muscle and heart enzymes.2In these conditions, a newly-formed intermediate band appeared in the electropherogram (figure 2, lane 31, which migrated to the same position as the intermediate band of normal human heart (figure 2, lane 2) and was not formed when the imidazole-citrate buffer was omitted from the medium (figure 2, lane 4). In agreement with the electrophoretic data, immunologic stutdiers (figure 3) showed that antiserum against purified skeletal muscle phosphorylase, in amlounts that completely inhibited enzyme activity of human muscle extracts, inhibited normal heart p hosphorylase by only approximately 50% and the patient’s heart phosphorylase by less than 10%. The discrepance between the major inhibition of normal heart phosphorylase activity and the faint muscle band seen by electrophoresis suggests that antibodies react not only with muscle isoenzyme but also with muscle-heart hybrid in cardiac extracts. On the other hand, the small degree of inhibition of the patient’s heart phosphorylase can be attributed to the structural similarity of all phonphclrylase i~oenzymes.~ The results of antibody inhilbition indicate that in normal human heart the lcardliac isoenzyme accounts for at least half of the total phosphorylase activity and is under separate genetic control from the muscle isoenzyme. This can explain the lack of symptoms or signs of heart disease in patients with myophosphorylase deficiency.
Acknowledgments The authors are grateful to Mr. Gomer Pound for technical assistance, Ms. Rhonda Bunin for typing the manuscript, and Drs. James Miller, Hannu Somer, and Lewis P. Rowland for their advice.
Virruid evoked potentials in infants with hydrocephalus
From the H. Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Diseases, Departments of Pathology (Drs. Miranda and Nette) and Neurology (Dr. DiMauro), Columbia University College of Physicians and Surgeons,and the NeurologicalInstitute, Presbyterian Hospital, New York, and the Departments of Pediatrics and Neurology, Medical College of Georgia, Augusta, Georgia (Dr. Hartlage). Supported by Center grant No. NS-11766 from the National Institute of Neurological and Communicative Disorders and Stroke and the Muscular Dystrophy Association. Accepted for publication April 17, 1979. Address correspondence t o Dr. Dilklauro,College of Physicians and Surgeons of Columbia University, 630 West 168 Street, New York, NY 10032.
References 1. Yunis AA,Fischer EH, Krebs EG: Comparative studies on glycogen phosphorylase:Piurification and properties of rabbit heart phosphorylase. J Biol Chem 237:2809-2815, 1962 2. Davis CH, SchliselfeldLH, Wolf DP, et al:Interrelationships among glycogen phosphorylase isozymes. J Biol Chem 242:4824-4833, 1967 3. Schliselfeld LH: Comparative studies of phosphorylase isozymes from the rabbit, Ann NY Acad Sci 210:181-191, 1973 4. Will H, Krause EG, Biihm M, et al: Kinetische Eigenschatten der Isoenzyme der Glykogm-phosphorylase b aus Herzund Skelettmuskulatur des Menschen. Acta Biol Med Ger 33:149-160, 1974 5. DiMauro S,Arnold S, Miraiida A, et al: McArdle disease: The mystery of reappearing pliosphorylase activity in muscle culture: A fetal isoenzyme Ann Neurol 3:60-66, 1978 6. DiMauro S: Storage-metabolic myopathies. In Vinken PJ, Bruyn GW (Editors): Handbook of Clinical Neurology. Amsterdam,NorthHollandPublishing Co.,vol. 41 (in press) 7. DiMauro S , Hartlage PL: Fatal infantile form of muscle phosphorylase deficiency. Neurology 28:1124-1129, 1978 8. Layzer RB, Rowland LP,Rmney HM: Muscle phosphofi-uctokinase deficiency. Arch Neurol 17:512-523, 1967
Article a b s t r a c t v i s u a l evoked potentials to flash stimuli were recorded in 15 infants with hydrocephalus. All demonstrated increased latencies for the prominent positive component (Pz)of the response, compared to the mean values for age-matched controls. In nine infants studied prior to and 1 week after a shunt procedure, the Pz latency decreased. NEUROLOGY 29: 1541-1544, November 1979
Albert Ehle and Frederick Sklar Currently available techniques for the evaluation of cortical function in infancy and early childhood are limited. Diseases can have profound effects upon the cerebral cortex and later behavior or intellectual function without immediate observable changes in behavior or in the results of traditional diagnostic techniques. Although the devel-
opment of computerized tomography (CT)has significantly enhanced the detection of morphologic abnormalities, this technique cannot evaluate the functional status of the brain. However, evoked cortical potentials in the and somatosensory2j6 systems are readily detected in premature and term infants, and provide a method for
November 1979 NEUROLOGY 29 1511
Visual potentials in hydrocephalic infants
320
1
, 1
-6
'
1
-2
1
1
2
1
1
6
1
1
10
1
1
1
14
1
18
1
1
1
22
AGE IN WEEKS FROM TERM
Figure 1. Mean latency of Pz and range for normal infants (stippled area) and values observed in hydrocephalic patients (closed circles). Connected points indicate pre- and postoperative studies on the same patient.
