Abstract
Lissencephaly (agyria) is a rare congenital brain anomaly characterized by a lack of apparent gyri formation. We reported previously a case of the lissencephaly syndrome. This paper presented a comparison between CT findings at the age of 5 months and neuropathological findings at 19 months.
The macroscopical examination showed smooth surface without cerebral convolution, incomplete sylvian fissure formation without operculum insulae, cavum septi pellucidi, enlargement of the lateral ventricles especially in the posterior portion, thicker white matter of frontal lobe than that of occipital lobe, thick cortex with poor demarcation between gray and white matter. These changes corresponded well to the findings in CT previously done; Therefore CT scan is useful for the diagonsis of this disease.
Golgi study revealed that there was a disorganization of neuronal disposition and developmental retardation or arrest of neurons, in addition to a maldevelopment of cortical cellular layers.
In the patient there was a history of acute exanthematous disease of her mother at 2 months of pregnancy. The patient showed hepatosplenomegaly with elevation of IgM and cytomegalovirus CF titer at 5 months, and chronic nonspecific infectious lesions with necrosis in lymphnodes, liver and submandibular glands at autopsy. These clinical and pathological findings may be considered to suggest that the intrauterine infection had caused the syndrome in this patient.
