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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.9.773 on 1 September 1979. Downloaded from Journal ofNeurology, Neurosurgery, andPsychiatry, 1979, 42, 773-785 Spinal internuncial neurones in progressive encephalomyelitis with rigidity D. A. HOWELL, A. J. LEES, AND P. J. TOGHILL From the Department of Neuropathology of the Derbyshire Royal Infirmary, Derby, and the Neurological Unit of University College Hospital and the General Hospital, Nottingham SU M M A R Y 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 myo- clonus 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 patho- logical evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar. Protected by copyright. Kasperek and Zebrowski (1971) described a patient of 56 years after a complex illness lasting 26 with a complex, progressive, neurological disorder months. A succession of neurological signs and eventually dominated by board-like rigidity of symptoms were superimposed one after another, muscles, abnormal postures, and painful muscular with only a few brief periods of partial remission spasms. Similar patients were described by Lher- or abrupt exacerbation on the progressive down- mitte et al. (1973) and Whiteley et al. (1976). The hill course. postmortem examination in all three patients Her symptoms began in May 1974, when she showed an encephalomyelitis of the spinal cord developed giddy spells lasting about half an hour. and brainstem, suggestive of a viral infection, During these spells her head and neck flushed, she affecting both grey and white matter. The pyra- sweated profusely, the rest of her body tingled, midal tract, and to a lesser extent the lower motor and her gait was unsteady. Occasionally they were neurones, were spared from destruction by the associated with a sense of rotation and diarrhoea. disease, producing a unique and distinctive appear- Four months later she complained of brief attacks ance. We report a fourth patient with the same of severe burning pain in the left side of the neck disease with a selective destruction of small and and head, each lasting 10 seconds or so. By intermediate sized spinal neurones, most of which December 1974, her gait was continuously un- were spinal internuncial neurones. We suggest that steady, and she held her head flexed towards the http://jnnp.bmj.com/ the abnormal discharges of the alpha motor right side. Stiffness of her fingers made housework neurones causing rigidity, abnormal postures, pain- difficult. ful muscle spasms, and myoclonus in our patient In February 1975 she was admitted to the were a direct consequence of their release from General Hospital, Nottingham, after a brief control by the destroyed spinal internuncial episode of loss of consciousness on rising from neurones. bed. This was followed by recurrent attacks of severe vertigo and vomiting, subsiding after four Case report days. She complained of pain in her neck and a on October 1, 2021 by guest. stinging feeling all over her body. The patient was a housewife who died at the age On physical examination at that time she was alert and co-operative. There was a flame-shaped Address for reprint requesL : Dr D. A. Howell, Department ofNeuro- pathology, Derbyshire Royal Infirmary, London Road, Derby haemorrhage in the left fundus. Elevation of the DE1 2QY. left eye was impaired, and there was a rotary Accepted 12 March 1979 nystagmus on lateral gaze to either side, after the 773 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.9.773 on 1 September 1979. Downloaded from 774 D. A. Howell, A. J. Lees, and P. J. Toghill vertigo had subsided. The head was drawn to the were present but there were no abnormal move- right and neck movements were limited, painful, ments of the tongue or palate. There was no and resisted. She had difficulty in relaxing her rigidity of the muscles of the face or jaws. Her muscles. There was ataxia of the arms and legs head was drawn to the right and slightly back by without weakness or wasting of muscles or any rigid neck muscles, and attempts to correct the loss of sensation in her limbs or trunk. Her gait posture were resisted involuntarily by increased was ataxic and shuffling. The left triceps and painful muscular spasm. Severe plastic rigidity was ankle jerks were absent, and the right plantar present in the right arm and left wrist. There was response was extensor. painful restriction of movement of the right Her past health had been good except for stress shoulder. The abdominal muscles were rigid and incontinence and episodes of dysuria after vaginal felt hard. The leg muscles were rigid, causing the hysterectomy in 1965. She had mild hypertension legs to lie extended and slightly internally