Introduction. This report presents a clinical case of an 11-month-old patient with a compound heterozygous mutation in the TRAPPC2L gene, associated with early infantile progressive encephalopathy (OMIM 618331). Starting from 2.5 months of age, the patient exhibited serial epileptic spasms, developmental regression, and pharmacoresistance to levetiracetam, valproic acid, vigabatrin, methylprednisolone, and pyridoxine, necessitating objective data for diagnosis confirmation and therapy efficacy assessment.

Methods. Analysis of clinical and instrumental data, including dynamic video-EEG studies, was performed. Given the challenges in interpreting parental reports and a clinical picture suggestive of epileptic spasms, with no ictal EEG patterns but presence of multiregional epileptiform activity—the BASED scale (Burden of Amplitudes and Epileptiform Discharges), developed in 2021, was applied for quantitative assessment of disease severity and objective evaluation of treatment response.

Results. Initial EEG showed modified hypsarrhythmia, corresponding to a high BASED score. Therapy with levetiracetam and valproic acid had no significant effect, while vigabatrin (100 mg/kg) provided only a brief response. A 39-day course of adrenocorticotropic hormone (ACTH) achieved clinical cessation of visible seizures by day 15. Follow-up video-EEG monitoring revealed multiregional epileptiform activity with a very low index, indicating a substantial reduction in the BASED score and objectively confirming positive dynamics with seizure resolution. Improvement included onset of cooing and emotional responses (laughter).

Conclusion. This case highlights the pivotal role of the BASED scale in diagnosing epileptic spasms in complex scenarios without typical ictal EEG patterns. The scale enabled objective confirmation of the epileptic nature of the events based on interictal activity, severity assessment, and justification for ACTH therapy, thereby supporting an effective treatment strategy aimed at seizure control and psychomotor development improvement.

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