Alternating Hemiplegia of Childhood: A Study of Neuropsychological Functioning

International Classification of Functioning, Disability and Health in subjects with alternating hemiplegia of childhood

“…Communication problems were reported with regard to activity and participation. In particular, consistent with previous studies [11,15,18], verbal and nonverbal comprehension skills seem to be better than production skills: from age 3 to 18 years, more than 30% of the participants showed difficulties in producing age-appropriate words, sentences or narratives. Since the early stages of development, patients with AHC showed significant impairments in interpersonal relationships in a contextually and socially appropriate manner, for example in tune with the feelings of others.…”

“…The delay can also explain why 76% of the individuals with AHC participating in this study show marked difficulties in sustaining attention. Consistent with Shafer et al's findings [18] these difficulties, which are age-independent, include both an impairment of attention regulation (e.g. moving the attention focus from one object to another) and attention sharing (e.g.…”

“…In line with a single case study of an 11-year-old girl [18], sleeping problems (falling asleep and staying asleep) were reported. This is particularly important because falling asleep plays a major role in the resolution of hemiplegia attacks [1].…”

“…Since the early stages of development, patients with AHC showed significant impairments in interpersonal relationships in a contextually and socially appropriate manner, for example in tune with the feelings of others. As already stated [18], interpersonal interaction skills can be markedly affected, not only by the cognitive delay but also by these communication difficulties. Consistent with the aforementioned neuromuscular problems, difficulties in independent movement were reported that are further responsible for a significant fall in quality of life.…”

The Expanding Spectrum of Neurological Phenotypes in Children With ATP1A3 Mutations, Alternating Hemiplegia of Childhood, Rapid-onset Dystonia-Parkinsonism, CAPOS and Beyond

“…As they reach adolescence, episodic hemiplegia, dystonia, and abnormal eye movements sometimes wane in frequency and severity, whereas concerns regarding behavior and autonomy become increasingly significant. 27 Unfortunately, only a few published studies to date help clarify specific details regarding the range and severity of neuropsychologic deficients in patients with AHC, 28 although multiple studies cite the nearly universal co-occurrence of both motor and cognitive developmental delay in children meeting clinical diagnostic criteria for the syndrome. 16–18,20 …”