Pearls cLongitudinally extensive transverse myelitis (TM) or TM with inconclusive CSF findings should raise concern for prion disease (also known as Creutzfeldt-Jacob disease [CJD]) especially in the setting of associated cognitive impairment. c CJD associated with focal findings should raise suspicion for spinal cord involvement and warrants spinal imaging. c Spinal cord involvement in CJD could suggest more rapid disease progression, although further investigations are warranted.
Oy-sters cThe MRI findings in the brain typically associated with CJD may not appear until later in disease progression. c Evaluation of a patient presenting with subacute to acute myelopathy should include CSF testing for prion disease when there is associated rapid decline in mental status.A 57-year-old man with a history of monoclonal B-cell lymphocytosis was admitted because of 2 months of progressive clumsiness, gait instability, and bowel/bladder incontinence. There was no family history of dementia or similar symptoms. Neurologic examination showed poor attention, tangential thinking, dysarthria, right leg weakness, right arm ataxia, tremors, and an upgoing right toe. There was no sensory level and jaw jerk was not tested. In addition to cerebral injury, these symptoms, together with incontinence, were clinically more suggestive of a primary myelopathy. However, uncertainty about spinal vs cortical pathology remained and thus MRI spine and brain were obtained. MRI spine ( figure, A and B) demonstrated a longitudinally extensive transverse myelitic (LETM) lesion in the cervicothoracic (C6-T1) region while MRI brain was unremarkable. CSF analysis demonstrated the following: glucose 93 mg/dL, protein 55 mg/dL, white blood cells <3, and red blood cells <3. Cytology showed no abnormal cells to suggest CNS lymphomatous involvement. Previous evaluation for monoclonal B-cell lymphocytosis revealed a kappa light chain restricted CD20+ population comprising 12% of the patient's lymphocytes. This was monitored biannually without treatment and was noted to have improved on its own by the time of hospital presentation. Further workup for LETM, including aquaporin-4 antibody, immunoglobulin G index, oligoclonal band index, antinuclear antibody, extractable nuclear antigen antibodies, vitamin B 12 , copper, vitamin E, autoimmune, and paraneoplastic antibody testing, were all unrevealing. Infectious evaluation was negative in the CSF (including HIV, herpes simplex virus 1, herpes simplex virus 2, cytomegalovirus, and varicella-zoster virus) and serum (including Lyme antibodies, human T-cell lymphotropic virus, fungal serologies, hepatitis, and syphilis). CT chest was negative for evidence of sarcoidosis.