assessment of cortical function. Visual evoked responses mature rapidly in the perinatal per i ~ d . ' , ~ -As ~ , a' potential technique for the evaluation of cortical function in infants, visual evoked responses offer the advantages of a short and defined peripheral pathway, ease of evocation and recording, and established normal physiology and development. For these reasons, we used visual evoked potentials t o study infants with hydrocephalus to evaluate their sensitivity for detecting abnormality in a potentially reversible disease process.
Methods. Visual evoked potentials were recorded from 0 1 and 02 locations of the international 10-20 electrode placement system referentially to C,. A 1- to 60-Hz bandpass was used. Responses were averaged with a total analysis time of 500 msec with either a Nicolet 1072 or a Med-80 computer system. Stimuli were delivered by a Grass PS-22 photic stimulator at a n intensity setting of 8, placed 25 cm from the eyes. Stimulus rates of 0.25 Hz were used for all premature infants, and 64 stimuli were averaged. In term or older infants, 128 stimuli were averaged at stimulation rates of 0.5 or 1 Hz. At least two separate trials were obtained, and the wave forms were compared for reproducibility. Reproducibility was gauged by general similarity of wave form, and by repeated measures of the latency of the major positive wave (Pz)being within 10 msec. Because of alterations in the evoked response that may occur with change in consciousness,8 all recording sessions were started while the infant was awake, and intermit-
1542 NEUROLOGY 29 November 1879
tent somatic or auditory stimuli were used if necessary to maintain arousal. Responses were analyzed by identifying Pz and measuring the latency to peak from the time of the flash. The mean value of the repeated measures was used. This wave was chosen because it is easily identified, and because it is the most stable of the latency measures in infants8 and is present in premature as well as term infants. We studied 15 infants with hydrocephalus documented by either CT or pneumoencephalogram. Infants with a history of meningitis were excluded. In 9 of the 15, follow-up studies were obtained 1 week after a CSF shunting procedure was performed. Twenty-three normal infants ranging in estimated age from 6 weeks premature to term were studied with the same techniques. The mean value for the PZlatency of the 10 infants of 39 to 42 weeks gestational age was 172 msec (range, 160 to 220 msec). This value and the values obtained for younger infants were similar to the results of 0thers.*.~*9 Our results were combined with these prior studies of larger numbers and greater age ranges to obtain a mean and range for normals (figure 1).
