‘Cognitive function in tetraplegic cerebral palsy with severe motor dysfunction’

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Kittang OB, Vesterhus P. (1998) 47, XYY syndrome, a significant diagnosis? Tidsskr Nor Laegeforen 118: 1563–1564. Kumra S, Wiggs E, Krasnewich D, Meck I, Smith A, Bedwell I. (1998) Brief report: association of sex chromosome anomalies with childhood-onset psychotic disorders. J Am Acad Child Adolesc Psychiatry 3: 292–296. Lachar D, Gdowski CL. (1979) Actuarial Assessment of Child and Adolescent Personality: An Interpretive Guide for the Personality Inventory for Children Profile. Los Angeles, CA: Western Psychological Services. Ratcliffe SG. (1999) Long-term outcome in children of sex chromosome abnormalities. Arch Dis Child 80: 192–195. Ratcliffe SG, Pan H, McKie M. (1992) Growth during puberty in the XYY boy. Ann Hum Biol 19: 579–587. Reitan MR, Davison LA. (1974) Clinical Neuropsychology: Current Status and Application. New York: J. Wiley & Sons. Robinson A, Bender B, Borelli J, Puck M, Salbenblatt J. (1983) Sex chromosomal abnomalities: prospective studies in children. Behav Genet 4: 321–329. Stray LL. (2001) Motor Function in AD/HD Children. (Thesis). Division for Physiotherapy Science, University of Bergen, Norway. Wechsler D. (1949) Wechsler Intelligence Scale for Children. New York: The Psychological Corporation. List of abbreviations ADHD Attention-deficit–hyperactivity disorder SCL Skin conductance level WISC Wechsler Intelligence Scale for Children 562 Developmental Medicine & Child Neurology 2005, 47: 559–562 Letter to the editor ‘Cognitive function in tetraplegic cerebral palsy with severe motor dysfunction’ SIR–We have recently examined this issue, using data from the same large database from California on cerebral palsy (CP) that was the source for earlier publications. 1–3 We defined severe motor dysfunction as tetraplegia with no functional hand use and inability to crawl, creep, scoot, or walk. According to the Client Development Evaluation Report Manual, 4 ‘intellectual functioning and adaptive behavior are [typically] measured by standard- ized tests, the results of which form the basis for the psy- chologist’s clinical diagnosis. Determination of the level of mental retardation*… must be consistent with Chapter 3 and Appendix A of the Classification in Mental Retardation [5] ’.We grouped mental retardation level as: (1) moderate, severe, or profound (IQ<50); (2) interme- diate (IQ approximately 50 to 70); or (3) no mental retar- dation (IQ 70 or higher). The latter was assumed if (a) the patients had ‘no mental retardation’ or an ‘unspecified’ mental retardation level, and (b) receptive language was at the highest level, namely: ‘Understands meaning of story plot and complex conversation.’ We focused on young adults, (15–35y), because cognitive assessments have generally stabilized by this age. There were 532 patients meeting these criteria, of whom 426 (80%) were classified as having spastic CP and 20 (4%) as having dyskinetic CP (this term includes athetosis and dystonia). Among those with spastic CP, 95% had severe mental retardation or worse and 75% were classed as having Profound Mental Retardation (IQ<25). Only 2% had no mental retardation. Among those with dyskinetic CP, however, only eight (40%) had profound mental retardation, and four (20%) had no mental retardation. A possible limitation of these results is that the relia- bility of the cognitive assessments is untested. Nevertheless, it seems reasonable to conclude that nor- mal intelligence is rare among young adults with severe spastic CP, but not uncommon among those with dyski- netic CP, even when there is profound motor impair- ment. These findings are consistent with those of Kyllerman 6 who demonstrated that more than half of his (child) patients with dyskinetic CP had intelligence with- in the normal or dull-normal range. These findings emphasize the importance of appreciat- ing that cognitive abilities continue to remain preserved in some young adults with dyskinetic CP who have very severe motor dysfunction. DOI: 10.1017/S0012162205001106 David Strauss* Lewis Rosenbloom Steven Day Robert Shavelle *Correspondence to: [email protected] References 1. Strauss D, Shavelle R. (1998) Life expectancy of adults with cerebral palsy. Dev Med Child Neurol 40: 369–375. 2. Strauss DJ, Shavelle RM, Anderson TW. (1998) Life expectancy of children with cerebral palsy. Pediatr Neurol 18: 143–149. 3. Strauss D, Cable W, Shavelle R. (1999) Causes of excess mortality in cerebral palsy. Dev Med Child Neurol 41: 580–585. 4. Department of Developmental Services. (1986) Client Development Evaluation Report Manual. Sacramento, California: Department of Developmental Services. 5. Grossman HJ, Editor. (1983) Classification in Mental Retardation. Washington DC: American Association on Mental Deficiency. 6. Kyllerman M. (1977) Dyskinetic cerebral palsy. An analysis of 115 Swedish cases. Neuropadiatria 8 (Suppl.): 28–32. *UK usage: learning disability.

