Diagnosis and Treatment of Catatonia

Transcription

Diagnosis and Treatment of Catatonia
Diagnosis and Treatment of Catatonia
Priya Gopalan, MD
Pierre N. Azzam, MD
Agenda
• Overview of Catatonia
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Typical Features of Catatonia
Historical Evolution of the Diagnosis
Diagnosis from DSM-IV to DSM-5
Epidemiology
Clinical Evaluation of Catatonia
• Prototypes of Catatonia
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Distant Mute
Waxy Stiff
Stubborn Grouch
Broken Record
Agenda
• Catatonia and its Underpinnings
– Differential Diagnosis
– Precipitating Conditions
– Pathophysiologic Theories
• Management of Catatonia
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Essential of Management
Mainstays of Therapy
Benzodiazepines in Challenge and Maintenance Therapy
Electroconvulsive Therapy (ECT) and Alternate Options
Management and Prevention of Complications
• Questions
Overview of Catatonia
Pierre N. Azzam, MD
Clinical Case #1: Pt. S.
• Demographic:
– 42y/o single white man
• Medical History:
– diabetes, HTN, strokes (R cerebellar, R temporal, R basal ganglia)
• Psychiatric History:
– schizophrenia, undifferentiated type
• Clinical Presentation:
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admitted with infectious eneterocolitis
multi-system organ failure  intubation
upon extubation, quetiapine 400mg po QHS
altered mental status and QT prolongation on EKG
Clinical Case #1: Pt. S.
• Mental Status:
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lying in bed, eyes closed, motionless
resisted opening his eyelids, then kept them shut
no spontaneous speech
no rigidity, catalepsy, waxy flexibility
• Evaluation/Management:
– initial primary diagnosis: multifactorial delirium
– EEG: slowing, MRI: multiple infarcts, age undetermined
– recommendations: PRN olanzapine (no improvement)
Clinical Case #1: Pt. S.
• Follow-up Visit:
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stupor, intermittent alertness
mutism, intermittent repetition: “I’m scared”
mild rigidity and waxy flexibility
purposeless blanket-bunching
frequent head movements (side-to-side)
• Management:
– lorazepam 1.5 mg trial
– perked up and requested to listen to Nat King Cole
– lorazepam 0.5 mg TID started as maintenance therapy
Clinical Case #1: Pt. S.
“I felt like I was stuck between
two planes in space… Like there
were pins in me everywhere
holding me in place… I
remember you asking questions,
and I knew some answers but
couldn’t say… I was scared.”
Catatonia at a Glance
• Syndrome (not a disease)
• Motor and behavioral
disturbances
• Various medical and
psychiatric precipitants
• Fluctuating time course
• Most common features:
– Mutism
– Interpersonal withdrawal
– Negativism (resistance to
instruction)
– Stupor/immobility
– Staring
– Catalepsy (posturing)
– Rigidity
– Stereotypy (repetitive behaviors)
– Mannerisms (odd behaviors)
– Excitement/combativeness
– Echophenomena
– Ambitendency (“stuck”)
History of Catatonia: Kahlbaum
(Fink 2003)
• Coined the term catatonia
• Stages of the condition:
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Melancholia, apathy
Mania, hyperactivity
Stupor, confusion
Dementia
• Credited with unifying
motoric and behavioral
features, outlining temporal
course of catatonia.
Karl Ludwig Kahlbaum. Psychiatrische Wochenschrift. Public domain image.
Wikimedia Commons
History of Catatonia: Taking Root in Schizophrenia
•
Late 1800s: Emil Kraepelin
folded catatonia into
dementia praecox,
conceptualized catatonia as a
more chronic disordered
state.
•
(Fink 2003)
Early 1900s: Eugen Bleuler
included catatonia as a
(marginal) feature of
schizophrenia, focused on
psychodynamic theories.
