[Non-hereditary +hyperekplexia. Study of a patient with unusual ictal EEG features and review of the literature]. / Hiperecplexia no hereditaria. Estudio de un paciente con características EEG ictales poco habituales y revisión de la bibliografía.

INTRODUCTION AND CLINICAL CASE: We present a male patient which presented distal muscular stiffness, from the first hours of the life. At this time, he also presented episodes of generalized muscular hypertonia with cyanosis and apnoea, started by somato-sensorial stimuli. Those episodes were terminated by passive flexion of the extremities and the head. Later, dream myoclonic jolts appeared. EEG tracings during hypertonic episodes showed an initial artefact potential followed by bursts of rhythmic and repetitive acute potentials in the bilateral fronto-central regions, at a 20-22 Hz frequency, similar to an epileptiform spike-wave burst. The EMG showed a continuous muscular activity, suppressed by rest and the administration of diazepam. Treatment with oral diazepam has been very effective. Now, the patient is four years old, is asymptomatic and continues treatment with oral diazepam. If the dose is decreased, the child starts unstable march, startle response on somatosensory stimuli with falls, and above all nocturnal myoclonia. The patient does not have familiar antecedents of hyperekplexia, even in its minor form. CONCLUSION: Clinical picture suggests us a not familiar form of hyperekplexia.

[Statistical (Poisson) motor unit number estimation. Methodological aspects and normal results in the extensor digitorum brevis muscle of healthy subjects]. / Estimación del numero de unidades motoras en un musculo mediante la técnica estadística (Poisson). Aspectos metodológicos y parámetros normales en el extensor digitorum brevis.

INTRODUCTION: Among the differents techniques for motor unit number estimation (MUNE) there is the statistical one (Poisson), in which the activation of motor units is carried out by electrical stimulation and the estimation performed by means of a statistical analysis based on the Poisson s distribution. OBJECTIVES: The study was undertaken in order to realize an approximation to the MUNE Poisson technique showing a coprehensible view of its methodology and also to obtain normal results in the extensor digitorum brevis muscle (EDB) from a healthy population. SUBJECTS AND METHODS: One hundred fourteen normal volunteers with age ranging from 10 to 88 years were studied using the MUNE software contained in a Viking IV system. RESULTS: The normal subjects were divided into two age groups (10 59 and 60 88 years). The EDB MUNE from all them was 184 49. Both, the MUNE and the amplitude of the compound muscle action potential (CMAP) were significantly lower in the older age group (p< 0.0001), showing the MUNE a better correlation with age than CMAP amplitude ( 0.5002 and 0.4142, respectively p< 0.0001). CONCLUSION: Statistical MUNE method is an important way for the assessment to the phisiology of the motor unit. The value of MUNE correlates better with the neuromuscular aging process than CMAP amplitude does.

[Guillain Barré syndrome in an intensive care unit]. / Síndrome de Guillain Barré en Unidad de Cuidados Intensivos.

INTRODUCTION AND OBJECTIVE: The Guillain Barré syndrome is an inflammatory process of the peripheral nervous system. It is potentially reversible. Nowadays it is the commonest cause of acute, generalized, flaccid paralysis in the western world. Our objective was to present the clinical and electrophysiological characteristics of patients with the Guillain Barré syndrome who required admission to an intensive care unit. We therefore report our experience over a period of four years with patients with this syndrome who were admitted to the intensive care unit and given mechanical ventilation. We analyze the current incidence of this syndrome, the proportion of patients requiring admission to the intensive care unit and connection to mechanical ventilators due to their muscle weakness, and the mortality rates of the major case series published. We analyze the electrophysiological findings which indicate worse prognosis. Greater axonal involvement is seen in patients with poorer recovery, whilst those who made a better recovery had a predominantly demyelinating pattern. We studied certain clinical features which implied more severe illness and worse functional recovery such as the appearance of signs of autonomic nervous system involvement, rate of progression until the maximum affectation occurred and advanced age. Finally, we discuss the role of current immunomodulation treatment and the evidence of its effectiveness. The Guillain Barré syndrome patients admitted to the intensive care unit with the greatest index of long term sequelae are characterized by being older, with a higher proportion of neurovegatative disorders and have an electrophysiological pattern showing signs of greater axonal degeneration.

Miastenia gravis generalizada seronegativa. Presentación de un caso de inicio precoz / Early onset seronegative generalised myasthenia gravis. Case report

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Síndrome de Miller-Fisher asociado a neuropatía axonal motora aguda: correlación clínico-inmunológica / Miller-Fisher syndrome associated with acute motor axonal neuropathy: clinic-immunological correlation

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