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Electrophysiological brainstem dysfunction in a child with Leigh disease
1Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Japan.
Pediatric Neurology
|May 1, 1997
Summary
Leigh disease can cause brainstem dysfunction, impacting sleep and neurological responses. Multimodality testing aids in assessing these complex neurological deficits.
Area of Science:
- Neurology
- Neurophysiology
Background:
- Leigh disease is a severe genetic neurometabolic disorder.
- Brainstem dysfunction is a significant complication in Leigh disease.
Observation:
- A patient with Leigh disease underwent comprehensive neuroradiological and neurophysiological evaluations.
- Brain magnetic resonance imaging and [123I]IMP SPECT revealed abnormalities in the basal ganglia and midbrain.
- Auditory brainstem responses (ABRs), short-latency somatosensory evoked potentials (SSEPs), and blink reflexes (BRs) demonstrated progressive brainstem disturbances.
- Polysomnography (PSG) indicated disruptions in REM sleep.
Findings:
- [123I]IMP SPECT showed reduced tracer accumulation in the basal ganglia and midbrain.
- Serial ABRs and SSEPs revealed progressive impairment of brainstem wave components.
- Impaired R2 components of blink reflexes were observed.
- Disturbances in both tonic and phasic REM sleep components were detected via PSG.
Implications:
- Multimodality testing, combined with neuroradiology, is valuable for evaluating brainstem dysfunction in Leigh disease.
- These assessments can help monitor disease progression and guide therapeutic strategies.
- Understanding brainstem involvement is crucial for managing Leigh disease patients.
