Stiff-person syndrome (SPS)15-21 | Progressive rigidity of the truncal muscles, painful spasms and continuous motor activity and an exquisite sensitivity to |
external stimuli Continuous co-activation of agonist and antagonist muscles, particularly “core muscles” - paraspinal and abdominal muscles |
Rigidity and painful spasms of the lumbar paraspinal, abdominal, and occasionally proximal leg muscles associated with a lumbar hyperlordosis |
Classic SPS, which affects the lumbar, trunk, and proximal limb muscles |
SPS-plus syndrome, which consists of (a) the stiff-limb subtype, in which symptoms are limited to the lower limbs; (b) jerking stiff-man syndrome, characterized by chronically progressive stiffness and myoclonus; and (c) acute onset and progressive encephalomyelitis with rigidity and myoclonus |
Cerebellar ataxia38 | Lack of voluntary coordination of muscle movements |
Epilepsy38 | Seizures - violent shaking and loss of alertness |
Limbic encephalitis41,43,44 | Short-term memory deficits, headache, irritability, sleep disturbance, delusions, hallucinations, agitation, seizures and psychosis |
Dancing eye syndrome40-45 | Multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia |
Miller Fisher syndrome48 | Acute onset of external ophthalmoplegia, ataxia, mild limb weakness, ptosis, facial palsy, or bulbar palsy. Patients have reduced or absent sensory nerve action potentials and absent tibial Hoffmann’s reflex |