Recenzija, Prikaz slučaja
STIFF PERSON SYNDROME AND THORACIC HYDROMYELIA
DENIS ČERIMAGIĆ
orcid.org/0000-0003-0743-2618
; Opća bolnica Dubrovnik, Odjel za neurologiju, Dubrovnik, Hrvatska
ERVINA BILIĆ
; Sveučilište u Zagrebu, Medicinski fakultet i Klinički bolnički centar Zagreb, Klinika za neurologiju, Zagreb, Hrvatska
VANJA BAŠIĆ KES
; Klinički bolnički centar Sestre milosrdnice, Klinika za neurologiju, Zagreb, Hrvatska
Sažetak
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.
Ključne riječi
stiff person syndrome; hydromyelia; glutamic acid decarboxylase; gamma aminobutyric acid
Hrčak ID:
259275
URI
Datum izdavanja:
19.6.2021.
Posjeta: 1.891 *