Lee Wachtel, MD, Medical Director, Neurobehavioral Unit, Kennedy Krieger Institute. Psychiatry Professor, Johns Hopkins School of Medicine. Baltimore, MD.
Dr. Wachtel, expert for this educational activity, has no relevant financial relationship(s) with ineligible companies to disclose.
CCPR: Let’s start by identifying what we mean when we talk about catatonia in children and adolescents.
Dr. Wachtel: Catatonia is marked by a change from the person’s baseline state with distinct motor, vocal, and behavioral symptoms. Clinicians usually think of a catatonic person as being like a statue—immobile, unresponsive, staring, and with waxy flexibility. But look at DSM-5—catatonia can also manifest as psychomotor agitation with repetitive, stereotypical, purposeless behaviors that escalate to aggression and self-injury and can include dangerous autonomic dysfunction. Agitation is common in children and adolescents, especially with autism. And while catatonia-related agitation may happen in the context of stressors, when there is no clear environmental reason for the agitation, it may be part of catatonia. (Editor’s note: See sidebar below for reference.)
CCPR: How common is catatonia in children and adolescents?
Dr. Wachtel: Studies on adults range from 6% in outpatient settings to 32% on specialized neurologic units. In pediatrics we have two studies. A French study 19 years ago found an incidence of 0.6% but in a specialized setting that may have reduced case identification (Thakur A et al, Aust N Z J Psychiatry 2003;37(2):200–203; Cohen D et al, J Am Acad Child Adolesc Psychiatry 1999;38(8):1040–1046). The other study from India found catatonia in about 6% of inpatients.
CCPR: What about in autistic patients?
Dr. Wachtel: The numbers for catatonia are higher in autism, ranging from 12% to 18% (Billstedt E et al, J Autism Dev Disord 2005;35(3):351–360; Hare DJ and Malone C, Autism 2004;8(2):183–195). There’s been more recognition in the past 20 years of a shared genetic susceptibility between schizophrenia, autism, and catatonia. Some researchers hypothesize that autism itself may be an early form of catatonia, which may become more evident as the child develops.
CCPR: What other conditions are associated with catatonia?
Dr. Wachtel: Affective illness and psychotic illness are the first and second most common comorbidities associated with catatonia. Trisomy 21, Prader-Willi, velocardiofacial, and Phelan-McDermid syndromes are associated with catatonia, and the latter three have susceptibility loci for psychosis as well. The behavioral phenotype at a younger age may look like autism and as time goes on catatonia may emerge, often complicated with affective and psychotic pathology. Anti-NMDAR receptor encephalitis and other encephalitic pictures also present with psychosis and catatonia.
CCPR: Is puberty a trigger?
Dr. Wachtel: Adolescence is often when illnesses like affective and psychotic disorders emerge, and these are associated with catatonia. From an endocrine perspective, catatonia is a state of GABAergic dysregulation, and puberty is when GABA tone increases to adult levels.
CCPR: How do we assess patients for catatonia?
Dr. Wachtel: Get the history from family members since patients vary significantly in what they can share. Ask about and look for the motor, vocal, and behavioral symptoms in the DSM-5 and/or the Bush-Francis Catatonia Rating Scale (www.tinyurl.com/4yzm27nf) and assess for autonomic symptoms. Look for Parkinsonian symptoms since those might be present in catatonia. Check the range of motion in your patient’s arms at the elbow and wrist joints, looking for rigidity, resistance to movement, tone, and any cogwheeling. Waxy flexibility really feels as if the patient’s limb was made of soft wax. Position the patient’s arm in the air and see if it remains there when you release it. Look for “getting stuck” during movements, unusual repetitive movements, facial grimacing, staring, and stupor.
CCPR: And what about lower body or walking?
Dr. Wachtel: Watch how the patient goes from sitting to standing, noting any difficulty in moving fluidly. Ask them to walk down the hallway. Do they have a shuffling gait? Are they slow? Do they lack an arm swing? What does their pivot look like? Evaluate the overall ability to initiate, change, and cease movement. Is there festination (speeding up) and/or retropulsion (walking backwards)?
CCPR: What about vocal and behavioral symptoms?
