Friday, July 3, 2020

Characterization of severe and extreme behavioral problems in patients with alternating hemiplegia of childhood

Keri Wallace, Julie Uchitel, Lyndsey Prange, Joan Jasien, Melanie Bonner, Richard D’Alli, Gary Maslow and Mohamad A. Mikati.  Characterization of Severe and Extreme Behavioral Problems in Patients with Alternating Hemiplegia of Childhood.  Pediatric Neurology.  In press.

About 41% of AHC patients have severe (26%) or extreme (15%) behaviors.
Genotype, age, puberty, and degree of ID do not correlate with behavior severity.
Medication adverse events may trigger behavioral problems, including psychosis.
Efficacy of medications for behavioral control varies among individual patients.
Collaboration with mental health professionals aids in management of AHC.


Alternating Hemiplegia of Childhood (AHC) often manifests severe or extreme behavioral problems, the nature of which remain to be fully characterized.

We analyzed 39 consecutive AHC patients for occurrence of behavioral problems and categorized those by severity: mild (not requiring intervention), moderate (requiring intervention but no risk), severe (minor risk to self and/or others) and extreme (major risk). We then analyzed behavioral manifestations, concurrent morbidity, and medication responses in patients with severe or extreme symptoms.

Two patients had mild behavioral problems, 5 moderate, 10 severe, 6 extreme and 16 none. Extreme cases exhibited disruptive behaviors escalating to assaults. Triggers, when present, included peer-provocation, low frustration tolerance, limits set by others, and sleep disruption. Reversible psychotic symptoms occurred in 2 patients; in one triggered by infection and trihexyphenidyl, and in another triggered by sertraline. Of the 16 patients with severe/extreme symptoms, 13 had concurrent neuropsychiatric diagnoses. Occurrence of severe/extreme symptoms did not correlate with age, puberty, severity of intellectual disability or mutation status (p>0.05). A multidisciplinary team including mental-health professionals co-managed all patients with severe/extreme symptoms with behavioral therapy and/or medications. When considering medications prescribed to a number of patients greater than 4, medicines that demonstrated efficacy or partial efficacy in > 50% of patients were alpha-adrenergic agonists and SSRIs.

AHC patients often (41%) experience severe/extreme behavioral problems and, rarely, medication-triggered psychotic symptoms. These observations are consistent with current understanding of underlying AHC brain pathophysiology. Increasing awareness of these behavioral problems facilitates AHC management and anticipatory guidance.

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