Headache as presenting symptom of neurosarcoidosis

Abstract

Headache may occur in neurosarcoidosis and diagnostic criteria are given in the International Classification of Headache Disorders (ICHD-II). We present a case series of patients suffering from neurosarcoidosis in whom headache was the presenting symptom. The aim of the present study was to analyze the possible clinical presentations and the corresponding cerebral lesions in a retrospective chart review of patients suffering from neurosarcoidosis in whom headache was the presenting symptom. Medical records and data of six patients were analyzed. The possible diagnoses of headache forms included in ICDH-II, and in particular the correspondence with the criteria for "headache attributed to neurosarcoidosis", as well as neuroimaging findings were evaluated in each patient. The ICHD-II criteria were fulfilled in all the six patients. As for as clinical presentation, in three patients (50%), headache had the clinical characteristics of Tolosa-Hunt syndrome, with evidence of a lesion into the cavernous sinus. In the remaining three cases headache was the only neurological symptom found in association with systemic features of sarcoidosis, and had the clinical features of tension-type headache. Our findings confirm that the clinical features of headache in patients with this disorder may have different presentations, which depend on neuropathologic involvement. Thus, a detailed neuroimaging study and CSF evaluation are needed to confirm diagnosis, particularly in patients with no sign of systemic sarcoidosis or in those in whom head pain may mimic a primary headache syndrome.