| 1. The following are true of dementia: | |
| A. Incidence is 5 % in the under 65’s | F |
| B. Incidence is about 30 % in the over 85’s | T |
| C. Alzheimer’s disease is the most common cause | T |
| D. Frontal lobe dementia is the second most common cause in the over 65’s | F |
| E. Reversible dementias account for about 5 % of all dementias | T |
| 2. Regarding the aetiology of Alzheimer’s disease: | |
| A. Females are affected twice as commonly as males | T |
| B. Familial forms are autosomal dominant | T |
| C. The presenilin-2 gene is on chromosome 14 | F |
| D. The gene for presenilin-1 is on chromosome 19 | F |
| E. Trauma is a risk factor | T |
| 3. Regarding the aetiology of Alzheimer’s disease: | |
| A. The apolipoprotein e gene is on chomosome 19 | T |
| B. The Beta-amyloid precursor protein is on chromosome 21 | T |
| C. Trisomy 21 is a risk factor | T |
| D. Presenilin-1 causes about 50 % of early onset dementias | T |
| E. Late onset dementia is associated with alpha-1 macroglobulin | F |
| 4. Pathological findings in Alzheimer’s include: | |
| A. Loss of cortical neurones | T |
| B. Amyloid plaques are the critical pathological feature of AD | T |
| C. Relative sparing of the outer 3 layers of cortex | F |
| D. Gliosis | T |
| E. Granulovacuolar degeneration | T |
| 5. Neurochemical findings in Alzheimer’s include: | |
| A. Cholinergic loss in the locus coeruleus | F |
| B. Serotinergic neuron loss correlates with depression | F |
| C. Noradrenergic loss is most marked in early-onset dementia | T |
| D. Increased GABA | F |
| E. Decreased somatostatin and CK | T |
| 6. Clinical features of Alzheimer’s dementia may include: | |
| A. Long-term memory loss | T |
| B. Disorders of Language and praxis | T |
| C. Depression | T |
| D. Loss of primitive reflexes | F |
| E. Persecutory delusion | T |
| 7. Investigations in AD may show: | |
| A. Increase in the dominant alpha rhythm on EEG | F |
| B. Shortening of the P300 potential | F |
| C. Reduced metabolism in the temporal lobes on PET | T |
| D. Reduction in the temporal lobe volume | T |
| E. Loss of theta and delta activity on EEG | F |
| 8. Lewy body dementia: | |
| A. Unlike AD, does not result in loss of cholinergic function | F |
| B. Is characterized by Lewy bodies in the cortex and substantia nigra | T |
| C. Results in increased dopaminergic function | F |
| D. May result in hallucinations and delusions | T |
| E. Fluctuations in the presentation exclude this diagnosis | F |
| 9. Multi-infarct dementia: | |
| A. Is more common in men | T |
| B. Has been linked to an autosomal recessive heritability | F |
| C. Causes cerebral atrophy and ventriculomegaly | T |
| D. Can be rated using the Hatchinski index | T |
| E. Can result in seizures | T |
| 10. In multi-infarct dementia: | |
| A. Depression is rare | F |
| B. Shows a slow, gradual deterioration | F |
| C. PET shows asymmetric changes in the cortex | T |
| D. SPECT shows increased blood flow | F |
| E. MRI shows areas of multiple infarction | T |
| 11. Binswanger’s disease: | |
| A. Is more properly called subcortical arteriosclerotic encephalopathy | T |
| B. Is characterized by abnormal pyramidal function | T |
| C. Gait is normal | F |
| D. Sphincter disturbance is common | T |
| E. MRI shows high intensity lesions distributed evenly throughout the cortex | F |
| 12. Pick’s disease: | |
| A. Is more common in men | F |
| B. Has been linked to an autosomal dominant gene | T |
| C. Is characterized by changes in personality and behaviour | T |
| D. CT shows enlargement of the frontal horns | T |
| E. EEG shows abnormalities in a similar pattern to Alzheimer’s | F |
| 13. Pathological findings in Pick’s disease may include: | |
| A. Atrophy of the frontal and parietal lobes | F |
