Catatonia

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INTRODUCTION

CATATONIA

Catatonia is a syndrome of specific motor abnormalities closely associated with disorders in

mood, affect, thought, and cognition. The principal signs of the disorder are mutism,

immobility, negativism, posturing, stereotypy, and echo-phenomena. This syndrome is a

significant clinical problem and in certain situations it represents a psychiatric and even vital

emergency because it is associated with dehydration, intercurrent infection, and pulmonary

embolism.

PRINCIPLE FEATURES OF CATATONIA

Mutism Verbal unresponsiveness, not always associated with immobility

Stupor Unresponsiveness, hypoactivity, and reduced or altered arousal

during which the patient fails to respond to queries; when severe,

the patient is mute, immobile, and does not withdraw from

painful stimuli

Negativism

(Gegenhalten)

Patient resists examiner’s manipulations, whether light or

vigorous, with strength equal to that applied, as if bound to the

stimulus of the examiner’s actions

Posturing

(catalepsy)

Maintains postures for long periods. Includes facial postures, such

as grimacing or Schnauzkrampf (lips in an exaggerated pucker).

Body postures, such as psychological pillow (patient lying in bed

with his head elevated as if on a pillow), lying in a jack-knifed

position, sitting with upper and lower portions of body twisted at

right angles, holding arms above the head or raised in prayer-like

manner, and holding fingers and hands in odd positions

Waxy flexibility Offers initial resistance to an induced movement before gradually

allowing him-self to be postured, similar to bending a candle.

Stereotypy Non-goal-directed, repetitive motor behaviour. The repetition of

phrases and sentences in an automatic fashion, similar to a

scratched record, termed verbigeration, is a verbal stereotypy.

The neurologic term for similar behaviour is palilalia, during

which the patient repeats the sentence just uttered, usually with

increasing speed.

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Automatic

obedience

Despite instructions to the contrary, the patient permits the

examiner’s light pressure to move his limbs into a new position

(posture), which may then be maintained by the patient despite

instructions to the contrary.

Ambitendency The patient appears “stuck” in an indecisive, hesitant movement,

resulting from the examiner verbally contradicting his own strong

non-verbal signal, such as offering his hand as if to shake hands

while stating, “Don’t shake my hand, I don’t want you to shake

it.”

Echophenomena Includes echolalia, in which the patient repeats the examiner’s

utterances, and echopraxia, in which the patient spontaneously

copies the examiner’s movements or is unable to refrain from

copying the examiner’s test movements, despite instruction to the

contrary

Mannerisms Odd, purposeful movements, such as holding hands as if they

were handguns, saluting passersby, or exaggerations or stilted

caricatures of mundane movements

CATATONIC SPECTRUM BEHAVIORS

Tiptoe walking, skipping, hopping

Repeating questions instead of answering

Manneristic hand or finger movements not typically dyskinetic

Inconspicuous repetitive actions, such as making clicking sounds before or after

speaking; automatically tapping or touching objects or body parts; tongue chewing,

licking; lip smacking; pouting; teeth clicking; grimacing; frowning; squeezing shut or

opening eyes wide

Oddities of speech, such as progressively less volume until speech is an almost inaudible

mumble (prosectic speech); using a foreign accent not typical for the patient; speaking

like a robot or like a child learning to read; speaking without the use of word contractions

(e.g., “I am not going to the store because I cannot do it” rather than using “I’m” and

“can’t”)

Holding head in odd positions

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Rocking, shoulder shrugging, sniffing and wrinkling of nose, opening eyes wide and then

squeezing them shut

Rituals, such as tapping the dishes or eating utensils in a specific order before eating;

tapping buttons before buttoning a shirt

THE CATATONIA SYNDROMES

Sl. no Subtype of catatonia Eponyms

1 Retarded catatonia

Benign stupor

Kahlbaum syndrome

2 Excited catatonia

Manic excitement

Delirious mania Manic delirium

Bell’s mania

Oneiroid state Onirisme

Oneirophrenia

3 Malignant catatonia (MC) Lethal catatonia

Pernicious catatonia

Acute fulminating psychosis

Neuroleptic malignant syndrome

(NMS)

