Uncommon findings in idiopathic hypertrophic cranial pachymeningitis

Abstract

In the patients here reported, absence of cranial nerve impairment, seizures, MRI findings resembling chronic subdural hematoma, and association with polineuropathy were unusual findings of IHCP. Moreover, the type of inflammatory infiltrate, lacking in previous reported cases, suggests a probable pathogenetic role for cell-mediated immunity of unknown origin.

Imaging Features of Meningeal Inflammatory Myofibroblastic Tumor

“…15,18,[21][22][23][24] Particularly, the nonenhancing central linear area in the enhancing thickened dura of diffuse IMT in the present study had already been reported as a feature of idiopathic hypertrophic pachymeningitis, representing thickened dura with extensive fibrosis and peripheral inflammatory cell infiltration. 21,25 In addition to similar clinical symptoms and laboratory findings, these similar radiologic features support the argument that both meningeal IMT and idiopathic hypertrophic pachymeningitis are part of a spectrum rather than discrete disorders, even if there were some pathologic differences between the 2 disease entities. 10,21,23,25 As for treatment, surgical resection with adjuvant steroid medication is the treatment of choice for meningeal IMT.…”

“…21,25 In addition to similar clinical symptoms and laboratory findings, these similar radiologic features support the argument that both meningeal IMT and idiopathic hypertrophic pachymeningitis are part of a spectrum rather than discrete disorders, even if there were some pathologic differences between the 2 disease entities. 10,21,23,25 As for treatment, surgical resection with adjuvant steroid medication is the treatment of choice for meningeal IMT. 26,27 In our study, there was no recurrence in any patient with localized meningeal IMT following surgical resection with steroid treatment, but all the patients with unresectable diffuse IMT showed various types of recurrence despite the use of multimodal treatments.…”

Idiopathic Hypertrophic Cranial Pachymeningitis Misdiagnosed as Acute Subtentorial Hematoma

“…The affected areas can be detected clearly by gadolinium-enhanced MRI studies. Other than these clinical findings, diffuse or focal hypertrophy of the meninges, brain edema, cavernous sinus thrombosis, venous congestion, secondary white matter changes, hydrocephalus, and intracranial hemorrhages may also be seen on a MRI 9) . The "dura tail sign," which is a characteristic MRI finding of meningiomas in T1WI, may also be observed 10) .…”

“…Generally, the presenting symptoms include headaches, cranial nerve palsies, cerebellar dysfunction, and seizures. The laboratory findings in patients with IHCP include mild-to-moderate elevation of C-reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) 1,8,9) . Cerebrospinal fluid (CSF) studies in most cases show aseptic inflammatory changes 3) .…”

Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

“…Nur selten ist der intrakraniale Druck erhöht, wodurch zusätzlich Kopfschmerzen ausgelöst werden kön-nen [12,38]. Die Zephalgien sind in der Regel kontinuierlich vorhanden, dumpf-drückend, von starker Intensität und im Verlauf der Erkrankung oft progredient [5,11,19,20,37,39,45]. Seltener sind die Kopfschmerzen stechend oder haben pulsierenden Charakter.…”