SUMMARY Anatomical study of 31 cases showed distinct pathological patterns characterizing craniocerebral injuries, closed skull traumas with fracture, and closed skull traumas without fracture. Morphological aspects, distribution, and evolution were noted. These injuries affected all tissues, including the neuromeningeal network, the glia, and the vessels; circulatory disturbances play an essential part in the development of the lesions and their physiopathological consequences. RÉSUMÉ Étude anatomique à propos de‐31 observations des schémas pathologiques distincts qui caractérisent les plaies cranio‐cérébrales, les traumatismes craniens fermés avec fracture, les traumatismes craniens fermés sans fracture. En sont precisés les aspects morphologiques, la répartition, l'évolutivité. Lew constitution implique une atteinte omnitissulaire, avec réaction de la trame neuroméningée, de la glie, des vaisseaux, les perturbations circulatoires jouant un rôle essentiel dans le développement des lésions et leurs conséquences physiopathologiques.

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Two siblings born from consanguineous tunisian parents are reported. They showed a severe form of Farber's disease with prominent involvement of the central and peripheral nervous system: low conduction velocity was noticed in both children. Macular cherry red spots were observed in one of them. The diagnosis for the girl investigated was confirmed by evidence of ceramidase deficiency in cultured fibroblasts. Here we report the pathological findings in the subcutaneous nodules using light and electron microscopy (one case), and in sural nerves using morphometric studies (both cases). Varying morphological aspects of intracellular inclusions, depending on the tissues involved, are described and discussed. A review of all cases reported since Farber's first paper in 1952 is given.

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Thirty eight extraocular muscle biopsies obtained from thirty patients (1 normal and 29 affected with various kinds of ocular motility disorders such as strabismus, Duane's syndrome, paralysis), were studied by electron microscopy. Except for the control biopsy considered as normal, they showed: atrophy, disorganization of myofilaments, double Z-disks, rods, curving arrays of myofibrils, concentrically disposed electron-dense sarcotubules, clusters of mitochondria with numerous modifications of their cristae, and subsarcolemmal inclusions, such as dense bodies, laminated bodies, lipofuscin granules and lipid droplets. In addition, subsarcolemmal granulo-fibrillar aggregates, characterized by periodic granulated foci spaced at about 120 nm intervals, were seen in all the biopsies, even in that of the control case. The authors stress the pecularity of the granulo-fibrillar aggregates which, up to now, have never been described in skeletal muscles and the function of which in the extraocular muscles fibers is impossible to define. They point out the difficulty in determining if the motility disturbances may be related to the various changes observed in extraocular muscles of squinting eyes.