[Nonsystemic vasculitic neuropathy]. / Neuropatía vasculítica no sistémica.

BACKGROUND: Nonsystemic vasculitic neuropathy (NSVN) is an infrequent type of vasculitic neuropathy that evolves without manifestations of vasculitis in other organs and in the absence of serological abnormalities. There are non clarified conjectures about the pathogenesis, outcome and treatment approach. PATIENTS AND METHOD: A retrospective study of a series of six patients diagnosed of NSVN during a period of 12 years. Clinical, electrophysiological and pathological features, as well as the response to therapy and outcome are analysed. RESULTS: Four cases presented with a pattern of multiple mononeuropathy, evolving towards a symmetrical sensory and motor polyneuropathy in two of them. One patient presented with an acute sensory neuropathy and another had a subacute asymmetric sensory and motor neuropathy. No signs of accompanying systemic vasculitis were observed during the follow-up (mean 35 months) and the only outstanding serological abnormality was the presence of antibodies against hepatitis B virus in four of them. The nerve conduction studies showed typical features of axonal degeneration. The diagnostic was obtained due to the presence of a necrotizing vasculitis in the sural nerve biopsy in all cases. The mean time from symptom onset to diagnosis was 11 months. All patients were treated with immunosuppressive therapy presenting a favourable response, except the case of the sensory neuropathy that remained stable. CONCLUSIONS: NSVN is a benign type of vasculitic neuropathy with a variable clinical pattern of presentation and favourable response to immunosuppression. This neuropathy requires a high index of suspicion for diagnosis, so nerve biopsy must be carried out in all neuropathy of unknown etiology. Careful follow-up of patients is necessary, so that life-threatening systemic vasculitis neuropathy can be diagnosed early.

[Creutzfeldt-Jakob disease with unilateral onset: clinical profile and neuroimaging]. / Enfermedad de Creutzfeldt-Jakob de inicio unilateral: clínica y neuroimagen.

The usual clinical profile of Creutzfeldt-Jakob disease (CJD) is that of subacute dementia and intractable myoclonus. Occasionally, some cases present peculiar clinical features. We report on a case of CJD with an unilateral onset showing remarkable neuroimaging features. The patient, aged 72 years, began to suffer from sudden anomia, initially restricted to persons; but in a few weeks it evolved into a global aphasia, right hemiparesis, severe gait disorder, and finally akinetic mutism and intractable myoclonus. He died 11 weeks after onset. Early in the course, an analysis of 14-3-3 protein in CSF was positive. In advanced disease, the EEG showed the typical periodic activity of CJD. FLAIR MRI study showed a mesencephalic and focal cortical hyperintensity. Autopsy was performed and confirmed the diagnosis of CJD with an extensive presence of generalised spongiosis in cerebral grey matter. This case illustrates the usefulness of the life recent paraclinical methods to diagnose CJD in life. New MRI techniques seems to be particularly relevant, as they are not limited to exclude other conditions but can also offer data with validity to a positive diagnosis, like grey matter hyperintensity, that in this case was present also in the midbrain.

[Descriptive study of a serie of patients affected by progressive supranuclear palsy]. / Análisis descriptivo de una serie de pacientes afectados de parálisis supranuclear progresiva.

INTRODUCTION: Progressive supranuclear palsy is a neurodegenerative disorder affecting diverse neurologic systems. The actual treatment response is poor in most patients. OBJECTIVE: review of a long series of patients affected by PSP in several aspects. PATIENTS AND METHODS: A series of patients was reviewed by means of the register questionnaire of PSP in Spain (from PSP Disabling Rating Scale and Staging System). This is carried out on the patients when the diagnostic is done. It was achieved a descriptive of the patients, in several aspects, and an evaluation of the treatment in relation to the dose and the duration. RESULTS: In general, the age of diagnostic is 66 years, there is not neurological illness in the family, falls and disorders of gait are the most representative parameters. The neuroimage shows fronto temporal atrophy. The treatment response is poor, despite the dose and the duration. CONCLUSIONS: Our series confirms the typical dates of the illness an the poor response to treatment with L Dopa.