Catatonia: The Forgotten Syndrome

CATATONIA: The Forgotten Syndrome

Mr. Jones was a 56-year-old husband of three children and a successful CEO. He was brought to the hospital by his wife who was concerned about her husband’s sudden change in behavior. “I woke up yesterday and found him lying in bed staring at the ceiling. He wouldn’t make eye contact with me and has spoken only three words in the past 24 hours. He makes odd noises and repeats phrases that don’t make any sense. Could he have suffered a stroke?” Mr. Jones suffers from post-traumatic stress disorder and bipolar disorder and has been adherent with his medications. He appeared disheveled and has not eaten in over 24 hours.
On examination, Mr. Jones was not responsive. He did not make eye contact, but his eyes were open, and he appeared awake. His eyes blink to threats which suggests he is not blind. He resists any attempts to move his upper and lower extremities. Neuroimaging of his brain appears normal, electroencephalography (EEG) shows no signs of epileptic foci (i.e., no seizure activity), and his labs are all normal. The astute clinician suggests an intravenous (IV) Lorazepam (Ativan) challenge for suspected Catatonia. After just two doses, Mr. Jones begins to respond normally, moves spontaneously, and asks why he was brought to the hospital.

What is Catatonia?

Catatonia is a psychomotor disorder whereby affected individuals display abnormal behaviors and movements. Catatonic behaviors can be categorized as withdrawn or excited, with the withdrawn state more common.
Symptoms of catatonia may include mutism or impoverished/quiet speech, reduced interaction with the environment (i.e., stupor), negativism (i.e., resisting movement by others), increased motor tone/rigidity, posturing and grimacing, automatic obedience, ambitendency, echolalia (i.e., repeating or mimicking others’ words), stereotypy (repetitive movements or speech), verbigeration (i.e., An obsessive repetition of meaningless words and phrases, especially as a symptom of mental illness), echopraxia (i.e., mimicking others’ movements), extreme anxiety/fear, and impulsive/bizarre behavior. 
Although the signs and symptoms of Catatonia have been described in the literature for hundreds of years, it wasn’t until the 1870s that Karl Kahlbaum characterized catatonia as a distinct syndrome.
In an effort to establish a nosology for mental disorders, Emil Kraepelin further expanded upon Kahlbaum’s work and classified catatonia, hebephrenia and dementia paranoides as a single entity he termed “dementia praecox” (dementia of the young).
Eugen Bleuler (1907), inspired by Kraepelin, adopted the view that catatonia was part of a group of severe idiopathic deteriorating psychoses, which he renamed the “schizophrenias.”
It is important to mention that Kraepelin and Bleuler both recognized catatonic symptoms could emerge from mood disorders, but it wasn’t added as a specifier until many years later. Today, we recognize catatonia as a syndrome associated with psychotic disorders, mood disorders, neurological diseases (e.g., stroke, multiple sclerosis, Parkinson’s disease, epilepsy), toxic-metabolic states (i.e., illicit drugs, withdrawal states, liver failure), infections, medications (i.e., immunosuppressants, antipsychotics, steroids, antibiotics), and other medical problems (e.g., post-operative delirium, liver transplant, cardiac surgery, orthopedic surgery).  

Quick Stats about Catatonia

  • The prevalence of catatonia in acute medical settings is about 1.6-6.3%
  • Incidence of catatonia during acute psychosis is about 7-17%
  • Incidence of catatonia during a mood episode is about 13-31%
  • The cause of catatonia is idiopathic/unknown in about 4-46% of cases

The diagnosis of Catatonia can be made when three or more of the following symptoms occur together

  • Stupor
  • Catalepsy (muscle rigidity)
  • Waxy flexibility
  • Mutism
  • Negativism (i.e., resisting movement by others)
  • Posturing
  • Mannerisms
  • Stereotypy (i.e., repetitive movements or speech)
  • Agitation/Impulsive/Bizarre behavior
  • Grimacing
  • Echolalia (i.e., repeating or mimicking others’ words)
  • Echopraxia (i.e., mimicking others’ movements)
In addition, there must be evidence from history, labs, and/or imaging studies that symptoms are from a medical cause and are not better explained by another mental disorder.
Controversially, the Diagnostic and Statistical Manual 5th Edition (DSM-5) precludes the diagnosis of Catatonia if it occurs exclusively during a period of delirium. The controversy exists because Catatonia and Delirium can present simultaneously. In fact, a recent study reported a 12-37% incidence of catatonia in patients with delirium.
Below is a photo of a man with catatonia. As you can see, he is posturing–a classic catatonic sign. Some patients may remain in the same position for many hours. Although we typically associate catatonia with this classic sign, in most cases posturing isn’t a prominent sign or symptom.

