Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study.

We studied the effects of intravenous immunoglobulin (IVIg) in 12 patients with motor neuron syndromes associated with high titers of anti-GM1 antibodies. Five of the patients had conduction blocks. The study design was a double-blind, placebo-controlled, crossover trial with IVIg (0.4 g/kg body weight per day injected for 5 consecutive days). We evaluated the patients before and 5, 28, and 56 days after drug administration using a computerized analyzer for muscle strength, the Norris scale for disability, motor nerve conduction velocities for patients with conduction blocks, and measurements of immunologic markers. Compared with placebo, IVIg induced a significant increase in muscle strength only in the patients with conduction blocks.

A controlled one-year trial of dextromethorphan in amyotrophic lateral sclerosis.

In a one-year parallel group double-blind placebo-controlled study of dextromethorphan (1.5 mg/kg) in amyotrophic lateral sclerosis, no significant differences were observed in the rate of progression (Norris scale) in comparing 24 patients randomly assigned to the dextromethorphan group and 25 patients randomly assigned to the placebo group. Of the 24 patients in the dextromethorphan group, 17 had limb onset and 7 had bulbar onset disease; average duration of disease was 12.5 +/- 6 months and sex ratio (M:F) was 1.4:1. Of the 25 patients in the placebo group, 18 had limb onset and 7 had bulbar onset disease; average duration of disease was 9.9 +/- 6 months and sex ratio (M:F) was 1.55:1. Dextromethorphan is a weak noncompetitive N-methyl-D-aspartate (NMDA) antagonist and higher doses or other potent NMDA receptor antagonists should be tested.

[Late onset myasthenia: 34 cases in patients over 65 years of age]. / La myasthénie de révélation tardive: 34 cas chez des patients de plus de 65 ans.

This study, a retrospective view of 34 patients with myasthenia gravis, compared the course of the disease for patients with onset before 65 and after 65 years. 70% of those under 65 were female while 55.8% of those over 65 were male. Bulbar symptoms were more frequent in older patients. Only 3 patients had another immune disease (rheumatoid arthritis, diabetes mellitus, thyroid pathology), and two a thymoma. All patients were treated with anticholinesterases. Prednisone was used in 44% of cases and rarely Azathioprine. In our cases and in the review of the literature there is no significant difference between age groups except for the sex ratio and the outcome in the older group in case of thymoma or respiratory failure.

[Hiramaya disease: disease or syndrome?]. / La maladie d'Hirayama, maladie ou syndrome?

Hirayama's disease, a benign juvenile amyotrophy of "oblique" topography of an upper limb, has been acknowledged in the international literature. 4 cases are reported in this paper. However there present some differences: presence of antigangliosides antibodies in 1 case, cryoglobulinemia in another case developing bilaterally, conduction blocks in a third case. Finally, in the patient illustrating the typical form, the neuroradiological abnormalities considered as characteristic of cervical spine compression by Hirayama are lacking.

[An anatomo-clinical case of dementia in endovascular large-cell lymphoma]. / Un cas anatomo-clinique de démence au couors d'un lymphome endovasculaire à grandes cellules.

Cerebral angiotropic large cell lymphoma is a rare fatal neurologic disorder characterized by multifocal intravascular proliferation of large pleomorphic cells within vessels of all caliber, predominantly skin and nervous system. Clinical manifestations in previously reported cases were dominated by focal neurologic signs, epilepsia and progressive dementia. We report a case of a 70 year-old man with subacute dementia, epileptic seizures and cerebrovascular events. There was no evidence of a systemic disease outside the nervous system. Cerebrospinal fluid contained 13 leukocytes/mm3 (49% of lymphocytic cells) and more than 100 mg/dl of protein. Cytology was negative. Cranial MRI demonstrated cerebral atrophy and an increased paraventricular signal in T 2 weighted images. A frontal brain biopsy revealed only neuronal dystrophy and astrocytic gliosis. Despite treatment with corticosteroids the patient died 18 months after the onset of the first symptoms. Autopsy was performed and revealed B cell lymphoma.

[Pharmaco-epidemiological study on the prophylactic treatment of migraine. National inquiry on attitude to prescription practices by primary care physicians and neurologists in Franceø]. / Etude pharmaco-épidémiologique sur le traitement de fond de la migraine.

A pharmacoepidemiological survey was conducted in order to understand the pattern of migraine prophylactic drug utilization by French physicians. Neurologists and primary care physicians completed a phone-mail-phone questionnaire which inquired about migraine prophylactic treatment. French neurologists and PCP made the same use of migraine prophylaxis in terms of indication, time interval between treatment onset and evaluation, and duration. The two most commonly chosen migraine prophylactic agents were dihydroergotamine and beta-blockers. This study also showed the importance of considering quality of life to evaluate efficacy of migraine prophylaxis.

Familial desmin myopathies and cytoplasmic body myopathies.

Clinicopathological, immunohistochemical and biochemical studies were performed on seven patients from five families showing an abnormal accumulation of desmin in the muscle fibers. Late onset myopathy was observed in all the cases studied. The clinical features were heterogeneous and usually nonspecific. However, some patients presented with dysphonia, dysphagia or cardiomyopathy. These features are highly suggestive of desmin myopathy. Using electron microscopy, desmin myopathy is characterized by an accumulation of granulofilamentous material. Depending on the distribution of the material, however, three different patterns of desmin accumulation can be observed: (1) large circumscribed inclusions, (2) intermyofibrillar areas of diffusely distributed material, and (3) deposits around large spheroid bodies. The second pattern is characterized by a rubbed-out appearance using oxidative enzyme reactions. For all the patients studied here, the immunohistochemical data showed that the desmin accumulation fitted these three patterns of distribution. For six patients, immunoblot analysis confirmed the desmin accumulation patterns and showed that an increase in the expression of the 53-kDa protein had occurred. The third pattern of desmin accumulation confirms the pathological heterogeneity of cytoplasmic and spheroid bodies. Desmin does not accumulate in all cytoplasmic and spheroid body myopathies, as observed in two other familial cases presented here.