HTLV-1 Associated Motor Neuron Disease Mimicking "Man-in-the-Barrel" Syndrome

The "man-in-the-barrel" syndrome is a descriptive term that was used in the past for patients with complete flaccid paralysis of the upper extremities caused by symmetric "watershed" brain infarction after cardiovascular arrest or cardiac surgery. However, it is now evident that this syndrome can occur in acute and chronic diseases of the upper and lower motor neuron. We describe a 48-year-old woman with rapid and severe progressive weakness and wasting of both upper extremities mimicking the "man-in-the-barrel" syndrome. The neurologic and electrophysiological findings were compatible with anterior horn cell disease affecting the cervical spinal cord segments. There were only minimal signs suggesting the presence of myelopathy. After the initial rapid onset, progression during the 7 years of follow up was minimal. An extensive etiologic workup disclosed infection with HTLV-1.