strokehe syringomyeliahe multiple sclerosishe tumourhe encephalitishe
he earaer aeraer asdsadas eerw dssdfsselleds Pre-ganglionic causes Pancoast's tumourhe cervical ribhe thyroidectomyhe traumahe
he earaer aeraer asdsadas eerw dssdfsselleds Post-ganglionic causes carotid artery dissectionhe internal carotid aneurysmhe cavernous sinus thrombosishe
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Each of the following are causes of peripheral neuropathy. Which one is associated with predominately motor loss?ia
A.A Vitamin B12 deficiencyia B.A Guillain-Barre syndromeia C.A Uraemiaia D.A Diabetesia E.A Leprosyia
Next question Peripheral neuropathy sqweqwesf erwrewfsdfs adasd dhe Peripheral neuropathy may be divided into conditions which predominately cause a motor or sensory loss he earaer aeraer asdsadas eerw dssdfsselleds Predominately motor loss Guillain-Barre syndromehe porphyriahe lead poisoninghe hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth he chronic inflammatory demyelinating polyneuropathy (CIDP)he diphtheriahe
he earaer aeraer asdsadas eerw dssdfsselleds Predominately sensory loss diabeteshe uraemiahe leprosyhe alcoholismhe vitamin B12 deficiencyhe amyloidosishe
he earaer aeraer asdsadas eerw dssdfsselleds Alcoholic neuropathy secondary to both direct toxic effects and reduced absorption of B vitaminshe sensory symptoms typically present prior to motor symptomshe
he earaer aeraer asdsadas eerw dssdfsselleds Vitamin B12 deficiency subacute combined degeneration of spinal cordhe dorsal column usually affected first (joint position, vibration) prior to distal paraesthesiahe
Which part of the brain does herpes simplex encephalitis characteristically affect?ia
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A.A Temporal lobeia B.A Parietal lobeia C.A Occipital lobeia D.A Cerebellumia E.A Medullaia
Next question
The inferior frontal lobe may also be affected Herpes simplex encephalitis sqweqwesf erwrewfsdfs adasd dhe Herpes simplex (HSV) encephalitis is a common topic in the MRCP. The virus characteristically affects the temporal lobes - questions may give the result of imaging or describe temporal lobe signs e.g. aphasia he earaer aeraer asdsadas eerw dssdfsselleds Features fever, headache, psychiatric symptoms, seizures, vomitinghe focal features e.g. aphasiahe peripheral lesions (e.g. cold sores) have no relation to presence of HSV encephalitishe
he earaer aeraer asdsadas eerw dssdfsselleds Pathophysiology HSV-1 responsible for 95% of cases in adultshe typically affects temporal and inferior frontal lobeshe
he earaer aeraer asdsadas eerw dssdfsselleds Investigation CSF: lymphocytosis, elevated proteinhe PCR for HSVhe CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patientshe MRI is betterhe EEG pattern: lateralised periodic discharges at 2 Hzhe
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he earaer aeraer asdsadas eerw dssdfsselleds Prognosis treated 25%he untreated 80%he
Which one of the following drugs is used in the management of multiple sclerosis?ia
A.A Beta-interferonia B.A Gamma-interferonia C.A Infliximabia D.A Rituximabia E.A Alpha-interferonia
Next question Multiple sclerosis: management sqweqwesf erwrewfsdfs adasd dhe Treatment in multiple sclerosis is focused at reducing the frequency and duration of relapses. There is no cure. High dose steroids (e.g. IV methylprednisolone) may be given for 3-5 days to shorten the length of an acute relapse. Baclofen is helpful in controlling spasticity. Hallucinations are occasionally seen on the withdrawal of baclofen he earaer aeraer asdsadas eerw dssdfsselleds Beta-interferon has been shown to reduce the relapse rate by up to 30%. Certain criteria have to be met before it is used: relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaidedhe secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided)he reduces number of relapses and MRI changes, however doesn't reduce overall disabilityhe
