Citations

We report a brief treatment format for young sexually abused boys that has both diagnostic and therapeutic implications. A general format is outlined, and then a group is described in more detail. Examples from each session are given that illustrate both therapeutic issues and issues related to the effects of sexual abuse on this sample. Implications for longer, more intensive treatment are given.

A sample of 31 boys sexually assaulted in the previous 18 months was compared on the Child Behavior Checklist with 33 boys who had a diagnosis of oppositional or conduct disorder. The boys ranged in age from 3 to 8 years old. The conduct-disordered boys were being seen in outpatient therapy with their parents. The two groups did differ significantly, with the sexually abused group being less externalizing and more sexualized than the conduct disorder group.

The congenital myasthenic syndromes include end-plate (EP) acetylcholinesterase deficiency, presynaptic abnormalities affecting the evoked release or size of transmitter quanta, and acetylcholine (ACh) receptor (AChR) channelopathies stemming from a kinetic abnormality and/or deficiency of AChR. A kinetic abnor mality predicts, and AChR deficiency may predict, one or more mutations in an AChR subunit gene. These clues have led to the identification of 53 mutations in different subunits of AChR in 55 kinships of the congenital myasthenic syndromes. The mutations either increase or decrease the response to ACh, produce AChR deficiency, or both. In the slow-channel syndromes, prolonged opening episodes of AChR cause cationic overloading of the EP and an EP myopathy; the mutations occur in different subunits and different domains of the subunits and have dominant positive effects. The M1 and M2 mutations slow channel closure, increase apparent affinity for ACh, and variably enhance desensitization, and the extracellular αG153S enhances affinity for ACh, promoting reopening of the diliganded receptor. In the low-affinity fast-channel syndrome, εP121L reduces affinity for ACh and reopening of the diliganded receptor, resulting in a de creased response to ACh and shorter burst durations. Severe EP AChR deficiency results from heterozy gous or homozygous mutations that terminate translation prematurely; these are concentrated in the ε subunit, probably because substitution of the fetal γ for the adult ε subunit can rescue the phenotype from fatal null mutations in ε. Variable AChR deficiency and variable functional abnormalities stem from hetero allelic nonsense and missense mutations in AChR subunit genes. NEUROSCIENTIST 4:185-194, 1998