Survey of adolescents with severe intellectual handicap

Article Abstract:

Severe mental retardation occurs in about 4 out of 1,000 people. In the past, specific causes for retardation were identifiable in half of all cases. It is important to identify the cause wherever possible in order to estimate the probable prognosis and to predict the probability of recurrence in other children and family members. In recent years, a new disorder, the fragile X syndrome, has been identified which is probably second only to Down syndrome as a cause of retardation. Males carry one X and one Y sex chromosome, while females have two X chromosomes. In fragile X syndrome, a mutation occurs near the tip of the X chromosome, which ultimately results in mental deficiency in most males and in 30 percent of females. It is unknown why some males with fragile X are apparently unaffected, but pass on the disorder, while many females with one fragile X are mildly or moderately impaired. Severely retarded children over the age of 11 who attended special schools in Tameside, England, and who had not been medically evaluated since early childhood were reassessed in this study. Of 82 affected children, 18 had Down's syndrome and one had suffered severe head trauma. The remaining 63 were offered reassessment so that families might be given genetic counseling; 53 families were subsequently evaluated. A diagnosis could be made in 25 out of 31 children for whom the cause of retardation was previously unknown. Of these, 15 had fragile X syndrome, and all but one of the affected families made use of genetic counselling and evaluation. Five girls were diagnosed with Rett's syndrome, a disorder in which developmental regression develops in girls after apparently normal births and early infancy, which is associated with a low risk of familial recurrence. In 12 of 82 children, disorders related to intellectual disability such as cerebral palsy were found. No cause for retardation could be determined in only nine cases. These results indicate that reassessment of older retarded children is of value, as new diagnoses may be made, and families welcome the review and advice made available by such evaluations. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Demographic aspects, Fragile X syndrome, Mental retardation

---

Treatment of epilepsy in people with multiple handicap

Article Abstract:

This report is an account of the treatment of 51 severely handicapped and epileptic children between the ages of 2 and 16 years. The cases are described with respect to patients' handicaps, types of seizures, general management, drug treatment, side effects, outcome, and cost. There were 22 boys and 29 girls (27 lived in a home for handicapped children, while 24 lived at home) who suffered from cerebral palsy, mental impairment, severe learning disability, autism, deafness or hydrocephalus. Some children had different types of seizures, which were classified into four groups: classic petit mal (1 case); complex partial seizures (26 cases); episodes involving muscle twitching (myoclonus) and akinetic (complete or partial loss of muscle movement) falls (10 cases); or generalized seizures involving loss of consciousness (43 cases). Changes in routines or schedules seemed to trigger episodes. Drug treatment for epilepsy was given to 44 children, of which 19 were receiving more than one drug and two were receiving three drugs at the same time. The goal was to adjust the drug dosage over time in order to reduce the severity and frequency of seizures. At the end of the two-year study period, nine patients (18 percent) were drug- and seizure-free, 36 patients had fewer seizures on less medication, and three patients receiving medication continued to have seizures several times a week. The most common drugs used were sodium valproate and carbamazepine. Overall, the cost was unchanged because fewer, more expensive drugs replaced more, less expensive drugs. Minor side effects included irritability, sleeplessness, and weight fluctuations, but serious liver damage may occur with sodium valproate. These results suggest that a delicate balance between seizure control and sedation is difficult to achieve, but handicapped, epileptic children can be treated so that they have few seizures with minimal sedation. The author stresses that periodic examination of the drug treatment regimen should be performed because situations constantly change, and thus the individual response to certain drugs may change too. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Drug therapy, Disabled children, Seizures (Medicine), Epilepsy in children, Pediatric epilepsy

---

Consent for children and adolescents who have an intellectual handicap

Article Abstract:

In the United Kingdom the law considers adolescents autonomous and legally competent to make decisions for themselves at the age of 16. However, in cases of adolescents over the age of 16 who are unable to give informed consent because of some intellectual handicap, physicians must assure themselves that some means of obtaining consent are used that protect the best interests of these patients. Although the decision may be left to the courts, short of this expensive and time-consuming method the legal requirements for obtaining informed consent are unclear. It is suggested that in certain situations involving minor procedures, the adolescent may be capable of giving consent if the situation is carefully explained. However, where more complex medical procedures are involved, or where socially sensitive issues such as sterilization or abortion must be faced, the physician is well advised both to confer with other physicians to obtain second or even third opinions and, where appropriate, to discuss the decision with the patient's family. Emergency care need not be delayed until a relative can be found. Further, particular care should be taken in using the patient for research if the patient is likely to receive little direct benefit. These guidelines should be communicated to physicians, nurses and other caregivers at facilities that treat legally incompetent patients. (Consumer Summary produced by Reliance Medical Information, Inc.)

United Kingdom, Laws, regulations and rules, Informed consent (Medical law), Informed consent, Mentally disabled children, Great Britain, Adolescent medicine, Disabled youth

Similar abstracts:

• Abstracts: Quality of Lyme disease tests. Pitfalls in the diagnosis and treatment of Lyme disease in children. Antibody testing in Lyme disease: a comparison of results in four laboratories
• Abstracts: Mucosal dysplasia of the liver and the intraductal variant of peripheral cholangiocarcinoma in hepatholithiasis
• Abstracts: Hepatocellular carcinoma in childhood: clinical manifestations and prognosis. Maternal transmission of hepatitis B virus in childhood hepatocellular carcinoma
• Abstracts: Genital and anal conditions confused with child sexual abuse trauma. Vaginal opening measurement in prepubertal girls

This website is not affiliated with document authors or copyright owners. This page is provided for informational purposes only. Unintentional errors are possible.