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.

-Autosomal recessive hereditary spastic paraplegia (AR-HSP) associated with thin corpus callosum was recently described in Japan, and most families were linked to chromosome 15q13-15. We report two patients from two different Brazilian families with progressive gait disturbance starting at the second decade of life, spastic paraparesis, and mental deterioration. One patient presented cerebellar ataxia. Magnetic resonance imaging (MRI) of the head of both patients showed a thin corpus callosum. AR-HSP with a thin corpus callosum is a rare disorder, mainly described in Japanese patients. We found only 4 Caucasian families with AR-HSP with thin corpus callosum described in the literature. .urther studies including additional Caucasian families of AR-HSP with thin corpus callosum are required to delineate the genetic profile of this syndrome in occidental countries.KEY WORDS: hereditary spastic paraplegia, corpus callosum, MRI.Paraplegia espástica hereditária associada a hipoplasia de corpo caloso RESUMO -A paraplegia espástica hereditária autossômica recessiva (PEH-AR) associada com hipoplasia de corpo caloso foi inicialmente descrita no Japão. Estudos de ligação genética mostram que a maioria das famílias estão relacionadas ao cromossomo 15q13-15. Relatamos dois pacientes de famílias brasileiras, não relacionadas, com distúrbio de marcha com início na segunda década de vida, paraparesia espástica e comprometimento das funções cognitivas. Um dos pacientes apresentava ataxia cerebelar. A ressonância magnética de encéfalo de ambos os pacientes mostrou hipoplasia de corpo caloso. PEH-AR associada com hipoplasia de corpo caloso é uma condição rara, descrita principalmente em pacientes do Japão. Encontramos apenas 4 famílias caucasianas com PEH-AR e hipoplasia de corpo caloso. Mais estudos com famílias caucasianas são necessários para delinear o perfil genético dessa síndrome em países ocidentais. PALAVRAS-CHAVE: paraplegia espástica hereditária, corpo caloso, ressonância magnética.

We evaluated 44 patients with tics and Tourettes syndrome (TS) emphasising the age of onset of symptoms, sex, classification and localization of tics, associated symptoms and signs and comorbidities. Thirtythree patients (75.2%) had TS defined criteria whereas 10 (22.7%) had chronic motor and/or vocal tics. Simple motor tics were found in 43 cases (97.7%), mainly affecting the eyes (43.2%), mouth (43.2%), face (34.1%). Simple vocal tics occurred in 33 (75%). Coprolalia was found in just 6 cases (13.6%) and copropraxia in just 2 (4.5%). Obsessive compulsive disorder and/or symptoms were found in 26 cases (59.1%) and attention deficit in 17 (38.6%). Eighteen patients (40.9%) had other disorders, such as alcoholism, tabagism, drug abuse, affective disorders, anxiety, sleep and learning disorders. The data obtained are similar to those found by other authors. We highlight the low frequency of coprolalia, as well as the associated neuropsychiatric disorders.KEY WORDS: Tourettes Syndrome, obsessive compulsive disorder, attention deficit disorder.Tiques e síndrome de Tourette: avaliação clínica de 44 casos RESUMO Avaliamos 44 pacientes com tiques e síndrome de Tourette (ST), enfatizando a idade de início dos sintomas, sexo, classificação e localização dos tiques, sinais e sintomas associados e presença de comorbidades. Trinta e três pacientes (75,2%) tinham TS definida, ao passo que 10 (22,7%) tinham tiques motores e/ou vocais crônicos. Tiques motores simples foram encontrados em 43 casos (97,7%), principalmente envolvendo os olhos (43,2%), boca (43,2%), face (34,1%). Tiques vocais simples ocorreram em 33 (75%). Coprolalia estava presente em apenas 6 casos (13,6%) e copropraxia em apenas 2 (4,5%). Sintomas e/ou transtorno obsessivo-compulsivo foi encontrado em 26 casos (59,1%) e déficit de atenção em 17 (38,6%). Dezoito pacientes (40,9%) tinham outras desordens, tais como alcoolismo, tabagismo, abuso de drogas. Os dados encontrados são semelhantes àqueles encontrados por outros autores. Nós enfatizamos a baixa incidência de coprolalia, assim como a presença de transtornos neuropsiquiátricos associados.

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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-Cerebellar hemorrhage is listed among the potential complications following neurosurgical procedures. In this scenario it is usually reported as a rare condition. However, it seems that epilepsy surgery patients are somewhat more prone to this kind of complication, compared to other surgical groups. Head positioning, excessive cerebral spinal fluid draining and the excision of non-expanding encephalic tissue (or combinations among the three) are likely to be cause underlying remote cerebellar hemorrhage. Out of the 118 ATL/AH performed at our institution, between 1996 and 2002, we identified 3 (2.5%) patients presenting with cerebellar hemorrhage. We report on such cases and review the literature on the topic.KEY WORDS: cerebellar hemorrhage, epilepsy surgery, neurosurgical complications.Hemorragia cerebelar como complicação de lobectomia temporal para epilepsia do lobo temporal medial: relato de três casos RESUMO -A hemorragia cerebelar faz parte das potenciais complicações dos procedimentos neurocirúrgicos. De forma geral, é considerada uma condição rara. Entretanto, há aparente propensão dos pacientes submetidos ao tratamento cirúrgico de epilepsia em apresentar este tipo de complicação, quando comparados com outros grupos cirúrgicos. O posicionamento da cabeça, excessiva drenagem de líquido cefalorraquidiano e a excisão de tecido cerebral não expansível (ou talvez combinações entre os três) constituem as potenciais causas da hemorragia cerebelar remota. Entre os 118 pacientes em nossa série de LTA ⁄ AH, identificamos 3(2.5%) casos de hemorragia cerebelar. Relatamos os três casos desta natureza, com revisão da literatura pertinente a esta complicação.PALAVRAS-CHAVE: hemorragia cerebelar, cirurgia de epilepsia, complicações neurocirúrgicas.Anterior temporal lobectomy (ATL) and amygdalohippocampectomy (AH) are effective treatment alternatives in patients with temporal lobe epilepsy refractory to medical treatment. Neuropsychological disturbances (language and memory) are the most common post-operatory derangements. Nevertheless, surgical complications are rarely expected, both locally and at remote sites. Recent papers have shed some light on the relative high frequency of cerebellar hemorrhage in patients submitted to anterior temporal lobectomy, when compared to other surgical groups. It seems that ATL/AH patients are particularly prone to this kind of complication. At our institution ATL ⁄ AH are always performed using the same surgical technique (that is, a trans-temporal approach). Out of the 118 ATL ⁄ AH performed at our institution we were able to identify 3(2.5%) patients presenting with cerebellar hemorrhage. Their cases are reported. CASESPatient 1. A 31 year-old male patient, presenting with a seizure disorder starting at the age of 9 y/o, characterized by an aura (epigastric sensation), shortly followed by a complex partial seizure and on occasion a generalized tonic-clonic seizure. He was tried on phenobarbital and carbamazepine, with unsatisfactory seizure control. A combination of valpr...

The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.