[Autoimmune familial myasthenia in 2 sisters with a follow-up of more than 30 years].

The clinical course of two sisters with myasthenia initially published in La Revue Neurologique in 1960 has been followed. Both had secondary severe respiratory impairment requiring a tracheotomy and mechanical ventilation. Thymectomy was performed in both and revealed residual thymic tissue.

[Recurrence of Guillain-Barré syndrome].

Fifteen of the 238 patients (6.3%) who recovered from an initial episode of Guillain-Barré syndrome had one or several acute relapses. Usually, there was only one relapse.

[Teaching emergency medicine].

In France, emergencies are taught: to all medical students in the course of their studies; a specific emergency course being rarely given as such; to physicians who want to practice in emergency system: transport, emergency rooms, in association with intensive care medicine. The head of the emergency service should have a full time position, and whatever his original specialty, should receive a specific education in critical care medicine. Nurses who attend different congresses to complete their formation are not yet rewarded with a specific status.

[Familial mitochondrial encephalopathy. A clinicopathologic study].

We report the cases of 2 siblings with progressive encephalopathy. The first symptoms were noted when they were 6 years old. The full clinical picture included myoclonus, seizures, cerebellar ataxia, blindness due to optic atrophy and retinal degeneration, deafness, swallowing difficulties with relatively spared intellectual functions.

[Coma and cerebral death].

The concept of cerebral death is universally accepted. It rests on a panel of signs which must all be present and constant, and provided this is the case no error is possible. No authentic case throwing doubt on this statement has ever been reported.

[Evolution of the conditions for EEG recording in the diagnosis of brain death].

The authors trace the evolution of conditions of EEG recording parallel to the clinical criteria of certainty of the diagnosis of brain death, since the first definition of irreversible coma given by M. Goulon and P. Mollaret in 1959.

[Treatment of severe myasthenia gravis with cyclosporin. A 12-month open trial].

Cyclosporine A, an immunosuppressor and immunomodulator, has been proposed as an alternative in patients with autoimmune disease not responding to the usual immunosuppressants. We treated with cyclosporine 19 patients with severe myasthenia gravis in a 12-month open uncontrolled trial. There were 14 women and 5 men aged 51.

[Extrapyramidal syndrome after cardiocirculatory incompetence].

A 43 year-old woman suffered a cardio-circulatory arrest with a post-anoxic coma during 24 hours. This was followed by and akinetic-hypertonic syndrome. There was also dystonia of both hands and of right big toe.

[Amyotrophic lateral sclerosis and respiratory assistance].

Respiratory insufficiency of restrictive origin, often aggravated by obstructive disorders, is constant in amyotrophic lateral sclerosis (ALS), and is usually progressive. Respiratory distress may be life-threatening while the neurologic disease is still limited. Furthermore, the diagnosis of ALS is not always obvious when urgent therapeutic decisions have to be made.

The clinical spectrum of alcoholic pellagra encephalopathy. A retrospective analysis of 22 cases studied pathologically.

A retrospective clinical study of 22 heavy alcohol drinkers is reported in which postmortem study showed diffuse chromatolysis of neurons identical to that found in neurological pellagra, associated in 13 cases with Marchiafava-Bignami disease and/or Wernicke-Korsakoff disease. The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus. Because of the frequent coexistence of other alcoholic encephalopathies in the same patient, alcoholic pellagra was often unrecognized.

[Neuropathologic study of 15 cases of multinucleated giant cell encephalitis in acquired immunodeficiency syndrome (AIDS)].

The central nervous system (CNS) of 40 patients who died of acquired immune deficiency syndrome (AIDS) between August 1982 and August 1987 was examined. In 15 cases, multinucleated giant cells (MGC) characteristic of Human Immunodeficiency Virus (HIV) infection were observed. In 3 cases ultrastructural examination disclosed HIV-like viral particles in the cytoplasm of some MGC.

[Anoxic encephalopathy after cardiocirculatory insufficiency. Neuropathological study apropos of 16 cases].

Sixteen cases of anoxic encephalopathy in adult patients admitted to the intensive Care Department of Raymond Poincaré Hospital (Garches) have been selected on the following criteria: resuscitation by external cardiac massage for cardio-respiratory failure, without any previous history of anoxia and with detailed data on the subsequent evolution of neurological signs and symptoms. Main clinical features were compared to the results of the neuropathological examination. The pattern and the distribution of changes are detailed.