Abstract Details

Title Endophenotypes of Non-Paraneoplastic Limbic Encephalitis in Childhood and Possible Underlying Immune Deficiencies/Dysregulations: A Case Series and Literature Review

Topic Autoimmune Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 15-011

Objective Our aim is to characterize features of non-paraneoplastic LE in children and correlate them with underlying immunodeficiencies.

Background Limbic encephalitis (LE) is a rare disease in children but with a well-recognizable signature. Beyond the association with anti-neuronal antibodies (Ab), no dysimmune phenotype was associated with LE. Natural killer (NK) cells have disease-limiting role in neuroinflammation.

Design/Methods Retrospective case series of eight patients with LE. We performed literature review from 2000-2017. Inclusion criteria included age < 18 years, symptoms of LE, absence of tumor, with either MRI, pathology or LE antibodies marker(s).

Results The age range at first symptom was between 1-16 years. The clinical presentation was either acute (n=5) or insidious (n=3) encephalopathy. Symptoms included temporal epilepsy (n=6), ADHD or autistic symptoms (n=2), and memory changes (n=3). Patients had low NK cells (n=3), both VGKC and TPO Abs (n=1), TPO Ab (n=2), low lymphocytes, low B cells, and low IgA, IgG, IgM levels (n=1), low lymphocyte count (n=1), and low IgA (n=1). We had a spectrum of outcomes, from responding to anti-epileptics to more aggressive immunotherapy. Five patients improved or underwent remission after first intervention and three others are still progressing. From literature review, we included 85. Among these 10% had a progressive course or death 2%. Neurological sequelae were seen in 89%. Immune dysregulation was reported among four patients of which, two had a progressive course.

Conclusions Even if most patient with non-paraneoplastic LE has a non-progressive course, most of them suffer a neurological sequalae. Among patients who underwent immunological workup half of them seem to have an immune dysregulation. Our findings although limited in number suggest a full immunological workup for any patient with suspected LE.

Authors/Disclosures
Abdulla Alawadhi, MBBS (Dubai health / AJCH) PRESENTER	Dr. Alawadhi has nothing to disclose.
	No disclosure on file
	No disclosure on file
Guillaume Sebire (McGill University)	No disclosure on file

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Abstract Details