Electroconvulsive Therapy, Catatonia, and Regression in People with Down Syndrome

Electroconvulsive therapy (ECT) is a treatment that involves sedating an individual with anesthesia and then inducing a seizure with a short electrical stimulation of the brain (more information can be found on the American Psychiatric Association’s website ). ECT can be frightening to some. Hollywood has done a good job of helping us remember disturbing, outdated ECT procedures, such as is seen in the movie One Flew over the Cuckoo’s Nest. ECT does cause the person to have a seizure and that can be frightening. However, today it is typically done with the person under anesthesia, so the person is not aware of the procedure, does not remember it, and is safe during the procedure. In addition, some other brain stimulation procedures are available and brief descriptions are provided on the American Psychiatric Association website .

ECT can be an effective treatment for some individuals with Down syndrome who have catatonia and/or severe depression as well as some other mental health conditions. Catatonia is an abnormality of movement and behavior. Catatonia may present in various ways in a person with DS. The main features include repetitive or purposeless overactivity and/or resistance to movement. Catatonia is a condition that traditionally was associated with psychoses, but it is now recognized that it can occur with a variety of psychiatric or medical conditions such as depression (Daniels, 2009). It has also been proposed that it be a separate diagnosis not associated with any psychiatric or medical conditions (Shorter, 2012; Padhy, Parakh, & Sridhar, 2014). Individuals Down syndrome who have what has been called regression syndrome, Down syndrome regression disorder, or Down syndrome disintegrative disorder may also have catatonia.

Catatonia has not been studied much in Down syndrome, but one group of researchers (Ghaziuddin et al., 2015) described catatonia in a small series of people with Down syndrome. The authors used high dose lorazepam combined with ECT to treat the four individuals with Down syndrome in their study and reported that they all recovered their baseline level of functioning. However, the patients in this study needed prolonged usage of ECT to successfully recover from catatonia.

Despite Ghaziuddin’s successful use of ECT in treating four patients with Down syndrome and catatonia, further investigation of the use ECT for this purpose is warranted. A recent meta-analysis of the use of ECT in people without Down syndrome who were diagnosed with catatonia found serious side effects in seven studies, including mental confusion, memory loss, headache, or adverse effects associated with anesthesia (Leroy et al., 2017). The study further concluded that the “literature consistently describes improvement in catatonic symptoms after ECT. However, the published studies fail to demonstrate efficacy and effectiveness.” As best as we can tell, in our experience, our patients have not typically had persistent mental confusion or memory loss. 

Additional study is needed to understand the role of ECT in people with Down syndrome and catatonia. However, in our experience and the experience other clinicians have reported to us, treatment with ECT does result in dramatic improvement in some individuals. We have seen improvement of symptoms for many and resolution for some.  It tends to be more likely to be beneficial if it is done earlier in the course of the regression and catatonia. However, at our Center, we have patients who had catatonia and regression symptoms for several years before undergoing ECT who still responded well to ECT.

If an individual with DS has treatment-resistant depression or catatonia, a discussion about ECT may be considered.  Review the indications and potential side effects.  Discuss with your provider and find a psychiatrist skilled in ECT.  Discuss the number of treatments recommended.  Many people with DS when being treated with ECT for catatonia will need an initial series of treatments.  This will vary but it may, for example, include twice weekly treatments for 6-8 weeks followed by follow-up or even ongoing treatments less frequently (for example, weekly or monthly). Before starting ECT at our hospital, a pre-anesthesia assessment, blood work, ECG (electrocardiogram), and spine x-rays are required.

The most important challenge in getting ECT is finding a psychiatrist who is trained in ECT and able to skillfully provide it for people with DS.  There may also be legal issues in your state.  This is particularly true when considering ECT in someone less than 18 years of age.

While treatment with ECT can be quite beneficial in people with DS with catatonia, it is important to remember it is not the only treatment available.  Medications, immunotherapy, counseling, occupational therapy, physical therapy, speech therapy, and other interventions may be recommended depending on the symptoms and the findings on the evaluation of the individual.  We have several articles and webinars in this section of our Resource Library. 

References

Daniels J. Catatonia: clinical aspects and neurobiological correlates. J Neuropsychiatry Clin Neurosci. 2009;21(4):371-380. doi:10.1176/jnp.2009.21.4.371 

Ghaziuddin N, Nassiri A, Miles JH. Catatonia in Down syndrome; a treatable cause of regression. Neuropsychiatr Dis Treat. 2015;11:941-949. Published 2015 Apr 2. doi:10.2147/NDT.S77307

Leroy A, Naudet F, Vaiva G, Francis A, Thomas P, Amad A. Is electroconvulsive therapy an evidence-based treatment for catatonia? A systematic review and meta-analysis. Eur Arch Psychiatry Clin Neurosci. 2018;268(7):675-687. doi:10.1007/s00406-017-0819-5

Padhy SK, Parakh P, Sridhar M. The catatonia conundrum: controversies and contradictions. Asian J Psychiatr. 2014;7(1):6-9. doi:10.1016/j.ajp.2013.07.006

Shorter E. Making childhood catatonia visible, separate from competing diagnoses. Acta Psychiatr Scand. 2012;125(1):3-10. doi:10.1111/j.1600-0447.2011.01788.x