Citations

A 23-year-old gentleman presented to the emergency department with new onset neurological dysfunction and painful oral and genital ulcers. Biopsies of the lesions noted non-specific inflammatory changes. MRI of the brain revealed a large mass-like lesion involving the pons, medulla, midbrain and surrounding cerebral tissue with T2 signal enhancement that was not amenable for biopsy. Both the ulcer biopsy results and the MRI findings could be attributed to either central nervous system (CNS) lymphoma or CNS Behçet's disease. A diagnosis of CNS Behçet's disease was thought to be most likely based on the clinical presentation and MRI findings. He was treated with high-dose immunosuppression and made a significant recovery.

Abstract BACKGROUND: Ventral spinal epidural meningeal cysts are rare entities for which the pathogenesis is poorly understood. OBJECTIVE: We present the clinical, radiographic, surgical, and pathologic findings of 4 patients with extensive ventral spinal epidural meningeal cysts and review the relevant literature. In addition, we discuss a suspected mechanism for pathogenesis. METHODS: Four patients with anterior spinal epidural meningeal cysts are retrospectively reviewed. RESULTS: Ventral spinal epidural meningeal cysts are often large, extending on average from C2 to L1 in our series. Patients typically present with a prolonged course of symptoms and signs, including segmental muscle weakness and atrophy, subtle myelopathy, mild to moderate spinal pain, and headache. Histopathologic analysis of the cyst wall demonstrates collagenous tissue consistent with dura but without arachnoid features. Dynamic computed tomographic myelography is the study of choice for localization of the primary dural defect. Patient symptoms and neurological deficits routinely improve after appropriate surgical intervention. CONCLUSION: Diverse signs and symptoms herald the presentation of ventral spinal meningoceles. Intraoperative, radiographic, and pathological findings are all suggestive of an intradural dissection as the etiology. Hence, they may be more appropriately named “ventral spinal intradural dissecting meningoceles.” Definitive treatment involves identification and obliteration of the dural defect.

Ipsilateral headache associated with hypoglossal nerve palsy is uncommon and is usually reported to be secondary to internal carotid artery dissection. Herein, we report three idiopathic cases of berign ipsilateral headache with hypoglossal nerve palsy.