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 – – – – – 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 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: – – – – 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 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: – – – – 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+) – – – – – – – – – – – – 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 1. 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? – – – – – 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 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: – – – – – 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 • 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 • • • • • • • • 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 – – – – – – – – 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 – – – – – – – – 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 • • • • • • • • • Aspiration Burn Cardiac failure Dehydration DIC DVT/PE GI bleed/obstruction Pneumonia Renal failure • • • • • • (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? 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