©1979.VOL.10,NO.3
CLINICAL ELECTROENCEPHALOGRAPHY
Visual Evoked Potentials in Reye's Syndrome L. Ch'ien, J. Belluomini, and H. Lemmi
Introduction Reye's syndrome is a childhood disorder characterized by the rapid onset of fulminating encephalopathy, cerebral edema, and severe systemic metabolic disturbances, sometimes following a nonspecific viral illness.' Fatty infiltration of the viscera is seen at postmortem examination. Early reports indicated a high mortality rate for this disorder. As milder forms of the disease were recognized and management improved, estimates of mortality rate ranged from 30 to 50%.2,3 Prompt detection of neurologic sequelae in survivors of Reye's syndrome is therefore important, particularly for evaluation of new treatments. We recently studied visual evoked potentials (VEPs) in one of our patients who had recovered from Reye's syndrome, and found this simple noninvasive method to be capable of detecting subtle lesions of the visual system. Case Report S.H., a 14-year-old girl, was admitted to the LeSonheur's Children's Hospital with lethargy, confusion and combativeness of 1 day's duration. She was recovering from a flulike illness that had persisted for about 1 week. On examination she was confused, disoriented and her pupils were slightly dilated, both measured 4 mm in diameter. Papilloedema and loss of pulsations of retinal venules were noted. Hyperreflexia and bilateral extensor plantar responses were also present. The diagnosis of Reye's syndrome was established on the basis of marked elevation of blood ammonia (245 mg/dl), serum glutamic oxalacetic transaminase (210 units/liter), and creatinine phosphokinase (882 units/dl). Cerebrospinal fluid was acellular; protein was 27 mg/dl and glucose 94 mg/dl.
To ensure constant and adequate cerebral perfusion, we inserted a Sokol subarachnoid screw to monitor intracranial pressure and placed a cannula in the radial artery to record systemic arterial pressure. A gradient of more than 40 mm of Hg was maintained throughout the whole course by intravenous bolus administration of mannitol, 1 gm/kg/dose. The patient was in a coma for 4 days. When she regained consciousness, she complained of diplopia, pain and blurred vision of the left eye. On examination, slight ptosis of the left eyelid, normal pupils, intact visual fields, and absence of papilloedema were noted. Visual acuity was 20/20 for both eyes. Three consecutive electroencephalograms (EEG) were obtained and showed progressive improvement (Fig. 1). The first VEP was obtained on the 25th day of illness, or 1 week after the last EEG.
Methods VEPs induced by flash stimulations were recorded by stimulating each eye separately. The other eye was covered with an eye pad. A Grass PS 22 photostimulator, at a flash intensity setting of 8, delivered one flash per second at a distance of 20 centimeters from the eye. One hundred and twenty-eight responses were recorded from occipital (0 , and O 2 ), parietal (P 3 and P 4 ) and vertex (C 2 ) areas referencing to ipsilateral ears (A 1 and A 2 ), and averaged by a Nicolet 1080 computer.
From the Section of Neurology, SI. Jude Children's Research Hospital, and the Neurophysiology Laboratory, Baptist Memorial Hospital, Memphis Tennessee. Partly presented at the 30th Annual Southern EEG Society Meeting, Chattanooga, Tennessee, October 27-29, 1977. Reprints requests should be sent to Lawrence T. Ch'ien, M.D., SI. Jude Children's Research Hospital, 332 N. Lauderdale, P.O Box 318, Memphis, Te. 38101.
