However, excessive cerebral IFN-I levels lead to pathologies, suggesting the need for tight regulation of responses. Protection of the brain from viral infections involves the type I IFN (IFN-I) system, defects in which render humans susceptible to herpes simplex encephalitis (HSE). 12 UK Dementia Research Institute at UCL, London, United Kingdom.11 Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, United Kingdom.10 Department of Immunology, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio, USA.9 Department of Clinical Medicine, University of Aarhus, Aarhus, Denmark.8 Institute of Innate Immunity, University of Bonn, Bonn, Germany.7 Institute of Neuroscience and Physiology, Department of Psychiatry and Neurochemistry, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Germany.6 Centre of Biomedical Research, SGPGI Campus, Lucknow, India.5 Center of Functionally Integrative Neuroscience, Aarhus University, Aarhus, Denmark.4 Department of Nuclear Medicine & PET Centre, Aarhus University Hospital, Denmark.3 Department of Psychiatry and Neurochemistry, Institute of Neuroscience and Physiology, Sahlgrenska Academy at the University of Gothenburg, Sweden.2 Department of Viroscience, Erasmus Medical Centre, Rotterdam, Netherlands.1 Department of Biomedicine, Aarhus University, Aarhus, Denmark.Cases still appear, given the disease’s long incubation period, but they are rare. The New Guinea government has discouraged the practice of cannibalism. Women and children were mainly infected because they were the primary participants in these rites. Kuru developed primarily in the Fore people of New Guinea when they ate the brains of dead relatives during funeral rites. You can contract the disease by eating an infected brain or coming into contact with open wounds or sores of someone living with it. These spongiform diseases, as well as kuru, create sponge-like holes in your brain and are fatal. They are inanimate, misshapen proteins that multiply in the brain and form clumps, hindering typical brain processes.Ĭreutzfeldt-Jakob, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia are other degenerative diseases caused by prions. Prions are not living organisms and do not reproduce. Infectious, abnormal proteins known as prions cause kuru. Unlike most infections or infectious agents, kuru is not caused by bacteria, viruses, or fungi. It primarily affects the cerebellum - the part of your brain responsible for coordination and balance. Kuru belongs to a class of diseases called transmissible spongiform encephalopathies (TSEs), also called prion diseases. These secondary symptoms can lead to death within a year. Starvation and malnutrition usually set in at the third stage, due to the difficulty of eating and swallowing. They may also exhibit dementia or behavior changes, causing them to seem unconcerned about their health.

Third stage: the person is usually bedridden and incontinent. Body tremors and significant involuntary jerks and movements begin to occur. Second stage: Also called the sedentary stage, the person is unable to walk. They may have difficulty balancing and maintaining posture. Since these are common symptoms, they are often missed as clues that a more serious disease is underway.įirst stage: A person with kuru exhibits some loss of bodily control.

It’s usually preceded by headaches and joint pain. Symptoms of more common neurological disorders such as Parkinson’s disease or stroke may resemble kuru symptoms. Today, the study of kuru still impacts research on neurodegenerative diseases. It led to the creation of a new class of diseases including Creutzfeldt-Jakob disease, Gerstmann-Sträussler-Scheinker disease, and fatal familial insomnia. It was the first neurodegenerative disease resulting from an infectious agent. The identification and study of kuru helped scientific research in a number of ways. It’s usually fatal within one year of contraction. Other symptoms include difficulty walking, involuntary movements, behavioral and mood changes, dementia, and difficulty eating. The symptoms of the disease include muscle twitching and loss of coordination. The name kuru means “to shiver” or “trembling in fear.” The Fore people contracted the disease by performing cannibalism on corpses during funeral rituals. Its highest prevalence occurred during the 1950s and 1960s among the Fore people in the highlands of New Guinea. Kuru is a rare and fatal nervous system disease.

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