High-dose intravenous gammaglobulin for myasthenia gravis.
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Biomedical subjects
Publications and source records attributed to M Goulon.
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The Neuroleptic Malignant Syndrome (NMS) is a rare but severe affection (spontaneous mortality 30 to 50 per cent), associating fever, hypertonia with myolysis, and respiratory impairment. Its mechanism remains debatable: The origin of the hypertonia might be central (as phenothiazines and butyrophenones induce a blockade of dopaminergic receptors in the hypothalamus) or it might be muscular (with an impairment of the sarcoplasmic reticulum uptake of calcium in a genetically abnormal muscle, as is proven in malignant hyperthermia). Whatever the actual mechanism, the oral or intravenous administration of sodium dantrolene, a peripheral muscle relaxant agent which does not affect the neuromuscular transmission but prevents the calcium-dependent contraction of actin and myosine, has proved to be effective in three recent cases of NMS.
Hemiplegia after carbon monoxide (CO) poisoning is rare since only 9 such lesions have been reported from among 1480 cases of poisoning. The patient reported was a 43 year-old man with a left hemiplegia with coma following CO poisoning. Right carotid angiography demonstrated a hypoglossal artery supplying both posterior cerebral arteries. Left carotid angiography showed a dolichocarotid artery supplying the territories of the anterior and middle cerebral arteries on both sides. Complete regression of the hemiplegia occurred after 2 months following rapid administration of hyperbaric oxygen. Normal CT scan images and cerebral blood flow rates on follow-up examination suggest that the left hemiplegia was due to the combined effects of arterial anomalies and CO poisoning.
Three cases of Aeromonas hydrophila septicaemia are reported. The first case concerned a 51 years old patient suffering from drowning who had previously been in good health and who died after a few days of refractory hypoxaemia. The second case was an 8 years old child with trisomy 21 operated for a congenital cardiac defect. The portal of entry was a catheter and the septicaemia was complicated by A. hydrophila mediastinitis, a complication which has not been described previously. The third case was a 75 years old man with no previous medical history, whose hobby was gardening. He developed septicaemia after a flu-like illness. A. hydrophila is a mobile Gram negative bacillus whose natural habitat is water. It appears to be an exceptional and temporary contaminant of the human G I tract. Clinical infection is rare and usually focal: gastroenteritis, infection of soft tissues after trauma in aquatic surroundings. In 60 out of 82 cases there was a documented deficiency in the patient's immune defenses. A digestive portal of entry was suspected in 63 cases but only rarely proved. In one of our cases the portal of entry was septic thrombophlebitis induced by an indwelling catheter; this complication has not previously been reported. The clinical picture of A. hydrophila septicaemia is characterised by the relative high incidence of Echtyma gangrenosum. The lethal outcome in 50 p. 100 of cases is often related to the patient's general condition. The sensitivity of the three cultures isolated was tested against third generation cephalosporins: they were effective in each occasion.
A typical case of glycyrrhizin-induced hypokalemic paralysis in a diabetic patient is presented. Hypokalemia was responsible for a generalized muscle weakness with a rise in serum muscular enzymes and cardiovascular disorders, which improved under potassium supplementation and spironolactone therapy. The pseudoaldosteronism syndrome with low plasma renin activity and aldosteron suggested aldosteron -like liquorice intoxication. Patient's interrogation revealed regular consumption of a common aperitive drink: a non-alcoholic pastis containing small amounts of glycyrrhizin. The discrepancy between the severity of clinical manifestations and the ingested amounts of glycyrrhizin is explained by the diabetic state.
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A randomized study was carried out in 14 myasthenia patients to compare the long-term effects of two therapeutic regimens. Group I patients received prednisone 1 mg/kg/24 h for one month, then in decreasing dosage; in case of failure at the end of the first month, cyclophosphamide 2 mg/kg/24 h was added to the prednisone treatment. Group II patients received the same treatment associated with 3 plasma exchanges over a 10-day period; these were continued, if required, at the rate of once a week. The minimum follow up was one year. The results (greater improvement in muscular strength and vital capacity) were better after one month in group I and thereafter similar in both groups. However, the mean daily dose of prednisone was higher in Group I. The number of exacerbations of myasthenia was greater in group II (11 versus 2 in group I over a 24-months period). The mean fall in anti-R Ach ab was about the same in both groups. This study confirms the rapid effectiveness of plasma exchanges and their value in severe myasthenia. The higher incidence of exacerbations in Group II was probably due to a rebound phenomenon and points to the need for combined immuno-suppressive treatment.
