Creutzfeldt-Jakob Disease

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Creutzfeldt-Jakob Disease (CJD) is an uncommon degenerative brain disorder that leads to dementia and is ultimately fatal. Its characteristics are similar to Alzheimer’s disease, but it progresses much more rapidly. The annual incidence is about 1-2/1 000 000. It usually affects people around the age of 60.

Walther Spielmeyer gave the disease its name in 1922, naming it after the German neurologists Hans Creutzfeldt (1885–1964) and Alfons Jakob (1884 –1931). It is also called subacute spongiform encephalopathy, transmissible spongiform encephalopathy, a neurocognitive disorder due to prion disease or a variation of those names. CJD belogs to a group of human and animal diseases known as the transmissible spongiform encephalopathies or prion diseases. Prions are infectious agents that is a small proteinaceous particle with no nucleus.

Creutzfeldt-Jakob Disease captured public attention in the early 1990s when a number of people in the UK developed variant CJD (vCJD) after eating meat from diseased cattle, the so-called ‘mad cow disease’. The significant public health response to this at the time was well covered in the news media globally. However, the classic and typical Creutzfeldt-Jakob disease is not linked to eating contaminated beef. It is thought that around 200 people a year do develop vCJD from eating contaminated meat and it is not contagious.

Aetiology:
Generally believed that abnormal forms or types of the prions (designated as PrP) cause CJD.
Most cases appear spontaneously, with no source identified. It is not easily contagious from person-to-person contact.
In rare cases, transmission of CJD has occurred through iatrogenic means (contact with brain tissue and cerebrospinal fluid, eg neurosurgery, corneal transplant; injections of contaminated pituitary hormone; contaminated surgical instruments).

Four types: Familial CJD (inherited type; autosomal dominant; up to 10% of cases); Sporadic CJD (most cases); Acquired or iatrogenic CJD (exposure to infected tissue during a medical procedure); Variant CJD (from exposure to bovine spongiform encephalopathy (BSE); Mad Cow Disease; very rare; usually much younger).

Pathophysiology:
The prion causes normal proteins to fold abnormally.
Develop a spongiform encephalopathy (a characteristic appearance of infected brains filled with holes, resembling sponges under a microscope); cerebral atrophy; neuronal loss.

Clinical Features:
Initial symptoms include memory problems; anxiety; fatigue; headache, dizziness; behavioral changes; changes in gait; falls; vision difficulties.
Later symptoms include a rapid progression of dementia; weakness and stiffness of limbs, involuntary movements (extrapyramidal lesion signs); blindness.
Electroencephalography (EEG) shows a specific type of abnormality in many cases.
Cerebrospinal fluid tests may be helpful in making a diagnosis.
MRI is starting to be shown to be useful in reaching a diagnosis.
Diagnosis is typically confirmed after death via autopsy.

Management:
70%-90% are fatal within 1 year of diagnosis, mostly from pneumonia. There is no specific treatment.
Any current treatments are aimed at relieving symptoms and making them as comfortable as is possible. Opioids may be given for pain relief; clonazepam or sodium valproate may be given for the involuntary movements.
Trials of different drugs are underway (eg monoclonal antibody).
In many countries CJD is a reportable disease and public health units are responsible for testing and surveillance as well as being responsible for precautionary measures.

Relevance to Podiatry:
Management of the foot problems of those with dementia is challenging. There are practical things that can help.
Ensure that carers are aware of the importance of proper foot care and foot hygiene.
The involuntary movements may make foot care and the treatment of foot problems a challenge.

Page last updated: Jun 11, 2022 @ 6:33 am

 
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