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[Central motor conduction evaluation in glycogenosis type III]
J A Alaejos Fuentes1, T López-Alburquerque, J De Portugal Alvarez
1Servicio de Rehabilitación, Hospital Universitario de Salamanca.
Anales De Medicina Interna (Madrid, Spain : 1984)
|May 1, 1997
Summary
Glycogenosis type III causes distal muscle weakness, particularly in the legs. Central motor pathways are not involved in this neuromuscular disorder.
Area of Science:
- Neurology
- Metabolic Myopathies
Background:
- Glycogenosis type III (GSD III) is a rare inherited metabolic disorder affecting glycogen metabolism.
- It leads to the accumulation of glycogen in muscles and liver, causing progressive muscle weakness.
Observation:
- A 20-year-old male patient presented with progressive distal muscle weakness, predominantly affecting the lower extremities.
- Electromyography (EMG) revealed myopathic changes, indicative of muscle fiber dysfunction.
Findings:
- Nerve conduction studies (NCS) were normal, ruling out peripheral neuropathy.
- Central motor conduction studies using magnetic brain stimulation showed no abnormalities, suggesting intact central motor pathways.
Implications:
- These findings indicate that the muscle weakness in GSD III is primarily due to peripheral myopathy.
- The central nervous system's motor pathways appear unaffected, differentiating GSD III from other neuromuscular conditions with central involvement.