Download Self Injurious behaviors

Psychopharmacological Treatment of
Behavioral Problems in Neurogenetic
Syndromes
By:
Dr. Mohammad Effatpanah
Child and Adolescent Psychiatrist
Assistant Prof. at TUMS
April 2014
1
Definition


Neurogenetics is a branch of genetics that
analyzes the impact of genes on the
structure and function of the brain and
peripheral nervous system
The role of genetics in the development and
function of the nervous systems
2
What are clinical Neurogenetic
syndromes?






Fragile X syndrome : most common cause OF ID
Prader-Willi syndrome
Angelman syndrome
Williams syndrome
Tuberous sclerosis
Neurofibromatosis
3
Neurogenetics syndrome
4
Some facts



Epidemiological surveys indicate that up to two
thirds of children and adults with Intellectual
disability have comorbid mental disorders
Epidemiological study found that 40.7 percent of
intellectually disabled children between 4 and 18
years of age met criteria for at least one psychiatric
disorder
A basic strategy of "Start low, Go slow" is
warranted (lower starting doses, increase more
slowly.)
5
Reasons for psychotropic medications
These drugs are used in five main areas:
For treatment of psychiatric disorders
For the control of behaviors problems
To reduce aberrant or uncontrolled sexual drive
For the treatment of epilepsy and associated disturbances
For other conditions
6
Some facts

Up to 75% of persons with ID who are referred for
psychiatric assessment have undiagnosed or
undertreated medical conditions, and nearly 50%
receive nonpsychotropic mediations that could have
behavioral side effects
7
Some facts

Between 20% and 50% of the residents in
institutions for ID receive psychotropic
drugs

As little evidence as there is for
psychopharmacology in adults, there’s
much less for children
8
Some facts


Various investigators have observed that only about
one-fourth of the mentally retarded people who
receive psychotropic medication have a psychiatric
Diagnosis
The drugs are most often prescribed to combat
particular behaviors that could not be tolerated by
others, principally aggression, SIB, destructive
behaviors, hyperactivity, withdrawal, and sleep
9
Approaches

Challenging behaviors

Syndromes
10
Challenging behaviors


Behaviors that interferes with an individual’s
or care's daily life
Common examples of challenging behaviors
are aggression, self-injurious behaviors,
property destruction, oppositional behaviors,
stereotyped behaviors, socially inappropriate
behaviors, and withdrawn behaviors
11
Challenging behaviors

Challenging behaviors can occur in the absence of a
psychiatric disorder and not all people with mental
illness exhibit challenging behaviors
12
Common Challenging behaviors
Attention-Deficit/Hyperactivity behaviors


Hyperactivity and ADHD are common traits
in ID children and adolescents and occur in
approximately 10%–20% of the patients
For these children , stimulants as well as
other drugs such as anti depressives,
neuroleptics , anticonvulsants, and sedatives
have been used
13
Attention-Deficit/Hyperactivity behaviors

Methylphenidate in particular are commonly
prescribed for ID children, especially those with
mild retardation and those suffering from attentiondeficit/hyperactivity
disorders
or
exhibiting
symptoms of disruptive and impulsive behaviors
and conduct disorders
14
Attention-Deficit/Hyperactivity behaviors


Stimulant medications are also prescribed for
children with autism and pervasive developmental
disorder who often exhibit the characteristic
symptoms of ADHD
The doses of methylphenidate usually vary between
0.3 and 1 mg/kg/day. The recommended dose
of methylphenidate is approximately 0.6 mg/kg/day
15
Attention-Deficit/Hyperactivity behaviors

Children with more severe
more biological disorders
nervous system (CNS) are
responding negatively and
side effects
retardation and
of the central
more at risk of
of having more
16
Stimulants-side effects


In addition to the well-known side effects of psycho
stimulants, there is evidence that ID children may
react to these with restlessness and anxiety and
may develop a psychotic state
In severely retarded children, an increase in
stereotyped behaviors has also been observed
Increase of self-injury has also been found
17
Attention-Deficit/Hyperactivity behaviors

Risperidone was found to be highly
beneficial in reducing symptoms of ADHD in
children and adolescents with moderate
Intellectual disability
18
Stereotypical Motor Movements

Antipsychotic medications, such as Risperidone,
haloperidol and chlorpromazine, decrease repetitive
self-stimulatory behaviors in intellectually disabled
children
19
Obsessive-Compulsive Symptoms

Serotonin Reuptake Inhibitors, such as
fluoxetine, fluvoxamine (Luvox), paroxetine, and
sertraline, have been shown to have efficacy in
treating obsessive-compulsive symptoms
20
Explosive Rage behaviors

Aggression

New admissions at 42% and readmissions 45%

Self-Injurious behaviors

up to 24%
21
Risperidone



All studies, however, showed a positive clinical
response in 60–70% of the cases
Eight of 29 side effects were significant: increased
weight gain, increased appetite, drowsiness,
tremor, being ‘‘too quiet’’ or ‘‘not themselves,’’ lack
of spontaneity, and nasal congestion
Weight gain was a serious side effect (ranging from
12 kg to 9.6 kg). It occurred in 70% of individuals
22


Relatively little psychotropic medication research
has been done in children/adolescents with ID
Studies are predominately open trials, case reports
or controlled trials with small samples. Risperidone
is an exception
23
Risperidone






Prolactin levels were also elevated while on
risperidone
Extra-pyramidal side effects were very low and few
serious extra-pyramidal symptoms were observed
Effects on cognitive performance at low doses
appeared to be slight
The overall severity of these side effects was
moderate and manageable
In 2006, the Food and Drug Administration
approved the use of risperidone for Autism
24
Prader-Willi Syndrome



