Article

Onset of Catatonia at Puberty Electroconvulsive Therapy Response in Two Autistic Adolescents

Department of Psychiatry, University of Michigan, Ann Arbor, MI 48109-5734, USA.
The journal of ECT (Impact Factor: 1.39). 12/2010; 26(4):274-7. DOI: 10.1097/YCT.0b013e3181de332e
Source: PubMed

ABSTRACT

Catatonia is a syndrome of motor and behavioral disturbance. It is a poorly understood condition, which is underrecognized and may go untreated despite intensive medical workup and numerous unsuccessful medication trials. However, with treatments known to be effective, such as benzodiazepines and/or electroconvulsive therapy, patients may return to their baseline functioning. Autism and catatonia have been previously reported together. We report 2 patients with autism and mental retardation who developed catatonic symptoms at the onset of puberty. Both patients experienced persistent symptoms over several years and presented with a history of motor disturbance, functional decline, and episodic aggression. Both patients were treated with electroconvulsive therapy resulting in a positive response and functional improvement. Catatonia may persist as a chronic condition, lasting over several months or years, if not recognized and treated.

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Available from: Daniel Gih, Mar 04, 2014
Onset of Catatonia at Puberty
Electroconvulsive Therapy Response in Two Autistic Adolescents
Neera Ghaziuddin, MD, Daniel Gih, MD, Virginia Barbosa, MD, Daniel F. Maixner, MD,
and Mohammad Ghaziuddin, MD
Abstract: Catatonia is a syndrome of motor and behavioral dis-
turbance. It is a poorly understood condition, which is underrecognized
and may go untreated despite intensive medical workup and numerous
unsuccessful medication trials. However, with treatments known to be
effective, such as benzodiazepines and/or electroconvulsive therapy,
patients may return to their baseline functioning. Autism and catatonia
have been previously reported together. We report 2 patients with autism
and mental retardation who developed catatonic symptoms at the onset of
puberty. Both patients experienced persistent symptoms over several years
and presented with a history of motor disturbance, functional decline, and
episodic aggression. Both patients were treated with electroconvulsive
therapy resulting in a positive response and functional improvement.
Catatonia may persist as a chronic condition, lasting over several months
or years, if not recognized and treated.
Key Words: catatonia, autism, ECT
(J ECT 2010;26: 274Y277)
C
atatonia is a poorly understood but debilitating condition
characterized by motor, behavioral, and autonomic symp-
toms. It is described in the Diagnostic and Statistical Manual
of Mental Disorders, Fourth Edition (DSM-IV ) in association
with 3 different diagnoses: general medical conditions, schizo-
phrenia, and mood disorders. Under each category, catatonia is
described primarily as a psychomotor disturbance that may in-
clude increased motor disturbance (usually purposeless) and/or
reduced motor activity (immobility, negativism, waxy flexibility,
stupor in extreme cases), disturbance of voluntary movements
(bizarre or inappropriate postures, repetitive or stereotyped move-
ments, prominent grimacing), and echophenomena (mimicking
the movements or words of other people). Potential consequences
include exhaustion, self-inflicted injury, dehydration, malnutri-
tion, and, occasionally, death.
Usually associated with affective or psychotic symptoms,
catatonia is more common than generally believed. A recent cross-
cultural study using standardized criteria found a frequency of
13.6% in India and 9.6% in Wales among psychiatrically hos-
pitalized adults.
1
Overall, there is increased awareness of this
disorder, the associated risks, and importance of using established
treatments.
2
Catatonia can also occur in the setting of autism spectru m
disorders. Wing and Shah
3
reported the first systematic study of
catatonic symptoms in a larger sample of 506 children with
autism spectrum disorders, who were referred to a tertiary re-
ferral center in the UK.
4
The authors found that 17% of the
sample, ranging in age from 15 to 19 years, displayed what the
authors described as Bsevere exacerbation of catatonic features.[
The diagnosis of catatonia was given if there was deterioration
of movement resulting in interference with daily functioning,
increased slowness affecting movements and action, difficulty in
initiating and completing actions, increased reliance on physical
or verbal prompting, and increased passivity and apparent lack
of motivation. Other authors have pointed out the similarities
between catatonia and autism spectrum disorders.
5
Symptoms
common to both autism and catatonia include communication
difficulties, stereotyped movements, mannerisms, and agitation.
