MORPHOLOGICAL-STUDY OF THE BRAIN-STEM IN FUKUYAMA TYPE CONGENITAL MUSCULAR-DYSTROPHY

We have observed sudden clinical death due to Fukuyama-type congenital muscular dystrophy (FCMD), In FCMD, brain abnormalities, such as poly microgyria, leptomeningeal neuroglial heterotopia and abnormal course of the corticospinal tracts, are well known, We investigated the brain stem of 10 FCMD and 7 control cases, Among the control cases, 5 with D uchenne type muscular dystrophy died of heart failure and 2 died accid ental death, In the brainstem, the catecholaminergic neurons character ized by reaction with antiserum to tyrosin hydroxylase showed notable reduction in the reticular formation, vagal nuclei, and nucleus tractu s solitarius. Delays or aberrations of neural control may contribute t o the pathogenesis of sudden infant death syndrome, and medullary glio sis occurs in the reticular formation of sudden infant death syndrome, The pathogenesis of neurons in the brainstem in FCMD may be similar t o that in sudden infant death syndrome, These findings suggest neurona l dysfunction in the brainstem and may be related to respiratory, circ ulatory, or sleep-wake regulation disorders. (C) 1996 by Elsevier Scie nce, Inc.