INTRODUCTION:Miller-Fisher syndrome is defined by the triad: ophthalmoplegia, ataxia and areflexia. This rare entity is generally regarded as a variant of the Guillain-Barré syndrome, although neurophysiological patterns differ. In the acute phase of the disease, sera of affected patients contain high titers of antiganglioside anti-GQ1b, which is a specific marker. Recurrences are exceptional.EXEGESE:We report the case of a man with three recurrences of Miller-Fisher syndrome within 16 years. Anti-GQ1b antibody titers were elevated during an episode, decreasing but not completely and vanishing 2 years later. Intravenous human immunoglobulin treatment probably accelerated improvement at the two last episodes.CONCLUSIONS:Some experimental and immunohistochemical data from the literature argue for a probable direct pathogenic role of antibodies against GQ1b ganglioside in this syndrome. This should be a rationale for the use of immunomodulating treatments.

Background Native valve strands might be related to the acute stage of thrombosis or might suggest a long-term valvular change. We aimed to estimate changes in the strands in patients with stroke through a serial transesophageal echocardiographic (TEE) study.Methods and Results A study was conducted among patients who were referred for TEE for stroke or cardiac pathology. Patients had TEE examinations with a 5-MHz multiplane TEE probe. Echocardiography was repeated 3 months later in patients with stroke. TEE was performed in 180 patients admitted to cardiology units and in 160 patients referred to neurology units. Among 34 patients with valvular strands, 30 were referred to neurology for stroke, whereas 4 patients were admitted to cardiology (18.8% versus 2.2%, difference 16.5%, 95% confidence interval 10% to 22.9%, P = .001). Strands were located on the mitral valve in 16 patients, the aortic valve in 6 patients, and both left heart valves in 8 patients. Among the 38 valves with strands, 17 (44.7%) were morphologically normal, 4 (10.5%) were thickened, 7 (18.4%) were redundant and 10 (26.3%) had both abnormalities. TEE showed other abnormalities in 16 (53.3%) patients, whereas 14 patients had only strands. Twenty-six (86.6%) patients had a second TEE study 3 months later. Strands were not found in 4 (15.4%) patients (95% confidence interval 4.3% to 34.9%).Conclusions Valvular thickening or redundancy may predispose valves to strand formation. Native valve strands usually persist and thus reflect a chronic valvular change.

Among the patients showing evidence of cerebral metastases without previously known cancer history, lung cancer has been found 37 times as the primary tumor in our institution. There were 34 men and three women and all but two were heavy smokers. Only one presented at diagnosis with thoracic symptoms but the chest radiograph was abnormal in 34. The histologic type of the primary tumor was obtained in 32 cases as a result of thoracic investigations and in five cases from metastatic tumor tissue. The primary tumor appeared to be non-small cell lung carcinoma in 26 cases and small cell lung carcinoma in 11 cases. These results show that patients treated with surgery (20 cases) have a better survival (median 10 months versus 4.5) than the others, and among surgically treated patients only those treated with bifocal resection (eight patients) are long-term survivors. Also, in four of six patients, objective regression of the neurologic symptoms was seen after radiation therapy alone. Central nervous system relapse was seen in 12 patients, but in none of the patients treated with postoperative radiation therapy. Conventional chemotherapy (11 patients) induced objective responses only in the small cell type and proved to be too toxic when used simultaneously with radiation therapy in inoperable patients.

Observation chez un arboriculteur de 58 ans. Il s'agit d'un accident isole, il est legitime que l'intoxication et ses sequelles soient prises en charge en tant qu'accident de travail

Eight cases of [absence status] are reported, occurring in old patients (7 females, 1 male), mean age 75 years, without any known history of epilepsy. The main clinical feature was a fluctuating state of consciousness, during which the EEG showed diffuse spikes and polyspikes with a frontal predominance. These abnormalities disappeared after intravenous benzodiazepine administration, resulting in most cases in a spectacular clinical improvement. Five of our patients had a depressive history and were taking antidepressive or psychotropic drugs. Hyponatremia or hypokalemia probably secondary to diuretic medications, was present in 4 cases. No recurrence occurred with or without long-term anti-epileptic medication.

A 32 year-old woman after voluntary intoxication with methanol presented, after coma with metabolic acidosis, a prolonged parkinsonian syndrome improved by L-dopa. Initial findings were areflexia and electromyographic signs of neurogenic atrophy of lower limbs. A moderate decrease in visual acuity was associated with altered visual evoked potentials indicating a global lesion of the optic tracts. CT scan during the first few days showed symmetrical low densities areas in the putaminal regions, which unchanged 18 months later. This case can be compared with the rare reported cases with extrapyramidal signs, EMG tracing evidence of peripheral neuropathy and analogous images on CT scanning. The clinical sequelae and CT scan imaging anomalies are the consequence of the characteristic lesions of methanol intoxication, in which the role played by circulatory disorders and that related to the action of the toxic substance itself on nerve cells remains a debatable subject.

At the age of 6 years a patient developed disorders of character, intellectual deterioration, tremor, falls and epileptic seizures. This was followed by extrapyramidal and pyramidal disorders with a fatal outcome at age 21. There was no family history. Histopathology showed evidence of Hallervorden-Spatz disease, remarkable by the diffusion of spheroids into the central nervous system gray matter and by the presence of innumerable Lewy bodies in the substantia nigra and locus coeruleus. Similar findings have been reported in only 3 other cases of typical Hallervorden-Spatz disease. They suggest a preferential affection of monoaminergic neurons.

CT scan in 4 patients with Marchiafava-Bignami's disease in its acute alcoholic form, who died after 12, 18, 30 and 50 days respectively, demonstrated large symmetrical bilateral hypodense areas in the hemispheric white substance. These areas were either widespread or more marked posteriorly, and were independent of the corpus callosum. Pathologic examination of the brains showed necrotic regions, often with cavities, or areas of demyelinization of the centrum ovale correlating with the hypodense areas as seen on CT scans. In two cases CT scan further showed regions of linear hypodensity in front of and medial to the frontal ventricular horns which could have corresponded to the corpus callosum necrosis found on the brain sections. CT scan can thus contribute to the diagnosis of Marchiafava-Bignami's disease.

A 66-year-old man presented with a primary malignant lymphoma of the cerebellum and brain stem. The lymphoma was of type V (Bryon's classification) with predominant B cells, and was associated, as in previously reported cases, with a peripheral blood T lymphocyte deficiency. This case was unusual, in that autopsy revealed an active multivisceral sarcoidosis (considered as being cured more than 10 years previously), a clear-cell renal adenoma demonstrating nearly all the characteristics of a Grawitz's tumor, and a papillary epithelioma of the thyroid gland. These findings lead to discuss the significance of immuno-surveillance lack in this particular case: was it dependent on the sarcoidosis (during which a reduction in T lymphocytes is known to occur), or was it primary and perhaps genetic, a son of the patient having Hodgkin's disease? Whatever the case may be, the encephalic proliferation of the B lymphocyte clone, the peripheral epitheliomas, the sarcoidosis, and the deficit in T lymphocytes in the peripheral blood constitute a group of factors singularly rich in questions, this being, apparently, the first case of this type reported in the published literature.