Abstract
Stiffness, a main clinical feature in stiff-person spectrum disorders (SPSD), can be mimicked by other phenomenologies such as spasticity, cramps, and dystonia. This chapter discusses how to differentiate stiffness from these entities. SPSD encompass various phenotypes and are classified into classic stiff-person syndrome (SPS) and SPS variants including stiff-limb syndrome, progressive encephalomyelitis with rigidity and myoclonus, and overlapping syndrome. The terms “jerking SPS” and “SPS-plus” have been used confusingly. Hyperekplexia shares some similarities, but it is not equivalent to SPSD.
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