rotated (blood pressure 160/1 10 mmHg). at the hips with inversion of the ankles. The left Investigations included normal full blood count, arm shook continuously with a two or three per blood urea and electrolytes, erythrocyte sedimenta- second coarse flapping tremor. A milder inter- tion rate, liver function tests, blood glucose, chest mittent but similar tremor affected the muscles and skull radiographs, and serum vitamin B12. rotating the left hip more than the right one. Serological tests for syphilis and a gamma scan Shock-like contractions of muscles of the left arm, of the brain were also negative. Cerebrospinal particularly the biceps brachii, and of the left leg fluid (CSF) examination, however, revealed clear, occurred spontaneously, and the brief muscular colourless fluid under normal pressure with 36 spasms spread a little to neighbouring muscles white cells per mm3 (95% lymphocytes) and 0.52 before subsiding slowly over several seconds. They g/l of protein. A course of adrenocorticotrophic were very painful. These painful myoclonic jerks hormone (ACTH) therapy was given with only or spasms were easily provoked by examination, Protected by copyright. transient slight improvement. In June 1975 a making the assessment of muscle power difficult, repeat lumbar puncture yielded CSF with a normal but it was thought that there was only a little cell count and glucose, and a protein level of generalised weakness. She was thin, but the only 0.81 g/l. In July 1975 the CSF contained five definite wasting was in the small muscles of the white cells per mm3 and 0.52 g/l of protein. hands. The left leg was ataxic in the heel-knee- Serological tests for syphilis were negative and the shin tests. All tendon reflexes were absent, and the Lange colloidal gold curve was flat. While in the plantar responses were extensor. Vibration sense ward she developed burning pains radiating around and joint position sense were normal, but there her chest. was patchy sensory impairment to pinprick over In October 1975 she was transferred to Univer- the limbs and trunk, and two-point discrimination sity College Hospital under the care of Dr G. M. was impaired in the hands. Stern for additional opinions about the nature of Routine haematological and biochemical investi- her illness. At this stage she complained mostly gations were normal, as was the urinary 4-hydroxy- of abnormal movements and postures. She kept 3-methoxymandelic acid (VMA). Two urinary 5- her eyes closed because she was distressed by the hydroxy-indoleacetic acid (5IAA) estimations were light, by double vision, and by jerky movements within the normal range of 15 to 75 jtmol/24 hr, of objects about her. Her arms and legs tingled but the third was 150 ,umol/24 hr. The Mantoux http://jnnp.bmj.com/ and were clumsy. She had difficulty in chewing test was 1 in 1000 and the tests for rheumatoid and and swallowing. On examination she was mildly antinuclear factor were negative. The electro- depressed but otherwise showed no mental abnor- cardiogram, electroencephalogram, skull radio- mality and gave a lucid and precise account of graphs, gamma scan of brain, vertebral angiogram, her illness. Her speech was slurred and the slightest and computerised axial tomograms of the brain exertion made her breathless. When she opened were all normal. Nerve conduction studies showed her eyes there was divergent strabismus with both normal motor nerve conduction velocities and a eyes tending to look downwards. Conjugate move- slightly reduced median nerve sensory action on October 1, 2021 by guest. ments were otherwise full, though continuously potential. interrupted by vertical or rotary conjugate jerks. A course of dexamethasone was given without It was these conjugate dancing eye movements benefit, but diazepam gave slight relief from the (opsoclonus) which were associated with the ap- painful spasms. parent movement of objects about her (oscillopsia). The patient returned to Nottingham in Novem- Frequent grimacing and lip smacking movements ber 1975 and remained in hospital until she died J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.42.9.773 on 1 September 1979. Downloaded from Spinal internuncial neurones in progressive encephalomyelitis with rigidity 775 in June 1976. In January the tremor, the oro- method, and fairly uniform throughout the grey facial dyskinesias, and the painful spasms sub- matter of the spinal cord, though the gliosis was a sided, perhaps in response to treatment with little less dense in the ventral horns of the grey chlorpromazine, but in March the painful spasms matter than in the intermediate zone, and much in the left arm and leg became almost continuous.

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