Comienzo con diplopía y parálisis descendente: un Guillain-Barré atípico / First onset of diplopia and descending paralysis: Atypical Guillain-Barré

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[Anti-NMDA receptor encephalitis in a 3-year-old girl with no associated pathology involving a tumour]. / Encefalitis antirreceptor de NMDA en una nina de 3 anos sin patologia tumoral asociada.

INTRODUCTION: Encephalitis due to NMDA receptors antibodies is a relatively common condition but it was under diagnosed until recently. It courses predictably and similarly in adults and children, although there are some differences, still less its association with tumours. CASE REPORT: A 3 years-old girl who was admitted to our hospital with symptoms compatible with acute encephalitis, so we started treatment with acyclovir. During admission she was alterning periods of poor response to stimuli with periods of agitation, and progressed to complete silence, adding sleep problems. She suffered epileptic seizures, dystonic movements and autonomic disturbances. Cranial MRI showed mild cortical atrophy and EEG generalized slowing of base tracing. Repeated samples of cerebral spinal fluid were normal from cytological and biochemical point of view. In view of the torpid evolution she began methylprednisolone therapy and later inmunoglobulins with no improvement. Upon confirmation of the positivity for NMDA receptors antibodies in cerebral spinal fluid and serum, cyclophosphamide was administered, with gradual improvement of symptoms until full recovery. After ten months of follow-up without treatment she has not presented relapses and has ruled out the presence of tumours. CONCLUSIONS. It is important to recognize encephalitis with behavioural changes and abnormal movements, because early diagnosis and the beginning of appropriate therapy could improve the prognosis.

[Severe cerebellar mutism after posterior fossa tumor resection]. / Mutismo cerebeloso grave tras cirugía de un tumor de fosa posterior.

Cerebellar mutism is an infrequent but important complication after posterior fossa surgery in children. Dysarthria, irritability and ataxia are among the signs and symptoms of this disorder, which are usually mild and self-limiting. However, in severe cases, there can be impairment of higher-level cognitive functions, affecting the child's future personal and social relations. This disorder has been described in many other situations and consequently pediatricians should be familiar with its symptoms, physiopathology, diagnosis, degrees of severity, treatment, and prognosis, since a multidisciplinary approach is required. We present the case of a 5-year-old boy who underwent surgery for a low-grade ependymoma in the fourth ventricle; 48 hours after surgical resection, the boy developed irritability, cranial nerve involvement and stereotyped movements in the context of active hydrocephalus. His symptoms progressively improved 6 weeks after the intervention. We review the literature on cerebellar mutism and discuss the physiopathology of this disorder, which seems to confirm that the cerebellum not only acts as a simple coordinator of motor function, but also plays an important role in higher-level cognitive functions, such as language.

Mutismo cerebeloso grave tras cirugía de un tumor de fosa posterior / Severe cerebellar mutism after posterior fossa tumor resection

El mutismo cerebeloso es una complicación infrecuente pero con gran trascendencia en los niños intervenidos de cirugía de fosa posterior. Disartria, irritabilidad y ataxia, son parte de sus síntomas y signos, que suelen ser leves y autolimitados, pero en algunos casos graves, pueden alterarse funciones cognitivas superiores, pudiendo verse comprometidas las relaciones personales y sociales futuras del niño. Está descrito en otras situaciones, por lo que es importante para el pediatra el conocimiento de sus síntomas, fisiopatología, diagnóstico, grados de severidad y enfoque terapéutico, así como su pronóstico, dado que requiere de un tratamiento multidisciplinario. Presentamos el caso clínico de un niño de 5 años intervenido de un ependimoma de bajo grado localizado en el cuarto ventrículo, que desarrolló 48 h después de la resección quirúrgica, un cuadro consistente en mutismo, irritabilidad, afectación de pares craneales y movimientos estereotipados, en el contexto de una hidrocefalia activa. La clínica del paciente mejoró progresivamente a partir de 1,5 mes tras la intervención. Revisamos la bibliografía existente sobre el mutismo cerebeloso y discutimos su fisiopatología, que parece confirmar que el cerebelo no participa únicamente como un simple coordinador de la función motora, sino que desempeña un papel relevante en funciones cognitivas superiores, como el lenguaje

