Status Epilepticus: an Epilepsy Emergency and the Response to Immunotherapy

سال انتشار: 1398
نوع سند: مقاله کنفرانسی
زبان: انگلیسی
مشاهده: 284

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EPILEPSEMED16_075

تاریخ نمایه سازی: 28 بهمن 1398

چکیده مقاله:

Background: Epilepsy emergencies include acute repetitive seizures and status epilepticus. Their prognosis depends on the etiology of the seizures and the time spent in status epilepticus. Status epilepticus (SE) is a condition in which patients suddenly experience a prolonged seizure or a flurry of very frequent seizures. Status epilepticus that persists despite at least 2 standard anticonvulsant medications is termed refractory status epilepticus (RSE). New-onset refractory status epilepticus (NORSE) is defined as a condition, not a specific diagnosis, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause in a patient without active epilepsyMost of the common causes of RSE can be identified within 24-72 hours of presentation. In up to half of the cases of NORSE, a possible or probable cause is ultimately found, most often autoimmune or paraneoplastic encephalitis, with infectious causes less common. In the remaining half, no cause is identified despite an extensive work-up. Summary: Autoimmune epilepsy is an underrecognized condition, and its true incidence is unknown. Identifying patients with an underlying autoimmune origin is critical because these patients condition may remain refractory to conventional antiseizure medications but may respond to immunotherapy. EEG seems as a limited diagnostic tool in differentiating epilepsy and/or encephalopathy patients with a possible autoimmune etiology from those without. However, antineuronal Abs associated with encephalitis should be considered in the etiology of status epilepticus forms. A possible autoimmune etiology for seizures may be considered in the presence of continuous slow waves, FIRDA, and delta brush pattern in the EEG. In two-thirds of NORSE cases, the course of the syndrome begins with a mild febrile illness, associated with malaise, fatigue and symptoms of upper respiratory tract or gastro-intestinal tract infection. Symptoms of meningeal inflammation, such as headache and photophobia, are uncommon. Behavioral and cognitive symptoms, such as apathy or agitation, amnesia, and sometimes hallucinations can be observed. The presence of hallucinations may suggest an autoimmune etiology, especially anti-NMDA receptor encephalitis. This initial phase lasts a few days to a week or two and is followed by the progressive onset of seizures. Both focal seizures with impaired awareness (previously known as complex partial seizures, and typically described as staring episodes) and bilateral tonic-clonic seizures can occur. They are initially intermittent but become increasingly more frequent and the patient’s consciousness declines as he/she transitions into status epilepticus. The most common causes of NORSE and FIRES are autoimmune/paraneoplastic disorders, such as encephalitis associated with anti-neuronal antibodies (anti-NMDAreceptor, anti-voltage-gated potassium channel complex, etc.), followed by viral encephalitis. The treatment of SE initially consists of benzodiazepines (lorazepam, diazepam, or clonazepam), followed by a standard anticonvulsant medication, as in most cases of SE. Preference is given to drugs that are available in IV form (valproic acid, phenytoin, levetiracetam, phenobarbital and lacosamide). By definition, NORSE do not respond to these first two lines of treatment, and additional drugs are required. The two options are either to try additional anticonvulsant medications and/or to induce pharmacological coma with an anesthetic drug.Medications available in an IV formulation are often favored but others (e.g., topiramate, pregabalin) are sometimes used later as add-on therapy. Of the three, midazolam has the best safety profile but may be associated with a higher risk of recurrent seizures. Barbiturates are associated with more prolonged coma and need for mechanical ventilation, with a higher rate of complications. Propofol carries a small risk of propofol infusion related syndrome (PRIS), a potentially lethal syndrome of acidosis, kidney and heart failure. When an underlying cause is identified it should be appropriately treated.it is common to use approaches that modulate the immune system. These options include IV steroids, IV immunoglobulins, plasma exchange therapy (plasmapheresis) and some monoclonal antibodies against inflammatory cells (e.g., rituximab). The efficacy of these strategies is suggested by small case series, though never investigated in controlled trials. Conclusion: Status Epilepticus is an epilepsy emergency and neurologists try to treat it emergency to reduce the morbidity and mortality of this grave disease recently one of the refractory causes of status epilepticus is autoimmune encephalitis and Response to Immunotherapy has been reported satisfactory in some cases. But more investigation must be performed.

نویسندگان

Sheida Shaafi

Associate Professor of Neurology Department of neurology-Tabriz University of medical scienc