Characterization of Severe and Extreme Behavioral Problems in Patients With Alternating Hemiplegia of Childhood
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Keri Wallace1, Julie Uchitel1, Lyndsey Prange1
1Division of Pediatric Neurology and Developmental Medicine, Duke Children's Health Center, Durham, North Carolina.
Insights
Alternating hemiplegia of childhood (AHC) frequently involves severe behavioral issues. Management strategies include behavioral therapy and medications like alpha-adrenergic agonists and SSRIs for AHC patients.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroscience
Background:
- Alternating hemiplegia of childhood (AHC) is a rare neurological disorder characterized by recurrent, usually unilateral, episodes of dystonia, choreoathetosis, and hemiparesis.
- Behavioral problems are common in AHC, but their spectrum and severity require further characterization.
Purpose of the Study:
- To analyze the occurrence and nature of behavioral problems in patients with Alternating hemiplegia of childhood.
- To categorize behavioral problems by severity and analyze manifestations, comorbidities, and treatment responses in severe/extreme cases.
Main Methods:
- Retrospective analysis of 39 consecutive patients diagnosed with Alternating hemiplegia of childhood.
- Categorization of behavioral problems into mild, moderate, severe, and extreme based on intervention needs and risk.
- Analysis of concurrent diagnoses and medication efficacy for severe/extreme behavioral symptoms.
Main Results:
- 41% of AHC patients experienced severe or extreme behavioral problems, with extreme cases showing assaults.
- Triggers included peer provocation, low frustration tolerance, and sleep disruption. Reversible psychotic symptoms occurred in two patients.
- 13 out of 16 patients with severe/extreme symptoms had concurrent neuropsychiatric diagnoses. Alpha-adrenergic agonists and SSRIs showed efficacy in over 50% of treated patients.
Conclusions:
- Severe behavioral problems, including rare medication-triggered psychosis, are frequent in AHC.
- These findings align with the known pathophysiology of AHC.
- Awareness and multidisciplinary management are crucial for effective AHC patient care.
Background:
Alternating hemiplegia of childhood often manifests severe or extreme behavioral problems, the nature of which remains to be fully characterized.
Methods:
We analyzed 39 consecutive patients with alternating hemiplegia of childhood 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, others, or both), and extreme (major risk). We then analyzed behavioral manifestations, concurrent morbidity, and medication responses in patients with severe or extreme symptoms.
Results:
Two patients had mild behavioral problems, five moderate, 10 severe, six 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 two patients: in one triggered by infection and trihexyphenidyl, and in another triggered by sertraline. Of the 16 patients with severe or extreme symptoms, 13 had concurrent neuropsychiatric diagnoses. Occurrence of severe or 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 comanaged all patients with severe or extreme symptoms with either behavioral therapy, medications, or both. When considering medications prescribed to more than four patients, medicines that demonstrated efficacy or partial efficacy in more than 50% of patients were alpha-adrenergic agonists and selective-serotonin-reuptake-inhibitors.
Conclusions:
Patients with alternating hemiplegia of childhood (41%) often experience severe or extreme behavioral problems and, rarely, medication-triggered psychotic symptoms. These observations are consistent with current understanding of underlying alternating hemiplegia of childhood brain pathophysiology. Increasing awareness of these behavioral problems facilitates alternating hemiplegia of childhood management and anticipatory guidance.
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