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Mobius syndrome: electrophysiologic studies in seven cases
S Jaradeh1, O D'Cruz, J F Howard
1Department of Neurology, Medical College of Wisconsin, Milwaukee 53226, USA.
Muscle & Nerve
|September 1, 1996
Summary
Mobius syndrome involves congenital facial diplegia and gaze impairment. Electrophysiologic studies suggest a brainstem dysfunction affecting facial nuclei, not muscles.
Area of Science:
- Neurology
- Neurophysiology
Background:
- Mobius syndrome presents with congenital facial diplegia and potential cranial nerve deficits.
- The precise location of the neurological dysfunction in Mobius syndrome is not well-established.
Observation:
- Electrophysiologic studies were conducted on seven individuals (five children, two adults) diagnosed with Mobius syndrome.
- Standard nerve conduction studies for sensory and motor functions in the limbs were within normal limits.
Findings:
- Facial muscle compound action potential amplitudes were diminished in all participants.
- Blink reflex R1 responses were absent in some patients, and R1/R2 latencies were prolonged.
- Electromyography indicated multifocal, chronic neurogenic alterations within the facial muscles.
Implications:
- These findings suggest a brainstem-level pathology in Mobius syndrome.
- The dysfunction appears to primarily involve the facial nerve nuclei and their connections, rather than supranuclear pathways or the muscles themselves.
