Spinal internuncial neurones in progressive encephalomyelitis with rigidity.

Abstract
The clinical and pathological features of a 4th patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. The muscular rigidity, abnormal postures, painful muscular spasms and myoclonus are a product of excessive and abnormal discharges of alpha motor neurons caused by their release from control by spinal internuncial neurons. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurons, most of which were spinal internuncial neurons. Experimental, clinical and pathological evidence concerning spinal internuncial neurons is reviewed and conforms to this theory. The pathogenesis of opsoclonus may be similar.

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