Some studies have suggested that 1 in 7 autistic patients may
have catatonia and that the diagnosis should be considered in
any autistic adolescent or adult presenting with significant func-
tional impairment.
6
Both misdiagnosis and missed diagnosis of catatonia have
been previously reported. For instance, a case was misdiagnosed
for a neurological disorder until catatonia was eventually diag-
nosed 15 years later.
7
That catatonia may persist over months
or years as a chronic condition was previously described by
Kocmur and Vodopivec
7
and Gaind et al
8
and also observed anec-
dotally by clinicians who treat patients with autistic disorders.
We report 2 adolescents with autism (18 and 16 years at
presentation) whose symptoms started around puberty and per-
sisted for 5 and 3 years, respectively. The earliest symptoms in
each case were motor disturbance associated with a significant
change in baseline functioning and increased aggression. Both
patients were admitted to our institution for a comprehensive
workup, which was followed by treatment with electroconvul-
sive therapy (ECT). Fictitious identifiers were used to protect
patient confidentiality in accordance with institutional review
board guidelines. The MECTA device (MECTA Corporation,
Tualatin, Oreg) was used for treating both patients.
CASE REPORTS
Case 1
LN is an 18-year-old man with diagnoses of congenital
sensorineural deafness, mild mental retardation (MR), autism,
and reported history of catatonia. His parents presented him for
possible treatment with ECT, given the persistent loss in func-
tioning despite intensive treatment for the past 5 years. The
family approached our institution because LN was from a state
where restrictive ECT regulations apply.
Birth history was unremarkable. Profound bilateral hearing
loss was noted early. LN had delayed developmental milestones,
reduced interpersonal relatedness, and preference for parallel
CASE REPORT
274 www.ectjournal.com Journal of ECT
&
Volume 26, Number 4, December 2010
From the Department of Psychiatry, University of Michigan, Ann Arbor, MI.
Received for publication November 2, 2009; accepted March 9, 2010.
Reprints: Neera Ghaziuddin, MD, Rachel Upjohn Bldg, 4250 Plymouth Rd,
Ann Arbor, MI 48109-5734 (e-mail: neerag@umich.edu).
Dr Daniel F. Maixner, MD, received funding from the Flinn Foundation.
There are no other financial disclosures.
Virginia Barbosa, MD, was completing a fellowship at the time of
manuscript preparation. She is presently connected with the Cumberland
Behavioral Health Center in Lexington, KY.
Copyright * 2010 by Lippincott Williams & Wilkins
DOI: 10.1097/YCT.0b013e3181de332e
Copyright @ 20 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.10
Page 1
play as a toddler. The diagnosis of pervasive developmental
disorder was given in his native country before his arrival to the
United States. LN has no family history of mental retardation or
other psychiatric disorders.
LN was a star student in special education and a Special
Olympics athlete. At age 12 years, LN had severe decline in
functioning (lost the ability to feed himself and use sign
language) and marked motor slowing. Home videos corrobo-
rated the family’s account. Soon after, he was briefly hospitalized
for severe dehydration and weight loss, which required tube
feeding. He was treated with haloperidol, fluoxetine, and ben-
zodiazepines, and his condition was diagnosed as catatonia.
However, benzodiazepines were discontinued after a brief trial
for unknown reasons. At discharge, LN continued to display
severe psychomotor retardation and required assistance with
activities of daily living (ADLs). From age 17 to 18, he was
treated at a specialized psychiatric facility for the deaf but
failed to return to his previous baseline functioning. He started
to engage in unpredictable episodic aggression, which required
his father to physically restrain him. These behaviors were
prominent at presentation to our institution. LN was evaluated
in the psychiatric emergency service and subsequently hospital-
ized for an evaluation, including the appropriateness of ECT.
Past psychotropic medication trials included neuroleptics
to treat aggressive behavior; however, there was worsening in
psychomotor retardation. His parents noted that LN did better
when treated with relatively larger doses of benzodiazepines.
Medical workup was unremarkable and included magnetic res-
onance imaging of the brain; 2 electroencephalograms (EEG),
which did not reveal seizure activity; karyotype and fragile X
testing; metabolic panels for serum lactic acid, amino acids,
acylcarnitine, copper, ceruloplasmin, thyroid-stimulating hor-
mone, and urine mucopolysaccharides.
