Catatonia in Autism and Neuroatypical Patients - Easy to miss, Harder to Treat

  -- More recently I have faced the diagnostic conundrum of catatonia in autism, and that is what I want to explore in more excruciating detail today.  There is surprisingly little literature on the subject, and that is concerning because being able to identify and treat catatonia can be life-saving, not to mention life-altering for patients and their caretakers.  Misidentifying catatonia as mere aggression or highly limited interests in autism can result in exactly the wrong medication being given or no medication being given and a worsening of the condition. --

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Transcript

Catatonia in Autistic and other Neuroatypical Patients

Welcome to PsyDactic.  Today is Saturday, August 10, 2024. and I am Dr. O’Leary, a psychiatrist and fellow in child and adolescent psychiatry in the national capital region.  This is a podcast about psychiatry and neuroscience that I produce in my free time to help inspire me to never stop learning.  I hope that I can help you learn as well.  I should remind anyone listening to this that I am not giving medical advice and any opinion that I present here, even when I try to report on other’s opinions, is only my own opinion and no one else’s.

One of the things that is both frustrating and fascinating about psychiatry is how uncertain everything is.  I consider myself a science-based clinician, but the kinds of science available generally answer only very specific questions.  We still know relatively little about how our brains work (though we are gaining knowledge fast).    Our brains are also not generating anything independent of their environment, so these complex interactions cannot be ignored.  Another difficulty is one of diagnosis, and that is what I want to focus on today.

Many psychiatric diagnoses can be difficult to easily distinguish from each other.  This is one of the reasons that the DSM has been so thoroughly revised so many times.   I have struggled, for example, to distinguish severe ADHD or borderline personality disorder with bipolar 2 disorder versus something else I don’t even know about.  PTSD might resemble agoraphobia and they may be both present simultaneously.  Many disorders that resemble each other are present at the same time.  Now that I am a Fellow in CAP, many of my patients are not neurotypical.  It can be difficult to distinguish narrow interests and repetitive actions from obsessions and compulsions in patients with autism spectrum disorder.  Often, a long period of waiting and observing is required.  Even with patience for our patients I often find myself making a judgment call being less than about 75% confident in what I am treating.  Often it is just a coin toss in my mind, but I have to make a decision anyway.

Let me take a moment to discuss a phenomenon called degeneracy.  To call someone a “degenerate” is an insult.  In English it means that they are a simpleton, they cannot understand anything complex, they cannot be taught, or they are primitive and immoral.  My wife, who is a Spanish instructor, informed me that in Spanish the ser form of degenerado, ser degenerado, means someone is a person who is a sexual deviant.  Tu estas degenerando te, means you are losing ground, you are regressing.  

In mathematics, a degenerate case is a statistical term and it is more difficult to explain, but that has rarely stopped me before.  So let me try.  If you make the radius of a circle 0, it degenerates into a point in space.  If you make one of the angles or sides of a triangle 0, it degenerates into a line segment.  Becoming more simple or having fewer dimensions is not what I mean here.  In fact, I mean that there can be an especially large number of different things that can appear to be the same thing.  If you rotate a two dimensional circle in a three dimensional plane, when it is exactly parallel to my perspective, it will look like a line segment, but so would a triangle, or a square, or a hexagon, or a trapezoid or any two dimensional shape, no matter how many angles it has.

Degeneracy in neuroscience is kinda like that.  It means that a phenomenon does not have to follow a predictable pattern through the brain to appear to be the same thing. For example the pattern of activity that produces the subjective state of anger can differ enormously between individuals or even within the same individual.  Even if you found a part of the brain that would reliably result in anger when you stimulated it, this does not mean that this is the brains anger center and it certainly does not mean that it is necessary to produce everything that appears to be anger.

This means that for any psychological construct (like depression), even though it is identifiable to some extent by its functional endstate (a loss of pleasure and motivation), there are many different patterns of neural activity that can produce it.  As far as we can tell, all of our emotions are degenerate phenomena.  This helps to explain the diagnostic difficulty that psychiatrists face and why the same treatment mechanism only ever works for a subset of patients.

More recently I have faced the diagnostic conundrum of catatonia in autism, and that is what I want to explore in more excruciating detail today.  There is surprisingly little literature on the subject, and that is concerning because being able to identify and treat catatonia can be life-saving, not to mention life-altering for patients and their caretakers.  Misidentifying catatonia as mere aggression or highly limited interests in autism can result in exactly the wrong medication being given or no medication being given and a worsening of the condition.  Antipsychotics at low doses are often helpful with aggression in autism, but care has to be taken when giving these to someone with catatonia because it can worsen the catatonia.  I want to inspire any psychiatrists listening to be extra-sure to rule out catatonia before they merely reach for risperidone in an aggressive patient with autism.

