Catatonia: Breathing life into an old concept

Dr. Vishnu Pradeep, Dr. Tareq Ghani, Dr. Louise Tansey CAMHS/Intellectual Disability Services

TABLE OF CONTENT• CASE PRESENTATION • HISTORY • SYMPTOMATOLOGY • PATHOPHYSIOLOGY • MANAGEMENT • PROGNOSIS • CONCLUSION

CASE PRESENTATION

INTRODUCTION

“The little patient lies perhaps for hours or days seemingly in a sort of mystical contemplation, with limbs more or less rigid, or fixed in strange postures; sometimes there is insensibility to impressions, while in other instances vague answers are given, or there is actual incoherent raving”

-Henry Maudsley on ‘cataleptoid insanity’ in children, 1867

BACKGROUND

Asked by the Paediatric team to review:

13 year old female with 4/52 history of psychomotor retardation, recent discontinuation of Risperidone 0.25 mg, seen by the ID Services for management of emotional and behavioural difficulties on the background of Moderate Intellectual Disability and ASD.

BACKGROUND

•13 year old female with Moderate Intellectual Disability• ASD• No diagnosis of epilepsy • No previous diagnosis of depression/psychosis• Attends East Limerick Children’s Services

SOCIAL HISTORY

• Lives at home with Mother and two younger brothers (12 & 6)• Father living abroad – moved out since 2006• Attends St. Vincent’s school in Lisnagry – 5 days a week• Gets respite with St. Joseph’s Early Intervention Team

PREVIOUS ADMISSION/BACKGROUND HISTORY • Previous admission (07.07.16) to paediatrics unit• Presenting complaints

• Progressive regression in behaviour for past 2/12• Since May/2016 – noticed to be intermittently staring into space • Needed constant prompting to do things, such as dressing and self hygiene • No prompting was needed prior• Behavioural changes coincided with mother leaving (?PTSD)

• On admission:• Noted periodic staring episodes • Neurological Exam – Normal • Bloods – Normal

PREVIOUS ADMISSION/BACKGROUND HISTORY• Social History on admission:• Mom had to go to Ghana for 2 weeks as her mother had passed away• Pt. had to go into respite (two different Places)

• Medication on Admission:• Risperidone 0.25mg nocte - ? Compliance

• Withheld then D/C (8.7.16)

INITIAL MANAGEMENT - 12th August, 2016 Management to-date: Admitted under Paediatric team 09.08.16 with raised temperature,

labile BP and tachycardia. Covered for Encephalitis with Acyclovir and Ceftriaxone for 3/7.

INITIAL MANAGEMENTInvestigations to-date: • CT-Brain: high signal in sub cortical white matter in Left Frontal and

temporal lobe. • MRI- Brain: focus of high-signal in the left frontal lobe and within the

left temporo-parietal lobe, broad differential: demyelination, micro vessel ischaemic changes less likely. • XR-Chest: Normal • ECG: Sinus tachycardia rate 144• EEG: No epileptiform changes (done 4 weeks ago) • ABG: Normal findings • LP: Not done

INITIAL MANAGEMENT• WBC 15.32 (raised) • Hb 14.1• MCV, MCH, PV – Normal • Neutrophil 9.61 (raised) • U&E, LFTs – Normal • Electrolytes – Normal • Urine Dipstick/tox screen – Negative • ASO – 319 (normal) • RF <12 (normal) • Prolactin – 180 (normal) • Glucose 5.9 • CK -1993 (raised) 622 • TFTs – Normal • CRP – unavailable

INITIAL MANAGEMENTAdditional team input: • Neurology: Ruled out NMS or Encephalitis; thorough

infective/autoimmune/toxicology screen sent. • Lyme – Negative• ANCA, ANA – Negative• HIV, Syphilis Serology – Negative

• Psychiatry-on-Call: Received stat dose 2 mg Lorazepam (the night before)

