Case Report of the Safe Administration of Electroconvulsive Therapy for Catatonia in a Patient with Fragile X Syndrome and Neuropsychiatric Lupus

Joseph Bond, MD, MPH

MGH/McLean Child and Adolescent Psychiatry Residency – Resident
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Scientific Abstract

Background: We report on a complex case of a 21-year old woman with catatonia refractory to benzodiazepines in the setting of neuropsychiatric lupus co-morbid with fragile X syndrome (FXS), which was successfully treated with electroconvulsive therapy (ECT). There are no published recommendations on using ECT to treat catatonia in a patient with FXS, and a single published case report suggests that ECT may have severe cognitive side effects in FXS.

Methods: Catatonia severity was scored using the BFCRS. Cognitive status was scored using the MOCA. Catatonia was initially treated with lorazepam with sustained doses of 2mg every 2 hours without improvement. She was treated with bilateral brief pulse ECT (PW 0.5 ms, frequency 40 Hz, duration 8 sec, current 800 mA, dose 256 mC) with an initial motor seizure duration of 29 sec and EEG seizure duration of 72 sec. Due to the patient’s history of myotonic dystrophy, anesthesia consisted of methohexital (1.25 mg/kg) with rocuronium (0.6 mg/kg) muscle relaxant. She received 5 ECT treatments over 9 days.

Results: Prior to ECT, BFCRS score remained high on hospital days 18-26 with average BFCRS score of 18.2. ECT was initiated on hospital day 27 with rapid reduction in catatonia symptoms following ECT. Average BFCRS score during the week after ECT was 8.0. Prior to first ECT, her MOCA score was 0 and rapidly improved and remained stable six months following ECT with a high score of 24. Clinical Global Impression-Improvement (CGI-I) score of 1 “very much improved” following fourth ECT treatment.

Conclusion: This is the first report of a patient with catatonia in the setting of both lupus and FXS treated with ECT. This patient did not experience an observable drop in cognitive functioning, however her gender and FXS phenotype differ from the previous case report of a patient with FX trait with cognitive decline following ECT. This case suggests that FXS alone is not a contra-indication to ECT but does warrant careful consideration of risks and benefits. We suggest that more observational data on patients with FXS and catatonia be collected and published to help guide clinicians in the use of ECT in these patients.

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research Areas

Authors

Joseph B. Bond, MD, MPH, Evelyne Baroud, MD, James Luccarelli, MD, DPhil, Mayowa Olusunmade, MD, MPH

Principal Investigator

Joseph B. Bond, MD, MPH