By contrast, clinical improvement in cases of anti-NMDAR encephalitis is not always achieved with TPE (22,23)

By contrast, clinical improvement in cases of anti-NMDAR encephalitis is not always achieved with TPE (22,23). present case report aims to raise awareness about the importance of MBQ-167 early implementation of this potentially life-saving therapy and continuing the treatment courses until full subsidence of symptoms. Keywords: anti-NMDA receptor antibodies, autoimmune encephalitis, MBQ-167 therapeutic plasma exchange, status epilepticus, EEG abnormalities Introduction Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is an autoimmune disorder with a wide spectrum of neuropsychiatric symptoms, which was first described in 2005 by Vitalini (1). It has recently been demonstrated that this potentially fatal disease is significantly underdiagnosed and may contribute to the majority of cases of non-infectious encephalitis of previously unknown aetiology Rabbit Polyclonal to UBAP2L (2). Vitalini (1) retrospectively analysed >500 cases of intensive care unit (ICU) admission and identified 7 patients with encephalitic signs (psychiatric and cognitive disturbances, as well as focal or generalized seizures progressing into status epilepticus), cerebrospinal fluid (CSF) inflammation and exclusion of bacterial or viral infection, and in 6 cases the presence of anti-NMDAR antibodies was confirmed. A positive serum or CSF sample screening for antibodies to the NMDAR subunit remains the gold-standard in diagnosing the disease and must be performed in all patients presenting with an acute onset of psychiatric symptoms with atypical features or unusual movements. MBQ-167 However, emerging data exists to suggest that electroencephalograms (EEG) can also be very useful in aiding clinicians to diagnose anti-NMDAR encephalitis (3). Over the past years, increasing awareness of autoimmune mediated forms of encephalitis with antibodies against neuronal surface antigens have led to an improvement in prognosis, predominantly due to wider application of immunomodulatory therapies. Notably, despite major progress in understanding the pathophysiology of anti-NMDAR encephalitis, there remains a clear requirement for a good quality data regarding the optimal treatment of the disease, predominantly since the type of immunotherapy that is most effective in controlling the symptoms of the disease remains a matter of debate. Numerous patients, particularly with a prolonged or severe form of the disease, do not respond to first-line immunotherapy [steroids or intravenous immunoglobulins (IVIg)] and may require therapeutic plasma exchange (TPE), which is commonly used to treat a number of neurological conditions, including Guillain-Barr syndrome, myasthenia gravis, chronic inflammatory demyelinating polyneuropathy, Lambert-Eaton syndrome, multiple sclerosis, MBQ-167 neuromyelitis optica, paraproteinemic polyneuropathy, Sydenham’s chorea and natalizumab-associated progressive multifocal leukoencephalopathy (4). Despite the increasing number of potential indications for TPE in the treatment of neurological disorders, the proven efficacy, side effects and costs must be taken into consideration prior to a decision being made to implement this therapy. Case report A 23-year-old female was admitted to a psychiatric ward, presenting with acute confusion, agitation and behavioural changes. The initial diagnosis was the first episode of schizophrenia or schizoaffective disorder. Her past medical history was non-significant and no prior psychiatric or psychological problems were reported. An episode of an upper respiratory tract infection, which preceded the psychotic symptoms by few days was notable. Upon admission, agitation and restlessness were observed, followed by progressive mutism and somnolence, which were the predominant symptoms of the disease within 5 days. The patient was administered standard antipsychotic treatment during her entire stay in the psychiatric ward, which included haloperidol, olanzapine and aposulpiryd, without any improvement in her psychiatric condition. On day 5, the patient’s neurological condition significantly deteriorated; decreased level of consciousness and loss of muscle tone were observed. Involuntary movements of upper limbs, jaw and eyes were also noticed, as well as clonic seizures, which were treated with intravenous diazepam. The initial diagnosis was changed to infectious encephalitis and the patient was transferred to a neurological ward at the state hospital, where CSF and blood samples were obtained and imaging studies of the brain, as well as EEG were performed (Fig. 1). Open in a separate window Figure 1. Encephalogram demonstrates generalized rhythmic delta activity.