Quiz Form

Question Date: 30/10/2023

Question: A 15-year-old girl is referred for a neurologic evaluation by her primary care physician because of choreic movements of several years duration. The movements interfere with her ability to participate in sports, crafts, and writing, but her mother does not think that they are progressively worsening. The patients father does not live with her but reportedly had similar movements for several years. Her medical history is remarkable for short stature, hypothyroidism, and moderately significant developmental delay<br><br>Which of the following is the most likely cause of her chorea?

Options:

• Ataxia-telangiectasia
• Benign hereditary chorea
• Chorea-acanthocytosis
• Mitochondrial disorder

Correct Answer: Benign hereditary chorea

Explaination: Many cases of childhood onset of nonprogressive chorea associated with short stature and an autosomal dominant inherited pattern are due to benign hereditary chorea. The involuntary movements in benign hereditary chorea can be quite severe and are not necessarily benign, and this disorder can be associated with short stature and developmental delay. Phenotypic heterogeneity within families is typical. Larger deletions of the causative gene can cause a multisystem disorder with congenital hypothyroidism, hypotonia, and pulmonary problems

Reference: Walker RH. Chorea. Continuum (Minneap Minn). 2013 Oct19(5 Movement Disorders)1242-63. doi 10.121201.CON.0000436155.46909.c3. PMID 24092289.