On admission, the patient was on a daily regimen of
40 mg of fluoxetine, 1.5 mg of lorazepam, 750 mg valproic acid,
and 3 mg of benztropine. He needed assistance with ADLs,
displayed periods of psychomotor retardation, unexplained ex-
citement (bolting out of his room for no apparent reason), pur-
poseless hand waving, unresponsiveness while posturing with
rotation of the upper extremities, which were maintained for
a minute or more, and frequent episodes of aggression requir-
ing restraints and injectable antipsychotic agents.
The patient’s condition was diagnosed as catatonia based
on prominent motor disturbance (psychomotor slowing and
posturing), episodic excitement, history of stupor, and stereo-
typed movements. LN was initially treated with high doses of
lorazepam, 10 mg or more a day for 9 days, whereas fluoxetine
and valproic acid were gradually discontinued. Trazodone was
added to reduce aggression during the day. However, he did not
respond to these treatments and continued to manifest multi-
ple daily aggressive outbursts. Two psychiatrists independently
evaluated the patient and reached a consensus that LN may
benefit from ECT. Based on the state mental health laws, LN’s
father was awarded guardianship, and he gave a signed consent
for treatment. Standard ECT guidelines for adults with mental
retardation were used.
LN received 12 bilateral ECT sessions (mean motor sei-
zure, 28 seconds; EEG seizure, 59 seconds; charge, 349 milli-
coulombs [mC]; pulse width, 0.6 milliseconds) The first signs
of improvement were reduced stereotyped movements, cessa-
tion of posturing, followed by fewer aggressive episodes and
reduced need for supervision. Behavioral problems were con-
trolled without antipsychotic medications after treatment num-
ber 6. There were no further aggressive outbursts at the time of
discharge, his bladder control improved from near incontinence
at admission, he became more independent with his ADLs
(able to shower and dress with assistance, and was able to feed
himself ). His handwriting became more legible. He was able to
communicate using the American Sign Language, which was
somewhat bizarre and stereotyped before ECT. He was dis-
charged to his parents with recommendation that LN was likely
to benefit from maintenance ECT. However, the family verbal-
ized that they anticipated problems with arranging treatment
because of restrictive laws in their home state.
The family contacted us 4 weeks after discharge to report
that the patient was doing better than before he received ECT
but that some symptoms had recurred including withdrawal
(staying in bed for several hours) and episodic excitement
(shouting without reason, and head banging). He did retain the
ability to shower independently. The parents added that they
were trying to arrange for maintenance ECT but had not been
successful up to that point.
Case 2
CE is a 16-year-old boy with moderate mental retardation,
autism, and 3 years of worsening physical aggression. Three
months before his presentation to us, aggressive outbursts had
resulted in psychiatric hospitalization at another facility where
a diagnosis of psychosis not otherwise specified was made. He
did not improve despite adequate trials of psychotropic med-
ications and behavior therapy. He was admitted to our institution
for continued aggressive and self-injurious behavior.
Antenatal history was positive for 3 viral infections
during the mother’s pregnancy. Development was normal until
20 months when speech and communication declined. Genetic
testing revealed a normal chromosomal array and no fragile X.
Autism was diagnosed by age 5 years. Family history was neg-
ative for mental retardation, developmental delay, or metabolic
disorders. At baseline, CE could feed, shower, and dress with
assistance. He had excellent rote memory and enjoyed spelling
despite little ability to form sentences. Psychotropic medications
were not used until age 13 years.
At presentation aggression would occur without any
precipitants, lasted a few minutes, and was accompanied by
yelling, kicking, biting, breaking things, and ripping clothes.
Before the onset of aggressive outbursts, the mother recalled
that CE would appear frozen in motion, crouching in a door-
way or standing on one leg for an hour or more. Spelling
difficulty and ritualistic behaviors (needing someone to touch
his arm while eating, and walking on tiles in a certain way)
were also noted. Unexplained regurgitation of food began ap-
proximately the same time as the motor symptoms. Extensive
gastroenterology workup (stool for ova and cysts, abdominal
computed tomographic scan, gastric emptying study, and cystic
fibrosis panel) was completed without positive findings. A
restricted diet based on the recommendation of an allergist was
tried but did not result in any changes in his behaviors.
