6533b873fe1ef96bd12d4cad

RESEARCH PRODUCT

Muscular alteration in agyria with pyramidal tract anomaly

A. BardosiHans H. GoebelUros RoessmannAkira Hori

subject

MaleDecussationMicrocephalyPyramidal TractsAnterior commissureCorpus callosum03 medical and health sciences0302 clinical medicineDevelopmental Neuroscience030225 pediatricsmedicineHumansMyopathyCerebral CortexPyramidal tractsMuscular hypotoniabusiness.industryMusclesGeneral MedicineAnatomymedicine.diseasemedicine.anatomical_structureChild PreschoolAgenesisPediatrics Perinatology and Child HealthNeurology (clinical)medicine.symptombusiness030217 neurology & neurosurgery

description

A 4-year-old boy with a history of muscular hypotonia, mental retardation, microcephaly, and generalized convulsions was found at autopsy to have agyria, agenesis of the anterior commissure and posterior corpus callosum as well as an abnormal decussation of pyramidal tracts which descended in the spinal dorsal columns. Postmortem muscular alterations included type IIc fiber hypertrophy and type I fiber grouping, variably expressed in individual muscles and intramuscular fascicles. This may represent a developmental delay compatible with a gestational age between the 34th and 40th week. These studies also indicate the importance of examining (a) multiple samples of postmortem muscles and (b) muscles from patients afflicted with cerebral malformations.

https://doi.org/10.1016/s0387-7604(86)80011-0