Results. In all 15 infants with hydrocephalus, the
Pz latency was greater than the mean value for age-matched normal controls (figure 1).This finding was significant at t h e p < 0.05 level. Nine of the 15 had values at or above the upper limits of normal. Most responses recorded from left and right hemispheres were symmetrical, but there were marked asymmetries in two cases (figure 2). In one (MG), abnormality was associated with CT evidence of interventricular hemorrhage from the left hemisphere. In the second, similar asymmetry was noted in the initial examination of a 17week-old patient (figure 2, LM), no lateralized abnormality was present on CT, and the asymmetry diminished after shunting. In 9 of the 15 patients, studies were obtained 1 or 2 days prior to shunt placement, and then repeated 1 week after surgery. In all, the Pz latency decreased postoperatively. These changes were all similar and approximately equal to the rapid rate of change seen in normals between 2 and 8 weeks. In addition to the change in PZlatency from shunting (figure 1) other alterations in wave form were observed (figure 3). Generally, the latencies of later waves of the evoked potential also decreased, but were not analyzed in detail.
Discussion. These data indicate that anatomic changes in the hydrocephalic infant are frequently accompanied by functional abnormality as measured by a change in the latency of the most stable component of the visual evoked potential. Prompt normalization of function appears within the first
1
-
Figure 2. Asymmetries observed in two infants with hydrocephalus; negativity at occiput is upward. Pz latencies are indicated by the arrow, except that a definite P2 could not be identified for MG at 0 1 .
Figure 3. Examples of pre- and postshunt evoked potentials obtained in infants of varying ages. Negativity at occiput is upward. P2 latency is indicated by arrow.
week after a shunt procedure. The localization of the insult that causes these changes is uncertain. The flash-evoked potential is relatively insensitive to pathology affecting the anterior visual pathways, suggesting that the insult is in the postgeniculate optic radiations or occipital cortex.l0 Because of the long pathway and its close relationship t o the ventricular system, compromise of the optic radiations could be responsible in part for the abnormality. Direct effects on occipital cortex are also possible, because the occipital horns of the ventricular system typically demonstrate the most prominent dilation in infantile hydrocephalus. A major handicap for clinical application of visual evoked potentials is the rapid maturational change during lale fetal development and the first 6 weeks of normal extrauterine growth. These changes involve both decreases in latency and in-
creasing complexity of the evoked potential wave form. Additionally, estimates of gestational age are imprecise. For these!reasons, only large deviations from normal mean values indicate abnormality.8 However, because of interaction with these early rapid maturational changes, chronic or subacute disease processes (such as hydrocephalus) may cause more abnormality than would be observed after an isolated acute insult. Although the normal variability of the infantile visual evoked potential may limit its utility as an individual diagnostic test, our results indicate that the test is useful in detecting dysfunction and monitoring responses tci therapy in infants at risk for cerebral injury. Hrbek and and Graziani, Weitzman, and Pineda13 also found visual evoked response useful in studying infants with hypoxic or anoxic insults, with similar initial abnormality and normalization after therapy. November 1979 NEUROLOGY 29 1543
Visual potentials in hydrocephalic infants
From the Departments of Neurology and Neurosurgery, Southwestern Medical School, University of Texas Health Science Center a t Dallas, Dallas, Texas. Accepted for publication April 11, 1979 Address reprint requests to Dr. Ehle, Department of Neurology, University of Texas Health Science Center at Dallas, 5323 Harry Hines Boulevard, Dallas, TX 75235.
Acknowledgments The authors would like to thank Mr. Fred Levingston, Miss Cara East, and Mr. Arthur Leventhal for technical assistance in these studies and Miss Leslie Sheaffer for manuscript preparation.
