Infinite Potential Program

Cortical Integrative Therapy in Congenital Microcephaly with
Associated Defective Brain Development:

Dr. Victor M. Pedro
Department of Clinical Sciences University of Bridgeport, Bridgeport, Connecticut, U.S.A.

ABSTRACT

A case study is described of a 17-year-old right-handed Caucasian female diagnosed with microcephaly and complicated by defective brain development (mild to moderate mental retardation) with likely craniosynostosis. Familial history suggested microcephaly and the mother's gestational diabetes was probably not causative. Female at age 14 months presented with relatively normal neurological scan, but by age 5 (during kindergarten screening) developmental delays associated with defective brain development became apparent. Family members observed frequent minor seizures, their onset signaled by excessive blinking episodes followed by disorientation. Subject continued to experience great difficulty in learning and retaining basic concepts. Observed performance indicators remained grossly deficit, with markers reaching a plateau before stagnating in the kindergarten to second grade (k-2) range for overall academic achievement. After eleven years of special education interventions with no significant improvement in any measurable area of function, a multimodal approach using techniques aimed at facilitating inter-hemispheric communication was provided. At completion of the Cortical Integrative Therapy program, a significant gain in verbal ability was independently observed and verified by testing; smaller gains in non-verbal and other diverse performance indicators were otherwise noted. Seizures still occurred, but less frequently.

KEY WORDS: microcephaly, Cortical Integrative Therapy, defective brain development, craniosynostosis

A description and not a specific diagnosis, microcephaly is a term for the condition of an abnormally small head. It can result from a number of different causes, the most common being abnormal brain growth -- which is a cause rather than an effect of impaired skull growth (Dorman C., 1991). Researchers frequently characterize microcephaly as primary or secondary. Its primary form can exist when a smaller than normal brain and skull often results in some degree of mental retardation. The microcephalic brain is not only smaller, but also the cerebral cortex - the layers of nerve cells that cover the brain's surface and are the seat of higher reasoning - is shrunken. (Travis J., 2002). Primary microcephaly usually suggests below normal skull and brain growth during pregnancy followed by relatively normal growth, size, and development during childhood and maturation. A more pervasive microcephaly that can present with similar manifestation is secondary microcephaly, a condition characterized by near normal or normal brain size noted at birth, but because of an unknown genetic predisposition (not known to be caused by a specific gene), the brain subsequently fails to grow normally. While secondary microcephaly often produces impaired intelligence and frequent seizures, these deficits are usually a consequence of an underlying brain disorder or environmental factors - most often chronic tobacco use during the gestational period (Mercuri et all, 2000) - or a combination of genetic and environmental factors. A common brain malformation caused by a genetic defect that results in a proliferation failure of neurons, craniosynostosis is the premature closure of one or more of the cranial sutures in a fetus or an infant. It can produce a chronic increase in intracranial pressure caused by tangential growth of the brain within an unyielding skull -- invariably resulting in defective brain development and serious impairment of intelligence. A large percentage of children with microcephaly, perhaps as many as 40% (McIntyre 1997) develop craniosynostosis associated with their microcephaly. Mental retardation in microcephaly can range from mild to severe, with the entire spectrum of intelligence stunted to sub-normal or below normal functioning.

CASE REPORT

E.F. is a 17-year-old right-handed female, born at 9 pounds, 14 ounces following a full term gestation complicated by the mother's diabetes. Head size at birth was 29 centimeters, slightly below the normal of 33 centimeters. The mother suspected that E.F. would be born microcephalic because of the presence of a familial strain involving smaller than normal head size in, at least, two ancestral relatives and previously diagnosed microcephaly in E.F's elder sister. While a prenatal ultrasound scan and a CT scan performed at two days of age appeared relatively normal, with no CT evidence of microcephaly (configuration of the cranial normal) in the latter scan, further testing revealed incipient microcephaly when the infant was 10 days of age. Although developmental delays were not immediately apparent, a comment alluding to forecast delays -- small head/"slow learner" -- appeared in E.F.'s medical record at that time. Because membranes were applied tight to the infant's head and difficult to rupture, and because her fontanels "felt small" and were difficult to identify, a complicating diagnosis of craniosynostosis was considered likely. There was no suggestion of infection, destructive lesion, or further chromosomal abnormalities. E.F. matured in a multi-cultural and bilingual (Portuguese-English) home environment. When E.F. was nearly 14 months of age, a physician's report re-confirmed familial microcephaly with an "autosomal recessive" condition described as causal. At this time, a neurological exam surprisingly determined E.F.'s functioning and development to be normal, although early intervention for prompt detection of developmental delays was recommended. E.F. became ambulatory at 12-13 months, but her speech was delayed until age 4. Toilet training was delayed until 4 years 10 months as chronic nocturnal enuresis was noted.

