Chapter 23. Ataxic Disorders Flashcards
Question 23-1:
Which of the following describe speech abnormality in patients with cerebellar dysfunction?
A. Slurred, mumbling, and excessively rapid. making it difficult to understand
B.Slow, slurred, with pauses and hesitations
C. Articulate with phoneme substitutions but otherwise normal content
D. Impaired content but otherwise normal expression
Answer 23-1: B.
Patients with cerebellar disorders have speech
which is slowed and slurred. There is
difficulty with articulation, such that speech is
hesitant with pauses at inappropriate times and
loss of pauses when they would be expected.
There is also inappropriate variability in
volume and pitch and errant breath control
Question 23-2: A 50-year-old man presents with gait ataxia. Examination shows good independent movement of the limbs on formal examination. Romberg is positive. Reflexes are absent. Where is the lesion? A. Corticospinal tract B. Basal ganglia C. Cerebellum D. Sensory nerves
Answer 23-2: D.
Patients with sensory ataxia have impaired
coordination due to proprioceptive deficit.
Formal examination would have shown
impaired proprioceptive function in the
extremities. The main clue in this clinical
presentation is areflexia
Question 23-3:
Which of the following ataxic disorders are inherited?
A. Friedreich’s ataxia
B. Mitochondrial encephalopathy with ragged red fibers
C. Ataxia-telangiectasia
D. Spinocerebellar ataxia type 2
E. All of the above
Answer 23-3:L
All of these are genetic causes of ataxia.
Friedreich’s ataxia and AT are autosomal
recessive. MERRF is mitochondriaL SeAs are
autosomal dominant. Another important
mitochondrial cause is MELAS
Question 23-4:
Which of the following statements are true regarding ataxia from the Arnold-Chiari malformation?
A. This is a disorder mainly of childhood
B. If a patient is not affected during childhood, they will not develop symptoms during adult life
C. Arnold-Chiari malformation produces ataxia on the basis of hydrocephalus
D. All of these are true
Answer 23-4: A.
Arnold-Chiari malformation is protrusion of
the cerebellar tonsils through the foramen
magnum. This usually becomes symptomatic
during childhood or young adult life, but it
may only become evident later in life.
Question 23-5: Hair loss with ataxia suggests which of the following conditions? A. Wilson's disease B. Ataxia telangiectasia C. Hypothyroidism D. Friedreich's ataxia
Answer 23-5: C
Hypothyroidism is associated with ataxia and
hair loss in addition to other clinical
manifestations of thyroid deficiency,
including reflex changes, dry skin, and weight
gain.
Question 23-6: Ataxia with Keyser-Fleischer rings suggests which of the following? A. Mitochondrial disease B. Gaucher's disease C. Friedreich's ataxia D. Wilson's disease
Answer 23-6: D.
‘Wilson’s disease is associated with ataxia
associated with liver disease and a disorder of
copper metabolism. Keyser-Fleischer rings in
the cornea are an important diagnostic feature.
Question 23-7: Ataxia associated with cataracts suggests which of the following? A. Wilson's disease B. Marinesco-Sjogren syndrome C. Friedreich's ataxia D. Gaucher's disease
Answer 23-7: B.
Marinesco-Sjogren syndrome is a rare genetic
disorder characterized by ataxia. cataracts
small stature, and cognitive change
Question 23-8: Ataxia in combination with internuclear ophthalmoplegia can suggest which of the following? A. Pontine ischemia B. Multiple sclerosis C. Spinocerebellar degeneration D. A&B E. All of the above
Answer 23-8: D.
Pontine ischemia and MS are common causes
of INO in combination with cerebellar ataxia.
Question 23-9: Downbeat nystagmus in association with ataxia suggests which of the following conditions? A. Amold-Chiari malformation B. Basilar invagination C. Lithium toxicity D. Spinocerebellar ataxia E. All of the above
Answer 23-9: E.
Any of these can produce ataxia along with downbeat nystagmus. SCA-6 can produce this
chnical presentation. Basilar invagination and
Arnold-Chiari malformation can be associated
with compression of brain stem and cerebellar
tissues
Question 23-10:
Which of the following would not be expected in patients with cerebellar ataxia due to multiple sclerosis?
A. MRI abnormalities in the white matter of the cerebellum
B. Visual loss
C. Relapsing-remitting course
D. Ptosis
E. Diplopia
Answer 23-10: D.
Ptosis is not a common finding with multiple
sclerosis although a host of other ophthalmologic findings are identified, including INO, other ocular motor palsies, and visual loss. A relapsing-remitting course is typical, and MRI abnormalities in the
cerebellum are expected
