Collet-Sicard syndrome associated with probable intracranial vasculitic arteriopathy: a case report

Abstract

Collet–Sicard syndrome is unilateral palsy of the glossopharyngeal, vagus, accessory, and hypoglossal nerves. It is most commonly caused by neoplastic, traumatic, or vascular lesions at the skull base. To our knowledge, its association with intracranial vasculitis has not been reported previously. A 35-year-old Sri Lankan woman presented with a 3-month history of dysphagia, dysphonia, and nasal dysarthria. She had had a left-sided cerebral infarction 1 year earlier. Examination showed right-sided cranial nerves IX, X, XI, and XII palsies, consistent with Collet–Sicard syndrome. Contrast-enhanced computed tomography and magnetic resonance imaging of the skull base were normal, with no evidence of jugular foramen pathology, skull-base osteomyelitis, venous thrombosis, or basal meningitis. Cerebrospinal fluid analysis revealed mild lymphocytic pleocytosis and elevated protein. Extensive investigations excluded malignancy, infection, sarcoidosis, IgG4-related disease, and systemic vasculitis. Review of prior neuroimaging showed a corpus callosal infarction. Because of this unusual stroke location, digital subtraction angiography was performed which demonstrated multifocal intracranial arterial stenoses and luminal irregularities suggestive of possible intracranial vasculitis. In the absence of another explanation, we hypothesised that the lower cranial neuropathies may have resulted from vasculitic involvement of the vasa nervorum. She was treated with corticosteroids, rituximab, azathioprine, and aspirin. Despite treatment, severe bulbar dysfunction persisted, and she later died from aspiration pneumonia. This case highlights a rare association between Collet–Sicard syndrome and intracranial vasculitic arteriopathy. When structural skull-base lesions are absent, vasculitic neuropathy should be considered as a potential cause of multiple lower cranial neuropathies. Corpus callosal infarction, an uncommon stroke pattern, should prompt investigation for unusual vascular aetiologies including central nervous system vasculitis.