A case of sarcoidosis of the central nervous system and orbita.

Metin Mercan,Yahya Karaman,Hayrunnisa Bolay,Aslı Akyol

doi:10.1155/2015/403459

Abstract

Sarcoidosis is a multisystemic disease characterized by granulomatous inflammation. Lung or lymph node involvement is common. We present a rare case of sarcoidosis that began with orbital involvement, and a month later, due to insufficient treatment, it involved the central nervous system. A 49-year-old female patient began suffering from swelling in her right eye, redness, ptosis, and limited eye movements two months ago. Gadolinium-enhanced orbital magnetic resonance imaging showed thickening of the lacrimal gland and the right medial rectus muscle. After three weeks of local antibiotic and steroid treatments, her symptoms were resolved. One month ago, the patient reported sudden weakness in her right arm and leg. After laboratory tests and imaging studies, the patient was diagnosed with probable neurosarcoidosis using the Zajicek criteria and treated with prednisone (1 mg/kg/day). Although sarcoidosis frequently presents with lung and lymph node involvement, it is rarely accompanied by orbital involvement. Patients with orbital symptoms may receive a late diagnosis and insufficient central nervous system treatment. Involvement of the central nervous system in sarcoidosis leads to high morbidity and mortality rates. Therefore, early diagnosis and treatment are very important.

Highlights

Sarcoidosis is a chronic multisystemic disease with progressive formation of noncaseating granulomas that can develop in organs, including the lungs, lymph nodes, eyes, and skin [1]
Ocular involvement typically presents as uveitis and, rarely, as orbital involvement affecting the lacrimal gland and extraocular muscles
When central nervous system involvement is detected, it commonly presents as cranial neuropathy, aseptic meningitis, pituitary dysfunction, or cognitive impairment

Summary

Introduction

Sarcoidosis is a chronic multisystemic disease with progressive formation of noncaseating granulomas that can develop in organs, including the lungs, lymph nodes, eyes, and skin [1]. While symptoms may appear at any age, peak occurrence is between 30 and 40 years of age [2]. Central nervous system involvement (neurosarcoidosis) occurs in 5–15% of cases. Patients with neurosarcoidosis present with neurologic symptoms, including headaches, visual impairment, diplopia, ataxia, motor deficits, seizures, and cognitive decline [3, 4]. Ocular involvement typically presents as uveitis and, rarely, as orbital involvement affecting the lacrimal gland and extraocular muscles. Isolated sarcoidosis unaccompanied by systemic involvement is rare and difficult to diagnosis [5, 6]

Case Presentation

Conclusions