An open study of botulinum-A toxin treatment of trigeminal neuralgia.

Thirteen subjects with trigeminal neuralgia were treated with botulinum-A neurotoxin (BoNT/A) in an open-label pilot study. After BoNT/A, visual analog scale score, surface area of pain, and therapeutic coefficient were reduced in all patients and for all branch trigeminal nerves studied. Therefore, BoNT/A is an efficient treatment. There were no major side effects. A placebo-controlled clinical trial is needed to confirm these findings.

[Internal carotid bilateral occlusion, meningovascular syphilis and AIDS: case report]. / Oclusão bilateral das artérias carótidas internas, sífilis meningovascular e SIDA. Relato de caso.

We report a case of bilateral occlusion of internal carotid arteries, presenting with right hemiparesis and hypoesthesia, associated to meningovascular syphilis in a patient with AIDS. CT scan showed few small hypodense lesions, with a predominance on the left side, and the angiography showed bilateral occlusion of the carotid arteries. The association between syphilis and AIDS is not unusual, but the paucity of symptoms, probably due to a slow and gradual occlusion is not commonly reported.

[Professor Antonio Austregésilo. The pioneer of neurology and of the study of movement disorders in Brazil]. / Professor Antonio Austregésilo. O pioneiro da neurologia e do estudo dos distúrbios do movimento no Brasil.

Professor Antonio Austregésilo was the pioneer of neurology in Brazil, creating the first neurologícal school, in Rio de Janeiro, of which he was the first professor. He was also the first to study the movement disorders in Brazil, publishing several works on this subject, primarily in "Revue Neurologique", and "L'Encephale", including a rival sign of Babinski and the first description of a posttraumatic dystonia.

Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2, MJD/SCA3 and DRPLA) in a large group of Brazilian patients.

Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by expansions of an unstable CAG repeat localized in the coding region of the causative genes. Another related disease, dentatorubropallidoluysian atrophy (DRPLA) is also caused by an unstable triplet repeat and can present as SCA in late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3 and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90 unrelated families with various patterns of inheritance and originating in different geographic regions of Brazil. We found mutations in 35 families (39%), 32 of them with a clear autosomal dominant inheritance. The frequency of the SCA1 mutation was 3% of all patients; and 6% in the dominantly inherited SCAs. We identified the SCA2 mutation in 6% of all families and in 9% of the families with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30% of all patients; and in the 44% of the dominantly inherited cases. We found no DRPLA mutation. In addition, we observed variability in the frequency of the different mutations according to geographic origin of the patients, which is probably related to the distinct colonization of different parts of Brazil. These results suggest that SCA may be occasionally caused by the SCA1 and SCA2 mutations in the Brazilian population, and that the MJD/SCA3 mutation is the most common cause of dominantly inherited SCA in Brazil.

Arterial graft reconstruction in an aneurysm of the middle cerebral artery.

A case of a large aneurysm of the middle cerebral artery that was treated by resection of the aneurysm sac and reconstruction of the vessel is presented. The middle cerebral artery was reconstructed using a graft from the superficial temporal artery (STA). Cerebral vessel reconstructions are usually carried out with veins as grafts. This paper shows the possibility of using the STA as a graft. When a vessel reconstruction is contemplated, the skin flap should be planned in order to save the STA.

[Isaacs syndrome: report of a case]. / Síndrome de Isaacs: relato de um caso.

The authors report a case of Isaacs syndrome observed in a 40 years old woman. The clinical picture included progressive muscular weakness, dysphagia, dysphonia, dyspnea and increased perspiration. Fasciculations, facial myokymia and pseudomyotonia were observed on physical examination. Electromyographic study at rest revealed continuous electrical muscle activity. Muscle histochemistry showed type II fibers atrophy and an ultrastructural study of the gastrocnemius muscle disclosed marked cysternal dilatation of the sarcoplasmatic reticulum. An excellent clinical response was observed with the use of carbamazepine. Some recent aspects of this rare syndrome are reviewed and discussed.