[A clinical analysis of idiopathic hypertrophic cranial pachymeningitis with a nodular space-occupying effect].
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 neutrophil 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.
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China.
Hailing
H
Zhang
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China.
Qinwen
Q
Dong
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China.
Bin
B
Xiong
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China.
Dongdong
D
Song
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China.
Jianguo
J
Liu
Department of Neurology, Navy Clinical Department, the Second Military Medical University, Beijing 100048, China. Email: bjqxk@sina.com.
Xiaokun
X
Qi
D000328:Adult; D003952:Diagnostic Imaging; D006801:Humans; D006984:Hypertrophy / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008581:Meningitis / Q000175:diagnosis* / Q000473:pathology*; D008875:Middle Aged; D012189:Retrospective Studies