Abstract
Vladan Ljubisavljevic, Paul Schneider, Division of Mental Health, Princess Alexandra Hospital, Brisbane, Australia:
The word catatonia derives from the Greek word meaning, ‘to stretch tightly’. It is a state of increased tone in muscles at rest. Over the years, catatonia has been viewed either as a disease entity, a syndrome, or a symptom. Sims [1] mentioned several variants of catatonia, including lethal (pernicious) catatonia, neuroleptic malignant syndrome (NMS), periodic catatonia, manic excitement and stupor in delirium. The question arises whether it is clinically important to distinguish these variants. The case to be described is of particular interest in this context, as it demonstrates importance of early recognition and prompt treatment of a severe variant of catatonia, so called lethal catatonia (LC).
Mr A, a 33-year-old, employed man was initially admitted to a medical unit for the assessment of possible organic cause for automatic instability and catatonia-like picture. Collateral history indicated gradual deterioration over one week, characterized by increased restlessness and agitation, neurovegetative disturbances, social withdrawal and decreased communicativeness. Acute deterioration occurred two days prior to presentation and was characterized by muteness, posturing and ambitendency. He also refused food and drink and was suffering from urinary incontinence.
Mr A was hospitalized three months previously with first psychotic episode and was commenced on Olanzapine 5 mg that he had stopped following his discharge. There was no other psychiatric history, no significant medical history or a history of substance abuse. There was also no family history of psychiatric disorders.
Examination revealed a mute, dishevelled man with muscular rigidity and profuse perspiration. Tachycardia (120/min), tachypnoea (20/min) and hypertension (160/90 mmHg) were noted. Investigations, including blood tests, sepsis screen, ECG, EEG, CT of head and lumbar puncture were inconclusive.
Mr A was admitted to a medical ward and all regular medication was stopped. Over the succeeding five days, the patient remained catatonic with minimal oral intake. Autonomic instability was evidenced by profuse perspiration, fluctuating pulse rate (between 100 and 140/min), blood pressure (between 140/70 and 160/95 mmHg) and temperature (between 36.9 and 38°C). Due to repeated physical and neurological examination and investigations revealing no new findings, Mr A was transferred to a psychiatric unit. Right unilateral ECT was first administered on day nine. On day 13 the patient developed left lower lobe pneumonia and intravenous antibiotics were commenced. Course of ECT continued throughout that period. Autonomic instability was first to settle. That was followed by an improvement in the patient's mental state. The last ECT (9th) was given on day 27. The patient was discharged on day 30, on Olanzapine 10 mg nocte with community mental health follow-up. At the time of discharge, affective blunting was the only remaining sign.
There has been a relative paucity of literature published in English language on LC. It is a neuropsychiatric disorder that was first described in early 1800s and was subsequently reported by numerous authors throughout the preneuroleptic era. In 1934 Stauder coined the term lethal catatonia. In their review of related literature from around the world, Mann et al. [2] identified a series of 292 cases of lethal catatonia. They reported a 60% mortality rate with a female/male ratio of 2 : 1. The mean age of patients in that review was 33 years. Main clinical features of LC can be divided into: core catatonic symptoms, autonomic instability and fever. Autonomic instability is manifested by diaphoresis, labile or high blood pressure, tachycardia and varying degrees of cyanosis. The syndrome can appear in a hyperactive form (twothirds of cases) or in a hypoactive or stuporous form (one-third of cases).
Our patient presented with the main clinical features of lethal catatonia, including catatonic syndrome, combined with autonomic instability and intermittent fevers. He presented with a hypoactive form. This presentation occurred on the background of schizophreniform psychosis and absence of organic pathology. This case underlies the importance of prompt recognition and treatment. Once developed, lethal catatonia, independent of its aetiology, undertakes an autonomous and frequently fatal course. Prolonged immobility and withdrawal with refusal to eat may expose catatonic patients to the risk of infection, dehydration, malnutrition, and pulmonary embolism. Our patient developed pneumonia, which is known to be a frequent complication of LC [3]. The evidence that neuroleptics are of benefit in the treatment of LC is limited [2]. Furthermore, it seems that neuroleptics are largely ineffective in controlling LC once in its full-blown form. Although controlled studies are lacking, ECT appears to be effective and widely used [2, 3]. Sporadic case reports also suggest effectiveness of ACTH and corticosteroids [2]. In their review of case reports and case series, Hawkins et al. [4] identified 178 patients with catatonia. The most commonly reported treatment was with benzodiazepines, which were effective in 70% of the cases. They found that ECT was more likely to provide a positive outcome in LC.