References 1. Ellingson RJ:Cortical electrical responses to visual stimulation i n the human infant. Electroencephalogr Clin 1960 Neurophysiol 12:663-677, 2. Hrbek A, Mares P: Cortical evoked responses to visual stimulation in full-term and premature newborns. Electroencephalogr Clin Neurophysiol 16:575-581,1964 3.F e r r i s GS, Davis GD, Dorsen MMcF, e t al: Changes in latency and form of the photically induced average evoked response i n human infants. Electroencephalogr Clin Neurophysiol 22:305-312,1967
Progressive supranuclear palsy and normal-pressure hydrocephalus
4.Engel R,Butler BV: Appraisal of conceptual age of newborn infants by electroencephalographic methods. J Pediatr 63:386-393,1963 5. Umezaki H, Morel1 F: Developmental study of photic evoked responses in premature infants. Eledroencephalogr Clin Neurophysiol 2855-63,1970 6.Desmedt J E , Brunko E, Debecker J: Maturation of the somatosensory evoked potentials in normal infants and children with special reference to the early Ni component. Electroencephalogr Clin Neurophysiol 40:43-58,1976 7. Karlberg P, Olsson T: Development of visual a n d somatosensory evoked responses in preterm infants. Electroencephalogr Clin Neurophysiol 34:225-232,1973 8. Ellingson RG: Variability of visual evoked responses in the human newborn. Electroencephalogr Clin Neurophysiol 29:lO-19.1970 9.Ellingson w: The study of brain electrical activity of infants. In Lipsitt LP, Spiker CC (Editors): Advances in Child Development and Behavior. New York, Academic Press, 1967,V O ~3, pp 64-98 10.Halliday AM, McDonald WI, Mushin J : Visual evoked potentials in patients with demyelinating disease. In Desmedt JE (Editor): New Developments in Visual Evoked Potentials in the Human Brain. London, Oxford University Press, 1977,pp 438-449 11.Hrbek A, Karlberg P, Kjellmer I, et al: Clinical application of evoked EEG responses in newborn infants: I. Perinatal asphyxia. Dev Med Child Neurol 19:34-44,1977 12.Hrbek A, Karlberg P, Kjellmer I, et al: Clinical application of evoked EEG responses in newborn infants: 11. Idiopathic respiratory distress syndrome. Dev Med Child Neurol 20:619-626,1978 13. Graziani U,Weitzman ED, Pineda G Visual evoked responses during neonatal respiratory disorders in low birth weight infants. Pediatr Res 6:203-210,1972
Article abstract-In three patients who fulfilled the clinical criteria of progressive supranuclear palsy, radiologic investigations suggested normal-pressure hydrocephalus. Shunt procedures in all three resulted in temporary improvement of gait, mentation, and bladder control, but gaze paralysis and extrapyramidal findings did not change. NEUROLOGY 29: 1544-1546. November 1979
Mircea A. Morariu, M.D.
Normal-pressure hydrocephalus (NPH) may follow subarachnoid hemorrhage,lS2t r a ~ r n a or ,~ chronic m e n i n g i t i ~It . ~has also been described in of the basilar artery,6 Alzheimer d i ~ e a s eectasia ,~ and hypertensive cerebral vascular d i ~ e a s eMost .~ cases, however, are i d i o p a t h i ~ .Progressive ~.~ supranuclear palsy (PSP)is a condition of unknown etiology in which antecedent encephalitis or viral infection has been suspected.10 NPH and PSP have many common clinical features. Unsteady gait and deterioration of mental functions were found in all 50 patients with NPH studied by Adams.BUrinary incontinence is usually a late and less constant manifestation. Gait unsteadiness and dementia are also manifestations of PSP, together with the vertical gaze paralysis and abnormal postures that are characteristic.1° Bilateral pyramidal signs, rigidity, and 1544 NEUROLOGY 29 November 1979
bradykinesia a r e encountered i n both syndromes.8~10*11 We recently observed three patients who fulfilled the clinical criteria of PSP, but in whom investigations revealed hydrocephalus. This association has not been previously reported. Case 1. This man was well until age 72, when his voice
seemed hoarse, One year later his gait became unsteady and he tended to fall backwards. Consciousness was retained. Dizziness and vertigo were denied. On occasion, he was incontinent of urine. On admission, he was alert and oriented with flat affect and a hoarse, monotonous voice. Blinking was infrequent, and there was hypomimia. Upward gaze was markedly impaired. Downward gaze and convergence were slightly restricted. Horizontal eye movements were full. His gait was slow and unsteady, with diminished associated movements of arms. There was
Virruid evoked potentials in infants with hydrocephalus Albert Ehle and Frederick Sklar Neurology 1979;29;1541 DOI 10.1212/WNL.29.11.1541 This information is current as of November 1, 1979 Updated Information & Services
including high resolution figures, can be found at: http://n.neurology.org/content/29/11/1541.full.html
Citations
This article has been cited by 2 HighWire-hosted articles: http://n.neurology.org/content/29/11/1541.full.html##othe rarticles
Permissions & Licensing
Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://n.neurology.org/misc/about.xhtml#permissions
Reprints
Information about ordering reprints can be found online: http://n.neurology.org/misc/addir.xhtml#reprintsus
Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1979 by the American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
Acknowledgments The authors are grateful to Mr. Gomer Pound for technical assistance, Ms. Rhonda Bunin for typing the manuscript, and Drs. James Miller, Hannu Somer, and Lewis P. Rowland for their advice.
