HDAC Inhibitors as Epigenetic Regulators

As a routine practice, we use 5% albumin as replacement fluid in all TPE procedures except in TTP and in chronic liver disease patients having deficiency of coagulation factors. Conclusion With early suspicion based on clinical features and rapid treatment initialization, even in the absence of detectable autoantibodies (due to lack of diagnostic kits and facilities) TPE plays an important role in the management of AIE, as it can even remove some hitherto undetected autoantibodies. exchange Introduction Autoimmune encephalitis (AIE) is usually a group of encephalitis syndromes.[1] Patients generally have impaired memory and cognition over a period of days or weeks. There may be clues to specific causes on history of physical examination, but often these specific signs are absent. In about 50% of suspected autoimmune encephalitis based on clinical features, no antibodies are identified despite extensive evaluation.[2] The first line of therapy consists of steroids, therapeutic plasma exchange (TPE), and intravenous immunoglobulins (IVIG). TPE is usually a potential first-line therapy for various subtypes of AIE. Aniracetam Here, we present a case of autoantibody-negative suspected AIE, managed successfully with TPE after steroid failed to improve the conditions. Case Report A 39-year-old diabetic male presented with altered behavior, frequent twitching of the left angle of the mouth along with clonic/dystonic movement of the left hand. He had a history of low-grade fever for 1 day followed by headache and intermittent slurring of speech along with deviation of angle of mouth on the left side. There was no present history of nausea, vomiting, rash, features suggestive of vasculitis, and no previous history of comparable events. These complex partial seizures rapidly progressed to status epilepticus in few hours. As patient’s general condition was poor, he was intubated and put on ventilator. His cerebrospinal fluid (CSF) picture showed neutrophilic pleocytosis, increased protein with normal sugar. The treatment was started around the lines of autoimmune and viral encephalitis. CSF HSV type I and II reports were unfavorable on polymerase chain reaction. Although, antivoltage-gated potassium channels (VGKC) antibody were also unfavorable, the left focal faciobrachial seizure along with dystonic posturing of the left upper limb was more in favor of AIE. Magnetic resonance imaging (MRI) obtaining on the 1st day Aniracetam revealed few altered area of signal intensity in bilateral cerebral hemispheres showing patchy enhancement along with enhancing leptomeninges. Patient was started with IV fluids, steroids, antibiotics, and antiviral and antiepileptic medicines. MRI on the 3rd day showed gyral swelling in bilateral cerebral hemisphere with restricted diffusion of the right fronto-temporo-parietal region. When compared to the last MRI, it also showed a significant increase in site and extent of involved areas. As there was no improvement with ongoing medications, the decision to start TPE was taken. We did a total of five sessions of TPE (initially one exchange every day for 3 days followed by 2 exchanges alternate days). All the TPE procedures were done with COM.TEC (Fresenius Kabi, Germany). One standard TPE session was 1.5 plasma volume exchanges using 5% albumin as a replacement fluid. Prophylactic administration of calcium gluconate (one ampoule diluted in 100 ml of 0.9% normal saline) was done for every 1000 ml of plasma exchanged. Informed consent was obtained from family members. All the procedures were done on double-lumen femoral dialysis catheter IMMT antibody under aseptic precautions. Compliance to TPE was excellent as all the sessions were Aniracetam uneventful. After five sessions, patient’s clinical condition improved significantly, and a repeated MRI was done after 5th cycle of TPE which revealed reduction in Aniracetam the areas of signal alteration in comparison to the previous MRI. This was suggestive of regression of disease [Table 1 and Physique 1]. The patient was discharged around the 10th day of hospital admission. Table 1 Relevant findings on cerebrospinal fluid Open in a separate window Open in a separate window Physique 1 Contrast magnetic resonance imaging pre- and post-therapeutic plasma exchange Discussion AIE is considered to be a rare disease. In a multicentric prospective study in the UK, it was shown that in one-third of the patients Aniracetam diagnosis of AIE was not conclusive and almost 50% of the patients were found unfavorable for the common autoimmune antibodies suspected.[3,4] California encephalitis project also demonstrated the same finding. In this cohort of patients (a cohort of 1500 adults as well as children), confirmed suspected etiologies were not found in almost two-third of the study populations.[5] The imaging and laboratory findings of many forms of autoimmune and infectious encephalitis are similar to AIE making it difficult to diagnose clinically.[6] For the diagnosis of definite autoimmune limbic encephalitis, all four of the following criteria have to be met: subacute onset, bilateral brain abnormalities on T2-weighted fluid-attenuated inversion recovery MRI highly restricted to the medial temporal lobes, CSF pleocytosis or electroencephalogram with epileptic or slow-wave activity around the temporal lobe, and reasonable exclusion of alternative causes.[1] Detection of autoantibodies is a definite diagnostic tool; however, there are case reports of patients presenting with features of AIE without any.