Language outcome following multiple subpial transection for Landau-Kleffner syndrome.

Landau-Kleffner syndrome is an acquired epileptic aphasia occurring in normal children who lose previously acquired speech and language abilities. Although some children recover some of these abilities, many children with Landau-Kleffner syndrome have significant language impairments that persist. Multiple subpial transection is a surgical technique that has been proposed as an appropriate treatment for Landau-Kleffner syndrome in that it is designed to eliminate the capacity of cortical tissue to generate seizures or subclinical epileptiform activity, while preserving the cortical functions subserved by that tissue. We report on the speech and language outcome of 14 children who underwent multiple subpial transection for treatment of Landau-Kleffner syndrome. Eleven children demonstrated significant postoperative improvement on measures of receptive or expressive vocabulary. Results indicate that early diagnosis and treatment optimize outcome, and that gains in language function are most likely to be seen years, rather than months, after surgery. Since an appropriate control group was not available, and that the best predictor of postoperative improvements in language function was that of length of time since surgery, these data might best be used as a benchmark against other Landau-Kleffner syndrome outcome studies. We conclude that multiple subpial transection may be useful in allowing for a restoration of speech and language abilities in children diagnosed with Landau-Kleffner syndrome.

Evaluating medication response in ADHD: cognitive, behavioral, and single-subject methodology.

Although evidence supports the use of double-blind placebo medication trials to evaluate methylphenidate (MPH) effects on the core behavioral symptoms of attention-deficit/hyperactivity disorder (ADHD), few studies have demonstrated their utility in examining MPH effects on the cognitive deficits associated with ADHD. This article presents a technique for evaluating behavioral and cognitive dose-response relationships at the single-subject level of analysis. Case study results and multivariate analyses suggest that systematic evaluation of behavioral and cognitive MPH dose-response relationships could lead to more accurate MPH titration and greater long-term multimodal treatment efficacy.

Developmental changes of parental-reported sleep disturbance symptoms in children with attention deficit disorder.

The primary goal of this retrospective study was to assess parental report of current sleep disorders in school-aged attention deficit disorder (ADD) children, as well as recalled sleep problems from when the children were infants (0-12 months) and toddlers (1-3 years). Results of a sleep questionnaire completed by mothers of 48 ADD children and a comparison group of 30 patients with school problems indicate that ADD children were perceived to have significantly more sleep problems and that these problems had onset in infancy. Specific items in the questionnaire which were increased included latency to sleep onset of more than 30 min at least 3 nights per week, fatigue upon awakening, and recall of nightmares. Pediatric clinicians should be alert to possible sleep disorders in children suspected of attention disorders and should consider "sleep hygiene" measures as a component of treatment.

Mismatch negativity in the neurophysiologic/behavioral evaluation of auditory processing deficits: a case study.

The subject of this case report is an 18-year-old woman with grossly abnormal auditory brain stem response (ABR), normal peripheral hearing, and specific behavioral auditory processing deficits. Auditory middle latency responses (MLRs) and cortical potentials N1, P2, and P300 were intact. The mismatch negativity (MMN) was normal in response to certain synthesized speech stimuli and impaired to others--consistent with her behavioral discrimination of these stimuli. Behavioral tests of auditory processing were consistent with auditory brain stem dysfunction. A neuropsychological evaluation revealed normal intellectual and academic performance. The subject was in her first year of college at the time of the evaluation. This case study is important because: (1) Although there have been several reports of absent/abnormal ABR with preserved peripheral hearing and deficits in auditory processing, little is known about the specific nature of the auditory deficits experienced by these individuals. Such information may be valuable to the clinical management of patients with this constellation of findings. (2) Of interest is the information that the mismatch negativity (MMN) cortical event-related potential can bring to the evaluation of patients with auditory processing deficits. The MMN reflects central auditory processing of small acoustic differences and may provide an objective measure of auditory discrimination. (3) From a theorectical standpoint, a patient with neural deficits affecting specific components of the auditory pathway provides insight into the relationship between evoked potentials and physiological mechanisms of auditory processing. How do various components of the auditory pathway contribute to speech discrimination? How might evoked potentials reflect the processes underlying the neural coding of specific features of speech stimuli such as timing and spectral cues?

The go-no go test in attention deficit disorder is sensitive to methylphenidate.

The go-no go test requires a subject to emit a simple motor response to one cue while inhibiting the response in the presence of another cue. This test has been effective in demonstrating impulsivity (elevated commission error rate) in children with attention deficit disorder (ADD). In this study, we examined the effects on go-no go test performance of two doses of methylphenidate (0.15 mg/kg and 0.3 mg/kg) administered in double-blind placebo-controlled fashion to children with ADD. Our results indicate that even modest doses of methylphenidate improve the go-no go performance of these children by decreasing their tendency to make impulsive commission errors. Thus the test is sensitive to the effects of methylphenidate and can be used to monitor a response to therapy.

The go-no-go paradigm in attention deficit disorder.

We administered the go-no-go paradigm to 44 boys with attention deficit disorder (ADD) and 32 control subjects who did not have ADD. This task requires a subject to emit a simple motor response to one cue while inhibiting the response in the presence of another cue. Commission errors suggest impulsivity, and omission errors suggest inattention. ADD subjects made more total errors than did control subjects (p less than 0.03), and more ADD subjects made multiple errors (p less than 0.001). Within the ADD group, the nonhyperactive (ADDnoH) subjects were characterized by a high number of commission errors early, and significant improvement with practice (p less than 0.01). In contrast, the hyperactive ADD subjects (ADD/H) did not differ from control subjects in number of early commission errors, but differed from both control subjects and ADDnoH subjects in their failure to improve with practice. In addition, the incidence of omission errors was highest in the ADD/H group. This paradigm can be easily incorporated into the assessment of children with suspected ADD and provides an objective measure of inattention and impulsivity. Our data provide cognitive support for the empirical distinction between hyperactive and nonhyperactive children with ADD.

Presurgical adjustment in male transsexuals with and without hormonal treatment.

The effects on hormonal treatment on the adjustment of adult males requesting sex reassignment surgery were examined in two studies. In study 1, the Minnesota Multiphasic Personality Inventory (MMPI) profiles of 19 patients who received no hormonal therapy and 22 patients who received 12 or more months of estrogen and medroxyprogesterone acetate treatment were compared. Patients receiving hormonal therapy not only showed significantly less psychopathology, but their scores on MMPI scales indicating neurotic and study 2, using correlational techniques to assess the effects of both hormonal treatment and cross-dressing on psychopathology. Length of hormonal treatment was related to emotional adjustment on four clinical scales of the MMPI. Scores indicating greater adjustment were associated with longer periods of treatment. Length of full-time cross-dressing, on the other hand, was not associated with patients' level of adjustment. These findings have important implications for those involved in personality evaluation of transsexuals and for future research. A more rigorous criteria for psychopathology may be required for those persons already exposed to hormonal therapy. It if further suggested that personality research with presurgical transsexuals include a drug hormone history.