Virruid evoked potentials in infants with hydrocephalus
From the H. Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Diseases, Departments of Pathology (Drs. Miranda and Nette) and Neurology (Dr. DiMauro), Columbia University College of Physicians and Surgeons,and the NeurologicalInstitute, Presbyterian Hospital, New York, and the Departments of Pediatrics and Neurology, Medical College of Georgia, Augusta, Georgia (Dr. Hartlage). Supported by Center grant No. NS-11766 from the National Institute of Neurological and Communicative Disorders and Stroke and the Muscular Dystrophy Association. Accepted for publication April 17, 1979. Address correspondence t o Dr. Dilklauro,College of Physicians and Surgeons of Columbia University, 630 West 168 Street, New York, NY 10032.
References 1. Yunis AA,Fischer EH, Krebs EG: Comparative studies on glycogen phosphorylase:Piurification and properties of rabbit heart phosphorylase. J Biol Chem 237:2809-2815, 1962 2. Davis CH, SchliselfeldLH, Wolf DP, et al:Interrelationships among glycogen phosphorylase isozymes. J Biol Chem 242:4824-4833, 1967 3. Schliselfeld LH: Comparative studies of phosphorylase isozymes from the rabbit, Ann NY Acad Sci 210:181-191, 1973 4. Will H, Krause EG, Biihm M, et al: Kinetische Eigenschatten der Isoenzyme der Glykogm-phosphorylase b aus Herzund Skelettmuskulatur des Menschen. Acta Biol Med Ger 33:149-160, 1974 5. DiMauro S,Arnold S, Miraiida A, et al: McArdle disease: The mystery of reappearing pliosphorylase activity in muscle culture: A fetal isoenzyme Ann Neurol 3:60-66, 1978 6. DiMauro S: Storage-metabolic myopathies. In Vinken PJ, Bruyn GW (Editors): Handbook of Clinical Neurology. Amsterdam,NorthHollandPublishing Co.,vol. 41 (in press) 7. DiMauro S , Hartlage PL: Fatal infantile form of muscle phosphorylase deficiency. Neurology 28:1124-1129, 1978 8. Layzer RB, Rowland LP,Rmney HM: Muscle phosphofi-uctokinase deficiency. Arch Neurol 17:512-523, 1967
Article a b s t r a c t v i s u a l evoked potentials to flash stimuli were recorded in 15 infants with hydrocephalus. All demonstrated increased latencies for the prominent positive component (Pz)of the response, compared to the mean values for age-matched controls. In nine infants studied prior to and 1 week after a shunt procedure, the Pz latency decreased. NEUROLOGY 29: 1541-1544, November 1979
Albert Ehle and Frederick Sklar Currently available techniques for the evaluation of cortical function in infancy and early childhood are limited. Diseases can have profound effects upon the cerebral cortex and later behavior or intellectual function without immediate observable changes in behavior or in the results of traditional diagnostic techniques. Although the devel-
opment of computerized tomography (CT)has significantly enhanced the detection of morphologic abnormalities, this technique cannot evaluate the functional status of the brain. However, evoked cortical potentials in the and somatosensory2j6 systems are readily detected in premature and term infants, and provide a method for
November 1979 NEUROLOGY 29 1511
Visual potentials in hydrocephalic infants
320
1
, 1
-6
'
1
-2
1
1
2
1
1
6
1
1
10
1
1
1
14
1
18
1
1
1
22
AGE IN WEEKS FROM TERM
Figure 1. Mean latency of Pz and range for normal infants (stippled area) and values observed in hydrocephalic patients (closed circles). Connected points indicate pre- and postoperative studies on the same patient.
