A 49‐Year‐Old Man with Progressive Cranial Neuropathies

Abstract

A 49-year-old man presented with a 2-month history of headache and recent 20-pound weight loss. He was diagnosed with cluster headaches at an outside hospital and treated with triptans. One week after the initial onset of headache, he developed right jaw and facial pain with facial weakness, accompanied by inability to fully close his right eye and mouth. He was given one dosage of steroids at an outside hospital for cluster headache with full resolution of his symptoms. However, a week later, his right facial weakness recurred and persisted. About 4 weeks later, he complained about similar jaw/ facial pain and weakness of his left side, which appeared to be more severe than those of his right side. He also developed occasional diplopia on far gaze and left lateral gaze. He did not experience any changes in hearing or taste. Magnetic resonance imaging of brain revealed abnormal enhancement of the third, fifth, seventh and eighth cranial nerves with various degree of diffuse enlargement (arrows pointing to trigeminal nerves in Figure 1a). Patchy enhancement of the nerve roots in the distal lumbar region was also noted. A gallium whole body scan did not reveal any significant abnormality. Repeated CSF studies showed low glucose levels, elevated protein levels and elevated cell counts. Extensive peripheral blood and CSF work-up for infectious disease, autoimmune, demyelinating disorders and neoplasia were negative except for a positive T-cell lymphotropic virus type 1 (HTLV-1) antibody. His HIV-1/2 ELISA was negative. Peripheral blood flow cytometry showed mature WBCs with slightly elevated CD4 : CD8 ratio. CSF flow cytometry revealed large granular T-cell lymphocytosis consisting of CD3/