Dr. Wachtel: Vocal symptoms include frank mutism and/or loss of other forms of communication (eg, use of an iPad or augmentative communication device). There is a wide range of behavioral symptoms such as automatic obedience, ambitendency, echophenomena (any automatic imitation), and purposeless agitation.
CCPR: Can you explain automatic obedience and ambitendency and how to assess for these?
Dr. Wachtel: Automatic obedience is when the patient does what you say even if it doesn’t make sense. It can be assessed pretty simply. I will often tell the patient: “Please stick out your tongue; I’m going to put a pin in it.” Ambitendency is hesitation or ambivalence when you challenge the patient with opposing gesture and command. To assess for this, I hold out my hand and ask the patient to shake it. Then I hold out my hand and instruct the patient to NOT shake it. With a smaller child, I might jump up and down and ask the child to do the same, then jump up and down and ask the child to NOT do the same.
CCPR: What about purposeless agitation? What does that look like?
Dr. Wachtel: A catatonic patient may be frenetic, engaging in purposeless motor activity. The agitation can progress to where a patient looks like a wind-up toy gone mad, with repetitive self-injurious behavior of incredible frequency and intensity when the patient is not restrained. Many autistic children engage in repetitive self-injurious behavior, and while in some children this behavior is a response to something in their environment, in others it is part of catatonia.
CCPR: What other questions should we ask to clarify symptoms of catatonia?
Dr. Wachtel: Ask about the patient’s ability to perform tasks, response times, and any loss of skills. Do they need a lot of prompting? These kids take a lot longer to do tasks than before or do not complete them at all. This isn’t childhood obstinance. Older patients with catatonia often withhold urine and stool, but children typically lose bladder or bowel continence. With developmental disabilities, skills that take years—reading, piano, or any learned ability—may be lost. The first population-based study on catatonia in autism spectrum disorders commented upon the frequent slowness, amotivation, and prompt-dependence in these patients (Wing L and Shah A, Br J Psychiatry 2000;176:357–362).
CCPR: Catatonia symptoms might be confused with mood disorders, psychosis, and autism.
Dr. Wachtel: There can certainly be overlap in some symptoms. Some depressed kids are quite unresponsive. But when an autistic patient very suddenly loses skills, becomes slow or unresponsive, or engages in new purposeless, repetitive behaviors, think about catatonia and look for other symptoms.
CCPR: Can this withdrawal or disinterest extend to appetite and eating?
Dr. Wachtel: Yes. Pay attention to food refusal as a manifestation of negativism. This is different from an autistic child who, for example, may only eat purple foods. Any child may develop avoidant/restrictive food intake disorder (ARFID), which is itself dangerous (Editor’s note: See The Carlat Child Psychiatry Report, July/Aug/Sept 2022 for more on ARFID), but in autism consider a catatonia diagnosis. Catatonia may progress to the point where patients totally refuse food and drink. We’ve had children end up on nasogastric or gastrostomy tubes.
CCPR: We’ve talked about motor, vocal, and behavioral symptoms. What about autonomic symptoms?
Dr. Wachtel: Symptoms of autonomic dysfunction include excessive sweating, flushing, acrocyanosis (blue extremities), or unstable vital signs including fever, high or low blood pressure, and elevated heart rate. These may indicate malignant catatonia, also known as neuroleptic malignant syndrome (NMS), a severe form of catatonia that can be fatal if left untreated and should prompt immediate treatment.
CCPR: Many of us trained during a time when NMS was not identified as a form of catatonia. Can you clarify how NMS was recognized as a form of malignant catatonia?
Dr. Wachtel: When NMS was first observed after chlorpromazine and other antipsychotics became more widely used, including antiemetics, the thinking was that NMS should be treated through dopamine agonism, supportive therapy like hydration, and bromocriptine. Malignant catatonia refers to hemodynamic and thermoregulatory instability in catatonic patients, where patients may have hyper- or hypotension, brady- or tachycardia, and elevated or decreased core body temperature. This can lead to global organ system collapse and death. NMS has this same rigidity, dystonia, and autonomic dysfunction, and is largely now conceptualized as malignant catatonia caused by an antipsychotic, so withdrawing the agent is the first step, with subsequent rapid implementation of anticatatonic treatment paradigms: benzodiazepines and electroconvulsive therapy (ECT). ECT is literally a lifesaver in NMS.
