Psychogenic Non-epileptic Seizures Secondary to Forced Normalization Phenomenon in a Patient with Eyelid Myoclonia with Absence Epilepsy (Jeavons Syndrome): A Case Report
Fernando Vasquez Lopez1, Juan Carlos Vera-Lopez2, Maximiliano Salgado-Deza2, Salvador Martinez-Medina2, Jimena Gonzalez-Salido2, Jimena Colado-Martinez2, Stefan Narvaez-Labuhn2, Mariana Peschard-Franco2, Andres Morcillo Muñoz2, Katherin Milagros Plasencia Correa2, Iris E. Martínez Juárez2
1Instituto Nacional de Neurología y Neurocirugía "Manuel Velasco Suárez", 2Epilepsy Clinic, Instituto Nacional de Neurología y Neurocirugía "Manuel Velasco Suárez"
Objective:
To describe the first reported case of psychogenic non-epileptic seizures (PNES) as a manifestation of forced normalization (FN) in a patient with drug-resistant Epileptic Eyelid Myoclonia (EEM), highlighting diagnostic and therapeutic challenges.
Background:
Epilepsy with eyelid myoclonia (EEM) is a rare subtype of genetic generalized epilepsy characterized by eye-closure–induced seizures, photosensitivity, and EEG paroxysms, typically beginning in childhood with a female predominance. Forced normalization (FN) refers to the emergence of psychiatric symptoms following seizure control. Psychosis is most common, but other manifestations, including PNES, may occur.
Design/Methods:
A 26-year-old female with long-standing, drug-resistant EEM began experiencing eyelid myoclonia and absence seizures at age 10, refractory to multiple antiseizure medications (oxcarbazepine, valproate, lamotrigine, lacosamide). At age 24, ethosuximide was introduced to target absence seizures. Eleven days later, she developed brief, fluctuating episodes characterized by asynchronous limb movements, forced eyelid closure, and upward gaze deviation, without postictal confusion. Video-EEG captured these events without electrographic correlates. Psychiatric evaluation diagnosed PNES and mixed anxiety-depressive disorder. Ethosuximide was discontinued, while levetiracetam and clobazam were maintained.
Results:
The patient met Krishnamoorthy and Trimble’s FN criteria, including ≥50% reduction in interictal spike frequency compared to baseline EEG. She achieved seizure freedom before PNES onset and has remained seizure-free for two years, though PNES persists 1–2 times per week despite psychiatric treatment.
Conclusions:
This is the first reported case of PNES as a manifestation of FN in EEM, representing the close interaction between epilepsy control and emergent psychiatric symptoms. Ethosuximide may act as a pharmacological trigger in susceptible patients. Early recognition of FN is crucial for preventing misdiagnosis and optimizing interdisciplinary management strategies.
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