A 52-year-old man is prescribed apomorphine for Parkinson's disease. What is the mechanism of action?ia
A.A Dopamine receptor agonistia B.A Dopamine receptor antagonistia C.A MAO-B inhibitoria D.A Decarboxylase inhibitoria E.A Antimuscarinicia
Next question Apomorphine is one of the older dopamine receptor agonists. Newer agents such as ropinirole and cabergoline have since been developed
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Parkinson's disease: management sqweqwesf erwrewfsdfs adasd dhe Currently accepted practice in the management of patients is to delay treatment until the onset of disabling symptoms and then to introduce a dopamine receptor agonist. If patient is elderly, levodopa is sometimes used as an initial treatment he earaer aeraer asdsadas eerw dssdfsselleds Dopamine receptor agonists bromocriptine, apomorphinehe newer agents: ropinirole, cabergoline, pergolidehe
he earaer aeraer asdsadas eerw dssdfsselleds Levodopa usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to prevent peripheral metabolism of levodopa to dopaminehe reduced effectiveness with time (usually by 2 years)he unwanted effects: dyskinesia, 'on-off' effect he no use in neuroleptic induced parkinsonismhe
he earaer aeraer asdsadas eerw dssdfsselleds Selegiline MAO-B inhibitorhe reduces dopamine metabolismhe
he earaer aeraer asdsadas eerw dssdfsselleds Antimuscarinics useful for tremorhe reduces inhibition of excitatory cholingeric neuronshe e.g. procyclidine, benzotropine, benzhexolhe
A 25-year-old female with a history of bilateral vitreous haemorrhage is referred due to progressive ataxia. What is the likely diagnosis?ia
A.A Neurofibromatosis type Iia B.A Neurofibromatosis type IIia C.A Tuberose sclerosisia D.A Von Hippel-Lindau syndromeia E.A Sarcoidosisia
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Next question Retinal and cerebellar haemangiomas are key features of Von Hippel-Lindau syndrome. Retinal haemangiomas are bilateral in 25% of patients and may lead to vitreous haemorrhage Von Hippel-Lindau syndrome sqweqwesf erwrewfsdfs adasd dhe Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3 he earaer aeraer asdsadas eerw dssdfsselleds Features cerebellar haemangiomas: he retinal haemangiomas: vitreous haemorrhagehe renal cysts (premalignant)he phaeochromocytomahe
Each one of the following is associated with the development of chorea, except:ia
A.A Haemochromatosisia B.A Ataxic telangiectasiaia C.A Carbon monoxide poisoningia D.A SLEia E.A Huntingdon's diseaseia
Next question Chorea sqweqwesf erwrewfsdfs adasd dhe Chorea describes rapid, jerky movements which often move from one part of the body to another. Slower, sinuous movement of the limbs is termed athetosis. Chorea is caused by damage to the basal ganglia, especially the caudate nucleus he earaer aeraer asdsadas eerw dssdfsselleds Causes 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 features is not associated with an oculomotor nerve palsy?ia
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A.A Miosisia B.A Ptosisia C.A Eye is deviated 'down and out'ia D.A Pain if due to a posterior communicating artery aneurysmia E.A Diplopiaia
Next question
Ptosis + dilated pupil = third nerve palsy; ptosis + constricted pupil = Horner's
Oculomotor nerve palsy is typically associated with a dilated pupil Third nerve palsy sqweqwesf erwrewfsdfs adasd dhe Features eye is deviated 'down and out'he ptosishe pupil may be dilated (sometimes called a 'surgical' third nerve palsy)he
he earaer aeraer asdsadas eerw dssdfsselleds Causes diabetes mellitushe vasculitis e.g. temporal arteritis, SLEhe false localizing sign due to uncal herniation through tentorium if raised ICPhe posterior communicating artery aneurysm (pupil dilated)he cavernous sinus thrombosishe Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokeshe other possible causes: amyloid, multiple sclerosishe
