Table 1

Neurological syndromes with positive anti-glutamic acid decarboxylase antibodies.

DisorderSymptom
Stiff-person syndrome (SPS)15-21Progressive 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 ataxia38Lack of voluntary coordination of muscle movements
Epilepsy38Seizures - violent shaking and loss of alertness
Limbic encephalitis41,43,44Short-term memory deficits, headache, irritability, sleep disturbance, delusions, hallucinations, agitation, seizures and psychosis
Dancing eye syndrome40-45Multivectorial eye movements, involuntary multifocal myoclonus, and cerebellar ataxia
Miller Fisher syndrome48Acute 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