A clinical analysis of idiopathic hypertrophic cranial pachymeningitis with a nodular space-occupying effect / 中华内科杂志

Objective To explore the clinical features,neuroimaging and histopathological findings in patients with idiopathic hypertrophic cranial pachymeningitis (IHCP) with a nodular space occupying effect.Methods Four IHCP cases with a nodular space occupying effect diagnosed in our hospital were retrospectively studied.Results All the 4 patients were men with a mean onset age of 40.25 (33 ～ 50) years old.They all had long disease duration and relapses.The common symptoms of IHCP were chronic headache,multiple cranial nerve palsies and epileptic seizures.CT and MRI of the brain revealed prominent dural partial thickening,which indicated a mass or nodular space occupying effect that mimicked intracranial tumour-like meningioma.The histopathological findings of dura in 2 cases revealed connective tissue proliferation,scattered neutrophile granulocytes and plasmacytes infiltration.Combination therapy of corticosteroid or/and immunosuppressive drugs was effective for the IHCP patients.Conclusions IHCP patients with a nodular space occupying effect usually onset with chronic headache and are often recurrent.The combination therapy of corticosteroid or/and immunosuppressive drugs is effective.The image of the brain presents prominent dural partial thickening,indicating a mass or nodular space occupying effect,which often lead to confusion with intracranial tumours or granulomatosis.

Right Superior Oblique Paralysis associated with Idopathic Hypertropic Cranial Pachymeningitis

PURPOSE: Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disase, which causes chronic progressive inflammation and thickening of the basal dura mater. We report a case of IHCP associated with superior oblique paralysis and present a review of the literature. METHODS: An 8-year-old boy presented with binocular diplopia and left side head tilting. Suspecting right superior oblique muscle paralysis, an alternating prism test, head tilt test, fundus examinations, neurologic examination, and brain MRI were performed. RESULTS: The brain MRI revealed abnormal enhancement of the right tentorium in the course of the right fourth cranial nerve, leading to a diagnosis of IHCP with paralytic strabismus and the patient was treated with systemic steroid therapy. CONCLUSIONS: We report a case of IHCP with right superior oblique paralysis. Patients with recent onset paralytic strabismus require appropriate neurolgic and neuroimaging examinations.

A Case of Idiopathic Hypertrophic Cranial Pachymeningitis Associated with Tolosa-Hunt Syndrome

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare disease, which causes chronic progressive inflammation and thickening of the basal dura mater and produces multiple cranial neuropathies, headache, ataxia, and seizure. A 40 year-old man presented with a continuous, diffuse, dull headache with left periorbital pain, exophthalmos, ptosis and loss of visual perception. Brain MRI revealed thickened, highly enhanced pachymeninges in all supratentorial areas in association with the left cavernous sinus. A subsequent meningeal biopsy showed non-specific chronic inflammations of the pachymeninges. The patient dramatically responded to steroid therapy. To our knowledge, this is an extremely rare case of IHCP associated with Tolosa-Hunt syndrome. (J Korean Neurol Assoc 19(1):56~59, 2001

Clinical,imaging and pathologic features of hypertrophic cranial pachymeningitis / 临床神经病学杂志

Objective To explore the clinical,imaging and pathologic features of hypertrophic cranial pachymeningitis (HCP).Methods The clinical data of one HCP patient who treated in our hospital and other 77 HCP patients from literatures were analyzed retrospectively.Results The clinical manifestation of all the 78 HCP patients had chronic headache,multiple cranial nerves impairment.The secondary was psychiatric disorder(10.3%),ataxia(9.0%) and seizure disorder(6.4%).Hemiplegia,menorrhea and galactosis were found few.Headache was the first onset symptom in the 74 cases (94.9%).HCP was often misdiagnosed subarachnoid hemorrhage,hypotensive cranial pressure headache and cerebral venous sinus thrombosis in early stage.MRI demonstrated local or diffused thickened dura,especially in cerebral falx and/or tentorium of cerebellum,which could be enhanced through reinforced scanning.Pathological evidence indicated an obvious proliferation of dura fiber tissue accompanied with inflammatory cells infiltration.Corticosteroid was effective to all the cases.Conclusions The clinical manifestation of HCP was multiplicity,but it mainly was chronic headache and multiple cranial nerves impairment.MRI demonstrates local or diffused thickened dura especially in cerebral falx and/or tentorium of cerebellum.MRI has an important significance for diagnosis.

Idiopathic hypertrophic cranial pachymeningitis: Report of 2 cases & Review of literatures

Idiopathic hypertrophic cranial pachymeningitis(IHCP) is a rare chronic pro gressive fibrosing inflammation of pachymeninges of unknown origin. Since the spreading of CT and MRI, there has been a few cases of IHCP have been reported. We describe two patients of IHCP with brain parenchymal involvement presented as epilepsia partialis continua which has not been described as a symptom of IHCP and review the previous reported literatures. IHCP commonly presents headache, multiple cranial nerve palsy, ataxia, and sometimes seizure. In many cases, the CSF finding is noninfectious inflammation, and the erythrocyte sedimentation rate is elevated. The brain MRI is the best noninvasive tool for diagnosis of IHCP and shows diffuse thickening and enhancing dura, especially posterior fossa. IHCP responds steroid initially but recurs frequently and progresses chronically.

Characteristic features of clinical manifestation, imaging and pathology of hypertrophic cranial pachymeningitis (report of 1 case) / 临床神经病学杂志

Objective To determine the characteristic features of clinical manifestation, imaging and pathology in the patients with hypertrophic cranial pachymeningitis (HCP). Methods All clinical data of 1 patient with HCP were studied retrospectively. Results The patient presented with heavy headache, progressive visual acuity decreased and hoarse voice. Brain MRI displayed abnormal strengthening signals in cerebral dura mater, especially in cerebral falx and tentorium of cerebellum. Pathological examination demonstrated chronic inflammation changes including numerous plasmocytes infiltration, accrementition and glassy degeneration of fibrous tissue, formation of granulation tissue. Therapy with corticosteroid hormone showed good effect for this patient.Conclusions HCP mainly presents with headache and paralysis of multiple cranial nerves. Distinctive sign on brain MRI is strengthening signal in cerebral dura. Chronic inflammation is the pathological change of this disease and pathological examination plays important role in diagnosis of HCP.