Starting in March of 2022, I produced a series on Catatonia in this podcast, during which I discussed many of the core diagnostic features, so I am not going to repeat all of that here.  However, it is worth reviewing from a 10,000 foot perspective what catatonia is so that you can understand how difficult it may be to distinguish between (for example) excited catatonia and a patient with autism who is also manic, or who is catatonic because they are manic.

Let's start with describing catatonia and why many of its features might be missed or misinterpreted in autistic persons, especially those individuals that have historically required substantial daily support.

Catatonia has been called a movement disorder, and this is evidenced by the fact that we often diagnose it based on the movements or the lack-there-of in catatonic individuals.  Catatonia has been divided into subtypes, retarded also-known-as akinetic, and excited as-known-as hyperkinetic.  It can also progress to malignant catatonia which is nearly indistinguishable from neuroleptic malignant syndrome where patients become stiff, there is autonomic instability, muscle break-down, kidney failure, arrhythmias, seizures and often death.

The retarded or akinetic symptoms of catatonia are generally characterized by a lack of interaction with the outside world.  A patient will move little if at all, fail to feed or care for themselves, they may stare forward and not respond or respond far less than they normally would.  Their body may appear stuck in a position, called postering, and if you move a body part against gravity it may stay in that same position for minutes to hours.  When you are able to move the patient around like a modeling clay statue, this is called catalepsy with lead-pipe rigidity.

The patient may also meaninglessly resist you when you try to move them.  The harder you push, the harder they resist.  They have no reason to resist, they simply cannot help themselves.  Instead of fully resisting, they might provide initial resistance and after you have pushed for a few seconds, they release.  The key to catatonia is that these actions appear motiveless to the examiner.  Another odd feature of catatonic patients is that they might do the opposite of what you say to do.  Again this is motiveless and for those catatonic patients that can remember the episode, they might tell you that they had no choice but to do the opposite.

The excited signs of catatonia include impulsive, meaningless movements.  They may jump up and run around, repeating meaningless phrases.  They may suddenly punch someone without any provocation or start knocking everything off the table that they pass by.  They may do normal things in bizarre ways.  I am going to stop there for now, but there is much more I could say about excited catatonia.

