ONE CASE OF REFRACTORY STATUS EPILEPTICUS AND AUTOIMMUNE ENCEPHALITIS

Guliko Kiliptari 1 and Ia Rukhadze 2 . 1. Head of Department of critical care., university central clinic after acad. N. Kipshidze ,MD, PhD. Prof.of TSMU (Tbilisi, Georgia). 2. Neurologist, Md, PHD (Tbilisi, Georgia). ...................................................................................................................... Manuscript Info Abstract ......................... ........................................................................ Manuscript History


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Several particular features raise the suspicion of an autoimmune cause in otherwise unexplained seizure disorders.
One of the most important challenges is the elucidation of the causes of seizure disorders. IgG autoantibodies against proteins on the surfaces of neurons were identified as markers and pathogens in autoimmune encephalitides that are accompanied by repetitive focal seizures. the antibodies against surface antigens contribute directly to the disease processes.In particular Anti-NMDA receptor encephalitis is a disease occurring when antibodies produced by the body's own immune system attack NMDA receptors in the brain .In the absence of a reliable biomarker, it is difficult to gain systematic and general insights into these patients.
Because autoantibody test results and response to therapy are not available at disease onset, treatment is based the initial diagnostic approach on neurological assessment and conventional tests that are accessible to most clinicians.
We presented one case of unexplained coma and refractory status epile-pticus .
Case report:-Patient, 20 yars old.men,Caucasian was admetted in hospital at 06.07.2017. with coma , stable hemodinamics, on mechanical ventilation (patient was transferred from another clinic) CT of brain could not discovered any pathological changing.MRI within admittion (07.07.2017) was without acute pathological intracerebral changing, EEG-discovered polymorphic dysrhythmia,bilateral activity( Fig.1). Treatment was started empirical , with antiviral(Acyclovir ) and antibacterial drugs . After 6 day from the beginning of investigation ,autoantibody test result was positive NMDAR AAB in CSF was 1:2 ,in serum 1:160.(serem and CSF was investigated in Hidelberg laboratory of Limbach) After 4 day EEG revealed status epilepticus (    b) In this laboratory screening for surface receptor antibodies (avidin-biotin-peroxidase technique; rat brain; IIFT Euroimmun) was negative for antibodies such as NMDAR, LGI1, CASPR2, AMPAR, GABA(B)R, and DPPX.A cell-based assay was negative for glycin-receptor antibodies (in-house; GlyR alpha1).
Institute has noticed that negative test result does not exclude autoimmune logically mediated syndromes of other origin.

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Later , after 36 day from admittion patient state has became stable, patient was weaned from artifitial ventilation . The level of consciousness has been gradually improved and finally patient has discharged from hospital .

Discussing:-
We presented drug-resistant status epilepticus that persisted following the continuous administration of intravenous anesthetics and anticonvulsive drugs for more than several days Acute onset, resistance to anti-seizure medication, evidence of CNSinflammation was clinical features suggestive of autoimmune epilepsy.
Many researchers have reported that, during prolonged seizures, the number of activated GABA-A receptors on the postsynaptic membrane gradually decreases, whereas the number of inactive GABA-A receptors increases .These changes cause a significant reduction in the efficacy of antiepileptic drugs (AEDs). Recent studies have found that, during prolonged seizures, when the numbers and activities of GABA receptors gradually decrease,simultaneously,the numbers and activities of glutamatergic NMDA receptors increase, Thus, the commonly used first-line and second-line AEDs gradually fail, often causing RSE and thus providing the possibility of the use of ketamine to treat RSE.Ketamine is a noncompetitive NMDA receptor antagonist that might play a role in treating SE by blocking NMDA receptor-mediated glutamatergic transmission .But After ketamin infusion its anticonvulsant effects has not been reached. For maximal captured and decreasion of brain bioactivity was used thiopental with high dose (400mg/h)infusion . We achieved absolute decreasion of brain bioactivity and full resolvation of convulsion.

Conclusion:-
An autoimmune cause was suspected based on frequent or medically intractable seizures and the presence of neural antibody, inflammatory changes indicated in spinal fluid and on MRI. Patients had abnormal findings on brain MRI, with extensive multifocal or diffuse cortical and subcortical involvement. EEG showed generalized periodic discharges and status epilepticus . Within investigation of IgG autoantibodies against proteins on the surfaces of neurons, we received difference result from two different laboratory,but refractory status epilepticus (Resistance to anti-seizure medication),radiological changes ,EEG pattern and progress of illness has indicated autoimmune character of process.