CATATONIA Moderator : Dr Keertish Presenter : Dr Kavya
DIAGNOSTIC EVALUATION DIFFERENTIAL DIAGNOSIS MANAGEMENT PROGNOSIS COMPLICATIONS TAKE HOME POINTS OUTLINE
WHY CATATONIA
Syndrome of specific motor abnormalities closely associated with the disorders in mood, affect, thought and cognition Max Fink, Michael Alan Taylor,2003
INTRODUCTION Catatonia is a severe psychomotor syndrome associated with various psychiatric disorders and medical conditions. Psychiatric emergency
INTRODUCTION Although catatonia has historically been associated with schizophrenia and is listed as a subtype of the disorder, it can occur in patients with a primary mood disorder and in association with neurological diseases and other general medical conditions Prompt recognition and treatment with benzodiazepines or electroconvulsive therapy decreases the lethality of the catatonia
HISTORICAL BACKGROUND
DIAGNOSTIC EVOLUTION
2013 APA DSM-5 Three forms (catatonia as a specifier ),” “Catatonic disorder due to another medical condition” “Unspecified catatonia.” Requires 3 or more of 12 symptoms
Catatonia from the first descriptions to DSM 5. Journal of Psychopathology 2015;21:145-151
1992 WHO ICD-10 Two contexts Organic Catatonic Disorder (ICD-10 code F06.1) Catatonic schizophrenia (F20.2)- Prominently exhibits at least one of the catatonic features, for at least 2 weeks
ICD 11
EPIDEMIOLOGY • 9%-17% of patients with acute psychiatric illnesses MC in mood disorders (50%) 10-20% in schizophrenia 2% in other psychoses • 20% in primary medical or neurologic disease • 8% in BZD withdrawal 1/3 rd
CLASSIFICATION Malignant Non-malignant Excited Retarded Retarded Excited NMS Lethal catatonia Delirious mania Classic catatonia Van Den Eede & Sabbe (2004)
TYPES OF CATATONIA RETARDED EXCITED AUTISTIC MALIGNANT / LETHAL PERIODIC ICTAL The more frequently observed subtype, associated with signs reflecting a paucity of movement Associated with severe psychomotor agitation Severe psychomotor agitation with hyperthermia, altered consciousness, and autonomic dysfunction Stober et al (2002), is the first subtype of schizophrenia with confirmed genetic linkage, the susceptibility site being 15q15 (Hare & Malone,2004)Occurring in people with developmental disorders Seizure manifests itself as catatonia, is postulated to be due to involvement of the limbic system (Lim et al, 1986)
TYPES OF CATATONIA Measure if Impairment Acute Chronic Catatonic signs Greater Fewer Nutritional compromise More likely Less likely Dehydration More likely Less likely Automatic instability Inpatient Outpatient or residential Medical complications More likely Less likely Recent diagnostic procedures More likely Less likely Impairment in ADLs More severe Less severe Acute Vs Chronic Catatonia
ETIOLOGICAL CLASSIFICATION 1 2
PRIMARY CATATONIA Affective disorders ( MC ) Schizophrenia Schizoaffective disorder Post-partum psychosis Abrupt withdrawal of clozapine or benzodiazepine Dissociative stupor Obsessive compulsive disorder Autistic spectrum disorder
SECONDARY CATATONIA Typhoid fever Neurocysticercosis Prion disease Viral encephalitis Subacute sclerosing pan encephalitis Neurosyphilis Infections Autoimmune and inflammatory Cardiovascular Renal Metabolic Neurodegenerative disorders CNS Hematology Medications SLE or antiphospholipid syndrome* Anti-NMDAR encephalitis* Paraneoplastic encephalitis Multiple sclerosis Takotsubo cardiomyopathy Renal failure in dementia with Lewy body disease Wilson’s disease Hyponatraemia or hypernatraemia Glucose-6phosphate deficiency Westphal variant of Huntington’s disease Parkinson’s disease Familial frontotemporal dementia Posterior reversible encephalopathy Subdural hematoma Pontine and extrapontine myelinolysis Stroke Pernicious anemia Thrombotic thrombocytopenic purpura Venlafaxine-associated hyponatraemia Pegylated interferon- α 2 b and ribavirin for hepatitis C Paliperidone palmitate Dexamethasone Quinolones Zolpidem withdrawal Temazepam withdrawal Lorazepam withdrawal Clozapine withdrawal* Manganese neurotoxicity Clonazepam/benzodiazepine withdrawal Ziprasidone Lithium toxicity Tramadol and meperidine Azithromycin Levetiracetam Efavirenz
ETIOPATHOGENESIS
PROPOSED ETIOLOGICAL MODELS Motor circuitry model Neurotransmitter model Epilepsy model Endocrine model Immune model Fear model Autonomic nervous system model
NEUROCHEMICAL BASIS
HYPOTHESIS Northoff (2002) An alteration in ‘top-down modulation’ of basal ganglia due to deficiency of cortical gamma-amino butyric acid (GABA). Hyperactivity of glutamate, the primary excitatory neurotransmitter Osman & Khurasani (1994)- sudden and massive blockade of dopamine. Yeh et al, 2004- Clozapine-withdrawal Catatonia. PET scan- Decreased density of GABA in sensori -motor cortex NMS- Reduced GABA in CSF.
