Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjögren’s syndrome

Li, J.-Y.; Lai, Ping-Hong; Lam, Hing‐Chung; Lu, Ling‐Ying; Cheng, He‐Hsiung; Lee, J.-K.; Lo, Yi-Jing

doi:10.1212/wnl.52.2.420

Cited by 60 publications

(28 citation statements)

References 11 publications

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“…HCPis considered to be an autoimmunedisorder because of its association with other autoimmune disorders. HCPis associated with disorders such as, mixed connective tissue disorder and rheumatoid arthritis (RA) (15,19,20). The present patient had a seropositive rheumatoid factor, but no multiple joint deformities.…”

Section: Discussionmentioning

confidence: 73%

Rheumatoid Factor Positive Hypertrophic Cranial Pachymeningitis in Association with Hypopituitarism and Multiple Cranial Nerve Palsies.

et al. 2001

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This is the first report of a patient presenting with rheumatoid factor (RF) positive hypertrophic cranial pachymeningitis (HCP) in association with hypopituitarism and multiple cranial nerve palsies. Our patient developed palsies of the left II and III, bilateral VI and VII, and right IX, X, and XII cranial nerves. A stimulation test showed hypopituitarism due to hypothalamic failure. The patient was seropositive for RF but had no multiple joint pain or deformities. Magnetic resonance imaging (MRI) showed thickened dura of the sellar and parasellar region, hypothalamus, bilateral cavernous sinuses and the tentorium all of which were enhanced by gadolinium (Gd). Treatment with prednisone improved clinical symptoms and MRIfindings concomitant with reduction of RF titer. Although the exact mechanism of HCPhas not been clearly elucidated, the present case suggests an autoimmune mechanism associated with RF. (Internal Medicine 40: 964-967, 2001)

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Section: Discussionmentioning

confidence: 73%

Rheumatoid Factor Positive Hypertrophic Cranial Pachymeningitis in Association with Hypopituitarism and Multiple Cranial Nerve Palsies.

et al. 2001

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“…The pathogenic mechanism of extradural enhancement may be attributable to the vascular dilatation of dural arteries and medullary and cortical veins to compensate for reduced CSF volume and a greater volume of gadolinium remaining in the dilated extradural microvasculature and diffusing into the extradural interstitial fluid (7,20). Non-hypotensive pachymeningeal enhancement may result from various dural tissue responses to infection, immunological mechanisms, systemic or regional granulomatosis, cancer metastasis, various connective tissue diseases, or other unknown causes (3,12,14,17,32). That is why the biochemical and microbiological examinations of CSF and the histopathological evaluation of dura and arachnoid mater were needed to assure the diagnosis of ICH.…”

Section: Discussionmentioning

confidence: 99%

A case of intracranial hypotension complicated with hydrocephalus

Özişik

Berker²,

Önal³

2009

Turkish Neurosurgery

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AIM: Intracranial hypotension may have variable clinical presentation. The imaging characteristics of intracranial hypotension are especially well depicted on magnetic resonance imaging studies. Although the clinical and radiological manifestations of spontaneous intracranial hypotension are increasingly recognized in many reports, many other abnormalities in this disorder and complicating cases still exist. MATeRIAL and MeTHodS:A 16-year-old patient suffering from nausea, vomiting and blurred consciousness was admitted to the hospital. MRI showed diffuse pachymeningeal thickening resembling intracranial hypotension and ventricular enlargement like compensated hydrocephalus. The patient was investigated and treated using endoscopic third ventriculostomy. CoNCLUSIoN: The authors presented a case of intracranial hypotension complicated with hydrocephalus. Specific causes for intracranial hypotension as well as additional new treatment options will also be discussed. KeywoRdS: Intracranial hypotension, Triventricular hydrocephalus, Lumbar puncture, Herniation ÖZ AMAç: İntrakranial hipotansiyon farklı klinik bulgular verebilen bir antitedir. MRI bulguları literatürde ayrıntılı olarak tarif edilmekle birlikte, nöroşirürji pratiğinde her zaman akla gelmemektedir. Aynı bulguların farklı sinir sistemi hastalıklarında da görülebilmesi, vakaları komplike hale getirmektedir. yÖNTeM ve GeReç: 16 yaşında bayan hasta bulantı-kusma ve bilinç bulanıklığı nedeniyle hastanemize yatırıldı. MRI intrakranial hipotansiyonla uyumlu difüz pakimeningeal kalınlaşma ve kontrast tutulumu ve eşlik eden triventriküler genişleme gösterdi. Hasta ayrıntılı olarak incelendi ve endoskopik yolla 3. ventrikülostomi yapılarak tedavi edildi. SoNUç: Yazarlar, arrest hidrosefali ile komplike olan bir intrakranial hipotansiyon vakasını sunmuş ve tedavi yöntemi ile birlikte tartışmışlardır. Endoskopik 3. ventrikülostomi, bu vaka için tanı koydurucu ve emniyetli bir seçenek olmuştur.

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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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Hypertrophic cranial pachymeningitis and lymphocytic hypophysitis in Sjögren’s syndrome

Abstract: The authors describe a patient with primary Sjögren's syndrome who developed pachymeningitis, hypopituitarism, and central diabetes insipidus. The patient improved with corticosteroid pulse therapy.

Cited by 60 publications

References 11 publications

Rheumatoid Factor Positive Hypertrophic Cranial Pachymeningitis in Association with Hypopituitarism and Multiple Cranial Nerve Palsies.

Rheumatoid Factor Positive Hypertrophic Cranial Pachymeningitis in Association with Hypopituitarism and Multiple Cranial Nerve Palsies.

A case of intracranial hypotension complicated with hydrocephalus

Paquimeningitis craneal hipertrófica idiopática.

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