Emil Kraepelin. Credit: Wellcome Library, London. Used with Permission. Wikimedia Commons
E. Bleuler. Beilage sur Munchener medizinischen Wochenschrift. Public domain image. Wikimedia Commons
History of Catatonia: Modern Progression
• 1950s – 1970s:
– Catatonia identified in patients
with mania, depression, medical
and neurologic conditions
– Up to 10% of psychiatric
inpatients
– “Lethal catatonia” identified,
along with neuroleptic malignant
syndrome (NMS)
• 1980s – 2010s:
– Catatonia first treated with
lorazepam
– DSM III as subtype of
schizophrenia
– DSM IV as subtype of
schizophrenia and specifier to
depression or mania
– DSM-5: Catatonia as a separate
syndromal entity
Catatonia and the DSM IV-TR
• Classifications
– Schizophrenia (subtype)
– Depressive, manic, mixed
episodes (specifier)
– Catatonia secondary to
general medical condition
(stand-alone diagnosis)
(APA 2000)
• Diagnostic Criteria (2+)
– Motoric immobility
– Excessive motor activity
– Extreme mutism or
negativism
– Peculiar voluntary
movements
– Echolalia or echopraxia
Catatonia and the DSM-5
(APA 2013)
• Classifications
– Catatonia associated with
another mental disorder
(specifier)
– Catatonic disorder due to
another medical condition
– Unspecified catatonia
• Distressing or functionally
impairing symptoms
• Contributing condition
unclear or full criteria not
met
• Diagnostic Criteria (3+)
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Stupor
Catalepsy
Waxy Flexibility
Mutism
Negativism
Posturing
Mannerism
Stereotypy
Agitation
Grimacing
Echolalia
Echopraxia
Epidemiology
• General Hospital Setting
– Prevalence: Unknown
– Believed to be under-recognized and -treated
• Inpatient Psychiatric Setting
– Prevalence: 7-20%
– Due to general medical condition: 20-25%
• Sex: 1.1–1.3 : 1 (female : male)
• Age: affects all ages, rare in childhood
Bush-Francis Catatonia Rating Scale
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2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
Excitement
Immobility/Stupor
Mutism
Staring
Posturing/Catalepsy
Grimacing
Echophenomena
Stereotypy
Mannerisms
Verbigeration
Rigidity
Negativism
Waxy Flexibility
Withdrawal
15.
16.
17.
18.
19.
20.
21.
22.
23.
(Bush 1997)
Impulsivity
Automatic Obedience
Mitgehen
Gegenhalten
Ambitendency
Grasp Reflex
Perseveration
Combativeness
Autonomic Abnormality
•
Screening: First 14 items (2+
positive)
•
Monitoring: All 23 items (each
measured for serial tracking)
Clinical Evaluation
(Fricchione 2011)
• Observe patient engaging in conversation
Look for: mutism, echolalia, verbigeration
• Scratch your head in exaggerated manner
Look for: echopraxia
• Check chart for 24-hour oral intake, vital signs, behaviors
Look for: autonomic instability, withdrawal
• Observe directly and indirectly at different times of the day
Look for: fluctuations in clinical presentation
Clinical Evaluation
(Fricchione 2011)
• Extend hand: “Do NOT shake my hand”
Look for: ambitendence
• Reach in pocket, and say: “Show me your tongue. I want to stick
a pin in it.”
Look for: automatic obedience
• Check for grasp reflex
Look for: frontal release signs
• Examine arms for tone
Look for: rigidity, waxy flexibility, gegenhalten
Prototypes of Catatonia
Priya Gopalan, MD
Clinical Case #2: Pt. C.
• Demographic:
– 55 y/o widowed African American female
• Medical History:
– Breast cancer
• Psychiatric History:
– Remote episode postpartum depression
• Present Illness:
– Begins course of chemotherapy, receives one dose of intramuscular
(IM) steroid, dexamethasone
– Three days later, family notes that Ms. C is pacing around the house,
“fidgety,” talking to herself, and seeing people that are not present
Clinical Case #2: Pt. C.