BEDSIDE ASSESSSMENT • Vitals: HR 141, O2 99%, BP 123/72 <-> 159/82, Temperature 36.9 • Appearance: young girl of Nigerian origin, casually dressed, appropriately

kempt, laying in bed, vacant gazing at the roof, laughing intermittently, breath holding, significant psychomotor retardation• Speech: muted• Affect: flat, restricted • Mood: (0bjectively) – ambivalent, stuporous • Not responding to external stimuli (unable to establish if psychotic features

present) • Waxy flexibility, Automatism

RECOMMENDATION • Lorazepam Challenge• 0.5 mg Lorazepam TDS to be increased to 1 mg TDS tomorrow • Monitor closely for any excessive sedation/respiratory compromise

• Continue monitoring in Paediatric HDU• DVT Prophylaxis • Input/output monitoring; IV Fluids 1.5-2 L/day • Risk of Silent Aspiration – SALT input • Repeat CK , U&E, Electrolytes• Daily Psychiatry Input

PROGRESS – 17th August to 22nd August, 2016 • Intervention: • Lorazepam increased to 1.5mg TDS x 5/7 • Further increase to 2mg TDS x 2/7 (22/8/16)

• Observation: • Vitals stable• Able to answer simple questions – one word answer• Lying in bed – watching TV; Intermittent staring • Holding saliva – needs prompting to swallow • Continues to require prompting for other activities

PROGRESS – VIDEO 18th August, 2016 • Consent Obtained • Recorded with Mom’s Permission

PROGRESS – 24th August, 2016 • Intervention: • Lorazepam increased to 2.5mg TDS x 2/7

• Observation: • Mom – Not back to previous functional level • No Significant improvement since admission

• Recommendation: • Continue observation for over sedation

PROGRESS – 26th August, 2016 • Intervention: • Lorazepam increased to 3mg TDS x 6/7

• Observation: • Observed in play room reading• Engaging & Responding well to 1:1 attendance • No over sedation • Mom – noted slight improvement

• Intermittent smiling and response

PROGRESS – 1st September, 2016 • Intervention: • Lorazepam increased to 3.5mg TDS x 4/7

• Observation: • Smiling more, more animated on commands • Core features of Catatonia – resolved

• E.g. Mutism, Waxy flexibility • Has tendency to stare into space• Automatic obedience – e.g. Sings on request

PROGRESS – 2nd to 5th September, 2016

2/9/16•Observation:

• Attending Ark School • Engaging and enjoying being back to school • Requires ongoing prompting with activities

5/9/16•Intervention:

• Lorazepam increased to 4mg TDS x 2/52

•Observation: • Bright and more aware of her surrounding

PROGRESS – 19th September, 2016 • Observation: • Maintaining improvement • Increased awareness of surroundings • Improved self care with less prompting • Better communication

• Intervention: • Began reducing Lorazepam by 0.5mg every 3/7• 4mg BD & 3.5mg nocte

PROGRESS – 22nd – 26th September, 2016

22/9/16•Intervention: • Increased reduction of Lorazepam by 1mg every 3/7• 4mg mane & 3mg BD

26/9/16•Observation: • Continued improvement

•Intervention: • Decided to reduce Lorazepam further to 3mg TDS

PROGRESS – 29th September, 2016 29/9/16

•Observation: • Doing well• Spent weekend with Mom at home:

• noted improvement – more like her previous self

•Intervention: • Decided to further reduce lorazepam to 3.5mg mane, 1mg tarde, 3.5mg

nocte

PROGRESS – 3rd -4th October, 2016 3/10/16

•Observation: • Mixed statements re-progress • Had a good day in school• In play-room: Less engaging, Less animated, Holding saliva • Monitor on current dose

4/10/16 •Observation: • Unusual hand movement noted• Not in good form & appeared in low mood (some what withdrawn)

PROGRESS – 6th October, 2016 • Observation: • Withdrawn, less interactive, not as bright • School concerned • Increased impulsivity • Over activity

