Diagnosis and Treatment of Catatonia - Pitt · PDF fileDiagnosis and Treatment of Catatonia...
Transcript of Diagnosis and Treatment of Catatonia - Pitt · PDF fileDiagnosis and Treatment of Catatonia...
Priya Gopalan, MDPierre N. Azzam, MD
Diagnosis and Treatment of Catatonia
• Overview of Catatonia– Typical Features of Catatonia– Historical Evolution of the Diagnosis– Diagnosis from DSM-IV to DSM-5– Epidemiology– Clinical Evaluation of Catatonia
• Prototypes of Catatonia– Distant Mute– Waxy Stiff– Stubborn Grouch– Broken Record
Agenda
• Catatonia and its Underpinnings– Differential Diagnosis– Precipitating Conditions– Pathophysiologic Theories
• Management of Catatonia– Essential of Management– Mainstays of Therapy– Benzodiazepines in Challenge and Maintenance Therapy– Electroconvulsive Therapy (ECT) and Alternate Options– Management and Prevention of Complications
• Questions
Agenda
Pierre N. Azzam, MD
Overview of Catatonia
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: – 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:– 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:– 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 betweentwo planes in space… Like therewere pins in me everywhereholding me in place… Iremember you asking questions,and I knew some answers butcouldn’t say… I was scared.”
• 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”)
Catatonia at a Glance
History of Catatonia: Kahlbaum (Fink 2003)
• Coined the term catatonia• Stages of the condition:
– 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 (Fink 2003)
• Late 1800s: Emil Kraepelin folded catatonia into dementia praecox, conceptualized catatonia as a more chronic disordered state.
• 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 CommonsE. Bleuler. Beilage sur Munchener medizinischen Wochenschrift. Public domain image. Wikimedia Commons
• 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
History of Catatonia: Modern Progression
Catatonia and the DSM IV-TR (APA 2000)
• Classifications– Schizophrenia (subtype)– Depressive, manic, mixed
episodes (specifier)– Catatonia secondary to
general medical condition (stand-alone diagnosis)
• 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+)– Stupor– Catalepsy– Waxy Flexibility– Mutism– Negativism– Posturing– Mannerism– Stereotypy– Agitation– Grimacing– Echolalia– Echopraxia
• 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
Epidemiology
1. Excitement2. Immobility/Stupor3. Mutism4. Staring5. Posturing/Catalepsy6. Grimacing7. Echophenomena8. Stereotypy9. Mannerisms10. Verbigeration11. Rigidity12. Negativism13. Waxy Flexibility14. Withdrawal
15. Impulsivity16. Automatic Obedience17. Mitgehen18. Gegenhalten19. Ambitendency20. Grasp Reflex21. Perseveration22. Combativeness23. Autonomic Abnormality
• Screening: First 14 items (2+ positive)
• Monitoring: All 23 items (each measured for serial tracking)
Bush-Francis Catatonia Rating Scale (Bush 1997)
• Observe patient engaging in conversationLook for: mutism, echolalia, verbigeration
• Scratch your head in exaggerated manner Look for: echopraxia
• Check chart for 24-hour oral intake, vital signs, behaviorsLook for: autonomic instability, withdrawal
• Observe directly and indirectly at different times of the dayLook 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 reflexLook for: frontal release signs
• Examine arms for toneLook for: rigidity, waxy flexibility, gegenhalten
Clinical Evaluation (Fricchione 2011)
Priya Gopalan, MD
Prototypes of Catatonia
• 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?– Echo-phenomena– Verbigeration– Rigidity– Catalepsy– Grasp reflex
• What factors predispose this patient to catatonia?– 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
Clinical Case #2: Pt. C.