| B. ‘Knife-blade’ atrophy | T |
| C. Senile plaques | F |
| D. Neurofibrillary tangles | F |
| E. Neuronal loss in the outer cortex | T |
| 14. Frontal lobe dementia: | |
| A. Is a common cause of early-onset dementia | T |
| B. Is more common in men | T |
| C. There is rarely a family history | F |
| D. Is easily seen on EEG | F |
| E. Pathological findings include gliosis and spongiosis | T |
| 15. Huntington’s chorea: | |
| A. Is more common in men | F |
| B. Is due to an autosomal dominant gene | T |
| C. Shows incomplete penetrance | F |
| D. The gene lies on chromosome 4 | T |
| E. Usually presents in the 3rd or 4th decade | T |
| 16. Huntington’s chorea: | |
| A. Demonstrates ‘imprinting’ | T |
| B. Demonstrates ‘anticipation’ | T |
| C. The gene codes for GABA | F |
| D. Results in reduced levels of GABA in the caudate nucleus | T |
| E. Decreased levels of dopamine in the basal ganglia | F |
| 17. Clinical features of Huntington’s disease may include: | |
| A. Dysarthria | T |
| B. Epilepsy | T |
| C. Paranoia and schizophreniform illness | T |
| D. Increased risk of suicide | T |
| E. Depression | T |
| 18. Findings in Huntington’s disease include: | |
| A. Caudate atrophy | T |
| B. Increased blood flow in the neostriatum | F |
| C. EEG shows spike-wave activity | F |
| D. Reduced striatal metabolism | T |
| E. Atrophy of the basal ganglia | T |
| 19. Prion diseases: | |
| A. Are more common in women | F |
| B. Are encoded on chromosome 10 | F |
| C. Individuals with the E4 apolipoprotein allele are at reduced risk | F |
| D. The familial form is autosomal recessive | F |
| E. Usually have a normal EEG | F |
| 20. Normal pressure hydrocephalus: | |
| A. Is more common in the pre-senium | F |
| B. May be preceded by a history of head injury | T |
| C. Is characterized by impairment of memory, pyramidal function, and attention | F |
| D. CT shows periventricular lucencies and abnormal sulci | F |
| E. Is due to blockage of the ventricular system | F |
| 21. The following are true of head injury: | |
| A. The duration of post-traumatic amnesia correlates with the extent of brain injury | T |
| B. The duration of retrograde amnesia correlates with psychiatric disability | F |
| C. Frontal lobe syndrome is due to brain damage | T |
| D. Depression is due to brain damage | F |
| E. Aggressive tendencies are not due to brain damage | F |
| 22. Regarding psychiatric illness following head injury: | |
| A. Schizophrenia is more common in mild injuries | T |
| B. Affective illness is more common | T |
| C. There is a higher incidence of hypomania than depressive psychosis | F |
| D. Suicide is more common | T |
| E. Suicide is associated with frontal and temporal lobe damage | T |
| 23. In ‘Punch drunk’ syndrome: | |
| A. Morbid jealousy is more common | T |
| B. Pyramidal signs are characteristic | T |
| C. CT scan shows cerebellar atrophy and normal ventricles | F |
| D. Pathological changes are similar to normal ageing | F |
| E. Commonly occurs in boxers | T |
| 24. The following symptoms are commonly seen in frontal lobe lesions: | |
| A. Magnetism | T |
| B. Contralateral optic atrophy | F |
| C. Ipsilateral spastic paresis | F |
| D. ‘Witzelsucht’ | T |
| E. Dyspraxia | F |
| 25. The following are seen in lesions of the non-dominant parietal lobe: | |
| A. Dysphasia | F |
| B. Prosopagnosia | T |
| C. Alexia | F |
| D. Constructional dyspraxia | T |
| E. Body image disorders | F |
| 26. Features of Gerstmann’s syndrome may include: | |
| A. Bilateral parietal lobe lesion | F |
| B. Right-left disorientation | T |
| C. Dysgraphaesthesia | F |
| D. Finger agnosia | T |
| E. Dysgraphia | T |
| 27. Dominant temporal lobe lesions may cause: | |
| A. Impaired visual memory | F |
