[Segmental motor paralysis caused by the varicella zoster virus. Clinical study and functional prognosis]. / Parálisis motora segmentaria por virus varicela-zoster. Estudio clínico y pronóstico funcional.

INTRODUCTION: Segmental motor paralysis of the limbs (SMP) complicates 2-3% of the cases of cutaneous herpes zoster. Viral invasion and inflammation of the motor neurons of the anterior horn cells by the varicella-zoster virus (VVZ) causes clinical weakness at the same time and site as the cutaneous eruption. OBJECTIVES: To analyze the clinical findings, complementary investigations and functional prognosis of patients with SMP at brachial plexus and lumbosacral levels. PATIENTS AND METHODS: We made a retrospective study of 10 patients with SMP admitted to the Hospital Universitario Gregorio Maranon de Madrid during 1989-1999, aged between 38 and 84 years (6 women, 4 men). Neurological examination was done, including muscle balance, complementary studies including microbiology (serum and CSF serology, viral PCR-ADN), neurophysiology using MNR of the spine and plexuses and functional prognosis on the NDS, NSS and RANKIN scales. RESULTS: There is a close relationship between dermatome and myotome involvement (90%). The brachial and lumbosacral plexuses were equally affected (50%). Plasma and CSF VVZ serology was positive in 50% of the cases, permitting diagnosis of a patient with no cutaneous lesions (zoster sine herpete). Denervation of the myotomes involved and the paraspinal muscles was shown on neurophysiological studies. In most cases there was functional improvement, with complete functional recovery in 80% of the cases after 12 months. CONCLUSIONS: VVZ should be considered amongst the aetiologies of SMP, even in the absence of cutaneous lesions (zoster sine herpete). The SMP coincides in time and place with the dermatome lesions. In most patients there is complete functional recovery within 12 months.

[Study of inpatient consultation for the neurological services]. / Estudio de la interconsulta intrahospitalaria a un servicio de neurología.

INTRODUCTION: Intrahospitalary interconsultation (IC) to a neurology department is an infrastudied task within daily neurologic health care. AIMS: To evaluate the IC to a neurology department. METHODS: A one-year retrospective study was performed on the IC received in the Department of Neurology in the Hospital General Universitario "Gregorio Marañón" (Spain), to evaluate the reasons for consultation, the urgency of the request, concordance between initial clinical suspicion and final diagnosis, specialties involved, final diagnosis and management/deviation to the IC. RESULTS: A total of 432 IC were analyzed. The most frequent reasons for consultation were cerebrovascular disease (19%) and control of already diagnosed neurological diseases (17%). The most frequent final diagnoses were cerebrovascular disease and acute metabolic encephalopathy (16% in both cases). With regard to management, three percent of the cases were admitted to the ward and 14% were sent to out patients for completing studies and/or follow up. CONCLUSIONS: This study underlines the importance of intrahospitalary IC within the daily tasks and health care carried out in a neurology department in relation to the number of IC undertaken during the study period. It is also an excellent method to evaluate the neurologic manifestations observed in systemic diseases and neurologically study patients with diverse pluripathy.

Miopatía hipotiroidea / Hypothyroidism myopathy

Presentamos dos caso de miopatía, como cuadro de debut de hipotiroidismo, destacando lo poco frecuente de este acontecimiento. En ambos casos, el desconocimiento de la existencia de dicho trastorno endocrino, llevaron a un enfoque inicial erróneo del cuadro muscular. El diagnóstico final correcto de miopatía tiroidea y su tratamiento con L-tiroxina, determinan una mejoría inmediata. Recordar, pues que un cuadro miopático aislado puede ser debido a hipofunción tiroidea, teniendo en cuenta la "dificultad" del diagnóstico de dicha endocrinopatía cuando faltan sus datos clínicos más típicos (AU)

Estudio de la interconsulta intrahospitalaria a un servicio de neurología / Study on intrahospitalary interconsultation to a neurology department