CCPR: So let’s talk about treatment. What should be our initial treatment approach?
Dr. Wachtel: The first-line treatment for catatonia is benzodiazepine therapy. I usually use lorazepam because it’s cheap, readily available, and can be administered with TID dosing. We usually start with 0.5 mg TID and then increase the dosage by 0.5 mg TID every two to three days based on response. It is often necessary to push the dosage quite high to effectuate a response, well beyond what a noncatatonic patient could tolerate. For example, I had a mute, unresponsive patient who needed 8 mg TID of lorazepam and would get up and dance upon administration. When benzodiazepines are insufficient, or the potential morbidity and mortality of the patient is high, then ECT is the definitive treatment for catatonia.
CCPR: Tell us more about using ECT for catatonia in children and adolescents.
Dr. Wachtel: ECT is a rapid and robust treatment for catatonia that hasn’t responded to benzodiazepines. It can rapidly and safely treat malignant catatonia/NMS, sometimes within two or three days with daily ECT (“en bloc”). Some patients are out of the ICU after two ECT treatments. ECT is one of the few interventions in child psychiatry where we can quickly save somebody’s life. Untreated, NMS carries a 10%–20% mortality rate (Fink M and Taylor A. Catatonia: A Clinician’s Guide to Diagnosis and Treatment. Cambridge University Press; 2006). Some patients have a chronic course and might require several years of ECT in gradually decreasing frequency, from several times per week to every three months.
CCPR: Child psychiatrists are often wary of ECT. How do you address their concerns?
Dr. Wachtel: In recent years, the American Academy of Child & Adolescent Psychiatry expanded teaching about catatonia and ECT. It is a paradigm shift for pediatric practitioners to imagine that a controlled seizure can have beneficial effects on the brain. Some families have been vocal about their children’s positive experiences with ECT, and they’ve paved the way for other families to feel comfortable pursuing ECT.
CCPR: How do families respond to your recommendation for ECT?
Dr. Wachtel: I have heard responses ranging from “that’s horrible” or “I would never do that” to “when can we start?” Education is key. I’m frank with them and I demystify the process by explaining exactly what happens. I make myself available to families to ask questions and gather information so they can make the best decision for their child. We also have an internal network to connect families with other families after they’ve had a chance to speak with me, so the educational process is not limited to talking to the professional.
CCPR: Do some families decline ECT?
Dr. Wachtel: Many families won’t consider ECT initially, but as the situation deteriorates, they realize that this can become a life-or-death situation. I had a parent who was tearful and upset until I showed him a video of the ECT process, and his response was “that’s it?” In terms of visible motor activity, not much happens in modern, modified ECT with anesthesia and neuromuscular blockade.
CCPR: How do you treat patients who have psychosis with catatonia?
Dr. Wachtel: Antipsychotics can worsen catatonia, and we typically discontinue them. Once the catatonia is gone, you can target the psychosis. We have had mute, unresponsive patients in whom it became clear that they were floridly psychotic during the initial course of ECT, and we subsequently were able to start an antipsychotic. During maintenance ECT, I use lithium or lamotrigine for maintaining clinical stability while decreasing ECT frequency. (Editor’s note: When using lithium and ECT, you may need to use lower doses of lithium to avoid delirium.)
CCPR: What do you do when patients do not respond or cannot tolerate lithium or lamotrigine?
Dr. Wachtel: Some respond to atypical antipsychotics. When using an antipsychotic, monitor the CPK and WBC, at least in the early stages of therapy, to pick up any early warning signs of malignant catatonia/NMS. Some patients remain moderately negativistic in terms of food refusal even after ECT and may anecdotally benefit from a low dose of olanzapine to increase appetite. I prefer using atypical over typical antipsychotics because of the increased risk profile with the latter.
CCPR: Any final thoughts?
Dr. Wachtel: Catatonia is more common than you might realize, especially if you work with patients with neurodevelopmental challenges. Recognition of catatonia is one of those areas in child psychiatry where you can save somebody’s life. We need to be vigilant just like we are about suicidal ideation because patients’ lives depend on our astuteness.
CCPR: Thank you for your time, Dr. Wachtel.
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