Creutzfeldt-Jakob disease (CJD) is a rare neurological condition affecting the nervous system. As the condition progresses, vision and mobility are affected. Behavioural changes and cognitive decline follow.
CJD belongs to transmissible spongiform encephalopathies (TSE), an umbrella term for conditions caused by infectious proteins called prions. The main characteristic of prion disease is the sponge-like appearance of the brain due to tiny, porous pockets forming in the brain tissue.
We have yet to find many answers to the causes of Creutzfeldt-Jakob disease, and researchers worldwide are finding ways to treat this fatal condition.
The effects on the brain
Prion is important for various cellular functions like crosstalk between cells, signalling, cell growth and formation of new neurons. But when this protein gets mutated, it creates havoc within the cells. The inherent cleaning mechanism inside cells cannot flush away the unwanted protein, resulting in its accumulation. Clumps of prion ravage nerve cells, causing their death.
Dr Venkatraman Karthikeyan, a neurologist at Gleneagles Global Hospital Chennai, says, “Generally, individuals after their 60s develop this [CJD] condition.”
However, he says it also affects young people, mainly when they unwittingly consume diseased or improperly cooked cattle meat. A form of TSE called bovine spongiform encephalopathy affects cattle, causing hobbling and violent behaviour. “However, this is a rare occurrence where the infectious agent transfers to humans,” adds Dr Karthikeyan.
The many reasons for prion mutations
The exact cause of why the prion protein mutates is unclear, but experts have identified various factors triggering prion mutation. Dr Karthikeyan states, “A minority of cases came up in the UK previously, after corneal transplants or tissues connected to the nervous tissue and brain.”
The above infographic depicts some known causes that could trigger Creutzfeldt-Jakob disease.
Also, read – Rare neurological conditions.