Cerebellar mutism is an infrequent but important complication after posterior fossa surgery in children. Dysarthria, irritability and ataxia are among the signs and symptoms of this disorder, which are usually mild and self-limiting. However, in severe cases, there can be impairment of higher-level cognitive functions, affecting the child's future personal and social relations. This disorder has been described in many other situations and consequently pediatricians should be familiar with its symptoms, physiopathology, diagnosis, degrees of severity, treatment, and prognosis, since a multidisciplinary approach is required. We present the case of a 5-year-old boy who underwent surgery for a low-grade ependymoma in the fourth ventricle; 48 hours after surgical resection, the boy developed irritability, cranial nerve involvement and stereotyped movements in the context of active hydrocephalus. His symptoms progressively improved 6 weeks after the intervention. We review the literature on cerebellar mutism and discuss the physiopathology of this disorder, which seems to confirm that the cerebellum not only acts as a simple coordinator of motor function, but also plays an important role in higher-level cognitive functions, such as language

[Giant arteriovenous malformation of the scalp. Case report]. / Malformación arteriovenosa gigante de cuero cabelludo: caso clínico.

Arteriovenous malformations (AVM) in the scalp are infrequent vascular lesions. Its clinical presentation varies from annoying and unaesthetic mass of the skin to devastating hemorrhages. Its origin can be congenital or traumatic. The diagnosis of AVM is based on physical examination and confirmed by internal and external carotid angiography. Nowadays the gold standard treatment is the surgical intervention although the endovascular approach is gaining field in order to reduce blood losses as presurgical or like lonely treatment. A 50 year old woman was admitted with a huge mass in scalp, with subcutaneous enlarged vessels and no other symptoms. A head traumatic antecedent had occurred 12 years before. The angiography evidenced a mottled AVM with blood supplies from the external and internal carotid arteries, with meningeal transosseous branches from both ophthalmic arteries. Endovascular treatment could not be performed due to high risk of uni or bilateral amaurosis. Thus, a conventional surgical treatment was done without complications. The treatment of AVM of scalp offers various possibilities but the individualization of each case becomes essential to decide the correct management in order to avoid complications.

Malformación arteriovenosa gigante de cuero cabelludo: caso clínico / Giant arteriovenous malformation of the scalp. Case report

Las malformaciones arteriovenosas (MAV) en el cuero cabelludo son lesiones vasculares muy infrecuentes. Sus síntomas son variados, desde molestas y antiestéticas tumoraciones de la piel, hasta hemorragias que pueden ser devastadoras. Su origen puede ser congénito o traumático. El diagnóstico de las mismas es clínico, mediante la inspección del enfermo, y la angiografía de las carótidas interna y externa confirma el diagnóstico. Las posibilidades terapéuticas son variadas, siendo la extirpación quirúrgica el tratamiento de elección, aunque cada vez se utiliza más el tratamiento endovascular como tratamiento único o prequirúrgico, para disminuir las pérdidas hemáticas. Presentamos el caso de una paciente de 50 años de edad que presentaba una gran tumoración en cuero cabelludo, con dilataciones vasculares muy marcadas y sin otra sintomatología asociada. Describió un antecedente traumático hacía 12 años. La angiografía mostró una MAV abigarrada, con aporte vascular dela carótida externa y también de la interna, a cargo de ramas meníngeas transoseas provenientes de arterias etmoidales y dependientes de ambas arterias oftálmicas. El tratamiento endovascular de los aportes intracraneales no fue posible, debido al alto riesgo de amaurosis uni o bilateral, por lo que la lesión se resecó quirúrgicamente, sin presentarse complicaciones. El tratamiento de las MAV de cuero cabelludo ofrece diversas posibilidades pero se hace imprescindible la individualización de cada caso a la hora de decidir el abordaje correcto para evitar las posibles complicaciones

Arteriovenous malformations (AVM) in the scalp are in frequent vascular lesions. Its clinical presentation varies from annoying and unaesthetic mass of the skin to devastating hemorrhages. Its origin can be congenitalor traumatic. The diagnosis of AVM is based on physical examination and confirmed by internal and external carotid angiography. Nowadays the gold standard treatment is the surgical intervention although the endovascular approach is gaining field in order to reduce blood losses as presurgical or like lonely treatment. A 50 year old woman was admitted with a huge mass in scalp, with subcutaneous enlarged vessels and no other symptoms. A head traumatic antecedent had occurred 12 years before. The angiography evidenced amottled AVM with blood supplies from the external and internal carotid arteries, with meningeal transosseous branches from both ophthalmic arteries. Endovascular treatment could not be performed due to high risk of uni-or bilateral amaurosis. Thus, a conventional surgical treatment was done without complications. The treatment of AVM of scalp offers various possibilities but the individualization of each case becomes essential to decide the correct management in order to avoid complications