Advertisement

Polysomnographical assessment of the pathophysiology of West syndrome

      Abstract

      In this brief review, the sleep studies on patients with West syndrome (WS) were summarized. In addition to the previously reported common finding for sleep in WS – reduction of the amount of rapid-eye-movement (REM) sleep – weakness of phasic suppression of chin muscle activity in WS patients has recently been found. The degree of this weakness is quantified by the phasic inhibition index (PII), which has been found to reflect a patient's prognosis as to convulsions. PII is proposed to be a useful parameter for assessing the prognosis of WS. Since the pontine tegmentum is involved in the production of the REM-related phasic loss of muscle activity in REM sleep, WS patients are hypothesized to have a functional instability of the pontine tegmentum. After adrenocorticotropin (ACTH) treatment, PII decreased significantly in all WS patients examined. Taken together with the effects of corticosteriods on PII, and the incidence of phasic chin muscle activity in patients with congenital adrenal hyperplasia and nephrotic syndrome, ACTH is hypothesized to suppress the spasms in WS patients not only through corticosteroids, but also through a direct action on the pontine tegmentum. Since PII has been reported to be elevated in patients with an autistic tendency, the appearance of an autistic tendency is also hypothesized to be involved in the functional disturbance of the pontine tegmentum.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Brain and Development
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • West W.J.
        On a peculiar form of infantile convulsions.
        Lancet. 1841; i: 724-725
        • Appleton R.E.
        Infantile spasms.
        Arch Dis Child. 1993; 69: 614-618
        • Shionaga A.
        • Iida Y.
        • Fukuyama Y.
        Polygraphical all night EEG in infantile spasms (in Japanese).
        Shouni-Geka Naika (Tokyo). 1976; 8: 1415-1423
        • Fukuyama Y.
        • Shionaga A.
        • Iida Y.
        Polygraphic study during whole night sleep in infantile spasms.
        Eur Neurol. 1979; 18: 302-311
        • Hashimoto T.
        • Ohara K.
        • Hiura K.
        • Suzue J.
        • Kawano N.
        • Kobayashi Y.
        • et al.
        Sleep polygraphic studies on infantile spasms.
        Acta Paediatr Jpn (Tokyo). 1978; 20: 1-7
        • Horita H.
        Overnight polygraphic studies of age-dependent epilepsy in infancy: part 1. Sleep states and changes in pulse, respiration and seizure activities during sleep states (in Japanese).
        No To Hattatsu (Tokyo). 1979; 11: 229-246
        • Hrachovy R.A.
        • Frost Jr, J.D.
        • Kellaway P.
        Sleep characteristics in infantile spasms.
        Neurology. 1981; 31: 688-694
        • Hashimoto T.
        • Hiura K.
        • Suzue J.
        • Kokawa T.
        • Fukuda K.
        • Endo S.
        • et al.
        Influence of ACTH therapy on overnight sleep polygrams in infantile spasms.
        Brain Dev (Tokyo). 1981; 3: 51-56
        • Okawa M.
        • Sasaki H.
        Sleep disorders in mentally retarded and brain-impaired children.
        in: Guilleminault C. Sleep and its disorders in children. Raven Press, New York, NY1987: 269-290
        • Kohyama J.
        Sleep as a window on the developing brain.
        Curr Probl Pediatr. 1998; 28: 73-92
        • Maquet P.
        • Péters J-M.
        • Aerts J.
        • Delfiore G.
        • Degueldre C.
        • Luxen A.
        • et al.
        Functional neuroanatomy of human rapid-eye-movement sleep and dreaming.
        Nature. 1996; 383: 163-166
        • Braun A.R.
        • Balkin T.J.
        • Wesensten N.J.
        • Carson R.E.
        • Varga M.
        • Baldwin P.
        • et al.
        Regional cerebral blood flow throughout the sleep–wake cycle. An H215O PET study.
        Brain. 1997; 120: 1173-1197
        • Hobson J.A.
        • Stickgold R.
        • Pace-Schott E.F.
        The neuropsychology of REM sleep dreaming.
        NeuroReport. 1998; 9: R1-R14
        • Neville B.G.R.
        The origin of infantile spasms: evidence from a case of hydranencephaly.
        Dev Med Child Neurol. 1972; 14: 644-656
        • Kohyama J.
        REM sleep atonia: responsible brain regions, quantification, and clinical implication.
        Brain Dev. 2000; 22: S136-S142
        • Kohyama J.
        • Shimohira M.
        • Hasegawa T.
        • Kouji T.
        • Iwakawa Y.
        Phasic motor activity reduction occurring with horizontal rapid eye movements during active sleep in human.
        Exp Brain Res. 1995; 107: 137-144
        • Seguin J.J.
        • Magherini P.C.
        • Pompeiano O.
        Cholinergic mechanisms related to REM sleep. III. Tonic and phasic inhibition of monosynaptic reflexes induced by an anticholinesterase in the decerebrate cat.
        Arch Ital Biol. 1973; 111: 1-23
        • Hendricks J.C.
        • Kline L.R.
        • Davies R.O.
        • Pack A.I.
        Effect of dorsolateral pontine lesions on diaphragmatic activity during REMS.
        J Appl Physiol. 1990; 68: 1435-1442
        • Hendricks J.C.
        • Kline L.R.
        Differential activation within costal diaphragma during rapid-eye-movement sleep in cats.
        J Appl Physiol. 1991; 70: 1194-1200
        • Kohyama J.
        • Ohsawa Y.
        • Shimohira M.
        • Iwakawa Y.
