Alternating hemiplegia of childhood (AHC) is a pretty rare disorder associated with recurrent attacks of hemiplegia (paralysis of half of the body) that can affect either side of the body, eye movement abnormalities, disorders of movement and progressive developmental delay in children.
What is the pathology behind alternating hemiplegia of childhood ?
Though the cause of Alternating Hemiplegia of Childhood (AHC) remains unclear, it is most likely a symptom complex with multiple contributory causes. The neurophysiologic findings during an attack have demonstrated impaired brainstem circuits.
There are abnormal smooth muscle cells in the blood vessels found in the brain which could be causing a functional vascular disorder causing transitory small blood vessel dysfunction in the brain.
How is the alternating hemiplegia of childhood passed on ?
It is a genetic disease, associated with gene mutations. The pathophysiology of AHC was totally unknown until the recent identification of mutations in ATP1A3, which encodes a neuron specific sodium/potassium ATPase involved in the regulation of neuronal excitability.
What are the symptoms of alternating hemiplegia of childhood ?
This condition is characterized by recurrent hemiplegic attacks that usually last from anything between a few minutes to several days. They are associated with slowly progressive neurologic deficits that accumulate over years. The syndrome mostly begins with abnormal eye or ocular movements (such as nystagmus or lagging of one eye behind the other) or dystonia in most patients by the age of 3 months.
Motor findings in an attack include a flaccid kind of paralysis, with lack of response to stimuli also. Attacks are commonly associated with environmental triggers such as temperature extremes, strong odours, water exposure, physical activities (like exercise and swinging), lights (like sunlight and fluorescent bulbs), or even foods (like chocolates and artificial coloring agents).
How is alternating hemiplegia of childhood diagnosed ?
- Presenting symptoms should be there before the age of 18 months, in infants.
- Repeated, recuurent attacks of hemiplegia.
- Episodes of quadriplegia (affecting all four limbs) either as a separate attack or as a part of the general hemiplegic attack.
- Symptomatic relief upon sleeping
- The other symptoms will include eye movement abnormalities, or other autonomic symptoms either concurrent with attacks or that occur independently, as well.
- Evidence of developmental delay or other neurologic findings.
How is alternating hemiplegia of childhood treated ?
Treatment of Alternating Hemiplegia of Childhood is divided both into acute management of attacks and prevention of attacks or episodes. Acute management will consist of removing all possible triggers and the prompt facilitation of sleep, as sleep relieves attacks. Rapid sedation will have to be administered. Episode prophylaxis will also consist of avoiding all triggers over and above long-term drug treatment. For its treatment, a wide range of medications have been proposed for Alternating Hemiplegia of Childhood but calcium channel blockers are the most effective drugs.
What is the prognosis for alternating hemiplegia of childhood ?
The long-term prognosis for patients with alternating hemiplegia of childhood is mostly poor because of associated developmental delays and progressive deterioration after severe recurrent attacks. It is still unclear if pharmacologic therapy improves learning, but definitely it lessens the severity and frequency of attacks leading to an enhanced quality of life.
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
