PHENOMENOLOGY OF CATATONIA
Catatonia is a syndrome that occurs in many diseases. It is not a diagnosis. No single definition exists. Its association with psychiatric disorders has been and continues to be rethought, and its association with medical diseases continues to expand. The term “catatonia” was coined in a famous monograph by Kahlbaum in 1874 (1) based on a Greek word meaning “to stretch tight.” Kahlbaum’s manuscript refers to catatonia as the “tension insanity.” The disorder was recognized as a brain disease with a remarkably diverse panoply of signs that cycled between a hypoactive state in which catalepsy and bizarre stereotypies predominated and a hyperactive physical and emotional state, sometimes associated with abnormal, nonsensical speech production. It is not a diagnosis. No single definition exists so that authorities disagree on how broadly to define it (2,3). Its association with psychiatric disorders has been and continues to be rethought, and its association with medical diseases continues to expand. In DSM-5, catatonia is considered a subtype of schizophrenia, a syndrome due to a systemic medical disorder, and a descriptor for affective disorders (4) (Table 38.1). Johnson (9) described catatonia as “a neuropsychiatric syndrome in which an abnormal mental state is associated with cataleptic phenomena, namely akinesia, posturing, and mutism.” “Catalepsy” is the term he reserved for akinesia, posturing, and mutism in the absence of “any psychiatric abnormality” (6). A syndrome of catatonia without psychosis has been described (6), and the term “medical catatonia” has been proposed for a catatonic syndrome, including mental abnormalities, that is due to an organic illness (10).
“Catalepsy,” which was described at least as early as Galen’s time, is a term derived from the Greek meaning “a seizure of the body and soul.” Catalepsy describes the development of fixed postures, either self or externally imposed. It could even be induced by psychosocial stressors, as has occurred in epidemic fashion in response to religious preaching (6).
Catatonia was “hijacked” (6) by Kraepelin and subsumed into the entity dementia praecox. Kraepelin interpreted the syndrome as a form of mental blocking, in contrast to Kahlbaum’s conceptualization of it as an organic brain disease. Bleuler (5) later reinforced this notion in his famous text on schizophrenia, in which 26 pages of his chapter on the symptoms of schizophrenia are devoted to “the catatonic symptoms” (Table 38.2). Catatonia was generally thought to be a schizophrenic subtype, that is, a particular form of schizophrenia, until 1994 officially recognized as occurring in affective disorders as well. The associations among neuroleptic malignant syndrome (NMS), neuroleptic-induced catatonia, catatonia, and lethal catatonia have been explored (2,11–15) and case reports attest to catatonic syndromes occurring in a wide variety of neurologic disorders, possibly as a frequent finding in anti-NMDA-receptor encephalitis (15), an increasingly recognized autoimmune disorder that usually includes psychotic behavior. Several series of open-label treatments of psychiatric catatonia have been reported, generally with excellent outcomes (8,16–18), but the few cases of catatonia occurring as part of a neurologic disorder are difficult to interpret because of the several concurrent treatment regimens usually employed.
Bleuler, 1950 (5)
Catalepsy
Stupor
Hyperkinesis
Stereotypies, motor and behavioral, including speech
Mannerisms
Negativism
Command: automation and echopraxia
Automatisms, motor and speech
Impulsiveness
Johnson’s Cataleptic Triad, Johnson, 1993 (6)
Immobility
Maintenance of imposed postures
Mutism
Bush et al., 1996 (7)
Excitement grimacing
Immobility, stupor echophenomena
Mutism stereotypy
Staring verbigeration
Posturing rigidity
Withdrawal waxy flexibility
Impulsivity, ambitendency
Automatic obedience grasp reflex
Mitgehen perseveration
Gegenhalten autonomic abnormality
Rosebush et al., 1990 (8)
Immobility negativism
Staring waxy flexibility
Mutism echolalia/echopraxia
Rigidity, stereotypy
Withdrawal verbigeration
Posturing, grimacing
CLINICAL SIGNS OF CATATONIA
Signs considered part of the catatonic syndrome are wide ranging (5,6,7,19,20). Multiple distinct but overlapping criteria have been proposed for the diagnosis of catatonia (19) with DSM-5 being the most recent. Taylor and Fink reported that only two signs are required for the diagnosis (Table 38.3) and suggested that catatonia should be classified as a movement disorder (19). In an attempt to objectively define the likely diagnostic criteria, one study compared 32 catatonics to 155 noncatatonic psychiatric patients. Using receiver operating characteristic (ROC) analysis, any cluster of 3 of 11 “classic” signs is discriminated between the two groups, with equal weight given to each sign: immobility/stupor, mutism, negativism, oppositionalism, posturing, catalepsy, automatic obedience, echophenomena, rigidity, verbigeration, and withdrawal (21). Bleuler (5) described patients maintaining postures for months at a time and considered this situation “not at all rare.” Bleuler noted that the patients exerted precise control over muscular contractions so that movements could be made “like a piece of wood” or “as a lever” with exact compensation of other parts of the body. More common than whole-body rigidity was waxy flexibility (cerea flexibilitias). Patients were akinetic but would maintain a posture imposed on them, apparently completely indifferent to discomfort. These imposed postures would persist for several minutes before resolving into more comfortable, albeit still bizarre, postures. In having a posture altered by an examiner, patients often participated, responding easily to mildly applied pressures, or they overresponded if moved quickly (mitgehen). At other times, however, displaying a negative approach, the patient might also resist alteration of a posture. The waxy flexibility or fixed posture might apply only to one part of the body, while another limb performed a variety of maneuvers. The catalepsy could sometimes be provoked or aborted by environmental changes, so that patients might suddenly become hyperactive, after being stuporous, if a particular person entered the room, or they would exhibit catalepsy only when observed, and appear normal when thinking themselves unobserved.
Akinesia
Mutism
Diminished responsiveness and alertness
Negativism
Waxy flexibility
Posturing
Excessive, purposeless motor activity
Staring
Echolalia or echopraxia
DSM-5 (10) defines catatonia as a movement disorder without a physiologic basis, characterized by signs of akinesia, mutism, decreased alertness and responsiveness, negativism, posturing, waxy flexibility, excess purposeless movement, staring, echolalia, or echopraxia. There are three subtypes: retarded, excited, and malignant. In a Canadian cohort of 148 catatonic patients, almost all exhibited immobility, mutism, and withdrawal—55% to 65% negativism, posturing, grimacing or rigidity. Less than 35% showed waxy flexibility, verbigeration, stereotypy, echolalia, or echopraxia (16).
1. Immobility, mutism, or stupor of at least 1 hour in duration, associated with at least one of the following: catalepsy, automatic obedience, or posturing, observed or elicited on two or more occasions
2. In the absence of immobility, mutism, or stupor, at least two of the following, which can be observed or elicited on two or more occasions: stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten, ambitendency