Neuroimaging (magnetic resonance imaging of the brain
and computed axial tomography) were unremarkable. Ambu-
latory and video EEG recordings (at ages 5, 13, and 16 years)
showed nonspecific theta and delta activity suggestive of en-
cephalopathy but no ictal activity. Aggressive outbursts were
captured on video EEG recordings, but no epileptiform dis-
charges or seizures were recorded. Lactate was marginally low
but not diagnostic; carnitine profile, pyruvate, and urine organic
acids were normal. Serum amino acids were low, suggestive of
a protein-restricted diet. Autoimmune disorders were ruled out
by rheumatology. Eventually, von Willebrand diseaseVunknown
type (based on platelet studies that showed delta granule s tor-
age pool deficiency) and subclinical hypothyroidism (normal
Journal of ECT
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Volume 26, Number 4, December 2010 Catatonia at Puberty
* 2010 Lippincott Williams & Wilkins www.ectjournal.com
275
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Page 2
thyroid-stimulati ng hormone and low thyroxine) were diag-
nosed. Consultation with an endocrinologist concluded that CE
had a sick thyroid syndrome from long-standing malnutrition
owing to his overly restrictive diet. The parents were advised
that thyroid replacement be stopped and his diet should be
slowly normalized.
Past medication trials included sertra line (for ritualistic
behaviors, but resulted in increased aggression), phenytoin,
topiramate, clonazepam, and gabapentin (for possible seizure
disorder), risperidone and quetiapine (resulted in increased
aggression), lorazepam (equivocal results), and guanfacine, meth-
ylphenidate, lithium, memantine, and nortriptyline (no change).
The mother believed that most medications were ineffective or
resulted in worsening of aggression.
Mental status examination revealed a boy who appeared
younger than his given age, absent eye contact, and persever-
ative speech. Brief periods of remaining motionlessness (main-
taining a posture for up to an hour) and staring followed by
aggression toward self or others were observed. At times, he
would hurtle himself against the wall, bite his father, speak un-
intelligibly, and display minimal social interactions with others.
Speech was limited, but there was no evidence of delusions or
hallucinations.
Catatonia was diagnosed based on episodic immobility
and periods of frantic excitement. Bilateral ECT was started
to target catatonia and aggression. American Academy of Child
and Adolescent Psychiatry (2004) guidelines, recommended for
the administration of ECT in minors, were followed
9
; these in-
cluded consensus opinion by 3 child and adolescent psychia-
trists, consultation with an anesthesiologist, and parental
informed consent.
The patient tolerated ECT well. He made remarkable
improvement (with no posturing, minimal agitation, and ag-
gression) by the sixth treatment. At the time of this report, CE
had received 55 ECT treatments over 14 months (mean motor
seizure, 37 seconds; EEG seizure, 64 seconds; charge, 261 mC;
pulse width, 0.6 milliseconds). Index course consisted of 29
treatments (3 treatments per week) followed by maintenance
treatment (a single treatment every 2 to 3 weeks). At the time
of writing this report, the patient was receiving 15 mg of loraz-
epam per day and continued to receive ECT, which is adjusted
in its frequency, whenever he displays increased aggression. The
overall plan for ECT is to lengthen the time interval between
treatments without precipitating return of aggression. Figure 1
illustrates the association between treatment frequency and
the number of aggressive incidents during a 40-week period of
maintenance ECT.
CE has a behavior plan for home and school. He con-
tinues to live at home, attends school (was excluded from school
before ECT), exhibits brief episodes of agitation but without
severe aggression. No adverse effects of ECT, including loss of
skills or intellectual functioning, have occurred. He was briefly
tried on propanalol while receiving maintenance ECT, which
was discontinued after 8 weeks because of return of severe
aggression.
DISCUSSION
We present here 2 male patients with pervasive develop-
mental disorder, who developed symptoms of catatonia in early
adolescence and remained severely impaired for several years.
Features common to both cases are aggression as the presenting
symptom; early motor symptoms, which were not recognized by
parents or physicians; symptom onset at puberty; significant
decline in functioning; failure to respond to multiple medica-
tion trials; and deterioration in response to most pharmacolog-
ical agents, particularly neuroleptics. Both patients functioned
relatively well until the onset of their catatonic illness.
Although the exact cause of catatonia in autism remains
uncertain, it has been linked to abnormalities of F-aminobutyric
acid metabolism postulated to occur in both catatonia and
autism.
