scholarly journals Case Report: Autoimmune Encephalitis Associated With Anti-glutamic Acid Decarboxylase Antibodies: A Pediatric Case Series

2021 ◽  
Vol 12 ◽  
Author(s):  
Changhong Ren ◽  
Haitao Ren ◽  
Xiaotun Ren ◽  
Weihua Zhang ◽  
Jiuwei Li ◽  
...  

Background: Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients, including stiff person syndrome, cerebellar ataxia, refractory epilepsy, and limbic and extralimbic encephalitis. There have been some case reports and investigations regarding anti-GAD65 antibody-associated encephalitis in adult populations, but pediatric cases are rare. We retrospectively analyzed the clinical data of three anti-GAD65 antibody-positive patients to explore the diversity and clinical features of anti-GAD65 antibody-associated pediatric autoimmune encephalitis.Methods: The clinical data of a series of three patients positive for anti-GAD65 antibody were retrospectively analyzed. GAD65 antibodies were determined in serum and CSF using a cell-based assay.Results: All three patients were female, and the onset ages were 4 years and 9 months, 6 years, and 16 years old. Their clinical phenotypes included autoimmune limbic encephalitis, extralimbic encephalitis, and encephalitis combining limbic and extralimbic encephalitis. The clinical symptoms included seizures, memory deficits, drowsiness, dysautonomia, and headache. All patients had abnormal carinal MRI and EEG. All patients received immunotherapy and had transiently good responsiveness, but one patient then experienced relapse. In follow-up, one patient with extralimbic encephalitis recovered completely, while two patients with limbic involvement had poor outcomes with refractory focal epilepsy.Conclusion: In addition to limbic encephalitis, extralimbic encephalitis is also an important phenotype in patients who are positive for anti-GAD65 antibodies. Early diagnosis and immunotherapy can improve the symptoms. However, patients with limbic encephalitis often have refractory epilepsy in the chronic phase and have a poor long-term outcome.

2020 ◽  
Vol 7 (3) ◽  
pp. e684 ◽  
Author(s):  
Megan E. Esch ◽  
Scott D. Newsome

ObjectiveTo describe 2 cases from a single academic institution of improvement in stiff-person syndrome (SPS) symptoms during pregnancy and to review the clinical outcomes of SPS in 6 additional pregnancies described in the literature.MethodsEvaluation of clinical symptoms and treatment changes of disease state during pregnancy.ResultsSeven patients with 9 pregnancies are described in women with a diagnosis of SPS. Six of 7 (86%) women were positive for glutamic acid decarboxylase (GAD65) antibody. In 5 of 9 (56%) pregnancies, symptomatic medications (antispasmodics) were significantly reduced with stabilization or improvement in symptoms through pregnancy. Nine live, healthy pregnancies resulted. All 7 (100%) women experienced worsening of symptoms after the birth of their children, and symptomatic therapies were resumed and/or increased.ConclusionsThe immune pathogenesis of SPS continues to be explored. Immunomodulatory shifts during pregnancy may influence changes of clinical SPS symptoms and provide insight into the unique pathogenesis of SPS. Some women with SPS may be able to reduce symptomatic medications related to clinical improvement during pregnancy. Women with SPS may safely carry pregnancies to term, delivering healthy and unaffected babies.


2014 ◽  
Vol 343 (1-2) ◽  
pp. 192-194 ◽  
Author(s):  
Ioannis Markakis ◽  
Harry Alexopoulos ◽  
Cornelia Poulopoulou ◽  
Sofia Akrivou ◽  
Athanasios Papathanasiou ◽  
...  

2020 ◽  
Vol 61 (3) ◽  
pp. 296-301
Author(s):  
Evan B. Fletcher ◽  
Miriam T. Fox ◽  
Oscar Joseph Bienvenu ◽  
Brent C. Pottenger

2011 ◽  
Vol 21 (3) ◽  
pp. 306-313 ◽  
Author(s):  
Souhel Najjar ◽  
Daniel Pearlman ◽  
Amanda Najjar ◽  
Vahid Ghiasian ◽  
David Zagzag ◽  
...  

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