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The authors report a fairly typical clinico-pathological case of acute hemorragic leucoencephalitis (A.H.L.E.). Both clinical and histological features appeared particularly acute. At autopsy a visceral evolving tuberculosis was diagnosed. Such an etiological circumstance has not been, apparently, reported in cases of A.H.L.E. published as such. Three other etiological circumstances were noticed: pyuria, treatment by cephalotin and treatment by gentamycine. The physiopathogenesis of A.H.L.E. remains obscure and will not be clarified before detailed immunological studies can be performed. But the disease is rare and brisk and diagnosis usually post-mortem.

The authors report an observation in which four siblings were affected by both multiple cutaneous epitheliomatosis and complex but relatively stereotyped neurological disorders. Clinically, the main syndrome was cerebello-spinal ataxia with involvement of the anterior horns of the spinal cord with less marked pyramidal and extra-pyramidal features. Neuropathological examination of one of the cases revealed lesions of essentially cerebello-spinal degeneration suggestive of Menzel's disease. The possible connection between the neural and cutaneous lesions is discussed. All the various etiological categories possible have been ruled out; not one being entirely satisfactory, except for the very broad category of genetic neuro-dermatoses.