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A 26-year-old housewife, born of consanguineous parentage, began to have gait and speech disturbance. Her brother had died from suffocation because of dysphagia. At thirty-two, she developed difficulty in swallowing, clumsiness and incontinence. When she was thirty-six she had pseudobulbar palsy, vertical gaze paresis, hyperreflexia and muscular atrophy of the upper half of the body. CT scan showed cerebral atrophy. Her mental function progressively deteriorated and amyotrophic lateral sclerosis associated with dementia was suspected. She died at the age of thirty-seven. Diagnosis was made only by autopsy. There was no particular general pathologic finding excepting aspiration pneumonia. Microscopical examination revealed numerous distended neurons with accumulation of light brown pigments by Luxol fast blue/HE stains, especially in hypothalamus, substantia nigra and nuclei of oculomotor nerves. To a lesser extent such neurons were noted ubiquitously. The stored material was mainly composed of lipofuscin and ceroid. Ultrastructurally they presented the various structures which have previously been reported, except for finger print profiles. The pigmentary deposits were shown to be immunoreactive with polyclonal antibody directed against amyloid beta-protein.

A boy, born after normal pregnancy and delivery, began to have fits at 3 days. The seizures were composed of tonic or tonic-clonic convulsions at the upper extremities but myoclonus was absent. These attacks were not easy to control. There was gross developmental delay. Laboratory investigations were almost normal except for cerebrospinal fluid: pleocytosis and high protein content. EEG showed "suppression-burst" and MRI revealed high signal intensity in the left temporo-occipital region on T2 weighted image. At three and a half months of age, EEG changed into hypsarrhythmia. The child died at 5 months of age. At post mortem neuropathological examination, the cortical ribbon in the bilateral parieto-occipital regions appeared thick, as if there were pachygyria. Microscopically polymicrogyria was noted in these areas as well as in the insular cortex. This lesion showed a symmetrical distribution. The cytoarchitectonic features of the polymicrogyric cortex did not consist of 4 layers. The other structures of the central nervous system were almost devoid of lesion. The number of clinico-pathological reports on Ohtahara's syndrome is very limited and the etiopathogenesis of polymicrogyria is discussed.

Two cases of multilocular cystic adenocarcinoma of the kidney are presented. Although ultrasonography (US) and x-ray computed tomography (CT) are generally considered to be excellent diagnostic methods for renal cystic lesions, in the 2 cases reported here they failed to give the correct diagnosis. These unusual cases altered us to the possibility that the capabilities of modern diagnostic imaging techniques can lead to a false sense of security regarding the prognosis. To our knowledge, there are no reliable clinical features which distinguish multilocular cystic adenocarcinoma from multilocular cystic nephroma.

A 22-year old male patient was admitted for deep coma probably of traumatic origin. There was neither fracture of the skull nor expansive intracranial lesion. The patient survived for 6 years and 8 months without any change in consciousness. Post-mortem neuropathological examination showed lesions which predominated in the white matter and had features that were compatible with diffuse axonal injury. The mechanism responsible for these lesions seems to be stretching and shearing of axones at the moment of impact. The exceptionally long duration of survival probably accounts for the severity of the lesions observed.

International audience; The prelimary results ofthe third part of the oceanographic survey by the J.Charcot Kaiko provide new dataon the Japan and Kuril trenches and their junction. South of the concerned area, the Kashima seamount is entering the subduction zone without any important compressive deformation of the inner wall. Instead, theseamount is cut, before being subducted, by newly created normalfaults in the oceanic crust. North of this area,. important gravity slidings occur on the innerwall, thus infilling the trenchwhich is reduced to asuccession ofsmall basins; all these [slidings are ihen subducted together with the oceanic crust and its sedimentarycover. Thisregion could be a modelfor tectonic erosion. Oceanic magnetic anomalies, which strike obliquely on the trench,can be followedbelow the innerwall on along distance wiih decreasing amplitude. At the Japan-Kuril trenchesjunction aleft-lateral strike-slip fault offsets theyinner wall. The Kuril trench is a wide flat plain stronglyconstrasting with the Japan trench small basins. The direction of the trench being paralill to the oceanic magneticanomalies, ancient normalfaultsinheritedfrom the oceanic accretion are reactivated when the oceanic lithosphereenters the subduction zone. The Erimo seamount, brought by the Pacific plate aithey corner between the two, trenches does not play any significant role in the curvature which is more likely related to the intracontinentalAmerica-Eurasiaplate boundary; Les premiers résultats de la troisième partie de la campagne océanographique du J. Charcot, Kaïko, apportentdes données nouvelles sur la fosse du Japon et sa jonction avec la fosse des Kouriles. Au Sud de la régionétudiée, le seamount Kashima entre en subduction sans que le mur interne soit déformé. Il est découpé, avant d'être subducté, par des failles normales nouvellement créées au front de la zone de subduction dans la croûteocéanique.Au Nord, le mur interne de la fosse du Japon est le lieu de glissements en masse qui vont remplirla fosse, alors réduite morphologiquement à une succession de bassins étroits; tous ces dépôts sont ensuitesubductés avec le reste de la croûte océanique et sa couverture. Cette légion pourrait être un modèle pourl'érosion tectonique. Les anomalies magnétiques de la croûte océanique,obliques sur la direction de la fosse se suivent sous le mur interne sur une grande distance. A la transition avec la fosse des Kouriles, un décrochementsenestre décale le mur interne et fait passer à une vaste plaine qui contraste avec les petits bassins de la fossédu Japon. La direction de la fosse étant alors parallèle aux anomalies magétiques de la croûte océanique, lesanciennes failles normales datant de l'accrétion océanique sont réactivées. Le seamount Erimo qui bouche lafosse dans le coin Kourile-Japon ne joue aucun rôle dans la courbure qui est plutôt à mettre en relation avec la limite intracontinentale entre les plaques Amérique et Eurasie.