Continuing Education Activity

Catatonia, a neuropsychiatric syndrome characterized by abnormal movements, behaviors, and withdrawal, is a condition that is most often seen in mood disorders but can also be seen in psychotic, medical, neurologic, and other disorders. Most studies on the incidence of catatonia find it to be between 5% - 20% in the acute inpatient psychiatric setting. This activity reviews the cause and presentation of catatonia and highlights the role of the interprofessional team in its management.

Objectives:

• Review the causes of catatonia.
• Describe the presentation of catatonia.
• Summarize the treatment of catatonia.
• Explain the importance of improving care coordination among interprofessional team members to improve outcomes for patients affected by catatonia.

Introduction

Catatonia, a neuropsychiatric syndrome characterized by abnormal movements, behaviors, and withdrawal, is a condition that is most often seen in mood disorders but can also be seen in psychotic, medical, neurologic, and other disorders. Most studies on the incidence of catatonia find it to be between 5% - 20% in the acute inpatient psychiatric setting. Most episodes of catatonia can be classified as excited, retarded, or malignant. Symptoms can wax, wane, or change during these episodes, and patients affected can have periods of withdrawal and periods of excitation. Studies have suggested a connected pathway between the cortex, basal ganglia, and thalamus underpins these different subtypes and results in catatonic symptoms. Recognition and treatment of catatonia can play an important part in both psychiatric and medical treatment as it can inhibit treatment, confusing diagnoses, and be potentially fatal if untreated.[1][2][3]

There are a few different catatonia rating scales that have been developed to both screen for and monitor the progression of symptoms during an episode (e.g., Bush-Francis Catatonia Rating Scale and the Northoff Catatonia Scale), but the DSM-V gives 12 categories for symptoms that can lead to a diagnosis of catatonia. These symptoms include stupor, catalepsy, waxy flexibility, mutism, negativism, posturing, mannerisms, stereotypy, agitation not influenced by external stimuli, grimacing, echolalia, and echopraxia. At least three of these symptoms must be present for the diagnosis of catatonia. Some other criteria used in the Bush-Francis rating scale include automatic obedience, autonomic abnormalities, and the presence of a grasp reflex. These together present a wide variety of symptoms that can be present, and while there is a crossover between them and aspects of other illnesses, findings such as waxy flexibility, posturing, and automatic obedience can be more specific for catatonia.

Etiology

Catatonia is often thought to precipitate secondary to another underlying illness. Psychiatric disorders can present primarily with symptoms of catatonia. Mood disorders such as bipolar disorder and depression are the most common disorders in which catatonia manifests. A psychotic disorder such as schizophrenia can also be associated with catatonia, and historically schizophrenia recognition and diagnosis included symptoms of catatonia or was subtyped if catatonic symptoms were present. When catatonic symptoms present, the cause is likely psychiatric, but many medical etiologies can lead to catatonia. Neurologic insults such as strokes, neoplasms, or other diseases such as Parkinson's can lead to catatonia. Autoimmune, paraneoplastic, infectious, metabolic, and certain drug exposures and poisonings can lead to the development of catatonia. The differential for the cause of catatonia is very broad, and a new case of catatonia without a significant psychiatric history should be a cause for further evaluation for an underlying insult.[4][5]

Epidemiology

A wide variety of illnesses can be associated with catatonia. The studies of incidence and epidemiology of catatonia center mainly around psychiatric patients. In retrospective studies, rates of catatonia among all psychiatric patients range from 0.5% - 2.1%; although, some prospective studies have found the incidence to be as high as 17.1%. One prospective and retrospective study by Stöber et al. using the DSM-III-R criteria found the rate of catatonia to be 24.4%. Among patients with mood disorders, rates have been higher, ranging from 13% - 27%. Historically, as schizophrenia has been associated with catatonia, studies have been performing measuring the rates of catatonia starting in the 1910s. Two studies in 1911 and 1913 found the incidence rates for catatonia to be 50% and 19.5%, respectively. One retrospective study published in 1979 found the rate, between 1938 and 1960, of 990 patients to be 16.9%. More recent studies vary, with one published in 1998 found the rate to be 40.2%, and another in 2002, found it to be 10.3%. Overall, in schizophrenia patients, rates have been found to be as low as 1% and as high as 50%.[6][7]

