Katatonia: A New Conceptual Understanding of Catatonia and a New Rating Scale

| December 9, 2008 | 0 Comments

by Brendan T. Carroll, MD; Rob Kirkhart, PhD, PA-C; Niraj Ahuja, MD, MRCPsych; Ilo Soovere, MD; Edward C. Lauterbach, MD; Dirk Dhossche, MD; and Rebecca Talbert, PharmD

Dr. Carroll is Associate Professor of Psychiatry, University of Cincinnati (Volunteer), Department of Veteran Affairs Medical Center, Chillicothe, Ohio; Dr. Kirkhart is Clinical Professor, Marietta College, Department of Veterans Affairs Medical Center, Chillicothe, Ohio; Dr. Ahuja is Consultant Psychiatrist and Honorary Clinical Lecturer, Northumberland, Tyne and Wear NHS Trust and Newcastle University, United Kingdom; Dr. Soovere is Staff Psychiatrist and Neurologist, Perry Point VAMC, Perry Point, Maryland; Dr. Lauterbach is Professor of Psychiatry, Neurology, and Radiology, Mercer University School of Medicine, Macon, Georgia (retired); Dr. Dhossche is Professor of Psychiatry, University of Mississippi Medical Center, Jackson, Mississippi; and Dr. Talbert is Clinical Pharmacist, Riverside Methodist Hospital, Columbus, Ohio.

Psychiatry (Edgemont) 2008;5(12):42–50

Disclaimer: This manuscript was developed by the authors and does not constitute research or opinions of the Department of Veterans Affairs.

Financial Disclosures: The authors have no financial conflicts of interest relevant to the contents of this manuscript.

Abstract

Modern psychiatric nosologies separate catatonia along the lines of presumed etiology: bipolar, major depression, schizophrenia, and/or due to a general medical condition. Catatonic signs have always possessed significant diagnostic, therapeutic, and prognostic value. Kahlbaum’s description of this syndrome in his monograph “Katatonia” included careful documentation of phenomenology. Kahlbaum selected the term katatonia to describe “tension insanity.” He felt that the neuromotor signs were more important than the content of delusions (e.g. megalomania). While he felt that he was describing a unitary illness, he did identify mood disturbance, psychosis, and medical factors in this new illness. In modern times, the term catatonia has become limited to describe a specifier of neuropsychiatric illnesses.

The authors of this article feel that the term katatonia should be used to describe a group of neuropsychiatric illnesses presenting with catatonic signs. This may prevent the misconception that “catatonia is schizophrenia” and improve the detection of katatonia in patients. Specifically, katatonia is also observed in mood disorders, general medical conditions, and pervasive developmental disorders. The literature also supports the view of Dr. Leo Kanner and his description for neuromotor and neuropsychiatric signs in autistic disorder. This scale is named in honor of Dr. Kanner. It was developed by the authors and includes some of Dr. Kanner’s core concepts. This paper will identify the clinical features of katatonia and introduce the KANNER scale (see APPENDIX 1) to improve conceptualization, detection, and measurement of this important clinical syndrome.

Key Words: catatonia, katatonia, stupor, Kahlbaum, bipolar disorder, schizophrenia, autism, KANNER

Introduction

Karl Ludwig Kahlbaum is best known for his description of catatonia. He presented his first description of catatonia in Innsbruck in 1869 and later wrote his monograph entitled Katatonia in 1873.[1] Since then, the presence of catatonic signs has always held diagnostic, therapeutic, and prognostic value.[2]

Kahlbaum’s description of this syndrome in his monograph included careful documentation of phenomenology. He selected the term katatonia to describe “tension insanity” and held that the neuromotor signs (e.g., waxy flexibility) were more important than the content of delusions (e.g., megalomania). While he was convinced that he was describing a unitary illness, he did identify mood disturbances, psychosis, and medical factors in this new illness.[2]

Kahlbaum described katatonia as a syndrome of motor abnormalities including mutism, negativism, stereotypies, catalepsy (waxy flexibility), and verbigeration.[1] Kraepelin included catatonia as belonging to the group of dementia praecox, but acknowledged that “catatonic morbid phenomena” occurred in different psychiatric disorders.[3]

