Idiopathic Cranial Pachymeningoencephalitis Focally Affecting the Parietal Dura Mater and Adjacent Brain Parenchyma: Case Report

Nishizaki, Takafumi; Iwamoto, Fuminori; Uesugi, Seiji; Akimura, Tatsuo; Yamashita, Katsuhiro; Ito, Hiroki

doi:10.1097/00006123-199704000-00037

Cited by 33 publications

(27 citation statements)

References 14 publications

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“…Intracranial hypertrophic pachymeningitis may involve the skull bones, the pericranial soft tissue and, rarely, the cerebral parenchyma (when inflammatory cells infiltrate through the Virchow-Robin spaces) (9). Pachymeningeal thickening may be diffuse or focal/nodular.…”

Section: Discussionmentioning

confidence: 99%

“…Such external stenosis at the base of the skull may also lead to occlusion of the internal carotid artery, resulting in cortical deficits (15). These lesions have a mass effect, but the inflammatory perivascular infiltration also plays an important part in the cortical irritative symptomatology (9,16). Intracranial hypertrophic pachymeningitis may occasionally also lead to occlusion of the venous sinus and obstructive hydrocephalus (15).…”

Section: Discussionmentioning

confidence: 99%

“…Headache, progressive affection of multiple cranial nerves and ataxia are the most common symptoms, and are manifested in about 90 %, 60 % and 30 % of the cases, respectively (4,5,8). Epileptic seizures also occur (9). The headache may be focal or diffuse and may be the only symptom for a number of years (10,11).…”

Section: Down-titration This Treatment Led To Regression Of Both Symmentioning

confidence: 99%

“…MRI findings are characteristic, and show various degrees of the lesion's inflammatory pattern (5,8). T1-weighted images show iso-or hypointense thickened dura (6,8,9,13) with intense enhancement after injection of paramagnetic contrast medium due to inflammatory reaction in the pachymeninges. The dura mater appears hypointense in T2-weighted images, in some cases surrounded by a hyperintense rim of the lesion (8,9,13,19).…”

Section: Case Reportmentioning

confidence: 99%

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En mann i 40-årene med hodepine og tåkesyn

Bjørke¹,

Wahl²,

Kalstad³

2012

Tidsskriftet

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Headache with increased cell number in the spinal fluid, fever and meningeal thickening immediately gives rise to associations with bacterial meningitis. When there is sub-acute onset of symptoms and an atypical course of disease with poor response to antibiotics, however, differential diagnostic possibilities should be considered. The patient is man in his 40s with severe complications of a rare meningeal condition.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Down-titration This Treatment Led To Regression Of Both Symmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

See 2 more Smart Citations

En mann i 40-årene med hodepine og tåkesyn

Bjørke¹,

Wahl²,

Kalstad³

2012

Tidsskriftet

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“…Steroid therapy represents the first choice and is often effective for IHSP [12,18,19]. Besides steroid treatment, radiotherapy [3], azathioprine therapy [1,12,15], and cyclophosphamide therapy [15] have been used.…”

Section: Discussionmentioning

confidence: 99%

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

et al. 2011

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Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a comparatively rare disease characterized by hypertrophic inflammation of the dura mater and clinical symptoms that progress from local pain to myelopathy. We report a case of IHSP followed up for 20 years in a 46-year-old man. Expansive laminoplasty was performed in 1991, and this case has been previously reported by a co-author. After 17 years, the patient's gait disturbance returned. Physical examination and imaging confirmed IHSP that had developed into syringomyelia at the T2-L1 conus level. This case was diagnosed as adhesive spinal arachnoiditis due to pachymeningitis caused by syringomyelia. T1-T4 laminectomy, a syringo-subarachnoid shunt (S-S shunt), and L2-L3 laminectomy were performed. The patient again developed dysesthesia and gait disturbance 3 years after the second operation. Most reports of IHSP have limited their focus to short-term follow-up after initial treatment with no long-term results. At present, there are only five reports referring to long-term results of greater than 5 years. All but one case needed additional surgery. To the best of our knowledge, this is the first case in which syringomyelia occurred in a patient with IHSP. It is important to note that syringomyelia may be a cause of symptom recrudescence during long-term follow-up in IHSP patients.

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