Abstracts

Rationale: Infantile spasms (IS) is an age-specific epilepsy syndrome that occurs during infancy and is characterized by tonic and/or flexor–extensor spasms, hypsarrhythmia on EEG, and neurodevelopmental regression. The most common treatments for IS includes hormonal therapy with adrenocorticotropic hormone (ACTH) - first line therapy - and treatment with Vigabatrin (VGB). ACTH has several common adverse effects, including hypertension, adrenal dysfunction, transient increase in liver enzymes, somnolence, irritability, electrolyte abnormalities, and osteoporosis. Disorders of movement have been rarely described as adverse events related to ACTH therapy. Our objective is to report an unusual ACTH-induced adverse effect seen during treatment of IS. Methods: This study presents a series of four cases taken from the epilepsy and neurophysiology department of Hospital das Clínicas do Paraná (Curitiba, PR, Brazil). The Ethics Committee at “Universidade Federal do Paraná” approved this study. All patient’s parents gave written consent for publication.  Results: We present four patients with IS that were first treated with VGB without success. They were then transferred to our service and started on treatment with ACTH. However, after a few days of hormonal therapy they developed involuntary movements in the form of facial (grimacing, repetitive mouth opening and tongue protrusion), and arms and legs dyskinesias. These involuntary movements were not associated with loss of consciousness or EEG changes and resolved within one to two months after discontinuation of hormonal therapy. One patient had normal brain MRI consistent with cryptogenic IS, whereas three patients had symptomatic IS, with malformation of cortical development, right middle cerebral artery stroke, and microcephaly and periventricular calcifications secondary to congenital cytomegalovirus (CMV) infection. Conclusions: ACTH treatment induced transient dyskinesias in patients with IS. This adverse effect could be mistaken by seizures leading to inappropriate management. Our patients received VGB prior to treatment with ACTH. Therefore, it remains unknown whether these unwanted involuntary movements would have appeared in our patients if ACTH had been started without prior use of VGB. The treatment of IS with ACTH was completed in all patients despite of the dyskinesias and these unwanted involuntary movements could be minimized by addition of Diphenhydramine.  Funding: None.