596 Brief clinical and laboratory observations Infectious diarrhea was originallysuspected because of acute onset with fever and leukocytosis, the severity of the diarrhea, and the presence of sheets of polymorphonuclear leukocytes in stool. The difficulty in culturing S. dysenteriae from the stool was a major factor in our failure to establish a correct diagnosis. This is not u n c o m m o n since 20% of patients known to be infected with Shigella may not have microorganisms cultured from their stool. The barium enema interpreted as ulcerative colitis added to confusion in diagnosisY H a d proctosigmoidoscopy been done initially, diagnostic dilemma would have been avoided, and a course of antimicrobial therapy might have reversed the pathologic,process before colectomy became necessary. Shigella dysenteriae type I. must be ~eturned to the list of causes of pseudomembranous colitis. REFERENCES 1. Formal SB, Gemski R, Gianella RA, et al: Mechanism at Shigella pathogenesis, Am J Clin Nutr 25:1427, 1972. 2. Saphir O: A text on systemic pathology, New York 1959, Grune & Stratton, Inc, pp 950-952; 962-963. 3. Sleisenger M, and Fordtran J: Gastrointestinal disease, Philadelphia, 1973, WB Saunders Company, pp 13731377. 4. Silverman A, Roy CC, and Cozzetto FJ: Pediatric clinical gastroenterology, St. Louis, 1971, The CV Mosby Company, pp 140-141. 5. Boekus HL, et al: Gastroenterology, in Dearing WH, editor: Infectious diarrheas, Philadelphia, 1976, WB Saunders Company, pp 956-957. The "'locked-in'" syndrome in children Gerald S. Golden, M.D.,* Norman Leeds, M.D., Bronx, N. ii., Martin W. Kremenitzer, M.D., New York, N. Y., and Barry S. Russman, M.D., Hartford, Conn. From the Departments of Pediatrics and Neurology, Albert Einstein College of Medicine; Department of Radiology, Montefiore Hospital and Medical Center," Departments of Pediatrics and Neurology, New York Medical College," and Departments of Pediatrics (end Neurology, University of Connecticut Medical School. *Reprint address: 1410 Pelham ParkwaySouth, Bronx, N. Y. 10461. The Journal of Pediatrics October 1976 6. Morson BC, and Dawson IMP: Gastrointestinal pathology, ed 2, Oxford, England, 1974, Blackwell Scientific Publications, p 259. 7. Reiner L, Schlesinger MJ, Miller GM: Pseudomembranous colitis following aureomycin and chloramphenicol, Arch Pathol 54:39, 1972. 8. Pettet JD, Baggenstoss AH, Dearins HH, et al: Postoperative pseudomembranous enterocolitis, Surg Gynecol Obstet 98:546, 1954. 9. Scott AJ, Nicholson GI, and Ken AR: Lincomycin as a cause of pseudomembranous colitis, Lancet 2:1232, 1973. 10. Cohen IE, McNeill CJ, and Wells RF: Clindamycin associated colitis, JAMA 223:1379, 1973. 11. Tedesco FJ, Barton RW, and Alpers DH: Clindamycin associated colitis, Ann Intern Med 81:429, 1974. 12. Goulston SH, and McGovern VJ: Pseudomembranous colitis, Gut 6:207, 1973. 13. Tedesco FJ, Stanley RJ, and Alpers DH: Diagnostic features of clindamycin associated pseudomembranous colitis, N Engl J Med 290:841, 1974. 14. Reller B, Gangarosa EI, and Brachman PS: Shigellosis in the United States-five-years review of nationwide surveillance, 1964-1968, Am J Epidemiol 91:161, 1970. 15. Levine VD, Overturf GD, and Mathies AW: Shigella dysenteriae type I--severe dysentery and sepsis with hematologic hepatic and renal complications, West J Med 121:501, 1974. 16. Willis KC, and Rosenblatt RM: Shiga bacillus dysentery complicated by bacteremia and disseminated intravascular coagulation, J PEmATR 83:90, 1973. 17. Shimkin PM, and Link RJ: Pseudomembranous colitis: A consideration in the barium enema differential diagnosis of acute generalized ulceration colitis, Br J Radiol 46:437, i973. THE TERM " L O C K E D - I N " SYNDROME refers to the condition of total motor paralysis with preservation of mesencephatic-controlled vertical eye movements and an intact sensorium. This has been described as "alert wakefulness accompanied by m u t e tetraplegia. TM As evidence for intact cerebral cortical function, the patient can be taught to communicate by means of a set of coded eye blinks. The syndrome differs from that of the akinetic mute. In the latter condition, the patient can move, although he rarely does, and despite a superficial appearance of alert wakefulness, communication cannot be established. No previous reports o f this condition in children have been found. CASE REPORTS Case 1. This 13-year-old boy had cyanotic congenital heart disease consisting of tricuspid atresia, ventricular septal defect, and pulmonic stenosis. At age 10 years a Blalock-Taussig anastamosis had been performed. Volume 89 Number 4 Fig. 1. Basilar artery occlusion. The lateral projection