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Journal of Child Neurology
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Familial Dystonia and Choreoathetosis in Three Generations Associated With Bilateral Striatal Necrosis

R.D. Craver, MD

Department of Pathology, Louisiana State University Medical Center, and Children's Hospital, New Rleans, LA, Department of Pediatrics Louisiana State University Medical Center, and Children's Hospital, New Orleans, LA

M.C. Duncan, MD

Department of Pediatrics Louisiana State University Medical Center, and Children's Hospital, New Orleans, LA

J.S. Nelson, MD

Department of Pathology, Louisiana State University Medical Center, and Children's Hospital, New Rleans, LA

Nine cases of dystonia and choreoathetosis (six females and three males) have developed in three generations of a single family. There has been one death. Neuropathologic examination disclosed bilateral striatal necrosis. In this family, the neurologic disorder has evolved gradually or in association with a febrile illness. There has been no neurologic recovery. The disease is worse in females, has been transmitted only through females, and shows incomplete penetrance and anticipation. The maternal inheritance pattern suggests either an autosomal dominant trait also affecting male reproductive ability or a defect involving the mitochondrial genome. (J Child Neurol 1996;11:185-188).

Journal of Child Neurology, Vol. 11, No. 3, 185-188 (1996)
DOI: 10.1177/088307389601100303
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