[MIM 184 850]

(Moersch and Woltman syndrome, stiff person syndrome)

Prevalence estimated at 1-9/106. Acquired neurological pathology (in contrast to hyperexplexia, which is congenital, see this topic). Women are especially affected. The peak incidence is around 45 years of age.

Clinical presentation:

-        fluctuating rigidity of the trunk, abdomen and limbs, of slow and gradual onset

-        abrupt and painful muscle spasms, either spontaneous or triggered by noise, emotion or physical contact; risk of falls

-        ankolyzing joint deformities.

The origin is probably autoimmune as one finds, in 70% of cases, antibodies against the isomerase 65 of the glutamate decarboxylase (GAD65), which blocks the synthesis of GABA, one of the main inhibitory neurotransmitters of the central nervous system. Anti GLY-R antibodies (receptors to glycine) have been observed in other cases. In 10 % of cases, antibodies against amphiphysin, an intracellular protein associated with the endocytosis of synaptic vesicles are present. Those patients suffer generally from thymoma, lymphoma or breast or lung cancer.

Diagnosis: clinical, presence of autoantibodies anti-GAD or anti-GLY-R, typical EMG .

There are two variants of the classical form:

-        a form limited to a limb, often of paraneoplastic origin

-        a form with progressive encephalomyelitis and myoclonus (PERM: Progressive Encephalomyelitis with Rigidity and Myoclonus) the prognosis of which is severe. It associates a fluctuating rigidity of the trunk and limbs, painful muscular spasms, pseudo-agoraphobia, heightened sensitivity to external stimuli and ankylosing joint deformities (by example, lumbar posture fixed in hyperlordosis)

Many patients suffering from this disease have also other autoimmune diseases: diabetes (30%), thyroiditis (10%), atrophic gastritis with pernicious anemia, adrenal insufficiency, vitiligo, cerebellar ataxia.

Treatments: diazepam, clonazepam, baclofen (p.os or intrathecal pump), gabapentin. Sometimes corticosteroid therapy, gamma globulin IV or plasmapheresis with mixed results. Propofol has shown dramatic effects in some cases, pending the result of an alternative treatment.

Anesthetic implications:

presence of other autoimmune diseases ? Drug interaction with diazepam and especially baclofen: major risk of acute withdrawal in case of cessation of the treatment. Risk of postoperative hypotonia.  It may be preferable to use propofol-based total IV anesthesia to reduce the risk of hypotonia associated with halogenated agents. Curarization monitoring: a case of clinical prolonged hypotonia  despite a complete electric decurarization has been described, but succinylcholine and  non-depolarizing muscle relaxants have been used without problems in many cases. Regional anesthesia is feasible but it necessitates some sedation to avoid emotions, a possible source of spasms, not controlled by a neuraxial block.

References : 

-        Johnson JO, Miller KA.
Anaesthetic implications in stiff-person syndrome.
Anesth Analg 1995; 8: 612-3.

-        Obara M, Sawamura S, Chinzei M, Komatsu K, Hanaoka K.
Anaesthetic management of a patient with Stiff-person syndrome.
Anaesthesia 2002; 57: 511

-        Shanthanna H.
Stiff man syndrome and anaesthetic considerations: successful management using combined spinal epidural anaesthesia.
J Anaesthesiol Clin Pharmacol 2010; 26: 547-8.

-         MA, Tran NV, Kaye AD.
Anesthesia considerations in stiff person syndrome.
M E J Anesth 2013; 22:217-21.

-        Cassavaugh JM, Oravitz TM.
Multiple anesthetics for a patient with stiff-person syndrome.
J Clin Anesth 2016; 31: 197-9.

-        Anesthesia considerations for patients suffering from stiff man syndrome.
Orphananesthesia.eu

-        Sanefuji M, Torisu H, Kira R, Yamashita H et al.
A case of childhood stiff-person syndrome with striatal lesions: a possible entity distinct from the classical adult form.
Brain & Development 2013; 35: 575-8.

-        Boettcher BT, Muravyea M.
Anesthetic management of a parturient with Stiff person syndrome for urgent cesarean delivery.
Int J Obstetr Anesth 2016; 85-88

-        Wallet F, A.Didelot A, Delannoy B, Leray V, Guerin C.
PERM sévère mimant un tétanos.
Ann Fr Anesth Réanim 2014 ; 33 : 530-2.

-        Bouw J, Leendertse K, Tijssen MAJ, Dzoljic M.
Stiff Person syndrome and anesthesia: case report.
Anest Analg 2003;97:486–7

-        Ledowski T, Russell P.
Anaesthesia for stiff person syndrome: successful use of total intravenous anaesthesia.
Anaesthesia 2006 ; 61 :725

-        Jaun O-K, Yum M-S, Kim E-H, Lee Y-J, Seo J-J, Ko T-S..
A rare case of childhood Stiff Person Syndrome associated with pleuropulmonary blastoma.
Pediatr Neurol 2015 ; 53 :448-51. 

-        Elsherbini N, Weingartshofer A, Backman SB.
Postoperative hypotonia in a patient with stiff person syndrome: a case report and literature review.
Can J Anesth 2022 ; 69:1419–25

-        Morita H, Kinoshita H, Kiyokawa M, Kushikata T, Hirota K.
Remimazolam and remifentanil anesthetics for an adolescent patient with Stiff-Person syndrome: a case report.
A&A Practice 2024;18:e01758.

Updated: March 2024