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. 2012:2012:843016.
doi: 10.1155/2012/843016. Epub 2012 Jul 30.

Clinic-based retrospective analysis of psychopharmacology for behavior in fragile x syndrome

Elizabeth Berry-Kravis  1 , Allison Sumis, Crystal Hervey, Shaguna Mathur
Affiliations

Clinic-based retrospective analysis of psychopharmacology for behavior in fragile x syndrome

Elizabeth Berry-Kravis et al. Int J Pediatr. 2012.

Abstract

Fragile X syndrome (FXS) is associated with behavior that limits functioning, including distractibility, hyperactivity, impulsivity, hyperarousal, anxiety, mood dysregulation, and aggression. Medication response and side effect data were reviewed retrospectively for 257 patients (age 14 ± 11 years, range 4-60 years, 203 M, 54 F) attending an FXS clinic. Treatment success rates were defined as the percentage of positive response in the form of documented clinical report of improvement in the behavior(s) being targeted over at least a 6-month period on the medication, without side effects requiring medication discontinuance, while failures were defined as discontinuance of medication due to lack of clinical effectiveness or side effects. Success rate for treatment of targeted behaviors with trials of individual medications was 55% for stimulants, 53% for antidepressants, 62% for alpha2-agonists, and 54% for antipsychotics. With sequential trials of different medications in the same class, success rate improved to 73-77%. Side effect-related failures were highest for antipsychotics. Systematic psychopharmacologic intervention targeted to behavioral symptoms appears helpful in the majority of patients with FXS.

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Figures

Figure 1
Figure 1
Age and gender demographics of the FXS cohort in this study.
Figure 2
Figure 2
Response rate for major classes of psychopharmacological agents utilized. Response rate for “trials” indicates percent of positive responses for individual trials of medications within the class. Response rate for “patients” indicates percent of patients who responded positively to any medication within the class. Only the trial response rate is shown for alpha2-agonists because virtually all of these treatments were with clonidine and there were no patients with trials of two different medications in this class, thus the patient response rate is the same as the trial response rate for this medication class. No response data is shown for alpha2-agonists in females because no females were treated; the fraction of responders is zero for alpha2-agonists in males > 18.
Figure 3
Figure 3
Reason for failure of various classes of medications during treatment trials. Green areas of charts indicate percent of individuals for whom no follow-up information was available, Red areas indicate failure due to medication ineffectiveness, and Blue sections of charts represent individuals failing due to a side effect that limited treatment. For antidepressants and antipsychotics data is presented separately for children and adolescents less than 18 and adults 18 and over to compare rates of intolerable side effects in these age groups. For alpha2-agonists and stimulants, only 1 and 5 individuals, respectively, aged 18 or over failed treatment, all due to medication ineffectiveness. Therefore, age groups were not presented separately for these medication classes.
Figure 4
Figure 4
Response rates in patients with FXS to trials of different types of stimulants, fractionated by age and gender. Numbers above bars represent total number of patients in the indicated category treated with the stimulant type.
Figure 5
Figure 5
Results of sequential stimulant trials for the subgroup of individuals with FXS who had trials of both APH and MPH preparations (N = 64).
Figure 6
Figure 6
Response rates to different antidepressants, fractionated by age and gender. For bars with an asterisk, there were <5 trials of the indicated medication for the patient group represented by the bar.
Figure 7
Figure 7
Response rates to different antipsychotics, fractionated by age and gender. Numbers above bars represent total number of patients in the indicated category treated with the antipsychotic agent.
Figure 8
Figure 8
Response and failure rates in patients with FXS treated with aripiprazole, fractionated by age. This figure includes response data for aripiprazole from 7 additional patients with FXS started on this treatment between the end of December of 2005 and July of 2006.
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