437
The Japanese Journal of Psychiatry and Neurology, Vol. 46, No. 2, 1992
Polysomnographical Study in the Patients with West Syndrome before Treatment Masayuki Shimohira, M.D., Satoshi Araki, M.D., Satoko Kumada, M.D., Masahiro Ito, M.D. and Yoshihide Iwakawa, M.D. Department of Pediatrics, Tokyo Medical and Dental University, Tokyo
The close relationship between the underlying brainstem abnormality of patients with West syndrome (WS) and sleep disorder has been pointed out. From the pathophysiological point of view, we performed some polysomnographies (PSGs) on the patients with WS before anticonvulsive treatment and analyzed the relationship between sleep parameters and the prognosis of the patients. Subjects and Methods The profile of six patients with typical WS is shown in Table 1. These patients have been followed-up for about 7 to 8 years after the ACTH therapy and three of them (group A) have a good prognosis for seizure, while the remaining patients (group B) have a poor prognosis and still have intractable seizures. Each PSG consisted of several electroencephalograms, horizontal electrooculo-
grams (EOGs), respiration, surface electromyograms of the chin, abdominal rectal and some limb muscles. Sleep stages were decided every minute according to the standards of the Association for Psychophysiological Study of Sleep.' Body movements (BMs) during sleep were classified into three types; gross movements (GMs), localized movements (LMs) and twitch movements (TMs). The percentages of the number of LMs or TMs that occurred with the REMs-bursts to the total number of LMs or TMs during SREM (%LMs-R or %TMs-R) and the Dissociation Index (DI), which is the ratio of the number of TMs against the number of (LMs TMs), were detected. Detailed definitions of the BMs, %LMs-R, %TMs-R and DIs were previously reported.* The rising angles of the rapid eye movements (REMs) on the EOGs were detected over 50 degrees on the paper speed of 1.5 cm/sec. PSGs were obtained for each patient before the anticonvulsive treatment. We compared them with some healthy and agematched children as controls.
+
Results Percent Sleep Stages
The average percentages of stage REM (%SREM) showed statistically significant
Table 1: Patient Profiles Group
A
Case No. 1 2
3 4 5
B
6 7 8
Age/Sex lly 9y 8y 7y
6m/m Im/f 9m/f 8m/f
1Oy 3m/f 9y 6m/m 8y llm/m 4y 3m/f
Age at Onset
Age at PSG
Neurological Findings
7m 3m 6m 7m
ly lm 5m 6m 8m
N N N N
5m 5m 5m 6m
5m 5m 6m 6m
N DPl N N
cT
Development (DQ)
N
N
N SN N SN
N CMA
(60) (60)
SH
(50) (50)
SE CA
N
N : normal, SE: subdural effusion, CA: cortical atrophy, SN: subnormal, Dpl: diplegia, SH: subdural hematoma, CMA : cortico-medurally atrophy.
Selected Papers: Child Epilepsy
43 8
Oroup
x
A lllr
3020
S l e e p
S t a l e
Sleep
-
0
I I
0
Stale
37-42U 1-41.1 5-1211 Concept weeks
.
I
Q?* T;?;I Sleep
Stale
Sleep
Stale
1-5Y
Fig. 2: %LM in REMs-burst. .Group A*, OGroup B**, tcontrol (mean* lS.D.), * p < 0.05, * * p< 0.02 vs. controls.
Fig. 1 : Gross movements and twitch movements. .Group A, OGroup B, Bcontrol ( m e a n t lS.D.), * p< 0.01, vs. controls.
reductions in both groups A and B (25.3% (p < 0.02), 28.4% (p< 0.03), respectively) as compared with those in normal healthy children (controls; 33.3 f4.5% ).
Body Movements (Fig. 1) Sleep-stage-dependent distributions of BMs: In group A, the distributions of GMs and TMs were slightly abnormal. In group B, those were more disturbed than group A.
Total number of BMs in the whole sleep time: In group A, the total number of GMs and TMs was within the normal range. In group B, the average number of GMs increased and that of TMs decreased. The difference between group B and controls was statistically significant in GMs and TMs. %LMs or %TMs in REMs-burst (%LMs-R or %TMs-R) (Figs. 2 and 3): Compared with controls2, %LMs-R were significantly higher in both groups A and B
301 20
II
0
I
I
37-42U 1-411 Concept weeks
.