NEURO-CHEMICAL BASIS GABA A GABA B D2 5 HT1A 5 HT2A Glutamate hyperactivity at NMDA receptor CATATONIA
GABA
NEURAL CIRCUITRY OF CATATONIA Low GABA in PFC Low DA in BG High glutamate in parietal cortex
AFFECTIVE SYMPTOMS Dysfunction in MOFC and GABAnergic MOTOR SYMPTOMS Alteration in “vertical modulation ” of basal ganglia by GABAnergic mediated OFC VEGETATIVE ABNORMALITIES alteration in midbrain and brainstem nuclei BEHAVIORAL SYMPTOMS Deficits in behavioral inhibition and LOFC activity
DIAGNOSTIC EVALUATION
CLINICAL FEATURES Ambitendency Automatic obedience Aversion Catalepsy Echolalia Echopraxia Excitement Forced grasping Gegenhalten Grimacing Immobility Logorrhoe Mannerisms Mitgehen Mutism Negativism Obstruction Perseveration Posturing Psychological pillow Rigidity Staring Stereotypies Stupor Verbigeration Waxy flexibility Withdrawa l IMMOBILITY and MUTISM are the MC signs, each present in over 90% of patients STUPOR is the classic and most striking catatonic sign. It is a combination of immobility and mutism
RATING SCALES Most commonly used is the Bush-Francis Catatonia Rating Scale Another scale is Modified Rogers Scale Rating scales are useful in Check and quantify catatonic signs in suspected patients Monitoring the progress and response to the treatment Disadvantage : Presence of non specific signs may lead to over diagnosis of catatonia
BUSH-FRANCIS CATATONIA RATING SCALE
BUSH-FRANCIS CATATONIA RATING SCALE Excitement Immobility/stupor Mutism Staring Posturing/catalepsy Grimacing Echopraxia/echolalia Stereotypy Mannerisms Verbigeration Rigidity Negativism Waxy flexibility Withdrawal Impulsivity Automatic obedience. Mitgehen Gegenhalten Ambitendency . Grasp reflex Perseveration Combativeness Autonomic abnormality Severity rated on a scale of 0-3 Uncertain items are rated as 0 ≥2 from 1-14 items are necessary Items 1-23 are for severity Brief and sensitive
EXAMINATION FOR CATATONIA PROCEDURE EXAMINES Observe patient while trying to engage in a conversation Activity level Movements Speech Examiner scratches head in exaggerated manner Echopraxia Attempt to reposture , instructing patient to "keep your arm loose" - move arm with alternating lighter and heavier force. Waxy flexibility
EXAMINATION FOR CATATONIA PROCEDURE EXAMINES Take the hand of the patient as if you are examining his pulse and leave his hand Posturing Patient does the exact opposite of what is asked to do Patient does not carry out any orders Active Negativism Passive Negativism Extend hand stating "DO NOT Shake my hand". Ambitendency Forced grasping
EXAMINATION FOR CATATONIA PROCEDURE EXAMINES Reach into pocket and state,"Stick out your tongue, I want to stick a pin in it". Automatic obedience Check for grasp reflex. Grasp reflex Some patients oppose all passive movements with the same degree of force as that of which is been applied by the examiner. (Asked to co-operate) Gegenhalten or opposition
EXAMINATION FOR CATATONIA PROCEDURE EXAMINES If examiner rapidly touches the palm and steadily withdraws his finger the patient’s hand follows the examiners hand like an iron following magnet. Magnet reaction Patients body can be put to any position without any resistance although he has been instructed to resist all movements. Incooperation or mitmachen
EXAMINATION FOR CATATONIA PROCEDURE EXAMINES Ask patient to extend arm. Place one finger beneath hand and try to raise slowly after stating, "Do NOT let me raise your arm". Mitgehen Anglepoise lamp
DIFFERENTIAL DIAGNOSIS Neuroleptic malignant syndrome Malignant hyperthermia Serotonin syndrome Anticholinergic syndrome Elective mutism Locked in state Stiff man syndrome Parkinson’s disease Metabolic induced stupor
