Contributions
Abstract: P1794
Type: Poster Sessions
Abstract Category: N/A
Background: The autoimmune encephalitides (AE) are a group of neuro-inflammatory disorders resulting from dysregulation of the immune system. This produces a heterogeneous group of neurological symptoms including drug refractory seizures, neuropsychiatric disturbance, movement disorders and cognitive dysfunction. These disorders are divided into subtypes based on a combination of serological testing (antibodies to synaptic or intracellular proteins) and clinical phenotypes. The cohorts from which these clinical characteristics have been identified are largely from the Northern hemisphere and may not reflect geographical variations.
Method: Medical records from 2008-2018 in 4 tertiary hospitals in Australia were searched systematically for patients with a diagnosis of AE. These were matched with serological laboratory results to define patients as seropositive (and subsequent serotype) or seronegative. Clinical, laboratory, electrophysiological and imaging information was then collected in a retrospective manner.
Results: 132 patients with AE identified. 56 (42.5%) had known antibodies identified: 20 NMDAR, 11 VGKC, 10 LGI-1, 4 Anti-Hu, 3 AMPAR, 2 GAD65, 2 SOX-1, and single patients with Anti-Ma2, GABABR, CASPR2 and 1 related to treatment with a PD-1 inhibitor.76 patients (57.5%) were seronegative. Seropositive patients were more likely to have seizures (71% compared with 50%). 75% seropositive and 67% seronegative patients received corticosteroids as first-line therapy while 32% of seropositive patients received second-line treatment (Rituximab or Cyclophosphamide) compared with 10% of seronegative patients. Of the seropositive patients, 64% had a 'good' functional outcome (defined as mRS 0-2) and 28% had a 'poor' outcome (mRS 3-6), while in the seronegative cohort 55% had a 'good' outcome and 38% had a 'poor' outcome. 10% of patients in both cohorts experienced a relapsing disease requiring further immunotherapy.
Conclusion: There is minimal difference in this cohort of AE patients compared with previously published cohorts from the Northern hemisphere.
Disclosure: Dr Robb Wesselingh: Dr Wesselingh has received personal compensation for speaking or other activities with Roche and Biogen.
Dr James Broadley: Nothing to disclose
Dr Chris Kyndt: Nothing to disclose
Dr Katherine Buzzard: Nothing to disclose
Professor Terence O´Brien: Prof O´Brien has received personal compensation for activities, compensation for consulting, serving on a scientific advisory board, speaking, or other activities with SciGen, Eisai, and UCB Pharma. Dr. O´Brien´s institution has received funding for research from Eisai, Janssen-Cilag, Sanofi-Genzyme, SciGen, Zynerba Pharmaceuticals, and UCB Pharma.
Dr Mastura Monif: Nothing to disclose
Abstract: P1794
Type: Poster Sessions
Abstract Category: N/A
Background: The autoimmune encephalitides (AE) are a group of neuro-inflammatory disorders resulting from dysregulation of the immune system. This produces a heterogeneous group of neurological symptoms including drug refractory seizures, neuropsychiatric disturbance, movement disorders and cognitive dysfunction. These disorders are divided into subtypes based on a combination of serological testing (antibodies to synaptic or intracellular proteins) and clinical phenotypes. The cohorts from which these clinical characteristics have been identified are largely from the Northern hemisphere and may not reflect geographical variations.
Method: Medical records from 2008-2018 in 4 tertiary hospitals in Australia were searched systematically for patients with a diagnosis of AE. These were matched with serological laboratory results to define patients as seropositive (and subsequent serotype) or seronegative. Clinical, laboratory, electrophysiological and imaging information was then collected in a retrospective manner.
Results: 132 patients with AE identified. 56 (42.5%) had known antibodies identified: 20 NMDAR, 11 VGKC, 10 LGI-1, 4 Anti-Hu, 3 AMPAR, 2 GAD65, 2 SOX-1, and single patients with Anti-Ma2, GABABR, CASPR2 and 1 related to treatment with a PD-1 inhibitor.76 patients (57.5%) were seronegative. Seropositive patients were more likely to have seizures (71% compared with 50%). 75% seropositive and 67% seronegative patients received corticosteroids as first-line therapy while 32% of seropositive patients received second-line treatment (Rituximab or Cyclophosphamide) compared with 10% of seronegative patients. Of the seropositive patients, 64% had a 'good' functional outcome (defined as mRS 0-2) and 28% had a 'poor' outcome (mRS 3-6), while in the seronegative cohort 55% had a 'good' outcome and 38% had a 'poor' outcome. 10% of patients in both cohorts experienced a relapsing disease requiring further immunotherapy.
Conclusion: There is minimal difference in this cohort of AE patients compared with previously published cohorts from the Northern hemisphere.
Disclosure: Dr Robb Wesselingh: Dr Wesselingh has received personal compensation for speaking or other activities with Roche and Biogen.
Dr James Broadley: Nothing to disclose
Dr Chris Kyndt: Nothing to disclose
Dr Katherine Buzzard: Nothing to disclose
Professor Terence O´Brien: Prof O´Brien has received personal compensation for activities, compensation for consulting, serving on a scientific advisory board, speaking, or other activities with SciGen, Eisai, and UCB Pharma. Dr. O´Brien´s institution has received funding for research from Eisai, Janssen-Cilag, Sanofi-Genzyme, SciGen, Zynerba Pharmaceuticals, and UCB Pharma.
Dr Mastura Monif: Nothing to disclose