[Autoimmune familial myasthenia in 2 sisters with a follow-up of more than 30 years]. / Myasthénie familiale auto-immune deux soeurs suivies pendant plus de 30 ans.

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. Complete remission was observed long after thymectomy even with the persistence of antiacetylcholine receptor antibodies which are still present in the older sister despite intercurrent autoimmune thyroiditis and in the younger sister despite the development of mediastinal lymphosarcoma leading to death 32 years after the onset of myasthenia.

[Recurrence of Guillain-Barré syndrome]. / Récidives du syndrome de Guillain-Barré.

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. The durations of the plateau and recovery phases of the relapses were longer than those of the initial episodes. The protein level in the cerebrospinal fluid was higher during relapses (1.37 +/- 1.30 milligrams) than during the initial episode (0.87 +/- 0.44 milligrams). The intensity of the symptoms during relapses was comparable to that of the initial episodes, but the occurrence of these relapses aggravated functional sequelae. Comparison of relapsing patients with those who experienced monophasic Guillain-Barré syndromes and were treated in the same unit revealed no element predictive of relapses.

[Teaching emergency medicine]. / Enseignement de la médecine d'urgence.

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. In addition, first aid measures should be thought to the general public, so it can help injured persons.

[Familial mitochondrial encephalopathy. A clinicopathologic study]. / Encéphalopathie mitochondriale familiale. Etude clinicopathologique.

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. The course was progressive and led to death within 8 years. The pathological findings included bilateral and almost symmetrical lesions involving the thalami, the colliculi, and the pontine and medullar tegmentum, similar to the changes described in Leigh disease. Neuronal loss and gliosis were noted in the dentate nucleus and in the inferior olive, as in MERRF syndrome. Laminar necrosis of the cerebral cortex could have been due to episodes of severe hypotension before death. Cytochrome c oxidase deficiency was found in case 2. The enzyme deficiency was present in muscle and in fibroblasts in culture.

[Coma and cerebral death]. / Coma et mort cérébrale.

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. It has been said that the term "coma dépassé" which we chose in 1959 could lead to confusion with the different degrees of coma and for this reason, be an obstacle to organ donation. I do not consider this a satisfactory explanation. In favour of the appellation "coma dépassé" are the originality of its first description and its use in the French medical language. It does not matter if "cerebral death" is preferred, both terms being synonymous. The individualization of "coma dépassé" and the possibility of grafting organs with success are acquisitions separated by only a few years. They have made it possible to obtain the remarkable successes known to every one, they have given medical thinking a new dimension and placed upon doctors an increased responsibility.

[Evolution of the conditions for EEG recording in the diagnosis of brain death]. / Evolution des conditions d'enregistrement de l'EEG pour le diagnostic de la mort cérébrale.

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. They notice that over the past 30 years, the duration of the EEG has been reduced, their frequency decreased, and the delay between the clinical diagnosis of brain death with flat EEG and the switching off of the cardio-respiratory ventilation have been reduced from 48 to 6 h, thus enabling extremely early removal of organs; the slow progress of this evolution is explained by the difficulty over the years in distinguishing between brain death and certain reversible pathological conditions which resemble it, and by the fact that some EEGs may take some time to become flat. Actually, once these special cases have been eliminated, after the clinical diagnosis of brain death has been ascertained, we observe that two flat EEGs at an interval of 6 h are never subsequently modified. Finally, the EEG, a non-invasive method, seems always to be the quickest and the easiest method to carry out and especially to repeat, without any particular problem of staff or of place and is, thus, a reliable testimony in the event of medico-legal controversy.

[Treatment of severe myasthenia gravis with cyclosporin. A 12-month open trial]. / Traitement de la myasthénie grave par la ciclosporine. Etude ouverte de 12 mois.

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 51.5 +/- 18.5 years (range 20 to 81 years) who had had the disease for 50.8 +/- 43.6 months. Myasthenia gravis was generalized in 18 and ocular in 1. Previously, 4 patients had been unsuccessfully treated with azathioprine, 1 with cyclophosphamide, 12 with steroids; 5 had undergone thymomectomy and 5 thymectomy. Anti-acetylcholine receptor antibodies (AChR-ab) were detected in the sera of 15. The severity of the disease was assessed by a myasthenic muscle score (MMS) ranging from 0 to 100 and a 5-grade functional scale (FS) from 1 = severe disability to 5 = complete remission. Depending on changes in MMS and FS, the effectiveness of cyclosporine was deemed very good, good or nil. Cyclosporine was administered orally 3 times daily in doses of 5.6 +/- 1.6 mg/kg/day over 11.6 +/- 1.6 months. The final dosage was targeted to achieve a peak serum level and a trough level respectively below 200 and 100 mg/ml, and was adapted if side effects occurred. No other cytotoxic drug was allowed. Seventeen patients completed the treatment (M12); 1 died of uterine cancer at M6 and 1 was lost to follow-up at M5. At M12 clinical results were considered very good in 6 patients, good in 8, nil in 3. The MMS increased significantly from 56.1 +/- 17.2 (n = 19) at D0 to 83 +/- 15 (n = 17) (P less than 0.0005), as did the FS, from 2 +/- 1 to 3.7 +/- 0.85 (P less than 0.0005). Subsequently, cyclosporine was discontinued in 7 of the 14 patients who responded well and maintained in 7 at a reduced dosage (3.4 +/- 0.8 mg/kg/day). In the former group 3 patients relapsed 1, 1 and 5 months respectively after the drug was withdrawn; the remainder had, at the last follow-up (29.6 +/- 8.5 months from D0), MMS 62.9 +/- 34.1 and FS 3 +/- 2. In the latter group, at the last follow-up (17.6 +/- 3.6 months), MMS was 86.7 +/- 16.6 and FS 4.2 +/- 0.8, differing significantly from D0 values. AChR-ab titers and CD4/CD8 were unaltered at M12.(ABSTRACT TRUNCATED AT 250 WORDS)

[Extrapyramidal syndrome after cardiocirculatory incompetence]. / Syndrome extra-pyramidal après incompétence cardio-circulatoire.

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. After and initial mutism, the patient spoke with dysarthria, a monotonous weak voice of poor timbre and low vocal volume. She had in addition mood disturbances with indifference to her condition and compulsive activity. Extrapyramidal syndromes after ischemic anoxia are rare, when compared to their relative frequency after carbon monoxide poisoning. Early CT scan with contrast can identify symmetrical and bilateral lenticulocaudal high densities and MRI is also useful for the diagnosis.

[Amyotrophic lateral sclerosis and respiratory assistance]. / Sclérose latérale amyotrophique et assistance respiratoire.

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. Results are reported in 16 patients with ALS treated by assisted ventilation: 14 by endotracheal and 2 by nasobuccal routes. Transient improvement in respiratory function in 9 patients allowed partial return of spontaneous respiration. Known mean duration of course (n = 14) from first sign to death (12 cases) or last control (2 cases) was 3 years 3 months (1 to 9 years). Survival from tracheotomy to death or last control (July 1988 in 12 cases) was 12 months (3 to 48 months). These patients can now return home under the care of associations providing respirators.

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. Fifteen patients received thiamine and pyridoxine therapy without niacin. It appeared to aggravate the neurological state or to trigger the development of alcoholic pellagra encephalopathy in 9 cases. The relationship between pellagra occurring during thiamine and pyridoxine therapy and 'nicotinic acid deficiency' is discussed. Multiple vitamin therapy should be given in the treatment of undiagnosed encephalopathies in alcoholic patients.