• Intervention: • Decided to increase Lorazepam to 3mg TDS

PROGRESS – 10th October, 2016 • Observation: • Showing signs of improvement• Remains somewhat overactive• Sleep disturbance – early wakening • Impulsive • Observed to be elated

• Intervention: • Commenced on Aripiprazole 2mg mane

PROGRESS – 13th – 14th October13/10/16

•Observation: • Remained Hyperactive • Over-eating (looking for food everywhere both home and school) • Sleep disturbed

14/10/16•Intervention: • Increased Aripiprazole to 5mg mane• Began reducing Lorazepam by 0.25mg every 7/7 to 3mg BD & 2.75mg nocte

PROGRESS – 18th October• Observation: • Impulsive behaviour subsided, No signs of agitation • Positive feedback from school• Sleep remains disturbed

• Intervention: • Reduced lorazepam further to 3mg BD & 2.5mg nocte

PROGRESS – 21st – 26th October 21/10/16

•Observation: • In much better form & Sleep has improved

•Intervention: • Increased Aripiprazole to 7.5mg mane

26/10/16•Observation: • Continues to improve• Engaging and needing less prompting

•Intervention: • Further reduction in Lorazepam to 3mg BD & 2.25mg nocte

DISCHARGE PLAN – 1st November, 2016 • Discharged on

• Aripiprazole 7.5mg mane • Lorazepam 3mg BD and 2mg nocte

• Home care (Bluebird care) organized - • Care staff to administer night medication and on weekends • To organize additional activities

• School (East-limerick School Services)• Staff to administer morning and afternoon doses

• Psychiatry team • Home visits • School visits

POST DISCHARGE• School feedback• Doing well• Engaging • Periodic saliva holding reported - ? Behavioural

• Home Feedback• Doing well• More talkative and engaging • Mom says back to previous self

ADDITIONAL SUPPORTS IN PLACE • Bluebird care• Monday to Friday – 1 hr/day in the evening to give meds • Weekends – 3 hrs/day (meds administration and social engagement)

• Respite • D.O.C • Overnight

MANAGEMENT CHALLENGES • During hospital stay • Pharmacological Management & Response • 1:1 special • Continues assistance • Hospital environment – Social isolation • Mom lack understanding – ? Cultural views

MANAGEMENT CHALLENGES • Post discharge • Mom lack of understanding of complexity • Mom extremely dependent on external support re-management • Ambivalence towards long term care • Unavoidable need for shared care • Difficulties in establishing aetiology:

• ? BPAD• Increased risk of relapse with further reduction in medication • Understanding Triggers

Catatonia: The past and the

present

INTRODUCTION

Photograph of a group of catatonic patients. This photograph appeared in the fifth edition of Emil Kraepelin's Psychiatrie (Leipzig Johann Ambrosius Barth, 1896).

INTRODUCTION “Ever since the introduction of the concept of catatonia,

it has been the focus of debate, a major point of discussion being its nosological status. The question

rises whether it is to be considered a syndrome with a wide variety of causes and clinical signs or a distinct

clinical entity”.

E. VANCAESTER and P. SANTENS Dept. of Neurology, University Hospital Gent, Gent, Belgium

ORIGINS

The concept of catatonia, literally meaning “to stretch tight”, goes back to the original description by Karl Ludwig Kahlbaum in 1874.

ORIGINS In his research of catatonia, he published the monograph, Die Katatonie oder das Spannungsirresein, in which he characterizes the disorder as• ‘disturbance in motor

functionality’ that represents a phase in a progressive illness that includes stages of mania, depression and psychosis that typically ends in dementia

ORIGINS• Two major view-points emerged during the early 19th

century: • one view supported the proposal of catatonia as a disease

of its own• The opposing view was that it was a complication of

different pathophysiology's and not a distinctive disease

ORIGINS• Karl Jaspers:• portrayed catatonia as an illness with special characteristics like opposing

pairs of symptoms (negativism vs automatic obedience).• Kurt Schneider: • psychology of catatonia, which he found unknowable:

