Stages and patterns of centrifugal arrest of diffuse neuronal migration disorders.
Centrifugal migration of newly generated neuroblasts toward the cortical surface can be arrested at different levels, resulting in anatomically different disorders. This is illustrated by three patients with diffuse or generalized neuronal migration disorders (NMDs): pachygyria, subcortical laminar heterotopia ('double-cortex' syndrome), and periventricular laminar heterotopia. All had medically intractable partial and generalized epileptic seizures, but there was no close correlation between the type of dysplasia and intelligence or clinical pattern. The low intelligence found in these patients may relate to the epileptic syndrome, rather than to the NMDs alone. MRI suggested that in this spectrum of disorders the migration process was arrested at different stages, depending on the extent, timing and site of damage to radial glial fibres.
Palmini
A
A
Department of Neurology and Neurosurgery, McGill University, Montreal, PQ, Canada.
Andermann
F
F
de Grissac
H
H
Tampieri
D
D
Robitaille
Y
Y
Langevin
P
P
Desbiens
R
R
Andermann
E
E
D000328:Adult; D001479:Basal Ganglia / Q000503:physiopathology; D001706:Biopsy; D001921:Brain / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis / Q000000981:diagnostic imaging; D001932:Brain Neoplasms / Q000175:diagnosis / Q000503:physiopathology; D002540:Cerebral Cortex / Q000002:abnormalities / Q000503:physiopathology; D002648:Child; D002828:Choristoma / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology; D008279:Magnetic Resonance Imaging; D012640:Seizures / Q000175:diagnosis / Q000503:physiopathology; D014057:Tomography, X-Ray Computed