I
5-1211
1
1-5V
Fig. 3: %TM in REMs-burst. .Group A, OGroup B*, tcontrol (mean*S.D.), * p< 0.02 vs. controls.
and %TMs-R were also significantly higher in group B. The Dissociation Index (DI)'
In group A, DIs were almost within the
The Japanese Journal of Psychiatry and Neurology, Vol. 46, No. 2, 1992 normal range or slightly decreased, while they were variously dispersed in group B. Discussion The distributions and the total number of BMs during sleep were thought to be related to the striato-nigral dopaminergic ~ y s t e m .LMs, ~ TMs, %LMs-R, %TMs-R and DIs reflect the function and maturation of the brainstem.2 The results of the present study revealed that BMs, %LMs-R and %TMs-R had some significant relationship to the seizure prognosis of West syndrome. We also emphasize that both groups A and B showed almost the Same abnormalities of some sleep parameters. In patients with WS, the pathological and clinico-pharmacological abnormalities of the dorsal pontine tegmental field’ and the cholinergic system6 were reported. These reports suggest that the brainstem disturbance in WS patients was related to the sleep parameters. We speculate that the WS patients would have the abnormalities of several systems in the brainstem as well as the dopaminergic system in the CNS and these would be the underlining mechanism of the characteristic seizures in WS.
439
References 1. Rechtschaffen, A. and Kales, A.: A manual
of standardized terminology, techniques and scoring system for sleep stages of human subjects. U.S. Government printing office, Washington, D.C., 1968. 2. Kohyama, J. and Iwakawa, Y.: Polygraphic features of a victim of sudden infant death syndrome and of infants with apparent lifethreatening event. Brain Dev (Tokyo) 11: 186-190, 1989.
3. Kohyama, J. and Iwakawa, Y.: Develop-
mental changes in phasic sleep parameters as reflections of the brain-stem maturation: Polysomnographical examinations of infants, including premature neonates. Electroenceph Clin Neurophysiol 7 6 325-330, 1990. 4. Segawa, M., Nomura, Y., Hakamada, S., Nagata, E., Sakamoto, M. and Oka, N.: Polysomnography-Functional topographical examination of the basal ganglia. Brain Dev (Tokyo) 8: 475-481, 1986. 5. Sato, J., Mizutani, T. and Morimatsu, Y.: Neuropathology of the brainstem in agedependent epileptic encephalopathy-Especially of cases with infantile spasms. Brain Dev (Tokyo) 8: 443-449, 1986. 6. Rektor, I., Svejdovh, M. and Mtnini, C.: Cholinergic system disturbance in the West syndrome. Brain Dev (Tokyo) 12: 790-794, 1990.
The Japanese Journal of Psychiatry and Neurology, Vol. 46, No. 2, 1992
Polysomnographical Study in the Patients with West Syndrome before Treatment Masayuki Shimohira, M.D., Satoshi Araki, M.D., Satoko Kumada, M.D., Masahiro Ito, M.D. and Yoshihide Iwakawa, M.D. Department of Pediatrics, Tokyo Medical and Dental University, Tokyo
The close relationship between the underlying brainstem abnormality of patients with West syndrome (WS) and sleep disorder has been pointed out. From the pathophysiological point of view, we performed some polysomnographies (PSGs) on the patients with WS before anticonvulsive treatment and analyzed the relationship between sleep parameters and the prognosis of the patients. Subjects and Methods The profile of six patients with typical WS is shown in Table 1. These patients have been followed-up for about 7 to 8 years after the ACTH therapy and three of them (group A) have a good prognosis for seizure, while the remaining patients (group B) have a poor prognosis and still have intractable seizures. Each PSG consisted of several electroencephalograms, horizontal electrooculo-
grams (EOGs), respiration, surface electromyograms of the chin, abdominal rectal and some limb muscles. Sleep stages were decided every minute according to the standards of the Association for Psychophysiological Study of Sleep.' Body movements (BMs) during sleep were classified into three types; gross movements (GMs), localized movements (LMs) and twitch movements (TMs). The percentages of the number of LMs or TMs that occurred with the REMs-bursts to the total number of LMs or TMs during SREM (%LMs-R or %TMs-R) and the Dissociation Index (DI), which is the ratio of the number of TMs against the number of (LMs TMs), were detected. Detailed definitions of the BMs, %LMs-R, %TMs-R and DIs were previously reported.* The rising angles of the rapid eye movements (REMs) on the EOGs were detected over 50 degrees on the paper speed of 1.5 cm/sec. PSGs were obtained for each patient before the anticonvulsive treatment. We compared them with some healthy and agematched children as controls.
