[Recurrent familial neuropathy: report of one family]. / Neuropathie recurrente familiale a propos d'une famille.

The authors report a case of a family with hereditary compression of peripheral nerves. The disease is traced through five generations. Six members were proved to be affected. 14 patients were examined. The review of the literature and the family studied suggest that the disorder is transmitted by an autosomal dominant gene with variable expressivity.

The intranuclear filamentous inclusions of a human glioma. Their relation with nuclear bodies.

The intranuclear filamentous inclusions of a human glioma were analysed with an electron microscope equipped with a goniometer stage. The inclusions consist of 6 to 8 filaments. Considering the organization of the constituent filaments we distinguish three basic types: 1. Filamentous bundles of more or less parallel filaments, forming a cigarshaped inclusion. 2. Crystalloid inclusions: a. Prisms. They consist of stacked layers of strictly parallel filaments. The angle formed by the filaments of adjacent layers if 60 degrees. b. Cylinders. The layers of filaments are bent up and may form either a circle or a spiral, when the inclusion is seen in cross-section. 3. Partially crystalloid or "intermediate" inclusions. We consider them to be transitional forms between types 1 and 2 inclusions. The crystalloid layers of such intermediate inclusions may form either prisms or cylinders. Finally, the similarity between the granulo-fibrillar capsules surrounding granular nuclear bodies and filamentous inclusions, as well as the existence of granular material dispersed between the filaments of some inclusions led us to investigate a relationship between these two structures.

[Maintenance therapy in cranio-facial pain: a controlled trial with tiapride (author's transl)]. / Traitement de fond des algies crânio-faciales. Expérimentation du tiapride.

Tiapride, which belongs to the anisamide family, has an anti-dopaminergic effect, mainly on the mesolimbic system but also directly at the vascular level. A controlled trial was performed in 25 patients suffering from intractable migraine or facial vascular pain. Results were excellent in ten cases. Prescription of tiapride is therefore advised whenever usual therapy has failed.

[Malignant gliomas in adult. Interest of immediate radiotherapy in the combined protocols : chemotherapy (VM26-CCNU), radiotherapy. A cooperative study on 137 cases (author's transl)]. / Gliomes malins hémisphériques de l'adulte. Intérêt de la radiothérapie "seconde" dans les protocoles combinés : chimothérapie (VM 26-CCNU) - radiothérapie. Etude coopérative sur 137 cas.

The results of a multicentric study of three combined therapeutic protocols for malignant gliomas are reported. The goal was the evaluation of "Delayed" versus "Immediate" Radiotherapy. All patients were operated on, the radiotherapy was 5 800 rad. whole brain. The drugs used were : Day 1 - Day 2 Epipodophylotoxine 60 mg/m2/day. Day 3 - Day 4 CCNU 60 mg/m2/day. The mean survival times were : "total" surgery versus "partial" surgery: "total" group : 52 cases - mean survival : 44 weeks ; "partial" group : 60 cases - mean survival : 48 weeks : 1) protocol A : surgery + chemotherapy alone : 60 patients/34 weeks ; 2) protocol B : surgery + radiotherapy + chemotherapy :22 patients/43 weeks ; 3) protocol C : surgery + chemotherapy 4 cycles + radiotherapy + chemotherapy : 51 patients/71 weeks. The percentage of survivors at 12 months was Prot. A : 57% - Prot. B : 32% - Prot. C : 70%. These results are in favor of "Delayed" radiotherapy. They are statistically significant, and prompt us to pursue such a trial for getting confirmation upon numerically broader groups of patients.

[The biological basis of therapeutic strategies in malignant gliomas. General oncological data, current methods and results, prospects (author's transl)]. / Bases biologiques des stratégies thérapeutiques des gliomes malins. Données oncologiques générales, place des moyens actuels et résultats, perspectives.