A.A Chromosome 4ia B.A Chromosome 11ia C.A Chromosome 16ia D.A Chromosome 17ia E.A Chromosome 22ia
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Next question One of our registered users sent the following mnemonic to us: NF1: chromosome 17 - as neurofibromatosis has 17 charactershe NF2: chromosome 22 - all the 2'she
Neurofibromatosis sqweqwesf erwrewfsdfs adasd dhe Overview two types, NF1 and NF2he both autosomal dominanthe
he earaer aeraer asdsadas eerw dssdfsselleds NF1 also known as von Recklinghausen's syndromehe caused by gene mutation on chromosome 17 which codes for neurofibrominhe affects 1 in 4,000he
he earaer aeraer asdsadas eerw dssdfsselleds NF2 caused by gene mutation on chromosome 22he affects 1 in 100,000he
he earaer aeraer asdsadas eerw dssdfsselleds NF1 features caf-au-lait spots (>= 6)he axillary/groin freckleshe peripheral neurofibromashe iris: Lisch nodules in > 90%he
he earaer aeraer asdsadas eerw dssdfsselleds NF2 features bilateral acoustic neuromashe
Which one of the following is least likely to produce a lymphocytosis in the cerebrospinal fluid?ia
A.A Systemic lupus erythematousia B.A Guillain-Barre syndromeia C.A Viral encephalitisia D.A Partially treated bacterial meningitisia
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E.A Behcet's syndromeia
Next question Cerebrospinal fluid: raised lymphocytes sqweqwesf erwrewfsdfs adasd dhe Normal values of cerebrospinal fluid (CSF) are as follows: pressure = 60-150 mm (patient recumbent)he protein = 0.2-0.4 g/lhe glucose = > 2/3 blood glucosehe cells: red cells = 0, white cells < 5/mmhe
he earaer aeraer asdsadas eerw dssdfsselleds The following conditions are associated with raised lymphocytes viral meningitis/encephalitishe TB meningitishe partially treated bacterial meningitishe Lyme diseasehe Behcet's, SLEhe lymphoma, leukaemiahe
Which one of the following features is most associated with temporal lobe lesions?ia
A.A Astereognosisia B.A Auditory agnosiaia C.A Visual agnosiaia D.A Disinhibitionia E.A Expressive (Broca's) aphasiaia
Next question Brain anatomy sqweqwesf erwrewfsdfs adasd dhe The following neurological disorders/features may allow localisation of a brain lesion: he earaer aeraer asdsadas eerw dssdfsselleds Parietal 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
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he earaer aeraer asdsadas eerw dssdfsselleds Occipital lobe lesions homonymous hemianopiahe cortical blindnesshe visual agnosiahe
he earaer aeraer asdsadas eerw dssdfsselleds Temporal lobe lesion Wernicke's aphasiahe superior homonymous quadrantanopiahe auditory agnosiahe
he earaer aeraer asdsadas eerw dssdfsselleds Frontal lobes lesions expressive (Broca's) aphasiahe disinhibitionhe perserverationhe anosmiahe
A.A Mild mental impairmentia B.A Round faceia C.A Frontal baldingia D.A Myotoniaia E.A Cataractsia
Next question Myotonic dystrophy sqweqwesf erwrewfsdfs adasd dhe Inherited myopathy with features developing at around 20-30 years old. Affects skeletal, cardiac and smooth muscle he earaer aeraer asdsadas eerw dssdfsselleds Genetics autosomal dominanthe a trinucleotide repeat disorderhe CTG repeat at the end of the DPMK gene on chromosome 19he
he earaer aeraer asdsadas eerw dssdfsselleds Facial features myotonic facies (long, 'haggard' appearance)he frontal baldinghe
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bilateral ptosishe cataractshe dysarthriahe
he earaer aeraer asdsadas eerw dssdfsselleds Other features myotonia (tonic spasm of muscle)he weakness of arms and legs (distal initially)he mild mental impairmenthe diabetes mellitushe testicular atrophyhe cardiomyopathyhe dysphagiahe
A 34-year-old man is reviewed in the neurology clinic. He has been established on sodium valproate for primary generalised epilepsy. Despite now taking a therapeutic dose he continues to have seizures and is troubled by weight gain since starting sodium valproate. He asks to stop the his current medication and try a different drug. Which one of the following drugs would be the most appropriate second-line treatment?ia
