Autosomal dominant HMSN with proximal involvement: new Brazilian cases.

We report four Brazilian siblings with Autosomal Dominant Hereditary Motor Sensory Neuropathy with Proximal Dominant Involvement (HMSN-P), a rare form of HMSN, that was characterized by proximal dominant muscle weakness and atrophy onset after the age of 30 years, fasciculation, arreflexia and sensory disturbances with autosomal dominant inheritance. Electrophysiological study and sural nerve biopsy were in the accordance with axonal sensory motor polyneuropathy and laboratorial analysis disclosed serum lipids and muscle enzymes abnormalities. Our report is the first done by a group outside Japan, where the disease initially seemed to be restricted and stressed the phenotypic variability from the original report.

[Stroke in a neurology ward: etiologies, complications and length of stay].

Introduction: Purposes of this study were: evaluate complications and length of stay of patients admitted with diagnosis of ischemic stroke (IS) in the acute or subacute phase, in a general Neurology ward in São paulo, Brazil; investigate the influence of age, risk factors for vascular disease, arterial territory and etiology.

Methods: Data from 191 IS patients were collected prospectively.

Results: Fifty-one patients (26.

Stroke management in a university hospital in the largest South American city.

Objective: To describe characteristics and provision of care for patients admitted with cerebrovascular disorders (CVD), focusing on ischemic stroke (IS), in a large, public, academic hospital in São Paulo, Brazil.

Method: We retrieved information about 357 patients with CVD admitted to the Neurology Emergency Department (NED) and Neurology Ward (NW) of our institution. We described patient characteristics and management of IS in NED and in NW.