[Cavitary orthochromatic leukodystrophy in the adult. Oligodendroglial proliferation and inclusions].

Abstract

A 41 year-old woman presented with two Grand Mal fits followed by progressive spastic paraparesis and intellectual impairment. After 18 months there was a remission which lasted for 4 years but thereafter inexorable progression of the disease and many fits led to a state of akinetic mutism. Death occurred nine and a half years after the onset of the illness. There was no family history. The neuropathological studies included light and electron microscopy of a cerebral biopsy and a complete post mortem examination. Myelin loss and cavitation of the white matter was associated with the presence of sudanophilic lipid and abundant cells at the periphery of the lesions. These cells had the characteristics of oligodendrocytes. The combination of sudanophilic leucodystrophy, cavitation of white matter and oligodendrocyte proliferation has previously been observed only in childhood.