When E.F. was age 5, she underwent educational testing for admission into kindergarten (pre-school). At this time, a spectrum of delays associated with defective brain development (mild to moderate mental retardation) became evident. Documented with pronounced weaknesses in cognition, reasoning, and problem solving, E.F. proved significantly below the average for her age in global functioning. While acquired knowledge related to number concepts was E.F.'s strongest area (slow learner classification), further weaknesses in verbal areas (word knowledge, verbal fluency, long term memory, ability to reason) revealed moderate mental retardation. Other area scores (non-verbal problem solving, visual discrimination, perception of part/whole relationships, spatial organization, eye-hand coordination, auditory short-term memory) were closer to peer norms, but still demonstrated significant delays (mild retardation).

Observations and further testing of E.F. in a kindergarten special needs learning environment confirmed overall global cognitive and language delays although her bi-lingual use of language might have hindered independent evaluation of her condition. Like many bilingual children, she did not speak or write consistently in English or Portuguese, complicating her ability to communicate and interact.

Little changed as E.F. matured. Although her mental retardation was never classified as "severe" or "profound," effects of defective brain development were always apparent. Frequent seizures, their onset signaled by excessive blinking, would result in disorientation and fatigue. She learned to communicate effectively (primarily in English), but only developed learning skills demonstrable at kindergarten to second grade (K-2) academic level and proved incapable of further advancement. Prior to age 8, certain skills incidental to academic performance and living at home did show improvement. These included maintaining eye contact, articulation, and volume of speech (speaking loud enough for listeners to comprehend what she was saying). Never mainstreamed (allowed to learn complex concepts with normal peers), her entire academic career was spent in a succession of special education academic environments. It was hoped that supportive small groups and structured multi-sensory language-based programs would best facilitate E.F.'s specialized needs. At age 11, E.F. was reported to be a "delightful child" with both receptive and expressive language delays and difficulty retrieving and retaining information. A school psychologist expressed comment about 11-year-old E.F. in the following manner: "Although she physically looks her age, her mannerisms, emotional presentation, and language communication are suggestive of a much younger child."

A series of intelligence tests administered to E.F. at age 16 by a school psychologist (she was now attending high school in an "ungraded class") conclusively confirmed previous testing and observations. The Slosson Intelligence Test was administered to E.F. and she scored in the moderately retarded range. Her strengths were in general knowledge and number concepts. Overall skills appeared stagnated at the five to five-and-a-half year level. On the Vineyard Adaptive Behavior Scale, strengths were noted in gross and fine motor skills and daily living skills. In daily living domestic skills, E.F. achieved a score at the 12-year level. (E.F. could perform household tasks with competence and independence, including cooking and planning simple meals.) A weaker area was written language. Here E.F. scored at the 7 year 5 month level. Finally, several Kaufman Tests of Educational Achievement were administered producing results exclusively in the mildly retarded range. For instance, E.F. could read at the first grade level and spell at the second grade level.

METHOD

12-week Cortical Integrative Therapy Program

RESULTS

At the conclusion of the twelve-week treatment program, E.F. showed considerable gains in her ability to verbally communicate as noted by an independent examiner, a school psychologist. Her overall academic performance, as noted by her classroom teachers and parents, was also significantly improved.

There were also other documented gains. Prior to her participation in the Cortical Integrative Therapy program, E.F. attained an overall standard verbal score of 46 on the Wechsler Intelligence Scale for Children (WISC-III). Subsequent to the WISC-III testing and after completing Cortical Integrative Therapy, E.F. attained a standard verbal score of 62. Although these are two different tests, the psychologist wrote, "Although these are two different tests, these results were unexpected and the use of two different tests would not solely account for these gains."

In addition, according to her family, E.F. was observed to have significantly fewer blinking episodes that were formerly the ominous prelude to disruptive and recurrent seizures.

DISCUSSION

Microcephaly complicated by craniosynostosis is often associated with major intellectual deficits and learning disabilities including mental retardation. Behavior problems such as distractibility and echolalia can also occur. E.F. was able to perform only up to the second grade level in her academic career, and her quality of life was severely restricted. But when a multimodal approach using techniques aimed at facilitating inter-hemispheric communication was provided, evidence was produced to indicate that when such techniques are applied, the prospect of indirect neuronal or network modulation occurring as cortical pathways are stimulated becomes a real possibility.

REFERENCES

Dorman, C. (1991). "Microcephaly and intelligence." Developmental Medicine and Child Neurology, v33, n3p267 (3)

Mercuri E., Ricci D., Cowan F.M., Lessing D., Frisone M.I., Haataja L., Counsell S.J., Dubowitz L.M., Rutherford M.A. "Head growth in infants with hypoxic-ischemic encephalopathy: correlation with neonatal magnetic resonance imaging." Pediatrics, August 2000.

McIntyre, F.L. "Craniosynostosis." American Family Physician, March 1997.

Travis, J. "Sizing up the Brain: mutations that produce small brains may reveal how human intelligence evolved." Science News, Nov 16 2002.