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Heterotopic neurons in congenital myotonic dystrophy with mental retardation
Author(s) -
Watanabe Chigusa,
Katayama Sadao,
Noda Koichi,
Kaneko Mayumi,
Inai Kouki,
Nakamura Shigenobu
Publication year - 1997
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1997.tb00046.x
Subject(s) - spinal cord , medicine , white matter , myotonic dystrophy , weakness , neuroscience , anatomy , pathology , biology , magnetic resonance imaging , psychiatry , radiology
We studied heterotopic abnormalities of the brain and spinal cord of a 48‐year‐old mentally retarded man with congenital myotonic dystrophy. He presented with a 2‐year history of progressive muscular weakness in the trunk and he developed dyspnea on effort and orthopnea 4 months pro to death. He died of acute renal failure and severe congestive heart failure. The characteristic neuropathological finding was a number of heterotopic neurons in the cerebrum. In the molecular layer of the cerebral cortex, medium‐sized neurons, pyramid‐shaped large neurons, and horizontal cells were noted. Many neurons were also distributed sporadically in the subcortical white matter. In the spinal cord, abnormally situated neurons were present in be posterior and lateral columns near the gray matter. These findings suggest that intrauterine maturational disturbance occurs during the period of migration and organization of neurons in the brain and spinal cord.