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Last updated: 04 January 2025 Print

Stiff Person Syndrome

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Stiff Person Syndrome

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:

  1. 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).
  2. Sensitivity to External Stimuli:

    • Patients exhibit heightened sensitivity to environmental factors such as noise, touch, and emotional stress, which can exacerbate spasms and rigidity.
  3. Electromyographic (EMG) Findings:

    • Continuous involuntary muscle fiber activity is observed in affected muscles, even at rest, which is pathognomonic for SPS.

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