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Sensory involvement in distal myopathy (Welander)
1Department of Neurology, Karolinska Hospital, Stockholm, Sweden.
Journal of the Neurological Sciences
|September 1, 1987
Summary
Welander distal myopathy patients show significant sensory impairments, particularly in thermal sensibility. This suggests a peripheral sensory neuropathy affecting both myelinated and unmyelinated nerve fibers.
Area of Science:
- Neurology
- Clinical Neuroscience
- Peripheral Neuropathy
Background:
- Welander distal myopathy is a rare genetic disorder affecting distal muscles.
- Sensory function in Welander distal myopathy has not been extensively studied.
Purpose of the Study:
- To investigate sensory perception thresholds in patients with Welander distal myopathy.
- To determine the extent and type of sensory neuropathy in this condition.
Main Methods:
- Detailed sensory testing, including vibration and temperature perception thresholds, was performed on 11 patients.
- Results were compared to age-corrected normal values and a control group with poliomyelitis.
Main Results:
- All patients exhibited impaired thermal sensibility.
- Nine patients had impaired vibration or thermal thresholds compared to normal values.
- Significant thermal and vibration abnormalities were noted when compared to paretic controls.
Conclusions:
- Sensory impairment, including both myelinated and unmyelinated fiber involvement, is a feature of Welander distal myopathy.
- Findings indicate a peripheral sensory neuropathy associated with this myopathy.