following injection of contrast medium into the right vertebral artery demonstrates reflux into the left vertebral artery. A thrombosis of the basilar artery is demonstrated in its mid-segment (arrow) (Case 1). On the day of admission he had sudden onset of dizziness followed by nausea, vomiting, and lethargy. Neurologic examination showed deep obtundation and bilateral decerebrate posturing. Pupils were small and reactive. Optic axes were conjugate, but there were no spontaneous ocular movements and no reaction to oculocephalic maneuvers. Ice water caloric testing showed a bilateral internuclear ophthalmoplegia only on the first day. Electroencephalogram had depressed, disorganized activity, but with organized sleep spindles and vertex sharp waves. Cerebral angiography demonstrated occlusion of the midporti0n of the basilar artery (Fig. 1). Four days after admission he remained quadriplegic but appeared to be awake and alert. He could blink, elevate, and depress his eyes and could use a blink code to answer questions. Over the following year and one-half his neurologic status remained virtually unchanged, except for some voluntary function of his left arm that permits use of a communication board and an electric typewriter. Case 2. A 5 1/2-year-old boy with transposition of the great vessels had an atrial septotomy by balloon catheter at age nine days, and an anastomosis of the right puhnonary artery to the superior vena cava at age 10 months. Because of increasing cyanosis and fatiguability, cardiac catheterization was carried out, and four hours later he became decerebrate with bilateral ptosis and fixed pupils. Extraocular movements could not be obtained, either with cold caloric testing or oculocephalic maneuvers. Some recovery occurred over the next three days, with partial Brief clinical and laboratory observations 59 7 return of lateral eye movements and responsiveness of the right pupil to light. Two months later he remained aphonic, but could move his eyes laterally on command, and responded by smiling. Four months later he began to vocalize and to make purposeful movements with his left arm. Two months later he had complete ptosis on the left and partial ptosis on the right. There was no convergence or vertical gaze. The right eye had some movement past midline. He had a bilateral intention tremor and a wide-based stance. Slow improvement has continued. Case 3. A 13-year-old girl presented with headache, facial numbness, and unsteady gait. She had dysfunction of the left fifth, sixth, and seventh cranial nerves, bilateral pyramidal tract signs with right-sided preponderance, ataxia of the left hand, and an ataxic gait. An intramedullary brainstem tumor was identified by cerebral angiography. She received a total dose of radiotherapy of 5,640 rad to the posterior fossa. She survived for 10 months with progression of her neurologic signs. For a period of several days she developed bilateral decerebrate posturing, was aphonic, and had facial diplegia and complete lateral gaze palsies. She was able to open and close her eyes and look up and down on command, however. Subsequently she again became comatose. DISCUSSION T h e t e r m "locked-in" s y n d r o m e has b e e n applied to patients w h o can p e r f o r m n o voluntary m o v e m e n t s o t h e r t h a n vertical eye m o v e m e n t s , but are able to c o m m u n i c a t e utilizing the latter. The u n d e r l y i n g p a t h o l o g y in the majority o f anatomically d o c u m e n t e d cases is nearly complete infarction of the basis pontis, owing to occlusion of the basilar artery. 2 Destruction of this region of the b r a i n s t e m interrupts b o t h corticospinal tracts p r o d u c i n g quadriplegia, often with decerebrate posturing. Vertical eye m o v e m e n t s are lost as a result of i n v o l v e m e n t o f the sixth n e r v e nuclei, p a r a a b d u c e n s region, a n d medial longitudinal fasciculus. Lack of i n v o l v e m e n t o f the m e s e n c e p h a l o n permits preservation of convergence a n d eye m o v e m e n t s in the vertical plane. To m a i n t a i n consciousness the bulk of the rostral p o n t i n e t e g m e n t u m must be spared. S i n c e severe vascular d a m a g e in the distribution o f the posterior circulation generally leads to fatal illness, the manifestations m a y be difficult to elicit a n d only be present transiently d u r i n g the patient's rapid decline. A few l o n g - t e r m survivors h a v e b e e n reported. E l e c t r o e n c e p h a l o g r a p h y has shown, in most cases, a l p h a a n d theta r h y t h m s w h i c h were reactive to external stimuli. 