• In the ED:
– Physical exams by emergency medicine and neurology note
audiovisual hallucinations, loose associations, and echolalia, but no
other abnormal findings
– Ms. C is admitted to the general medical floor
• During Admission:
– Primary team withholds chemotherapy and starts antipsychotics for
delirium
– She receives 10mg PO olanzapine and 7.5mg IV haloperidol
– Medical work up, including: basic labs, CPK, UDS, MRI, and EEG
reveal no abnormalities
Clinical Case #2: Pt. C.
• Psychiatry is consulted for evaluation of “altered mental
status”
• Family confirms lack of prior psychiatric symptoms or
substance abuse
• Ms. C is observed watching TV in her room, repeating the
words on commercials
• She is minimally interactive with the examiner and mumbles
nonsensically throughout the exam.
Clinical Case #2: Pt. C.
• No fevers, diaphoresis, or flushing
• Rigidity noted in upper extremities, but no cog-wheeling
• Catalepsy in upper extremities
• No echopraxia, automatic obedience, or ambitendency
• Positive (abnormal) grasp, glabellar, and snout reflexes
• Normal deep tendon reflexes
• No clonus or tremors
Clinical Case #2: Pt. C.
• Would you call this catatonia?
• What features of catatonia does this person exhibit?
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Echo-phenomena
Verbigeration
Rigidity
Catalepsy
Grasp reflex
• What factors predispose this patient to catatonia?
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History of a mood disorder
Medical illness
Steroid exposure
Antipsychotic exposure
Clinical Case #2: Pt. C.
• Ms. C is diagnosed with catatonia, likely due to steroids
• The psychiatry consultation service recommends stopping
all anti-dopaminergic agents
• Ms. C is given 1mg IV lorazepam x 1 and re-examined one
hour later.
• At that time, she responds to questions, but is confused and
gives inappropriate answers
Clinical Case #2: Pt. C.
• Psychiatry team starts lorazepam 1mg IV three times daily
(TID)
– The next day she is interactive, but still has echolalia
– She continues to have intermittent agitation and motor exam is
limited by restraints
• Lorazepam is increased to 2mg IV four times daily (QID)
– Catatonia symptoms resolve
– Hallucinations resolve
– She continues to have tangential thoughts and delusions that the
physicians are other people
Clinical Case #2: Pt. C.
• Aripiprazole is initiated for psychosis, and she is transferred
to inpatient psychiatry for further management
• Psychiatric symptoms resolve on a regimen of aripiprazole
20mg daily and lorazepam 2mg QID, and Ms. C is
discharged to home
• Aripiprazole and lorazepam are both tapered off as an
outpatient with no return of symptoms
Prototypes in Psychiatry
• Carl Jung: Archetypes
• Kahana and Bibring: Personality types
• Groves: “Hateful Patient”
• Prototype models exist for numerous psychiatric conditions
(e.g., bipolar disorder, personality disorders)
Prototype Models
• Advantages:
• Disadvantages:
– Effective
– Oversimplified
– Practical
– Risk of stereotyping
– Matches natural clinical
reasoning
– Risk of symptom exclusion
– Facilitates teaching and
pattern-guided recognition
– Associated with high falsepositive rates
Retarded Catatonia (Kahlbaum Syndrome)
• Clinical features:
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Stupor
Mutism
Rigidity
Withdrawal
Waxen Flexibility
• Typical associations:
depression, psychosis,
neurologic disease
A Woman Diagnosed as Suffering from Melancholia. Credit:
Wellcome Library, London. Adapted with Permission.
Excited Catatonia (Delirious Mania)
• Clinical features:
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Nightmarish derealization
Excessive activity/agitation
Disorganized speech
Confusion, disorientation
Stereotypy, echophenomena
• Typical associations:
mania, mixed states,
toxidromes
A Woman Diagnosed as Suffering from Mania. Credit: Wellcome
Library, London. Adapted with Permission.
