Focal hypertrophic cranial pachymeningitis associated with temporal arteritis

Marano, E; D’Armiento, Francesco Paolo; Scarano, Valentina; Tortora, Fabio; Mignogna, Chiara; Briganti, Francesco

doi:10.1007/s00415-003-0914-6

Cited by 17 publications

(13 citation statements)

References 8 publications

(8 reference statements)

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“…The causes of pachymeningitis are, among others, vasculitides, including primary9 10 and secondary CNS vasculitis such as temporal arteritis 6 7 8. An alternative classification distinguishes between systemic—for example, Wegener's disease,3—and local organ related vasculitides.…”

Section: Discussionmentioning

confidence: 99%

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Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology

Brüggemann

Gottschalk

Holl‐Ulrich

et al. 2010

Journal of Neurology, Neurosurgery & Psychiatry

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Cranial pachymeningitis is a poorly understood syndrome, defined by leptomeningeal thickening and typical gadolinium enhanced MRI. The heterogeneous clinical and aetiological features of five patients with both focal and diffuse pachymeningitis are presented. The initial symptoms included headache (n=3), sensory Jackson seizures (n=1), hemiparesis (n=1), episodes of short lasting hemiataxia (n=1), hemihypaesthesia (n=1), aphasia (n=1) and confusion (n=2). MRI scans revealed focal (n=3) or diffuse (n=2) leptomeningeal gadolinium enhancement and cortical swelling (n=4). In addition, one case presented with a subarachnoid and a second with an intracerebral haemorraghe. CSF findings were variable and showed clear lymphomonocytic pleocytosis in 3/5 cases. Infectious diseases were extensively excluded in all cases. Leptomeningeal biopsies of two cases revealed perivascular inflammation, indicating central nervous system vasculitis. In the cases presented, pachymeningitis was caused by primary central nervous system vasculitis (n=2) and rheumatoid arthritis (n=2). In one case, the cause remained unclear.

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Section: Discussionmentioning

confidence: 99%

“…Pachymeningitis should be considered as a neurological syndrome with heterogeneous aetiology attributed to one of the following causative groups: (a) PCNSV,9 10 (b) secondary CNS vasculitis,3 6 7 8 (c) general systemic inflammation, especially tuberculosis,1 2 (d) neoplastic disorders and (e) idiopathic causes 11…”

Section: Discussionmentioning

confidence: 99%

Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology

Brüggemann

Gottschalk

Holl‐Ulrich

et al. 2010

Journal of Neurology, Neurosurgery & Psychiatry

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“…This difference in headache origin results in different clinical characteristics. A vascular headache is commonly characterized by throbbing and pulsating pain in the scalp, especially in the swollen superficial temporal artery (1,9), while a meningeal headache has no tenderness in the temporal artery itself (4,6). Thus, clinicians should note that the absence of tenderness in the temporal arteries does not necessarily rule out the diagnosis of temporal arteritis, and in such cases, the headache might result from hypertrophic pachymeningitis.…”

Section: Discussionmentioning

confidence: 99%

“…It sometimes occurs concurrently with disorders such as infections, autoimmune diseases and neoplasms. However, few case reports have described hypertrophic pachymeningitis associated with temporal arteritis (3)(4)(5)(6)(7).…”

Section: Introductionmentioning

confidence: 99%

Hypertrophic Pachymeningitis as a Potential Cause of Headache Associated with Temporal Arteritis

et al. 2016

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We herein describe a rare case of temporal arteritis associated with hypertrophic pachymeningitis. An 81-year-old man presented with a right temporal headache that had persisted for one month. A right superficial temporal artery biopsy revealed intimal hypertrophy with increased elastic fibers, consistent with temporal arteritis. Brain MRI using gadolinium enhancement showed thickened dura mater on the right frontal and temporal lobes, which led to the diagnosis of hypertrophic pachymeningitis. Intravenous methylprednisolone and oral prednisolone improved the patient's symptoms. According to our findings, hypertrophic pachymeningitis may be a potential cause of an ipsilateral temporal headache associated with temporal arteritis.

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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

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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Focal hypertrophic cranial pachymeningitis associated with temporal arteritis

Cited by 17 publications

References 8 publications

Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology

Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology

Hypertrophic Pachymeningitis as a Potential Cause of Headache Associated with Temporal Arteritis

Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

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