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Understanding Pediatric Acute-onset Neuropsychiatric Syndrome

October 23, 2019


(water splashing) (thunder rumbling) (rain pattering) – As a healthcare provider,
you’ll likely hear these and similar chief complaints from the families of PANS patients. PANS is a problem that
dramatically changes a previously healthy and developmentally
normal child overnight. It presents with a number of emotional and neurological changes
that start at the same time. At first, one may suspect
such an abrupt change could stem from psychosocial trauma, such as bullying or abuse. Or it could represent the onset
of a transient tic disorder, a urinary tract infection, or reactions to challenges of a new situation, each of which could explain
one or two of these symptoms. But Occam’s Razor seeks
a unitary explanation. And there is one, so that
when these various changes begin suddenly and simultaneously, PANS and other inflammatory
brain disorders should be considered in
your differential diagnosis. Inflammatory brain responses occur when a misguided immune
response affects the brain. Sydenham chorea, Hashimoto’s encephalitis, anti-NMDA receptor
encephalitis, CNS lupus, and pediatric multiple sclerosis are examples of brain disorders thought to be caused by
immune dysregulation. Each of these presents with its own course and characteristic neurological
and psychiatric symptoms. Some brain inflammation is thought to be mediated by the innate immune system, involving nonspecific
inflammatory processes, while others are thought to involve the adaptive immune system,
involving antibodies that recognize epitopes in the CNS. Some inflammatory brain disorders may very well involve both systems. Antecedents for pathological
immune responses may be infection, trauma, tumors, seizures, metabolic or endocrine disturbances, or otherwise, antecedents
may remain unidentified. All of these possibilities
should be considered with any change in
psychiatric or mental status. Clinicians should always suspect some sort of inflammatory brain disorder when a previously healthy
child presents with acute or subacute symptom change, such as the development of seizures, neurological deficits, cognitive
or memory difficulties, movement abnormalities,
or psychiatric symptoms. For further information about evaluation and treatment of pediatric
inflammatory brain disorders, please see articles by
Dale, Grouse, and Britton in the reference section. In the case of PANS, the
precipitant need not be specified and may not be known. PANDAS, a subtype of PANS, is
by definition associated with antecedent group A beta hemolytic
streptococcal infection. Of course, not all
group A strep infections are followed by PANDAS, just as not all group A strep infections are followed by rheumatic
fever or Sydenham chorea. Please consult the reference section for PANS and PANDAS diagnostic guidelines for detailed information
on differential diagnosis and a workup. For the sake of clarity and consistency, I will use the term PANS
to refer to both PANDAS and other subtypes of PANS
throughout this course. To meet diagnostic criteria for PANS, the onset must be sudden and severe. This onset is often described as fu-dreyon, or lightning-like, where multiple psychiatric
symptoms and neurological changes start and develop dramatically
over less than 48 hours. PANS is characterized
by the sudden onset of obsessive-compulsive symptoms
or severe food restriction. PANDAS’ cardinal symptoms are different. When the PANDAS criteria
were first defined its cardinal symptoms included the sudden onset of tics or OCD. In both PANDAS and the
umbrella disorder, PANS, OCD symptoms are present
and are associated with at least two other
neuropsychiatric symptoms whose emergence is equally
as sudden and debilitating. This sudden onset of symptoms
is typically followed by a slow remission. Much like asthma, Crohn’s disease, and other inflammatory disorders, PANS can repeatedly flare up
and then improve over time leading to a sawtooth course of illness. To make the diagnosis of PANS, particular co-occurring symptoms must be considered as a group. The symptoms may not seem
related at first glance. Why would separation anxiety and enuresis or rage attacks and
handwriting deterioration come on together? When looked at as a whole,
a clear picture of PANS comes into view as a
constellation of symptoms. Here are the PANS diagnostic
guidelines established in 2013 by an experienced group of
researchers and clinicians to unify future research. They were created to broadly capture what PANS clinical
presentation looks like. Namely, the guidelines describe three distinct criteria
for PANS diagnosis. One, abrupt, dramatic onset of OCD or severely restricted food intake. Two, concurrent presence of
neuropsychiatric symptoms from at least two of the following: A, anxiety, B, emotional
lability and/or depression, C, irritability, aggression, and/or severely oppositional behaviors, D, behavior regression, E,
deteriorating school performance related to ADHD-like
symptoms, memory deficits, and/or cognitive changes, F,
sensory or motor abnormalities, G, somatic signs and symptoms,
including sleep disturbances, enuresis, or urinary frequency. Three, finally, the criteria states that the symptoms are not better explained by a known neurologic or medical disorder, such as Sydenham chorea. We’ll discuss these
criteria in greater detail later in the course. Because PANS symptoms are diverse, families may first seek help from a diverse array of medical specialists, all of whom should be aware of PANS, in order to expedite
appropriate treatment. If these practitioners do not have PANS in their differential diagnosis, the search for an accurate diagnosis can resemble something like the parable of the blind men and the elephant. In that parable, a
number of wise blind men were tasked with identifying
an unusual creature. Each man felt the animal
from his unique angle and deduced very different conclusions. One examined the leg and thought the animal was actually a tree. Another felt the tail and
declared it a ropy animal. The blind man on the trunk thought the animal was something like a fire hose. Another felt the skin and
mistook it or leather and so on. Individually, none
could declare the animal to be an elephant. But put their observations together and the conclusion becomes clear. And just like the blind men, different specialists who
detect specific PANS symptoms but are unfamiliar with PANS will not suspect it in the first place. Please refer to our course
materials for more information on the way children with PANS may present to various pediatric specialists. Regardless of the setting,
when diverse psychiatric and neurological symptoms present abruptly with OCD, tics, or food
restriction over a couple days, please consider PANS in
your differential diagnosis so that your patient may
receive appropriate treatment. PANS treatment integrates
three primary strategies. One, identification and
treatment of active infection, as appropriate. Two, assessment and
modulation of inflammation, as appropriate. And three, initiation of supportive behavioral, cognitive
behavioral, and, if needed, pharmacologic treatment, in
order to facilitate workup, address psychiatric symptoms,
optimize family behavior in response to symptoms,
and decrease suffering. The three strategies need to be undertaken nearly simultaneously. At the same time
infection and inflammation are typically identified and treated, patients must be managed and
their families must be educated about the syndrome and its management. From the start, children will require mental health evaluation and treatment. They should continue in
school as much as possible while participating in therapy in order to initiate rehabilitation, much like other brain injury rehab. Most PANS patients do miss some school and school administrators and teachers will request information and guidance. Often, PANS patients require
referral to specialists such as cognitive behavior specialists, child psychiatrists,
occupational therapists, dieticians, and physical therapists. Depending on what the case
presentation warrants, they may require consultation
with additional specialists like a pediatric
rheumatologist, otolaryngologist or an infectious disease specialist. We’ll cover first-line
and secondary options for referral and treatment in greater detail later in the course. Let’s now move to module three, where we’ll use the case
of a seven-year-old boy to illustrate our
approach to the diagnosis and initial management of PANS. (upbeat, peaceful music)

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