| B. Homonymous hemianopia | T |
| C. Impaired verbal memory | T |
| D. Impaired visual memory | F |
| E. Dysprosody | F |
| 28. Bilateral temporal lobe lesions may cause: | |
| A. Amnesic syndrome | T |
| B. Cortical blindness | F |
| C. Hyperorality | T |
| D. Hypersexuality | T |
| E. Social-emotional agnosia | T |
| 29. Features of occipital lobe lesions may include: | |
| A. Complex visual hallucinations | T |
| B. Visual field defects | T |
| C. Anton’s syndrome | T |
| D. Sensory neglect | F |
| E. Anomia | F |
| 30. Regarding Multiple Sclerosis : | |
| A. It is more common in women | T |
| B. Survival is less in women | F |
| C. Survival is greater if onset is at a younger age | T |
| D. Lhermitte phenemenon is almost pathognomonic in a young person | T |
| E. Visual evoked potentials are abnormal in 10 - 20 % | F |
| 31. In Multiple sclerosis : | |
| A. Depression is about as common as in the general population | F |
| B. Dementia can occur | T |
| C. IgG ratios are lower than normal | F |
| D. Oligoclonal bands are seen in the IgM region on electrophoresis | F |
| E. Epilepsy is more common than normal | T |
| 32. The following are characteristics of Parkinson’s disease : | |
| A. Bradykinesia | T |
| B. Intention tremor | F |
| C. Cogwheel rigidity | T |
| D. Impaired postural reflexes | T |
| E. Cognitive impairment | F |
| 33. In Parkinson’s disease : | |
| A. The risk decreases with age | F |
| B. It is more common in females | F |
| C. Smoking has a negative association | T |
| D. Pesticide exposure is a risk factor | T |
| E. There is a loss of axons in the substantia nigra | F |
| 34. In Parkinson’s disease : | |
| A. Rigidity is due to enhancement of long-latency stretch reflexes | T |
| B. Tremor is associated with alpha-gamma co-activation | T |
| C. Tremor is present in only 25 % of patients | F |
| D. Dementia is seen in 15-20 % | T |
| E. Depressive illness is seen in 85 % of patients | F |
| 35. Regarding Wilson’s disease : | |
| A. It is autosomal recessive | T |
| B. Onset is always in childhood | F |
| C. Kayser-Fleischer rings are due to copper deposition in the iris | F |
| D. Psychiatric symptoms are related to the severity of the hepatic involvement | F |
| E. Around half of all patients will have psychiatric symptoms | T |
| 36. After Cerebrovascular Incidents: | |
| A. Residual deficits occur in 90 % | T |
| B. Personality change is rare | F |
| C. Depression is common with left hemisphere lesions | T |
| D. Anxiety is common | T |
| E. Emotional lability is more common in those with right temporal lesions | F |
| 37. Multiple sclerosis: | |
| A. Is more common in men | F |
| B. Is more common in the northern hemisphere | T |
| C. Progressive conditions are more common than relapsing-remitting ones | F |
| D. Depression is common | T |
| E. Dementia is not a feature | F |
| 38. Regarding the features of MS: | |
| A. Epilepsy is less common than in the general population | F |
| B. Erectile dysfunction can occur | T |
| C. CSF examination shows oligoclonal bands in the IgA region | F |
| D. Optic neuritis results in painless visual disturbance | F |
| E. Symptoms can be improved by a hot bath | F |
| 39. Parkinson’s disease: | |
| A. Is less common in women | T |
| B. Is associated with pesticide exposure | T |
| C. Is less common in non-smokers | F |
| D. There is loss of dopaminergic cell bodies in the substantia nigra | T |
| E. Lewy bodies are often seen | T |
| 40. Clinical features of Parkinson’s disease include: | |
| A. Increased rate of blinking | F |
| B. Tremor inhibited by purposeful movement | T |
| C. Dementia | T |
| D. Higher incidence of depressive illness | T |
| E. Dementia is less common in men | F |
| 41. In Klinefelter’s syndrome : | |
