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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 Stupor Verbal unresponsiveness, not always associated with immobility 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. 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.” 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

Ambitendency

Echophenomena

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 1 Subtype of catatonia Retarded catatonia Benign stupor 2 Excited catatonia Manic excitement Eponyms Kahlbaum syndrome

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) 4 Periodic catatonia Mixed affective state 5 Primary akinetic mutism Apallic syndrome Stiff man syndrome Locked-in syndrome TSS; Serotonin syndrome Rapid cycling mania

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 sideeffects

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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 Reverse hyperthermia

Measures to take Aspirin or acetaminophen suppositories; place patient under cooling blankets or give alcohol bath; gastric lavage with ice water 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

Reverse dehydration

Maintain pressure rhythm

stable and

blood Blood pressure and pulse rate are monitored. cardiac Hyperkalemia from muscle breakdown is prevented or resolved. Hypertension is controlled with labetolol or esmolol; and hypotension vasopressors by increasing blood volume, and giving

Ensure oxygenation

adequate 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 Critical care nursing, moving of limbs, changing of position, immobility: thrombosis, skin care

embolism, aspiration pneumonia, bed sores 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 dopaminedepleting 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