Chronischer Kopfschmerz mit Depression durch Pachymeningitis bei Wegener-Granulomatose

Bierbrauer, A. von; Pelzer, C; Fischer, Vernon W.

doi:10.1055/s-2008-1017494

Cited by 3 publications

(4 citation statements)

References 16 publications

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“…It appears that the presence of hypertrophic pachymeningitis identifies patients with vasculitic cranial neuropathy, who are clinically also distinct from those with normal brain scans. These patients have further complaints and symptoms pointing to CNS disease, for example severe headaches, nausea, cerebellar deficits or epileptic seizures [25].…”

Section: Diagnosis Of Active Vasculitis ■ Brain Imagingmentioning

confidence: 99%

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

et al. 2009

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The problem of diagnosing vasculitic neuropathy is discussed based on case reports of two patients with Wegener's granulomatosis. One patient developed de novo 6(th) nerve palsy as an isolated relapse manifestation and the second patient a sequence of multiple cranial nerve palsies. Brain imaging with CT and MRI and the laboratory provided no clues suggesting active vasculitis. However, in both patients the neuropathies fully recovered in response to standard induction protocols of vasculitis. In the absence of organ-specific proof of vasculitis, these treatment decisions were guided by the overall clinical presentations. Cranial neuropathy may be the first obvious vasculitic manifestation preceding other organ disease, and since single reliable tests for its diagnosis are lacking, a multidisciplinary approach is advocated here to detect vasculitic manifestations in other organs.

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Section: Diagnosis Of Active Vasculitis ■ Brain Imagingmentioning

confidence: 99%

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

et al. 2009

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“…In our research, the main neurological manifestation at the beginning was headache in both kinds of vasculitides, though severe headache was more commonly observed in PCNSV. Although headache is a nonspecific symptom for ARD, CNS vasculitis should be searched for these patients if they present it [17][18][19][20][21][22]. In our study, focal and non-focal neurological manifestations were similar in both groups of vasculitides, except for the focal motor deficit which was significantly more frequent in PCNSV.…”

Section: Discussionmentioning

confidence: 52%

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

et al. 2014

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The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid, pleocytosis and proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients. After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05). Neurological manifestations and neuroimaging findings were similar in both groups of vasculitides, but general symptoms, joint, musculoskeletal, and peripheral neuropathy were preponderant in SCNSV. After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.

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“…Primary manifestation in the nervous system is rare. In these cases inflammatory thickening and fibrosis of the dura mater is usually associated with headaches, whereas cranial nerve lesions, cerebellar symptoms or epileptic seizures occur only rarely (1,4,7). Atypical cases of WG presented as ischemic stroke (10) as subarachnoid hemorrhage (2) or pituitary disease (9).…”

Section: Discussionmentioning

confidence: 99%

“…A number of reports demonstrated secondary spread of WG to the meninges. In these cases inflammatory thickening and fibrosis of the dura mater is usually associated with headaches, whereas cranial nerve lesions, cerebellar symptoms or epileptic seizures occur only rarely (1,4,7). In addition, a case of diffuse involvement of the meninges from Wegener's granulomatosis followed by chronic aseptic meningitis with communicating hydrocephalus and papillary edema was reported (8).…”

Section: Discussionmentioning

confidence: 99%

A 54‐year‐old Woman With a Meningeal Lesion Compressing the Medulla Oblongata

Kołodziej¹,

Benes²,

Nimsky³

et al. 2010

Brain Pathology

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A 54-year-old woman had nausea, vomiting and personality changes since a few weeks. Paresis of the abducens and hypoglossal nerves on the left side, a right sided reduction of the visual field, discrete dysarthri×a and ataxia were diagnosed. Cranial magnetic resonance imaging demonstrated a contrast enhancing circular meningeal lesion of the foramen magnum. Histological examination revealed a granulomatous lesion of the meninges with focal necrosis, vasculitis and neutrophils indicating immune complex reactions. A diagnosis of primary meningeal Wegener's disease was made. Medication with low dose prednisolone led to complete remission of the lesion 1.5 years later.

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Chronischer Kopfschmerz mit Depression durch Pachymeningitis bei Wegener-Granulomatose

Cited by 3 publications

References 16 publications

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

Cranial nerve palsy in Wegener’s granulomatosis – Lessons from clinical cases

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

A 54‐year‐old Woman With a Meningeal Lesion Compressing the Medulla Oblongata

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