‘‘Sometimes it seems as though the patient is like a dead camera: He sees everything, hears everything, understands everything and yet is capable of no reaction, of no affective display, and of no action. Even though fully conscious he is mentally paralyzed.’’• He considered Catatoinia a complication of many illnesses and rejected

Kraepelin’s formulations

ORIGINS• Mayer-Gross:• Felt that catatonia was resolutely Kraepelinian that catatonia was a type of

schizophrenia.• George Kirby (1913) • pictured catatonia as typically occurring among patients with manic-

depressive illness• August Hoch(1921) • described 25 psychiatric patients in stupor. Thirteen with manic-depressive

illness had a favorable prognosis and 12 with general medical illnesses or schizophrenia had a poor prognosis

ORIGINS • Lange (1922) • reported an experience with 200 patients found catatonia to be more

common among the manic-depressive patients than among those with dementia praecox.

• Taylor and Abrams: • 4 publications between 1973 and 1979, reported catatonia to be more

common among manic and depressed patients• Gelenberg • described catatonia among patients with neurologic and general medical

illnesses

CATATONIA AND ICD • 6th Ed. 1948: ‘‘catatonic type’’ among the ‘‘schizophrenic disorders.’’• 10th. Edition 1992: unchanged;

‘‘For reasons that are poorly understood, catatonic schizophrenia is now rarely seen in industrial countries, though it remains common else where.’• Catatonic schizophrenia (category F20.2)• Pt. With severe depression is in a stupor- a diagnosis of ‘severe depressive episode with

psychotic symptoms’ (F32.3)• manic stupor will be diagnosed as having ‘mania with psychotic symptoms’ (F30.2)• Catatonia due to physical causes is diagnosed as ‘organic catatonic disorder’ (F06.1).

DEFINITION • Catatonia is a syndrome that encompasses

more than two dozen signs that describes in essence a “disturbance in motility” • Stupor is the classic and most striking

catatonic sign. It is a combination of immobility and mutism• The patient is able to maintain the same

posture for long periods. An extreme version of posturing is catalepsy (rigid; cant feel pain)• highly uncomfortable postures, which are

maintained for a considerable period of time

FEATURES • Stupor• Posturing• Waxy flexibility (cerea flexibilitas)• Negativism (Gegenhalten)• Automatic obedience• Ambitendency• Psychological pillow• Forced grasping• Obstruction

• Echopraxia• Aversion• Mannerisms• Stereotypies• Excitement• Speech abnormalities• Echolalia, logorrhoea and

verbigeration

RATING SCALES • In general the syndrome is poorly

recognised, some of the commonly used rating scales are as indicated below: • Bush–Francis Catatonia Rating Scale (BFCRS)

• most widely used• 23 items• shorter, 14-item screening version

• Bush–Francis Catatonia Screening Instrument (BFCSI)• Modified Rogers Scale (MRS)• Lorazepam Challenge Test (Fink & Taylor, 2003)

• Involves administering 1-2mg Lorazepam IV• High response rate – 75-100% (Hawkins et al. 1995)

PREVALENCE • Very rare • Commonly under-recognised and under-diagnosed•9%–15% of patients admitted to a typical acute care

psychiatric service meet diagnostic criteria for catatonia (Rosebush, 2010)•More common in mood disorders (28%-31% of catatonic

patients had mixed mania or mania) and only 10%-15% had underlying diagnosis of schizophrenia

SUBTYPES• Non-malignant

• Psychomotor retardation/withdrawn- appear awake and watchful, but with minimal spontaneous speech and movement. Stupor, mutism, negativism, and posturing are common signs

• Excited - excessive purposeless motor activity associated with disorganised speech, disorientation, aggression, and violence

• Periodic – most frequently associated with Bipolar disorder. Involves fluctuations between stupor and excitement (Fink & Taylor, 2003)

