Clinical Characterization of Definite Autoimmune Limbic Encephalitis: A 30-case Series

Autor: Takayuki Jo, Yuri Shojima, Masao Watanabe, Kazumasa Yokoyama, Kazuyuki Noda, Yasuyuki Okuma, Kenya Nishioka, Takao Urabe, Nobutaka Hattori, Hiroshi Takashima, Keiko Tanaka
Rok vydání: 2019
Předmět:
Male
Paraneoplastic Syndromes
medicine.medical_treatment
030204 cardiovascular system & hematology
Early initiation
Gastroenterology
0302 clinical medicine
Limbic system
Japan
Modified Rankin Scale
Age of Onset
biology
Limbic encephalitis
VGKC
General Medicine
Middle Aged
autoimmune limbic encephalitis
Magnetic Resonance Imaging
Temporal Lobe
NMDAR
Treatment Outcome
medicine.anatomical_structure
Potassium Channels
Voltage-Gated
Child
Preschool
Original Article
Female
030211 gastroenterology & hepatology
Immunotherapy
Antibody
Adult
medicine.medical_specialty
Receptors
N-Methyl-D-Aspartate
Autoimmune Diseases
Young Adult
03 medical and health sciences
Limbic Encephalitis
Internal medicine
Internal Medicine
medicine
Humans
Cognitive Dysfunction
Autoantibodies
Retrospective Studies
business.industry
Autoimmune limbic encephalitis
medicine.disease
biology.protein
Atrophy
business
Complication
Zdroj: Internal Medicine
ISSN: 1349-7235
0918-2918
DOI: 10.2169/internalmedicine.3029-19
Popis: Objective Limbic encephalitis (LE) is an inflammatory condition of the limbic system that has an acute or subacute onset. Several types of antibodies are related to the onset of LE, including anti-N-methyl D-aspartate receptor (NMDAR) antibodies and voltage-gated potassium channel (VGKC)-complex antibodies. However, the characteristics and prevalence of LE remain unclear, especially in Asian cohorts, due to the rarity. We aimed to survey their characteristics. Materials and Methods Data of 30 cases clinically defined as "definite autoimmune LE" (based on the standard criteria) were retrospectively collected. These patients were categorized into four subtypes: NMDAR (+) (n=8), VGKC (+) (n=2), antibodies related to paraneoplastic syndrome (n=2), and an antibody-negative group (uncategorized) (n=18). Results LE is rare in Japan, and affected only 30 of 16,759 hospital patients (0.2%) over a ten-year period. The NMDAR (+) group showed distinctive symptoms, while the other three groups had similar indications. Brain MRI indicated significant medial temporal lobe atrophy at one year follow up after discharge. The prevalence of cognitive dysfunction as a complication was 64% (9/14). First-line immunotherapy resulted in a good outcome. A drastic improvement was seen from 4.0±1.1 to 1.1+ on the modified Rankin Scale. A good treatment outcome was observed in all groups (NMDAR, VGKC, and uncategorized), suggesting the importance of an early clinical diagnosis and the early initiation of treatment. Furthermore, we reviewed 26 cases that were clinically diagnosed as definitive autoimmune LE in previous case reports. Conclusion Our findings show that the establishment of a clinical diagnosis based on the clinical criteria of definitive autoimmune LE is important for the initiation of immunotherapy.
Databáze: OpenAIRE
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