CASE REPORT
B.R. is a 6-year-old right-handed male, born at 6 pounds, 10 ounces following a normal (39 weeks) gestation. Mother was of advanced age (35) at pregnancy. Delivered at term, but his umbilical cord was discovered knotted and wrapped around B.R.’s neck at birth. An initial feeding tube for cholesterol supplements was inserted at age 19 months. Failure to thrive, indicative of the metabolic disorder Smith-Lemli-Opitz Syndrome had been observed. Global development delays were first observed at age 6 months. Medical history is extensive and significant for Dyspraxia of Speech Disorder, Apraxia of Speech Disorder, Ulcers, Mild Mental Retardation, and Autistic Spectrum Disorder. Surgery performed on B.R. at age 4 weeks for pyloric stenosis. He was hospitalized at age 8 months for bacterial pneumonia. When anoxia developed, B.R. was given oxygen. Four subsequent feeding tubes were inserted in separate operations. The final feeding tube was removed at age 5 when B.R. became able to take cholesterol supplements orally. B.R. had not completed toilet training at age 5 years, 10 months. He received early educational intervention ages 1-3 and experienced pre-school and kindergarten environments ages 4-6. Difficulties noted with B.R.’s peer interactions and social skills. In kindergarten, B.R. would often “get too close to others” (unable to discern appropriate boundaries) and hit, push, or touch other children indiscriminately. For these reasons, it was nearly impossible for B.R. to “make friends.” As a kindergarten student, B.R. was observed to be making academic progress at a slower rate than other children. He could identify a few letters (i.e. ‘B’ ‘E’) but was unable to articulate sounds associated with either letter. He was unable to identify any numbers as an early kindergartener, but by the end of the kindergarten year was able to count to 10, clap to the syllables in words, graph data, and participate in a daily writing workshop through drawing and writing. He was also able to name colors blue and green, and point to several distinct colors. B.R. attended kindergarten with an instructional assistant and was receiving several special education services: occupational therapy, physical therapy, and speech therapy. Although he could write his name, he was able to recognize only the first and second letters of his first name. His attention and focus were often deficient and his “language issues” for receptive and expressive language were especially prominent and indicative of severe developmental delays. He spoke in one and two word utterances and possessed a limited vocabulary. B.R. required frequent repetition for directions.
At age 5 years 8 months, B.R. received a complete psychological assessment to obtain a baseline of his cognitive and adaptive functioning skills. He presented as a “very friendly” child, and appeared to enjoy the one-to-one attention he received during the testing sessions. It was apparent that B.R. desired to interact and engage in conversation with the examiner, but due to severe dyspraxia was unable to communicate effectively. He also experienced significant difficulty in understanding some of the standardized verbal testing instructions. Administered the Stanford Binet Intelligence Scales – Fifth Edition (SB-V), a standardized test of intelligence, B.R.’s full-scale intellectual abilities fell in the mildly impaired range, as his overall verbal and visual reasoning skills exceeded only .2% of other children his age. Significant cognitive weaknesses were found (both on verbal and nonverbal assessments) with fluid reasoning, visual spatial reasoning, and on working memory tasks. The evaluator felt that B.R. met the criteria for mild mental retardation, but was also capable of learning.
He received an occupational therapy evaluation at age 5 years, 10 months. When administered the Beery-Buktenica Developmental Test of Visual-Motor Integration, B.R. received a raw score of 6, and a standard score of 63. This score was in the ‘very low’ range, where average includes a spectrum of 83-117. He had difficulty with visual motor skills, especially with forming and duplicating shapes. He also scored in the ‘very low’ range when administered VMI tests of motor coordination and visual perception. As part of a three-year educational assessment, B.R. underwent further testing, including the Young Children’s Achievement Test (YCAT) and the Kaufman Survey of Early Academic and Language Skills (K-SEALS). In the YCAT, B.R. scored “very poorly” in areas of general information, reading, mathematics, writing, and spoken language. Although he peaked out with a 7 percentile in reading skills, his overall composite was less than 1 percentile, equivalent to a child aged 3 years, 6 months. His K-SEALS scores were even less encouraging. His best result among the subtests was “Numbers, Letters, and Words,” where he received a standard score of ’80’ – ranking him as “well below average” among similarly aged peers. All other subtests were in the “lower extreme” range, and received standard scores between 55 and 69.