Serum creatine kinase levels after succinylcholine in children with "muscle, eye and brain disease"
Four boys belonging to a group of children affected by a rare farm of muscular dystrophy with eye aud brain involvment, termed the "muscle, eye and brain disease" (MEB), were anaesthetized for ~'arious eye examinations and surgery. On some occasions suceinyicholine was used during anaesthesia and the initially elevated serum creatine kinase (CK) values increased from a range of 122 to 1200 units.L -t to a range of 4350 to 9690 units'L -J 22 hours after anaesthesia. CK values after anaesthesia without succinylcholine remained at the initially elevated levels. Rectal temperatures of the children were normal. These findings suggest that succinylchollne should be avoided itJ patients with M~LB dlsease.From 1974 to 1977, four mentally retarded boys who also suffered from severe muscular dystrophy underwent general anaesthesia for diagnostic eye examinations and surgery for glaucoma, or muscle biopsy. In 1977, Santavnori et al. included these patients in a report of children with the rare condition termed "muscle, eye and brain disease" (MEB), t The aetiology of this condition remains unknown and in order to determine the degree of muscle damage, serum creatine kinase {CK) activity of the children had been repeatedly analyzed in the paediatric unit and found to be increased. Recent interest in MEB disease prompted the presentation of this report to deter- mine whether anaesthesia and surgery had influenced the initially elevated serum CK levels of these patients.
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Case reportsFour boys aged 2 to 44 months and weighing 5.3 to 13.0 kg were anaesthetized 9, 3, 4 and 3 times for various ophthalmological procedures or muscle biopsy. The children were mentally retarded and suffered from severe muscle weakness; they were bedridden, able to sit when supported and although they could swallow food, rt'tained their secretions. Examinations revealed large c,trneal diameters and myopia. Three of the patients had de~ elopmental glaucoma which required goniopuncture or trabeculectomy.Serum CK values were measured variably on the first postoperative day. Possible correlation between these values and the mode of anaesthesia with and without succinylcholine administration, as well as to other CK values of the same children was sought. Rectal temperatures had been measured one hour after some of the anaesthestics: the CK activity was measured at 250 C with test sets (Boehringer, Mannheim). Normal values for adults were 0 to 50 units,L -t.Serum CK detemunations were made on eight occasions 20-24 hours after anaesthesia. These values, along with the mode of anaesthesia and the patient's rectal temperatures one hour following anaesthesia, are shown in the Table. High CK values of 5200, 5500, 9690 and 4350 units-L -t were noted following anaesthesia which included intravenottsly administered succinylcholine for trabeculectomy or eye examinations. Anaesthesia without use of suceinylcholine, for muscle biopsy or trabeculectomy, was as...
