Clinical spectrum of glutamic acid decarboxylase antibodies in a Taiwanese population
Journal
European Journal of Neurology
Journal Volume
26
Journal Issue
11
Pages
1384-1390
Date Issued
2019
Author(s)
Kuo Y.-C.
Abstract
Background and purpose: High levels of autoantibodies against glutamic acid decarboxylase (GAD-abs) are associated with stiff-person syndrome (SPS). However, the full clinical spectrum associated with GAD-abs in Asians is unclear. The clinical and immunological features of patients positive for GAD-abs were reviewed in a large Taiwanese series. Methods: Retrospective case series and immunological investigations were conducted between July 2007 and July 2017 at a tertiary referral centre in Taiwan. Amongst 361 patients with GAD-ab reactivity, 185 with detailed clinical records were included. Results: Twenty-seven patients (14.59%), with a mean age at assessment of 54.8?±?13.9?years, presented with neurological symptoms. The major neurological presentations (mean GAD-ab concentrations) were SPS (n?=?9, 33.3%; 135.45?±?27.84?U/ml), cerebellar ataxia (n?=?3, 11.1%; 95.61?±?49.63?U/ml), encephalopathy (n?=?2, 7.4%; 51.8?±?49.64?U/ml) and epilepsy (n?=?1, 3.7%; 83.3?U/ml). Notably, eight patients fulfilling the clinical diagnosis of multiple system atrophy had relatively lower GAD-ab concentrations (2.57?±?0.82?U/ml), which has not been reported previously. There was no correlation between disease severity and GAD-ab concentration. Patients presenting with comorbid endocrinopathies (n?=?15, 55.5%) had higher GAD-ab concentrations than those without endocrinopathies (n?=?12, 44.4%; 104.45?±?22.51?U/ml vs. 34.08?±?21.83?U/ml, P?=?0.04). Of 158 patients (85.4%) without a neurological presentation, 133 had type 1 diabetes mellitus and 20 had diabetes of other aetiologies (type 2 or gestational diabetes mellitus, or diabetes secondary to pancreatitis); the remaining four patients had pure thyroid disorders. Conclusions: A clinical and immunological evaluation of East Asian patients positive for GAD-abs is presented and a different clinical spectrum of anti-GAD syndrome is identified compared to Caucasians. ? European Academy of Neurology 2019
Subjects
ataxia; epilepsy; glutamic acid decarboxylase antibodies; multiple system atrophy; neurological syndrome; stiff-person syndrome
SDGs
Other Subjects
azathioprine; glutamate decarboxylase antibody; hydroxychloroquine sulfate; immunoglobulin; levodopa; methylprednisolone sodium succinate; mycophenolic acid; pramipexole; prednisolone; steroid; antibody; autoantibody; glutamate decarboxylase; adult; aged; antibody blood level; Article; ataxia; brain disease; cerebellar ataxia; clinical feature; comorbidity; diabetes mellitus; disease severity; endocrine disease; epilepsy; female; gait disorder; Guillain Barre syndrome; human; hyperlipidemia; hypertension; immunotherapy; insulin dependent diabetes mellitus; major clinical study; male; malignant neoplasm; middle aged; myasthenia gravis; neuroimaging; neurologic disease; non insulin dependent diabetes mellitus; nuclear magnetic resonance; nuclear magnetic resonance imaging; orthostatic hypotension; pancreatitis; parkinsonism; plasmapheresis; population research; pregnancy diabetes mellitus; priority journal; retrospective study; Shy Drager syndrome; stiff man syndrome; Taiwan; Taiwanese; tertiary care center; thyroid disease; Asian continental ancestry group; follow up; immunology; neurologic disease; Shy Drager syndrome; stiff man syndrome; Adult; Aged; Antibodies; Asian Continental Ancestry Group; Autoantibodies; Comorbidity; Female; Follow-Up Studies; Glutamate Decarboxylase; Humans; Male; Middle Aged; Multiple System Atrophy; Nervous System Diseases; Retrospective Studies; Stiff-Person Syndrome; Taiwan
Publisher
Blackwell Publishing Ltd
Type
journal article