Sarcoidosis Is A Chronic Multi Organ Inflammatory Disease Of Unknown Etiology

Sarcoidosis Is A Chronic Multi Organ Inflammatory Disease Of Unknown Etiology

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Sarcoidosis is a chronic multi-organ inflammatory disease of unknown etiology that manifests as noncaseating granulomas. [1] The lungs are the most common organ involved but sarcoid can affect virtually any organ. The prevalence of sarcoid is more common in African Americans and is more common in women than men. Commonly occurring in adults aged 25-50 years of age. Neurosarcoidosis is seen in approximately 5-10% of sarcoidosis patients with systemic symptoms. Isolated neurosarcoid without systemic involvement is very rare [2]. We present a patient with ataxia, confusion and multiple falls attributed to an unusual presentation of neurosarcoidosis.

Case Presentation:

A 50-year-old African-American male with a history of cerebrovascular accidents leading to right lower extremity residual weakness who ambulates with a walker at baseline was brought into the emergency room by his son due to ataxia, confusion and multiple falls over 3 weeks. The physical exam was remarkable for unsteady posture while sitting upright and standing. Additionally, he had a right hemiplegic gait during ambulation with baseline 4/5 strength in the right lower extremity. Routine blood work including complete blood count, complete metabolic panel, and vitamin B12 came back normal. Computed tomography (CT) head without contrast demonstrated communicating hydrocephalus with cerebral edema and transependymal edema. Magnetic resonance imaging of the brain exhibited hydrocephalus, T2 signal abnormality in the periventricular white matter of the bilateral frontal regions, and bifrontal leptomeningeal enhancement. A lumbar puncture revealed an opening pressure of 19 cm H2O, a lymphocyte predominant white blood cell count of 35 cells/microliter, and a protein count o...

... middle of paper ... lymphadenopathy, and an EBUS guided TBBx revealing non-necrotizing granulomas. Given our patient’s diagnosis of hydrocephalus and subsequent need for high dose corticosteroid treatment, along with an anticipated chronic course, a long steroid taper was the prefered treatment modality.

In conclusion, when confronted by radiologic findings of leptomeningeal enhancement, the differential may be broad. We had a low index of suspicion for infection given the absence of fever, negative cultures and low CSF WBC count. Malignancy was high on our differential, and in the process of ruling it out we identified lymphadenopathy in the thorax. Thereby, we avoided invasive tissue sampling of the leptomeninges and avoided aggressive diagnostic modalities. We also avoided unnecessary placement of ventriculoperitoneal shunting by treating with a steroid course of steroid therapy.

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