Objective: To extend spectrum of glutamic acid decarboxylase(GAD) associated disorders by reporting a case

Background: GADA associated disorders include a broad and clinically heterogeneous group of conditions. Among them, stiff-person syndrome (SPS),cerebellar ataxia(CA) and limbic encephalopathy(LE) are common neurologic presentation. So far, more and more cases have showed that multiple neurologic syndromes could occur with anti-GADA,which can coexist in a single patient with different kinds of combination.

Method: A 29-year-old Chinese woman presented with paroxysmal numbness in left-sided limbs, slight memory loss and stiffness in trunk 22 months prior to admission,followed by gait problems and stiffness in left leg in 1 year.Neurological examination showed slight impairment of memory and calculation. Muscle tone increased in left limbs with predominance in left lower limb. Pathological signs were not noted. A stiff,split-step gait and disturbances of initiating left lower limb were present. Accompanied movements in left arm reduced during walking. Cranial nerve function, muscle strength and sensory examination were normal. No ataxia was present. No specific history was noted. EEG demonstrated spikes in frontal and temporal lobes with T2/T2flair hyperintensities in bilateral hippocampi on brain MR. EMG demonstrated continuous motor unit activity in left sacrospinal muscle and left rectus abdominis. A  titer of 1:320 GAD65 was identified in both serum and CSF. Other antibodies such as NMDA, AMPA1, AMPA2, GABA2,LGI-1 and CASPR-2 was negative.Tumor markers,organic acids,ANA and ENA related autoimmune antibodies were absent.Seizures and motor symptoms relieved after treatment of IVIG and continuous glucocorticoid. Titer of GAD65 in serum decreased to 1:100 during follow-up.

Results: We diagnose the patient as GADA associated neurological syndrome presenting with limbic encephalitis and stiff-person syndrome, which responded to IVIG and glucocorticoid.

Conclusion: LE and SPS associated with anti-GADA could coexist and respond to immunomodulatory therapy.

References: 1.Damato,V. et al. Mov Disord, 2018. 33(9): p.1376-1389. 2.Incecik, F.et al. Acta Neurol Belg, 2018. 118(3): p. 411-414. 3.Jung, Y.J.et al.J Mov Disord, 2014. 7(1): p.19-21.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/stiff-person-syndrome-and-limbic-encephalopathy-associated-with-gad-antibody-a-case-report/