Naslov
The Effect Of Delayed Anti-NMDR Encephalitis Recognition On Disease Outcome

Autori
Šulentić, Vlatko ; Bujan Kovač, Andreja ; Đerke, Filip ; Santini, Marija ; Bažadona, Danira ; Nankovic, Sibila ; Ozretić, David ; Jovanović, Ivan ; Petelin Gadže, Željka

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, stručni

Izvornik
Epilepsia / - , 2019, 212-213

Skup
33rd International Epilepsy Congress

Mjesto i datum
Bangkok, Tajland, 22.06.2019. - 26.06.2019

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Anti-NMDAR Encephalitis, treatment

Sažetak
Purpose: Anti- N- Methyl- D- aspartate receptor (NMDAR) is an acute form of brain inflammation characterized by the presence of specific IgG antibodies against heteromers of NR1 and NR2 subunits of cell-surface NMDA receptors. Although it can be a pure autoimmune disorder, anti-NMDAR encephalitis is frequently associated with an underlying malignancy, commonly occult ovarian teratoma. In the majority of cases is evident as encephalitis presenting exclusively with psychiatric symptoms with usually good outcome and high probability for recovery after treatment. Thus, in some cases can be potentially lethal, especially when presenting with atypical and unpredictable symptoms leading to a delay in the diagnosis establishment. Method: We present three cases of patients with epilepsy and anti-NMDAR encephalitis diagnosed one or more years after the initial presentation with psychiatric symptoms. Results: Symptomatology, clinical semiology of the epileptic seizures, disease progression, MRI findings, and frequency and time of relapses were different in all three cases. Thus, after diagnosing anti-NMDAR encephalitis and inadequate response to corticosteroid therapy as a first line of treatment in relapses, all patients received successful immunosuppression treatment with immunoglobulins, cyclophosphamide, or rituximab. Conclusion: Although the disease symptoms of anti- NMDAR encephalitis as an multiphasic disorder are relatively well-known, sometimes they can be unpredictable and atypical, leading to a delay in diagnosis. Unawareness of psychiatric symptoms, as possible initial presentation, and lack of the characteristic ones that would imply the need for specific antibody testing, are major problems which usually lead to prolonged and more demanding treatment with increased tendency of progression of consciousness impairment, autonomic dysfunction, respiratory insufficiency, and need for intensive care unit treatment. Thus, the timely diagnosis establishment is of the highest priority, considering that early treatment could lead to better disease outcome, less cognitive impairment, unpredictable relapses and pharmacoresistant epilepsy.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

POVEZANOST RADA