Is It Possible To Diagnose a Case of Creutzfedlt-Jakob Disease (CJD) in a Small Suburban Community in Upstate New York?

Abstract

ISSUE: A 66-year-old female with a history of anxiety was admitted to the psychiatric unit at a hospital located in a small suburban community in upstate New York. This patient was experiencing acute changes in her mental status over a two week period of time involving confusion, auditory and visual hallucinations, paranoia and waxing and waning levels of consciousness. Diagnostic tests including a spinal tap, an electroencephalogram (EEG), a magnetic resonance imaging (MRI) brain scan and computerized tomography (CT) brain scan were completed. The tests were inconclusive until a second EEG was performed and read by the neurologist. This EEG was abnormal and combined with the patient's deteriorating condition, and symptoms of unsteady gait, myoclonus and startled response the diagnosis of CJD was suspected. However a brain biopsy was needed to confirm the diagnosis. PROJECT: To confirm the diagnosis of CJD a brain biopsy had to be performed. The procedure would be performed along with the implementation of the hospital's CJD policy and procedure which had been developed based on the World Health Organization (WHO) guidelines for transmissible was implemented. RESULTS: With only a few hours notice, the operating room(OR)team implemented the policy and procedure as written including notification to the response team, “CJD Precautions” in the OR and readied the operating suite with the disposable equipment including the manual drill. The neurosurgeon performed a left temporal biopsy. The biopsy revealed the brain was shrunken and full of holes, like a sponge which is a classic description of CJD. The sample was sent to the National Prion Disease Pathology Surveillance Center in Cleveland. A definitive diagnosis of sporadic CJD was delivered within five days. Since there is no treatment to cure or control CJD, alleviating the symptoms and making the patient as comfortable as possible was the plan of care. The patient remained hospitalized and died within two weeks after the confirmation of the diagnosis. LESSONS LEARNED: The step-by-step CJD policy and procedure developed two years prior was invaluable in the management of the case. The neurosurgeon credited the hospital for having this in place for this rare disease. The education that was conducted through group lectures, individual one-on-one sessions, local media coverage including the newspaper and the television stations provided to be very crucial in reducing the hysteria that this rare disease can cause in a community.