A.A Lamotrigineia B.A Ethosuximideia C.A Pregabalinia D.A Gabapentinia E.A Tiagabineia
Next question Monotherapy with another drug should be attempted before combination therapy is started. Caution should be exercised when combining sodium valproate and lamotrigine as serious skin rashes such as Steven-Johnson's syndrome may be provoked Epilepsy: treatment sqweqwesf erwrewfsdfs adasd dhe Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present: the patient has a neurological deficithe brain imaging shows a structural abnormalityhe the EEG shows unequivocal epileptic activity he the patient or their family or carers consider the risk of having a further seizure unacceptable he
he earaer aeraer asdsadas eerw dssdfsselleds Sodium valproate is considered the first line treatment for patients with generalised seizures with carbmazepine used for partial seizures he earaer aeraer asdsadas eerw dssdfsselleds Tonic-clonic seizures
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sodium valproatehe second line: lamotrigine, carbamazepinehe
he earaer aeraer asdsadas eerw dssdfsselleds Absence seizures (Petit mal) sodium valproate or ethosuximidehe sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsyhe
he earaer aeraer asdsadas eerw dssdfsselleds Myoclonic seizures sodium valproatehe second line: clonazepam, lamotriginehe
he earaer aeraer asdsadas eerw dssdfsselleds Partial seizures carbamazepinehe second line: sodium valproate, lamotriginehe
As part of a routine cranial nerve examine the following results are obtained: he earaer aeraer asdsadas eerw dssdfsselleds Rinne's test: Air conduction > bone conduction in both ears Weber's test: Localises to the right side he earaer aeraer asdsadas eerw dssdfsselleds What do these tests imply?ia
A.A Left conductive deafnessia B.A Normal hearingia C.A Right conductive deafnessia D.A Right sensorineural deafnessia E.A Left sensorineural deafnessia
Next question Rinne's and Weber's test sqweqwesf erwrewfsdfs adasd dhe Performing both Rinne's and Weber's test allows differentiation of conductive and sensorineural deafness he earaer aeraer asdsadas eerw dssdfsselleds Rinne's test
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tuning fork is placed over mastoid process, followed by repositioning just over external acoustic meatushe air conduction (AC) is normally better than bone conduction (BC)he if BC > AC then conductive deafnesshe
he earaer aeraer asdsadas eerw dssdfsselleds Weber's test tuning fork is placed over middle of forehead, patient is asked which side is loudesthe in unilateral sensorineural deafness, sound is localised to the 'good' sidehe in unilateral conductive deafness, sound is localised to the 'bad' sidehe
A.A Ataxiaia B.A Confusionia C.A Ophthalmoplegiaia D.A Confabulationia E.A Nystagmusia
Next question An inability to acquire new memories and confabulation suggests the development of Korsakoff's syndrome Wernicke's encephalopathy sqweqwesf erwrewfsdfs adasd dhe Wernicke's encephalopathy is a neuropsychiatric disorder caused by thiamine deficiency which is most commonly seen in alcoholics. Rarer causes include: persistent vomiting, stomach cancer, dietary deficiency. A classic triad of nystagmus, ophthalmoplegia and ataxia may occur. In Wernicke's encephalopathy petechial haemorrhages occur in a variety of structures in the brain including the mamillary bodies and ventricle walls he earaer aeraer asdsadas eerw dssdfsselleds Features nystagmushe ophthalmoplegiahe ataxiahe confusion, altered GCShe peripheral sensory neuropathyhe
he earaer aeraer asdsadas eerw dssdfsselleds Investigations decreased red cell transketolasehe MRIhe
he earaer aeraer asdsadas eerw dssdfsselleds Treatment is with urgent replacement of thiamine