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Malignant catatonia secondary to sporadic encephalitis lethargica
  1. HOLLY A SHILL,
  2. MARK A STACY
  1. Muhammad Ali Parkinson Research Center, Barrow Neurological Institute, Phoenix, Arizona, USA
  1. Dr Mark A Stacy mstacy{at}bng.chw.edu

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Catatonia is a neuropsychiatric syndrome characterised by a combination of psychosocial withdrawal and various movement disorders. Kahlbahm first described this syndrome in 1868 when he noted this condition of “profound mental anguish”. In 1893, Kraeplin limited catatonia to a subtype of dementia praecox, later redefined by Bleuler in 1906 as catatonic schizophrenia. Since then, it has become increasingly apparent that the catatonic syndrome can be seen not just in schizophrenia, but in affective disorders as well as secondary to various underlying medical conditions, leading the DSM-IV to broaden its categorisation of catatonia to include these other entities. In the early 1900s, a condition known variously as epidemic encephalitis, encephalitis lethargica, or Von Economo's disease was described, affecting more than 65 000 patients from 1919 to 1929.1Case descriptions reported during this time bear striking similarities to our modern definitions of catatonia. Throughout recent years, isolated cases of encephalitis lethargica have been reported.2 3 We present a patient with features of sporadic encephalitis lethargica and discuss management of this entity in the context of catatonia.

The patient was a 22 year old previously psychologically and neurologically healthy woman who was transferred to the Barrow Neurological Institute after a 4 week stay in hospital for progressive immobility, mutism, posturing, and tremor. Initial evaluation had shown a CSF lymphocytosis, increased liver transaminases, and an EEG with bifrontal slowing. Further investigation as to aetiology of her meningoencephalitis was negative including brain MRI, brain biopsy, vasculitic profile, and comprehensive infectious disease evaluation for both endemic as well as epidemic encephalitis. An FDG-PET showed bilateral cortical hypometabolism and asymmetric thalamic hypometabolism. Over the course of her initial stay in hospital, she was intermittently agitated and chanting with frequent tremors, posturing, and occulogyric crisis. She had intermittent fever and tachycardia for which no source was found. Before her transfer, she became mute, stopped eating, and was bedridden with a percutaneous feeding tube and indwelling urinary catheter. Her initial evaluation at Barrow Neurological Institute showed her to be febrile (38.4°C) and tachycardic (130 bpm). She was mute with a staring, frightened expression and, although she seemed to attend to conversation at times, would not follow commands. Tone was diffusely increased with active resistance to passive movement of the limbs and catalepsy (waxy flexibility). She had a diffuse, asymmetric tremor, repetitive tongue thrusting, and occasional dystonic posturing of the arms. She had remarkable insensitivity to noxious stimuli, normal tendon reflexes, and flexor plantar responses. Repeat metabolic and infection evaluation was negative. Based on her history of intermittent agitation and verbigeration with progression into a mute, immobile state punctuated with random tremors, stereotypies, and abnormal tone, a diagnosis of catatonia was made. She had a brief trial of intravenous lorazepam that improved her motor symptoms but produced excessive sedation and respiratory compromise. She was then referred for electroconvulsive therapy (ECT) and had four treatments over 2 weeks with dramatic improvement in her symptoms. Repeat FDG-PET was normal. She was discharged to a rehabilitation facility and at 6 month follow up, she had made a full recovery and returned to full time employment.

The diagnosis of catatonia has not been standardised but instead relies on a range of typical clinical features that combine an alteration of behaviour with stereotypic movement disorders. Catalepsy, although considered by Bleuler to be intrinsic to the condition, is currently not considered mandatory for the diagnosis. Cardinal signs are felt to immobility, mutism, and withdrawal with secondary features including staring, rigidity, posturing or grimacing, negativism, waxy flexibility (catalepsy), echophenomenon, stereotypy, and verbigeration. Criteria have been proposed which include many of the above signs in an effort to standardise diagnosis and treatment.4 Lethal (or malignant) catatonia has additional features of hyperthermia, autonomic instability, and rigidity often severe enough to lead to death through rhabdomyolysis, renal failure, and cardiovascular collapse.

Aetiologies of catatonia are varied and although its association with schizophrenia is accepted, it is most often seen with affective disorders. Medical conditions are increasingly becoming recognised as causes of a catatonic syndrome. When first described, encephalitis lethargica produced three relatively distinct, although often overlapping neurological syndromes.1 The first, and most common, began with a flu-like illness that progressed with increasing sleepiness, ocular motility problems (including oculogyric crisis), and pupillary abnormalities and is known as the somnolent-ophthalmoplegic form. The parkinsonian subtype generally presented with bradykinesia, catalepsy, and mutism and most closely resembles catatonia. The final variety, recognised as the hyperkinetic form, had a more psychiatric presentation with agitation, motor restlessness, obsessional behaviour, psychosis, and dyskinesis. There are no contemporary criteria for diagnosis of encephalitis lethargica, however based on historical data, we think that our patient represents a progression of the hyperkinetic form into a more parkinsonian picture punctuated by occasional dyskinesias.

The pathological substrate for catatonia is largely unknown. When it is produced by anatomical derangement, abnormalities are most often seen in the thalamus, subthalamus, and substantia nigra. In patients dying from encephalitis lethargica, severe destructive changes were seen in the substantia nigra and, to a lesser extent, in the subthalamic nuclei and other basal ganglia structures. Our patient had a normal brain MRI and FDG-PET suggesting asymmetric thalamic hypometabolism which resolved with ECT, suggesting at least functional impairment in these anatomical areas.

Evaluation for the aetiology of catatonia is outlined in our report. Treatment is aimed at addressing any underlying medical conditions that may be producing the syndrome and once this is done, directly treating the catatonia itself. Historically, this has been varied, but recent studies suggest excellent efficacy for both high dose intravenous benzodiazepines and ECT.5 Our patient began responding within 24 hours of her first ECT and although spontaneous recovery remains a possibility, we think that her improvement is due to ECT. Data regarding outcome in epidemic encephalitis lethargica reports a mortality up to 35% with an additional 40%–50% experiencing neurological and psychiatric sequelae.1 Post-encephalitic parkinsonism could be seen as far out as 20 years in patients who seemed to have recovered from the acute infection. Recovery in our patient has been complete without evidence for a progressive or relapsing neurological or psychiatric disorder, although follow up has been limited to 1 year.

In conclusion, catatonia may be produced by various causes, both neurological and psychiatric. Without a history of previous psychiatric impairment, aggressive investigation should be pursued for treatable medical conditions. Catatonia due to medical conditions may be successfully treated with therapies typically reserved for psychiatric indications. The clinical syndrome of encephalitis lethargica, although no longer epidemic in nature, is still sporadically seen and the underlying inflammatory cause is, as yet, unknown.

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