Abstract
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.
Keywords
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Article info
Publication history
Published online: June 27, 2020
Accepted:
June 20,
2020
Received:
May 26,
2020
Footnotes
Conflict of interest: Dr. Mohamad Mikati reports that he has a pending patent application for therapy of alternating hemiplegia of childhood. The remaining authors declare that there are no conflicts of interests regarding the publication of this article.
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