[Polyneuropathy in patients with periodic leg movements during sleep]. / Estudio de polineuropatía en pacientes con movimientos periódicos de las piernas durante el sueño.

INTRODUCTION: Periodic legs movements of sleep (PLMS) are rhythmic, standard and repetitive contractions of muscles of the extremities during the sleep. It is known that the patients with restless legs syndrome (RLS) have disorders during the sleep: increase in the latency of the sleep, increased number of arousal, etc.; most of them have also periodic movements of the legs during the sleep. OBJECTIVE: The relationship of the periodic movements of the legs during the sleep with polyneuropathy is not clear. Some authors have found evidence of electrophysiological and pathological of signs of axonal mild polyneuropathy in patients with restless legs syndrome. In this work, we evaluated nine patients that were diagnosed of PLMS, to determine the prevalence of neuropathy in such sample. METHOD: Polysomnography of nocturnal sleep of 7-8 hours was performed, including electromyographic recording of both anterior tibialis muscles; and electroneurographic study of peroneal, sural, ulnar and median nerves. DISCUSSION: Just in none of the nine studied cases were obtained electrophysiological signs of neuropathy; though it has been able to demonstrate the existence of mild alteration of the peripheral nervous system, fundamentally of sensory character; nevertheless, C we think that it would have to be studied the existence of polyneuropathy in all the patients with PLMS in order to discard potentially tractable organic causes.

[Neurophysiological study in Alpers syndrome]. / Estudio neurofisiológico en el síndrome de Alpers.

INTRODUCTION: Infantil progressive polydystrophy was described by Alpers in a child with psychomotor retardation, crises which were resistant to treatment and diffuse loss of cortical neurons. OBJECTIVE: The aim of this study was to review the neurophysiological aspects of Alpers syndrome and their clinical correlation. MATERIAL AND METHODS: We present three children with subacute encephalopathy, progressive psychomotor retardation, myoclonic epilepsy which was resistant to treatment and crises of apnea, who had degeneration of the cerebral grey matter. Serial EEG, polysomnographs, auditory evoked potentials of the brain stem and visual evoked potentials were done. RESULTS: The electroencephalogram findings showed the presence of complex bursts of acute waves, small many-pointed or slow waves of great amplitude which were irregular and arrhythmical, lasting one to five seconds, separated by periods of inactivity on the tracing which lasted from three to ten seconds. The EEG was distinctive, changing over the course of the illness, and with increasing numbers and duration of the bursts of suppression of cerebral bioelectric activity. Polysomnography showed cerebral bioelectric activity which was markedly unstructured and with little difference between the tracings when asleep and when awake, together with a large number of apneas of obstructive and mixed types. The PEAT showed reduced amplitude and altered morphology in all the waves, and even absence of some of them. The visual evoked potentials were asymmetrical and with delay in the latency of the P100 wave. CONCLUSIONS: Although definite diagnosis of progressive neurone degeneration requires post mortem examination of the brain, clinico-pathological studies, including electrophysiological, radiological and biochemical studies are sufficiently characteristic to suggest the diagnosis during life.

[Neurophysiological aspects of Proteus syndrome]. / Aspectos neurofisiológicos en el síndrome de Proteus.

INTRODUCTION: The Proteus Syndrome was defined in 1983 by Wiedeman. However, the first case mentioned in the literature was that of Joseph Merrick, the Elephant Man, presented by Sir Frederick Treves in 1884. It is a rare pathological condition. Its multiple clinical features include; partial gigantism of hands and/or feet, pigmented nevi, hemihypertrophy of the body, tumors, skeletal anomalies, growth disorders and visceral anomalies. Hereditary transmission has not been clearly defined. Diagnosis and treatment require the participation of experts from several medical and surgical specialties. CLINICAL CASE: We present a case sent to our hospital for the surgical correction of cranio-facial malformations. Epileptic crises post-operatively indicated the need for neurological and neuro-physiological study. This was done by means of conventional electro-encephalography: brainstem, somato-sensorial and visual auditory evoked potentials, together with imaging techniques which showed the structural and functional asymmetry of the central nervous system at both cerebral and brainstem levels. CONCLUSIONS: Few neuro-physiological studies are included in the literature we reviewed for this paper. Therefore we do not know whether the functional anomalies of the central nervous system which we describe should be considered to be part of the syndrome.