164 Downloaded from eeg.sagepub.com at NORTHWESTERN UNIV LIBRARY on June 5, 2016
CLINICAL ELECTROENCEPHALOGRAPHY
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CLINICAL ELECTROENCEPHALOGRAPHY
Visual Evoked Potentials in Reye's Syndrome L. Ch'ien, J. Belluomini, and H. Lemmi
Introduction Reye's syndrome is a childhood disorder characterized by the rapid onset of fulminating encephalopathy, cerebral edema, and severe systemic metabolic disturbances, sometimes following a nonspecific viral illness.' Fatty infiltration of the viscera is seen at postmortem examination. Early reports indicated a high mortality rate for this disorder. As milder forms of the disease were recognized and management improved, estimates of mortality rate ranged from 30 to 50%.2,3 Prompt detection of neurologic sequelae in survivors of Reye's syndrome is therefore important, particularly for evaluation of new treatments. We recently studied visual evoked potentials (VEPs) in one of our patients who had recovered from Reye's syndrome, and found this simple noninvasive method to be capable of detecting subtle lesions of the visual system. Case Report S.H., a 14-year-old girl, was admitted to the LeSonheur's Children's Hospital with lethargy, confusion and combativeness of 1 day's duration. She was recovering from a flulike illness that had persisted for about 1 week. On examination she was confused, disoriented and her pupils were slightly dilated, both measured 4 mm in diameter. Papilloedema and loss of pulsations of retinal venules were noted. Hyperreflexia and bilateral extensor plantar responses were also present. The diagnosis of Reye's syndrome was established on the basis of marked elevation of blood ammonia (245 mg/dl), serum glutamic oxalacetic transaminase (210 units/liter), and creatinine phosphokinase (882 units/dl). Cerebrospinal fluid was acellular; protein was 27 mg/dl and glucose 94 mg/dl.
To ensure constant and adequate cerebral perfusion, we inserted a Sokol subarachnoid screw to monitor intracranial pressure and placed a cannula in the radial artery to record systemic arterial pressure. A gradient of more than 40 mm of Hg was maintained throughout the whole course by intravenous bolus administration of mannitol, 1 gm/kg/dose. The patient was in a coma for 4 days. When she regained consciousness, she complained of diplopia, pain and blurred vision of the left eye. On examination, slight ptosis of the left eyelid, normal pupils, intact visual fields, and absence of papilloedema were noted. Visual acuity was 20/20 for both eyes. Three consecutive electroencephalograms (EEG) were obtained and showed progressive improvement (Fig. 1). The first VEP was obtained on the 25th day of illness, or 1 week after the last EEG.
Methods VEPs induced by flash stimulations were recorded by stimulating each eye separately. The other eye was covered with an eye pad. A Grass PS 22 photostimulator, at a flash intensity setting of 8, delivered one flash per second at a distance of 20 centimeters from the eye. One hundred and twenty-eight responses were recorded from occipital (0 , and O 2 ), parietal (P 3 and P 4 ) and vertex (C 2 ) areas referencing to ipsilateral ears (A 1 and A 2 ), and averaged by a Nicolet 1080 computer.
From the Section of Neurology, SI. Jude Children's Research Hospital, and the Neurophysiology Laboratory, Baptist Memorial Hospital, Memphis Tennessee. Partly presented at the 30th Annual Southern EEG Society Meeting, Chattanooga, Tennessee, October 27-29, 1977. Reprints requests should be sent to Lawrence T. Ch'ien, M.D., SI. Jude Children's Research Hospital, 332 N. Lauderdale, P.O Box 318, Memphis, Te. 38101.
164 Downloaded from eeg.sagepub.com at NORTHWESTERN UNIV LIBRARY on June 5, 2016
CLINICAL ELECTROENCEPHALOGRAPHY
DAva ...... .,...,.,
@1979.VOL.10,NO.3
10
"10-.10
1-70"
~'~~"-''''''''''~\''--'~'v'''~~~V\,J'v-J~V~ 0,-4, o.-Aa
. - ..........
a.H. • 7'70408 t4 y ... WP 3/1/n
...
....
F,-1S
~-
Fk-F•
,
---....---.-
."
._ ~~~,~.,~iIT1 ,,, 1\-14
of. .. ''' . . . . . . . . 4.,.......
1f'Ih/tf
