[Progressive degenerative myoclonic epilepsy. Systematized olivo-cerebellar lesions]. / Epilepsie myoclonique progressive dégénérative. Lésions olivo-cérébelleuses systématisées.

A 15 year-old North-African female showed typical symptoms and evolution of Progressive Myoclonus Epilepsy of the Unverricht type. Pathological examination failed to show either inclusion bodies or any other storage material. The only relevant findings included degenerative changes in the inferior olives and, to a lesser extent, in the cerebellar cortex. The site of lesions was remarkable: in the inferior olives, lesions were bilaterally and symmetrically restricted to the external angles (lateral lamellae); in the cerebellum, loss of Purkinje cells and ascending fibres of the molecular layer was prominent in the lateralmost part of the hemispheres (semilunar lobules). Such a topography implies a system disorder involving the olivo-cerebellar pathway, particularly in that part which projects to the neocerebellum. Twelve other clinico-pathological cases of progressive myoclonus epilepsy of the degenerative group are reviewed. It is suggested that, here again, lesions--although more diffuse--may be related to a primarily olivo-cerebellar involvement.

[Striatonigral degeneration associated with olivopontocerebellar atrophy. Anatomo-clinical study of 3 cases. Nosologic discussion]. / Dégénérescence striato-nigrique associée à une atrophie olivo-ponto-cérébelleuse. Etude anatomo-clinique de trois cas. Discussion nosologique.

Results of a clinicopathologic study in 3 cases of nigrostriatal degeneration associated with olivopontocerebellar atrophy are reported. The clinical picture was primarily that of parkinsonism but associated disorders were atypical: sphincter and deglutition disorders, pyramidal signs and action myoclonia; paralysis of vertical gaze (with a deficit of convergence in 1 case) and a cerebellar syndrome in 2 cases; postural hypotension with an invariable pulse, amyotrophy of the hands and a paradoxical response to Dopa: worsening of akineto-hypertonic symptomatology and deglutition disorders in 1 case. Pathologic examination showed similar lesions in the 3 cases: atrophy with pigmentation of the putamen and changes in the substantia nigra, associated with evidence of olivopontocerebellar degeneration. There was marked loss of neurones in the intermediolateral tract in the cord of the patient with the Shy and Drager's syndrome. All cases reported of this abiotrophic association have been of a sporadic nature. The predominance of females, the age of onset and duration of the disease are features that do not differ much from those of pure nigrostriatal degenerative disorders. These cases cannot therefore be considered as a pathological entity but as a particular form of degeneration affecting multiple systems.

[Plasmocytic meningitis in multiple myeloma. An anatomo-clinical observation]. / Méningite plasmocytaire au cours d'un myélome multiple. Observation anatomo-clinique.

A confusional state due to hypercalcemia led to the discovery of multiple myeloma in a 73 year old man. The recurrence of a confusional state, related to plasma cell meningitis called for hospitalization. Serum, urine and cerebrospinal fluid immunoelectrophoresis revealed monoclonal immunoglobulines of lambda light chains. The pathologic study showed an invasion of the subarachnoid space by abnormal plasma cells. The dura mater and brain substance were not invaded. The leptomeningeal invasion by multiple myeloma plasma cells in a rare occurrence. We have found only three such cases published before. The analysis of our case and the three other leads to some comment. The clinical features are those of chronic meningitis revealed by a confusional study or epilepsy. The existence of abnormal plasma cells in the cerebrospinal fluid and their absence in the blood stream seems to suggest a local production of these cells. The histopathological study confirmed, in our case as in the others published, the massive invasion of the subarachnoid space by plasma cells while the dura mater is not invaded.

[Development of aphasic symptomatology with age. Discussion apropos of an anatomo-clinical case]. / Evolution de la sémiologie aphasique avec l'âge. Discussion à propos d'une observation anatomo-clinique.

Clinical data suggesting that aphasic semeiology undergo some evolution with age are summarized. Three kinds of hypotheses may account for this evolution: (1) a possible evolution with age of the site and extent of ischaemic lesions: (2) an evolution according to age of brain functional organization for language; and (3) evolution with age of psychological and/or social aspects of individuals in whom aphasia occurs. These hypotheses are discussed in the light of a clinico-pathological case of Wernicke's aphasia with logorrheic jargon due to an infarct involving both anterior and posterior parts of the left cortical speech area. This observation does not support the first set of hypotheses implying the lesion's site. On the other hand, as regards the second hypothesis, it is conceivable that the most instrumental features of speech functional organization may undergo a progressive subcorticalization process with age so that it possibly accounts for some age specificity in aphasic semeiology. Finally, the last set of hypotheses--individual's psychological and/or social aspects--although untested here, is thought to be also of some relevance.

[Progressive multifocal leukoencephalopathy. Clinical, CT scan and neuropathologic findings. Apropos of 4 cases]. / Leucoencéphalopathie multifocale progressive. Données cliniques, scanographiques et neuropathologiques. A propos de quatre observations.

