Hypertrophic pachymeningitis and undifferentiated connective tissue disease: a case report and review of the literature

Lampropoulos, Christos; Zain, Mollyza Mohd; Jan, Wajanat; Nader-Sepahi, Ali; Sabin, Ian; Cruz, David P. D’

doi:10.1007/s10067-005-0018-z

Cited by 13 publications

(7 citation statements)

References 7 publications

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“…Dural venous sinus occlusion/thrombosis in relation to IHPM is uncommonly reported, 3,5-13 as an incidental or a late imaging finding during the course of the disease. 7,8,10 Diagnosis of IHPM has also been established after visual loss following central retinal vein occlusion, 5 stroke, 9 dural arteriovenous fistula 11 or internal carotid occlusion. 13 In our patients the fibrosing inflammation within the dura mater probably encased the sinuses within it, resulting in their progressive occlusion, obliteration or both.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Bhatia

Tripathi

Srivastava

et al. 2009

Journal of Clinical Neuroscience

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Section: Discussionmentioning

confidence: 99%

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Bhatia

Tripathi

Srivastava

et al. 2009

Journal of Clinical Neuroscience

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“…Among these cases, two of them were hypertrophic cranial pachymeningitis (HCP), and the other one was reversible posterior leucoencephalopathy syndrome (RPLS). After symptomatic treatment, low-dose corticosteroid or immunosuppression treatment such as azathioprine or methotrexate, all the patients’ symptoms and radiologic abnormalities of brain were improved [10–12]. However, up to now, there is still lack of definite researches about clinical course, prognosis and organ involvement of early UCTD.…”

Section: Discussionmentioning

confidence: 99%

Intracranial lesion as onset symptom in a patient with early undifferentiated connective tissue disease: a case report

Du¹,

Li²,

Zhao³

et al. 2017

BMC Neurol

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BackgroundUndifferentiated connective tissue disease (UCTD) is widely considered to be a distinct clinical entity, and now divided into two subgroups: stable UCTD and early UCTD. The most frequent onset symptoms of UCTD include arthralgias, arthritis, Raynaud’s phenomenon, mucocutaneous involvement, and sicca symptoms. However, Neurologic involvement is rare, and intracranial lesion as onset symptom in a patient with early UCTD has not yet been reported.Case presentationA 51-year-old Chinese female experienced progressive left leg weakness for 14 days before hospitalizing in our department. The lesion on right parietal lobe was initially detected by brain magnetic resonance imaging. Although the patient declined a cerebral biopsy, the possibility of stroke, cerebral venous sinus thrombosis, NMOSD, MS, autoimmune encephalitis, intracranial infections, and malignant tumors as cause of the lesion was excluded by intracranial angiogram, CSF study, MRI enhancement and MRS examination. Moreover, immunologic studies showed high titer of antinuclear antibody, increased erythrocyte sedimentation rate and C-reactive protein. These results led to a diagnosis of early UCTD with central nerve system (CNS) involvement. After low dose corticosteroid and azathioprine therapy, the patient’s symptoms, abnormalities in immunologic tests and cerebral radiologic examinations were all greatly improved within a short duration.ConclusionsThis is the first report of intracranial lesion as onset symptom in a patient with early UCTD. Our case suggested that central nerve system (CNS) involvement could be the onset symptom in early UCTD, and should be recognized quickly with exclusion of other causative factors in the differential diagnosis. Prompt and adequate treatment with low-dose steroid and immunosuppressive drugs could improve the prognosis of both early UCTD and CNS involvement.Electronic supplementary materialThe online version of this article (doi:10.1186/s12883-017-0868-4) contains supplementary material, which is available to authorized users.

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“…An increasing number of case reports suggested an association of HP with autoimmune diseases, most commonly Wegener's granulomatosis and ANCA-positive vasculitis [5,6]. An association with connective tissue diseases, such as undifferentiated connective tissue disease [7], mixed connective tissue disease [8], rheumatoid arthritis [9], Sjogren's syndrome [10,11], antiphospholipid syndrome [12] had also been reported.…”

Section: Discussionmentioning

confidence: 99%

A pachymeningitis as an unusual cause of headache in systemic lupuserythematosus

Larbi¹,

Hamzaoui²,

Mrouki³

et al. 2016

Lupus Open Access

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We report a case of systemic lupus erythematosus (SLE) complicated by hypertrophic pachymeningitis (HP) four year after the onset of SLE. A 25-year-old woman with a previous diagnosis of SLE was admitted for severe headaches and vomiting without signs of meningeal irritation or papilloedema on fundus examination. Brain magnetic resonance imaging showed diffuse thikening of the dura matter with hyperintense signal on fluidattenuated inversion recovery sequence and gadolinium contrast enhancement. There was no sinus thrombosis on MR venography. Both clinical symptoms and imaging findings improved after steroid therapy.

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Hypertrophic pachymeningitis and undifferentiated connective tissue disease: a case report and review of the literature

Abstract: A 63-year-old man with hypertrophic pachymeningitis (HP) and an undifferentiated connective tissue disease is described. Combined therapy with prednisolone and azathioprine improved his symptoms. The association between an undifferentiated connective tissue disease and HP is discussed.

Cited by 13 publications

References 7 publications

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Idiopathic hypertrophic cranial pachymeningitis and dural sinus occlusion: two patients with long-term follow up

Intracranial lesion as onset symptom in a patient with early undifferentiated connective tissue disease: a case report

A pachymeningitis as an unusual cause of headache in systemic lupuserythematosus

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