Neuropsychiatric and brain CT findings in polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

Abstract

Seven patients with polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy (PLO-SL or membranous lipodystrophy) were examined clinically and with CT scanning. The degree of dementia was severe in five cases, marked in one case, and mild in one case. All patients suffered from marked or severe prefrontal psychosyndrome, impairment of memory and signs of upper motor neuron involvement. Epileptic seizures and primitive reflexes were observed in six cases. Degree of agnostic-aphasic-apraxic symptoms varied. In all cases CT revealed general and diffuse brain atrophy, which was accentuated in frontal areas. In the basal ganglia bilateral calcifications (five cases) or a slight hyperdensity (two cases) were detected. CT finding in PLO-SL may lead to an early diagnosis of this fatal disease.