Stiff Person Syndrome
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Definition[edit | edit source]
Stiff person syndrome (SPS) is a rare acquired neurological disorder characterized by fluctuating muscle rigidity and stiffness, painful spasms, and continuous motor unit activity (1, 2, 3). This condition was originally described as “stiff man syndrome” (SMS) by Moersch and Woltman in 1956. They reported observations from 14 cases of patients who experienced progressive stiffness, episodic painful muscle spasms, and difficulty walking without any other neurological signs (4). The rigidity and continuous motor unit activity is most pronounced in muscles of the trunk (thoracolumbar paraspinals and rectus abdominis), but may also occur in the legs and arms, and persists even when relaxation is attempted (3).
SPS includes the following variants: classical SPS, focal SMS, Stiff Leg Syndrome (SLS), jerking SMS, progressive encephalomyelitis with rigidity and myoclonus (PERM) paraneoplastic SMS, gait ataxia, dysarthria, and abnormal eye movements (1, 3).
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