Details

Autor(en) / Beteiligte

• Proietti, Jacopo
• Fiorini, Elena
• Cantalupo, Gaetano
• Fontana, Elena
• Lo Barco, Tommaso
• Bonin, Cecilia
• Bernardina, Bernardo Dalla
• Darra, Francesca

Titel

Refractory tonic-myoclonic status epilepticus with catamenial recurrence in epilepsy with myoclonic atonic seizures: A case report

Ist Teil von

• Heliyon, 2024-01, Vol.10 (2), p.e24747, Article e24747

Ort / Verlag

England: Elsevier Ltd

Erscheinungsjahr

2024

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• In epilepsy with myoclonic-atonic seizures (EMA), status epilepticus (SE) may occur during the onset phase, uncommonly in post-puberal patients. We report a post-puberal patient with EMA who presented SE with insidious onset and catamenial recurrence. She had a stormy epilepsy onset at 4 years, with tonic seizures, atypical absences, and myoclonic-atonic seizures, in the absence of SE. After the onset phase, sporadic nocturnal tonic seizures persisted and a mild intellectual disability appeared. At the age of 7, after gonadotropin-releasing hormone analog administration due to central precocious puberty, she presented with SE characterized by recurrent atypical absences, tonic seizures, and awareness impairment, which was successfully treated in 4 days. At 11 years, one week before menstruation, the patient presented with analogous SE that lasted 8 days. One week before the subsequent menstruation, she presented again with SE, initially characterized by atypical absences alternating with phases of awareness and motor impairment related to fast low-voltage EEG activity in the central regions; later, tonic and myoclonic seizures occurring even in the awake state increased, and the “atonic-akinetic status” related to fast EEG activity worsened. After conventional antiepileptic drugs had failed to control the seizures, a progestin was added, with subsequent gradual complete recovery.