In modern times the terms catatonic and catatonia have unfortunately become limited to describe specifiers for neuropsychiatric illnesses. The clinical term catatonia therefore holds minimal conceptual importance in clinical practice. For instance, Larner defines catatonia as a state of unresponsiveness with maintained immobility, posturing, mutism, and refusal to eat or drink. He lists several signs that may or may not be present (including incontinence).[4] Meanwhile Rogers has argued that catatonia is an extrapyramidal disorder.5 Fink and Taylor point out that in the two centuries since catatonia was first described, psychiatric terminology has changed many times.[6]

The authors propose that the term katatonia (with a “k”) be used to describe this cross-sectional clinical syndrome to avoid continuation of the more recent nosological confusion. In the published literature, katatonia has staged a remarkable comeback over the past 15 to 20 years, with several rating scales, brain imaging and genetic studies, and neurochemical theories that underscore the importance of this clinical entity.[6,7] However, research on katatonia has been limited by the lack of a psychopathological definition and clear conceptual understanding of the subject. This may explain why the existing catatonia rating instruments differ from each other in terms of the definition and number of individual symptoms.[7–11]

There is some variability of katatonia across various psychiatric disorders.[11] Thus, reexamination of the reliability and validity of catatonia rating scales across the various neuropsychiatric illnesses is recommended. Furthermore, the need for a new conceptual understanding of katatonia has been suggested because of the fluidity of the boundaries of the concept.[12] This review addresses the clinical concepts and attempts to describe katatonia over the past few decades. A new rating scale and a conceptual outline for this important clinical entity will be presented.

Research Criteria Defining Katatonia

Kanner’s autism. Kanner identified autism in 11 children between 2 and 8 years of age.[13–15] He described stereotypy, echolalia, and other neuromotor and neurodevelopmental signs. His classification was quickly adopted into the psychiatric nomenclature. Currently, autism (autistic disorder) has been placed as a subcategory of pervasive developmental disorders. Consequently, more emphasis has been placed on the social and emotional domains than the motor signs. There are several observations suggesting that catatonia and autism may have common underlying pathophysiology and treatment response.[16] The Autism Behavioral Checklist (ABC) does not define catatonic signs to the extent of detecting catatonic symptomatology in patients with autism. The autism rating scales fail to quantify items to show treatment response to the catatonic features. Consequently, we feel that there is a need for a new rating scale to assess katatonia in autism and pervasive developmental disorders. To date, there have been no studies of systematic ratings of catatonia in autism.

Lohr and Wisniewski (1987). Lohr and Wisniewski[17] proposed criteria for the catatonic syndrome, requiring one cardinal feature and two secondary features.[1] Northoff found that all patients who met this criteria scored at least 8 (>7) on the Northoff Catatonia Scale (NCS).[9] Catalepsy, positivism, or negativism (1 of these 3) must be present to meet Lohr and Wisniewski’s criteria.[17] Their criteria may not take into account immobility, excitement, and withdrawal (refusal to eat and drink) and may exclude a number of patients with catatonic symptomatology.

These criteria advanced the study of catatonia and highlighted important concepts that underlie catatonia. The clinician, however, may be challenged with some patients with katatonia who fail to meet this set of criteria but not others. Unfortunately, no rating scale was developed from these criteria.

Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) (1994). The development of DSM-IV brought about a new set of criteria for catatonia as a subtype of schizophrenia as well as a specifier for bipolar disorder and major depression. DSM-IV also included catatonia due to a general medical condition. DSM-IV catatonia criteria required 2 out of 5 signs, except for catatonia due to a general medical condition, where only 1 out of 5 was needed. We have previously pointed out the deficiencies of the DSM-IV and DSM-IV-TR (Text Revision) criteria.[18] There is currently no rating scale for DSM-IV or DSM-IV-TR catatonia criteria. Stompe et al[19] found that the sensitivity was greater for diagnoses with a higher number of items in katatonia criteria.

Rosebush et al (1990). Rosebush et al[20] described the Kahlbaum criteria with more than two cardinal features with more than one secondary feature. They used this criteria to identify catatonic features and measure treatment response. The Rosebush rating scale was proposed but it has never been published. Northoff examined this proposed rating scale and found it to have high correlation with other catatonia rating scales.[9] The Rosebush criteria do not take into account catatonic excitement and positivism (e.g., automatic obedience, mitgehen, ambitendency). This set of criteria is helpful but may exclude a number of patients with catatonic signs.