Aggression-Risperidone has been used successfully;
however, PWS patients are already prone to weight
gain. The SGAs with low weight-gain potential
(ziprasidone and possibly aripiprazole) may be
better choices but haven’t been studied
An alternative to consider would be an anti-epileptic
drug with a low potential for weight gain
(carbamazepine, oxcarbazepine, lamotrigine)
Valproic acid should probably be avoided, due to its
tendency to promote weight gain; a similar caution
exists for lithium
25
Prader-Willi Syndrome


Self Injurious behaviors
As there is a strong compulsive component to the
self-injury in these patients, treatment with an SSRI
should be considered although worsening of picking
behaviors related to SSRI should be monitored
26
Prader-Willi Syndrome



Self Injurious behaviors
If the patient fails two reasonable trials with an
SSRI,low-dose risperidone (starting at 0.5 mg/d)
may help
One study of adults with PWS demonstrated a
significant reduction in skin picking behaviors with
the use of topiramate; its use in younger patients
may be considered if other treatments are
unsuccessful
27
Fragile X Syndrome


Patients with fragile X often have high levels of
arousal and clonidine and β-blocking drugs may be
valuable in reducing these
When fragile X is associated with pervasive
developmental disorder, the drugs suggested for
the treatment of autism should be considered
28
Fragile X Syndrome


Aggression-if aggressive behaviors persist after
treating comorbid conditions, risperidone or
aripiprazole may be tried
Self Injurious behaviors-These behaviors will
usually respond to a combination of adequate
pharmacologic treatment of comorbid psychiatric
conditions (e.g. ADHD, depression, anxiety) and
behaviorsal interventions
29
Tuberous Sclerosis Complex (TSC)


Aggression-Much of the aggressive behaviors in
TSC is probably related to the seizure disorder;
therefore the first step in management should be
maximizing the AED regimen. However, the
epilepsy in TSC tends to respond poorly to the
AEDs
Risperidone has shown efficacy in reducing
problematic behaviors (aggression) in TSC
30
Tuberous Sclerosis Complex

The use of any medication that may affect cardiac
conduction should be delayed until the TSC patient
has had a thorough cardiac evaluation. About ½ of
these patients will have a cardiac Rhabdomyoma,
and about 20% of those will be associated with
arrhythmias
31
Autistic Spectrum Disorders (ASD)




approximately 75% of persons affected with autism have
some degree of ID
Aggression In the absence of a clearly defined mood disorder,
risperidone is the agent of choice to treat aggression in
children with autistic disorder, and perhaps with other ASD
In smaller studies Ziprasidone and aripiprazole also
demonstrated reductions in aggressive symptoms and
Olanzapine was ineffective Quetiapine increased aggression
Aripiprazole: FDA-approved for “irritability” in children and
adolescents with autism
32
Autistic Spectrum Disorders


Self-Injurious behaviors
In the event that the SIB in autistic patients doesn’t
respond to the AAs, or the medications are not
tolerated, a trial of the opioid antagonist naltrexone
may be beneficial
33
Autistic Spectrum Disorders




Self-Injurious behaviors
Risperidone has been found to reduce SIB in people
with autistic disorder; the other AAs may also be
helpful in this regard
Risperidone limitations : Although SIB frequency is
reduced in autistic patients, duration and severity of
SIB may not be significantly altered
Another concern is AA-related weight gain and
obesity, which is a risk factor for the development
of the metabolic syndrome
34
Aggression and Self-Injurious behaviors


Lithium-Some evidence from controlled and
uncontrolled studies indicates that lithium has been
useful in decreasing aggression and self-injurious
behaviors
a small number of case reports suggest that lithium
is effective for treating aggressive behaviors and
self-injurious behaviors
35
Aggression and Self-Injurious behaviors

Narcotic antagonists such as Naltrexone have
not been systematically shown to diminish
aggression or self-injurious behaviors
36
Aggression and Self-Injurious behaviors

Anticonvulsants,
such
as
carbamazepine
(Tegretol) and valproic acid (Depakene), have been
used clinically for aggressive behaviors in children
and adolescents
37
Antidepressants


Selective serotonin reuptake inhibitors are
increasingly being used with persons with
intellectually disabled children
Antidepressants are usually prescribed in ID
children for depression, anxiety disorders, ADHD,
and obsessive-compulsive disorder
38
Antidepressant Drugs

Antidepressants are used less frequently for people
with developmental disabilities, five times less
frequently than antipsychotics perhaps because of
the difficulties in diagnosing depression in this
population
39
40
41
42
43
References
1.
Psychotherapeutic Medication Treatment guidelines, University of
Florida, www.medicaidmentalhealth.org
2.
Sadock, Benjamin James; Sadock, Virginia Alcott, 'Kaplan &
Sadock's Synopsis of Psychiatry: Behavioral Sciences/Clinical
Psychiatry, 10th Edition'
Martin, Andres; Volkmar, Fred R., 'Lewis's Child and Adolescent
Psychiatry: A Comprehensive Textbook, 4th Edition'
3.
4.
5.
6.
Patterson, Marc C. “Neurogenetic developmental disorders :
variation of manifestation in childhood.“, 2007
Johannes Rojahn, Stephen Schroeder, Theodore Hoch, ‘’
Self-Injurious Behavior in Intellectual Disabilities, 2007
Robert L. Findling, ‘Clinical manual of child and adolescent
psychopharmacology’, 2008
44
Thank you
45