[PMID]: | 28723776 |
[Au] Autor: | Huang Y; Chen J; Gui L |
[Ad] Dirección: | aDepartment of Neurology bDepartment of General Surgery and Center of Minimal Invasive Gastrointestinal Surgery, Southwest Hospital, The Third Military Medical University, Chongqing, China. |
[Ti] Título: | A case of idiopathic hypertrophic pachymeningitis presenting with chronic headache and multiple cranial nerve palsies: A case report. |
[So] Fuente: | Medicine (Baltimore);96(29):e7549, 2017 Jul. |
[Is] ISSN: | 1536-5964 |
[Cp] País de publicación: | United States |
[La] Idioma: | eng |
[Ab] Resumen: | RATIONALE: Idiopathic hypertrophic pachymeningitis (IHP) is a rare condition, characterized by a chronic fibrosing inflammatory process usually involving either the intracranial or spinal dura mater, but rarely both. Here, we report a rare case of IHP affecting both the intracranial and spinal dura mater. We also discussed the diagnosis, management, and outcome of IHP. PATIENT CONCERNS: We reviewed the case of a 60-year-old woman presenting with chronic headache, multiple cranial nerve palsies and gait disturbance. Magnetic resonance imaging (MRI) of her head revealed thickened and contrast-enhanced dura in the craniocervical region as well as obstructive hydrocephalus and cerebellar tonsillar herniation. The patient had a suboccipital craniectomy and posterior decompression through C1 plus a total laminectomy. The dura was partially resected to the extent of the bony decompression, and a duroplasty was performed. DIAGNOSES: Microscopic examination of the surgically resected sample showed chronic inflammatory changes, lymphoplasmacytic cell infiltration, fibrous tissue hyperplasia, and hyaline degeneration. Blood tests to evaluate the secondary causes of hypertrophic pachymeningitis (HP) were unremarkable. INTERVENTIONS: Steroid was used to treat suspected IHP. OUTCOMES: Postoperatively, the patient showed gradual improvement in her headache, glossolalia, and bucking. Prior to discharge, a follow-up MRI showed improvement of the dura mater thickening. LESSONS: IHP is a chronic inflammatory disorder of the dura mater that usually causes neurological deficits. Clinical manifestations of IHP, MRI findings, and laboratory abnormalities are the essential components for making an accurate diagnosis. When the radiological or laboratory evaluation is uncertain, but neurological deficits are present, a prompt surgical approach should be considered. Postoperative steroid therapy and close observation for recurrence are necessary to ensure a good long-term outcome. |
[Mh] Términos MeSH primario: |
Enfermedades de los Nervios Craneales/complicaciones Enfermedades de los Nervios Craneales/diagnóstico Trastornos de Cefalalgia/complicaciones Trastornos de Cefalalgia/diagnóstico Meningitis/complicaciones Meningitis/diagnóstico
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[Mh] Términos MeSH secundario: |
Encéfalo/diagnóstico por imagen Enfermedades de los Nervios Craneales/patología Enfermedades de los Nervios Craneales/terapia Diagnóstico Diferencial Duramadre/diagnóstico por imagen Duramadre/patología Duramadre/cirugía Femenino Trastornos de Cefalalgia/patología Trastornos de Cefalalgia/terapia Seres Humanos Meningitis/patología Meningitis/terapia Mediana Edad
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[Pt] Tipo de publicación: | CASE REPORTS; JOURNAL ARTICLE |
[Em] Mes de ingreso: | 1708 |
[Cu] Fecha actualización por clase: | 170808 |
[Lr] Fecha última revisión: | 170808 |
[Sb] Subgrupo de revista: | AIM; IM |
[Da] Fecha de ingreso para procesamiento: | 170721 |
[St] Status: | MEDLINE |
[do] DOI: | 10.1097/MD.0000000000007549 |