Uncommon Neurologic Complications of Burns in Infants: A Parkinsonian Extrapyramidal Disorder and Massive Cerebral Infarction Eli Shahar, MD,* Ilan Keidan, MD,” Natan Brand, MD,* Mira Frand, MD,® and Zohar Barzilay, MD‘ Tel Hashomer, Israel We report uncommon neurologic complications of moderate to severe burns in two infants aged 13 and 19 months, respectively. The first patient suffered a 25% total body surface area burn to her lower limbs; 3 days later she became mute and irritable, with increasing rigidity of limbs and trunk. Her face was expressionless and she also had mild dysphagea. Muscle-stretch reflexes were brisk and accompanied by episodes of coarse jitteriness. A diagnosis of an extrapyramidal parkinsonian disorder was made, The infant was treated with amantadine hydrochloride, and she recovered completely over a period of 2 months. The second infant was admitted with an extensive burn to his trunk and limbs; 3 days later he became comatose and had intractable seizures, which were more prominent over his right side. Dysphasia and right-sided hemiplegia became apparent; these conditions were and remained permanent, accompanied by focal seizures and a marked cognitive delay. Although relatively uncommon, central nervous system involvement may be a hazardous complication of burns in infants, who thus represent a high-risk group. (J BURN CARE REHABIL 1991;12:54-7) Neurologic complications are currently uncommon in both children and adults with burns. Cogswell and Chu! did not report even a single case with neuro- logic complications in their study series of 580 chil- dren with severe burns. A generalized encephalopa- thy is the most common complication of burns in children, clinically represented by alterations of con- sciousness, hallucinations, and scizures.?* Transient focal deficits were also reported in three of 20 patients by Antoon and coworkers.” Young children may oc- casionally experience cerebral edema, intractable sci- zures, and hemiplegia after even minor burns.5 We report here on the cases of two infants with moderately severe burns who had unusual neurologic complications. An extrapyramidal parkinsonian syn- drome was present in the first infant and massive cerebral infarction was present in the second. From: Child Neurology Unit,* Department of Pediatrics A,’ and Pe- diatric ICU, Division of Pediatrics, The Chaim Sheba Medical Center, Tel Hashomer, the Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel. Reprint requests: Eli Shahar, MD, Child Nenvology Unit, Division of Pediatrics, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel. 30/1/24424 54 CASE REPORTS Patient 1 A 13-month-old, previously healthy female infant was admitted with a second- to third-degree burn to her lower extremities and buttocks, approximately 20% to 25% total body surface area (TBSA). On admission she was fully alert and responsive, with a normal neurologic examination. Topical treatment with silver nitrate applications and baths was initi- ated, On the third day she began having recurrent episodes of high-grade fever. Repeated blood cul- tures remained sterile. On the fifth day she became mute and withdrawn. Eye contact with this patient could not be easily achieved, she showed no interest in her surroundings, and she intermittently uttered high-pitched cries. Her face became expressionless, with intact ocular movements. She maintained a se- verely flexed and rigid posture of limbs and neck (Figure 1). Recurrent vigorous episodes of coarse jitteriness involving mainly the right upper limb were noted both cither spontaneously and in reaction to tactile stimuli. Examination revealed marked cog- wheel rigidity of her limbs, trunk, and neck muscles. When pulled to a sitting position, she came forward like a log. Muscle-stretch reflexes were brisk, with extensor plantar response. Volume 12 Number 1 January/February 1991 Laboratory data on admission disclosed a marked hyperglycemia with a glucose level of 540 mg/di and a leukocytosis of 43,000/mm%, all of which gradually subsided. Her sodium level on admission was 125 mEq/L and was rapidly corrected over 4 hours to 135 mEq/L. Her sodium and glucose levels re- mained normal thereafter. The marked hypomimia, severe truncal rigidity, cogwheel limb rigidity, brisk muscle-stretch reflexes, and withdrawn behavior suggested a parkinsonian extrapyramidal syndrome. An clectroencephalogram showed mild generalized slowing. Magnetic reso- nance imaging of the head was unremarkable. No response was noted after a trial therapy with phe- nobarbital and clonazepam. Oral amantadine was then administered at a dose of 5 mg/kg; this treat- ment resulted in overt improvement over the course of a week. She became less irritable, more alert, and more interested in her surroundings. Her marked rigidity gradually subsided and disappeared over a period of 2 months. Her muscle-stretch reflexes be- came normal and her plantar response became flexor. A follow-up assessment 4 months after admission showed her to be normal in alertness, muscle tone, and muscle-stretch reflexes. Patient 2 A 19-month-old previously healthy male infant was admitted with an extensive second- to third- degree scald of approximately 60% TBSA. His trunk and limbs were affected, sparing his head. On arrival he was irritable and hyperpyrexic but fully alert and responsive. Intravenous treatment with lactated Ringer’s solution and topical applications of silver nitrate were initiated. Thirty-six hours after admis- sion, he suddenly became comatose and experienced severe generalized seizures involving mainly the right side of his body. Acute apnea necessitated nasotra- cheal intubation and positive-pressure artificial ven- tilation. Intravenous treatment with high doses of phenytoin and phenobarbital initially failed to con- trol his intractable seizures. Laboratory data from the beginning of this acute event revealed severe meta- bolic acidosis and severe hypoglycemia with glucose levels of 13 mg/dl, both of which were promptly corrected. Computerized tomography of the cere- brum demonstrated an extremely low density in the distribution of the left middle cerebral artery, a finding suggestive of massive cerebral infarction. Single-photon emission computerized tomography using technetium 99m labeled hexamethyl propy- lenamine oxine demonstrated decreased regional ce- rebral blood flow in the distribution of the left middle cerebral artery and over the right hemisphere.