        Phasic motor activity reduction occurring with horizontal rapid eye movements during REM sleep is disturbed in infantile spasms.
        J Neurol Sci. 1996; 138: 82-87
        • Kohyama J.
        • Sugimoto J.
        • Itoh M.
        • Sakuma H.
        • Shimohira M.
        • Hasegawa T.
        • et al.
        REM sleep components predict the response to initial treatment of infantile spasms.
        Epilepsia. 1999; 40: 992-996
        • Kohyama J.
        • Shimohira M.
        • Tanuma N.
        • Hasegawa T.
        • Iwakawa Y.
        ACTH activates rapid eye movement-related phasic inhibition during REM sleep in patients with infantile spasms.
        Acta Neurol Scand. 2000; 101: 145-152
        • Rektor I.M.
        • Svejdová M.
        • Ménini C.
        Cholinergic system disturbance in the West syndrome.
        Brain Dev. 1990; 12: 790-794
        • Morimatsu Y.
        • Murofushi K.
        • Handa T.
        • Shinohara T.
        • Shiraki H.
        Pathological studies of severe physically and mentally handicapped – with special reference to four cases of infantile spasms (in Japanese).
        Shinkei Kenkyu no Shinpo (Tokyo). 1972; 16: 465-470
        • Satoh J.
        • Mizutani T.
        • Morimatsu Y.
        Neuropathology of the brainstem in age-dependent epileptic encephalopathy-especially of cases with infantile spasms.
        Brain Dev (Tokyo). 1996; 8: 443-449
        • Tominaga I.
        • Yanai K.
        • Kashima H.
        • Kato Y.
        • Sekiyama S.
        • Yokochi A.
        • et al.
        Observation anatomo-clinique d'une séquelle d'encéhalopathie aiguë.
        Rev Neurol (Paris). 1986; 5: 524-529
        • Hayashi M.
        • Itoh M.
        • Araki S.
        • Kumada S.
        • Tanuma N.
        • Kohji T.
        • et al.
        Immunohistochemical analysis of brainstem lesions in infantile spasms.
        Neuropathology (Tokyo). 2000; 20: 297-303
        • Maton B.
        • Hirsch E.
        • Vergnes M.
        • Depaulis A.
        • Marescaux C.
        Dorsal tegmentum kindling in rats.
        Neurosci Lett. 1992; 134: 284-287
        • Chugani H.T.
        • Shewmon D.A.
        • Sankar R.
        • Chen B.C.
        • Phelps M.E.
        Infantile spasms: lenticular nuclei and brainstem activation on positron emission tomography.
        Ann Neurol. 1992; 31: 212-219
        • Baram T.Z.
        Pathophysiology of massive infantile spasms: perspective on the putative role of the brain adrenal axis.
        Ann Neurol. 1993; 33: 231-236
        • Kohyama J.
        A quantitative assessment of the maturation of phasic motor inhibition during REM sleep.
        J Neurol Sci. 1996; 143: 150-155
        • Kohyama J.
        • Tachibana N.
        • Taniguchi M.
        Development of REM sleep atonia.
        Acta Neurol Scand. 1999; 99: 368-373
        • Mori S.
        • Nishimura H.
        • Kurakami C.
        • Yamamura T.
        • Aoki M.
        Controlled locomotion in the mesencephalic cat: distribution of facilitatory and inhibitory regions within pontine tegmentum.
        J Neurophysiol. 1978; 41: 1580-1591
        • Garcia-Rill E.
        The basal ganglia and the locomotor regions.
        Brain Res. 1986; 396: 47-63
        • Rye D.B.
        Contributions of the pedunculopontine region to normal and altered REM sleep.
        Sleep. 1997; 20: 757-788
        • Konishi Y.
        • Hayakawa K.
        • Kuriyama M.
        • Saito M.
        • Fujii Y.
        • Sudo M.
        Effects of ACTH on brain midline structures in infants with infantile spasms.
        Pediatr Neurol. 1995; 13: 134-136
        • Zaphiropoulos A.
        • Charnay Y.
        • Vallet P.
        • Constantinidis J.
        • Bouras C.
        Immunohistochemical distribution of corticotropin-like intermediate lobe peptide (CLIP) immunoreactivity in the human brain.
        Brain Res Bull. 1991; 26: 99-111
        • Kohyama J.
        • Ohinata J.
        • Hasegawa T.
        Disturbance of phasic chin muscle activity during REM sleep.
        Brain Dev. 2001; (in press)
        • Snead III, O.C.
        • Benton J.W.
        • Myers G.J.
        ACTH and prednisone in childhood seizure disorders.
        Neurology. 1983; 33: 966-970
        • Diomedi M.
        • Curatolo P.
        • Scalise A.
        • Placidi F.
        • Caretto F.
        • Gigli G.L.
        Sleep abnormalities in mentally retarded autistic subjects: Down's syndrome with mental retardation and normal subjects.
        Brain Dev. 1999; : 548-553
        • Jambaque I.
        • Chiron C.
        • Dulac O.
        • Raynaud C.
        • Syrota P.
        • Benninger C.K.
        Visual inattention in West syndrome: a neuropsychological and neurofunctional imaging study.
        Epilepsia. 1993; 34: 692-700
        • Hashimoto T.
        • Tayama M.
        • Murakawa K.
        • Yoshimoto T.
        • Miyazaki M.
        • Harada M.
        • et al.
        Development of the brainstem and cerebellum in autistic patients.
        J Autism Dev Disord. 1995; 25: 1-18
        • Rodier P.M.
        • Ingram J.L.
        • Tisdale B.
        • Nelson S.
        • Romano J.
        Embryological origin for autism: developmental abnormalities of the cranial nerve motor nuclei.
        J Comp Neurol. 1996; 370: 247-261
        • Rodier P.M.
        • Ingram J.L.
        • Tisdale B.
        • Croog V.J.
        Linking etiologies in humans and animal models: studies of autism.
        Reprod Toxicol. 1997; 11: 417-422