In this 88‑minute video, Dr. Deepan Singh discusses what anxiety looks like in PWS, what leads to it, and how can it be treated. Learn from this PWS mental health presentation, which concludes with Dr. Singh answering questions from the audience at the 2020 FPWR Virtual Conference.
Click below to watch the video. If you're short on time, scroll down for timestamps to find the portions you're most interested in.
Presentation Summary With Timestamps
2:56 Introduction by FPWR Executive Director Susan Hedstrom. Dr. Singh:
- Holds a board certification in general psychiatry and one in child and adolescent psychiatry.
- Has helped treat PWS patients with symptoms from mild attention deficit, to severe aggression and psychosis.
- Has published on the occurrence of cycloid psychosis in PWS.
- Is the first to have published on the use of extended release guanfacine in the management of behavioral issues associated with PWS.
5:40 Dr. Singh presents
- Look at the big picture: don’t get lost on the symptom, and rather try to decipher the underlying reason why the behavior is occurring.
- Anxiety manifests itself differently in PWS than in the general population.
- Important to understand compulsivity and “response perseveration.”
- Presentation objectives; session will end with a list of commonly used treatment strategies against anxiety.
8:00 Defining anxiety from a pathological perspective
- Anxiety is a response to threats: flight or fright response. Perseverative cognition negatively affects functioning; or when you cannot stop thinking about something that is not directly harming or threatening you, but you always have it “playing” in the background.
- A transdiagnostic process contributes to a range of psychopathologies and helps determine if it’s a disorder.
- Teamwork: Working with patients and families to draw a timeline; differentiate a chronic anxiety to episodic anxiety.
11:11 Types of Anxiety Disorders
- The DSM‑5 Anxiety Disorders are the following.
- The two most common ones are Generalized Anxiety Disorder (GAD), and a Specific Phobia.
- The one most commonly found in the PWS population is GAD, not attributable to one particular thing.
- A third anxiety disorder is Separation Anxiety, more common in kids, but can also be seen in adults. PWS patients feel very attached to caregivers, and there is a perseverative anxiety linked to them.
- Social anxiety is also common, and it occurs when there comes a time to speak or open up in front of others.
17:15 Types of Obsessive‑Compulsive and Related Disorders
- An obsession consists of the inability to get a negative or distressing thought out of your head even if you don’t like it, and can be like images or thoughts playing like a broken record.
- Compulsions are recurrent actions that can be consciously or unconsciously done to get rid of anxiety. Sometimes they go hand in hand with obsessions.
- Body Dysmorphic Disorder is another type of disorder, not often seen in PWS.
- Hoarding disorder is very common in patients with PWS.
- Excoriation disorder (or skin-picking) is another type of behavior regularly seen in PWS.
- The PWS related obsessive-compulsive behavior seems to be different from all these.
22:26 Can be hard to categorize PWS behaviors as fitting either the Anxiety or Obsessive-Compulsive related disorders.
- Response Perseveration is the inappropriate repetition of a particular behavior despite the absence or cessation of reward.
- In PWS, it’s related to compulsivity. The repetitive questioning and intrusive behavior occurs despite the negative response that might be given by their caregivers.
- Many caregivers might ask: “Is it anxiety, or is it compulsivity?”
- Response monitoring is the capacity to flexibly adapt to dynamic environments. It’s a crucial component of optimal daily functioning, and it comes from a physical difference in their prefrontal cortex.
- Their prefrontal cortex is able to hold less volume, making it more difficult to perform executive functions.
- The diagnosis in PWS is “Obsessive-Compulsive and Related Disorder due to PWS with OCD-like symptoms, hoarding symptoms, and skin-picking symptoms.”
32:00 Potential treatment and medication
- Benzodiazepines are most used to treat anxiety symptoms. They work on the GABA receptor.
- Benzodiazepines are a powerful calmer, or downer, and it can be a powerful medication.
- The medication can affect the reserves for cognitive function in PWS patients, and it’s usually not a long-term solution. It can make aggressive behaviors worse.
- Serotonin Receptor Inhibitors (SRI) can be very effective, but they can cause mania in patients prone to develop bipolar-type symptoms or psychosis. Used very seldom in PWS.
- Antipsychotics are the third type of medications, and they are used for particular situations where anxiety is coming from psychotic reasons (hearing voices, feeling paranoid).
- Mood stabilizers are the fourth and final type of medications to treat anxiety: lithium or anticonvulsants.
- Other medications Dr. Singh mentioned are Buspirone, Mirtazapine, Bupropion, NAC, which have been tested. There is early evidence coming from treatment with DCCR, pitolisant, and CBD.
- The Alpha-2 Agonists Guanfacine XR is available right now with the brand name of Intuniv. It has been used as treatment of ADHD and it has been tested in autism.
- It has been shown to help with impulse control, aggression and self-injury, and it also increases the activity of the lateral prefrontal cortex.
- The most common side effect of guanfacine is excessive daytime sleepiness, which takes longer for some patients to get used to while taking the medicine for the first time. It takes about four days to get used to the medication and then Dr. Singh mentions he will usually readjust the dosage at that point.
53:00 Q&A
