Case report

STIFF PERSON SYNDROME AND THORACIC HYDROMYELIA

DENIS ČERIMAGIĆ orcid.org/0000-0003-0743-2618 ; Dubrovnik General Hospital, Department of Neurology, Dubrovnik, Croatia
ERVINA BILIĆ ; Department of Neurology, Zagreb University Hospital Centre, School of Medicine, University of Zagreb, Zagreb, Croatia
VANJA BAŠIĆ KES ; Sestre milosrdnice University Hospital Centre, Department of Neurology, Zagreb, Croatia

Abstract

Here we present for the fi rst time a case of a patient with stiff person syndrome and associated thoracic hydromyelia and positive antibodies to glutamate decarboxylase. To our knowledge, only one case of antiglutamate decarboxylase positive stiff dog syndrome with associated cervical syringomyelia due to Arnold-Chiari malformation has been described in the literature so far. Glutamate decarboxylase is an enzyme necessary for the synthesis of gamma-aminobutyric acid, an inhibitory neurotransmitter. Gamma-aminobutyric acid defi ciency leads to disturbance of the equilibrium of excitatory and inhibitory effects on the anterior horn alpha-motoneurons at the level of spinal cord and consequent continuous muscle stimulation, which is clinically presented by rigidity and uncontrolled muscle spasms. Hydromyelia hypothetically may cause damage to and loss of GABA-ergic neurons of the spinal cord leading to an immune response to the newly exposed glutamate decarboxylase antigens. We can assume that a number of patients with stiff person syndrome have not received complete diagnosis and therefore are not optimally treated.

Keywords

stiff person syndrome; hydromyelia; glutamic acid decarboxylase; gamma aminobutyric acid

Hrčak ID:

259275

URI

https://hrcak.srce.hr/259275

Publication date:

19.6.2021.

Article data in other languages: croatian

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