NMS

Syndrom malin

Neuroleptic induced catatonia

Toxic serotonin syndrome (TSS) TSS; Serotonin syndrome

4 Periodic catatonia

Mixed affective state

Rapid cycling mania

5 Primary akinetic mutism Apallic syndrome

Stiff man syndrome

Locked-in syndrome

NEUROLOGIC CONDITIONS ASSOCIATED WITH CATATONIA

Encephalitis (particularly when limbic structures and temporal lobes are involved, as

with herpes, encephalitis lethargica)

Post encephalitic states (particularly with Parkinsonism)

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Parkinsonism

Subacute sclerosing panencephalitis

Bilateral lesions of the globus pallidus

Bilateral infarction of the parietal lobes

Temporal lobe infarction

Thalamic lesions

Periventricular diffuse pinealoma

Anterior cerebral and anterior communicating artery aneurysms and hemorrhagic infarcts

Frontal lobe traumatic contusions, arteriovenous malformations, neoplasms

Primary frontal lobe degeneration

Traumatic hemorrhage in the region of the third ventricle

Subdural hematoma

General paresis

Tuberous sclerosis

Paraneoplastic encephalopathy

Multiple sclerosis (particularly the mid-life onset form which often affects the

cerebellum)

Familial cerebellar-pontine atrophy

Epilepsy (particularly psycho-sensory)

Creutzfeldt–Jakob’s disease

Alcohol degeneration andWernicke’s encephalopathy

AIDS related dementia and other white matter dementias

Narcolepsy

METABOLIC DISORDERS THAT ARE ASSOCIATED WITH CATATONIA

Diabetic ketoacidosis

Hyperthyroidism

Hypercalcemia from a parathyroid adenoma

Pellagra, vitamin B12 deficiency

Acute intermittent porphyria

Addison’s disease

Cushing’s disease

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Syndrome of inappropriate anti-diuretic hormone secretion (SIADH)

Hereditary coproporphyria, porphyria

Homocystinuria, uremia, glomerulonephritis

Hepatic dysfunction or encephalopathy

Thrombocytopenic purpura

Lupus erythematosus or other causes of arteritis

Infectious mononucleosis

Langerhans carcinoma

Tuberculosis

Typhoid

Malaria

Toxic states secondary to mescaline, amphetamine, phencyclidine, cortisone, disulfiram,

aspirin, antipsychotic agents, illuminating gas, organic fluorides

LETHAL COMPLICATIONS OF CATATONIA

Profound negativism

Immobility and refusal to take in fluids predisposes patients to malnutrition, dehydration

Rhabdomyolysis

Aspiration pneumonia

Obstructive nephropathy

Azotemia

Deep vein thrombosis

Pulmonary embolism

DRUGS ASSOCIATED WITH MALIGNANT CATATONIA AND NEUROLEPTIC

MALIGNANT SYNDROME (NMS)

Antipsychotic drugs of all types and potencies, both typical and atypical

Abrupt withdrawal of antipsychotic drugs, or benzodiazepines

Antidepressant drugs combined with antipsychotic drugs

Disulfiram intoxication

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Corticosteroid intoxication

Phencyclidine intoxication

Metoclopramide

Anticholinergic and antihistamine toxicity and abrupt withdrawal

Lithium combined with phenelzine

Phenelzine combined with dothiepin

Clozapine withdrawal

Levodopa, amantadine, or bromocriptine abrupt withdrawal

Carbamazepine and valproic acid

Tetrabenazine and alpha-methyltyrosine combined

Cocaine intoxication

Cyclobenzaprine

FEATURES SUGGESTING GOOD OUTCOME IN A CATATONIC PATIENT

Previous episode with recovery

Present (or past) diagnosis of mania or depression

Hyperactivity, rapid and pressured speech, and lability of mood

Rapidity of episode onset (days or weeks from wellness to full illness)

Good social functioning prior to the episode

RATING SCALES FOR CATATONIA

The Bush–Francis Catatonia Rating Scale (BFCRS)

The BFCRS has 23 items, and there is also a shorter, 14-item screening version.

Modified Rogers Scale (MRS)

The MRS rates abnormalities in movement, volition, speech and overall behaviour, and

also aids in the distinction of catatonic signs from seemingly similar extrapyramidal side-

effects

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MANAGEMENT OF CATATONIA

PAST TREATMENTS OF CATATONIA

Iron and Quinine

The use of iron and quinine, combined with a diet and with a regulation of daily routine of

the patient (when necessary implemented against his will) appear to have contributed greatly

to the favourable outcome.