• Malignant catatonia- escalating fever and autonomic instability • Resembles neuroleptic malignant syndrome (NMS) • Some authors also consider toxic serotonin syndrome as a subtype of malignant

catatonia

ETIOLOGY • Wide variety of causes reported (Takaoka & Takata, 2003)• Psychiatric conditions:

• Schizophrenia, Bipolar disorder, PTSD, Eating disorder, Depressive disorder • Medical conditions:

• Autoimmune disorders, Metabolic disturbance• Neurological conditions:

• Encephalitis, Stroke, Seizure disorders • Others

• Ecstasy, phencyclidine, inhalant, steroids, neuroleptics, abrupt withdrawal of BNZ

PATHOMECHANISM • Exact cause of catatonia has not been

elucidated• PET Scan has identified abnormalities in

metabolism bilaterally in the thalamus and frontal lobes• Moskowitz (2004) suggests that catatonia may

be understood as an evolutionary fear response, catatonic stupor may represent a common ‘end-state’ response to feelings of imminent doom.

Catatonia in children & Adolescents • Literature regarding catatonia in children & adolescents is sparse• Wing & Shah, 2000. described Features particular to child and

adolescents presentation:• Increased slowness affecting motor and verbal responsiveness• Difficulty initiating and competing actions• Increased reliance on cueing by others• Increased passivity and lack of motivation • Reversal of circadian rhythms • Parkinsonian features• Excitement and agitation• Increased repetitive and ritualistic behaviour

INVESTIGATIONS• Comprehensive physical examination,

with specific emphasis on neurological signs• Bloods- FBC, Renal, LFT, TFT, Glucose, CK• Drug Screen• ECG• CT/ MRI• EEG• Culture• LP• Auto-Antibody Screen

MANAGEMENT• Supportive care/ high level of nursing care • Treatment with subcutaneous heparin, urinary catheterization• May require IV fluids, Nasogastric tube feeds or PEG tube placement. • Benzodiazepines are the drugs of choice for catatonia

• Lorazepam was the most commonly used treatment, resolving symptoms in 70% of reported cases.

• Other benzodiazepines such as diazepam, oxazepam, and clonazepam have also been reported to treat catatonia

• 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

• continue the benzodiazepines until the causative illness has been fully treated

MANAGEMENT• ECT

• ECT alone resulted in resolution of symptoms in 85%

• In malignant catatonia, the response to ECT was 89%,

• Emergency ECT is the treatment of choice for malignant catatonia

• Identified as a definitive treatment (Fink & Taylor, 2009)

• NMDA Antagonists (Amantadine, Memantine) • When ECT or Benzodiazepines fail

• Topiramate, Carbamazepine or combination of lithium and an antipsychotic may be an option in treatment-resistant catatonic stupor

ECT & ETHICS • Although ECT has been reported to effective in treating catatonia,

several adverse affects are unavoidable (Takaoka & Takata, 2003)• Several studies reported transient but frequent adverse effects:• Post ECT asthenia • Complaints of amnesia • Memory loss• Headaches• Post ECT confusion • Nausea, Vomiting & Vertigo

ECT & ETHICS • In an article published by Cohen et al. he insisted that there no valid

grounds to banning ECT as a treatment for catatonia is adolescents• Zaw et al., 1999. sought two independent second opinions in their

ECT treatment of a catatonic child. Proposed that:• “For children aged 12 and under the evaluation and concurrence of two

psychiatrists who are experienced in the treatment of children and in ECT and otherwise not involved with the treatment and care of the patient are necessary”• “For those aged 13 and above, one external reviewer should be involved”

MANAGEMENT – ANTIPSYCHOTIC USE • Not recommended during a catatonic phase• Catatonia represents a highly significant risk factor for subséquent

neuroleptic malignant syndrome• Atypical antipsychotics may have a role in the treatment of non-

malignant catatonia (Van Den Eede et al 2005) • Multiple case reports and retrospective studies indicating the successful

treatment of catatonia with atypical antipsychotics (olanzapine, risperidone, ziprasidone, aripiprizole, and clozapine)• Few reports of atypical antipsychotics causing catatonia, though these

studies were largely in patients with schizophrenia and only one focused on a patient with a medical illness

