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[Peripheral neuropathies simulating amyotrophic lateral sclerosis in gammopathies]
Revue Neurologique
|January 1, 1988
Summary
Certain benign gammapathies are linked to amyotrophic lateral sclerosis (ALS). Early detection of infra-clinical neuropathy in ALS patients is crucial for identifying associated gammapathies.
Area of Science:
- Neurology
- Immunology
- Pathology
Context:
- Amyotrophic lateral sclerosis (ALS) can present with unique clinical features when associated with benign gammapathies.
- Observational study of 12 patients aged 57-74 with benign gammapathy and ALS symptoms.
Purpose:
- To identify particular characteristics of ALS associated with benign gammapathies.
- To investigate the relationship between gammapathy and ALS pathology and clinical presentation.
Summary:
- Observed 12 patients with benign gammapathy (IgG, IgA, IgM) and progressive motor neuron involvement.
- Key findings include asymmetrical onset, rare bulbar signs, sub-clinical sensory neuropathy, elevated CSF protein with monoclonal component, and paraprotein fixation on nerve biopsies.
- The association between ALS and gammapathy is supported by epidemiological, experimental, and pathological evidence.
Impact:
- Highlights the importance of screening for gammapathy in ALS patients, especially those with infra-clinical neuropathy.
- Suggests a non-fortuitous link between ALS and gammapathy, warranting further investigation into immunological treatments.