LETHAL / MALIGNANT CATATONIA Severe form of Catatonia Presents with initial catatonic excitement, followed by Stupor Agitation Autonomic symptoms-fever, hypotension and diaphoresis Supportive care, stop offending agent ECT should be administered early, since the condition has a high rate of mortality if it is not rapidly and effectively treated
CATATONIA AND NMS Catatonic signs and low serum iron prior to the onset of neuroleptic malignant syndrome 0.07%-1.8% of catatonic patients treated with antipsychotic medications developed neuroleptic malignant syndrome In view of high mortality associated with NMS (20-30%), it is recommended to avoid antipsychotic drugs in acutely catatonic patients Once catatonic symptoms have been treated by benzodiazepines or ECT and patients are eating, drinking, and walking, antipsychotic treatment can be initiated safely
CATATONIA AND NMS
DIFFERENTIAL DIAGNOSIS Diagnosis Characteristics similar to catatonia Distinctive characteristics Non-catatonia stupor Immobility, mutism , absence of response to stimuli Precipitating cause (e.g. cranial trauma, anoxia, drug intoxication Encephalopathy Acute onset, bizarre behavior, altered mental state Generally associated with a somatic condition, reversible with treatment of the underlying medical condition Stroke Acute onset, can present with immobility, mutism and /or altered mental state History of cerebrovascular disease, focal neurological signs, CT/MRI confirmation Stiff man syndrome Immobility, fixed posture Rigidity and spasms caused by sudden stimuli Parkinson’s disease Immobility, altered mental state, comorbid mood disorder Symptoms improve with administration of dopamine agonists and anticholinergics, cogwheel rigidity Locked-in syndrome Immobility, mutism Total paralysis with only vertical eye movement and only winking, associated with lesions of the pons and cerebral peduncles
DIFFERENTIAL DIAGNOSIS Diagnosis Characteristics similar to catatonia Distinctive characteristics Malignant hyperthermia Immobility, mutism , altered mental state, instability of autonomic nervous system Hyperthermia due to inhaled anaesthetics , autosomal dominant, diagnosed with muscle biopsy Epileptic state Immobility, mutism , altered mental state, nizarre behaviors Epileptiform activity by EEG Autism Mutism , immobility, echolalia, echopraxia Chromic with onset at infancy Obsessive-compulsive disorder (severe forms) Echolaloa /repeated echopraxia , comorbid mood disorder Anxiety, knowledge of compulsive behavior Elective mutism Mutism Possible personality disorder or underlying paranoia
DIAGNOSTIC EVALUATION History Detailed psychiatric and neurological examination Investigations to rule out organicity Investigations to look for NMS Assessment of co-occurring medical/physical problems Based on the history and examination patient may need to admitted either in a psychiatric or a medical ward
FIRST LINE INVESTIGATIONS Catatonia is functional or organic CBC Renal fuction test LFT TFT Blood glucose Creatine phosphokinase ECG Chest X-ray CT / MRI EEG CSF analysis Urine culture Blood culture Test for HIV / Syphilis Auto antibody(IgG against NMDAR) Heavy metal & drug screen Serum iron OTHER INVESTIGATIONS
MANAGEMENT Care possibly in ICU setting Attempt to observe patient indirectly, at least for a brief period, each day Check chart for reports of previous 24-hour period -oral intake, I/O Chart, vital signs, and any incidents Adequate hydration, correction of electrolyte disturbances Management similar to comatose patients
TREATMENT Majority of patients respond to Benzodiazepines (GABA-A Agonism ) Patients are not responding / partially responding to Benzodiazepines can be effectively treated with ECT In organic catatonia, treat the underlying cause. Even in these patients Benzodiazepines and / or ECT can be very useful Lorazepam challenge test 1 mg intravenous lorazepam If no response after 5 min, administer another 1 mg If positive Treat with lorazepam increasing the dose up to 24 mg/day If negative Bilateral electroconvulsive therapy
