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 TolosaHunt 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.

Common Clinical and Molecular Pathways between Migraine and Sarcoidosis

“…However, the clinical presentation can be different; some authors have indeed found that headache had the clinical features of Tolosa-Hunt syndrome in 50% of NS patients in their study, citing as evidence a lesion in the cavernous sinus [47]. The lesion could be associated with cranial neuropathy, as the most common clinical presentation of NS, present in approximately 55% of patients and manifests usually with the characteristics of painful paralysis of the third cranial nerve, although any nerve of the cavernous sinus can be affected [46].…”

“…In a meta-analysis that analyzed 29 articles and included 1088 patients diagnosed between 1965 and 2015, headache was the second most common manifestation, affecting 31% (95% CI: 28-35%) patients [46]. Headache is usually characterized by a tension-like form [47] and could be classified among the secondary headaches; the 7.3.1 group of the International Classification of Headache (ICHD-3) states that "headache attributed to non-vascular intracranial disorders/noninfectious inflammatory intracranial disease/headache attributed to NS" and the diagnosis is based on the temporal relationship between the diagnosis of NS and headache onset [33].…”

“…The lesion could be associated with cranial neuropathy, as the most common clinical presentation of NS, present in approximately 55% of patients and manifests usually with the characteristics of painful paralysis of the third cranial nerve, although any nerve of the cavernous sinus can be affected [46]. Headache, therefore, can manifest differently according to the neuropathologic involvement, and a detailed imaging study is recommended along with a cerebrospinal fluid (CSF) examination to achieve the correct diagnosis and treatment approach [47]. Biopsy is less frequently needed; it is most often required in isolated forms where a radiological picture and a liquor analysis are not conclusive, or in those cases that mimic tumors and a correct diagnosis is needed before starting an appropriate therapy (corticosteroid treatment for sarcoid lesions, surgery or chemotherapy for tumors) [48].…”

A Rare Case of Steroid-Resistant Neurosarcoidosis of the Cavernous Sinus With Optic Neuropathy: A Case Report

“…In 7 of these cases, corticosteroid monotherapy alone was shown to be effective in the resolution of symptoms. [4][5][6][7][8] In one of these patients, early combination therapy with corticosteroid plus methotrexate therapy was used as first-line treatment and led to rapid improvement within a few days and near-complete clinical resolution of symptoms at 3 months. 9 Given the lack of clinical trial data, the treatment of neurosarcoidosis is largely based on observations from case series and case reports.…”