Four cases of progressive multifocal leukoencephalopaty are reported, 3 of them with an ultrastructural study and 2 with a C.T. scan study. In 2 cases, there were no other associated pathological condition. In 1 case chronic alcoholism only was present; in the last case a primitive hemochromatosis with a prevailing hepatic dysfunction was evidenced. In 2 of these cases, the initial clinical picture was made of mental disorders while it was made of a progressive left hemiparesis in one case and of a brain stem syndrome in the other case. The results of neuroradiological and isotopical investigations remained negative throughout the evolution in the 4 cases except for some C.T. scan abnormalities. Indeed, in 2 cases the C.T. scan evidenced brain lesions identified as large demyelinated areas. These were low density areas non altered by contrast and unaccompanied by any mass effect. These areas were not seen when the first clinical signs were present and thus, normality of C.T. scan appears not to allow infirmation of the diagnosis of progressive multifocal leukoencephalopathy. In the 4 cases the pathological study showed characteristic lesions of the white matter. Mononucleated cells inflammatory infiltration was observed at the periphery of the demyelinizated regions. This may be related with the primitive aspect of these cases. An ultrastructural study was performed in 3 cases: viral inclusions were present not only in the nucleus of oligodendrocytes but also in their cytoplasm and in some astrocytes.

[Clinical and pathological study of a peculiar form of tuberculous meningitis: diagnostic and therapeutic problems (author's transl)]. / Etude anatomo-clinique d'une méningite tuberculeuse "crypto-bacillaire". Difficultés diagnostiques et thérapeutiques.

The authors report a case of tuberculous meningitis in which multiple bacteriological samples were negative during the eleven months of the clinical course. There were no other visceral localizations. The fatal outcome was due to vascular lesions (multiple cerebral infarcts), and to CSF flow disorders. The findings of cranial computerized axial tomography and of the pathological examination are reported.

Primary cerebral neuroblastoma in an adult man.

Survey of the literature reveals 10 cases of cerebral neuroblastoma in adults. An additional case, confirmed by electron microscopy, is reported here. The patient had a voluminous supratentorial calcified intraventricular tumor with clinical signs dating back more than two years. Despite the size and cellular immaturity of the lesion, surgical removal of the tumor was apparently satisfactory on macroscopic grounds. Treatment also included postoperative radiotherapy of the brain and spinal cord. The present case is discussed in light of the scarce data from the literature.

[Vascular epilepsy: clinical, electroencephalographic, and computerized tomographic aspects (author's transl)]. / Epilepsies vasculaires: aspects cliniques électroencéphalographiques et tomodensitométriques.

The authors report 31 cases of "vascular epilepsy" among 280 cerebral strokes confirmed by cranial computerized tomography. A high incidence of ischemia (28 cases : 90%) is noted. Epileptic seizures are initial (14 cases) or sequellar (17 cases) manifestations of cerebral stroke. Partial seizures are the most frequent (58%), particularly "Jacksonian" motor fits, which, when initial, often lead to status epilepticus. Frequency and bad prognosis of initial status epilepticus are pointed out.

[Status epilepticus and multiple sclerosis (author's transl)]. / Etat de mal épileptique et sclérose en plaques.

Status epilepticus was a complicating feature of the last bout before the fatal outcome in a patient with a 11 years history of multiple sclerosis. The fits were seen as clonic movements of the eyes in a lateral direction, lasting for 30 to 90 seconds and repeated every 5 minutes on average. Electroencephalographic recordings demonstrated periodic slow spikes on the right hemisphere, starting in the anterior region. Neuropathological data eliminated an associated pathological condition and suggested that the development of acute cortico-subcortical demyelinating lesions was responsible of the epilepsy. The type of electrical activity observed, localized to one hemisphere, is found with recent ischemic lesions, and less frequently in cases of cerebral tumor, necrotizing encephalitis, metabolic encephalopathies or post traumatic disorders. A case with similar clinical and electroencephalographic features in a patient with multiple sclerosis has been reported in the literature.

[Cerebral neuroblastoma in the adult. Clinical and C.T. scan aspects (author's transl)]. / Neuroblastome cérébral de l'adulte. Aspects cliniques et scanographiques.

The case of a thirty two years old patient with a frontal syndrome developing over the last three years is reported. CT scan showed a large calcified lesion, situated on the median line enhanced by iodine. The patient was operated. Ultrastructural and histologie studies concluded that it the tumor was a neuroblastoma. After operation an unquestionable amelioration of the frontal disorders appeared. Facial paralysis with a inverse automatic-voluntary dissociation and an underuse of motricity, both left-sided, after cortectomy of the right-sided premotor area were observed. We therefore suggest that the lesion of the external premotor cortex was responsible of the facial paralysis with an inverse automatic voluntary dissociation and of the underuse the left side.