Transcript of ‘Cognitive function in tetraplegic cerebral palsy with severe motor dysfunction’

Kittang OB, Vesterhus P. (1998) 47, XYY syndrome, a significantdiagnosis? Tidsskr Nor Laegeforen 118: 1563–1564.

Kumra S, Wiggs E, Krasnewich D, Meck I, Smith A, Bedwell I. (1998)Brief report: association of sex chromosome anomalies withchildhood-onset psychotic disorders. J Am Acad Child Adolesc

Psychiatry 3: 292–296.Lachar D, Gdowski CL. (1979) Actuarial Assessment of Child and

Adolescent Personality: An Interpretive Guide for the

Personality Inventory for Children Profile. Los Angeles, CA:Western Psychological Services.

Ratcliffe SG. (1999) Long-term outcome in children of sexchromosome abnormalities. Arch Dis Child 80: 192–195.

Ratcliffe SG, Pan H, McKie M. (1992) Growth during puberty in theXYY boy. Ann Hum Biol 19: 579–587.

Reitan MR, Davison LA. (1974) Clinical Neuropsychology: Current

Status and Application. New York: J. Wiley & Sons.

Robinson A, Bender B, Borelli J, Puck M, Salbenblatt J. (1983) Sexchromosomal abnomalities: prospective studies in children.Behav Genet 4: 321–329.

Stray LL. (2001) Motor Function in AD/HD Children. (Thesis).Division for Physiotherapy Science, University of Bergen, Norway.

Wechsler D. (1949) Wechsler Intelligence Scale for Children. NewYork: The Psychological Corporation.

List of abbreviations

ADHD Attention-deficit–hyperactivity disorderSCL Skin conductance levelWISC Wechsler Intelligence Scale for Children

562 Developmental Medicine & Child Neurology 2005, 47: 559–562

Letter to the editor

‘Cognitive function in tetraplegic cerebral palsy with severemotor dysfunction’SIR–We have recently examined this issue, using datafrom the same large database from California on cerebralpalsy (CP) that was the source for earlier publications.1–3

We defined severe motor dysfunction as tetraplegia withno functional hand use and inability to crawl, creep,scoot, or walk. According to the Client DevelopmentEvaluation Report Manual,4 ‘intellectual functioning andadaptive behavior are [typically] measured by standard-ized tests, the results of which form the basis for the psy-chologist’s clinical diagnosis. Determination of the levelof mental retardation*… must be consistent with Chapter3 and Appendix A of the Classification in MentalRetardation[5]’.We grouped mental retardation level as:(1) moderate, severe, or profound (IQ<50); (2) interme-diate (IQ approximately 50 to 70); or (3) no mental retar-dation (IQ 70 or higher). The latter was assumed if (a) thepatients had ‘no mental retardation’ or an ‘unspecified’mental retardation level, and (b) receptive language wasat the highest level, namely: ‘Understands meaning ofstory plot and complex conversation.’ We focused onyoung adults, (15–35y), because cognitive assessmentshave generally stabilized by this age.

There were 532 patients meeting these criteria, ofwhom 426 (80%) were classified as having spastic CP and20 (4%) as having dyskinetic CP (this term includesathetosis and dystonia). Among those with spastic CP,95% had severe mental retardation or worse and 75%were classed as having Profound Mental Retardation(IQ<25). Only 2% had no mental retardation. Among

those with dyskinetic CP, however, only eight (40%) hadprofound mental retardation, and four (20%) had nomental retardation.

A possible limitation of these results is that the relia-bility of the cognitive assessments is untested.Nevertheless, it seems reasonable to conclude that nor-mal intelligence is rare among young adults with severespastic CP, but not uncommon among those with dyski-netic CP, even when there is profound motor impair-ment. These findings are consistent with those ofKyllerman6 who demonstrated that more than half of his(child) patients with dyskinetic CP had intelligence with-in the normal or dull-normal range.

These findings emphasize the importance of appreciat-ing that cognitive abilities continue to remain preservedin some young adults with dyskinetic CP who have verysevere motor dysfunction.

DOI: 10.1017/S0012162205001106

David Strauss*

Lewis Rosenbloom

Steven Day

Robert Shavelle

*Correspondence to: [email protected]

References1. Strauss D, Shavelle R. (1998) Life expectancy of adults with

cerebral palsy. Dev Med Child Neurol 40: 369–375.2. Strauss DJ, Shavelle RM, Anderson TW. (1998) Life expectancy

of children with cerebral palsy. Pediatr Neurol 18: 143–149.3. Strauss D, Cable W, Shavelle R. (1999) Causes of excess

mortality in cerebral palsy. Dev Med Child Neurol 41: 580–585.4. Department of Developmental Services. (1986) Client

Development Evaluation Report Manual. Sacramento,California: Department of Developmental Services.

5. Grossman HJ, Editor. (1983) Classification in Mental

Retardation. Washington DC: American Association onMental Deficiency.

6. Kyllerman M. (1977) Dyskinetic cerebral palsy. An analysis of115 Swedish cases. Neuropadiatria 8 (Suppl.): 28–32.*UK usage: learning disability.