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Creutzfeldt-Jakob disease presenting as complex partial status epilepticus: a report of two cases
  1. J H REES,
  2. S J SMITH,
  3. D M KULLMANN,
  4. N P HIRSCH,
  5. R S HOWARD
  1. National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
  1. Dr J H Rees, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.

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Creutzfeldt-Jakob disease is a transmissible human spongiform encephalopathy which may be familial, iatrogenic, or sporadic. The classic clinical features include a rapidly progressive dementia with the patient retaining clear consciousness until the terminal stages of the disease. We report on two patients presenting with a rapidly declining level of consciousness, in whom the clinical picture and EEG were suggestive of complex partial status epilepticus.

The first patient was a 58 year old woman who was admitted to a psychiatric unit with a short history of mood disturbance, confusion, and unsteadiness. A provisional diagnosis of agitated depression was made and she was started on lofepramine. She then became unsteady on her feet and required support when walking. She had had occasional complex partial seizures for 30 years but at presentation was not taking any anticonvulsant drugs.

On examination, she appeared perplexed, tearful, and agitated, and was unable to give a coherent history. She was intermittently confused and her gait was ataxic. There were no other cerebellar signs. The rest of the neurological examination was unremarkable although limited by poor cooperation.

She became more withdrawn and uncommunicative with incontinence of urine. She would occasionally jump when sitting in a chair.

Brain CT and MRI were normal, as was her CSF. An EEG showed frequent, almost continuous variable amplitude sharp waves in all areas, although with a right sided emphasis, with a repetitive appearance up to 2 per second (figure). The record was thought to be in keeping with partial status epilepticus.

Initial EEG at presentation in an acute confusional state, showing virtually continuous semirepetitive sharp waves with some right sided predominance. Although seizure-like evolution of discharges was not seen, the electrographic picture was considered to be in keeping with complex partial status.

Her level of consciousness deteriorated despite intravenous valproate and phenytoin and she was transferred to the intensive care unit for continuous EEG monitoring. On arrival, she was deeply unconscious and despite aggressive management of her presumed complex partial status she died 3 weeks later. Histology of the brain was diagnostic of the sporadic form of Creutzfeldt-Jakob disease.

The second patient was a 68 year old man who was admitted with a short history of confusion and inappropriate behaviour. He appeared not to recognise his family. Initially he was dysphasic and obtunded. His conscious level then deteriorated and he became mute with evidence of right sided weakness.

All investigations including contrast enhanced brain CT and CSF examination were normal. An EEG was reported as showing frequent bilateral epileptiform activity, but there was no improvement in his clinical state after a loading dose of intravenous phenytoin. A repeat EEG was dominated by periodic lateralised epileptiform discharges (PLEDs), more marked on the left side. A third EEG 3 weeks later again showed frequent predominantly left sided epileptiform discharges, which were attenuated by a bolus of intravenous diazepam but without any improvement in his clinical condition.

He was transferred to this hospital for artificial ventilation because of the concern that he was in complex partial status. On admission he was mute, his eyes were closed, and he flexed to pain on the left side only. Intermittent twitching of both sides at a rate of between 1 Hz–2 Hz was seen. Reflexes were brisk and symmetric. His right plantar response was extensor, his left flexor.

A further EEG 5 days later showed generalised bisynchronous continuous periodic sharp waves occurring at a frequency of 1.3 Hz, at times in the form of biphasic or triphasic complexes. Myoclonic jerks occurred during the recording.

It was considered that overall these features were now consistent with a diagnosis of Creutzfeldt-Jakob disease. His condition continued to deteriorate and he died 2 weeks later. A request for a postmortem examination was refused.

These two cases illustrate a previously unrecognised presentation of Creutzfeldt-Jakob disease, namely presumed complex partial status.

In the first case, the interpretation of the EEG findings was made more difficult by the patient’s depressed conscious level and the previous history of complex partial seizures, albeit mild. The initial psychiatric presentation, with mood and behaviour disturbance, as well as fluctuating confusion, was compatible with complex partial status. The initial EEG report, suggesting partial status epilepticus, prompted treatment, unsuccessfully, with anticonvulsant drugs and subsequent transfer for continuous EEG monitoring. This disclosed marked fluctuations, including discrete runs of rhythmic sharp waves that were considered to be electrographic seizures. Even after sustained burst suppression, the recording fluctuated between generalised periodic discharges and periods of relative inactivity within a matter of seconds.

In the second case, the patient developed focal seizures and PLEDs on the EEG. The initial recordings were suggestive of complex partial status, with asymmetric discharges abolished by diazepam but without any observable clinical change. Subsequent recordings were more characteristic of Creutzfeldt-Jakob disease, particularly as the patient had developed myoclonus. Although the electrographic changes were abolished by diazepam, suggesting seizure activity, the modification of both clinical and EEG activity in Creutzfeldt-Jakob disease by benzodiazepines has been reported1 giving rise to further confusion with epileptiform sharp wave activity. The focal nature of the patient’s signs and the lateralisation on the EEG is well recognised in Creutzfeldt-Jakob disease as are periodic PLEDs, which are often associated with contralateral myoclonic jerks.2

The two cases described here illustrate that a diagnosis of Creutzfeldt-Jakob disease should be considered where a rapid decrease in consciousness is accompanied by EEG changes apparently compatible with complex partial status. When there is a clinical suspicion of Creutzfeldt-Jakob disease, the ideal method of monitoring such patients is with continuous EEG recording, allowing documentation of rapid fluctuations. The present cases are atypical in that the progression from presentation to death was rapid, but they underline the fact that minute to minute changes in EEG rhythm, asymmetry, and electrographic responsiveness to benzodiazepines can all be seen in Creutzfeldt-Jakob disease.

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