Blood-letting

Laxatives

Withdrawal of fluids in dieting

Taking “the waters at spas”

Belladonna

Zinc oxide

Potassium bromide

Opium

Fever therapy:

Wagner-Jauregg (1918) injected fever inducing agent malaria by injection of blood from

malaria patients and treated with quinine. He did so in nine patients, reporting that three had

obtained dramatic relief after six weeks of treatment; three had some improvement; and three

showed little benefit

Sleep therapy

Insulin coma therapy (Sakel in 1933),

Chemical convulsive therapy (Meduna in 1934)

Lobotomy (Moniz in 1935)

Electroconvulsive therapy (Cerletti in 1938)

Sedatives

Morphine, hyoscyamus, hyoscine (scopolamine), chloralhydrate, and sodium bromide were

among many sedatives used to treat catatonic patients.

Barbiturates

The discovery of barbiturates in 1903, with their ability to induce sleep, was a boon to

therapy.

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PRESENT TREATMENT OF CATATONIA

SPECIFIC MANAGEMENT

ACUTE TREATMENT

Catatonic patients, especially those with syndromes of acute onset, need protection and care,

usually best done in a hospital. Catatonia responds well to treatment, regardless of the

underlying cause.

RETARDED CATATONIA (KAHLBAUM SYNDROME)

Patients are in a prolonged stupor or rigid posture. Self-care is compromised and they become

dehydrated and lose weight. Skin care is poor, and if they are bed-ridden, bedsores develop.

If allowed to remain in a posture for extended periods, contractures develop. Stasis of the

blood leads to thrombosis, pulmonary embolism, and death.

The severely retarded and often stuporous catatonic patient requires careful nursing care for

hydration, nutrition, mobilization, and skin care. Patients should be clothed daily and urged to

take part in group activities.

For re-hydration of a patient in reasonably good cardiovascular health, 500–1000 ml of

intravenous fluids and electrolytes can be infused quickly

When patients will not eat, parenteral lorazepam (1–2 mg), given 30–60 minutes before meals

often facilitates the patient taking fluid and food to maintain homeostasis. Soon after

injection, patients often eat and drink with nursing assistance. They may then, within an hour

or two, return to the stuporous state. This tactic can be repeated several times each day until

the catatonia is resolved.

If a specific cause is identified, its treatment takes priority (e.g., non-convulsive status

epilepticus). If no specific cause is considered likely, the stuporous patient is best treated with

barbiturates or benzodiazepines and patients are simultaneously evaluated for ECT.

Lorazepam and diazepam can be also used. Lorazepam is initially prescribed at 3–4 mg a

day. If well tolerated, and catatonia does not resolve in two days, the dosage may be doubled,

and increased progressively to 8–16 mg a day. If, after a few days, catatonia does not show

signs of resolving, or does so transiently with each dosing, ECT becomes a prime

consideration.

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Patients with chronic illness and persistent catatonic features are also less likely to respond to

benzodiazepines, making ECT the principal treatment in such patients.

EXCITED PATIENTS WITH CATATONIA (DELIRIOUS MANIA)

Treatment with sedatives is the first option, but the doses need to be large. It is not unusual

for the patient to need large doses repeated at frequent intervals.

Protocol

A common protocol is1-2mg lorazepam (5mg diazepam) every 20–30 minutes, up to 10 mg

lorazepam (40 mg diazepam) within a few hours. (The amobarbital equivalent is 0.5–1 Gm

intravenously every few hours.) In extreme instances, general anaesthesia has been required.

High potency antipsychotic drugs, especially haloperidol, are commonly used to reduce

excited and aggressive behaviour, but in these patients such use risks the development of

malignant catatonia or NMS.

Delirious mania is a life-threatening condition that warrants rapid escalation of parenteral

benzodiazepines. If these are not quickly effective, or the patient’s temperature rises, or

dehydration cannot be effectively treated, daily or twice daily ECT for three to four days may

be required to achieve a rapid result.

In patients in acute manic states rapid dosing with valproic acid or lithium is recommend but,

such a prescription is problematic when the patient is febrile, dehydrated, or catatonic. One

recommended regimen is to give a single afternoon dose of valproic acid (20–30 mg/kg of

body weight) on the first day of treatment. The same, or double the dose, is given on the

second day and daily thereafter. Such dosing is expected to establish effective serum levels

and interrupt excitement.

