[Hypertrophic cranial and spinal pachymeningitis. A description of four new cases and a review of the literature]. / Paquimeningitis hipertrofica craneal y espinal. Descripcion de cuatro casos nuevos y revision de la bibliografia.

INTRODUCTION: Hypertrophic pachymeningitis is an infrequent disease that is characterised by inflammatory hypertrophy of the dura mater. There are cranial, spinal and craniospinal forms of the disease. They may be due to underlying infectious, autoimmune or neoplastic processes, although most of the cases reported in recent years have no base pathology and are known as idiopathic hypertrophic pachymeningitis. The ideal treatment is unknown, but most cases usually respond well to therapy with corticoids. CASE REPORTS: We report two cases of cranial forms, one idiopathic with typical clinical features consisting in cranial polyneuropathy and good response to corticoids, and another case secondary to rheumatoid arthritis that began with epileptic seizures. Both had a parenchymatous oedema. The two forms of spinal pachymeningitis presented as progressive paraparesis that evolved well after surgical removal of the lesion. CONCLUSIONS: On observing pachymeningitis in neuroimaging tests it becomes necessary to conduct a comprehensive aetiological study in search of infectious, autoimmune and neoplastic diseases. In the cranial forms there may be parenchymatous oedema, which would explain the epileptic seizures and the cognitive deterioration of our patients. Response to corticoids is spectacular, although it is often necessary to continue to administer them for prolonged periods of time.

Paquimeningitis hipertrófica craneal y espinal. Descripción de cuatro casos nuevos y revisión de la bibliografía / Hypertrophic cranial and spinal pachymeningitis. A description of four new cases and a review of the literature

Introducción. La paquimeningitis hipertrófica es una enfermedad infrecuente caracterizada por la hipertrofia de carácter inflamatorio de la duramadre. Existen formas craneales, espinales y craneoespinales. Pueden deberse a procesos infecciosos, autoinmunes o neoplásicos subyacentes, aunque la mayoría de los descritos en los últimos años no presenta patología de base, es la paquimeningitis hipertrófica idiopática. Se desconoce el tratamiento idóneo, pero la mayoría de los pacientes suele responder a la terapia corticoidea. Casos clínicos. Presentamos dos formas craneales, una idiopática con clínica típica de multineuropatía craneal y respuesta buena a los corticoides y otro caso secundario a artritis reumatoide que comenzó con crisis epilépticas. Ambos tenían un edema parenquimatoso. Las dos formas de paquimeningitis espinal se presentaron como una paraparesia progresiva con buena evolución tras extirpación quirúrgica de la lesión. Conclusiones. La observación de una paquimeningitis en las pruebas de neuroimagen obliga a un amplio estudio etiológico en busca de enfermedades infecciosas, autoinmunes y neoplásicas. En las formas craneales pueden existir edema parenquimatoso, lo que explicaría las crisis epilépticas y el deterioro cognitivo de nuestros pacientes. La respuesta a los corticoides es espectacular, aunque muchas veces se requiere su mantenimiento de forma continua

Introduction. Hypertrophic pachymeningitis is an infrequent disease that is characterised by inflammatory hypertrophy of the dura mater. There are cranial, spinal and craniospinal forms of the disease. They may be due to underlying infectious, autoimmune or neoplastic processes, although most of the cases reported in recent years have no base pathology and are known as idiopathic hypertrophic pachymeningitis. The ideal treatment is unknown, but most cases usually respond well to therapy with corticoids. Case reports. We report two cases of cranial forms, one idiopathic with typical clinical features consisting in cranial polyneuropathy and good response to corticoids, and another case secondary to rheumatoid arthritis that began with epileptic seizures. Both had a parenchymatous oedema. The two forms of spinal pachymeningitis presented as progressive paraparesis that evolved well after surgical removal of the lesion. Conclusions. On observing pachymeningitis in neuroimaging tests it becomes necessary to conduct a comprehensive aetiological study in search of infectious, autoimmune and neoplastic diseases. In the cranial forms there may be parenchymatous oedema, which would explain the epileptic seizures and the cognitive deterioration of our patients. Response to corticoids is spectacular, although it is often necessary to continue to administer them for prolonged periods of time