The spinocerebellar ataxias (SCAs) are a heterogeneous group of neurodegenerative disorders varying in both clinical manifestations and mode of inheritance. Six different genes causing autosomal dominant SCA are mapped: SCA1, SCA2, Machado-Joseph disease (MJD)/SCA3, SCA4, SCA5, and dentatorubropallidoluysian atrophy (DRPLA). Expansions of an unstable trinucleotide CAG repeat cause three of these disorders: SCA type 1 (SCA1), MJD, and DRPLA. We determine the frequency of the SCA1, DRPLA, and MJD mutations in a large group of unrelated SCA patients with various patterns of inheritance and different ethnic backgrounds. We studied 92 unrelated SCA patients. The frequency of the SCA1 mutation was 3% in the overall patient group and 10% in the non-Portuguese dominantly inherited SCA subgroup. We found that DRPLA mutation in only one Japanese patient, who was previously diagnosed with this disease. We identified the MJD mutation in 41% of the overall patient group, which included 38 autosomal dominant kindreds of Portuguese origin; the frequency of the MJD mutation among the non-Portuguese dominantly inherited cases was 17%. These results suggest that SCA may be occasionally caused by the SCA1 mutation and rarely caused by the DRPLA mutation and that, to date, the MJD mutation seems to be the most common cause of dominantly inherited SCA. Finally, our results suggest that recessively inherited cases of SCA are not caused by the known trinucleotide repeat expansions.

Primary headache such as tension type headache and migraine may occur during pregnancy 1,2,3 either as a recurrent symptom of a previous condition or as a new onset manifestation.Secondary headache that mimics migraine may occur in the gestational period. 2,3 This type of headache may be caused by vasculitis, cerebral tumor, choriocarcinoma, pituitary tumor, arteriovenous malformation, sinus disorders, idiopathic intracranial hypertension, subarachnoid hemorrhage, cerebral arterial and venous episodes, pre-eclampsia and eclampsia. 3,4 Migraine improves during gestation in 55 to 90% of patients.5-8 Most of these women present with migraine without aura. When migraine gets worse during pregnancy it usually occurs during the first trimester. 5,6 The incidence of new onset ABSTRACT: Objective: The purpose of this study was to evaluate the presence of headache in women with a previous history or newonset headache during the current gestation, classify the findings, and describe the clinical characteristics and outcome of the headache. Methods: From January/1998 to June/2002 we prospectively evaluated 1101 pregnant women (12-45 years old), with a history of headache, at two prenatal clinics and an inpatient obstetric public hospital. Women were interviewed using a semi-structured questionnaire during the first, second, and third gestation trimesters and immediately after delivery. All interviews were conducted by one of the authors, using the International Headache Society Classification (IHSC-2004

No abstract

Idiopathic stabbing headache (ISH) is defined as the occurrence of short-lasting, painful jabs, restricted to the ophthalmic division of the trigeminal nerve. It is closely related to other forms of headache (such as migraine and tension-type headache) and has been reported among all age groups, including children and adolescents. As pathogenic mechanisms of the disease remain unclear, management decisions are empirical and limited to few options. Classically, indomethacin has been considered the first option, but therapeutic failure occurs in up to 35% of cases. In this setting, we report four patients with young-onset indomethacin-resistant ISH which had good responses to gabapentin and discuss the use of this drug in the presenting situation.

In this article, a group of experts in headache management of the Brazilian Headache Society developed through a consensus strategic measurements to treat a migraine attack in both the child and the adult. Particular emphasis was laid on the treatment of migraine in women, including at pregnancy, lactation and perimenstrual period.