Catatonia

Kristen Shirey, M.D.

Duke University Hospital

Internal Medicine and Psychiatry

Catatonia

• Common

• Signs are easily identifiable

• Many faces

• NMS is a form of malignant catatonia

• Differential diagnosis

• Syndrome of motor dysregulation

• Good-prognosis condition

• Easy to treat

Catatonia is common

• Prevalence is estimated at 6-15% of adult psychiatric inpatients

• Approximating incidence at 10%, catatonia may be 2-3 times more common than suicide in the United States

• Immobility & mutism often recognized while whispered/robotic voice, pacing, or other purposeless movement missed

• Rating scale has inter-rater reliability >0.90 [Norhoff et al. Movement Disorders 1999 14/3; 404-416]

Definition of catatonia

• First described by Kahlbaum in 1874 as a specific motor dysfunction, phase of progressive illness including stages of mania, depression, psychosis ending in dementia

• Includes 3 distinct categories of symptoms: hypo/hyperkinetic, affective & behavioral

• Excellent review by Taylor & Fink AMJP 2003; 160:1233-41

Classic signs of catatonia

• StuporExtreme hypoactivity, immobility, minimally responsive to stimuli (including pain)

• MutismVerbally minimally responsive

• NegativismInvoluntary/amotivational resistance, oppositional behavior (Gegenhalten)

Additional signs

• Automatic obedienceExaggerated cooperation with examiner’s request; mitgehen

• StereotypyRepetitive, non-goal-directed motor acitivity, echopraxia, echolalia, verbigeration

• Catalepsy/PosturingMaintains postures ie. pillow-sign,waxy flexibility

Other sx• Excitement

• Staring

• Grimacing

• Mannerisms: odd, purposeful movements

• Rigidity

• Impulsivity

• Ambitendency (alternating cooperation and opposition)

• Combativeness

ProcedureBush Examines

Observe patient while trying to engage in a conversation Activity level Abnormal movementsAbnormal speech

Examiner scratches head in exaggerated manner Echopraxia

Examine arm for cogwheeling. Attempt to reposture, instructing patient to "keep your arm loose" - move arm with alternating lighter and heavier force.

NegativismWaxy flexibilityGegenhalten

Ask patient to extend arm. Place one finger beneath hand and try to raise slowly after stating, "Do NOT let me raise your arm".

Mitgehen

Extend hand stating "Do NOT shake my hand". Ambitendency

Reach into pocket and state,"Stick out your tongue, I want to stick a pin in it".

Automatic obedience

Check for grasp reflex. Grasp reflex

Check chart for reports of previous 24-hour period. In particular check for oral intake, vital signs, and any incidents.

Attempt to observe patient indirectly, at least for a brief period, each day.

DSM IV => specifier of schizophrenia, mood disorder or general medical

condition

2 out of following 5 criteria:

• Motoric immobility ( catalepsy, waxy flexibility, stupor)

• Excessive motor activity (purposeless, not influenced by external stimuli)

• Extreme negativism (rigid posture, resistance to instructions, gegenhalten, mutism)

• Peculiarities of voluntary movement (grimacing, bizarre postures, stereotyped movements)

• Echolalia or echopraxia

Fink’s proposed catatonia classification• Diagnostic criteria:

A. Immobility, mutism or stupor for at least 1 hour + one of the following:Catalepsy, automatic obedience, posturing, observed or elicited at least twice

B. In the absence of immobility, mutism or stupor, need to observe or elicit at least twice at least two of the following: stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, ambitendency

• Catatonia:1. Non-malignant catatonia:

criteria A2. Delirious mania (excited

catatonia): criteria B + severe mania or excitement

3. Malignant catatonia A or B + fever and autonomic instability

• Modifiers:i. 2/2 Mood disorderii. 2/2 General medical

condition or toxic stateiii. 2/2 a Brain disorderiv. 2/2 Psychotic disorder

Malignant Catatonia

• Dopamine loss, sympathetic overdrive lead to hyperadrenergic state.