TREATMENT - BENZODIAZEPINES Characteristic feature is its striking responsiveness to benzodiazepine treatment Low dose lorazepam is the drug of choice, as it has the strongest evidence base from case reports It can be given orally(3-6mg) or intra – muscularly(1-2mg) Diazepam orally / by IV infusion is another option Response to Benzodiazepines, is immediate and dramatic
ADVANTAGE OF BZD’S OVER ECT Easily administered High margin of safety Short latency Even at high doses, it doesn’t pose the cardiovascular challenge as the electroconvulsive therapy; Doesn’t require anesthesia; ECT is associated with greater stigma Benzodiazepines are the first line of treatment
ELECTROCONVULSIVE THERAPY Oldest and best modality of treatment Partial or no improvement to benzodiazepines Lethal catatonia To treat psychiatric comorbidities Response rate 80%-100% Superior to any other therapy
TREATMENT – OTHER OPTIONS NMDA receptor antagonists – Amantadine and Memantine Lithium Carbamazepine Antiepileptics – topiramate and valproic acid Atypical antipsychotics
TREATMENT
TREATMENT ALGORITHM Exclude of treat underlying physical illness Rules out NMS Lorazepam up to 4 mg/day – start with 2 mg and give a further 2 mg if not effects after three hours. Use IM route from then on Loranzepam high dose 8-24 mg/day Consider SGA, e.g. clozapine, olanzapine ECT 4 Follow benzodiazepine/ECT protocol oppsite Stupor in the context of affective/conversion disorder Stupor in the context of psychotic illness NMS Possible No response after 1-2 days Not taking antipsychotics NMS ruled out No response after 1-2 days No response after 1-2 days
PROGNOSIS Early diagnosis and treatment is the key In general, the prognosis for the acute catatonic phase seems to be good Patients with longstanding catatonia or a diagnosis of schizophrenia may be less likely to respond. However, the long-term prognosis probably depends on the underlying cause of the catatonia.
COMPLICATIONS Dehydration and its attendant complications. Deep-vein thrombosis McCall et al (1995) have highlighted the increased risk of death due to pulmonary embolism in patients with persistent catatonia Significant risk of harm to self and others
SUMMARY Catatonia is an important phenomenon in both psychiatry and general medicine Common in psychiatric patients especially affective disorders Early recognition is of utmost importance in order to provide optimal treatment and to reduce the associated morbidity and mortality The lorazepam challenge test validates the diagnosis of catatonia. Catatonic patients are most susceptible to NMS, it is recommended to avoid antipsychotic drugs in acutely catatonic patients. An integrated multidisciplinary approach is important for both diagnosis and treatment of the catatonic patient.
REFERENCES Fink M, Michael Alan Taylor. Catatonia : a clinician’s guide to diagnosis and treatment. Cambridge ; New York: Cambridge Univ Pr ; 2006. Casey PR, Brendan K. Fish’s clinical psychopathology : signs and syptoms in psychiatry. London: Royal College Of Psychiatrists; 2007. Freedman AM, Sadock BJ, Kaplan HI. Comprehensive textbook of psychiatry, 3. Vol. 1. Baltimore ; London: Williams & Wilkins; 1980. World Health Organization. The ICD-10 classification of mental and behavioural disorders = ICD-10 : clinical descriptions and diagnostic guidelines. Geneve: World Health Organization; 1992. American Psychiatric Association. DSM-5 classification. Arlington, Va : American Psychiatric Association; 2013.