[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.

[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.

[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.

[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.

[Treatment of status epilepticus in the adult. Retrospective analysis of 192 cases treated in intensive care units]. / Traitement de l'etat de mal épileptique de l'adulte. Analyse rétrospect 192 cas traités en réanimation.

The results of therapy have been analyzed in a series of 192 patients admitted for status epilepticus over 7 years in two intensive care units. Most (142 cases without any prior epilepsy) corresponded to secondary forms. In 2/3 of the cases, the patients were admitted because of failure of benzodiazepines and/or phenobarbitone. Sodium thiopentone achieved control of seizures in 75%; short-acting barbiturates should be especially prescribed in grand mal status with impending brain anoxia. Diphenylhydantoin would appear suitable in non-life-threatening conditions such as serial seizures or partial status. Chlormethiazole often succeeds in controlling convulsive status which has proved refractory to other treatment. Supportive management is mandatory: 52% of patients required respiratory assistance. Fatalities (36%) exclusively correspond to the underlying cerebral conditions and systemic disorders.

[Status epilepticus in the adult. Epidemiologic and clinical study in an intensive care unit]. / Etat de mal épileptique de l'adulte. Etude épidémiologique et clinique en réanimation.

The present study, conducted between January 1975 and June 1983, includes 282 adult patients admitted for status epilepticus (SE) in 2 intensive care units (3.5% of all admissions). In their great majority, i.e., 201, patients had no previous history of epilepsy, and initiated the SE during the course of recent affections such as brain or systemic diseases. In 81 patients with previous seizure, epilepsy was idiopathic in 20, and symptomatic in 61, the 3 main causes being cranial traumatism, cerebrovascular accident and chronic alcoholism. SE was of various causes. The most frequent were cerebro-vascular disease, post-anoxic encephalopathy, intoxication, bacterial or viral meningo-encephalitis. In some patients, SE was preceded by manifestations such as convulsions, behavorial disturbances or conscience impairment. One should be aware of these facts and undertake or increase an anticonvulsivant medication at their onset. Duration of SE, when clearly established, was highly variable but had no apparent correlation to sequelae or death occurrences. SE itself was directly related to death in only 2 cases; in all other instances death was the consequence of the primary disease. These results are consistent with previous reports: presently SE occurs more frequently in the course of other affections than in prior epileptic patients. The better vital prognosis of SE is due to the simultaneous conjunction of new anticonvulsivant medication and supportive care associated with the cure of the causative disease.

[Non-ketotic hyperglycaemic coma with diabetic lipaemia and hyponatraemia (author's transl)]. / Coma hyperglycémique non cetosique avec lipémie diabétique et hyponatrémie.

A 68-year old male patients without personal or family history of diabetes mellitus developed diabetic lipaemia with severe hyponatraemia and became deeply comatose. The low blood sodium level (110 mmol/l) was consecutive to hyperglycaemia (45 mmol/l), hyperlipaemia (65 g/l) and true sodium depletion. This rare form of type V hyperlipidaemia is attributed to deficiency of heparin-activated lipoprotein lipase; it rapidly responds to insulin therapy. Hyponatraemia is a prominent feature of the syndrome, but it is partly due to blood dilution resulting from hyperglycaemia and therefore fictitious. For accurate measurement of sodium depletion blood sodium levels must be adjusted taking into account blood lipoprotein and glucose levels.

[Generalized saturnine paralysis. Discovery of a double congenital disease: glucose-6-phosphate dehydrogenase deficiency (new variant) and distal tubular acidosis]. / Paralysie saturnine généralisée. Découverte d'une double affection congénitale: déficit en glucose-6 phosphate déshydrogénase (variante nouvelle) et acidose tubulaire distale.

A case of severe lead poisoning with haemolytic anemia, extensive polyneuropathy and arterial hypertension is reported in a patient for whom G-6-P-D deficiency and renal tubular acidosis were discovered. Both traits were present in his family. Incomplete regression of paralysis and persistant biological abnormalities after chelating treatment were demonstrative of heavy saturnine load even though the toxic exposure was brief. The role of the two deficiencies in the mechanism of intoxication is discussed: facilitation of haemolysis by enzymopathy and bone lead redistribution resulting from osteolysis probably due to the tubulopathy. The authors emphasize the fact that thorough and serial biological investigation is essential in the discovery of factors which may precipitate lead poisoning, as well as in the determination of follow-up criteria of efficient toxic epuration with sufficient delay. Typing of the enzyme has led to the identification of a new variant of glucose-6-phosphate dehydrogenase deficiency.