Cerebral Venous Sinus Thrombosis Secondary to Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report and Review of Literature.

Abstract

Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare fibrosing inflammatory process involving the dura mater. Currently, there is no consensus on the treatments for IHCP, and the usefulness of immunosuppressive agents as a first-line option remains controversial. Cerebral venous sinus occlusion (CVSO) and cerebral venous sinus thrombosis (CVST) secondary to IHCP, which may cause progressive intracranial hypertension and venous obstructive parenchymal lesions, make the diagnosis and treatment of IHCP more complicated. We present a case of IHCP. We also review previous cases of IHCP with secondary CVSO/CVST and then summarize the clinical characteristics of these patients. A 52-year-old female patient with IHCP developed secondary CVST. She had a severe headache with a hyperintense lesion on computed tomography, which was considered as subarachnoid hemorrhage. Lumbar tapping with a cerebrospinal fluid test, in addition to gadolinium contrast-enhanced magnetic resonance imaging, suggested IHCP. Secondary CVST was identified by digital subtraction angiography and magnetic resonance venography. Fatal intracranial hypertension with severe neurologic deficits occurred, despite mannitol, furosemide, and corticoid therapy. After administration of intravenous pulse cyclophosphamide, she obtained complete remission. We experienced a patient with CVST secondary to IHCP, who was successfully treated with cyclophosphamide pulse therapy. Because IHCP with secondary venous obstruction has various differential diagnoses, venography is necessary to avoid misdiagnosis. The use of immunosuppressive agents may be promising but needs further verification.