Rare, fatal brain disease confirmed in New Hampshire patient

By Rina Shah, National Monitor | September 22, 2013

Rare, fatal brain disease confirmed in New Hampshire patient

Creutzfeldt-Jakob disease is a degenerative brain disorder.

According to CNN, a patient that died after neurosurgery at a New Hampshire hospital had Creutzfeldt-Jakob disease, a fatal brain disease.  Unfortunately, by the time doctors suspected this diagnosis, the same equipment used for the deceased patient was used in several other operations.  As a result, those patients may have been exposed to the disease.

Exposure could have occurred by contaminating the equipment.  Normal sterilization procedures are unable to get rid of the proteins, known as prions, which are tied to Creutzfeldt-Jakob disease.  Without knowing prions may have been present, the medical teams using the equipment in subsequent surgeries did not know to use different sterilization procedures.  As a result, they potentially exposed the other patients to infection.

Earlier this summer, five patients that underwent surgery at a Cape Cod hospital may have been exposed to a fatal brain disease, reports another CNN article.  The condition, called Creutzfeldt-Jakob disease, may have come from specialized medical equipment that was contaminated.  These five patients all underwent spinal cord surgery and the Massachusetts Department of Public Health indicates that their risk of infection is low.  At the time, they were waiting for confirmation that the patient in New Hampshire had Creutzfeldt-Jakob disease.

The Mayo Clinic describes Creutzfeldt-Jakob disease as a degenerative brain disorder.  The condition presents with symptoms that are similar to dementia.  However, disease progression is much more rapid with Creutzfeldt-Jakob.  A variant form of this disease is better known as mad cow disease.  However, normal Creutzfeldt-Jakob disease has no connection to contaminated beef.  Creutzfeldt-Jakob disease has been recognized since the 1920s, reports the Centers for Disease Control and Prevention.  In the U.S., there have been between 279 and 352 cases in the last five-year period studied.  While mad cow disease is known as variant Creutzfeldt-Jakob disease, it is not actually related to standard or classic Creutzfeldt-Jakob disease.

According to the National Institute of Neurological Disorders and Stroke, this disease is not transmitted through the air, touching, or most forms of casual contact.  As a result, the medical professionals that handled the contaminated equipment are not at risk.  For the patients that have been exposed, their spouses and family members are no more likely to also become exposed than members of the general public.  They would only be at risk if they somehow came into contact with the brain tissue and spinal cord fluid from an infected individual.

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