Determine the abnormalities in cognitive, behavioral and adaptive functions in patients with Alternating Hemiplegia of Childhood (AHC).

AHC is a complex neurological disorder. Despite two prior single case reports the full spectrum of neuropsychological profile in AHC patients has not yet been studied.

In this institutional review board approved study, 25 sequential AHC patients were administered assessments irrespective of the nature of prior complaints. These included, as applicable to level of development, CBCL (Child Behavior Checklist), Wechsler Intelligence Quotient (WISC) tests, clinical history, developmental milestones, school placement, Ages Stages Questionnaires (ASQ) problem solving score, and Capute Cognitive Adaptive Test (CAT). Results were entered into a prospective AHC database and analyzed by comparing them to published population means using 1-sample two sided t-test.

Cognitive function level was typical in 4/25 (16%), borderline in 3/25 (12%), mildly impaired in 6/25 (24%), moderately impaired in10/25 (40%) and severely impaired in 2/25 (8%). 10/25 had a functioning capacity high enough to complete the WISC. In those both the nonverbal (mean + SD 68.7+21.4) and verbal IQ (78.4+26.4) were lower than population means (100+15, p=0.0013, and p=0.0293). There was no significant difference between verbal and nonverbal IQs (p=0.913, Wicoxon test). The following CBCL domains were different from those of the general population: Thought, Social Problems, Pervasive Developmental, Affective, Sleep, Aggressive, Oppositional Defiant,  Somatic Complaints, Withdrawn Anxiety, Anxious/Depressed, Social Attention, and ADHD (p<0.0148 in all comparisons). Additionally, we diagnosed 20 individuals with neuropsychiatric DSM V diagnosis: ADHD (10) disruptive behavior (7) and anxiety disorder (3). 8/9 ADHD patients treated with stimulant therapy responded to it.

 

The majority of the patients with AHC have a behavioral and intellectual disability related problems that should be screened for. Several neuropsychiatric disorders coexist with ADHD being the most common. Stimulant therapy helps the majority of the ADHD patients.