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OBJECTIVE:To study tremor in patients with X-linked recessive spinobulbar muscular atrophy or Kennedy's disease.METHODS:Ten patients (from 7 families) with a genetic diagnosis of Kennedy's disease were screened for the presence of tremor using a standardized clinical protocol and followed up at a neurology outpatient clinic. All index patients were genotyped and showed an expanded allele in the androgen receptor gene.RESULTS:Mean patient age was 37.6 years and mean number of CAG repeats 47 (44-53). Tremor was present in 8 (80%) patients and was predominantly postural hand tremor. Alcohol responsiveness was detected in 7 (88%) patients with tremor, who all responded well to treatment with a β-blocker (propranolol).CONCLUSION:Tremor is a common feature in patients with Kennedy's disease and has characteristics similar to those of essential tremor.

BACKGROND: Blepharospasm (BS) is a form of central focal dystonia recently associated with psychiatric disorders, particularly obsessive and compulsive symptoms. Hemifacial spasm (HFS) represents a focal myoclonus with peripheral origin in the facial nerve. OBJECTIVE: To determine the frequency of obsessive and compulsive symptoms in patients with BS in comparison with patients with HFS. METHODS: 30 patients from each group (BS and HFS) followed by the botulinum toxin clinic at the HC-UFPR were evaluated using a structured interview based on the DSM-IV criteria and the Yale-Brown scale. RESULTS: were compared by the mean two-tailed t test. RESULTS: We found obsessive or compulsive symptoms in 20 (66.6%) patients with BE and 21 (70%) with HFS. Yale-Brown scale scores for each group were higher among BS patients; however, diferences were not statisticaly significant. CONCLUSION: Our study did not show a significant diference in the comparison of the prevalence of obsessive and compulsive symptoms among patients with BS and HFS.

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Marcel Proust is one of the most important French writers of the 20th century. His relationship with medicine and with neurology is possibly linked to the fact that his asthma was considered to be a psychosomatic disease classified as neurasthenia. Stendhal's syndrome is a rare psychiatric syndrome characterized by anxiety and affective and thought disturbances when a person is exposed to a work of art. Here, the authors describe neurological aspects of Proust's work, particularly the occurrence of Stendhal's syndrome and syncope when he as well as one of the characters of In Search of Lost Time see Vermeer's View of Delft during a visit to a museum.

Charcot name became very famous around the world, firstly because of the work of Professor Jean-Martin Charcot, the founder of Clinical Neurology, and, secondly, because of his son, Jean-Baptiste, the world famous maritime explorer.Key words: Charcot, neurology, Jean-Baptiste Charcot. RESUmOO nome Charcot tornou-se muito famoso em todo o mundo, primeiramente, por causa do trabalho do professor Jean-Martin Charcot, o fundador da Neurologia Clínica e, posteriormente, devido ao seu filho, Jean-Baptiste, o famoso explorador marítimo conhecido mundialmente.Palavras-Chave: Charcot, neurologia, Jean-Baptiste Charcot.

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There is controversy in the neurological literature about where Joseph Babinski was born, including a myth propounded by various important authors that he was born in Lima, Peru. However, according to the most consistent biographical data, he was in fact born in Paris, France, and became a medical celebrity there and in Poland as well as around the world.