Non-hereditary multiple telangiectasias of the central nervous system. Report of two clinicopathological cases.

We describe 2 clinicopathological cases of non-hereditary multiple telangiectasias of the nervous system. In the first case, the general course of the disease was characterized by spells over a period of 7 years and the major lesions successively involved cranial nerves, spinal cord and brain. Neuropathological examination showed both ischemic and hemorrhagic changes. Systematized degenerative changes were found and were similar to those observed in spinocerebellar heredodegenerations (Friedreich's ataxia). In the second case, the 3 years of evolution were characterized by spells of the encephalitic type only. A review of the literature indicates the rarity of this kind of disease, the usual localization of capillary malformations and the clinical polymorphism (epilepsy, strokes, multifocal syndromes masquerading as multiple sclerosis). The pathogenesis of pathological changes is discussed.

[Stuporous state in an epileptic treated with sodium valproate. Diagnostic problems]. / Etat stuporeux chez un épileptique traité par le valproate de sodium. Problèmes diagnostiques.

The authors report a case of stupor secondary to valproic acid, in a patient treated with this drug and phenobarbital for a complex partial form of epilepsy. The pathogeny of this rare complication is discussed. In this patient, a paradoxical epileptic reactivation is excluded, and the authors favor a metabolic mechanism: either hyperammoniemia or a perturbation in cerebral neuro-transmitter systems are proposed.

[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.

[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.

[Myoclonal encephalopathy due to dopamine]. / Encéphalopathie myoclonique à la dopamine.

Onset of confusion with myoclonus occurred in a 55-year-old patient hospitalized for infectious endocarditis and under intravenous dopamine. The speculative responsibility of dopamine in the occurrence of this myoclonic encephalopathy is discussed. Indeed, although dopamine does not cross the blood-brain barrier, inflammation of the wall of the cerebral vessels (immunologic vasculitis) may have enabled dopamine to reach the central nervous system.

[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.

[Present data of Fahr's syndrome. Report of two cases of idiopathic strio-pallido-dentate calcifications revealed by computerized tomography (author's transl)]. / Aspects actuels de la maladie de Fahr. A propos de deux observations de calcifications strio-pallido-dentelées idiopathiques révélées par la scanographie.

The authors report two computed tomographic observations of Fahr's disease without disturbances in calcium-phosphorus metabolism; the first case is a by chance discovery and the second one is characterized by a cerebellar tremor. From the literature data they discuss successively clinical, radiological and computed tomographic, neuropathological, aetiopathogenetic and therapeutic aspects actually presented by strio-pallido-dentate calcifications (SPD) or Fahr's disease.

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.

[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.

[Neuropathological study of adult intracranial hemorrhage. III. Analysis of 107 meningeal hemorrhages due to rupture of arterial aneurysms]. / Etude neuropathologique des hémorragies intracraniennes de l'adulte. III. Analyse de 107 hémorragies sous-arachnoïdienns par rupture anévrysmale.

The findings in 107 pathological studies of meningeal hemorrhages due to rupture of arterial saccular aneurysms are reported. There were 62 women (58 p. 100) and 45 men (42 p. 100). Mean age was 56, lower in men (53) than in women (58). 45 aneurysms of the Anterior Communicating Artery, 26 of the Middle Cerebral Artery, 15 of the Internal Carotid Artery, 10 of the Anterior Cerebral Artery, 1 of the Anterior Choroidal Artery and 10 of the Posterior System were studied. These ruptured aneurysms were compared to 31 non ruptured ones. Mean size of the ruptured aneurysms was 10, 62 mm, not statistically different from that of non ruptured aneurysms (9, 05 mm). Multiple aneurysms represent 10 p. 100 of the whole ruptured aneurysms population. They affected mostly the Middle Cerebral Artery. High blood pressure had been present in 56/107 cases of ruptured aneurysms (52 p. 100). The prevalence of high blood pressure in patients with ruptured aneurysms (men: 60 p. 100 between 35 and 49, 63 p. 100 between 50 and 64; women: 31 p. 100 between 35 and 49, 54 p. 100 between 50 and 64) was statistically higher than in the same age and sex ranges of the general French population. The other causes of bleeding were rare: 4 liver cirrhosis (2 of the patients were also hypertensive) and 5 anticoagulant therapies (2 of which were also associated with high blood pressure). Intracerebral hematomas were found in 43 cases (40 p. 100): 39 lobar, more frequent in the frontal (26) than in the temporal (13) lobes, due mainly to Anterior Communicating Artery and Anterior Cerebral Artery aneurysms; very few were in the basal ganglia (3) or brainstem (I). Intraventricular hemorrhage was found in 39 cases (36 p. 100). Forty (37 p. 100) cerebral infarcts had occurred. They were located in the territory of the same artery in 20 cases (19 p. 100), in a different territory in 11 cases (10 p. 100), in both in 9 cases (8. p. 100).

[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.

[318 cases of intracerebral hemorrhage. A pathologic study (author's transl)]. / Etude anatomique de 318 hémorragies intra-parenchymateuses de l'adulte.

The systemic analysis of cerebral hemorrhages (large single and multiple hematomas and small slit-hemorrhages) has been practiced in 318 patients. The emphasis is put on the site of the bleeding and the etiological data. Single hematomas were situated: 1) in 127 cases in the basal ganglia region (26 were medial, 44 intermediate, 48 lateral, 8 quadrilateral and one subthalamic), 2) in 67 cases in cerebral lobes (24: front; 22: temporal; 21 parietal) and 3) in 33 cases in the posterior fossa (19: cerebellum; 14: brain stem). Large hematomas in 71 cases and slit-hemorrhages in 20 others were multiple. In more than one case out of three, lateral hematomas extended into the temporal lobe and in one case out of two, the internal capsule was affected. The incidence of high blood pressure is significantly higher in patients with intermediate haematomas and slit hemorrhages. This factor is often found in lateral or cerebellar hematomas. Anticoagulant therapy is more frequent in lobar hematomas. In 43 cases without any proved etiology, the patients' mean age of death is inferior to that of the whole studied population. These findings are discussed in relation with the literature data.