A CLINICAL NOTEWORTHY BRIEF REVIEW ON PRION DISEASE

Kirti Rani* and Pragya

ABSTRACT

Prion disease is a rare inherited autosomal neurodegenerative disorders whose exact reported prevalence is still unknown. Prions are proteinaceous infectious agents without any nucleic acid. These agents are also termed ‗Transmissible Spongiform Encephalopathies (TSEs)‘ which also caused ‗Mad cow disease‘ in other animals as termed ‗Brain eater agents‘. But various clinical studies are suggested that this disease affects about one person per million worldwide each year. This disease is deteriorated the neuronal conditions with the time that impair brain function, loss in memory, personality disorder and behavior fluctuation, a decline in intellectual function (dementia) and abnormal gait movements and ataxia. The signs and symptoms of prion disease is typically begun in adulthood and progressively worsen with time, leading to brain death within a few months to several years. In, this disease, one copy of the altered PrP gene in each cell is sufficient to cause this neural disorder. In most of the cases, an affected person inherits the altered gene from one affected parent. So, genetic counseling and effective genetic analysis are played very important role to diagnose Prion disease at right time with their existing lethal symptoms during onset of this disease. This disease must be diagnosed, treated and managed well at right time in recorded cases of Prion diseased patients with effective neuroprotective and suggestive clinical measures along with palliative care. Then, it can be medically managed to control its clinical and genetic burden in affected population of any country worldwide.

Keywords: Prion disease; neurodegenerative disorder; ataxia; PrP gene; Prion diseased patients.