[Primary diffuse leptomeningeal leiomyomatosis].

A female patient aged 28 fell ill with manifestations of sensory aphasia, headache and signs of cerebral compression. Except for a bilateral papilledema there were no further pathological clinical and paraclinical findings. Later on, amaurosis, deafness, anosmia and generalized muscular hypotonia developed. The nuclear magnetic resonance image revealed a major accumulation of contrast medium in the leptomeninx. Biopsy demonstrated a mesenchymal neoplasm in the leptomeninx. After a strong rise in intracranial pressure, the patient died from a bulbar brain syndrome. Microscopy revealed a diffuse neoplasm limited to the leptomeninx of brain and spinal cord as well as the immediate neighbourhood of small cortical vessels which, by morphological criteria, was classified as low-grade malignant. With the aid of electron microscopy, the tumour cells could be identified as descendants of smooth muscle cells.