Movement disorders J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2014-309098 on 19 November 2014. Downloaded from REVIEW Movement disorders in catatonia Subhashie Wijemanne, Joseph Jankovic ▸ Additional material, ABSTRACT between 7.6% and 38% among all psychiatric including videos, is published Catatonia is a complex neuropsychiatric syndrome patients.10 The percentage of catatonia due to a online only. To view please characterised by a broad range of motor, speech and general medical condition is reported to range visit the journal online (http:// 11 dx.doi.org/10.1136/jnnp-2014- behavioural abnormalities. ‘Waxy flexibility’, ‘posturing’ from 20% to 39%. Catatonia may be subtle and 309098). and ‘catalepsy’ are among the well-recognised motor overlooked, which may account for reports suggest- Department of Neurology, abnormalities seen in catatonia. However, there are ing a declining incidence. People with bipolar disor- Parkinson’s Disease Center and many other motor abnormalities associated with ders probably constitute the largest subgroup of Movement Disorders Clinic, catatonia. Recognition of the full spectrum of the catatonic patients.51012In a minority of cases, no Baylor College of Medicine, phenomenology is critical for an accurate diagnosis. cause is found and the current prevalence of idio- Houston, Texas, USA Although controlled trials are lacking benzodiazepines pathic catatonia is unknown. Correspondence to are considered first-line therapy and N-Methyl-D- Owing to the wide range of underlying diagnoses, Dr Joseph Jankovic, aspartate receptor antagonists also appears to be patients with catatonia may present as a medical or Department of Neurology, effective. Electroconvulsive therapy is used in those psychiatric emergency13 or develop symptoms ’ Parkinson s Disease Center and patients who are resistant to medical therapy. An during hospitalisation, such as in the intensive care Movement Disorders Clinic, fi Baylor College of Medicine, underlying cause of the catatonia should be identi ed unit (ICU), which can be challenging from a diag- 13 14 6550 Fannin, Suite 1801, and treated to ensure early and complete resolution of nostic standpoint. Catatonia usually presents Houston, TX 77030, USA; symptoms. acutely but may present insidiously, and can be tran- [email protected] sient or chronic, and last for weeks, months and 15 Received 31 July 2014 even years. Catatonic patients are at risk for severe complications such as pneumonia, decubitus ulcers, Revised 8 October 2014 INTRODUCTION Accepted 28 October 2014 malnutrition, dehydration, contractures and throm- Catatonia is a complex neuropsychiatric syndrome Published Online First bosis and delays in diagnosis and management are 19 November 2014 characterised by a broad range of motor, speech and 13 associated with increased morbidity. Although it copyright. behavioural abnormalities. ‘Wa x y flexibility’, ‘postur- may become life-threatening,16 catatonia has an ing’ and ‘catalepsy’ are among the well-recognised excellent prognosis if recognised and treated early. motor abnormalities associated with catatonia. However, there is a wide spectrum of speech and other neurological abnormalities seen in this condi- DIAGNOSTIC CRITERIA AND RATING SCALES tion. This article attempts to summaries the clinical The diagnosis of catatonia is based on clinical obser- features of catatonia; discuss some diagnostic chal- vations. The revised diagnostic criteria were pub- lenges, possible mechanisms and available treatment lished in the fifth Diagnostic and Statistical Manual options in this poorly understood condition. of Mental Disorders (DSM-V) in 2013.17 While the Catatonia was first described by German psycho- DSM-IV used different sets of criteria for diagnosis pathologist Karl Kahlbaum in Die Katatonie oder of catatonia in schizophrenia and primary mood dis- das Spannungsirresein in 1874 as a motor syn- orders versus neurological/medical conditions, the http://jnnp.bmj.com/ drome in patients with behavioural disorders.12 revised DSM-V criteria can be applied across all of He considered catatonia as a distinct clinical entity the different clinical settings. According to DSM-V with progressive symptoms. Catatonia was subse- criteria, to make a diagnosis of catatonia one has to quently classified by psychopathologists Kraepelin have a minimum of 3 of the following 12 clinical and Bleuler as ‘dementia praecox’ (premature features, either observed or elicited during examin- dementia), a condition which was later classified as ation: (1) mutism, (2) stupor, (3) catalepsy, (4) waxy schizophrenia.3 The uncertainty about its definition flexiblity, (5) agitation, (6) negativism, (7) posturing, was partly responsible for the long-standing neglect (8) mannerisms, (9) stereotypies, (10) grimacing, on October 2, 2021 by guest. Protected Editor’s choice of catatonia in clinical and scientific literature and (11) echolalia, or (12) echopraxia.17 The criteria Scan to access more 4 free content for its frequent underdiagnosis. It is clear that seem rather arbitrary, and the list of associated fea- catatonia is no longer limited to schizophrenia, and tures highlights the clinical heterogeneity of this that it can be seen in the setting of a variety of neuropsychiatric disorder. other conditions such as psychiatric disorders other Several rating scales have been developed for the than schizophrenia, medical, neurological and sur- assessment of catatonia.18 The Bush-Francis gical conditions, as well as in the setting of certain Catatonia Rating Scale (BFCRS) is the most widely drugs and toxins.567 used scale. This includes 23 items and up to 30 The frequency of catatonia in acute psychiatric signs. Some of the signs (described below) are not admissions is approximately 10%, but estimates listed in the DSM-V criteria, such as excitement, To cite: Wijemanne S, range from 5% to 20% based on diagnostic criteria staring, rigidity, withdrawal, automatic obedience, Jankovic J. J Neurol used in prospective studies conducted during 1–12 impulsivity, ambitendency, grasp reflex, verbigera- Neurosurg Psychiatry months of observation at psychiatric units.489 tion, mitgehen, autonomic abnormality, combative- – 2015;86:825 832. Other surveys have reported a prevalence ranging ness and perseveration. Wijemanne S, et al. J Neurol Neurosurg Psychiatry 2015;86:825–832. doi:10.1136/jnnp-2014-309098 825 Movement disorders J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp-2014-309098 on 19 November 2014. Downloaded from There is also a screening version of BFCRS known as external stimuli, including pain (see online supplementary Bush-Francis Catatonia Rating Screening Instrument (BFCSI), videos 1–3). The positions assumed by the patient may be which contains 14 most common catatonic signs (excitement, unusual and appear uncomfortable to the observer. The patients immobility/stupor, mutism, staring gaze, posturing/catalepsy, can adopt statuesque postures with minimal movement lasting grimacing, echopraxia/echolalia, stereotypes, mannerisms, verbi- for several hours without any apparent fatigue or discomfort. geration, rigidity, negativism, waxy flexibility and withdrawal). Other examples include twisting of the body, standing on one If two or more of the BFCSI signs are present for 24 h or leg like a stork, holding one arm outstretched for a long time, longer, catatonia should be considered as a possible diagnosis. and squatting with extension of arms. Another dramatic postur- To avoid overdiagnosis, signs such as ‘impulsiveness’ and ‘com- ing is the ‘psychological pillow’ where the patient lies in bed bativeness’ were excluded from the screening instrument.19 with the head and shoulder raised as if there is an imaginary Items from the BFCRS are scored on a 0–3 point scale, whereas pillow. The head is raised a few inches above the bed surface items from the BFCSI are scored as ‘absent’ or ‘present’. which is maintained for prolonged period of time. Another catatonia rating scale, the Modified Rogers Scale In negativism there is increasing resistance to passive manipu- (MRS), has also been validated. lation of the limbs which is known as gegenhalten or paratonia. The primary aim of this review is to draw attention to the When eliciting the phenomenon of gegenhalten, it appears to broad spectrum of phenomenology associated with catatonia by the examiner as if the patient is deliberately opposing the highlighting the most characteristic clinical features and provide passive movement.22 Social negativism may include turning illustrative videos. away when addressed, refusing to open the eyes and closing the mouth when offered food or liquids. CLINICAL FEATURES Stereotypy is a common movement disorder seen in catatonia The catatonic syndrome is seen in two principal forms: hypoki- (see online supplementary videos 1 and 3) which is defined as netic (withdrawn type) or hyperkinetic (excited type).10 Some involuntary, coordinated, patterned, rhythmic, seemingly pur- patients, however, may display features of both types during the poseless movement or utterance performed repeatedly over course of the illness. Patients with hypokinetic or withdrawn time. Some of the motor stereotypies that are seen in catatonia type of catatonia, typically appear awake and watchful, but with include body rocking, shoulder shrugging, hand waving, minimal spontaneous speech and movement. It is commonly opening eye wide and then squeezing them shut, nose wrinkling, associated with mutism, stupor, negativism, obsessional slowness