Stiff Person Syndrome (SPS) is a rare neurological disorder typically affecting adults, with infrequent cases reported in children and adolescents. The condition is characterized by the following clinical and pathophysiological features:
Clinical Features:
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Progressive Rigidity and Muscle Spasms:
- Predominantly involves the trunk and proximal limb muscles.
- Muscle rigidity persists during wakefulness and is relieved during sleep.
- Painful muscle spasms are common and can be spontaneous or triggered by external stimuli (e.g., sudden noise, touch, or emotional stress).
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Sensitivity to External Stimuli:
- Patients exhibit heightened sensitivity to environmental factors such as noise, touch, and emotional stress, which can exacerbate spasms and rigidity.
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Electromyographic (EMG) Findings:
- Continuous involuntary muscle fiber activity is observed in affected muscles, even at rest, which is pathognomonic for SPS.