Paroxysmal hemipareses in childhood

Area of Science:

• Pediatric Neurology
• Neurophysiology
• Clinical Neuroscience

Background:

• Presents three pediatric cases with recurrent, transient paresis since infancy.
• Paresis episodes manifest as flaccid hemiparesis, monoparesis, or quadriparesis with variable laterality and severity.
• Attacks are characterized by preserved consciousness, sometimes preceded by ocular motor disturbances or seizures.

Purpose of the Study:

• To investigate the underlying etiology of recurrent transient paresis in infants.
• To characterize the clinical presentation and neurophysiological findings in affected children.
• To explore potential neuroanatomical correlates, specifically brain-stem dysfunction.

Main Methods:

• Clinical case series observation.
• Neurological examination including assessment of motor function, consciousness, and ocular movements.
• Electromyography (EMG) during paretic episodes to assess motor neuron integrity.
• Electroencephalography (EEG) to evaluate brain electrical activity.
• Neuroradiological imaging and biochemical analyses to exclude other causes.

Main Results:

• Patients exhibited transient, episodic paresis with preserved consciousness.
• Interparoxysmal periods revealed hypotonia, extrapyramidal signs, and intellectual disability (oligophrenia).
• EMG indicated central motor neuron lesions; EEG showed non-specific paroxysmal activity. Neuroradiology and basic biochemistry were unremarkable.

Conclusions:

• Transient hemiparesis attacks in infancy, coupled with specific neurological signs, point towards a central nervous system disorder.
• The findings suggest a potential brain-stem dysfunction as the underlying etiological factor.
• Further investigation is warranted to elucidate the precise mechanisms and diagnostic markers for this condition.