Sample Questions
1. A 70 year-old man, who was previously healthy, sustained a
stroke in the left side of this structure. Examination reveals dysarthria,
Horner's syndrome, and ipsilateral facial hypalgesia with crossed body
hypalgesia. Which other symptom will most likely be present?
a. Aphasia
b. Dementia
c. Intention tremor
d. Diplopia
e. Epilepsy
Answer c: The structure that has been sketched is the medulla, which
should be identified by the paired scalloped, concave nuclei. The lateral
medulla contains the cerebellar outflow tracts and several important brainstem
nuclei. He has developed the "lateral medullary syndrome." As
part of that syndrome, injury of the cerebellar outflow tract will cause
an ipsilateral intention tremor. More important, lesions of the brainstem
generally do not cause aphasia, dementia, or epilepsy - all manifestations
of cerebral injury.
2. Regarding genetic diseases that result from
excessive trinucleotide repeats, which of the following statements is false?
a. Juvenile onset Huntington's disease is an example.
b. Characteristically in a phenomenon "anticipation,"
successive generations show signs of the disease at an earlier age.
c. Maternal rather than paternal inheritance confers a greater
degree of illness.
d. Huntington's disease developing in middle-age men is an example.
Answer c: Paternal rather than maternal inheritance confers a greater
degree of illness. Genetic material in sperm is apparently more unstable
than in eggs. Myotonic dystrophy, fragile-X syndrome, certain spinocerebellar
ataxias and Huntington's disease result from expansion of abnormal genes
characterized by excessive trinucleotide repeats.
3. A 4 year-old mentally retarded boy eats compulsively
and voraciously. He eventually develops extreme obesity. Other than poor
coordination, his neurologic examination shows no particular abnormality.
Also, his routine blood tests, an MRI of the brain, and endocrine tests
are normal. His father has mental impairment and obesity, but not to the
same extent. At this point, which of the following illnesses is indicated?
a. Prader-Willi syndrome
b. Gilles de la Tourette syndrome
c. Trisomy 21
d. A hypothalamic tumor
Answer a: While all the choices might be considered, the familial incidence,
lack of neurologic abnormalities, and normal MRI and endocrine studies
eliminate almost all of them. The most likely condition is the Prader-Willi
syndrome, which results from abnormal genetic material that is usually
inherited from the father.
4. A 72 year-old man developed dementia. Then
he became reticent, but when he spoke his speech was aggressive and contained
many sexual innuendos. He was ambulatory and had no focal physical neurologic
signs. He had marked frontal release signs and tended to put inedible objects
into his mouth. Visuo-spatial relationships are preserved. Which of the
following is the most likely diagnosis?
a. Alzheimer's disease
b. Frontotemporal dementia
c. Bovine encephalopathy
d. Multi-infarct (vascular) dementia
Answer b: He has uninhibited behavior, often with a sexual flavor, and
other signs of frontal lobe dysfunction. Notably, he has no lateralized
signs, but preserved visuo-spatial relationships. This is a case of fronto-temporal
dementia, which typically has aspects of the human Kluver-Bucy syndrome.
Pick's disease is probably the commonest cause of this variety of dementia.
5. Which of the following visual fields is usually
found with nonfluent aphasia?
a. 
b. 
c. 
d.
e.
Answer: a. Nonfluent aphasia, which usually results from a left cerebral
hemisphere lesion, is associated with a right homonymous hemianopsia. Remember
that visual fields are portrayed from the patient's perspective.
• Top of page •
