Fig. 1

Pathophysiology of movement disorder emergencies (“storms”) including dystonic storm. This figure demonstrates proposed the pathophysiology of several movement disorder emergencies including dystonic storm (green), serotonin syndrome (increased serotonin leading to brainstem-mediated process; turquoise), malignant hyperthermia (purple) from peripheral muscle disorders such as secondary to ryanodine receptor (RYR1) mutations, neuroleptic malignant syndrome (from dopaminergic blockade leading to hypothalamic storm; blue), and others which lead to increased spinal-mediated rigidity (such as intrathecal baclofen withdrawal, autoimmune disesase e.g. stiff person syndrome or progressive encephalomyelitis with rigidity and myoclonus, and toxin-mediated disorders e.g. tetanus; yellow). These disorders lead to the final common pathway characterized by increased spinal-mediated muscle tone, fever, rigidity, elevated creatine kinase and leukocytosis. Abbreviations: BBGD, biotin-thiamine-responsive basal ganglia disease; CK, creatine kinase; ITB, intrathecal baclofen; ODS, osmotic demyelinating syndrome; WBC, white blood cells; WD, Wilson’s disease