Case ReportCerebral Venous Sinus Thrombosis Secondary to Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report and Review of Literature
Introduction
Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare fibrosing inflammatory process involving the dura mater.1 The crude prevalence of hypertrophic pachymeningitis (HP) is 0.949/10,000, according to a recent nationwide investigation of Japan, and IHCP comprises approximately half of HP.2 Diagnosis of IHCP is based on characteristic neuroimaging findings in addition to pathology and the exclusion of secondary causes. These secondary causes include infection, malignancy, and systematic autoimmune diseases such as vasculitis, lupus erythematosus, or Wegener granulomatosis.3 Because nonspecific clinical manifestations and relatively low sensitivity and specificity of computed tomography (CT), IHCP is likely to be misdiagnosed when first encountered. Currently, there is no consensus regarding treatment for patients with IHCP.4 While cortisol therapy can relieve symptoms in most cases of IHCP, improvement is often transient, and clinical recurrence or steroid dependence is also frequently reported. Additionally, secondary cerebral venous sinus occlusion/cerebral venous sinus thrombosis (CVSO/CVST) increases the incidence of progressive increased intracranial hypertension, vasogenic encephaledema, seizures, and even cerebral hernia in patients with IHCP.5 IHCP can be missed because the sensitivity of normal contrast magnetic resonance imaging (MRI) is relatively low, and performance of venography in patients with IHCP is not regular. Therefore, understanding of this complicated disease and its differetial diagnosis is needed for diagnosis and treatment.
We present a patient with IHCP who developed secondary extensive CSVT, who was first considered to have subarachnoid hemorrhage. The condition of this patient deteriorated rapidly, and she developed fatal secondary intracranial pressure (ICP) and vasogenic encephaledema. We emphasize the importance of imaging for differential diagnosis and management of IHCP together with CVST.
Section snippets
Methods
We experienced a case of CVST secondary to IHCP. Because this is an unusually reported phenomenon, we performed a literature search using a combination search therapy with the following terms: “venous,” “sinus,” “thrombosis,” “occlusion,” “idiopathic,” and “hypertrophic pachymeningitis.” PubMed, Ovid, Web of Science, and ScienceDirect databases were searched. Only English articles that included patients with IHCP who developed secondary CVSO/CVST, which was identified by neuroimaging, were
Previous Cases of IHCP with Secondary CVSO/CVST
A total of 11 reports of 16 patients with IHCP who developed CVSO/CVST were included (Table 1).6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16 Headache occurred in all of these patients. Neurologic deficits occurred in 11 patients, a fall in consciousness occurred in 4 patients, seizures occurred in 2 patients, and papilledema as a sign of intracranial hypertension occurred in 8 patients. Intracranial pressure was reported in 8 patients (ranging from 170–360 mmH2O), 6 of whom had intracranial
Case
A 52-year-old woman presented with worsening dizziness and headache for 4 days and was admitted to our department. She had a history of chronic headache for 4 years, diabetes mellitus for 1 year, and level II hypertension for 17 years. A neurologic examination at presentation was unremarkable, except for neck stiffness. A CT scan showed a diffuse hyperintense lesion along the tentorium, falx, and subdural space (Figure 1A and B). MRV and digital subtraction angiography (DSA) were performed and
Diagnosis and Differential Diagnoses of IHCP with Secondary CVSO/CVST
IHCP is a rare fibrosing inflammatory process involving the dura mater.1 Clinical symptoms of IHCP are variable and nonspecific, which often reflect the mechanical compression of vascular and nerve structures causing secondary ICP or neurologic deficits. Laboratory tests of IHCP often reflect nonspecific chronic intracranial inflammation, which is probably related to autoimmune diseases. Diagnosis of IHCP is based on characteristic neuroimaging findings and histopathology of a dural biopsy.
Conclusion
Our case was 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 usefulness of immunosuppressive agents is promising, but they need further verification.
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2018, World NeurosurgeryCitation Excerpt :The main point of discussion concerned the expected effectiveness of the endoscopic procedure in this kind of hydrocephalus, as one of the most common reasons for its failure depends on an underlying malabsorption component.30 Moreover, venous hypertension, caused by engorgement of the posterior fossa and stenosis of the dural sinuses, might have been an important contributing factor for the hydrocephalus pathogenesis, not necessarily related to complete cerebral sinus thrombosis.31,32 Actually, the pachymeningitis appeared localized in the infratentorial compartment inducing only a compressive effect with overcrowding of the posterior fossa and did not involve directly either the arachnoidal granulations or the dural sinuses.
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Conflict of interest statement: This work was supported by grants from the National Natural Science Foundation of China (81501065), Zhejiang Provincial Natural Science Foundation (LY16H090004), Zhejiang Provincial Traditional Chinese Medicine Science and Technology Plan (2016ZA115), and Zhejiang Provincial Medical and Health Science and Technology Plan (2015KYA091).