5
Benzodiazepines and ECT are effective treatments.
However, legislation in several states in the US prohibits the use
of ECT in children, thus limiting their access to this type of
FIGURE 1. Association between treatment frequency of ECT and aggressive incidents during a 40-week maintenance treatment period
in a 16-year-old boy with pervasive developmental disorder and catatonia.
Ghaziuddin et al Journal of ECT
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Volume 26, Number 4, December 2010
276 www.ectjournal.com * 2010 Lippincott Williams & Wilkins
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Page 3
intervention. The American Academy of Child and Adoles-
cent Psychiatry guidelines recommending a consensus of 3
child psychiatrists
9
may also be a barrier for early access to ECT
in youth, as many child and adolescent psychiatrists are un-
familiar with the use of ECT,
10
and there is little or no consen-
sus on the use of ECT in children and adolescents.
Several clinical points are noteworthy in the cases de-
scribed. For both cases, the early symptoms of catatonia were
only elicited after obtaining a careful histor y from the parents.
It was apparent that parents and physicians had failed to appre-
ciate the significance of early motor disturbance (marked motor
slowing in LN, alternating episodes of increased and reduced
activity in CE, and sustained posturing in both patients). Despite
the past diagnosis of catatonia given to LN, he did not receive
the standard treatment for this condition. Both patients improved
after cessation of medications and treatment with ECT. These
cases are similar in their presentation to 2 brothers with mental
retardation who were described by Gaind et al,
8
one of who was
catatonic for 5 years before he was successfully treated with
lorazepam.
This report underscores the importance of correct diagno-
sis and treatment of catatonia, even among patients with symp-
toms that have persisted for months or years. Onset at puberty
is noteworthy, which leads us to hypothesize that it may be a
period of elevated risk for the condition. Physicians should be
aware that a protracted treatment course (including treatment
with ECT) may be necessary in some cases. Early treatment
may prevent years of suffering, and there is little evidence to
suggest that antidepressants, mood stabilizers, antiepileptics, or
neuroleptic agents offer any benefit while catatonic symptoms
are evident.
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[see comment]. Br J Psychiatry. 2000;176:357Y362.
4. Wing L, Shah A. A systematic examination of catatonia-like
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9. Ghaziuddin N, Kutcher SP, Knapp P, et al. Practice parameter for use
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Journal of ECT
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Volume 26, Number 4, December 2010 Catatonia at Puberty
* 2010 Lippincott Williams & Wilkins www.ectjournal.com
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Page 4
    • "We carried out a search in PubMed and could locate 10 reports[6789101112131415] of use of ECT in adolescents with catatonia published after the review of Consoli et al.[4] Eight out of the 10 reports, described 11 cases in which ECT was used in adolescents for treatment of catatonia in patients with varied clinical conditions [Table 2].[678910111213] One case series described the use of maintenance ECT in three children with autism, in which ECT had led to resolution of acute symptoms of catatonia.[14] "
    [Show abstract] [Hide abstract] ABSTRACT: There is lot of skepticism about the use of electroconvulsive therapy (ECT) in children and adolescents. However, available literature suggests that use of ECT can be at times life-saving in adolescents, especially those presenting with severe catatonia. We treated a 16-year-old female who presented to us with catatonia with a course of nine ECTs, with which she showed marked improvement. Review of the literature suggests that ECT should be considered as the second line treatment in the management of catatonia in adolescents.
    No preview · Article · Apr 2014 · Indian Journal of Psychological Medicine
  • No preview · Article · Dec 2010 · The journal of ECT
  • [Show abstract] [Hide abstract] ABSTRACT: Autism spectrum disorders (ASD), labeled as pervasive developmental disorders (PDD) in the DSM-IV-TR (APA, 2000), are characterized by a distinct pattern of social and communication deficits with rigid ritualistic interests. While autism (Kanner, 1943) is the main category, other conditions include Asperger syndrome (Asperger, 1944) and pervasive developmental disorder not otherwise specified (PDDNOS). Rett disorder and childhood disintegrative disorder, although classified in the same category, are often not referred to as ASDs. As discussed elsewhere in this volume, the DSM-V is likely to collapse autism, Asperger syndrome, and PDDNOS into a single category called ASD.
    No preview · Chapter · Dec 2010
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