MANAGEMENT – ANTIPSYCHOTIC USE • During a prospective follow-up of 82 patients that had received

antipsychotics at some point when catatonic, NMS developed in three cases (3.6%), a substantially higher incidence than the estimated incidence of 0.07–1.8% in all antipsychotics-treated patients. (Rosebush et. al 2010) • The risk of worsening catatonia appears greater with neuroleptics and

antipsychotics with higher D2-blockade and a higher potential of causing extrapyramidal side effects, but a worsening of catatonia and precipitation of NMS has also been reported in association with, e.g., olanzapine.

PROGNOSIS • Two-thirds show marked improvement or remission• High incidence of recurrent catatonic episodes was

reported for idiopathic catatonia and catatonia due to affective disorders• Following ECT high relapse rate within a year• continuation ECT is an efficacious treatment for

maintaining response (Suzuki et al 2005)• “Chronic catatonia” in the context of schizophrenia is

phenomenologically different and less responsive to either benzodiazepines or ECT

PROGNOSIS • A longer illness duration, the presence of mutism, third-person

auditory hallucinations and ‘made phenomena’ (in which the individual feels he is being made to do something) predicted a poor response, whereas the presence of waxy flexibility predicted a good response• Van Waarde et. al have examined predictors of response to ECT in 27

catatonic patients and found improvement to be significantly associated with younger age, and the presence of autonomic dysregulation, especially higher body temperature

DIAGNOSTIC DIFFICULTIES

CATATONIA AND AUTISM • Motor disorder

characterized by stereotypy, rigidity, mutism and posturing • Rigidity is less in Autism • Unable to clearly

delineate prodromal features in Autism

CATATONIA AND NMS • Life-threatening • Antipsychotic can

worsen or trigger NMS in Catatonia• Rigidity in NMS• Automatism in NMS

MENTAL HEALTH/CULTURAL IMPACT• Mental health is a socially constructed and

defined concept, implying that different societies, groups, cultures, institutions and professions have diverse ways of conceptualising its nature and causes, determining what is mentally healthy and unhealthy, and deciding what interventions, if any, are appropriate. • Mental illness is a taboo subject that attracts

stigma in much of Africa. (Mary Amuyunzu-Nyamongo, 2014)

MENTAL HEALTH/CULTURAL IMPACT• A study conducted in Uganda revealed that the term ‘depression’ is

not culturally acceptable amongst the population.• While another study conducted in Nigeria found that people

responded with fear, avoidance and anger to those who were observed to have a mental illness. • The stigma linked to mental illness can be attributed to lack of

education, fear, religious reasoning and general prejudice

MENTAL HEALTH/CULTURAL IMPACT• Social stigma has meant that in much of Africa mental illness is a

hidden issue equated to a silent epidemic. Many households with mentally ill persons hide them for fear of discrimination and ostracism from their communities. • Girls from homes known to have mental illness are disadvantaged

due to the fact that a history of mental illness severely reduces their marriage prospects

CONCLUSION/TAKE AWAY POINTS • Catatonia is a severe psychomotor syndrome with an excellent prognosis if

recognized and treated appropriately. The treatment of catatonia in children and adolescents should follow the same principles as in adults. • Great care should be taken to avoid (medical) complications.• Although a number of pharmacological agents have been tried successfully in

catatonia, rarely, if ever, the effect is as immediate and dramatic as seen with benzodiazepines. • If benzodiazepines fail (inadequate or transient response, excessive sedation),

ECT should be started without delay. If the underlying condition warrants ECT-treatment, or in life-threatening situations like malignant catatonia or NMS, ECT is the treatment of first choice.

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