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Primary lateral sclerosis with MR diffusion tensor image and tract tracking.

This report shows that magnetic resonance diffusion tensor imaging and fiber tracking can identify changes of the corticospinal tract in clinically diagnosed primary lateral sclerosis patients. A 67-yr-old man presented with quadriparesis and dysarthria. Slowness and clumsiness of the left hand became evident at the age of 62 yrs. Spasticity involved the left arm and soon spread to all four limbs. In addition, spasticity caused spastic dysarthria. At age 65, he showed gait disturbance and occasional falling. He was unable to walk unaided by age 67. He showed respiratory distress and aspiration occasionally. On examination, there was motor weakness (MRC score of 4–5) of both upper and lower extremities, bilateral ankle clonus, bilateral Barbinski responses, and spasticity, but no atrophy or fasciculations were observed. Brain magnetic resonance imaging, cerebral spinal fluid examination, and standard laboratory tests, including serum glucose, Hba1c, thyroid hormones, vitamin B12 and folate levels, viral (HIV, hepatitis B), and long-chain fatty acid were normal. Vasculitis markers (ANA, cANCA, pANCA, anti-DNA antibodies) and cerebral spinal fluid oligoclonal band were negative. Distal latencies, motor conduction velocities, F waves, and sensory nerve conduction were normal. Needle electromyography was performed in the muscles of both upper and lower extremities, tongue, and lower thoracic spine and showed normal resting potential and normal motor unit potential. Fractional anisotropy (FA) and mean diffusivity of pyramidal tract were evaluated with magnetic resonance diffusion tensor imaging. The FA values were 0.452 and 0.460 at the left and right midbrain, 0.080 and 0.105 at the left and right ventral pons, and 0.074 and 0.110 at the left