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1/3. myotonic dystrophy and progressive cognitive decline: a common condition or two separate problems?

    We report the case of NG, a 43-year old woman with myotonic dystrophy (MYD) who has shown a slow decline in both motor and cognitive abilities since her referral to us at age 32. MYD is an autosomal dominant disorder characterised by weakening and wasting of the muscles together with impaired muscle relaxation. Cognitive abilities are usually little affected in the adult onset form, although there is a high risk of cognitive impairment in those with childhood onset. Cognitive decline is also typically associated with maternal inheritance. NG, who was diagnosed with MYD at the age of 18, inherited it from her father. We report the decline in NG's cognitive abilities over 11 years of longitudinal assessment, and consider whether she is an atypical MYD patient or whether the MYD and cognitive decline are attributable to two separate pathological processes. ( info)

2/3. Assessing mental status in persons with mental retardation.

    When assessing a client, avoid abstract questions, instead ask concrete, open-ended questions. All clients should be assessed for risk of suicide, elopement, or a danger to self or others. Be aware of underlying causes or confounding variables that may affect a client's mental status. ( info)

3/3. Surgical treatment of epilepsy in children.

    In carefully selected children, as well as adults, intractable seizures may be eliminated or greatly reduced by cortical resection or hemispherectomy. Critical features of surgical candidacy include intractable disabling epilepsy, a localized epileptogenic zone, and a low risk of new postoperative deficits; however, the analysis may be complicated in children. Compared with adults, pediatric patients are especially likely to present with poorly localizing electroencephalographic features because of their high incidence of extratemporal localization and developmental pathology. Maturation factors may result in unusual epilepsy manifestations, for example, infantile spasms and hypsarrhythmia caused by a focal cortical lesion. The cognitive and psychosocial costs of continued frequent seizures during infancy and childhood must be assessed differently from those in adults and may include stagnation of developmental progression. The risk for new postoperative deficits may be modified if surgery is performed during stages of active brain maturation with developmental plasticity. For each individual child, the potential risk/benefit ratio for surgery must be carefully weighed on the basis of results of an extensive preoperative evaluation. Results from pediatric epilepsy surgery series are encouraging, with percentages of seizure-free patients similar to those in adult series. In some series, delaying surgery for childhood-onset epilepsy into adulthood was associated with greater permanent psychosocial, behavioral, and educational problems. The available data suggest that children should be considered for surgical evaluation at whatever age they present with severe intractable localization-related epilepsy. Complicated cases warrant referral to specialized centers with extensive pediatric epilepsy surgery experience. ( info)

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