Pathophysiology

The pathophysiology of catatonia is not fully understood, but as imaging studies have improved more structures and pathways have been implicated in the pathogenesis of this syndrome. Using FMRI imaging, dysfunction has been seen in the right medial orbitofrontal and lateral orbitofrontal prefrontal cortex. The right motor cortex has shown atypical lateralization after patients who were suffering from catatonia were given lorazepam. Dysfunction in GABA, glutamate, serotonin, and dopamine transmissions have been implicated in the initiation and progression of catatonia symptoms through clinical findings of catatonia as a result of agents that disrupt these pathways or agents that affect these pathways relieving the symptoms of catatonia.

History and Physical

The initial presentation for catatonia can vary greatly due to the different subtypes that this syndrome can present. A lot of causes for catatonia can also make a typical presentation difficult to describe. As most patients with catatonia have a psychiatric illness primarily causing catatonia, a progression of psychiatric illness will be the most likely history.

A patient will often present with worsening depression, mania, or psychosis antecedent to catatonia symptoms beginning. These symptoms can present as excited, withdrawn, or a mixture. A patient presenting with excited catatonia will often have odd mannerisms such as performing actions without purpose or at inappropriate times (e.g., saluting). They may be agitated, hold odd positions against gravity, or have stereotypic and repetitive movements such as picking at their clothes or making odd gestures repeatedly. Their speech may be repetitive or mimic the interviewer’s speech or actions. A patient with withdrawn catatonia will likely be stuporous, hold an odd position, have no response or opposition to outside stimuli, and have very little speech. These symptoms may be present at some times and not at others, may be present in a combination, and vary in intensity throughout the hospital course. If these symptoms begin because of a secondary medical illness that illness may also cause other psychiatric symptoms such as mania or psychosis.

The physical exam for a patient with suspected catatonia can help to diagnose and differentiate it between other conditions such as neuroleptic malignant syndrome. Passive movement of limbs and the type of resistance encountered can reveal what the underlying condition is. If the patient has waxy flexibility and catalepsy (holds a posture against gravity when passively moved into a posture,) catatonia is high on the differential. If there is lead-pipe rigidity, neuroleptic malignant syndrome should be suspected. Spastic rigidity would indicate potential serotonin syndrome. Cogwheel rigidity is more concerning for extrapyramidal symptoms from neuroleptics.

A specific examination for catatonia using the Bush Francis Catatonia Rating Scale consists of:

• Observe the patient during normal conversation.

• Scratch the head in an exaggerated manner while speaking with the patient to see if they will copy the movement.

• Passively move the patient’s arm to examine for cogwheeling, varying the amount of force provided while telling the patient to keep their arm loose.

• Have the patient extend their arm and with one finger apply light pressure on their index finger while telling the patient, “Do not let me raise your arm.”

• Extend your hand for a handshake while telling the patient, “Do not shake my hand.”

• Reach into your pocket and state, “Stick out your tongue; I want to stick a pin in it.”

• Check for grasp reflex.

• Check oral intakes, vital signs, and any instances of agitation.

• Each day, observe the patient indirectly for other symptoms of catatonia, including their activity level, abnormal movements and speech, echopraxia and echolalia, rigidity, negativism, waxy flexibility, gegenhalten (resistance equal to the amount of pressure applied), mitgehen (the patient raising their hand to the light pressure), ambitendency, automatic obedience, and grasp reflexes.

