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A 35 year-old caucasian man suffered from paroxysmal nocturnal haemoglobinuria (PNH) or Marchiafava-Micheli's disease diagnosed in 1976 and complicated by several thrombotic episodes. He developed a benign intracranial hypertension. A digitalized intravenous angiography showed occlusion of both lateral sinuses. Partial improvement followed lombo-peritoneal(More)
A woman presented with a history of three regressive comas of undetectable etiology between the age of 52 and 57 years. An IgG lambda benign monoclonal dysglobulinemia was combined with a papular mucinosis (myxedematous lichen or the generalized form of Arndt-Gotton's scleromyxedema). In the 6 analogous cases documented in the literature the onset of coma(More)
A 34-year-old woman with no family history of orthochromatic leukodystrophy (OLD) developed progressive intellectual deterioration, a frontal syndrome and spastic tetraparesis. She died four years after the onset of the clinical illness. Neuropathological studies included light and electron microscopy of cerebral and nerve biopsies, and a complete(More)
A case of progressive supranuclear palsy characterized by a loss of self-activation and a compulsive behaviour of the obsessive type is reported. The pathological examination was remarkable for the intensity of pallidal lesions and their diffusion to both the external and internal segments. While the loss of self-activation seemed to result from a damaged(More)