A similar loading dose of 600–900 mg of lithium carbonate (depending on the patient’s body

weight) may also be effective for simple mania with excitement. Supplementary

benzodiazepine dosing may be needed. If excitement is not controlled by these loading-dose

tactics, or the tactics cannot be used because of the patient’s poor general health, or the

presence of catatonic features, then benzodiazepines or barbiturates are useful.

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MALIGNANT CATATONIA / NEUROLEPTIC MALIGNANT SYNDROME

Malignant Catatonia is a life-threatening condition with the characteristics of catatonia, and

associated fever and autonomic instability. It warrants intensive treatment. The immediate

need in management is the discontinuation of antipsychotic medicines, protection of the

patient when excited and delirious, control of temperature and hydration, and intensive

nursing care

Goal Measures to take

Reverse hyperthermia Aspirin or acetaminophen suppositories; place patient under

cooling blankets or give alcohol bath; gastric lavage with ice

water

Reverse dehydration Intravenous normal or half normal saline. Ringer’s lactate is

avoided as it may increase acidosis. Glucose loads may

precipitate a Wernicke’s encephalopathy in a chronic alcoholic

or other persons with chronically low thiamine levels

Maintain stable blood

pressure and cardiac

rhythm

Blood pressure and pulse rate are monitored.

Hyperkalemia from muscle breakdown is prevented or resolved.

Hypertension is controlled with labetolol or esmolol; and

hypotension by increasing blood volume, and giving

vasopressors

Ensure adequate

oxygenation

Continuously monitor oxygen saturation. Maintain airway

artificially if rigidity blocks air exchange. Use 100% oxygen if

oximetry shows blood saturation less than 95%

Avoid complications of

immobility: thrombosis,

embolism, aspiration

pneumonia, bed sores

Critical care nursing, moving of limbs, changing of position,

skin care

Avoid renal failure Frequent monitoring of serum creatinine phosphokinase (as an

indicator of muscle necrosis), creatine, and urea nitrogen. Check

urine for myoglobinuria to monitor renal function. Dialysis may

be required if the syndrome is not promptly and fully resolved

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ECT is effective in malignant catatonia. It needs to be administered early and intensively in

febrile patients to contain the illness. When treatments are deferred, mortality increases

sharply. The grace period for effective ECT is within the first five days of hospital care.

TREATMENT ALGORITHMS FOR MALIGNANT CATATONIA / NEUROLEPTIC

MALIGNANT SYNDROME

There are two approaches

a) Benzodiazepine / ECT approach

Benzodiazepine Treatment

Benzodiazepines are a common initial treatment for patients with catatonia.

If a specific cause of catatonia is identified treatment of the cause takes priority. If no specific

cause is quickly recognized, the patient with catatonia is best treated with benzodiazepines.

Benzodiazepines are safe, and cardiac arrhythmia is extremely rare.

Electroconvulsive Therapy

When a benzodiazepine challenge does not elicit measurable improvement, preparation for

ECT begins immediately. A failed challenge test (i.e., after 2– 3 mg of lorazepam or

equivalent) predicts a prolonged and often failed clinical trial of benzodiazepines. ECT is

given at a customary frequency with bi-temporal electrode placement and brief-pulse

currents. About 90% of catatonic patients remit with ECT, even those who have not

responded to benzodiazepines. ECT not only will relieve the catatonia but also may improve

the underlying psychopathology, particularly manic-depressive illness. ECT is safe and

effective in patients with general medical conditions that may be the cause of or comorbid

with catatonia. Mortality rate may be lower if ECT used as the initial treatment, particularly

within 5 days of the onset of the illness

b) Dopamine-muscle relaxant approach

Dantrolene effect is almost immediate and, thus hyperthermia also improves This treatment

approach considers NMS as an idiosyncratic response to the dopamine blockade

accompanying antipsychotic drugs, both the typical and the atypical varieties, and dopamine-

depleting drugs such as tetrabenazine and alpha-methyltyrosine.

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The strategies used in this approach are:

(1) The presynaptic dopamine agonist amantadine (100 mg given 2–4 times daily);

(2) The post-synaptic dopamine receptor agonist bromocriptine (5–45 mg daily is the usual

dose, with a starting dose of 2.5 mg orally 2–3 times daily);

(3) The muscle relaxant dantrolene sodium (100–300 mg daily in divided doses, starting with

an IV dose 1–2.5 mg/kg of body weight).