8 This is characteristic of conscious patients a n d differs f r o m the unresponsive fixed a l p h a frequencies sometimes seen in coma; it m a y be useful in confirming the diagnosis. Patient 1 clearly m e t all of the clinical criteria of this syndrome, a n d the site o f the lesion was confirmed 598 Brief clinical and laboratory observations angiographically. This has not been previously reported in children, probably owing to the rarity of posterior circulation occlusive vascular disease in children.' Children with cyanotic congenital heart disease are predisposed to vascular accidents and basilar occlusion;' but not to the "locked-in" syndrome. The Blalock-Taussig procedure can be associated with basilar insufficiency, but rarely occlusionY Although the second patient was mute and tetraparetic but awake, alert, and responsive, he rapidly regained some horizontal eye movements, while maintaining bilateral ptosis and inability to carry out vertical movements. This implies involvement of mesencephalic structures as well as of the pons. A patient with bilateral midbrain infarcts as a cause of the "locked-in" state has been previously reported. 7 The third child is unusual, since only one previous case of this syndrome in a patient with a brainstem tumor has been reported, s This was an adult who had a reticulum cell sarcoma involving the thalamus, mesencephalon, and pons. Although rare in its occurrence, it is important that this condition not be overlooked. Any patient seemingly comatose should be tested for the presence of vertical eye Normal leukocyte glutathione peroxidase activity in patients with chronic granulomatous disease The Journal of Pediatrics October 1976 movements in response to verbal commands. The electroencephalogram may be a useful ancillary tool in diagnosis. Recognizing the "locked-in" syndrome is important for humane patient care, especially in the case of children in w h o m long-term survival is possible. REFERENCES 1. Plum F, and Posner JB: The diagnosis of stupor and coma, Philadelphia, 1966, FA Davis Company, pp 92-93. 2. Nordgren RE, Markesberry WR, Fukada K, and Reeves AG: Seven cases of cerebromedullospinal disconnection: The "locked-in" syndrome, Neurology 21:1140, 1971. 3. Hawkes CM, and Bryan-Smyth L: The electroencephalogram in the "locked-in" syndrome, Neurology 24:10t5, 1974. 4. Harwood-Nash DC, McDonald P, and Argent W: Cerebral arterial disease in children, Am J Roentgenol 111:672, 1971. 5. Cottrill CM: Cerebral vascular accidents in cyanotic congenital heart disease, Am J Dis Child 125:484, 1973. 6. Clarkson PM, Gomez MR, Wallace RB, and Weidman WM: Central nervous system complications following Blalock-Taussig operation, Pediatrics 39:18, 1967. 7. Karp JS, and Hurtig MI: "Locked-in" state with bilateral midbrain infarcts, Arch Neurol 30:176, 1974. 8. Cherington M, Stears J, and Hodges J: Locked-in syndrome caused by a tumor, Neurology 26:180, 1976. consumption, hexose monophosphate shunt activity, nitroblue tetrazolium dye reduction, and hydrogen peroxide and superoxide anion production. ~ At least two forms of the disease exist; the classic disease is described in male children and appears to be inherited in an X-linked pattern? A similar syndrome has been described in females and in some males in which cases there are no demonstrable heterozygotes in the families; the inheritance of C G D in these cases may be autosomal recessive. 4 Lawrence R. DeChatelet, Pamela S. Shirley, and Linda C. McPhail, Winston-Salem, N. C. C H R O N I C GRANULOMATOUS DISEASE of childhood is a syndrome characterized by an inability of the patient's leukocytes to kill many types of bacteria.' This is associated with defective oxidative metabolism which usually accompanies the phagocytic event. Leukocytes from affected patients do not exhibit increases in oxygen From the Department of Biochemistry, The Bowman Gray School of Medicine. Supported by a grant from the Forsyth Cancer Service and by United States Public Health Service Grant A 1-10732from the National Institute of Allergy and Infectious Disease. Abbreviation used CGD: Chronic granulomatous disease [ The precise molecular defect is controversial in the X-linked disease) but Holmes and associates '~ reported a deficiency in leukocyte glutathione peroxidase activity in two female patients with the disease. To the best of our knowledge, this observation has not been verified in the literature and so we initiated an investigation of leukocytic gluthathione peroxidase activity in patients with CGD. MATERIALS AND METHODS The seven patients studied had a positive diagnosis of C G D as evidenced by metabolic studies (including oxygen