1. Prevalence - much, much higher in neurodevelopmental disorders.  10-20% in autism.  This means if you randomly sampled every person on earth with autism, about 15% of them would be experiencing some level of catatonia, but only a small number of them are diagnosed and being appropriately treated.
2. Catatonia in neurotypical people is easier to identify vs in individuals with autism, but is underdiagnosed in both populations.  The challenge for autistic individuals is distinguishing it from their baseline autistic traits.
   1. This is even more difficult to diagnose if it presents with excited or hyperkinetic symptoms, because these are often present in autism intermittently.  Stereotyped movements, which are movements that occur and appear to have no purpose, and also are not tics or tremors, are common in some autistic individuals.  Mannerisms, which are odd ways of doing purposeful things, are also common in autism.  The main things to consider here is
      1. New-onset symptoms - are the movements not typical of that individual.
      2. Intensity or frequency of symptoms is obviously increased and sustained over time.  They may seem to perseverate and are not as redirectable as before.
      3. Also, if the movements are bizarre, completely out of character, like kicking strangers they walk by or suddenly throwing furniture.
      4. Finally, if any of the symptoms are associated with failure to care for themselves they way they previously did because of the amount of time they spend doing these things or a loss of ability to do other previously learned tasks is a big red flag for catatonia.
   2. Often, there is a kind of prodromal or kindling phase.  The movements or lack there-of may start with small changes, such as eating less, making less eye-contact.  Staring into space.
   3. New movements that resemble tics, such as facial grimacing and shoulder shrugging are also common.
      1. Tics or repetitive movements without any other features should not be the only criteria used to diagnose Catatonia.
   4. Some of the features may appear to be obsessions or compulsions and OCD might be diagnosed.
   5. If the autistic patient is on long-term antipsychotics for aggressive or unmanageable actions, then new, unexplained movements might be mistaken for tardive dyskinesia.
   6. If any new movements are associated with a regression in ability to interact with the outside world, to communicate, or to care for self, this is highly suspicious for catatonia, but might also be interpreted as depression.  The two conditions are not mutually exclusive and mood disorders are one of the most common causes of catatonia.
   7. They may start having difficulty sleeping, when they did not before and be found awake, mumbling to themselves or staring forward with eyes open instead of sleeping.
   8. They may start refusing to do things, or they may do the opposite of what you ask.
   9. You may notice that they appear to have to put in a lot of effort to do simple tasks they could do before like brushing teeth, feeding themselves, shutting doors, or walking on uneven ground or stairs.  If they used to be able to, for example, fold up one of those complicated board games and now they appear confused and stuck, this could catatonia.
3. Prognosis is different for autistic individuals or individuals with neurodevelopmental disorders than with neurotypical individuals - catatonia is often more chronic, harder to treat to remission, more likely to recur without warning.
   1. May take weeks or many months to treat to their baseline.
      1. Start with lorazepam, but may require a longer acting benzodiazepine for maintenance.
      2. Some clinicians with experience treating many neuroatypical catatonic patients report they have to increase the dose of ativan even after an initial good response to a lower dose due to relatively quick adaptation to the new dose.
   2. There are not many controlled clinical trials in catatonia in general and even fewer in autistic individuals, so it may be necessary to rely on case series describing responses to treatments.  Some case series report that autistic individuals often require additional medications, and there are variable ways this is done.  I will link to two presentations available on YouTube from providers with experience treating catatonia in Autism if you are interested.
      1. Some will add Amantadine or Memantine.
         1. Benzodiazepines stimulate gaba receptors which will block glutamate release directly, but you can also try reducing glutamate transmission by giving NDMA receptor antagonists.  This may complement increasing gaba-ergic transmission by generally slowing down brain traffic
      2. If there is self harm, might add on N-acetyl-cysteine.  This is often given in children to reduce self-harming behaviors, so it is reasonable to try if an autistic patient with catatonic features starts to skin pick, pull their hair, or hit themselves.
      3. If there is emotional incontinence, like sudden inappropriate laughter or crying or anger, (also given the fancy name pseudobulbar affect), can add on Nuedexta (Dextromethorphan: sigma 1 agonist/NMDA antagonist; Quinidine: CYP2D6 inhibitor can be used to treat or prevent malaria or as an antiarrhythmic blocks the rapid sodium channel).
      4. Valproate or carbamazepine have been used adjunctively.  I had in the past, seen IV valproate used in an attempt to break catatonia in an elderly patient, but it was not effective.  However, there are certainly many possible etiologies for catatonia, so using anticonvulsants in some instances is reasonable to try.
   3. ECT is the gold standard because response and remission rates are very high.  80 to 90%.  Waiting for treatment resistance may prolong suffering.  However, finding a place that will do ECT in children may be extremely hard.
      1. ECT can treat the catatonia itself as well as any underlying mood component and may improve the psychotic features, though psychosis by itself is not an indication for ECT.
         1. Can shock away mania/depression/catatonia but unlikely to resolve a more purely psychotic process.
      2. Case series in neuroatypical patients report a significant response within 3 treatments, but often further treatments are needed for full resolution.  Less frequent maintenance ECT is often necessary to prevent relapse.  Patients will also likely need to continue benzodiazepines for more than a year and even indefinitely to prevent recurrence.
4. Tracking progress may require a scale other than the Bush Francis, which was not designed or tested in neuroatypical patients.  There is also the KANNER scale and something called the Catatonia Impact Scale.
   1. What is the purpose of the scale?
      1. To Make a diagnosis -  Bush Francis - very reliable
      2. To track daily progression inpatient - KANNER
      3. To give to parents to track weekly progress after discharge - Catatonia Impact Scale
5. When will I know if I need ECT?
   1. Little or no response to medication
   2. Progress halts
   3. Severe mood disorder underlying
   4. Catatonia worsens despite appropriate treatment
   5. Autonomic instability
   6. Uncontrolled muscle movements - concern for rhabdomyolysis

I hope that this episode helps anyone working with autistic and other neuroatypical patients to be able to recognize and treat catatonia, because it is often right under our noses and we don’t see it.  

Thank you for listening.  I am Dr. O and this has been an episode of PsyDactic.

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Peter-Ross EM. Molecular hypotheses to explain the shared pathways and underlying pathobiological causes in catatonia and in catatonic presentations in neuropsychiatric disorders. Med Hypotheses. 2018 Apr;113:54-64. doi: 10.1016/j.mehy.2018.02.009. Epub 2018 Feb 15. PMID: 29523295.

Vaquerizo-Serrano J, Salazar De Pablo G, Singh J, Santosh P. Catatonia in autism spectrum disorders: A systematic review and meta-analysis. Eur Psychiatry. 2021 Dec 15;65(1):e4. doi: 10.1192/j.eurpsy.2021.2259. PMID: 34906264; PMCID: PMC8792870.

Hutton J, Goode S, Murphy M, Le Couteur A, Rutter M. New-onset psychiatric disorders in individuals with autism. Autism. 2008 Jul;12(4):373-90. doi: 10.1177/1362361308091650. PMID: 18579645.

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