The authors summarize briefly the biological basis of therapeutic regiments against malignant gliomas. They outline some factors able to contribute to the poor results that are obtained with the now in use strategies: paucity of drugs, tumoral sites out of drug access, inadequate timing of prescriptions, variable and unpredictable drug sensitivity of cells in one given tumor. They encompass the eventuality of progress upon the use of new laboratory methods allowing a better under standing of tumor biology.

[Drugs and indications for medical treatment in Parkinson's disease (author's transl)]. / Moyens thérapeutiques et indications du traitement médical de la maladie de Parkinson.

The authors present a review of the actual drugs efficient in the treatment of Parkinson's disease. They emphasize the frequency of the side-effects and consequently to the difficult management of Parkinsonism. The indications are schematically summed up.

[Progressive myoclonic encephalopathy in dialysis patients: presence of high concentrations of aluminium in the lysosomes of the cerebral cells (author's transl)]. / Encéphalopathie myoclonique progressive des dialysés: présence d'aluminium en forte concentration dans les lysosomes des cellules cérébrales.

Sampling of brain tissue of two patients who died of dialysis encephalopathy syndrome have been studied using a combined electron microscope-electron microprobe x-ray analyzer. X-ray microanalysis showed that aluminium is present in high concentration in lysosomes of brain cells. This concentration is approximately one thousand-fold that which is normally measured in the grey matter. In these lysosomes, aluminium is present in needle like microcrystalline form of 0.006 micron diameter and 0.08 micron long on the average; in these crystals, aluminium is associated with phosphorus in a ratio of one atom of aluminium per atom of phosphorus. Similar lesions have been observed in the rat after intraperitoneal injections of water-soluble aluminium salts.

[A case of familial muscle weakness corrected by exercise (author's transl)]. / Un nouveau cas de faiblesse musculaire corrigée par l'exercice (forme familiale).

The symptom described as muscle weakness corrected by exercise appears to be part of the recessive form of congenital myotonia, of which it can constitute the dominant clinical manifestation in some cases. This symptom coincides with decrements of the action of potentials to repetitive stimulations in the electromyogram, which may be particularly severe in these same cases. These observations contribute to the modification and precision of the description of the recessive form of Thomsen's disease, but their pathogenicity remains unclear.

[Shy and Drager syndrome. Physiopathological and pharmacological approach based upon one case (author's transl)]. / Syndrome de Shy et Drager. Approche physiopathologique et pharmacologique à propos d'un cas.

The authors report a new case of Shy and Drager syndrome characterized by the severity of the extrapyramidal signs as well as that of orthostatic hypotension and urinary dysfunction. Peripheral adrenergic disturbance was proven, associated with an abnormality of the renin-angiotensin system. The authors describe the various drugs tried experimentally in treatment of the three main symptoms of the disease. A combination of Trihexyphenidyl and Dibenzepine finally appeared to be the most effective. Six months later, there remained a marked improvement in extrapyramidal signs and orthostatic hypotension. By contrast there was no improvement in urological symptoms.

[Arguments in favor of the genetic origin of malformed syringohydromyelic pictures]. / Arguments en faveur de l'origine génétique des tableaux malformatifs syringohydromyéliques.

After a critical analysis of the criteria usually used for arguing in favor of the hydrodynamic determinism of the syringohydromyelic malformations, the authors report convincing data according to which such dysmorphic status would be the result of abnormalities in the genetic programs of the morphogenesis of the central canal.

[Neuropharmacological data on the striatum]. / Données neuropharmacologiques sur le striatum.

The striatum constitutes the most voluminous basal ganglia in man. It is issued from ganglionic eminences which are very early bound by limbic kernels. If the cortical and reticulo-spinal projections have been first described the existence of anatomical connexions with the limbic system offers a large number of functional possibilities. The knowledge of the distribution of the different chemical substances which are present within this structure as well as the enzymes necessary for their synthesis and destruction permits to establish a chemical mapping, the dopaminergic one being the best known. The dopaminergic synaptic function in the striatum helps to understand the respective roles of the pre and post-synaptic receptors as well as the mechanisms by which the other neuromediators can modulate the dopaminergic activity, the cyclic nucleotides being often necessary for this action. These fundamental data subtend the mechanism of action of most of the drugs which are involved in extrapyramidal phenomenons (neuroleptics, dopaminergic agonists) and allows to put forth physiopathological hypothesis on Parkinson disease, Huntington chorea, as well as certain induced or spontaneous dyskinetic states. The functions of the striatum are then evoked: if the role of this structure in motor control is critical, its involvement in complex behaviours is strongly suggested.

