Yale University
Neurology

Author Of 2 Presentations

Observational Studies Poster Presentation

P0891 - Neurological manifestations of GAD-65 antibodies (ID 1922)

Speakers
Presentation Number
P0891
Presentation Topic
Observational Studies

Abstract

Background

Glutamic Acid Decarboxylase (GAD65) antibodies may be asymptomatic or associated with a variety of neurological manifestations. It is frequently associated with Type 1 diabetes.

Objectives

The main objective is to determine the clinical characteristics and treatments used for patients with GAD-65 antibodies.

Methods

This was an observational, retrospective, single-center study enrolling all patients found to be positive for GAD65 antibodies between January 2010 and December 2019. To select for cases thought to have neurologic manifestations of GAD65 autoantibodies, we further selected for subjects who were evaluated and assessed by the neurology service.

Results

1015 patients tested positive for GAD65 autoantibodies, but only 123 (12.1%) patients were evaluated by the neurology service. Among the subset evaluated by Neurology, the mean age was 47.1 (±20.8) and 67 (54.5%) were female. Diabetes mellitus (DM) was seen in 60 (48.8%) of the cases. The most common neurological manifestation was as follows: seizure 32 (26%), stiff-person syndrome 30 (24.4%), encephalitis 17 (13.8%), ataxia 16 (13%) and nystagmus 6 (4.9%). Thirty nine (31.7%) patients were diagnosed with peripheral neuropathy, which was likely secondary to DM in most cases. Seizure and stiffness were observed more frequently in females, although this was not a statistically significant difference.

Abnormal MRI brain was seen in 60 (48.8%) of the cases. Patient were treated with: intravenous steroid 45 (36.6%), oral steroid 59 (48%), intravenous immunoglobulin 58 (47.2%), plasmapheresis 14 (11.4%), rituximab 25 (20.3%), azathioprine 15 (12.2%), mycophenolate mofetil 12 (9.8%) and cyclophosphamide 6 (4.9%).

Conclusions

At least one tenth of GAD-65 antibody positive cases needed a neurological evaluation. The most common clinical presentations in our cohort were seizures and stiff-person syndrome.

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Observational Studies Poster Presentation

P0903 - Real world patterns and outcomes of seropositive autoimmune encephalitis (ID 1931)

Speakers
Presentation Number
P0903
Presentation Topic
Observational Studies

Abstract

Background

Seropositive autoimmune encephalitis is a heterogeneous and rapidly expanding diagnostic category with variable presentations and treatment responsivity.

Objectives

To determine the distribution, clinical characteristics, responses to treatment and clinical outcomes for real-world cases of confirmed seropositive autoimmune encephalitis.

Methods

Patients within a single, tertiary medical center identified to have positive blood or CSF autoantibodies on panels sent to Mayo Laboratories between January 2010 and December 2019 were identified and retrospective chart reviews were performed. Patient with low antibody titers and clinical features not suggestive of an autoimmune syndrome were excluded.

Results

1858 patients were tested for autoimmune encephalitis antibody panel. Two hundred nineteen (11.79%) had positive autoantibodies but only 42 cases fulfilled the inclusion criteria. The mean age of the patients was 53.4 (± 21.4) with 71.4% female predominance. The majority of the patients presented with altered mental status (n=15, 35.7%) or new onset seizure (n=13, 30.9%).The most common autoantibodies detected were anti NMDA (n=12, 28.6%) followed by anti LGI (n=11, 26.2%) and anti-neuronal nuclear antibody (ANNA, or anti-Hu, n=9, 21.4%). The most common treatments used were steroids (n=37, 88.1%) and IVIg (n=31, 73.8%). Rituximab was the most common second line treatment (n=20, 47.6%). The worst clinical outcomes were seen with ANNA-associated syndromes; these patients had a mean modified Rankin score of 5.8(± 0.3). Six of these patients had a small cell lung cancer. Patients with NMDA and LGI autoantibodies had a better response to treatment with mean mRS of 1.8 (± 1.03) and 1.5 (± 1.36) respectively.

Conclusions

Anti-NMDA and LGI were the most commonly detected autoantibodies in our cohort and also had the best clinical outcomes. Patient with ANNA had the worst clinical outcomes and small cell lung cancer was the most common associated malignancy.

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