4.2 Article

Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus

Journal

INTERNAL MEDICINE
Volume 62, Issue 12, Pages 1835-1842

Publisher

JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.0465-22

Keywords

cryptogenic new-onset refractory status epilepticus; intensive immunotherapy; reversible brain atrophy; MRI; autoantibodies

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C-NORSE is a neurologic emergency condition characterized by refractory status epilepticus (RSE) of unknown cause. Brain atrophy in patients with C-NORSE is usually irreversible, but a 33-year-old woman showed recovery of brain atrophy after treatment, along with improved cognitive function.
Cryptogenic new-onset refractory status epilepticus (C-NORSE) is a neurologic emergency condition characterized by refractory status epilepticus (RSE) of unknown cause. Brain atrophy in a setting of C-NORSE is usually irreversible. A 33-year-old woman who was highly suspected of C-NORSE once showed mild frontotemporal atrophy on brain magnetic resonance imaging (MRI), but follow-up MRI revealed recovery of the brain atrophy. Her cognitive function also gradually improved, with a reduction in seizure frequency. Early initiation of intensive immunotherapy with anti-seizure medications may have minimized irreversible brain damage associated with RSE, resulting in a relatively good outcome.

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