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Abnormal expression of cell adhesion molecule L1 in migration disorders: a developmental immunohistochemical study.
Clin Neuropathol
August 1997
Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, NCNP, Tokyo, Japan.
We studied immunohistochemically the expression pattern of a neural cell adhesion molecule, L1, in various human migration disorders associated with polymicrogyria: 4 fetuses and 11 infants having Fukuyama type congenital muscular dystrophy (FCMD) (4 cases), Zellweger syndrome (6) or thanatophoric dysplasia (3) and intrauterine brain damages (2) at different development stages, comparing to age-matched controls. There were different patterns of L1 expression, which suggested at least 3 pathogenetic mechanisms: high expression associated with neuoraxonal overgrowth (fetal FCMD and destructive event at intermigratory period); delayed expression with neuronal dysmaturation and dysmyelinogenesis (late infantile stage of Zellweger syndrome); no expression in toxic or destructive brain injury (Zellweger syndrome or destructive events at inter-or postmigratory periods).
View Article and Find Full Text PDFIntracranial arachnoid cysts in myotonic dystrophy.
Neuroradiology
October 1991
Service Hospitalier Frédéric Joliot, CEA, Orsay, France.
Clinically apparent brain dysfunction is common in myotonic dystrophy. In a sample of fourteen adult patients with the definite form of this disease, brain magnetic resonance imaging detected frequent white matter abnormalities and ventriculomegaly. In addition, two patients exhibited an intracranial arachnoid cyst, a condition of neurosurgical interest that could be related to the generalized dysmaturational process present in this disease.
View Article and Find Full Text PDFDysmaturation neuromyopathy: correlation with minimal neuropathy in sural nerve biopsies.
J Child Neurol
October 1988
Department of Pathology (Neuropathology), UCLA Center for Health Sciences 90024-1732.
Dysmaturation (neuro)myopathy without specific histochemical or cytoarchitectural characteristics accounts for many cases of hypotonia in infancy. We obtained a maturation profile for type I and type II fibers from birth to 6 years of age, from which a classification of fiber dysmaturation based on fiber-type hypotrophy and coefficient of fiber variation is presented. We analyzed the morphometric, ultrastructural, and single fiber teasing findings in the sural nerve of ten infants with hypotonia and dysmaturation myopathy based on the above classification.
View Article and Find Full Text PDFWe examined the pathologic findings in four infants of mothers with myotonic dystrophy. Four of the anomalies present in these infants (nesidioblastosis, renal blastema, cryptorchidism, and patent ductus arteriosus) represent persistence of fetal organ structures or configurations. We suggest that a maturational defect may be present not only in muscle, but also in other tissues.
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