Evaluation

While the diagnosis of catatonia is a clinical diagnosis that does not require specific lab tests or imaging, certain testing can help determine the underlying etiology of the catatonia.  An EEG in a patient with primary catatonia due to a psychiatric disorder will likely have diffuse slowing on EEG. As a post-ictal state can cause catatonia, an EEG may be helpful in detecting seizure activity driving the syndrome. While an MRI or CT scan cannot show catatonia, brain imaging could show abnormalities that are causing the catatonia. Imaging of the rest of the body may reveal neoplasms causing paraneoplastic encephalitis. Metabolic screens for illnesses such as diabetic ketoacidosis, glomerulonephritis, hepatic dysfunction, or other abnormalities may reveal a reversible cause. Inflammatory markers and autoantibodies may show autoimmune causes for catatonia. Vital signs should be monitored frequently as autonomic instability may indicate the onset of malignant catatonia - a catatonic syndrome characterized by fever, hypertension, tachycardia, and tachypnea which can progress to death if not rapidly treated. Overall in a patient with new-onset catatonia, psychiatric causes should be considered first, but somatic causes should not be ignored, especially if an underlying mental illness does not easily explain the clinical picture.[8][9]

Treatment / Management

The initial treatment, once potential catatonia causing agents such as neuroleptics, steroids, stimulants, anticonvulsants, dopamine depleters, and others, are stopped, is to provide a lorazepam “challenge.” By giving a dose such as lorazepam 2 mg IV slowly, 60% - 80% of patients with catatonia will have some or significant improvement in catatonia symptoms within 15 min - 30 min. If the patient responds to this lorazepam challenge, lorazepam can be subsequently scheduled at interval doses, often three times a day (though different patients respond at different rates.) The dose of lorazepam can be titrated until catatonia symptoms resolve. Paradoxically catatonic patients do not become sedated on benzodiazepines. Once the dose is titrated for efficacy, patients will be alert and interactive. Throughout this titration, the patient’s underlying cause should also be treated. This lorazepam dose can be slowly tapered as tolerated. If this tapering occurs too quickly, catatonia symptoms may return. Some patients require tapering over months. When the dose makes the patient sedated instead of active, it can likely be reduced.[10][11][12]

If the patient’s catatonic symptoms do not respond to benzodiazepines usually within 1 week, and the underlying cause either cannot be treated or treating it does not improve the symptoms, electroconvulsive therapy (ECT) can be used to reverse the symptoms. ECT is also recommended for malignant catatonia often used in combination with benzodiazepines. 

Differential Diagnosis

• Anterior circulation stroke

• Cardioembolic stroke

• Central pontine myelinolysis

• Cerebral venous thrombosis

• Complex partial seizures

• Lewy body dementia

• Fibromuscular dysplasia

• Glioblastoma multiforme

• Vascular surgery for atrioventricular malformations

Prognosis

Catatonia has historically responded well to the lorazepam challenge, thus, the prognosis is generally positive. Studies exhort response rates of up to 80% following the administration of lorazepam. 

Complications

• May engage in violence or be involved in trauma as a result of the excited stage
• Develop autonomic instability
• Refuse to eat
• Develop dehydration
• Develop pneumonia
• Develop neuroleptic malignant syndrome 
• Risk of DVT and pulmonary embolus
• Develop pressure ulcers
• Develop muscle contractions

Deterrence and Patient Education

Educating family and friends to be able to identify the onset of catatonia can obviate complications of catatonia. The earlier the behavioral aberration is acknowledged, the sooner it can be treated, ultimately reducing the overall morbidity of the patient.

Enhancing Healthcare Team Outcomes

Catatonia is not an uncommon presentation to the emergency department. However, because of its vague presentation, it can be mistaken for a variety of other medical disorders. The condition has a very high morbidity and mortality and is best managed by an interprofessional team that includes a mental health nurse, a psychiatrist, a psychologist, an internist, a neurologist, and an ophthalmologist. With treatment, the response rate varies from 50% to 70% and the prognosis is good. However, those who fail to respond to medications have a very poor prognosis. Many of these individuals require continuous mental healthcare and suicides are commonly reported. Further, patients with catatonia are at very high risk for thrombosis and pulmonary embolism. Finally, those who have catatonia in the presence of schizophrenia have a much worse prognosis than those who do not have schizophrenia.[13][14] (Level V)