[Biopercular polymicrogyria associated with congenital ophthalmoplegia caused by nuclear lesion of the common oculomotor nerve].
Developmental pseudobulbar palsies seem to be different from the adult form described by Foix, Chavany and Marie. They usually include a major speech delay and severe epileptic seizures. In one clinicopathological case, neuroradiological imaging showed a macrogyric aspect of both rolandic operculi and unilateral destruction of pes pedunculari. Microscopic examination showed a four-layered polymicrogyria involving the first temporal gyrus and in the brainstem a selective destruction of the left oculomotor nucleus. Thus, the macrogyric aspect could be related to post migratory disorder occurring late in the cortical development. The brain stem lesion, destroying unilaterally the third cranial nerve nucleus gives a good example of the complex somatotopia of this oculomotor nucleus.
Routon
M C
MC
Laboratoire de Neuropathologie, Hôpital St-Vincent-de-Paul.
Expert-Bezançon
M C
MC
Bursztyn
J
J
Mselati
J C
JC
Robain
O
O
D000015:Abnormalities, Multiple; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000000981:diagnostic imaging; D003389:Cranial Nerve Diseases / Q000151:congenital* / Q000175:diagnosis; D004827:Epilepsy / Q000209:etiology; D017809:Fatal Outcome; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D009802:Oculomotor Nerve; D009886:Ophthalmoplegia / Q000151:congenital* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed
Polymicrogyrie bioperculaire associée à une ophtalmoplégie congénitale par atteinte du noyau du nerf moteur oculaire commun.