PMID: 8972533Nov 1, 1996Paper

Morphological study of the brainstem in Fukuyama type congenital muscular dystrophy

Pediatric Neurology
M ItohE Ohama

Abstract

We have observed sudden clinical death due to Fukuyama-type congenital muscular dystrophy (FCMD). In FCMD, brain abnormalities, such as polymicrogyria, leptomeningeal neuroglial heterotopia and abnormal course of the corticospinal tracts, are well known. We investigated the brainstem of 10 FCMD and 7 control cases. Among the control cases, 5 with Duchenne type muscular dystrophy died of heart failure and 2 died accidental death. In the brainstem, the catecholaminergic neurons characterized by reaction with antiserum to tyrosin hydroxylase showed notable reduction in the reticular formation, vagal nuclei, and nucleus tractus solitarius. Delays or aberrations of neural control may contribute to the pathogenesis of sudden infant death syndrome, and medullary gliosis occurs in the reticular formation of sudden infant death syndrome. The pathogenesis of neurons in the brainstem in FCMD may be similar to that in sudden infant death syndrome. These findings suggest neuronal dysfunction in the brainstem and may be related to respiratory, circulatory, or sleep-wake regulation disorders.

References

Jan 5, 1979·Brain Research·J ChampagnatV Leviel
Apr 1, 1991·Acta Paediatrica Japonica; Overseas Edition·M OsawaY Hirayama
Jul 1, 1984·Journal of Neuropathology and Experimental Neurology·K TakadaJ Tanaka
Jun 1, 1981·The American Journal of Forensic Medicine and Pathology·C G Summers, J C Parker

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Citations

Feb 23, 2012·Fukushima Journal of Medical Science·Naoya Sekiya
May 23, 2020·AJNR. American Journal of Neuroradiology·A Hirasawa-InoueM Sasaki

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