assessment of cortical function. Visual evoked responses mature rapidly in the perinatal per i ~ d . ' , ~ -As ~ , a' potential technique for the evaluation of cortical function in infants, visual evoked responses offer the advantages of a short and defined peripheral pathway, ease of evocation and recording, and established normal physiology and development. For these reasons, we used visual evoked potentials t o study infants with hydrocephalus to evaluate their sensitivity for detecting abnormality in a potentially reversible disease process.
Methods. Visual evoked potentials were recorded from 0 1 and 02 locations of the international 10-20 electrode placement system referentially to C,. A 1- to 60-Hz bandpass was used. Responses were averaged with a total analysis time of 500 msec with either a Nicolet 1072 or a Med-80 computer system. Stimuli were delivered by a Grass PS-22 photic stimulator at a n intensity setting of 8, placed 25 cm from the eyes. Stimulus rates of 0.25 Hz were used for all premature infants, and 64 stimuli were averaged. In term or older infants, 128 stimuli were averaged at stimulation rates of 0.5 or 1 Hz. At least two separate trials were obtained, and the wave forms were compared for reproducibility. Reproducibility was gauged by general similarity of wave form, and by repeated measures of the latency of the major positive wave (Pz)being within 10 msec. Because of alterations in the evoked response that may occur with change in consciousness,8 all recording sessions were started while the infant was awake, and intermit-
1542 NEUROLOGY 29 November 1879
tent somatic or auditory stimuli were used if necessary to maintain arousal. Responses were analyzed by identifying Pz and measuring the latency to peak from the time of the flash. The mean value of the repeated measures was used. This wave was chosen because it is easily identified, and because it is the most stable of the latency measures in infants8 and is present in premature as well as term infants. We studied 15 infants with hydrocephalus documented by either CT or pneumoencephalogram. Infants with a history of meningitis were excluded. In 9 of the 15, follow-up studies were obtained 1 week after a CSF shunting procedure was performed. Twenty-three normal infants ranging in estimated age from 6 weeks premature to term were studied with the same techniques. The mean value for the PZlatency of the 10 infants of 39 to 42 weeks gestational age was 172 msec (range, 160 to 220 msec). This value and the values obtained for younger infants were similar to the results of 0thers.*.~*9 Our results were combined with these prior studies of larger numbers and greater age ranges to obtain a mean and range for normals (figure 1).
Results. In all 15 infants with hydrocephalus, the
Pz latency was greater than the mean value for age-matched normal controls (figure 1).This finding was significant at t h e p < 0.05 level. Nine of the 15 had values at or above the upper limits of normal. Most responses recorded from left and right hemispheres were symmetrical, but there were marked asymmetries in two cases (figure 2). In one (MG), abnormality was associated with CT evidence of interventricular hemorrhage from the left hemisphere. In the second, similar asymmetry was noted in the initial examination of a 17week-old patient (figure 2, LM), no lateralized abnormality was present on CT, and the asymmetry diminished after shunting. In 9 of the 15 patients, studies were obtained 1 or 2 days prior to shunt placement, and then repeated 1 week after surgery. In all, the Pz latency decreased postoperatively. These changes were all similar and approximately equal to the rapid rate of change seen in normals between 2 and 8 weeks. In addition to the change in PZlatency from shunting (figure 1) other alterations in wave form were observed (figure 3). Generally, the latencies of later waves of the evoked potential also decreased, but were not analyzed in detail.
