Pathology of Pregnancy, Childbirth and the Puerperium Codexery

Chorea gravidarum

Rare pregnancy complication with involuntary movements, linked to prior chorea.

Chorea gravidarum is a rare complication of pregnancy characterized by abrupt, brief, nonrhythmic, nonrepetitive involuntary movements of the limbs and nonpatterned facial grimaces. It is not a distinct pathological entity but a generic term for chorea of any cause arising during gestation, often associated with a history of Sydenham's chorea. The condition typically occurs in young patients, with an average age of 22 years, and its incidence has declined, likely due to reduced rates of rheumatic fever.

Quick Facts

Average age of patients
22 years

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Pathophysiology

Several pathogenetic mechanisms have been proposed but none proven. A history of rheumatic fever or chorea is suspected; estrogens and progesterone may sensitize dopamine receptors at a striatal level, inducing chorea in individuals with preexisting basal ganglia pathology. Studies show women with normal pregnancies before rheumatic fever developed chorea in subsequent pregnancies. Oral contraceptives are an infrequent cause; one patient without prior Sydenham's chorea or recent streptococcal infection had anti-basal ganglia antibodies, suggesting an immunological basis.

Diagnosis

The differential diagnosis includes Pelizaeus–Merzbacher disease, pantothenate kinase-associated neurodegeneration, Ramsay Hunt syndrome, Huntington's disease, striatonigral degeneration, Lesch–Nyhan syndrome, systemic lupus erythematosus, Lyme disease, torticollis, multiple system atrophy, Tourette syndrome and other tic disorders, neuroacanthocytosis, viral encephalitis, neuronal ceroid lipofuscinosis, Wilson's disease, olivopontocerebellar atrophy, familial paroxysmal choreoathetosis, and benign hereditary chorea. Chorea may also result from drug toxicity (e.g., anticonvulsants, antiparkinson agents, neuroleptics, steroids, estrogen) or infectious diseases such as meningovascular syphilis, Lyme disease, and viral encephalitis.

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