Rogers Scale (1991). The Modified Rogers Scale rates both extrapyramidal signs (EPS) and catatonic signs.[5] It contains several conceptual approaches to catatonia and catatonic signs. It is founded upon the concept that catatonic features are a form of EPS. Consequently the phenomenology is rated independent of presumed etiology and medications (e.g., antipsychotics). Rogers lists 36 motor signs and describes a neuropsychiatric motor examination. Each item is rated on a scale of 0 to 2 with scores ranging from 0 to 72. The Rogers scale has not been widely used in treatment. The Modified Rogers Scale provides definitions for gegenhalten, mitgehen, stereotypy, mannerism, echopraxia, bizarre gait, aprosodic speech, excitement, and negativism. It also describes overcompliance, iteration (the repetition of movements), and hypermetamorphosis. This scale has been used in institutional populations, including patients with mental retardation. It was also used by Starkstein et al[21] in a treatment study of catatonic features in patients with Parkinson’s disease and depression.

The Bush-Francis Catatonia Rating Scale (1996). The Bush-Francis Catatonia Rating Scale (BFCRS) was the first instrument constructed for the systematic, standardized, and quantifiable examination of catatonia using operationally defined signs and symptoms.[22] The BFCRS consists of 23 items accompanied by specific instructions to standardize the examination. Seventeen items are scored on a 0 to 3 scale while the remaining six are rated as either absent (“0”) or present (“3”). Selection of the items was based on a review of the classical and contemporary literature. The inter-rater reliability of BFCRS was established on 28 acutely ill patients presenting with catatonic syndrome that arose in the context of various psychiatric disorders. Catatonia ratings with the BFCRS predicted a high probability of treatment responsiveness to lorazepam.[23] Since its development, the BFCRS has been the most commonly used scale in research in catatonia. It is readily available for clinical use and has been translated into several languages. Our research team has used the BFCRS and considers it the gold standard compared to other scales.

The Northoff Catatonia Scale (1999). The Northoff Catatonia Scale (NCS) was based upon Kahlbaum’s descriptions of catatonia including 1) hypokinesias, 2) hyperkinesias, 3) affective symptoms, and 4) behavioral alterations. The NCS contains 40 items, rated from 0 to 2, with the scores ranging from 0 to 80. Northoff et al[9] performed extensive evaluation of the NCS and compared it to several rating scales. They found the NCS to be valid and reliable for rating catatonia. Also, hypokinesias and hyperkinesias were independent of antipsychotic medication. There was no significant difference between the NCS and the modified Rogers scale in the detection of catatonic patients. A score of >7 on the NCS separated catatonic from noncatatonic psychiatric patients. A factor analysis yielded four components that were characterized as 1) affective, 2) hypoactive, 3) hyperactive, and 4) behavioral.

In their editorial, Rosebush and Mazurek[7] pointed out that catatonia seemed to disappear from the landscape over the last century because psychiatry sought out verbal, introspective, and ‘interviewable’ patients. They expressed concerns regarding the affective and behavioral sections of the NCS because catatonic patients may be difficult to interview. These two sections may reduce the accuracy of the NCS in patients who are mute and exhibit negativism.[7]

The Braunig Catatonia Rating Scale (2000). Braunig, Kruger, and Shugar developed Catatonia Rating Scale (CRS)[10] for catatonia that has 21 items with possible scores of 0 to 4 (absent, minimal, moderate, marked, severe). These are defined by time intervals of observation during a 45-minute examination (<2 minutes, 2–10 minutes, 10–20 minutes, >20 minutes). The scores range from 21 to 84.

The scale is based on the work of several German language authors (Wernicke, Kraepelin, Kleist, Kahlbaum, Blueler, Meyer-Gross, and Leonhard). They group mitgehen and echophenomena under “abnormal suggestibility of will” (i.e., gegengriefen). This scale has been studied under factor analysis, but has not been used to measure treatment response.[10,11]

Fink and Taylor Criteria (2003). Fink and Taylor made a strong argument for a separate classification for catatonia (katatonia) in DSM-V.[6] They proposed a set of criteria for the detection and diagnosis of catatonia that is based on the earlier work of Taylor.[24] They did recommend the use of the BFCRS for research and clinical use. Consequently, the Fink and Taylor criteria (and the earlier version) have been used in screening for catatonia in at least two case series studies.[25,26]