• Tachycardia, fever (hotness of body), hypertension, diaphoresis ensue

• Increased vagal tone in the heart stimulates mechanoreceptors, which in turn modulate sympathetic tone -> hypotension, bradycardia, and even asystole…

Clinical features of MC/NMS

• Fever

• Muscle rigidity

• Dyskinesia

• Posturing, waxy flexibility, catalepsy, mutism

• Dysarthria, dysphagia, sialorrhea

• Altered consciousness, may appear comatose

• Autonomic instability: lability of blood pressure, tachycardia, vasoconstriction, diaphoresis

Early signs of MC/NMS

• Mania with fever

• Any catatonic features within 24h of antipsychotic initiation

• Autonomic instability or sialorrhea within 24 h of antipsychotic initiation

• Rapidly developed EPS symptoms with administration of low dose of antipsychotic

Drugs associated with MC/NMS

• All antipsychotics• Metoclopramide• ATD combined with AP• Lithium• MAOIs• CBZ• Valproic acid• Cyclobenzaprine• Alpha-methyltyrosine

• Abrupt withdrawal from antipsychotic

• dopamine agonists BZDantihistaminesanticholinergics

• Intoxication with:disulfiramcorticosteroidsPCP, cocaineantihistaminergicsanticholinergics

Abnormal labs in MC/NMS

• Proteinuria• Myoglobinuria• Very high CPK• High LDH• Leukocytosis (10-25k)• Thrombocytosis• Low serum iron

• Diffuse EEG slowing• Abnormal electrolytes:

Low calciumLow magnesiumHigh potassium

• Elevated LFTs:AST, ALT, rarely bilirubin

Clinical risk factors for MC/NMS

• Dehydration• Exposure to high

temperatures• Agitation• Thyrotoxicosis• Basal ganglia

disorder:TDakathisia

• EPS from Rx

• Past hx catatonia

• Past hx NMS

• Receiving high potency AP or >1 AP

• IM antipsychotic

• AP + ATD or mood stabilizers

• Recent alcohol abuse with liver dysfunction

Most commonly associated with psychiatric disorders

• Bipolar and schizophrenia• Withdrawal from dopaminergic

medications or cocaine (also implicated in NMS)

• Withdrawal from BZD (must exclude nonconvulsive status epilepticus)

• Medical causes (up to 16%)

Medical conditions associated with catatonia

• Endocrinopathies: hypoparathyroidism, thyrotoxicosis, pheochromocytoma

• Infections: viral, HIV, typhoid fever

• Tumors: esp fronto-temporal lesions

• Stroke: esp anterior brain regions

• Traumatic brain injuries: subdural hematoma

• Epilepsy: post-ictal immobility & NCSE

• Autoimmune: SLE

• Heat stroke

• Toxins: tetanus, staph., fluoride, strychnine

• Poisoning: salicylates, inhalational anesthesics

False positives

• Mutism alone is not sufficient; need at least 1 or 2 other motor symptoms

• Stupor alone is not sufficient (EEG)

• Parkinson disease, in particular akinetic parkinsonism, OCD, tic disorder, Tourette syndrome

• Malignant hyperthermia (rare AD genetic d/o)

• Stiff-person syndrome and locked-in syndrome

Catatonia has a good prognosis

• No RCTs of in Rx of catatonia

• Management is well-defined

• Important to treat emergently, as catatonia can evolve in life-threatening condition

• “When properly treated almost all episodes of catatonia fully resolve” Fink & Taylor

• “Most likely cause of failure is … prolonged inadequate treatment” Fink & Taylor

Treatment of catatonia

• Benzodiazepines, most studied are lorazepam and clonazepam at high doses: 70% response within 4 days

• ECT bilateral usually 8- 15 sessions 3x/week

• Benzodiazepines suspend the symptoms; ECT treats underlying disease

• Antipsychotics ineffective, often detrimental

Acute management• Hospitalization• BZD challenge• BZD treatment trial• Maintain fluid and electrolyte balance• Avoid antipsychotic agents• Avoid prolonged immobility• Identify and correct underlying neuropsychiatric

or medical cause• If not improved in 4 days => ECT• Tx may be augmented with NMDA antagonists

amantadine or memantine

Conclusions

• Common

• Signs are easily identifiable

• Many faces

• NMS is a form of malignant catatonia

• Differential diagnosis

• Syndrome of motor dysregulation

• Good-prognosis condition

• Easy to treat