Malignant Catatonia
• Malignant Catatonia
– Clinical features:
• Posturing, rigidity
• Neuroleptic Malignant
Syndrome
– Clinical features:
• Confusion
• Severe rigidity
• Intermittent excitement
• Altered mental status
• Fever, dysautonomia
– Course: sudden; rapid
progression; fluctuation
– Mortality: 10-30%
• Fever, dysautonomia
• Recent neuroleptic use
– NMS and MC clinically
indistinguishable
– Two sides of a coin?
–Vancaester 2007
The Distant Mute
•
ID: 57y/o man
•
PMH: epilepsy, on phenytoin
•
PPH: major depression,
alcohol dependence
•
Admission: seizures one week
prior to admission, followed by
dehydration and FTT
•
Presentation
– No speech
– Averted gaze; when finally made
eye contact, no blink for > 2 mins
– Refused to interact, engage in
care, eat or drink
– Did opposite of most commands
The Distant Mute
• Presentation: Abnormal
eye contact, prominent
mutism, uncommunicative
• Key Point: Mutism may be
the most frequently
observed sign in an acute
care setting
The Waxy Stiff
•
ID: 45y/o man
•
PMH: none
•
PPH: alcohol and opioid
dependence
•
Admission: found by
parents kneeling in fixed
position; three days of
comments about being the
Antichrist; two weeks of
depression
•
Presentation:
– Minimal speech. Intermittent
eye contact
– Maintenance of unusual
postures in UE, rigidity in LE
The Waxy Stiff
• Presentation: Sole
presence of motor findings
(catalepsy, waxy flexibility)
• Key Point: Classic
catatonia presentation is
not necessarily the most
common
The Stubborn Grouch
•
ID: 62y/o woman
•
PMH: DM, kidney disease, HTN
•
PPH: MDD, alcohol dependence
•
Admission: intra-abdominal
abscess after nephrectomy
•
Presentation:
– Pulled off anything placed on her
(clothes, lines, tape, blankets)
– Laying in her own urine
– “Get out! I want you out!”
– Doesn’t look at the interviewer
– Later: fists clenched, eyes shut
– Oppositional paratonia
The Stubborn Grouch
• Presentation: Prominent
negativism
• Key Points: Negativism is
often mistaken for
volitional behavior, and
may be more common in
the general medical setting
The Broken Record
•
ID: 31y/o man
•
PPH: bipolar disorder; PCP and
dextromethorphan dependence
•
Admission: punched in the face
•
Injuries: multiple facial fractures
and head injury
•
Presentation:
– Intermittently pulled at lines
– Shrugged shoulders repeatedly
– Parroted snapping fingers, hands
up, scratching head
– “I don’t know,” “That’s okay”
– Automatic obedience
The Broken Record
• Presentation: Purposeless
agitation, hyperactivity,
echophenomena
• Key Points: Challenges
traditional images of
catatonia, can be confused
with delirium
Prototypes in Catatonia: Summary
Distant Mute
Waxy Stiff
Broken Record
Stubborn Grouch
Inertia
Perseveration
Obstinance
Mutism
Catalepsy
Echophenomena
Negativism
Immobility
Waxy flexibility
Verbigeration
Interpersonal
withdrawal
Rigidity
Hyperactivity
Repetitive
movements
Overarching Disconnection
Theme
Diagnostic
Features
Challenges
in the
General
Hospital
Excitement
Reflexive
Distinguishing
misattribution to
sedation from
catatonic stupor psychopathology
Accusations of
volitional
symptom
production
Risk of medical and
surgical
complications due
to immobility
Management of
patient safety by
unit staff
Incomplete medical
evaluation due to
negativism
Misdiagnosis
leading to
neuroleptic
administration
Consideration of
medical decisionmaking capacity
with refusal of care
Catatonia and its Underpinnings
Pierre N. Azzam, MD
Differential Diagnosis
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Mutism (akinetic, elective, post-ictal)
Parkinson’s disease-related akinesia
Gilles de la Tourette’s disorder
Hyperactive delirium
Hypoactive delirium
Locked-in syndrome
Abulia
Coma
Precipitating Conditions
