Nota Clínica

Hypertrophic cranial and spinal pachymeningitis. A description of four new cases and a review of the literature

P.E. Jiménez-Caballero, J. Diamantopoulos-Fernández, I. Camacho-Castañeda [REV NEUROL 2006;43:470-475] PMID: 17033980 DOI: https://doi.org/10.33588/rn.4308.2006097

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Volumen 43 | Number 08 | Nº of views of the article 15.549 | Nº of PDF downloads 1.258 | Article publication date 16/10/2006

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ABSTRACT

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. KeywordsCerebral oedema Cranial polyneuropathy Epileptic seizures Hypertrophic pachymeningitis Meningeal biopsy Meningeal enhancement CategoriesEpilepsias y síndromes epilépticos Infecciones Nervios periféricos, unión neuromuscular y músculo

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Nota Clínica

Hypertrophic cranial and spinal pachymeningitis. A description of four new cases and a review of the literature