Uncommon Presentation of a Common Disease: Neurosarcoidosis as Initial Presentation of Sarcoidosis

Abstract

INTRODUCTION: Sarcoidosis is a systemic granulomatous disease of unknown cause. Pulmonary, Inthoracic lymphnode involvement, skin and eye are the most common organ involvements on presentation. Neurosarcoidosis is present in 25 % of patients with sarcoidosis but is asymptomatic in majority of patients. Only 10 % of patients with sarcoidosis have clinical neurosarcoidosis. Out of these patientaTMs only a few present with neurological symptoms on initial presentation. Symptoms range from nonspecific symptoms like headache, visual disturbances and lethargy to more severe and potential life threatening presentations like seizures, encephalitis, meningitis and cranial nerve palsies. CASE PRESENTATION: 68 year old Caucasian male with history lupus nephritis and coronary artery disease was seen several times over a course of 6-8 months. On initial presentation patient had daily headaches and vertigo which were treated as migraine and Positional vertigo. Over next couple of months patient developed mild cognitive impairment with visual hallucinations, vertigo and headaches. Diagnostic workup including MRI was negative except for mild cognitive decline on neuropsychological testing and vestibular neuropathy. Over next 4-6 months patient experienced significant neurological decline with moderate to severe cognitive decline, worsening vertigo, frequent falls and continued headaches. Repeat MRI showed extensive Pia Mater enhancement diffusely and compensated hydrocephalus . CSF showed lymphocytic predominance. CT showed enlarged lymph nodes in the chest and retroperitoneum. PET scan showed hypermetabolic activity in the chest and abdomen and a right parietal scalp lesion. Scalp lesion biopsy showed noncaseating granulomas with vasculopathy consistent with sarcoidosis. Patient was diagnosed with neurosarcoidosis and was started given loading dose of intravenous steroids followed by prednisolone 60 mg/day. DISCUSSION: Neurosarcoidosis is difficult to diagnose as initial presentation of sarcoidosis as it masquerades lymphoma, viral encephalitis/meningitis and vasculitis. Steroids are mainstay of treatment initially. Immunosuppressive agents are useful for patient not responding to steroids or to reduce the dose of steroids. In patients not responding to medications radiation therapy has been tried. Prognosis of patients with neurosarcoidosis is poor with 10 % mortality rate. CONCLUSIONS: In a patient with worsening neurological symptoms over course of time neurosarcoidosis should be considered in differential diagnosis. Delay in diagnosis and treatment might lead to worse prognosis.