Hypertrophic pachymeningitis: case report

Deus-Silva, Leonardo; Queiróz, Luciano de Souza; Zanardi, Verônica A.; Ghizoni, Enrico; Pereira, Hoyama da Costa; Malveira, George Linard Silva; Pirani, C L; Damasceno, Benito Pereira; Cendes, Fernando

doi:10.1590/s0004-282x2003000100021

Cited by 16 publications

(10 citation statements)

References 24 publications

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“…Cranial gadolinium MRI is more sensitive than contrast enhanced CT in demonstrating the thickened abnormal enhancing dura-mater; non-contrast CT should be used to exclude neoplasia [39]. The definitive diagnosis is made by histophatological findings, which usually show an inflammatory process with lymphomononuclear cell infiltration and a thickened dura-mater characterized by a dense hypocellular fibrous tissue [1, 40].…”

Section: Discussionmentioning

confidence: 99%

“…Serum angiotensin converting enzyme, c and p-ANCA, rheumatoid factor and anti-nuclear antibody must be tested; an evaluation of Lyme, syphilis, tuberculosis and fungi is also necessary [8, 14]. The CSF findings are dependent on the etiology; when infection is not present, CSF frequently shows non-specific pleocytosis, predominantly lymphocytic; there may be an increase in protein content [39, 51]. …”

Section: Discussionmentioning

confidence: 99%

“…IMT is also known as “myofibroblastoma” or “inflammatory pseudotumor”, and is characterized by proliferation of myofibroblastic spindle cells with mixed inflammatory infiltrates of plasma cells [59]; IMTs often include orbital involvement [60]. THS is a retro-orbital pseudotumor that involves the cavernous sinus, with imaging findings similar to IMT [39, 58]; it causes a painfull oftalmoplegia due to a nonspecific granulomatous inflammation of the cavernous sinus. Some described cases of THS have concurrent pachymeningitis in other portions of the cranial vault, raising the possibility that THS may be a focal manifestation of idiopathic HCP [61].…”

Section: Discussionmentioning

confidence: 99%

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Hypertrophic Cranial Pachymeningitis and Skull Base Osteomyelitis by Pseudomonas Aeruginosa: Case Report and Review of the Literature

et al. 2012

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Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder characterized by localized or diffuse thickening of the dura mater, and it usually presents with multiple cranial neurophaties. It has been associated with a variety of inflammatory, infectious, traumatic, toxic and neoplasic diseases, when no specific cause is found the process is called idiopathic. The infectious cases occur in patients under systemic immunosuppression, which have an evident contiguous source or those who have undergone neurosurgical procedures. We describe a case of a 62-year-old immunosuppressed woman with diabetes and rheumatoid arthritis, which had HCP and osteomyelitis of the skull base caused by pseudomonas aeruginosa, presenting with headache and diplopia. We believe this is the second documented case of pachymeningitis secondary to this microorganism. As a multifactorial disease, it is essencial to determine the specific causative agent of HCP before making treatment decisions, and great care is needed with immunocompromised patients.KeywordsPseudomonas aeruginosa; Hypertrophic pachymeningitis; Ophtalmoplegia, optical neuropathy; Osteomyelitis; Skull base

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hypertrophic Cranial Pachymeningitis and Skull Base Osteomyelitis by Pseudomonas Aeruginosa: Case Report and Review of the Literature

et al. 2012

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“…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

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 T2- weighted imaging, hypo-and hyperintense images can be observed, depending on the degree of fibrosis and of the active inflammatory process. Peripheral enhancement and T2 hyperintensity are common in the early stages of the disease and correspond to inflammatory activity, whereas hypointensity is attributed to fibrosis 13 . The location and pattern of dura mater enhancement is described as linear, nodular, or both.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Hypertrophic Pachymeningitis Manifesting with Frontal Brain Edema

Júnior

Felix²,

Leão³

2001

Rev Neurocienc

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Introduction. Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that results in thickening of the dura mater. Atypically, it can progress to include the involvement of the cerebral parenchyma. Method. In this paper, we report the rare case of a 31-year-old man with a three-week history of headaches, seizures, impairments of cognitive function, and changes in behavior and mood. Magnetic resonance imaging (MRI) of the brain showed a thickening and an abnormal enhancement of the dura mater over the falx cerebri with extensions into the adjacent cranial base and with brain edemas in the frontal lobes. Histopathological study of meningeal and brain biopsies showed an inflammatory process that was compatible with HP. The results of an extensive laboratory investigation were unremarkable and did not provide additional information on the cause of the meningeal disease. The patient exhibited relapses despite immunosuppressive therapy. Conclusion. This case shows the challenges associated with the management of the disease and the importance of early diagnosis to avoid worsening of the condition and cerebral damage.

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Hypertrophic pachymeningitis: case report

Cited by 16 publications

References 24 publications

Hypertrophic Cranial Pachymeningitis and Skull Base Osteomyelitis by Pseudomonas Aeruginosa: Case Report and Review of the Literature

Hypertrophic Cranial Pachymeningitis and Skull Base Osteomyelitis by Pseudomonas Aeruginosa: Case Report and Review of the Literature

Paquimeningitis craneal hipertrófica idiopática.

Idiopathic Hypertrophic Pachymeningitis Manifesting with Frontal Brain Edema

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