Neurosarcoidosis Without Systemic Sarcoidosis. Case Report.

Abstract

A 20-year-old male presented with symptoms of isolated neurosarcoidosis including epilepsy. Magnetic resonance imaging disclosed multiple enhanced right temporal and frontal lesions. Cerebrospinal fluid examination identified mild lymphocytic pleocytosis, and histological examination of a stereotactic brain biopsy specimen demonstrated noncaseating granulomas, so fungal or other inflammatory or granulomatous diseases were excluded. The diagnosis was cerebral sarcoidosis, despite the absence of systemic manifestations. Corticosteroid therapy improved his neurological state and radiological findings. Neurosarcoidosis is a well-recognized occurrence in systemic sarcoidosis, but diagnosis may be difficult in the absence of extracerebral manifestations.

Diagnosis and Treatment in Neurosarcoidosis

“…In 2 cases followed in our clinic, sarcoidosis had been previously diagnosis, and all cases were admitted with various neurological symptoms, such as epileptic seizures, cranial neuropathy, diplopia, dysarthria, trigeminal neuralgia, and hemiparesis. Diagnosis of NS remains challenging, particularly in cases that present with only neurological symptoms, without a previous diagnosis of sarcoidosis (16,17). Cases can be diagnosed by histopathological detection of non-caseified granulomas that accompany clinical and radiological features (18,19).…”

Neurosarcoidosis: Clinical Experience and Diagnostic Pitfalls

“…In our series, only 3 patients were diagnosed with systemic sarcoidosis before the onset of the first neurological symptoms. There are several case reports of patients presenting with isolated intracranial sarcoidosis [5, 6], but no systematic data regarding the frequency of disease onset with neurological symptoms alone [11]. …”

“…Thus, intensive diagnostic workup in cooperation with medical/pulmonary specialists seems to be crucial. However, a number of patients with neurosarcoidosis may present with neurological symptoms before systemic involvement becomes obvious [5, 6]. In the absence of diagnostic markers with sufficient specificity in those cases, a high degree of suspicion in unusual cases of systemic or local CNS inflammation is recommended to overcome the current obstacles in diagnosing this disease.…”

“…Besides a large number of anecdotal reports [3, 4, 5, 6], the literature concerning neurosarcoidosis predominantly consists of retrospective case series from departments of internal medicine. In these studies, patients with systemic sarcoidosis and additional neurological symptoms were analyzed [2, 7, 8, 9, 10].…”

Clinicopathological Conference: A Case of More than a Sprained Ankle

“…The most common neurologic symptom of sarcoidosis is a cranial nerve palsy, 9 and the most common ocular manifestation is uveitis. 10 Macular edema is the most common sight-threatening consequence of sarcoid uveitis.…”