• Psychiatric Illness
– Affective disorders *
– Schizophrenia *
– Autistic spectrum
disorders*
– Conversion disorder
– Obsessive compulsive
disorder (OCD)
(Fricchione 1997, 2011)
• Neurologic Insults
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Epilepsy *
Encephalopathy *
Encephalitis*
Infarct (cingulate,
temporal, parietal) *
CJD
Hemorrhage
Meningitis
Traumatic Brain Injury
Precipitating Conditions
• Medical Conditions
– Acute intermittent
porphyria *
– Systemic lupus
erythematosis*
– Addison’s disease
– AIDS
– Cushing’s disease
– Hepatitis
– Hyperparathyroidism
– Tuberculosis
– Uremia
(Fricchione 1997, 2011)
• Medications/Drugs
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Corticosteroids *
Metoclopramide *
Neuroleptics *
Phencyclidine (PCP) *
Disulfiram
MDMA (ecstasy)
Stimulants
Withdrawal states:
benzodiazepines *,
dopaminergic drugs
Pathophysiologic Theories
“Similar symptomatology in neuroleptic
catatonia (including NMS) and psychogenic
catatonia may reflect a common
pathophysiology involving dopamine and
GABA neurons in mesostriatal, mesolimbic,
and hypothalamic pathways.”
– Fricchione 1997
Proposed Neural Networks
(Daniels 2009, Fricchione 2011)
• Diffuse rather than focal lesions
• Striatal-thalamocortical tracts
– Basal Ganglia
motor regulation
– Orbitofrontal Cortex
motivation; perseveration
• Other proposed involvement
– Hypothalamus
hyperthermia, dysautonomia
– Limbic System
subjective fear
– Parietal Cortex
visuospatial processing
Proposed Neurochemistry
• Dopamine HYPOactivity
(Daniels 2009)
• Glutamate HYPERactivity
– Mesolimbic
– Posterior parietal
– Nigrostriatal
– Frontal GABAA inhibition
– Hypothalamic
• GABAA HYPOactivity
– Orbitofrontal
– Limbic connections
– Striatal DA inhibition
• 5-HT and possible link to
serotonin syndrome?
Management of Catatonia
Priya Gopalan, MD
Essentials of Management
• Observe closely and monitor vital signs
• Manage precipitating conditions
• Discontinue offending medications
• Restart recently withdrawn dopaminergic agents
• Monitor for medical complications
• Involve health care proxy
Additional Workup
(Fink 2003, Daniels 2009)
• Collateral Information: history of drug use or psychiatric
illness, presentation course
• Vital Signs: ↑HR, BP, RR
• Serum Labs: ↑CPK, WBC ↓Na, Fe
• Head imaging
• EEG: no consistent findings; may wax and wane with frontal
slowing
Potential Medical Complications
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Aspiration
Burn
Cardiac failure
Dehydration
DIC
DVT/PE
GI bleed/obstruction
Pneumonia
Renal failure
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•
(Gross 2008)
Respiratory distress
Rhabdomyolysis
Seizures
Sepsis
Urinary retention
Urinary tract infection
Death: up to 25% in malignant
catatonia
Preventive Measures
• DVT precautions (anticoagulation)
• Aspiration precautions
• Hydration
• Nutrition (parenteral)
• Stretching (PT/OT)
• Wound Care
• Mobilization
Mainstays of Catatonia Treatment
• Barbiturates: historical first-line treatment
• Benzodiazepines: current first-line treatment
• Dopamine Agonists: adjunctive agents
– Amantadine: pre-synaptic DA agonist
– Bromocriptine: post-synaptic DA agonist
• Dantrolene: used in cases of MC (rigidity)
• Electroconvulsive Therapy: treatment in BZD nonresponsive patients, malignant catatonia
Benzodiazepines in Catatonia
• BZD Challenge
– Lorazepam 1-2mg IV x 1, monitor response
– Lorazepam 1mg IV Q30min until response (up to 4-6mg)
• BZD Maintenance
– Response to challenge predicts response to maintenance treatment
(~90%)
– Dosing based on response to challenge
– Typical start dose: lorazepam 1-2mg po/IV TID
– Daily adjustment based on response
ECT in the Treatment of Catatonia
