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A Journal on Anesthesiology, Resuscitation, Analgesia and Intensive Care

Official Journal of the Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care
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Minerva Anestesiologica 2002 November;68(11):861-5

language: English

The stiff-person syndrome. Case report

Piovano C., Piattelli M., Spina T., Iervese G., Bosco G.

1 Chair of Anesthesia and Resuscitation “D’Annunzio” University, Chieti
2 Unit of Anesthesia, Resuscitation and Pain Therapy “Spirito Santo” Hospital, Pescara


The ­stiff-per­son syn­drome is a ­rare and dis­abling dis­or­der, char­ac­ter­ized by mus­cle rigid­ity ­with super­im­posed pain­ful ­spasms involv­ing ­axial and ­limb mus­cu­la­ture. The clin­i­cal symp­toms are con­tin­u­ous con­trac­tion of ago­nist and antag­o­nist mus­cles ­caused by invol­un­tary ­motor-­unit fir­ing at ­rest and the ­spasms ­that are pre­cip­i­tat­ed by tac­tile stim­u­li, pas­sive ­strach, voli­tion­al move­ment of affect­ed or unaf­fect­ed mus­cles, star­tling nois­es and emo­tion­al stim­u­li. ­Both the rigid­ity and the ­spasms are ­relieved by ­sleep, gen­er­al anaesthe­sia, myo­neu­ral block­ade and periph­er­al ­nerve block­ade. The ­cause of the ­stiff-per­son syn­drome is ­unknown but an auto­im­mune path­o­gen­e­sis is sus­pect­ed ­because 1) the pres­ence in the cere­bro­spi­nal ­fluid (CSF) of anti­bod­ies ­against glu­tam­ic ­acid decar­box­y­lase (GAD), the ­rate-lim­it­ing ­enzyme for the syn­the­sis of the inhib­i­to­ry neu­ro­tras­mit­ter γ-ami­no­bu­tyr­ic ­acid (­GABA), 2) the asso­ci­a­tion of the dis­ease ­with oth­er auto­im­mune dis­or­ders, 3) the pres­ence of var­i­ous auto­an­ti­bod­ies and 4) a ­strong immu­nog­e­net­ic asso­ci­a­tion. The ­stiff-per­son syn­drome is clin­i­cal­ly elu­sive but poten­tial­ly treat­able and ­should be con­sid­ered in ­patients ­with unex­plained stiff­ness and ­spasms. ­Drugs ­that ­enhance ­GABA neu­ro­tran­smis­sion, ­such as diaz­e­pam, vigab­a­trin and baclof­en, pro­vide mod­est ­relief of clin­i­cal symp­toms. Immu­nom­o­du­la­to­ry ­agents ­such as ster­oids, plas­ma­pher­e­sis and intra­ve­nous immu­no­glob­u­lin, ­seem to ­offer sub­stan­tial improve­ment.

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