To report an uncommon presentation of Neurosarcoidosis without other systemic clinical features of sarcoidosis.
Sarcoidosis presenting with isolated central nervous system clinical features is very rare and could be misdiagnosed, leading to poor clinical outcomes. To our knowledge, a similar clinical diagnostic dilemma is not reported. Timely diagnosis and treatment allow early treatment, improving prognosis and reducing complications and hospitalization time.
37-year-old Afro-American male with a history of sickle cell trait, longstanding paresthesias, chronic migraine, intermittent horizontal diplopia for two years presented with worsening headaches, blurred vision with intermittent horizontal diplopia for eight months, lost 20 pounds with insomnia had a nonfocal neurological examination.
Initial MRI brain with and without contrast done two years prior to hospital admission was unremarkable. MRI brain at the time of presentation showed extensive nodular leptomeningeal enhancement involving the basilar cisterns, suprasellar cisterns, both cerebral hemispheres, and, to a lesser extent, the cerebellum along with moderate hydrocephalus and trans ependymal flow of CSF. MRI of the cervical spine showed scattered nodular leptomeningeal enhancement within the cervical and upper thoracic region, similar to findings demonstrated within the MRI of the brain. CSF: Elevated opening pressure at 29 cm. Elevated IgG index and protein at 56, ACE levels 5.2. Cytology was negative for malignancy. No signs of CSF infection. Elevated ESR at 38. Transbronchial biopsy showed noncaseating granuloma suggestive of sarcoidosis. Gamma interferon release and QuantiFERON were negative. Hospital course was complicated by cognitive decline, hydrocephalus, and cerebral edema, which improved with ventriculoperitoneal shunt, hallucinations, and hyperglycemia due to steroid therapy. Steroids were switched to methotrexate with clinical improvement.
Extensive diagnostic investigations and aggressive treatment of refractory cases decrease morbidity and mortality associated with Neurosarcoidosis. Clinical trials would determine optimal treatment strategies.