Screening Tools

The Bush Francis Catatonia Rating Scale (BFCRS) is the most widely used screening tool for Catatonia.

Diagnostic Tests

Electroencephalography (EEG) is typically normal in catatonia but may show generalized slowing if there is a medically related cause. Brain imaging such as Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) studies are typically normal if not medically related. Lumbar Puncture (LP) may be performed to rule out encephalitis or other central nervous system inflammatory, infectious, or autoimmune processes.

Treatment and Management of Catatonia

Catatonia is a medical emergency and should be treated in a medical hospital.
The treatment of the catatonic patient begins with investigating potential underlying causes and treating them appropriately. This includes discontinuation of any offending medications. Medical conditions and medications associated with catatonia are tabulated below. 
MEDICAL CONDITIONS MEDICATIONS | DRUGS
  • Anti-NMDA-receptor Encephalitis (ANRE)
  • Paraneoplastic Limbic Encephalitis
  • Systemic Lupus Erythematous (SLE)
  • Seizures
  • Brain infections
  • Intracranial Mass Lesions (e.g., tumors)
  • Multiple Sclerosis
  • Delirium (Encephalopathy)
  • Glucose-6-phosphate dehydrogenase (G6PD) Deficiency
  • Vitamin B12 Deficiency
  • Hyponatremia
  • Stroke, Dementia, and Other Brain Degeneration Disorders
  • Takotsubo Cardiomyopathy
  • Wilson’s Disease
  • Ferropenia (low iron)
  • Post Liver Transplant
  • Post Cardiac Surgery
  • Post Orthopedic Surgery
  • Hepatic Encephalopathy (elevated ammonia)
  • Uremia
  • Sepsis
  • Tacrolimus
  • Cyclosporine
  • 3,4-methylenedioxy-N-methylamphetamine (MDMA)
  • Corticosteroids (prednisone, methylprednisolone)
  • Fluoroquinolone antibiotics (ciprofloxacin, levofloxacin)
  • Beta-lactam antibiotics (Cephalosporins)
  • Macrolide antibiotics (clarithromycin, erythromycin)
  • Disulfiram
  • Benzodiazepines and Barbiturates (withdrawal)
  • Baclofen
  • Cocaine (wuithdrawal)
  • Phencyclidine (PCP)
  • Ketamine
  • Antipsychotics
  • Withdrawal from dopamine agonists
In general, antipsychotics should be discontinued and/or avoided. An immediate trial of intravenous (IV) lorazepam at a dose of 2mg-3mg should be administered. The patient should be monitored for a response to this “lorazepam challenge.”  If no response within 60 minutes, consider administering a second lorazepam dose. If still no response, consider adding either a dopamine agonist (e.g., Amantadine, bromocriptine, L-dopa), memantine (Namenda), or zolpidem (Ambien). In cases of malignant catatonia, electroconvulsive therapy (ECT) is strongly recommended and potentially lifesaving. 
If a response to Lorazepam occurs, lorazepam should be administered at a dose of 3mg-20mg per day in divided doses. Usually, patients will require a few weeks of oral lorazepam before slowly tapering.
In addition to Lorazepam, supportive measures are important. These include reversing hyperthermia (if present) and intravenous hydration to prevent kidney injury. Providing adequate nutrition, monitoring oxygenation, and preventing deep vein thrombosis (i.e., compression stockings or anticoagulation), pressure ulcers (i.e., constantly change the patient’s position), muscle contractures, and aspiration are important.
Of note, Electroconvulsive Therapy (ECT) has a 90% response rate and is considered a first line treatment for malignant catatonia, severe malnutrition from refusing to eat, or partial response from medication trials.
For more information about Catatonia, click here.

References

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  • Fink, Max, and Michael Alan Taylor. “Catatonia: A History.” Catatonia (n.d.): 1-18. Web.
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