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      An atypical case of febrile infection-related epilepsy syndrome following acute encephalitis: impact of physiotherapy in regaining locomotor abilities in a patient with neuroregression

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          Abstract

          Encephalitis refers to inflammation of the brain parenchyma. It is potentially life-threatening with the highest incidence and severity in younger children. Febrile infection-related epilepsy syndrome (FIRES) is a condition, in which a child develops a nonspecific febrile illness that may not persist when the initial seizure activity begins. However, an electroencephalogram (EEG) shows that the child is in status epilepticus. We report the case of a five-year-old male who presented with difficulty to maintain sitting posture, and inability to stand and walk without support, following viral encephalitis at the age of one year. He had motor, visual, speech and cognitive impairment along with a seizure disorder. The physiotherapy interventions including neurodevelopmental treatment (NDT) and sensory integration (SI) helped in regaining locomotion ability in the child. The study aims to assess the impact of physiotherapy interventions on regaining locomotor ability in a child with FIRES following infective encephalitis.

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          New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

          New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are relatively rare clinical presentations. They are characterized by de novo onset of refractory status epilepticus (RSE) without clearly identifiable acute or active cause (structural, toxic, or metabolic). We reviewed the literature using PubMed reports published between 2003 and 2019 and summarized the clinical, neurophysiological, imaging, and treatment findings. Focal motor seizures, which tend to evolve into status epilepticus, characterize the typical presentation. Disease course is biphasic: acute phase followed by chronic phase with refractory epilepsy and neurological impairment. Aetiology is unknown, but immune-inflammatory-mediated epileptic encephalopathy is suspected. Electroencephalograms show variety in discharges (sporadic or periodic, focal, generalized, or more frequently bilateral), sometimes with a multifocal pattern. About 70% of adult NORSE have abnormal magnetic resonance imaging (MRI); in paediatric series of FIRES, 61.2% of patients have a normal brain MRI at the beginning and only 18.5% during the chronic phase. No specific therapy for FIRES and NORSE currently exists; high doses of barbiturates and ketogenic diet can be used with some effectiveness. Recently, anakinra and tocilizumab, targeting interleukin pathways, have emerged as potential specific therapies. Mortality rate is around 12% in children and even higher in adults (16-27%).
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            Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents

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              Understanding and managing acute encephalitis

              Encephalitis is an important cause of morbidity, mortality, and permanent neurologic sequelae  globally. Causes are diverse and include viral and non-viral infections of the brain as well as autoimmune processes. In the West, the autoimmune encephalitides are now more common than any single infectious cause, but, in Asia, infectious causes are still more common. In 2006, the World Health Organization coined the term “acute encephalitis syndrome”, which simply means acute onset of fever with convulsions or altered consciousness or both. In 2013, the International Encephalitis Consortium set criteria for diagnosis of encephalitis on basis of clinical and laboratory features. The most important infectious cause in the West is herpes simplex virus, but globally Japanese encephalitis (JE) remains the single largest cause. Etiologic diagnosis is difficult because of the large number of agents that can cause encephalitis. Also, the responsible virus may be detectable only in the brain and is either absent or transiently found in blood or cerebrospinal fluid (CSF). Virological diagnosis is complex, expensive, and time-consuming. Different centres could make their own algorithms for investigation in accordance with the local etiologic scenarios. Magnetic resonance imaging (MRI) and electroencephalography are specific for few agents. Clinically, severity may vary widely. A severe case may manifest with fever, convulsions, coma, neurologic deficits, and death. Autoimmune encephalitis (AIE)  includes two major categories: (i) classic paraneoplastic limbic encephalitis (LE) with autoantibodies against intracellular neuronal antigens (Eg: Hu and Ma2) and (ii) new-type AIE with autoantibodies to neuronal surface or synaptic antigens (Eg: anti-N-methyl-D-aspartate receptor). AIE has prominent psychiatric manifestations: psychosis, aggression, mutism, memory loss, euphoria, or fear. Seizures, cognitive decline, coma, and abnormal movements are common. Symptoms may fluctuate rapidly. Treatment is largely supportive. Specific treatment is available for herpesvirus group and non-viral infections. Various forms of immunotherapy are used for AIE.
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                Author and article information

                Journal
                Pan Afr Med J
                Pan Afr Med J
                PAMJ
                The Pan African Medical Journal
                The African Field Epidemiology Network
                1937-8688
                17 June 2020
                2020
                : 36
                : 101
                Affiliations
                [1 ]Government Physiotherapy College, Raipur, India,
                [2 ]Ravi Nair Physiotherapy College, Datta Meghe Institute of Medical Sciences, Wardha, India
                Author notes
                [& ]Corresponding author: Waqar M. Naqvi, Ravi Nair Physiotherapy College, Datta Meghe Institute of Medical Sciences, Wardha, India

                Domain: Neurology (general), Pediatric neurology

                Article
                PAMJ-36-101
                10.11604/pamj.2020.36.101.23855
                7392866
                32774660
                687d9e05-21c8-4a4a-805f-0817e5adf42d
                © Chanan Goyal et al.

                The Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 30 May 2020
                : 09 June 2020
                Categories
                Case Report

                Medicine
                post encephalitis sequelae,neurodevelopmental treatment,sensory integration
                Medicine
                post encephalitis sequelae, neurodevelopmental treatment, sensory integration

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