• If inadequate response within
5 days of med trial (sooner if
malignant catatonia), seek
ECT or alternative treatment
• Prospective study (n=28): 4
patients who did not improve
with benzodiazepines after 5
days improved with ECT
treatment (Bush 1996)
• Case series: delay in ECT for
malignant catatonia, by 5
days  death for 14 patients;
versus 16 of 19 patients who
survived when they received
ECT within 5 days of
diagnosis (Philbrick 1994)
• Pennsylvania: Court order for
ECT when patient is unable to
consent
Case-Reported Treatments
• Antiepileptic Drugs
– Carbamazepine
– Topiramate
– Valproic Acid
• Atypical antipsychotics
– Aripiprazole
– Olanzapine
(Babington 2007; Bastiampillai 2008; Bowers
2007; Carroll 2007; Chang 2009; Munoz 2008;
Prowler 2010)
• Levodopa
• Lithium
• Methylphenidate
• NMDA-antagonists
– Memantine
– Amantadine
• Zolpidem
Misconceptions in the Medical Setting
• Catatonia must be “psychiatric”
Catatonia presents in the setting of medical and psychiatric conditions
(additive effects?).
• “The patient is just playing possum”
Many behaviors are not ones people typically choose when they are
being “problematic.”
• Unresponsive = Asleep
BZDs remain the treatment of choice.
• Response to Ativan proves it’s psychiatric
BZDs treat catatonia, often not the precipitating condition.
Treatment Outcomes
(Hawkins 1995)
• Non-Malignant Catatonia
• Malignant Catatonia
– BZD response: 70%
– BZD response: 40%
– ECT response: 85%
– ECT response: 89%
– AP response: 7.5%
– AP response: 0%
When to Maintain Treatment
• Less Likely to Relapse
– Rapid, dramatic response
to BZD
– Episodic course
– High inter-episode
functioning
– Primary mood disorder
– If ECT: adequate seizures,
easily induced
(Daniels 2009; Carroll 2004)
• More Likely to Relapse
– High-dose BZD required
– Coarse neurologic disease
– Oneiroid state
– Chronic mania
– Alcohol dependence
– Substance-induced mood
disorder with catatonia
Summary
• Catatonia is a heterogeneous syndrome of motor and behavioral
disturbances
• Various neurologic, medical, psychiatric etiologies (affective
disorders, general medical conditions, psychotic disorders)
• Pathophysiology related to DA and GABA depletion
• Rapid identification, supportive measures, somatic treatments are
life-saving
– BZD – first line
– ECT – gold standard, use if BZD ineffective/contraindicated
– Dopaminergic agents – use adjunctively
• Prolonged and malignant cases require aggressive management
Questions?
References
American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. 4th ed – Text
Revision. Washington, DC: American Psychiatric Association, 2000.
Babington PW, Spiegel DR. Treatment of catatonia with olanzapine and amantadine. Psychosomatics
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Bastiampillai T, Dhillon R. Catatonia resolution and aripiprazole. Australian & New Zealand Journal of
Psychiatry 42:907, 2008.
Bowers R, Ajit SS. Is there a role for valproic acid in the treatment of catatonia? Journal of Neuropsychiatry
and Clinical Neuroscience 19:197-198, 2007.
Bush G, et al. Catatonia and other motor syndromes in a chronically hospitalized psychiatric hospitalization.
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Carroff SN, et al. Catatonia: From Psychopathology to Neurobiology. Arlington, VA: American Psychiatric
Press, 2004.
Carroll BT, et al. Review of adjunctive glutamate antagonist therapy in the treatment of catatonic syndromes.
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Chang CH, et al. Treatment of catatonia with olanzapine: A case report. Progress in NeuroPsychopharmacology & Biological Psychiatry 33:1559-1560, 2009.
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