Introducción: La interconsulta intrahospitalaria a un servicio de neurología es un trabajo infraestudiado dentro de la asistencia neurológica diaria. Objetivos: Evaluar la interconsulta intrahospitalaria a un servicio de neurología. Métodos: Se ha realizado un estudio retrospectivo de un año de extensión sobre las interconsultas intrahospitalarias recibidas en el Servicio de Neurología del Hospital General Universitario Gregorio Marañón de Madrid, valorando los motivos de consulta, urgencia de la petición, adecuación entre la sospecha clínica inicial y el diagnóstico final, especialidades implicadas, diagnóstico final y tratamiento/derivación de las interconsultas intrahospitalarias. Resultados: Se han analizado un total de 432 interconsultas. Los motivos de consulta más frecuentes fueron la enfermedad cerebrovascular (19 por ciento) y el control de enfermedades neurológicas ya diagnosticadas (17 por ciento). Los diagnósticos finales más frecuentes fueron la enfermedad cerebrovascular y la encefalopatía metabólica aguda (el 16 por ciento en ambos casos). En cuanto al tratamiento, se trasladaron a planta el 3 por ciento y se derivaron a consultas externas para completar el estudio y/o seguimiento al 14 por ciento. Conclusiones: Con este trabajo se pretende destacar la importancia de la interconsulta intrahospitalaria dentro del trabajo y la asistencia diaria de un servicio de neurología en relación con el número de interconsultas realizadas en el período estudiado, así como por ser un medio excelente para valorar las manifestaciones neurológicas de las enfermedades sistémicas y estudiar neurológicamente a pacientes con pluripatología diversa (AU)

[Intracranial hypertension as the first clinical manifestation of gliomatosis cerebri]. / Hipertensión intracraneal como primera manifestación clínica de gliomatosis cerebri.

A case of gliomatosis cerebri which clinically presented with a syndrome of intracranial hypertension (ICH), involvement of bilateral sixth cranial nerves, and oppressive holocranial headache of one week of evolution. Cranial MR and CT were performed demonstrating diffuse hypodense cortical-subcortical lesions on tomography and in T1 sequences and hyperdense lesions in T2 sequences with irregular contrast enhancement. Intracranial pressure was measured by ventricular catheter with the appearance of high, maintained pressure waves (Lundberg A waves). Ventricular LCR study and cerebral angiography did not provide additional data. Meningeal and cerebral biopsies showed infiltration by pleomorphous glioma leading to the diagnosis of gliomatosis cerebri. The patient was treated with steroids, hyperosmolar agents, external LCR derivation and tumoral radiotherapy. Nonetheless, the patient dies at six months of initiation of the symptoms. Gliomatosis cerebri should be taken into account in the differential diagnosis of clinical pictures presenting with ICH.

Hipertensión intracraneal como primera manifestación clínica de gliomatosis cerebri / Intracranial hypertension as the first clinical manifestation of gliomatosis cerebri

Se describe un caso de gliomatosis cerebri que se inició clínicamente con un síndrome de hipertensión intracraneal, afectación del VI par craneal bilateral y cefalea opresiva holocraneal de una semana de evolución. Se realizaron una TAC y una RMN de cráneo que demostraron lesiones difusas corticosubcorticales hipodensas en la tomografía y en las secuencias T1 e hiperdensas en las secuencias T2, con captación irregular de contraste. Se midió la presión intracraneal mediante catéter ventricular con la aparición de ondas de presión elevadas y mantenidas (ondas A de Lundberg). El estudio del líquido cefalorraquídeo ventricular y la angiografía cerebral no aportaron nuevos datos. Las biopsias meníngea y cerebral demostraron una infiltración por glioma pleomorfo, permitiendo el diagnóstico de gliomatosis cerebri. El enfermo fue tratado con esteroides, agentes hiperosmolares, derivación externa de líquido cefalorraquídeo y radioterapia tumoral, a pesar de lo cual falleció a los 6 meses del inicio de los síntomas. La gliomatosis cerebri debe considerarse en el diagnóstico diferencial de aquellos cuadros clínicos que se inicien con hipertensión intracraneal (AU)

[Clinical and prognostic heterogeneity in Aicardi's syndrome: a description of two cases]. / Heterogeneidad clínica y pronóstica en el síndrome de Aicardi: a propósito de dos casos.

INTRODUCTION: Aicardi's syndrome is characterized by infantile spasms, agenesis of the corpus callosum and ocular lesions. Clinically it presents as severe mental retardation, severe limitation of motor development and of language, with a prognosis of survival for only a few months or years. We present two new cases of this uncommon syndrome and describe the heterogeneity of its clinical and prognostic severity. CLINICAL CASES: Case 1. A ten-month old patient had flexion spasms of the limbs at the age of 4 months, bilateral corioretinal lesions and generalized hypoplasia of the corpus callosum. During the clinical course of the disorder, the epileptic crises were controlled, there was mental retardation, the head was held steady and the baby could sit. Case 2. A nine year old patient had had flexion spasms when aged 2 months, had bilateral retinal lesions and generalized hypoplasia of the corpus callosum. During his clinical course the epileptic crises were controlled, there was severe mental retardation, the patient could pay attention and collaborate, articulate single words, walk on his own and manipulate objects. CONCLUSION: Aicardi's syndrome should be considered to be a syndrome in which the clinical findings and prognosis are heterogeneous, as seen from new cases with less clinical and functional limitation than the patients first described.