Discussion. These data indicate that anatomic changes in the hydrocephalic infant are frequently accompanied by functional abnormality as measured by a change in the latency of the most stable component of the visual evoked potential. Prompt normalization of function appears within the first
1
-
Figure 2. Asymmetries observed in two infants with hydrocephalus; negativity at occiput is upward. Pz latencies are indicated by the arrow, except that a definite P2 could not be identified for MG at 0 1 .
Figure 3. Examples of pre- and postshunt evoked potentials obtained in infants of varying ages. Negativity at occiput is upward. P2 latency is indicated by arrow.
week after a shunt procedure. The localization of the insult that causes these changes is uncertain. The flash-evoked potential is relatively insensitive to pathology affecting the anterior visual pathways, suggesting that the insult is in the postgeniculate optic radiations or occipital cortex.l0 Because of the long pathway and its close relationship t o the ventricular system, compromise of the optic radiations could be responsible in part for the abnormality. Direct effects on occipital cortex are also possible, because the occipital horns of the ventricular system typically demonstrate the most prominent dilation in infantile hydrocephalus. A major handicap for clinical application of visual evoked potentials is the rapid maturational change during lale fetal development and the first 6 weeks of normal extrauterine growth. These changes involve both decreases in latency and in-
creasing complexity of the evoked potential wave form. Additionally, estimates of gestational age are imprecise. For these!reasons, only large deviations from normal mean values indicate abnormality.8 However, because of interaction with these early rapid maturational changes, chronic or subacute disease processes (such as hydrocephalus) may cause more abnormality than would be observed after an isolated acute insult. Although the normal variability of the infantile visual evoked potential may limit its utility as an individual diagnostic test, our results indicate that the test is useful in detecting dysfunction and monitoring responses tci therapy in infants at risk for cerebral injury. Hrbek and and Graziani, Weitzman, and Pineda13 also found visual evoked response useful in studying infants with hypoxic or anoxic insults, with similar initial abnormality and normalization after therapy. November 1979 NEUROLOGY 29 1543
Visual potentials in hydrocephalic infants
From the Departments of Neurology and Neurosurgery, Southwestern Medical School, University of Texas Health Science Center a t Dallas, Dallas, Texas. Accepted for publication April 11, 1979 Address reprint requests to Dr. Ehle, Department of Neurology, University of Texas Health Science Center at Dallas, 5323 Harry Hines Boulevard, Dallas, TX 75235.
Acknowledgments The authors would like to thank Mr. Fred Levingston, Miss Cara East, and Mr. Arthur Leventhal for technical assistance in these studies and Miss Leslie Sheaffer for manuscript preparation.
References 1. Ellingson RJ:Cortical electrical responses to visual stimulation i n the human infant. Electroencephalogr Clin 1960 Neurophysiol 12:663-677, 2. Hrbek A, Mares P: Cortical evoked responses to visual stimulation in full-term and premature newborns. Electroencephalogr Clin Neurophysiol 16:575-581,1964 3.F e r r i s GS, Davis GD, Dorsen MMcF, e t al: Changes in latency and form of the photically induced average evoked response i n human infants. Electroencephalogr Clin Neurophysiol 22:305-312,1967
Progressive supranuclear palsy and normal-pressure hydrocephalus
