Rituximab Treatment for Idiopathic Hypertrophic Pachymeningitis

BACKGROUND AND PURPOSE: Hypertrophic pachymeningitis (HP) is a rare disease caused by autoimmunity in the meninx that causes various neurologic symptoms, including headache, seizures, weakness, paresthesia, and cranial nerve palsies. Although the first-line therapy for HP is steroids, many HP cases are refractory to steroids or recur when the steroids are tapered. Here we report three HP cases that were successfully treated with rituximab (RTX). METHODS: From an institutional cohort recruited from April 2012 to July 2016, three HP cases that were identified to be steroid-refractory were treated with RTX (four weekly doses of 375 mg/m²). Clinical improvement was assessed by the number of relapses of any neurologic symptom and the largest dural thickness in MRI. RESULTS: All three patients were recurrence-free of neurologic symptoms and exhibited prominent decreases in the dural thickness after RTX treatment. No adverse events were observed in the patients. CONCLUSIONS: We suggest RTX as a second-line therapy for steroid-refractory HP. Further studies are warranted to confirm this observation in a larger population and to consider RTX as a first-line therapy.

Idiopathic Hypertrophic Pachymeningitis in the Craniocervical Junction

Idiopathic hypertrophic pachymeningitis (IHP) is a rare disease, and it is characterized by chronic progressive inflammatory fibrosis and thickening of the dura mater with resultant compression of the spinal cord or neural structure without any identifiable cause. It can occur in the intracranial or spinal dura mater alone or as a craniospinal form. The spinal form is rarer than the cranial form and the craniospinal form is extremely rare. We report a rare case of IHP in the craniocervical junction involving both the cranial and spinal dura mater and discuss the diagnosis and management of the disease.

Acute Bilateral Visual Loss with Idiopathic Hypertrophic Pachymeningitis

PURPOSE: To report a case of bilateral optic neuropathy related with idiopathic hypertropic pachymeningitis. CASE SUMMARY: A 66-year old woman presented with acute visual loss that developed 6 days previously in the right eye and 3 days prior in the left eye. During the initial evaluation, her visual acuity was light perception in the right eye and counting fingers at 30 cm in the left eye. A relative afferent papillary defect (RAPD) was noted in the patient's right eye. On brain MRI, the duramater was thickened and enhanced by contrast. The erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) level were increased and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) as positive. Other neurological and medical abnormalities were not found. Under the suspicion of bilateral optic neuropathy due to idiopathic hypertrophic pachymeningitis, the patient was treated with methylprednisolone pulse therapy. Two days after the treatment, the visual acuity was hand movements at 20 cm in the right eye and 0.6 in the left eye. Three months after the treatment, the visual acuity was counting fingers at 20 cm in the right eye, and 0.7 in the left eye. The right eye showed optic disc pallor and diffuse retinal nerve fiber layer (RNFL) defect. The left eye showed a suspicious RNFL defect, and was otherwise normal. CONCLUSIONS: The authors report a rare case of optic neuropathy related with idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis should be considered as one of the various causes of optic neuropathy.

Rapid Recurrence of Spinal Idiopathic Hypertrophic Pachymeningitis: A Case Report

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disorder of the dura mater. Spinal involvement is extremely rare and there are few case reports. We present a 36 year-old female of idiopathic hypertrophic spinal pachymeningitis compressing thoracic spinal cord which showed rapid recurrence.

A Case of Idiopathic Localized Hypertrophic Pachymeningitis Presented with Partial Seizures / 대한간질학회지

Idiopathic hypertrophic pachymeningitis is a clinical disorder caused by a localized or diffuse thickening of the dura mater, with an associated chronic inflammation. This can be diagnosed when there is no evidence of other etiologies such as trauma, infection, tumors, and Wegener's disease. Clinical manifestations are chronic headache with or without neurological manifestations such as cranial nerve palsies, cerebellar ataxia, neuro-ophthalmologic complications, and rarely clinical seizures. We described a patient with simple partial seizures with focal sensory and motor symptoms in the right hand as an initial and the only clinical manifestation, accompanied by a tumor-like lesion in the left parietal convexity on brain MRI. The patient underwent a lesionectomy, and the seizures have been well controlled so far without immunosuppressant treatment.

Idiopathic Hypertrophic Cranial Pachymeningitis: Case Report

Idiopathic hypertrohpic cranial pachymeningitis is rare, and is essentially a diagnosis of exclusion. A 53-year-old man presented with headache and visual loss in the right eye, first experienced a month earlier. MR images depicted a mass in the right cavernous sinus. At T1-weighted imaging, both the mass and the thickened dura mater present in both fromted lobes were isointense, while at while T2-weighted imaging, the signal intensity of both the mass and the dura mater was low. After the injection of contrast medium, pachymeningeal enhancement was observed. We report the radiologic findings in a case of idiopathic hypertrophic cranial pachymeningitis, confirmed surgically and pathologically.

Idiopathic Hypertrophic Spinal Pachymeningitis: A Case Report

Idiopathic hypertrophic pachymeningitis (IHP) is a rare, chronic nonspecific and granulomatous inflammatory disorder of the dura with unknown etiology. The diagnosis can be established by open biopsy and exclusion of all other specific granulomatous and infectious diseases. We report a typical case of spinal IHP occurring in a long segment of cervical and thoracic dura from C6 to T8. The patient was 56-yr-old female, who had been suffered from pain on her upper back and both arms for 3 months and recent onset motor weakness of both legs. During the 9 months of follow-up period, she experienced the improvement of her neurologic symptoms with combined therapy of partial excision and corticosteroid medication. Since early surgical intervention and subsequent pulse ste-roid therapy are mandatory for this disease to avoid irreversible damage of nervous system, the identification of this unique disease entity is essential on frozen diagnosis. A few cases have been reported in Korean literature.

The Therapeutic Effect of Azathioprine in Four Patients with Idiopathic Hypertrophic Pachymeningitis

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disease of unknown origin in which the recurrence is frequently observed despite an initial response to steroid therapy. Four patients, two men and two women aged 63 to 67 years, with severe headaches were evaluated by a brain MRI, and two patients were evaluated by follow up MRI receiving azathioprine therapy. All patients were given initial oral prednisolone 60mg or steroid pulse therapy followed by oral prednisolone and azathioprine therapy. Four patients improved with prednisolone but became steroid depen-dent. Azathioprine therapy permitted a reduction of the corticosteroid which may lead to clinical and radiological improvement. At present, high dose corticosteroid therapy is the treatment of choice, followed by immunosuppressive agents, such as azathioprine, if necessary. Further long-term follow-up studies of these patients are needed to clarify the outcome of this rare disease.

The Therapeutic Effect of Azathioprine in Four Patients with Idiopathic Hypertrophic Pachymeningitis

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disease of unknown origin in which the recurrence is frequently observed despite an initial response to steroid therapy. Four patients, two men and two women aged 63 to 67 years, with severe headaches were evaluated by a brain MRI, and two patients were evaluated by follow up MRI receiving azathioprine therapy. All patients were given initial oral prednisolone 60mg or steroid pulse therapy followed by oral prednisolone and azathioprine therapy. Four patients improved with prednisolone but became steroid depen-dent. Azathioprine therapy permitted a reduction of the corticosteroid which may lead to clinical and radiological improvement. At present, high dose corticosteroid therapy is the treatment of choice, followed by immunosuppressive agents, such as azathioprine, if necessary. Further long-term follow-up studies of these patients are needed to clarify the outcome of this rare disease.