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  • Treatment of IgG4-Related Pachymeningitis in a Patient With Steroid Intolerance: The Role of Early Use of Rituximab

    Final Number:

    Authors:
    Vadim Gospodarev BS; Justin Câmara MD; Vikram Chakravarthy MD; Arie Perry MD; Matthew Wood; Robin MD Dietz; Kenneth De Los Reyes MD, MSc; Ravi Raghavan MD

    Study Design:
    Other

    Subject Category:

    Meeting: Congress of Neurological Surgeons 2016 Annual Meeting

    Introduction: IgG4-related pachymeningitis is a serious autoimmune condition that can present with symptoms of mass effect and related focal deficits. According to a general consensus on management of IgG4-related diseases, the first-line therapy is steroids and chemotherapy, such as methotrexate. We describe a patient with IgG4-related pachymeningitis in whom steroid use was contraindicated and methotrexate was ineffective.

    Methods: A 54-year-old woman presented to her neurologist with a three-year history of gradual onset sensorineural hearing loss, right side greater than left, and worsening left sided headaches and dizziness. Given the patient’s increasingly debilitating headaches, dizziness, and lack of diagnosis despite an exhaustive evaluation, she underwent a left temporal craniotomy and biopsy of the dural lesion. Stereotactic navigation was used to plan the craniotomy. A perforator drill was used to make a burr hole, after which a craniotome was used to turn the bone flap. With the aid of stereotactic navigation, the most thickened and abnormal dura was identified.

    Results: Grossly, the dural sample was white, thick and avascular. Histopathological analysis revealed a striking number of IgG4-positive plasma cells (greater than 200 per high-powered field in more than 5 high-powered fields; IgG4/IgG ratio estimated at 58%). Postoperatively, the patient responded well to steroids; however, their continued administration was contraindicated due to her history of gastrointestinal surgery. Methotrexate dose was increased and continued for months with suboptimal response. During the course of her treatment, the patient presented to the emergency department with receptive and expressive aphasia, slurred speech, right-sided neglect, and loss of sensation. After a single infusion of rituximab and initiation of anticonvulsants, her symptoms resolved.

    Conclusions: Although IgG4 disease generally responds well to steroids and chemotherapeutic agents, the clinician must always take into account the patient’s individual circumstances. In this case, early rituximab might have been the most appropriate treatment

    Patient Care: The general consensus on treatment of IgG4-related diseases is that administration of steroids typically produces a favorable response with attenuation of clinicoradiological signs and symptoms. In our case, where high dose steroid use was relatively contraindicated, oral methotrexate was administered with escalating dose up to 25 mg per week; however, as evidenced by the worsening of the patient’s symptoms and eventual episode of aphasia and sensorimotor deficits, response to methotrexate was suboptimal. Rituximab treatment was associated with resolution of neurological symptoms in our patient. Clinicians should thus consider early administration of rituximab treatment in patients with aggressive forms of IgG4-related pachymeningitis.

    Learning Objectives: By the conclusion of this session, participants should be able to: 1) Review the etiology of IgG4-related pachymeningitis. 2) Understand the importance of neurohistopathology in the diagnosis of IgG4-related pachymeningitis. 3) Recognize that the patient's individual circumstances must always be the basis for choosing the most appropriate treatment.

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