Chicken-pox and the Guilain Barre syndrome.

D.H., male, age 9 years 11 months, developed chickenpox in March 1960. He had an extensive rash and showed more general illness at the outset than did his younger brother who infected him. After a week, he complained of headache and pain in his limbs and began to vomit. When he tried to get up, he cried with pain in his legs. These symptoms persisted. When seen for the first time 14 days after the onset of his illness, he was afebrile. He had neck stiffness and the Kemig sign was positive. There was some tenderness of the muscles of the left thigh, the tendon reflexes in the legs were absent apart from a very weak response in the left ankle jerk while those in the arms appeared normally brisk except for the supinator jerks which could not be elicited. Muscle power appeared normal. The right lower abdominal reflex was absent. Cranial nerve function was normal, there was no nystagmus and both sensation and co-ordination appeared normal. He was admitted to hospital (March 29, 1960), and lumbar puncture produced a clear fluid under normal pressure, containing two white cells (both lymphocytes) per c.mm., an excess of globulin, protein 240 mg./100 ml., chloride 760 mg./100 ml., sugar 75 mg./100 ml., and gold curve 0110000000. Blood count-haemoglobin 97%, white count 6,400/c.mm. (50% neutrophil, 2% eosinophil, 2% basophil polymorphonuclears, 43% lymphocytes and 3% monocytes). Sedimentation rate (Wintrobe) 20 mm. in one hour, P.C.V. 42%. Urine was normal and Heaf test was negative. He was given soluble aspirin as his only therapy, and for the next nine days, during which time his temperature rose slightly above normal on only two occasions, his condition was watched. The neck stiffness persisted, lessening after a week; the Kemig sign remained positive. All abdominal reflexes disappeared. The tendon reflexes in the arms became more difficult to elicit and less brisk, but in the triceps and biceps never completely disappeared. The knee jerks and right ankle jerk could never be elicited but an inconstant and occasional flicker could be observed in the left. Wasting of the leg muscles became apparent and the power became weaker. Plantar responses were flexor or equivocal. Some muscle tenderness and pain were complained of in the left deltoid and biceps muscles on one occasion. No sensory changes were apparent to testing and, though he became severely constipated in the first few days in hospital, there were no signs of sphincter disturbance. There was a slight improvement in his general well-being after three or four days. A second lumbar puncture was carried out on April 6, 1960, and showed 4 cells/c.mm., all lymphocytes, globulin excess, protein 500 mg./100 ml., chloride 720 mg./ 100 ml., sugar 45 mg./100 ml., Lange 0000012210, Wassermann reaction negative. Next day he was given his first dose of methyl prednisolone ('medrone'), 4 mg., continuing this four-hourly for six days and then reducing to eight-hourly for six days, 12-hourly for eight days and then 2 mg. eight-hourly for a week, 12hourly for a week and daily for four days. The neck stiffness and positive Kemig sign disappeared within 36 hours of starting treatment and the child became much better. Weak tendon reflexes in knees and ankles were obtained after 12 days, the abdominal responses were likewise discovered to be present on that day, and a week later all tendon and skin reflexes appeared normal. Muscle power returned rapidly and he was allowed up after a month. Further lumbar punctures showed the following: On April 20, 1960, 1 cell/c.mm., protein 140 mg./ 100 ml., and globulin in excess; on May 2, 1960, 1 cell/ c.mm., protein 40 mg./100 ml., globulin in slight excess; and on May 19, 1 cell/c.mm., protein 40 mg./100 ml., and globulin no excess. Apart from a slight irritative reaction after the last lumbar puncture (headache and vomiting) and later some angular stomatitis, there was no further trouble and he was discharged well on June 4, 1960. When last seen on July 15, 1960, he was well but was said to get a little tired after walking two or three miles.