Febrile infection-related epilepsy syndrome (FIRES) is an epilepsy syndrome in which new-onset refractory status epilepticus (NORSE) is preceded by febrile illness 24 hours to 2 weeks prior to the onset of seizures. The term was previously used for a paediatric syndrome but was redefined to include all ages.
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| - متلازمة الصرع المرتبطة بالحمى الناتجة عن الخمج (ar)
- Febrile infection-related epilepsy syndrome (en)
- Syndrome FIRES (fr)
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| - Le Syndrome de l'épilepsie fébrile liée à l'infection (FIRES) est une forme d'épilepsie qui touche les enfants de trois à quinze ans. Un enfant en bonne santé qui peut avoir été malade au cours des derniers jours, ou avec une belle persistance de la fièvre va dans un état de permanence des crises. Les crises sont résistantes aux médicaments et des traitements, bien que les barbituriques peuvent être administrés. Médicaux tests de diagnostic peut, au départ, de retour pas de diagnostic clair et ne peut pas détecter de toute évidence une tuméfaction sur le cerveau. Le syndrome est très rare: il ne peut affecter qu'un enfant sur 1 000 000. (fr)
- متلازمة الصرع المرتبطة بالحمى الناتجة عن خمج هي متلازمة صرعية حيث يسبق الصرع حديث الظهور بمرض حموي قبل 24 ساعة إلى أسبوعين من بداية النوبات. تم استخدام المصطلح سابقًا للإشارة إلى متلازمة الأطفال ولكن تم إعادة تعريفه ليشمل جميع الأعمار. (ar)
- Febrile infection-related epilepsy syndrome (FIRES) is an epilepsy syndrome in which new-onset refractory status epilepticus (NORSE) is preceded by febrile illness 24 hours to 2 weeks prior to the onset of seizures. The term was previously used for a paediatric syndrome but was redefined to include all ages. (en)
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| - Febrile infection-related epilepsy syndrome (en)
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| - Febrile infection-related epilepsy syndrome (en)
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| symptoms
| - Prior febrile illness with or without upper respiratory tract infection and/or gastroenteritis. Convulsive seizures within two weeks of fever. (en)
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| - Unknown, preceded by unspecified febrile illness (en)
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| - Acute phase lasts 1-12 weeks. Chronic phase is lifelong pharmacoresistent epilepsy and intellectual disability. (en)
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| synonyms
| - Acute encephalitis with refractory, repetitive partial seizures , new-onset refractory status epilepticus , devastating epilepsy in school-aged children . (en)
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| - متلازمة الصرع المرتبطة بالحمى الناتجة عن خمج هي متلازمة صرعية حيث يسبق الصرع حديث الظهور بمرض حموي قبل 24 ساعة إلى أسبوعين من بداية النوبات. تم استخدام المصطلح سابقًا للإشارة إلى متلازمة الأطفال ولكن تم إعادة تعريفه ليشمل جميع الأعمار. كانت المتلازمة تشير سابقًا إلى الإصابة في الأطفال الذين تتراوح أعمارهم بين ثلاثة وخمسة عشر عامًا. الطفل السليم الذي قد يكون مريضًا في الأيام القليلة السابقة أو يعاني من حمى طويلة يمر بحالة من النوبات المستمرة. النوبات مقاومة لأدوية وعلاجات الصرع، على الرغم من أنه يمكن إعطاء الباربيتورات. قد لا تُرجع الاختبارات التشخيصية الطبية في البداية أي تشخيص واضح وقد لا تكتشف أي تورم واضح في الدماغ. المتلازمة نادرة جدًا: قد تصيب طفلًا واحدًا فقط من بين مليون طفل. (ar)
- Febrile infection-related epilepsy syndrome (FIRES) is an epilepsy syndrome in which new-onset refractory status epilepticus (NORSE) is preceded by febrile illness 24 hours to 2 weeks prior to the onset of seizures. The term was previously used for a paediatric syndrome but was redefined to include all ages. FIRES was previously to refer to this syndrome in children aged three to fifteen years old. A healthy child that may have been ill in the last few days or with a lingering fever goes into a state of continuous seizures. The seizures are resistant to seizure medications and treatments, though barbiturates may be administered. Medical diagnostic tests may initially return no clear diagnosis and may not detect any obvious swelling on the brain. The syndrome is very rare: it may only affect 1 in 1,000,000 children. Febrile infection-related epilepsy syndrome (FIRES) is an infrequent, severe, and potentially deadly childhood epileptic syndrome. It can be described as potentially deadly or infrequent catastrophic epileptic encephalopathy. The characteristics of FIRES are repeated seizures, and febrile illness but no signs of infections. Febrile infection-related epilepsy syndrome (FIRES) was introduced by van Baalen and colleagues in 2010. FIRES also points to the severe clinical progression often consequential in global brain atrophy and poor outcome. NORSE describes a condition in which a healthy person who has not had seizures before, begins having seizures. The clinical progression of FIRES is frequently consequential in global brain atrophy, the outcome of that is very poor. Initial mortality and morbidity are significant, and most survivors are left with drug-resistant multifocal epilepsy and variable degrees of intellectual disability. A fever in an otherwise healthy child is supposedly a risk factor for Febrile Infection-Related Epilepsy Syndrome frequent. The consequence of FIRES is unfortunate, the death rate of up to 30%. FIRES is an immune disorder triggered by an infection. Between 10 and 11.7% of patients die during the acute phase of FIRES either due to obstinate status epilepticus or complications of treatment, including sepsis. It can be assumed that FIRES is associated with metabolic problems, infection, and autoimmune disorders. Affected patients have an unremarkable neurological history and, in close relation with a febrile illness (mainly upper respiratory tract infection or, less frequently, gastroenteritis), abruptly develop several seizures. Electroencephalography (EEG) abnormalities in the hyperacute phase of FIRES. FIRES is characterized by the onset of fever between 24 hours and 2 weeks before the onset of SE and the fever has usually resolved when the seizures start. In the acute phase, SE is particularly obstinate to treatment and can last for many weeks. This is followed by a chronic phase of intractable epilepsy, usually accompanied by cognitive impairment of variable severity. An inflammation can results in acetonemia, raises of fatty acids, inflection of glycemia, and comparative caloric limit. (en)
- Le Syndrome de l'épilepsie fébrile liée à l'infection (FIRES) est une forme d'épilepsie qui touche les enfants de trois à quinze ans. Un enfant en bonne santé qui peut avoir été malade au cours des derniers jours, ou avec une belle persistance de la fièvre va dans un état de permanence des crises. Les crises sont résistantes aux médicaments et des traitements, bien que les barbituriques peuvent être administrés. Médicaux tests de diagnostic peut, au départ, de retour pas de diagnostic clair et ne peut pas détecter de toute évidence une tuméfaction sur le cerveau. Le syndrome est très rare: il ne peut affecter qu'un enfant sur 1 000 000. (fr)
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| complications
| - Severe intractable epilepsy, status epilepticus (en)
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| prognosis
| - Poor, 82% of patients experience some level of permanent intellectual disability ranging from mild to vegetative state. (en)
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| complications
| - Severe intractable epilepsy, status epilepticus
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