UCL Queen Square Institute of Neurology


Creutzfeldt-Jakob Disease

Medline NLM definition: A rare, usually fatal, transmissible encephalopathy occurring in middle life, in which there is partial degeneration of the pyramidal and extrapyramidal systems accompanied by progressive dementia and sometimes muscle wasting, tremor, athetosis, and spastic dysarthria. A familial form has been shown to exhibit autosomal dominant inheritance. The more common sporadic form is probably caused by mutations that produce abnormal prion proteins (PRIONS). A variant of CJD has been reported in patients younger than 30 years old, probably caused by exposure to beef harboring the bovine spongiform encephalopathy agent.

CJD: factsheet from NINDS, the National Institute of Neurological Disorders and Stroke (US site)

CJD: information from the NHS website

CJD: information from Patient UK website

PubMed Medline search on Creutzfeldt-Jakob Disease

The Many Faces of Dementia This free online course was developed by Dr Tim Shakespeare of UCL's Dementia Research Centre. The course provides a unique insight into dementia through the stories, symptoms and science behind four less common diagnoses of dementia.

Support Groups

National Prion Clinic at the National Hospital for Neurology and Neurosurgery
The National Prion Clinic (NPC)'s multidisciplinary team comprises consultant neurologists, clinical research fellows and clinical nurse specialists.      

The UK Creutzfeldt-Jakob Disease Surveillance Unit
The National CJD Surveillance Unit was setup in 1990 in response to a recommendation in the Report of the Working Party on Bovine Spongiform Encephalopathy (The SouthWood Committee). The unit brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease.

Creutzfeldt-Jakob disease foundation
A US non-profit organisation which provides information and support about Creutzfeldt-Jakob Disease (CJD) to patients, health professionals and the general public, and promotes research into the disease and the dissemination of research findings. (US site)

Music for dementia
This is a great website for anyone who works with or cares for people living with demntia. If you are running a dementia friendly music event or are looking for one, they're all here, as well as suggestions about ways of engaging with music. To ensure every person living with dementia has access to music, they are taking the lead by creating a taskforce of stakeholders from across the music and dementia fields to embed music into dementia care pathways.

Rare Dementia Support service
Rare Dementia Support is a unique, world-leading service providing support, guidance and information to everyone affected by or at risk of a rare dementia, in a space of mutual respect and community. It builds on the pioneering research of the UCL Dementia Research Centre (DRC) and its expertise in young-onset, inherited and unusual dementias. With the generous support of the Selfridges Group Foundation and expertise from their digital and creative teams, they have designed a new, innovative online support platform. People living with rare dementias informed every step of the development process: from website layout, accessibility and content to design, fonts and colours.

Brain and Spine Foundation
Information on living with a neurological condition and on diagnosis. There are publications and resources. The useful glossary of neurological conditions (under 'Information and support') includes links to some other organisations as well as to Brain and Spine Foundation information.

Healthtalk: nerves and brain
Personal experiences of people living with various neurological conditions.

Neurological Alliance
Collective voice for 80 organisations, including stroke and brain injury. Organisations are listed at https://www.neural.org.uk/our-members/, a good place to start if looking for information on those conditions. Site also includes information about living with a neurological condition, a glossary, and information about onset and diagnosis as well as a small set of real life stories.

NHS Inform: brain, nerves and spinal cord 
NHS inform is Scotland's national health information service, with the aim of providing the people in Scotland with accurate and relevant information to help them make informed decisions about their own health and the health of the people they care for.