+
Results Percent Sleep Stages
The average percentages of stage REM (%SREM) showed statistically significant
Table 1: Patient Profiles Group
A
Case No. 1 2
3 4 5
B
6 7 8
Age/Sex lly 9y 8y 7y
6m/m Im/f 9m/f 8m/f
1Oy 3m/f 9y 6m/m 8y llm/m 4y 3m/f
Age at Onset
Age at PSG
Neurological Findings
7m 3m 6m 7m
ly lm 5m 6m 8m
N N N N
5m 5m 5m 6m
5m 5m 6m 6m
N DPl N N
cT
Development (DQ)
N
N
N SN N SN
N CMA
(60) (60)
SH
(50) (50)
SE CA
N
N : normal, SE: subdural effusion, CA: cortical atrophy, SN: subnormal, Dpl: diplegia, SH: subdural hematoma, CMA : cortico-medurally atrophy.
Selected Papers: Child Epilepsy
43 8
Oroup
x
A lllr
3020
S l e e p
S t a l e
Sleep
-
0
I I
0
Stale
37-42U 1-41.1 5-1211 Concept weeks
.
I
Q?* T;?;I Sleep
Stale
Sleep
Stale
1-5Y
Fig. 2: %LM in REMs-burst. .Group A*, OGroup B**, tcontrol (mean* lS.D.), * p < 0.05, * * p< 0.02 vs. controls.
Fig. 1 : Gross movements and twitch movements. .Group A, OGroup B, Bcontrol ( m e a n t lS.D.), * p< 0.01, vs. controls.
reductions in both groups A and B (25.3% (p < 0.02), 28.4% (p< 0.03), respectively) as compared with those in normal healthy children (controls; 33.3 f4.5% ).
Body Movements (Fig. 1) Sleep-stage-dependent distributions of BMs: In group A, the distributions of GMs and TMs were slightly abnormal. In group B, those were more disturbed than group A.
Total number of BMs in the whole sleep time: In group A, the total number of GMs and TMs was within the normal range. In group B, the average number of GMs increased and that of TMs decreased. The difference between group B and controls was statistically significant in GMs and TMs. %LMs or %TMs in REMs-burst (%LMs-R or %TMs-R) (Figs. 2 and 3): Compared with controls2, %LMs-R were significantly higher in both groups A and B
301 20
II
0
I
I
37-42U 1-411 Concept weeks
.
I
5-1211
1
1-5V
Fig. 3: %TM in REMs-burst. .Group A, OGroup B*, tcontrol (mean*S.D.), * p< 0.02 vs. controls.
and %TMs-R were also significantly higher in group B. The Dissociation Index (DI)'
In group A, DIs were almost within the
The Japanese Journal of Psychiatry and Neurology, Vol. 46, No. 2, 1992 normal range or slightly decreased, while they were variously dispersed in group B. Discussion The distributions and the total number of BMs during sleep were thought to be related to the striato-nigral dopaminergic ~ y s t e m .LMs, ~ TMs, %LMs-R, %TMs-R and DIs reflect the function and maturation of the brainstem.2 The results of the present study revealed that BMs, %LMs-R and %TMs-R had some significant relationship to the seizure prognosis of West syndrome. We also emphasize that both groups A and B showed almost the Same abnormalities of some sleep parameters. In patients with WS, the pathological and clinico-pharmacological abnormalities of the dorsal pontine tegmental field’ and the cholinergic system6 were reported. These reports suggest that the brainstem disturbance in WS patients was related to the sleep parameters. We speculate that the WS patients would have the abnormalities of several systems in the brainstem as well as the dopaminergic system in the CNS and these would be the underlining mechanism of the characteristic seizures in WS.
439
References 1. Rechtschaffen, A. and Kales, A.: A manual
of standardized terminology, techniques and scoring system for sleep stages of human subjects. U.S. Government printing office, Washington, D.C., 1968. 2. Kohyama, J. and Iwakawa, Y.: Polygraphic features of a victim of sudden infant death syndrome and of infants with apparent lifethreatening event. Brain Dev (Tokyo) 11: 186-190, 1989.
3. Kohyama, J. and Iwakawa, Y.: Develop-
mental changes in phasic sleep parameters as reflections of the brain-stem maturation: Polysomnographical examinations of infants, including premature neonates. Electroenceph Clin Neurophysiol 7 6 325-330, 1990. 4. Segawa, M., Nomura, Y., Hakamada, S., Nagata, E., Sakamoto, M. and Oka, N.: Polysomnography-Functional topographical examination of the basal ganglia. Brain Dev (Tokyo) 8: 475-481, 1986. 5. Sato, J., Mizutani, T. and Morimatsu, Y.: Neuropathology of the brainstem in agedependent epileptic encephalopathy-Especially of cases with infantile spasms. Brain Dev (Tokyo) 8: 443-449, 1986. 6. Rektor, I., Svejdovh, M. and Mtnini, C.: Cholinergic system disturbance in the West syndrome. Brain Dev (Tokyo) 12: 790-794, 1990.
