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[Alternating hemiplegia of childhoood and oculomotor anomalies]
J Bursztyn1, Y Mikaeloff, A Kaminska
1Consultation d'Ophtalmologie, Hôpital Saint Vincent de Paul, 74-82, avenue Denfert Rochereau, 75674 Paris Cedex 14.
Insights
Alternating hemiplegia of childhood (AHC) is a rare neurological disorder presenting with recurrent hemiplegic attacks and abnormal eye movements. Early diagnosis, aided by oculomotor signs, is crucial for managing this non-epileptic condition with a poor prognosis.
Area of Science:
- Pediatric Neurology
- Neuroscience
- Rare Diseases
Background:
- Alternating hemiplegia of childhood (AHC) is a rare neurological disorder characterized by recurrent episodes of unilateral or bilateral hemiplegia or hemiparesis.
- Onset typically occurs within the first year of life, often accompanied by paroxysmal manifestations like dystonia, episodic nystagmus, and neurovegetative disturbances.
Observation:
- Neurological anomalies may appear in the neonatal period, but typical attacks usually manifest after one year of age.
- Oculomotor anomalies, including paroxysmal nystagmus, strabismus, and ocular deviations, are key diagnostic indicators, present in most cases.
- Attacks are transient, lasting from 30 seconds to 3 minutes, and are not associated with epileptic EEG findings.
Findings:
- The diagnosis of AHC can be made before one year of age based on oculomotor signs and other symptoms, even without clear EEG evidence of epilepsy.
- Paroxysmal nystagmus, often horizontal and pendular, is a consistent finding, sometimes associated with mydriasis or ocular deviations.
- AHC is a non-epileptic, sporadic condition with an unknown pathogenesis and a generally poor prognosis.
Implications:
- The presence of specific oculomotor signs strongly suggests a diagnosis of Alternating Hemiplegia of Childhood.
- Early identification of AHC is critical for appropriate management and supportive care, despite the condition's poor prognosis.
- Further research into the pathogenesis of AHC is warranted to improve treatment strategies and patient outcomes.
Background:
Alternating hemiplegia of childhood is a syndrome which begins in the first year of life. It is characterized by repeated attacks of uni-or bilateral hemiplegia or hemiparesia. In most cases paroxysmal manifestations are observed: movements or dystonia++ attacks, episodic nystagmus, abnormal eye movements and disturbance of the neurovegetative system, predominantly in the first year of life.
Analysis:
In half of the cases, neurological anomalies begin during the neonatal period with a non characteristic aspect. Typical attacks take place after one year of life, sometimes associated with partial epilepsy. In a quarter of cases, the oculomotor anomalies have been known since early life. The diagnosis is made prior to one year on the basis of associated oculomotor anomalies and other symptoms without EEG arguments for epilepsy. Paroxysmal nystagmus is always found. One eye is affect in most cases, generally with horizontal and seldom with vertical movements of large variable pendular amplitude. One eye with nystagmus and the other with mydriasis is sometimes reported. Most attacks last from 30 sec to 3 min. Paroxysmal strabismus described in half of the cases seems to be generally unilateral internuclear transitory ophthalmoplegia. Finally, ocular deviations on the hemiparetic side are described. They are generally unique or sometimes associated with head deviation. Spontaneous blinking is reduced.
Conclusion:
Alternating hemiplegia of childhood is a non-epileptic sporadic, paroxysmal manifestation of unknown pathogenesis. Prognosis is poor. The presence of oculomotor signs suggests the diagnosis.