Objects Jumping, Body Bumping, Hearing Slumping…Quick, He is Crumping (Slides)

A 45 y/o man developed insidious unsteadiness and bilateral hearing impairment. A neurosurgeon diagnosed possible myelopathy, and despite an anterior cervical discectomy, he noted progressive gait difficulty, poor handwriting, and inability to play the guitar. Additionally, dysarthria, oscillopsia and dizziness with rapid head movement developed. He had ~ 40% sensorineural hearing loss in his left ear, and eventually hearing loss in his right ear. When we first saw him, he used a walker at home, but had frequent falls. Finally, he had disabling episodes of vertigo, nausea and vomiting about twice per month, lasting several hours at a time. His PMH was significant for testicular seminoma, s/p resection in 2007, (left orchiectomy), and had normal oncologic monitoring in July 2017. Family history of ataxia or hearing loss was negative. He did not drink alcohol. The exam showed limb and truncal ataxia, dysarthria, primary gaze downbeat nystagmus, saccadic pursuit and bilateral decreased hearing with normal VOR. A head MRI (pre and postcontrast was normal). He had a chest/abdomen and pelvis CT scan that showed two enlarged lymph nodes, one periaortic (2 x 2.2 cm), and one near the left renal artery (3 x 1, 8 cm). A biopsy showed small lymphocytes and a focus of non-necrotizing granulomatous inflammation, without evidence of carcinoma on H&E or pancytokeratin immunostains. CD68 identified hystiocytes (non-necrotizing granulomatous inflammation). Staining of the tissue after the pathologist became aware of the seminoma history showed rare seminoma cells and immunostaining with OCT4 was positive, leading to final diagnosis of "metastatic seminoma" CSF examination showed 13 white cells (all lymphocytes), protein: 28.56 mg/dL, and glucose: 50 mg /dL there was no local IGG synthesis and no oligoclonal bands. The anti-Ma antibody was negative, 14-3-3 protein level was 2 ng/mL (normal). A paraneoplastic panel performed at the Mayo Clinic was negative