TREATMENT OF COMPLICATIONS

Prophylactic treatments for hypercoagulability are given based on stratified risk.

Deep vein thrombosis and pulmonary embolism

Various formulations of heparin are used in treatment and prevention of Deep vein

thrombosis and pulmonary embolism. Heparin works as an anticoagulant by binding to ant

thrombin III, producing a change that allows anti-thrombin III to more rapidly inhibit

thrombin.

Vitamin K antagonists, such as warfarin, are not favoured for prophylaxis due to their

complex management and delayed onset of action. Instead, warfarin is used as secondary

therapy after a patient has sustained a pulmonary embolism

Other nonpharmacologic prophylactic options include placement of elastic stockings, devices

for intermittent pneumatic compression, and ambulation or exercises.

GENERAL MANAGEMENT

Benzodiazepines

Benzodiazepines are all GABA-A agonists. It has been hypothesized that GABA-A agonists

correct deficient GABA-ergic function in the orbito-frontal cortex. Lorazepam is the most

commonly used benzodiazepine in the treatment of catatonia, but other benzodiazepines such

as diazepam, oxazepam, and clonazepam have also been reported to treat catatonia. it has

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been found that giving lorazepam 1–2 mg intravenously to a catatonic patient can

dramatically reduce catatonic signs and symptoms within minutes; this can be helpful but not

definitive in making a diagnosis of catatonia. It has been noted that patients with catatonia

due to a medical or affective illness may respond better to lorazepam than do patients with

schizophrenia with catatonic features.

Zolpidem

Zolpidem, like the benzodiazepines, is a GABA- A agonist and has been reported in one case

series to be effective in the treatment of catatonia. Zolpidem has a quick onset (15 to 30

minutes) lasts for 3–4 hours, requiring frequent administration. It was reported to be effective

in one case where lorazepam failed.

Electroconvulsive Therapy

Available studies and case reports suggest a high success rate in treating all types of catatonia

with ECT. It has been found to be effective in catatonia refractory or partially refractory to

lorazepam. Most reports of successful treatment of catatonia use bitemporal ECT.

Repetitive Transcranial Magnetic Stimulation (rTMS)

Repetitive Transcranial Magnetic Stimulation (rTMS) can be used successfully to treat

catatonia and showed success where lorazepam had failed.

NMDA Antagonists

N-methyl-D-aspartic acid antagonists are another option when benzodiazepines and ECT fail

or are not an option. Amantadine can have anticholinergic side effects and can also increase

dopaminergic tone. The effect of NMDA antagonists is slower than that of benzodiazepines

and the effects can be seen within 24 hours with complete recovery occurring within 3 weeks.

Antiepileptics

Topiramate is a novel antiepileptic agent that is thought to increase GABA-ergic tone and

augment GABA activity as well as indirectly reduce the activation of AMPA (non-NMDA

glutamate receptor).

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Atypical Antipsychotics

The role of atypical antipsychotics in the treatment of catatonia remains unclear; it is possible

that atypicals directly treat catatonia. In addition to causing a D2 receptor blockade, atypicals

have weak GABA-agonism and 5HT2 antagonism that could stimulate dopamine release in

the prefrontal cortex and help alleviate catatonic symptoms.

CONCLUSION

Catatonia is a neuropsychiatric syndrome that can occur due to medical or psychiatric

disorder.

REFERENCES

Ignatowski M, Sidhu S, Rueve M. Pulmonary Embolism as a Complication of Major

Depressive Disorder with Catatonic Features, Psychiatry (Edgmont). 2007 June; 4(6):

51–56.

Daniels J .Catatonia: Clinical Aspects and Neurobiological Correlates Journal of

Neuropsychiatry.2009 march; 21:371-380

Riba M B., Ravindranath D. clinical manual of Emergency Psychiatry; 1st ed. USA,

American Psychiatric Publishing, Inc.;2010

Sadock BJ, Sadock VA. Kaplan and Sadock’s synopsis of psychiatry, Behavioural

sciences/clinical psychiatry.10th

ed. Philadelphia: Lippincott Williams and Wilkins

publishers; 2007

Oldham J M, Riba, M B. Emergency Psychiatry; 1st ed. USA, American Psychiatric

Publishing, Inc.;2010

Fink M, Taylor MA. Catatonia, clinicians guide to diagnosis and treatment; 1st ed. UK

Cambridge University Press,2003

Catatonia

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