| A. The usual karyotype is XYY | F |
| B. Urinary gonadotrophin levels are reduced | F |
| C. Intelligence is normal | F |
| D. Criminal behaviour is an established association | F |
| E. EEG shows slowed alpha waves | T |
| 42. In Turner’s syndrome : | |
| A. There is secondary amenorrhoea | F |
| B. Congenital rectal abnormalities are common | T |
| C. Verbal intelligence is normal | T |
| D. Visuospatial abilities are also normal | F |
| E. The incidence of psychotic illness is increased | F |
| 43. In Wilson’s disease : | |
| A. Most cases present in the first two decades | T |
| B. It is an autosomal dominant condition | F |
| C. Hepatocytes are forced to store copper due to increased levels of caeruloplasmin | F |
| D. Diagnosis is by raised plasma caeruloplasmin levels | F |
| E. Urinary copper excretion is usually elevated | T |
| 44. Symptoms of Wilson’s disease include : | |
| A. Changes in personality and behaviour | T |
| B. Epilepsy | T |
| C. Rigidity and dystonia | T |
| D. Visual symptoms | F |
| E. Cognitive impairment | T |
| 45. The following are true in Wilson’s disease : | |
| A. The ventricles are normal | F |
| B. The basal ganglia show lesions on CT | T |
| C. Treatment is with penicillamine | T |
| D. It is important to avoid vitamin B6 during treatment | F |
| E. Neurological disturbance responds better than hepatic disturbance | T |
| 46. The following are true for porphyria : | |
| A. Haem precursors are readily oxidized to porphyrins | T |
| B. All cases are autosomal recessive | F |
| C. Abdominal pain and neuropathy are rarely seen | F |
| D. Episodes can be precipitated by tricyclic antidepressants | T |
| E. EEG is always abnormal | F |
| 47. Regarding mitochondrial myopathy : | |
| A. Transmission is usually maternal | T |
| B. Most cases present after the age of 20 | F |
| C. Smooth muscle biopsy (such as from the rectum) is diagnostic | F |
| D. Limb weakness can occur on its own | T |
| E. It may present as a chronic fatigue syndrome | T |
| 48. In Neuroacanthocytosis : | |
| A. Red blood cells are abnormal | T |
| B. Tics are seen | T |
| C. Frontal lobe pictures can be seen | T |
| D. Intellectual impairment rules out the diagnosis | F |
| E. Shrinkage of the caudate head on CT can differentiate this condition from Huntington’s chorea | F |
| F. May present with OCD | T |
| G. Anxiety and depression are frequent | T |
| 49. The following are true for Cerebral Ceroid Lipofuscinosis : | |
| A. It is also called Leigh disease | F |
| B. It can occur in infancy | T |
| C. Only autosomal dominant forms have been recognized | F |
| D. It commonly presents as dementia with motor symptoms | T |
| E. Death is within 6-12 months | F |
| 50. In Subacute Necrotizing Encephalomyopathy : | |
| A. It is inherited as an autosomal recessive condition | T |
| B. It is due to a disturbance of thiamine metabolism | T |
| C. The condition usually presents in adolescence | F |
| D. Death is prolonged | F |
| E. It can also occur in adults, when it presents as impaired intellect and motor symptoms | T |
| 51. Hallorvorden-Spatz syndrome : | |
| A. Is a rare pyramidal disorder | F |
| B. Typically presents at a young age | T |
| C. Changes in personality also occur | T |
| D. EEG is normal | F |
| E. CT scan resembles Huntington’s chorea, with prominent atrophy of the basal ganglia | T |
| 52. The following are true of the Sphingolipidoses : | |
| A. They are autosomal recessive conditions | T |
| B. Patients with Gaucher’s disease have a greatly reduced life-span | F |
| C. Most cases of Niemann-Pick disease present in early life | T |
| D. Jewish people have a reduced incidence of Niemann-Pick disease | F |
| E. Tay Sachs disease usually results in death before the age of 2 | T |