RT Journal Article SR Electronic T1 Spinal internuncial neurones in progressive encephalomyelitis with rigidity. JF Journal of Neurology, Neurosurgery & Psychiatry JO J Neurol Neurosurg Psychiatry FD BMJ Publishing Group Ltd SP 773 OP 785 DO 10.1136/jnnp.42.9.773 VO 42 IS 9 A1 D A Howell A1 A J Lees A1 P J Toghill YR 1979 UL http://jnnp.bmj.com/content/42/9/773.abstract AB The clinical and pathological features of a fourth patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. It is suggested that the muscular rigidity, abnormal postures, painful muscular spasms, and myoclonus are a product of excessive and abnormal discharges of alpha motor neurones caused by their release from control by spinal internuncial neurones. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurones, most of which were spinal internuncial neurones. Experimental, clinical, and pathological evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar.