Related Experiment Videos
Isaacs' syndrome associated with chronic inflammatory demyelinating polyneuropathy
Z Odabasi1, J L Joy, G C Claussen
1Department of Neurology, University of Alabama at Birmingham, Veterans Affairs Medical Center 35294, USA.
Muscle & Nerve
|February 1, 1996
Summary
This study details the first documented case of Isaacs' syndrome with inflammatory demyelinating neuropathy. Treatment with phenytoin, prednisone, and azathioprine effectively managed symptoms and relapses over 17 years.
Area of Science:
- Neurology
- Immunology
- Histopathology
Background:
- Isaacs' syndrome, characterized by muscle stiffness and spasms, is typically associated with peripheral nerve hyperexcitability.
- Inflammatory demyelinating neuropathy has not been previously documented histologically in Isaacs' syndrome.
Observation:
- A patient presented with a 9-month history of progressive weakness, muscle spasms, stiffness, fasciculations, and myokymia.
- Clinical examination revealed motor conduction block and abnormal nerve excitability on electrophysiological studies.
- Sural nerve biopsy confirmed an inflammatory demyelinating neuropathy.
Findings:
- Electromyography showed continuous motor unit potentials at rest, responsive to peripheral nerve block and curarization, but not spinal anesthesia.
- Phenytoin provided relief for muscle cramping and stiffness.
- Prednisone and azathioprine led to gradual improvement in weakness and controlled three relapses over 17 years.
Implications:
- This case highlights the potential for an inflammatory demyelinating process in Isaacs' syndrome.
- The findings suggest that immunosuppressive therapy can be effective in managing this condition long-term.
- Histological confirmation aids in understanding the neurobiology of Isaacs' syndrome and guides therapeutic strategies.