A 64-year-old woman who had amyotrophic lateral sclerosis (ALS) with disturbance 
of vertical ocular movement was presented. She was admitted to our hospital with 
progressive dysphagia, dysarythria and weakness of the extremities. Neurological 
examinations revealed disturbance of vertical ocular movement with normal doll's 
eye phenomenon (supranuclear origin), bulbar palsy, muscle weakness of the 
extremities, extensor plantar signs, and fasciculations of the costal and 
interosseal muscles. EMG studies showed denervation potentials, and muscle 
biopsy demonstrated group atrophy, fiber type grouping and small angular fibers. 
TRH injections resulted in improvement of disturbance of vertical ocular 
movement, but no effect was seen on the weakness of the limb. There was about 20 
Japanese cases with disturbance of ocular movement in ALS, but it was rare to 
see ocular movement disorder from the early stage of ALS. The pathophysiology of 
ocular movement disorder in ALS has been thought to be due to supranuclear 
origin, i.e., the disturbance in the pathway from the frontal cortex to the 
mesencephalon. In this case, TRH might effect at some point of the 
frontomesencephalic pathway.