[Opsoclonus-myoclonus-ataxia syndrome: two anatomo-clinical case reports].

Introduction: Opsoclonus-myoclonus-ataxia syndrome (OMAS) is characterised by the acute or subacute development of chaotic eye movements and diffuse myoclonus. On some occasions it is associated with ataxia and encephalopathy. In adults there are multiple causations and a possible paraneoplastic origin must always be taken into account.

[Electrooculography findings in Friedreich's ataxia].

Introduction: Friedreich's ataxia (FA) is the most frequent of the recessive hereditary ataxias. AIMS. Our aim was to analyse the findings from electrooculography studies in subjects with FA attended in our Service over a 30-year period.

[Facial nerve tumours].

Introduction: Tumours originating in the facial nerve are extremely rare and their therapeutic approach requires the aid of specialists from a number of disciplines.

Aims: Our aim was to analyse the cases treated in our centre over a five-year period.

Patients And Methods: All the subjects submitted to surgical interventions to treat facial nerve tumours between January 1992 and December 1996 were evaluated retrospectively.

[Electrooculographic findings in patients with neurofibromatosis].

Neurofibromatosis are frequent genetic syndromes, with the electrooculographic examination (EOG) as an useful tool in their evaluation. We present the EOG findings of 16 patients with this entity which were seen at our Department between 1969 and 1991. Mean of age was 31 years.

Spanish family with Machado-Joseph disease: neurophysiological features and neuropathy study.

Objectives: We have carried out electrophysiological studies and sural nerve biopsy evaluation in a Spanish family with genetically proven Machado-Joseph disease (SCA3/MJD) phenotype III.

Patients And Methods: Two symptomatic and other two asymptomatic members of the family were clinically examined. Electrophysiological evaluation included multimodal evoked potentials, quantitative electromyography and nerve conduction studies, and central motor conduction time.