chapter 78-79-80 Flashcards
A 60-year-old man presents with gait ataxia associated with no arm ataxia. There are no signs of
brainstem dysfunction on examination, although he has a prominent cerebellar-type gait disorder.
There is no family history of ataxia. He smokes and drinks, but does not use any other drugs.
Which is the most likely etiology.
A. Alcoholic cerebellar degeneration
B. Brain tumor
C. Hypothyroidism
D. Spine-cerebellar degeneration
a
A 65-year-old female presents with progressive ataxia over two months. She had ovarian cancer
two years ago. Examination shows dysarthria and oscillopsia along with mild cognitive
impairment. MRI is normal. CSF shows a mononuclear pleocytosis. Which is the most likely
diagnosis?
A. Neoplastic meningitis
B. Paraneoplastic cerebellar degeneration
C. Spinocerebellar degeneration
D. Wernicke’s encephalopathy
b
A 45-year-old male presents with cerebellar ataxia and is found on examination to have mild
memory loss and upgoing plantar responses. His family reports some behavioral changes over
the past two months. He then develops myoclonus. EEG shows periodic discharges. Which of
the following is the most likely diagnosis?
A. Creutzfeldt-Jakob disease
B. HIV encephalopathy
C. Herpes encephalitis
D. Spinocerebellar degeneration
a
A patient presenting with cerebellar ataxia has positive anti-gliadin antibodies. Which is the
correct interpretation of this finding?
A. The anti-gliadin antibodies confirm the diagnosis of celiac disease
B. The anti-gliadin antibodies are suspicious but not confirmatory for celiac disease
C. The anti-gliadin antibodies are normal
B
An 18-year-old male presents with cerebellar ataxia, dysarthria, extensor plantar responses, and
areflexia in the legs. There is no family history of similar disorder. Peripheral
electrophysiological studies show a peripheral neuropathy with ax^nai loss. Which is the most
likely diagnosis?
A. Celiac disease
B. Friedreich’s ataxia
C. Olivopontocerebellar degeneration
D. Subacute combined degeneration
b
A 7-year-old female presents with progressive cerebellar ataxia and is found on examination io
have hypotonia, areflexia, and peripheral neuropathy. Eye examination is shown in the figure.
Which is the most likely diagnosis?
A. Ataxia-telangiectasia
B. Ataxia with vitamin E deficiency
C. Autosomal recessive ataxia with oculomotor apraxia
D. Infantile-onset OPCA
a
A patient presents with progressive ataxia. His mother and her father both died after being in
wheel-chair or bed-bound for years for unknown reasons. Examination shows cerebellar ataxia
affecting gait and limbs, dvsarthria, and brisk tendon reflexes with extensor plantar responses.
Which is me correct interpretation of the pyramidal tract sign in the setting of an otherwise
typical presentation of an autosomal dominant ataxia?
A. The patient has another disorder, not an autosomal dominant ataxia
B. The patient has an autosomal dominant ataxia plus another cause of the pyramidal tract signs
C. Pyramidal tract signs are common early in the autosomal dominant ataxia
c
What is the appropriate use of DNA analysis for ataxia?
A. All patients with ataxia
B. All patients with ataxia with a family history suggesting dominant inheritance
C. Ail patients with ataxia associated with no cause identified on MRI of the brain and spinal
cord
c
A 6-year-old child develops cerebellar ataxia following an acute febrile illness with associated rash. MRI is shown in the figure. CSF shows elevation in protein and a mild mononuclear pleocytosis. Which is the most likely diagnosis? A. Acute cerebellar ataxia of childhood B. Ataxia-telangiectasia C. Cerebellar astrocytoma D. Medulloblastoma
a
What is the etiology of cerebellar ataxia in patients with cystic fibrosis?
A. Anoxia
B. Idiopathic degeneration of Purkinje cells
C. Vitamin B12 deficiency
D. Vitamin E deficiency
d
A 12-year-old girl presents with short neck, low hairline, and limitation of motion around the
cervical spine. There is no family history. X-ray of the cervical spine show fused cervical
vertebrae. Which is the correct diagnosis?
A. Arnold Chiari malformation
B. Klippel-Feil anomaly
C. Paget’s disease
D. Spinal dysraphism
b
The figure shows the sagittal MRI of a patient who presents with episodic headaches with
nausea, vomiting, and photophobia. Neurologic examination is normal, and there are no
complaints other than the headache.
Which is the correct interpretation of the clinical and imaging features?
A. Chiari I malformation with resultant headaches
B. Chiari II malformation with resultant headaches
C. Migraine headaches with no imaging abnormalities
D. Migraine headache with unrelated Chiari I malformation
d
A patient with Chiari I malformation presenis with increasing headache and ataxia, and is found
to have significant interruption in CSF flow. MRI of the cervical spine show syringomyelia.
Which is the best method of treatment?
A. Shunting of the syrinx
B. Suboccipital decompression
C. Suboccipital decompression and shunting of the syrinx
D. No surgical treatment
b
A 25-year-old man presents with leg weakness. One year ago he had a motor vehicle accident
with transient leg weakness. Now he has several weeks of progressive weakness in the legs with
pyramidal trart signs, in addition, he has milder weakness of the arms with brisk triceps reflex
and absent biceps reflex. An image from the MRI is shown below.
Which is the correct diagnosis?
A. Cervical disk
B. Syringomyelia
C. Spinal cord edema
D. Transverse myelitis
b
Which of the following would suggest tethered cord?
1. Lower motor neuron deficit in one or both legs.
2. Upper motor neuron deficit
3. Sphincter dysfunction
4. Scoliosis
Select: A = 1,2.3. B = 1, 3. C = 2. 4. D - 4 only. E = All Question 79-6:
e