* Uncommon neurologic complications of burns 55 Figure 1, Extrapyramidal parkinsonian syndrome in a 2- month-old female infant with 25% TBSA thermal burn. Note the rigidly flexed position of limbs and the expres- sionless facies while the patient is awake. : His course of treatment was complicated by Staph- ylococcus aureus septicemia, for which he was vigor- ously treated with intravenous antibiotics and from which he eventually recovered. Mechanical ventila- tion was discontinued after 18 days. Marked right- sided hemiparesis remained, as did recurrent focal motor seizures over the right side of his body ac- companied by dysphasia. Initially, eye contact was unattainable, and he uttered high-pitched, irritable cries. Over a 12-month follow-up period, he became more alert, more attentive, and more amenable to commands, although he still exhibited significant cognitive delay. He also exhibited very mild improve- ment in receptive and expressive speech skills. His seizures proved partially responsive to a combination of carbamazepine, phenobarbital, and clonazepam. DISCUSSION The most common neurologic complication seen in children with burns is a generalized disturbance of cerebral activity, termed burn encephalopathy. This 56 Shahar ct al. disturbance may range from irritability, personality changes, and hallucinations to seizures, coma, and even death. This complication occurred in 5% to 15% of cases of children with burns.?* A change in the severity and prevalence of burn encephalopathy has been reported over the last 20 years. Twenty years ago, more children suffered from intractable seizures and coma secondary to cerebral edema leading to herniation and death than do now.>”° Mohnot and coworkers* recently reported a 5% incidence of burn encephalopathy, with a very low mortality rate. This lower mortality was attributed to improved medical and surgical management, including appropriate fluid replacement and possibly the avoidance of neu- rotoxic compounds such as hexachlorophene for the topical disinfection of burned areas.'® The first infant in this report experienced an ex- trapyramidal parkinsonian neurologic disorder, form which she recovered completely. Five days after a thermal burn of approximately 25% TBSA, she be- came mute and withdrawn, had expressionless facies, and displayed increasing rigidity. Tactile and audi- tory stimuli induced recurrent episodes, mainly over her right upper limb, of coarse jitteriness with in- creasing tone. Treatment with amantadine hydro- chloride seemed effective after the patient’s disorder failed to respond to clonazepam or phenobarbital therapy. To the best of our knowledge, an extrapy- ramidal disorder as a complication of severe burns in an infant has not been reported previously. The un- derlying pathogenesis of such a disorder is still ob- scure and open to speculation. This infant had hy- ponatremia of 124 mEq/L on admission, which was rapidly corrected over a period of less than 4 hours. Central pontine myelinosis has been associated with rapid correction of hyponatremia.’! This condition was found by McKee and coworkers’? in autopsies of 10 out of 139 patients with severe burns; they attributed it to a hyperosmolar state secondary to rapid correction of hyponatremia. A striatal extra- pontine syndrome was recently reported after rapid correction of hyponatremia.’ This patient’s symp- toms included mutism, bradykinesia, and rigidity, and he also showed improvement with antiparkin- sonian drugs similar to that of the infant described here. One may speculate that in the infant described here a similar striatal extrapontine parkinsonian dis- order may have resulted from rapid correction of hyponatremia; the disorder’s rapid onset was simi- larly followed by marked improvement and full re- covery. Journal of Burn Care & Rehabilitation The second infant described here suffered a mas- sive cerebral infarction as a complication of an ex- tensive burn. Burn encephalopathy is sometimes as- sociated with transient focal deficits.** Antoon and coworkers? reported four cases of children who had seizures and transient focal weakness late in the course of their disorder, three of whom fully recov- ered and one of whom died of Pseudomonas sepsis. The patient reported here exhibited intractable sei- zures and apparently massive hemispheral infarction, which appeared 36 hours after admission. He re- mained with complete left hemiplegia, intractable partial complex seizures, and marked developmental delay. Similarly, Emery and Campbell-Reid® re- ported the case of a 5-month-old infant who suffered a massive cerebral infarction and died. A few factors may have played a role in the evolution of massive cerebral infarction. A hypercoagulable state during the period immediately after the burn and marked hypoglycemia, as detected here, may be associated with generalized encephalopathy and uncommonly even with focal motor deficits.’* This infant, in fact, has widespread damage to his brain, as manifested by his marked cognitive and motor delay. This is delineated by a °°™Tc-labeled hexamethyl propylen- amine oxine, single-photon emission computerized tomographic study of regional cerebral blood flow, which showed several areas of decreased regional ce- rebral blood flow including that region responsible for his severe motor deficit. Our experience suggests that young infants with burns run a higher risk of neurologic complications than do older children. Vigorous treatment for these infants is necessary and should include slow correc- tion of hyponatremia, close monitoring of metabolic derangements, and treatment of hypoglycemia or hy- pocalcemia, in addition to the usual prompt man- agement of infection. REFERENCES 1. Cogswell JJ, Chu AC, Severe burns in children, 1964-1974. Arch Dis Child 1976;51:67-72. 2. Antoon AY, Volpe JJ, Crawford JD. Burn encephalopathy in children. Pediatrics 1972;50:609-16. 3. McManus WF, Hunt JC, Pruitt A. Postburn convulsive dis- orders in children. J Trauma 1974;14:396-401. 4. 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