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Symptoms and Signs – Differential Diagnosis of Huntington’s Disease
• Benign hereditary chorea: autosomal dominant with onset in childhood. There is no progression of symptoms and no associated dementia or behavioral problems.
• Chorea gravidarum: presents during first 4 to 5 months of pregnancy and resolves after delivery
• Dentatorubropallidoluysian atrophy: autosomal dominant, triplet repeat disease; presentation is variable and includes chorea, myoclonus, dementia, and ataxia; more common in Japan; can be confirmed by genetic testing
• Drug-induced chorea: dopamine, stimulants, anticonvulsants, antidepressants, and oral contraceptives have all been known to cause chorea.
• Neuroacanthocytosis: autosomal recessive; chorea, dystonia, tics, and orolingual dyskinesias that can result in self-mutilation; must look for acanthocytes in peripheral smear
• Paraneoplastic: seen most commonly in small cell lung cancer and lymphoma
• Postinfectious
• Senile chorea
• Sydenham’s chorea: decreased incidence with decline of rheumatic fever
• Systemic lupus erythematosus (SLE): can be the presenting feature of lupus; occurs in only about 1% of individuals with lupus; pathophysiology unknown
• Wilson’s disease: autosomal recessive; tremor, dysarthria, and dystonia are more common presentations than chorea. Of patients with neurologic manifestations, 95% will have Kayser-Fleischer rings.
• Benign hereditary chorea: autosomal dominant with onset in childhood. There is no progression of symptoms and no associated dementia or behavioral problems.
• Chorea gravidarum: presents during first 4 to 5 months of pregnancy and resolves after delivery
• Dentatorubropallidoluysian atrophy: autosomal dominant, triplet repeat disease; presentation is variable and includes chorea, myoclonus, dementia, and ataxia; more common in Japan; can be confirmed by genetic testing
• Drug-induced chorea: dopamine, stimulants, anticonvulsants, antidepressants, and oral contraceptives have all been known to cause chorea.
• Neuroacanthocytosis: autosomal recessive; chorea, dystonia, tics, and orolingual dyskinesias that can result in self-mutilation; must look for acanthocytes in peripheral smear
• Paraneoplastic: seen most commonly in small cell lung cancer and lymphoma
• Postinfectious
• Senile chorea
• Sydenham’s chorea: decreased incidence with decline of rheumatic fever
• Systemic lupus erythematosus (SLE): can be the presenting feature of lupus; occurs in only about 1% of individuals with lupus; pathophysiology unknown
• Wilson’s disease: autosomal recessive; tremor, dysarthria, and dystonia are more common presentations than chorea. Of patients with neurologic manifestations, 95% will have Kayser-Fleischer rings.
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