[Preserving hearing function after Gamma Knife radiosurgery for unilateral vestibular schwannoma].

Objective: The majority of the patients still lose hearing function in spite of the technical advances in microsurgery. We wanted to evaluate preservation of hearing function potential after Gamma Knife Surgery.

Material And Method: In Marseille, we performed 1000 Gamma-Knife procedures for vestibular schwannomas between July 1992 and January 2002.

[Functional outcomes of radiosurgical treatment of vestibular schwannomas: 1000 successive cases and review of the literature].

Rationale: To evaluate the functional results of Gamma Knife surgery of vestibular schwannomas relying on a large and prospective series of consecutive cases.

Material And Method: The first 1000 patients with cerebello-pontine angle schwannomas were consecutively treated by Gamma Knife in Marseille Timone University Hospital between July 1992 and March 2001. Patients without NF2 and or clinico-radiological arguments in favor of a facial origin accounted for a population of 927 patients (414 males, for 513 females) including 843 treated in first intention.

[Molecular diagnosis of hereditary neuropathies such as Charcot-Marie-Tooth disease].

During the last decade, molecular biology has demonstrated the extraordinary heterogeneity of genetic abnormalities in Charcot-Marie-Tooth disease (CMT). The main phenotypes are either of the demyelinating or axonal type, transmitted with dominant or recessive autosomal inheritance. X-linked CMT is less rare than it was initially described and is often misdiagnosed as autosomal dominant type.

[Multifocal motor neuropathies with persistent conduction blocks: a form of inflammatory demyelinating neuropathy].

Since the first cases described in 1986, multifocal motor neuropathy with persistent conduction blocks (MMN) appears to be a well-defined nosological entity. Clinical features include male-predominant occurrence, weakness often accompanied by cramps and fasciculations; topography of the motor involvement is characterized by assymetry, upper limb predominance, relation to the distribution of individual nerves or roots. Atrophy is variable but can lack despite severe weakness.

[Non hypertrophic amyloid myopathy with muscular inflammation in plasma cell dyscrasia].

The case of a 41 year old woman with amyloid myopathy is reported. Clinical involvement consisted of limb girdle muscle weakness, mild scapular muscle atrophy and dysphagia. In contrast with the published cases, abnormal firmness, pseudohypertrophy of the musculature and macroglossia were absent.

Increased prevalence of neurologic complications among insulin dependent diabetic patients of Algerian origin.

This study was undertaken to ascertain a clinical impression that the prevalence of complications is higher in insulin-dependent Algerian diabetics than in their European counterparts. Forty-one Algerian patients under regular follow-up for 2 years or more were closely matched to 41 French insulin dependent diabetics with regard to sex (34M/7F) and duration of disease (10.5 +/- 1 year; range 2 -32 years).