Concerns over Catatonia Rating Scales

In spite of the classical history of catatonic signs, there is much confusion over the reference definitions for the terms used in these rating scales. Definitions tend to vary from one scale to the other.[27] These include mannerisms versus stereotypy, catalepsy versus waxy flexibility, and mitgehen versus mitmachen. Most North American psychiatry textbooks fail to adequately define these terms. Each rating scale has the support of its own research group leading to lack of uniformity in catatonia rating scales. In research and clinical institutions, there may be differing views regarding the weight of scale items. Consequently, most of these scales are not routinely used to measure treatment response. The BFCRS is most commonly cited for treatment studies in the North American publications. These scales may also not be sensitive enough to measure treatment response.[27]

In view of the variety of neuropsychiatric and general medical conditions that can manifest with catatonic features, a generic catatonia rating instrument would be insufficient to capture catatonia in specific patient populations and consequently would need modification.[6] Wong et al[28] tested the BFCRS in a sample of patients with chronic schizophrenia where catatonic features are frequently encountered. Using Rasch analysis, the authors constructed a 20-item scale from the BFCRS that was useful in their population of chronic catatonics. They suggested that rigidity, grasp reflex, and autonomic abnormality (and perhaps impulsivity) do not measure the same construct as other BFCRS signs in chronically ill patients with schizophrenia.[28] The BFCRS Revised version was shorter and simpler that the original BFCRS and better suited to identifying catatonic signs in patients with chronic schizophrenia.[28]

Treatment Response in Katatonia

Peralta et al[29] and Barnes et al[30] remind us that catatonia is found across a wide range of psychiatric, neurological, and medical disorders, and this generally supports Kahlbaum’s idea of a unitary phenomenology and pathology. The response to lorazepam or electroconvulsive therapy (ECT) does not follow the primary psychiatric diagnosis. In fact, it is katatonia that responds to these treatments. While some authors have noted a trend toward less favorable improvement for catatonic symptoms in schizophrenia as compared to mood disorders, this has not been consistently or conclusively demonstrated. Nonetheless, the presence of katatonia predicts a favorable response of these signs to interventions like intravenous amobarbital, lorazepam (and other benzodiazepines), and ECT. Katatonia seems to be associated with a favorable prognosis, when diagnosed and treated promptly. There is clinical evidence to suggest that failure to diagnose katatonia or provide effective psychiatric treatment may result in a poor outcome.[30]

Directions for Future Research

The KANNER scale should be compared against a gold standard. We propose testing the KANNER rating scale against the BFCRS. While other scales have merit, there is limited experience with these scales in North America. The KANNER scale has three components; each should be tested separately (APPENDIX 2 PT 1 , APPENDIX 2 PT 2 , APPENDIX 2 PT 3 , APPENDIX 3 , APPENDIX 4). It may be useful to call together a consensus panel to propose criteria, evaluate rating scales, and identify treatment standards. This has been done for other neuropsychiatric illnesses, such as Lewy body dementia. Furthermore, since the KANNER is a neuromotor examination, a training video or DVD with patients or simulation actors might be helpful to achieve improved understanding of katatonia and create a core standardization in the rating of katatonia (Table 1).

Proposal

We propose the KANNER scale as a unifying instrument for quantifying katatonic core features (a) across the broad range of neuropsychiatric illnesses and (b) in the specific contexts of autism and pervasive developmental disorders. The KANNER scale is named after Leo Kanner (1894–1981), who described the neuromotor and neurodevelopmental features of autism, which are also features of katatonia.[13–15] We feel it is necessary to identify katatonia in patients with other neuropsychiatric illnesses, including schizophrenia and mood disorders, and due to general medical conditions (Table 1).
Based on our review and analysis from a large body of literature, the clinical signs for a possible and a probable diagnosis of catatonia are contained within the KANNER scale. Since there is no true gold standard for diagnosis, the sensitivity and specificity of this scale cannot be easily ascertained. Because the KANNER scale is based on an exhaustive and comprehensive review, we propose that it be adopted and tested prospectively for its clinical utility, sensitivity, and specificity.

Acknowledgment
This work is dedicated to the late Tressa D. Carroll who provided conceptual input.

References
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Category: Bipolar Disorder, Mental Disorders, Mood Disorders, Past Articles, Pervasive Developmental Disorders, Psychiatry, Review, Schizophrenia

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