Absence attacks and diffuse neuronal disease

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Abstract
An 8-yr. -old girl presented with frequent absence attacks. Her electrogram showed long bursts of generalized symmetrical and synchronous 3/sec. spike and wave discharges. From the age of 9-1/2 yr., she developed progressive visual loss and mental deterioration became apparent. A diagnosis of juvenile cerebromacular degeneration was confirmed by the finding of lipid storage in neurons of the myenteric plexus. Diffuse cortical and subcortical neuronal disease usually leads to the appearance of bilaterally synchronous paroxysmal slow wave or slow spike and wave discharges but it may, as in this case, induce regular 3-cycles/sec. attacks. Even in retrospect, it would not have been possible to distinguish this patient''s seizure and electroencephalo-graphic pattern from that of children with absence attacks for which no neuropathological cause is found. Findings in patients such as this suggest that the abnormality in absence attacks and the 3/sec. spike and wave discharge involve both cortical and subcortical neuronal populations.

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