4.Engel R,Butler BV: Appraisal of conceptual age of newborn infants by electroencephalographic methods. J Pediatr 63:386-393,1963 5. Umezaki H, Morel1 F: Developmental study of photic evoked responses in premature infants. Eledroencephalogr Clin Neurophysiol 2855-63,1970 6.Desmedt J E , Brunko E, Debecker J: Maturation of the somatosensory evoked potentials in normal infants and children with special reference to the early Ni component. Electroencephalogr Clin Neurophysiol 40:43-58,1976 7. Karlberg P, Olsson T: Development of visual a n d somatosensory evoked responses in preterm infants. Electroencephalogr Clin Neurophysiol 34:225-232,1973 8. Ellingson RG: Variability of visual evoked responses in the human newborn. Electroencephalogr Clin Neurophysiol 29:lO-19.1970 9.Ellingson w: The study of brain electrical activity of infants. In Lipsitt LP, Spiker CC (Editors): Advances in Child Development and Behavior. New York, Academic Press, 1967,V O ~3, pp 64-98 10.Halliday AM, McDonald WI, Mushin J : Visual evoked potentials in patients with demyelinating disease. In Desmedt JE (Editor): New Developments in Visual Evoked Potentials in the Human Brain. London, Oxford University Press, 1977,pp 438-449 11.Hrbek A, Karlberg P, Kjellmer I, et al: Clinical application of evoked EEG responses in newborn infants: I. Perinatal asphyxia. Dev Med Child Neurol 19:34-44,1977 12.Hrbek A, Karlberg P, Kjellmer I, et al: Clinical application of evoked EEG responses in newborn infants: 11. Idiopathic respiratory distress syndrome. Dev Med Child Neurol 20:619-626,1978 13. Graziani U,Weitzman ED, Pineda G Visual evoked responses during neonatal respiratory disorders in low birth weight infants. Pediatr Res 6:203-210,1972
Article abstract-In three patients who fulfilled the clinical criteria of progressive supranuclear palsy, radiologic investigations suggested normal-pressure hydrocephalus. Shunt procedures in all three resulted in temporary improvement of gait, mentation, and bladder control, but gaze paralysis and extrapyramidal findings did not change. NEUROLOGY 29: 1544-1546. November 1979
Mircea A. Morariu, M.D.
Normal-pressure hydrocephalus (NPH) may follow subarachnoid hemorrhage,lS2t r a ~ r n a or ,~ chronic m e n i n g i t i ~It . ~has also been described in of the basilar artery,6 Alzheimer d i ~ e a s eectasia ,~ and hypertensive cerebral vascular d i ~ e a s eMost .~ cases, however, are i d i o p a t h i ~ .Progressive ~.~ supranuclear palsy (PSP)is a condition of unknown etiology in which antecedent encephalitis or viral infection has been suspected.10 NPH and PSP have many common clinical features. Unsteady gait and deterioration of mental functions were found in all 50 patients with NPH studied by Adams.BUrinary incontinence is usually a late and less constant manifestation. Gait unsteadiness and dementia are also manifestations of PSP, together with the vertical gaze paralysis and abnormal postures that are characteristic.1° Bilateral pyramidal signs, rigidity, and 1544 NEUROLOGY 29 November 1979
bradykinesia a r e encountered i n both syndromes.8~10*11 We recently observed three patients who fulfilled the clinical criteria of PSP, but in whom investigations revealed hydrocephalus. This association has not been previously reported. Case 1. This man was well until age 72, when his voice
seemed hoarse, One year later his gait became unsteady and he tended to fall backwards. Consciousness was retained. Dizziness and vertigo were denied. On occasion, he was incontinent of urine. On admission, he was alert and oriented with flat affect and a hoarse, monotonous voice. Blinking was infrequent, and there was hypomimia. Upward gaze was markedly impaired. Downward gaze and convergence were slightly restricted. Horizontal eye movements were full. His gait was slow and unsteady, with diminished associated movements of arms. There was
Virruid evoked potentials in infants with hydrocephalus Albert Ehle and Frederick Sklar Neurology 1979;29;1541 DOI 10.1212/WNL.29.11.1541 This information is current as of November 1, 1979 Updated Information & Services
including high resolution figures, can be found at: http://n.neurology.org/content/29/11/1541.full.html
Citations
This article has been cited by 2 HighWire-hosted articles: http://n.neurology.org/content/29/11/1541.full.html##othe rarticles
Permissions & Licensing
Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://n.neurology.org/misc/about.xhtml#permissions
Reprints
Information about ordering reprints can be found online: http://n.neurology.org/misc/addir.xhtml#reprintsus
Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright © 1979 by the American Academy of Neurology. All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
