The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine.
Neurophysiological investigations of a patient suffering from the stiff-man syndrome revealed that exteroceptive reflexes, in particular those elicited from the skin, were excessively enhanced. In contrast, no abnormalities were found within the monosynaptic reflex arc. Clomipramine injection severely aggravated the clinical symptoms whereas diazepam, clonidine, and tizanidine decreased both muscular stiffness and abnormal exteroceptive reflexes. The hypothesis is put forward that the stiff-man syndrome is a disorder of descending brain-stem systems which exert a net inhibitory control on axial and limb girdle muscle tone as well as on exteroceptive reflex transmission. Detection of abnormal exteroceptive reflex activity in conjunction with neuropharmacological testing might help in the diagnosis of this rare disease.
D000328:Adult; D001933:Brain Stem / Q000503:physiopathology; D002997:Clomipramine / Q000009:adverse effects*; D003000:Clonidine / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D003975:Diazepam / Q000627:therapeutic use; D004576:Electromyography; D005260:Female; D006801:Humans; D009127:Muscle Rigidity / Q000188:drug therapy / Q000503:physiopathology*; D009135:Muscular Diseases / Q000503:physiopathology; D010812:Physical Stimulation; D012021:Reflex, Abnormal / Q000503:physiopathology*; D013035:Spasm / Q000503:physiopathology; D013577:Syndrome
C023754:tizanidine; D003000:Clonidine; D002997:Clomipramine; D003975:Diazepam