|  | Conditions Treatments | Neurological Disorders

Differences Between Sporadic & Familial CJD

Creutzfeldt-Jakob disease, or CJD, is a fatal degenerative brain disease with no cure. While the pathology of CJD is not well understood, researchers believe it results from a mutation in proteins known as prions. Sporadic and familial CJD share many symptoms but occur for different reasons.

  1. Prevalence

    • Both familial and sporadic CJD are extremely rare and occur in only one in a million people, according to Medline Plus. Of these cases, about 85 percent are the sporadic type, while the remaining cases are genetic in origin. Both forms of CJD frequently strike people in their late 50s, though the disease can occur any time between ages 20 and 70.

    Symptoms

    • Aside from their differing origins, familial and sporadic CJD are nearly identical in most respects. The National Institute of Neurological Disorders and Stroke lists memory loss, poor coordination and behavioral changes as early symptoms. As the disease progresses, cognitive and emotional symptoms worsen and may be accompanied by blindness, involuntary movements and coma. Other possible symptoms include anxiety, depression and insomnia. Certain symptoms may be more prominent depending on which parts of the brain are affected first.

    Prevention/Treatment

    • Currently, there is no cure for CJD. Both sporadic and familial forms progress rapidly and are invariably fatal. The National Institute of Neurological Disorders and Stroke states that roughly 90 percent of all CJD patients die within a year of diagnosis. In rare cases, patients with familial CJD survive a few years beyond the onset of symptoms. Treatment focuses on making the patient comfortable rather than halting the disease itself. Medications such as opiate painkillers, clonazepam and sodium valproate are sometimes used for symptom management.

    Misconceptions

    • Familial and sporadic CJD are sometimes confused with Mad Cow disease -- a transmissible prion disease in cattle. While classic CJD and Mad Cow disease are not related, a more recently discovered prion disease known as variant CJD (vCJD) can be spread from cows to humans. To date, no cases of vCJD have been identified in the U.S. Due to their extremely rapid progression, classic and variant CJD are rarely misdiagnosed as Alzheimer's disease or other kinds of dementia.

Neurological Disorders - Related Articles