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

Manabe, Yoshie; Narai, Hisashi; Warita, Hitoshi; Hayashi, Takeshi; Sakai, Kenichi; Abe, Kōji

doi:10.2169/internalmedicine.40.964

Cited by 11 publications

(4 citation statements)

References 21 publications

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“…As in the case described here, four of these reports noted the presence of cranial nerve palsy. In addition, although patients were RF positive, no involvement of autoimmune mechanisms was observed 18 – 21. The average age of the patients in these studies was 69 years, and most of them had RF concentrations of 100 IU/ml or higher.…”

Section: Discussionmentioning

confidence: 77%

A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase

Nakamagoe

Hosaka²,

Kondo³

et al. 2010

Case Reports

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This report concerns a 53-year-old male patient with idiopathic hypertrophic cranial pachymeningitis who presented with multiple cranial nerve palsies (I, II, III, IV, V, VI). Brain magnetic resonance imaging showed diffuse thickening and gadolinium enhancement of the cerebral dura mater. A biopsy of the cerebral dura mater showed granulomatous vasculitis with histiocyte infiltration. Although both the serum rheumatoid factor (RF) and matrix metalloproteinase-3 (MMP-3) were high, the patient showed no signs of arthritis. He was anti-cyclic citrullinated peptide antibody negative, which makes the presence of comorbid chronic rheumatoid arthritis (RA) unlikely. The aetiology of the pachymeningitis was unknown, which led to the diagnosis of idiopathic hypertrophic cranial pachymeningitis. Steroid pulse therapy successfully diminished the patient's pachymeningitis and lowered both RF and MMP-3. High values of RF suggest the possible involvement of an autoimmune mechanism, and the MMP value may be an important indicator of the aetiology of pachymeningitis with granulomatous vasculitis.

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Section: Discussionmentioning

confidence: 77%

A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase

Nakamagoe

Hosaka²,

Kondo³

et al. 2010

Case Reports

View full text Add to dashboard Cite

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“…1). So wurde die HP bei rheumatischen Erkrankungen und Vaskulitiden öfter beschrieben: An erster Stelle ist hier der M. Wegener [13,18,32,42,43] zu nennen, ferner auch die Arteriitis temporalis [15,19,23,26], rheumatoide Arthritis [16,25,33,35] und Panarteriitis nodosa [9]. Seltener wird die HP bei Kollagenosen wie dem Sjögren-Syndrom [22] oder Sharp-Syndrom ("mixed connective tissue syndrome") [8] beobachtet.…”

Section: äTiologieunclassified

“…Des Weiteren wurde über Stauungspapillen, Liquorabflussstörungen, Funktionsstörungen der Hypophyse und epileptische Anfälle berichtet [8,25,39,51]. In seltenen Fällen wird die Erkrankung durch ein Hirnödem, Thrombosen der Hirnsinus und sogar eine Stenosierung der A. carotis interna kompliziert [2,10,21] (.…”

Section: Klinische Symptomatikunclassified

Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

et al. 2006

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Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.

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“…Rheumatoid vasculitis is one of the extra-articular complications of rheumatoid arthritis (RA). Although there are no reports of the exact incidence of central nervous system (CNS) complications of RA [ 1 ], they are considered relatively rare [ 2 ]. Among such complications, there have been extremely rare cases of sudden-onset focal brain dysfunction that resemble a stroke, which is known as rheumatoid meningitis presenting with a stroke-like attack (RMSA) [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Rheumatoid meningitis presenting with a stroke-like attack treated with recombinant tissue plasminogen activator: a case presentation

et al. 2018

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BackgroundRheumatoid meningitis presenting with a stroke-like attack (RMSA) is a rare manifestation of rheumatoid arthritis (RA). When the patients arrive within the time-window for recombinant tissue plasminogen activator (rt-PA) infusion therapy, no diagnostic protocol has been established.Case presentationA 55-year-old woman was brought by ambulance to our hospital with complaints of sudden-onset dysarthria and left arm numbness. The National Institutes of Health Stroke Scale (NIHSS) score was 5, and the Alberta Stroke Program Early CT Score was 8. She was diagnosed with acute embolic stroke. At 4 h, 6 min after onset, intravenous administration of rt-PA (alteplase, 0.6 mg/kg) was started. Her neurological deficits improved rapidly, and her NIHSS score was 1. Brain MRI was then performed. There was no hemorrhagic transformation, but the MRI findings were not compatible with ischemic stroke. She had a past history of RA diagnosed 6 months earlier, and she had been treated with methotrexate (10 mg daily). She was diagnosed with RMSA, and continuous infusion of methylprednisolone 1000 mg daily was started for 3 days. The high signal intensity on the FLAIR image disappeared.ConclusionCT-based decision-making for rt-PA injection is reasonable, but MRI is needed for the early diagnosis of RMSA. In this case, it is particularly important that neither adverse events nor bleeding complications were observed, suggesting the safety of CT-based thrombolytic therapy in RMSA.

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

Cited by 11 publications

References 21 publications

A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase

A case of idiopathic hypertrophic cranial pachymeningitis presenting high values of matrix metalloproteinase

Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

Rheumatoid meningitis presenting with a stroke-like attack treated with recombinant tissue plasminogen activator: a case presentation

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