Disorders of the Pleura |

EGPA Presenting as a Movement Disorder FREE TO VIEW

Donovan Mabe, MD; Nikunj Bhatt, MD; Michael Perkins, MD
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Walter Reed National Military Medical Center, Bethesda, MD

Chest. 2014;146(4_MeetingAbstracts):457A. doi:10.1378/chest.1988928
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Eosinophillic Granulomatosis with Polyangiitis (EGPA) is a small-to-medium sized vessel vasculitis that affects multiple organ systems. In addition to severe asthma, EGPA frequently presents with cutaneous and peripheral nerve manifestations. Among peripheral nerve manifestations, mononeuritis multiplex predominates. We present a case of EGPA presenting with a movement disorder.

CASE PRESENTATION: A 22 year-old man with a medical history significant for childhood asthma and occasional migraines presented with blurry vision, mild headache, and uncontrolled movements. He reported 1-2 weeks of cough, wheezing, and dyspnea. He also reported numerous episodes of protracted wheeze, cough and dyspnea, at times associated with infiltrates on CXR over the previous 3 years. The patient was noted to have myoclonic jerking and choreiform movements in addition to end expiratory wheezes on pulmonary auscultation. A chest CT revealed multilobar groundglass opacities with areas of tree-in-bud nodularity. Two trials of antibiotics (Augmentin followed by Azitrhomycin) failed to relieve his pulmonary symptoms. Further evaluation revealed eosinophilia (9.6%), elevated IgE (620.9) and severe left maxillary sinusitis. Pulmonary function tests deomonstrated severe obstruction. Bronchoalveolar lavage produced negative cultures, but a gram stain did reveal numerous eosinophils. Corticosteroids were started with complete resolution of dyspnea, choreiform movements, myoclonic jerks, and blurry vision. Additionally, PFTs normalized, and the CXR infiltrates and maxillary sinusitis completely resolved. He was given a diagnosis of EGPA based on his multiple supportive clinical features.

DISCUSSION: In this case we discuss an atypical presentation of EGPA, mimicking a movement disorder with myoclonic-appearing jerks and choreoform movements. Neurological involvement is common with EGPA, but typically presents as mononeuritis multiplex or polyneuropathy. This patient met American College Rheumatology diagnostic criteria for EGPA and responded promptly to corticosteroid administration. This case highlights the variable nature of EGPA, specifically with the neurological findings.

CONCLUSIONS: We report a unique case of EGPA presenting as a movement disorder. The multi-system and variable presentation of this disease frequently presents a diagnostic dilemma to clinicians. Awareness of atypical cases may lead to earlier diagnosis and initiation of appropriate therapy.

Reference #1: Masi, A, et al. Arthritis Rheum. 1990;33(8):1094

Reference #2: Hattori, N, et al. Brain. 1999 Mar;122 ( Pt 3):427-39.

Reference #3: Comarmond, C, et al. Arthritis Rheum. 2013;65(1):270

DISCLOSURE: The following authors have nothing to disclose: Donovan Mabe, Nikunj Bhatt, Michael Perkins

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