from Section 3 - Specific Syndromes and Diseases
Published online by Cambridge University Press: 27 January 2022
Seizures are common in all types of autoimmune encephalitis, but their frequency and severity are particularly relevant in anti-NMDAR, anti-GABAbR, anti-GABAaR, and anti-LGI encephalitis. Seizures occur in>70% of patients in the acute phase of anti-NMDAR encephalitis without specific or distinctive features that may suggest this disorder. In anti-GABAbR and particularly anti-LGI1 encephalitis, seizures may precede in weeks the development of cognitive and psychiatric symptoms. Faciobrachial dystonic seizures (FBDS) are very typical of anti-LGI1 encephalitis and their recognition is important to make an early diagnosis. New-onset refractory status epilepticus (NORSE) is a clinical presentation of epilepsy that occurs in patients without previous history of seizures. When NORSE is preceded by a febrile episode, the term FIRES (febrile infection-related epilepsy syndrome) is frequently used, particularly in the paediatric literature. FIRES is considered a subtype of NORSE that may occur at any age. Only a small number of patients with anti-NMDAR or anti-GABAbR encephalitis presents as NORSE. The term FLAMES (FLAIR-hyperintense Lesions in anti-MOG associated encephalitis with seizures) has been used to describe the encephalitis of some patients with MOG antibodies. The clinical presentation includes seizures associated with isolated or predominantly unilateral cortical hyperintense lesions in FLAIR MRI images.
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