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A 54-year-old woman, with no previously documented thyroid disease, treated with amiodarone (200 mg/day, five days a week for 33 months) for paroxysmal tachyarrhythmia complicating mitral stenosis, suddenly developed extremely severe thyrotoxicosis. After therapeutic failures with carbimazole and propylthyrouracil (PTU) associated with beta-blockers, she was transferred to intensive care for plasma exchange (PE). Two PE were performed, temporarily aggravating the cardiovascular status of the patient, with no secondary improvement. The quantity of T3 removed was very small, about 1,000 ng per exchange. On the 14th day PTU had to be discontinued (toxic thrombopenia) and only symptomatic treatment was maintained (assisted ventilation, digitalis, hyperalimentation). In the 4th month, while the patient had a high total serum iodine, hypothyroidism developed due to partial block of the organification of the iodine with high TSH and fixation; this state also lasted 4 months. Spontaneous recovery was observed after 8 months. In addition a severe peripheral neuropathy was observed during the hyperthyroid phase confirmed by electromyography, distinct from the signs of thyrotoxic myopathy. This gradually regressed over 7 months and may be attributed to amiodarone therapy. The association of these two successive types of thyroid disorder due to amiodarone is an exceptionally rare phenomenon. Severe thyrotoxicosis generally requires long-term symptomatic therapy, its natural course being towards spontaneous regression. PE are ineffective on the circulating hormonal levels and were dangerous because of the underlying cardiac disease. The development of hypothyroidism at the 4th month is explained by the persistent iodine overload, and therefore prolonged surveillance after withdrawal of therapy is advised. The neurological complication of amiodarone was quite distinct from the hyperthyroid myopathy.
Two schizophrenic patients developed muscular rigidity, stupor, and hyperpyrexia consistent with neuroleptic malignant syndrome, 8 to 10 days after starting haloperidol therapy. Muscle rigidity was not affected by etybenzatropine or diazepam, but dantrolene, a direct-acting skeletal muscle relaxant, provided muscle relaxation with a concomitant decrease of fever and serum creatine kinase. Neuroleptic malignant syndrome and malignant hyperthermia are clinically similar, and dantrolene is effective in both; suggesting a muscular origin of fever in these two diseases.
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Sixty patients including forty two males and eighteen females, age range: 18-87 years, received antibacterial single drug treatment with latamoxef for septicemia. Forty nine patients had underlying conditions including multiple trauma, neoplasm, cardiovascular, metabolic and respiratory tract diseases. Causative pathogens were isolated in all cases. The predominant isolates were E. coli (thirty), Klebsiella pneumoniae (tent) and Enterobacter (seven). A single organism was isolated in fifty seven cases; in the other three cases two organisms were isolated from blood cultures. Mean daily dosage was 46.6 +/- 6.1 mg . kg-1 (range: 14-113 mg . kg-1). In the majority of cases (fifty two) dosage was 3 g . d-1 or less; in thirty cases it was no higher than 2 g . d-1. Duration of therapy ranged from six to thirty eight days. Serum titer was measured in many cases and latamoxef blood levels were assayed in nine patients. A satisfactory clinical response was achieved in fifty eight cases and fifty eight bacteriological cures were also obtained. There was no statistically significant difference in therapeutic response between the 2 g and 3 g daily dosage groups. Tolerance was very good; untoward effects were few and required drug discontinuation in one case only.
Twenty-three cases of disseminated candidiasis (DC) were observed between 1965 and 1981. In 23 cases the DC was a complication of a preexisting disease process (16 abdominal surgery, 18 bacterial septicemia, 16 acute renal failure, 4 carcinoma) requiring polytherapy (23 antibiotherapy, 5 parenteral nutrition, 4 corticotherapy). The most frequent foci were ocular (6), peritoneal (5), renal (4), and pulmonary (3). Diagnosis was confirmed by blood culture (20), urine culture (12), culture of the catheter tip (7), isolation of candida from a localised focus and by anti candida antibodies in some patients. Nine patients were not treated and died; 14 patients were given antifungal agents: 6 died, three despite cure of DC. The recognition of favouring circumstances, early diagnosis and systematic treatment of all foci of candidiasis allow effective prophylaxis against DC; the last case in our series was in 1978.
A cellulose diacetate membrane was tested in 60 plasma exchanges (PE) carried out in 16 patients. Access to the vascular compartment was obtained through one single puncture by inserting a catheter into the femoral vein, the blood circuit being provided with an alternating clamp. The exchange of 2 plasma volumes (72.3 +/- 16 ml/kg) reduced IgG, IgA and IgM concentrations to 30.9 +/- 12.1%, 31.6 +/- 14.5% and 28.3 %/- 15.0% respectively of pre-PE concentrations. Similarly, the C3 and C4 complement fractions and factor B were reduced to 32 +/- 12%, 33 +/- 15% and 36 +/- 16% respectively of pre-PE values. The membrane was used several times in 7 patients; its performance remained the same after 3 consecutive uses. In view of its effectiveness and ease of application, the cellulose diacetate membrane appears to be particularly suitable for plasma exchanges in intensive care units.
Sixteen patients, 9 to 81 years old, with Gram-negative septicaemia have been treated with cefotaxime. In all cases, the patients were hospitalised in an intensive care unit for a severe infection distinct from septicaemia. Cefotaxime was given alone to 11 patients and was administered by infusion over 20 to 30 minutes, every 4 or 6 hours, in a daily dose of 67 to 94 mg/kg to patients with normal of subnormal renal function. Gram-negative pathogens were sensitive to cefotaxime (MIC less than 1 mcg/ml in most cases). A clinical cure was obtained in 14 patients. In all the cases, tolerance was good. The very low MIC's of cefotaxime and the results in severe Gram-negative septicaemia justify the use of cefotaxime in monotherapy, in patients with severe life-threatening infections, when blood cultures appear to be positive for Gram-negative pathogens, even without awaiting antibiograms.
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