Encephalopathy associated with lamellar residual bodies in astrocytes (Towfighi, Grover and Gonatas 1975):a new observation.

A 7-year-old male patient presented a few episodes of convulsions during the first year of life. The psychomotor development has been normal during that period of time after which a steadily progressive deterioration occurred. Increasingly severe pyramidal signs, optic atrophy, quadriparesis and progressive evolution towards a decorticate state were noted. Skin and neuromuscular biopsies were not contributory. A brain biopsy showed the presence of lamellar inclusions in astrocytes and, to a lesser extent, in neurons. Such features have only been reported once in two siblings by Towfighi et al. (1975). The nosological situation of this disease is discussed.