• Carl Jung: Archetypes
• Kahana and Bibring: Personality types
• Groves: “Hateful Patient”
• Prototype models exist for numerous psychiatric conditions (e.g., bipolar disorder, personality disorders)
Prototypes in Psychiatry
Prototype Models
• Advantages:– Effective
– Practical
– Matches natural clinical reasoning
– Facilitates teaching and pattern-guided recognition
• Disadvantages:– Oversimplified
– Risk of stereotyping
– Risk of symptom exclusion
– Associated with high false-positive rates
Retarded Catatonia (Kahlbaum Syndrome)
• Clinical features:– 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:– 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– Clinical features:
• Posturing, rigidity• Confusion• Intermittent excitement• Fever, dysautonomia
– Course: sudden; rapid progression; fluctuation
– Mortality: 10-30%
• Neuroleptic Malignant Syndrome– Clinical features:
• Severe rigidity• Altered mental status• Fever, dysautonomia• Recent neuroleptic use
– NMS and MC clinically indistinguishable
– Two sides of a coin?–Vancaester 2007
Malignant Catatonia
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 opioiddependence
• 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
Overarching Theme
Disconnection Inertia Perseveration Obstinance
Diagnostic Features
Mutism
Immobility
Interpersonal withdrawal
Catalepsy
Waxy flexibility
Rigidity
Echophenomena
Verbigeration
Hyperactivity
Negativism
Repetitive movements
Excitement
Challenges in the General Hospital
Distinguishing sedation from catatonic stupor
Accusations of volitional symptom production
Reflexive misattribution to psychopathology
Risk of medical and surgical complications due to immobility
Management of patient safety by unit staff
Misdiagnosis leading to neuroleptic administration
Incomplete medical evaluation due to negativism
Consideration of medical decision-making capacity with refusal of care
Pierre N. Azzam, MD
Catatonia and its Underpinnings
Differential Diagnosis
• 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 (Fricchione 1997, 2011)
• Psychiatric Illness– Affective disorders *– Schizophrenia *– Autistic spectrum
disorders*– Conversion disorder– Obsessive compulsive
disorder (OCD)
• Neurologic Insults– Epilepsy *– Encephalopathy *– Encephalitis*– Infarct (cingulate,
temporal, parietal) *– CJD– Hemorrhage– Meningitis– Traumatic Brain Injury
Precipitating Conditions (Fricchione 1997, 2011)
• Medical Conditions– Acute intermittent
porphyria *– Systemic lupus
erythematosis*– Addison’s disease– AIDS– Cushing’s disease– Hepatitis– Hyperparathyroidism– Tuberculosis– Uremia
• Medications/Drugs– 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
• Dopamine HYPOactivity– Mesolimbic– Nigrostriatal– Hypothalamic
• GABAA HYPOactivity– Orbitofrontal– Limbic connections– Striatal DA inhibition
• Glutamate HYPERactivity– Posterior parietal– Frontal GABAA inhibition
• 5-HT and possible link to serotonin syndrome?
Proposed Neurochemistry (Daniels 2009)
Priya Gopalan, MD
Management of Catatonia
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 (Gross 2008)
• Aspiration• Burn• Cardiac failure• Dehydration• DIC• DVT/PE• GI bleed/obstruction• Pneumonia• Renal failure
• 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 non-responsive 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
• 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
ECT in the Treatment of Catatonia
Case-Reported Treatments
• Antiepileptic Drugs– Carbamazepine– Topiramate– Valproic Acid
• Atypical antipsychotics– Aripiprazole– Olanzapine
• Levodopa• Lithium• Methylphenidate• NMDA-antagonists
– Memantine– Amantadine
• Zolpidem
(Babington 2007; Bastiampillai 2008; Bowers 2007; Carroll 2007; Chang 2009; Munoz 2008; Prowler 2010)
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 = AsleepBZDs remain the treatment of choice.
• Response to Ativan proves it’s psychiatricBZDs treat catatonia, often not the precipitating condition.
• Non-Malignant Catatonia– BZD response: 70%
– ECT response: 85%
– AP response: 7.5%
• Malignant Catatonia– BZD response: 40%
– ECT response: 89%
– AP response: 0%
Treatment Outcomes (Hawkins 1995)
• Less Likely to Relapse– Rapid, dramatic response
to BZD
– Episodic course
– High inter-episode functioning
– Primary mood disorder
– If ECT: adequate seizures, easily induced
• More Likely to Relapse– High-dose BZD required
– Coarse neurologic disease
– Oneiroid state
– Chronic mania
– Alcohol dependence
– Substance-induced mood disorder with catatonia
When to Maintain Treatment (Daniels 2009; Carroll 2004)
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. Psychosomatics48:534-536, 2007.
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. Schizophrenia Research 27:83-92, 1997.
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. Journal of Neuropsychiatry and Clinical Neuroscience 19:406-412, 2007.
Chang CH, et al. Treatment of catatonia with olanzapine: A case report. Progress in Neuro-Psychopharmacology & Biological Psychiatry 33:1559-1560, 2009.
Daniels J. Catatonia: Clinical aspects and neurobiological correlates. Journal of Neuropsychiatry and Clinical Neuroscience 21:371-380, 2009.
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