A Young Woman With Vessel Dissection and Brainstem Lesions

A 21-year-old woman with baseline depression, 1-year history of recurrent, painful, oral and vaginal ulcers, and cellulitis had a new, severe, acute-onset, left posterior headache with left shoulder pain. On neurologic examination, she had mild right oculomotor and abducens nerve weakness and marked left upper extremity and moderate left lower extremity upper motor neuron–type paresis. Initial brain magnetic resonance imaging showed acute ischemia involving the right pons, right midbrain, right cerebral peduncle, and internal capsule, extending into the right diencephalic region. Neck computed tomography angiography identified a right vertebral artery dissection at the C3 level. Cerebrospinal fluid analysis showed a marked neutrophilic pleocytosis with a reported “high” white blood cell count. HLA-B51 testing was positive. Biopsies of her ulcers indicated nonspecific inflammation with no infectious sources. Her severe headache preceding the manipulations, along with recurrent, painful, oral and genital ulcers (recurrent aphthous stomatitis), cerebrospinal fluid pleocytosis, and brainstem involvement after a vertebral artery dissection, raised concern for Behçet syndrome and eventually neurologic involvement of Behçet syndrome. Recurrent corticosteroid-responsive oral ulcers plus recurrent genital ulcers and skin lesions (cellulitis) fulfill the criteria for Behçet syndrome, with possible neurologic involvement (neuro-Behçet syndrome). The positive HLA-B51 testing was consistent with the diagnosis of Behçet syndrome. Treatment was initiated with low-dose aspirin and intravenous methylprednisolone, after which her neurologic status started to improve. Oral prednisone and azathioprine were added for long-term treatment. At age 25 years, she discontinued azathioprine because she planned pregnancy. At age 27 years, she started having recurrence of oral and genital ulcers, along with axillary ulcerative skin lesions. New-onset diplopia and left-sided weakness also developed before corticosteroid and azathioprine could be reinitiated. Magnetic resonance imaging of the brain showed a new left pontine and cerebellar peduncle lesion with subtle contrast enhancement. Intravenous methylprednisolone was initiated, followed by reinitiation of oral prednisone and the azathioprine regimen for long-term maintenance immunotherapy. The prednisone was slowly tapered after 3 months. The final diagnosis for this patient was relapsing neuro-Behçet syndrome because she had 2 recurrent neurologic episodes associated with 1) vertebral artery dissection and 2) brainstem involvement. If she were to have further relapses, the plan was to administer a tumor necrosis factor-α‎ inhibitor. The case of this patient highlights 3 aspects of Behçet syndrome: 1) diagnosis of systemic Behçet syndrome is made on clinical grounds only, but even if the diagnostic criteria are not fulfilled, once neuro-Behçet syndrome develops, treatment should be initiated to curtail significant morbidity; 2) although rare, arterial involvement in neuro-Behçet syndrome should be recognized; and 3) there are notable sex-dependent factors in the evolution of Behçet syndrome.