Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options

Hatano, Norikazu; Behari, Sanjay; Nagatani, Tetsuya; Kimura, Masaaki; Ooka, Keiji; Saito, Kiyoshi; Yoshida, Jun

doi:10.1097/00006123-199912000-00014

Cited by 102 publications

(117 citation statements)

References 21 publications

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“…The natural history of cranial hypertrophic pachymeningitis has been well documented. The course of the disease follows one of the three patterns: (1) sustained remission, (2) relapse with corticosteroid resistance, or (3) relapse with corticosteroid dependence [6,21]. In contrast, the course of the spinal form has been less studied, since it is uncommon, but it is believed to run a progressive course.…”

Section: Discussionmentioning

confidence: 99%

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

et al. 2011

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Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.

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Section: Discussionmentioning

confidence: 99%

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

et al. 2011

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“…Parney et al encontraron en la literatura informes sobre 33 casos hasta 1996 en Medline (3). Al utilizar esta misma base de datos, identificamos 38 casos adicionales hasta abril de 2004 (1,(5)(6)(7)(8)(9). La entidad afecta especialmente a adultos mayores, generalmente después de los 50 años, con un ligero predominio del sexo masculino (1,5,7).…”

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“…La resonancia magnética también es de ayuda en el seguimiento de los pacientes; con la disminución de la inflamación como consecuencia del tratamiento, el realce anormal dural disminuye o desaparece. De igual manera, permite detectar la reactivación de la enfermedad al presentarse, nuevamente, los hallazgos característicos (1,(5)(6)(7). Recientemente, se ha llamado la atención acerca de la utilidad de la tomografía computarizada por emisión de fotón único (SPECT, por su sigla en inglés) con talio 201; este trazador se acumula significativamente en la duramadre de los pacientes con paquimeningitis hipertrófica (11).…”

Section: Discussionunclassified

“…El diagnóstico diferencial es amplio con todas las demás patologías que producen engrosamiento de la duramadre (cuadro 2) (1,(3)(4)(5)(6)(7)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22) La patogénesis de la paquimeningitis hipertrófica idiopática no está clara. Su estrecha relación con enfermedades del tejido conjuntivo como la granulomatosis de Wegener, la artritis reumatoidea o la enfermedad mixta del tejido conjuntivo y su reciente asociación con los p-ANCA en Japón, sugieren que pudiera tratarse de un síndrome vasculítico o de una enfermedad del tejido conjuntivo.…”

Section: Discussionunclassified

“…La paquimeningitis hiper trófica ha sido frecuentemente atribuida a etiologías específicas como la tuberculosis y la sífilis (3,4). Sin embargo, los casos más recientes parecen ser idiopáticos ya que una intensa búsqueda para definir la etiología fue negativa (1,(5)(6)(7).…”

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Paquimeningitis craneal hipertrófica idiopática.

Lizarazo¹,

Parra²,

Gutiérrez³

et al. 2004

biomedica

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La paquimeningitis craneal hipertrófica idiopática es un raro proceso inflamatorio crónico de etiología desconocida que produce engrosamiento de la duramadre y alteraciones neurológicas progresivas debido a la compresión de estructuras vecinas. Se presenta el caso de una mujer adulta con un cuadro clínico crónico de cefalea, fiebre, pérdida progresiva de la visión y neuropatía óptica bilateral. El diagnóstico se basó en la visualización de la duramadre engrosada en las neuroimágenes y en la exclusión de causas conocidas mediante estudios histopatológicos. La terapia con corticoesteroides mejoró la cefalea pero no el déficit visual. El diagnóstico y el seguimiento de esta entidad se han facilitado con la utilización de la resonancia magnética cerebral con medio de contraste. La biopsia de duramadre sigue siendo la regla de oro para el diagnóstico definitivo de la enfermedad. La terapia con esteroides produce mejoría en un buen número de pacientes; sin embargo, son frecuentes las recaídas por lo que es necesario el uso concomitante de otro inmunosupresor como la ciclofosfamida o la azatioprina. La mortalidad es baja, pero son comunes las secuelas neurológicas definitivas.Palabras clave: paquimeningitis, neuropatía óptica, cefalea, meningitis, esteroides. Idiopathic hypertrophic cranial pachymeningitisIdiopathic hypertrophic cranial pachymeningitis is an infrequent chronic inflammatory process of unknown etiology which causes thickening of the dura mater and progressive neurologic alterations due to the compression of adjacent structures. A case is presented of an adult woman with a clinical syndrome consisting of headache, progressive visual loss and bilateral optic neuropathy. The diagnosis was based upon visualization of the thickened dura mater in neuroimaging studies and the exclusion of known causes by histopathological examination. Diagnosis and follow-up of this condition are currently easier with the use of nuclear magnetic resonance with contrast medium. Biopsy of the dura mater continues to be the gold standard for the definitive diagnosis of this disease. Steroid therapy causes clinical improvement in most of the patients; however, relapses are frequent, making necessary the concomitant use of other immunosuppressive agents such as cyclophosphamide or azathioprine. Mortality is low but definitive neurologic sequelae are common.

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In vivo 3D analysis of the adipose tissue in the orbital apex and the compartments of the parasellar region

Weninger

Prokop

2004

Clinical Anatomy

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As tissue dissections carried out on formaldehyde-fixed anatomical specimens demonstrate, the parasellar region (PSR), traditionally referred to as the cavernous sinus, is composed of three distinct compartments: orbital, pterygopalatine, and lateral sellar. The aim of our study was to identify and measure these compartments in the living and to describe the topography of their adipose body tissues. For this purpose data sets of 35 patients, who had undergone thin-section multislice computed tomography (CT), were examined using 3D-reconstruction software. The pterygopalatine and orbital compartments of the PSR could be identified in the images by their adipose bodies. We provide more exacting measurements of their size than have been presented in earlier studies. Furthermore, we include data about the uni- and bilateral presence of the single compartments and analyze the topography of the adipose tissue bodies in the orbital apex region. Our quantitative data and topographical descriptions confirm the compartmentalization concept of the PSR, allow for correct interpretation of CT scans of the orbital apex and anterior PSR, and provide baseline information for individual planning of surgical and radiological interventions.

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Idiopathic Hypertrophic Cranial Pachymeningitis: Clinicoradiological Spectrum and Therapeutic Options

Abstract: Idiopathic hypertrophic cranial pachymeningitis exhibits varied clinical courses. It is important to prevent irreversible cranial neuropathy during the active phase of the disease, using daily administration of corticosteroids, pulse corticosteroid therapy, or surgical decompression.

Cited by 102 publications

References 21 publications

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

Paquimeningitis craneal hipertrófica idiopática.

In vivo 3D analysis of the adipose tissue in the orbital apex and the compartments of the parasellar region

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