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J Neurol Neurosurg Psychiatry 66:406-407 doi:10.1136/jnnp.66.3.406
  • Letters to the editor

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.

    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    She became more withdrawn and uncommunicative with incontinence of urine.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 …