[Progressive myoclonic encephalopathy in dialysis patients. Clinical, electroencephalographic and neuropathological study. Pathogenetic discussion]. / L'encéphalopathie myoclonique progressive des dialysés (E.M.P.D.). Etude clinique, électroencéphalographique et neuropathologique. Discussion pathogénique.

Clinical and Neuropathological data on sixteen cases of progressive myoclonic encephalopathy are reported. This neurological syndrome appears after an average duration of thirty two months of haemodialysis and leads to death in four and a half months, and is characterized by myoclonus, speech disorder, epileptic seizures, and mental-status changes. At first, clinical signs and symptoms are related to haemodialysis, later they become permanent. An early diagnosis is based on EEG which is the only useful laboratory test, demonstrating bisynchronous slow-wave bursts. The caracteristic histopathologic findings are neuronal depopulation, lipofuscin accumulation, and appearance of Neurofibrillary degeneration, especially in Motor cortex, red nucleus and dentato-olivary systems. It seems to be justified to attribute P.M.D.E. to aluminium chronic poisonning; the source of the aluminium intoxication is not aluminium containing phosphate-binding gels but intravenously administreted tape-water. The intracellular binding of aluminium is shown from a histochemical study employing fluorescent stain Morin.

[Progressive myoclonic encephalopathy in dialysis patients. The role of the water used for haemodialysis (author's transl)]. / Encéphalopathie myoclonique progressive des dialysés. Rôle de l'eau utilisée pour l'hémodialyse.

The observation in 14 dialysis patients of an encephalopathy associating myoclonia, dysarthria, generalised seizures in some cases, worsening over a few months, led to an aetiological inquiry based upon comparative study of patients with or without encephalopathy treated in the same centre or at home, and controls. Higher levels of aluminium were found in the frontal cortex grey matter of encephalopathy patients as compared to the control group. The same applies to manganese in the white matter. Copper, zinc and iron contents were not different. Aluminium levels in blood, dialysis bath and tap water supply were higher in center dialysis than in home dialysis. Blood aluminium levels at the end of hemodialysis were correlated with bath aluminium levels. The ingestion of alumine gels was not greater in the encephalopathy patients than in other hemodialysis patients; its estimation, in each case, was not related to the blood aluminium levels at the begining of hemodialysis. These finding indicate the need of a routine measure of metal content - mainly aluminium and manganese - in tap water used for dialysis, in order to treat this water if necessary.

[A case of bilateral lesions in the temporal convexity: attempt to define symptoms]. / Un cas de lesions bilaterales de la convexite temporale: tentative de definition des symptomes

The case is reported of a boy aged 15 who suffered a bilateral lesion of the temporal convexity after a brain traumatism; he died 5 years later and the symptoms during that period are described. The clinical picture is adequately described neither under the title of dementia, nor under that of aphasia. The concept of an abolition involving the systems of cultural mediation (linguistic, technical) is introduced. The status of echolalia and echo-praxia is discussed.

[Major histocompatibility system in multiple sclerosis]. / Etude du système majeur d'histocompatibilité dans la sclérose en plaques

The comparison of Histocompatibility Testing in 82 MS Patients and 368 controls is presented. The increase in HL-A7 and decrease in HL-A12 are confirmed. A significant increase in HL-A8 is reported. Mixed Lymphocyte Reaction confirms the LD7a increase (19 out of 24 Multiple Sclerosis patients tested); 100% of the patients bearing the HL-A7 determinant are found to be LD7a. The presence of specific Immune Response genes in Multiple Sclerosis is discussed.