Probable Sporadic Creutzfeld-Jakob Disease in a 59 year old male.
Sporadic Creutzfeld-Jakob disease (sCJD) is the most common human prion disease. Prion diseases are generally rare however. Creutzfeld-Jakob disease (CJD) is characterized by rapid neurologic deterioration manifesting as spongiform changes in the brain, loss of neurons, and prion protein accumulation leading to mortality usually occurring within a year of disease onset. CJD is most often sporadic, but can exist in genetic, iatrogenic, and variant forms as well. Diagnosis requires histopathologic confirmation, however, that is not always readily available, or patients become lost to follow-up after discharge from the hospital. When histopathologic confirmation is not performed, a probable diagnosis of CJD can still be made.
We report the peculiar case of a 59 year old man who presented to the emergency department with a ground level fall and neurologic complaints. Following admission to the hospital, the patient received an extensive work-up to determine the cause of his inability to walk, dysarthria, and progressively worsening neurologic symptoms. Magnetic resonance imaging and cerebrospinal fluid analysis was obatined as part of working toward the diagnosis. Ultimately, the patient died following discharge from the hospital, but a probable diagnosis of Sporadic Creutzfeld-Jakob disease (sCJD) was made. Unfortunately, an autopsy of the brain was not performed.
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