[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.

[Dysautonomia in acute primary polyradiculoneuritis]. / La dysautonomie des polyradiculonévrites aiguës primitives

In this research involving 172 cases of acute primary polyradiculoneuritis, the authors draw attention to the frequency and seriousness of the autonomic disorders, notably circulatory disorders (arterial hypertension, bradycardia), water retention, disorders in glucose metabolism. They have found a close correlation between the development of arterial hypertension and levels of VMA and the cathecholamines and between the appearance of hyperglycaemia and the level of urinary 17 OH. Free water clearance is often negative and becomes positive as the paralytic syndrome improves. The biological picture is identical with that brought about by inappropriate secretion of HAD. From the therapeutic point of view, caution is advised in using certain procedures and in prescribing certain drugs.

[Methyl bromide poisoning. 3 cases, 1 fatal. Neruopathological study of one case of coma with myoclonus followed for 5 years]. / Intoxication par le bromure de méthyle Trois observations, dont une mortelle Etude neuro-pathologique d'un cas de stupeur avec myoclonies, suivi pendant cinq ans

Three new cases of methyl bromide poisoning (leakage from a fire extinguisher) are reported. Two of these were characterized, after an initial period of coma and myoclonic status, by an action myoclonus. Electrical data are presented. A follow up of 4 and 8 years allowed to study the effect of drugs, two of which (clonazepam and baclofene) being pecularly contributive. The third patient outlived for 5 years in a stuporous state with myoclonus. The anatomical findings consisted mainly of a necrosis of both inferior colliculi with gliosis in the upper brain stem reticular formation and moderate changes in the dentate and pontine nuclei. Their significance is discussed in view of physiological and experimental knowledge.

[Solitary listerial abscess of the brain stem. Cure with antibiotic treatment]. / Abcès listerien solitaire du tronc cérébral. Guérison sous traitement antibiotique.

A healthy 26-year-old man, without initially presenting fever, rapidly developed a focal right pontomedullary deficit associated with an aseptic lymphocytic meningitis. The diagnosis of Listeria infection was confirmed by blood cultures. CT and MRI demonstrated an abscess extending from the superior cerebellar peduncle to the lateral portion of the medulla. Immunological controls gave no indication of deficiency. With ampicillin therapy, started on the 5th day, clinical recovery was almost complete, but a soft palate right paresis persisted as the unique sequel. Antibiotic therapy was maintained for 5 months up to normal CSF and CT. One year after the onset, MRI was also normal. The rare nature of listerial abscess in the brainstem is discussed with regard to rhombencephalitides.

[Diabetic hyperlipemia with or without acute pancreatitis in patients with chronic alcoholism. A study of 4 cases]. / Lipémie diabétique avec ou sans pancréatite aiguë chez l'alcoolique chronique. Etude de 4 observations.

Diabetic lipemia with and without acute pancreatitis in chronic alcoholism. A report of 4 cases. Diabetic lipemia was observed in 4 chronic alcoholic men after ingestion of high doses of alcohol and/or sugar-rich beverages, including one patient who was treated for insulin-dependent diabetes. None had a previous history of serum lipid disturbances. All had marked hyperglycemia, hyperosmolality and hypertriglyceridemia (mean: 60.8 mmol/l), 2 of undetermined type and 2 of type IV with eruptive xanthomas. Factitious hyponatremia was present in 3 cases, but true serum sodium was normal (138 mmol/l) or elevated (154, 156, 182 mmol/l) after correction. Three patients developed acute pancreatitis ascribed to high serum triglyceride levels and/or to alcohol ingestion. Serum and urine amylase activity was inhibited by hypertriglyceridemia. The diagnosis of pancreatitis was assessed twice by echography and computed tomographic scan, and once by tomographic scan and an elevation of the amylase on creatinine clearance ratio. It is likely that hypertriglyceridemia predisposed these patients to develop pancreatitis, alcoholism being a precipitating factor. We suggest that the diagnosis of acute pancreatitis should be systematically considered in any case of diabetic lipemia without true hyponatremia.

[Prognosis of post-anoxic coma]. / Pronostic des comas post-anoxiques.

The outcome of anoxic coma following cardiac arrest depends on the aetiological circumstances, on pre-existing visceral deficiencies and on the duration of inefficient circulation. Outside the extreme cases of prompt return to consciousness or early death, in many patients this course is marked by neurological sequelae of varying severity which may result in a persistent vegetative state. Initially, there is nothing that can predict the quality of survival, but within 72 hours the neurological examination usually makes it possible to foresee irreversible situations with permanent loss of consciousness. The decisional problems that ensue are discussed.