List II - Less Common 'Know of' Conditions Flashcards
What is Pick’s disease?
- Type of fronto-temporal dementia - atrophy of frontal and temporal lobes
How can Pick’s disease be distinguished from Alzheimer’s disease?
- Pick’s - not usually associated with memory loss in its early stages
- Especially known for the aphasia it causes
- Behaviour problems and personality changes are often a primary first symptom
What are the symptom’s of Pick’s disease?
- Hesitant speech
- Difficulty articulating
- Stuttering
- Ungrammatical speech
- Difficulty recalling words or names
What are the behaviour problems and personality changes associated with Pick’s disease?
- Unusual behaviour
- Disinhibition
- Passivity
- Hyperactivity
- Anxiety
- Slovenly appearance
- Delusions and paranoia
- Lack of empathy
What is the underlying cause of Pick’s disease?
- Caused by the build up of tau proteins, called ‘Pick bodies’ in the brain
What are the more common features of frontotemporal lobar dementias?
- Onset before 65
- Insidious onset
- Relatively preserved memory and visuospatial skills
- Personality change and social conduct problems
What is the appearance of Pick’s disease atrophy?
- Focal gyral atrophy with a knife-blade appearance is characteristic of Pick’s disease
What are the macroscopic changes seen in Pick’s disease?
- Atrophy of the frontal and temporal lobes
What are the microscopic changes seen in Pick’s disease?
- Pick bodies - spherical aggregations of tau protein (silver-staining)
- Gliosis
- Neurofibrillary tangles
- Senile plaques
What is the management of Pick’s disease?
- NICE do not recommend that AChE inhibitors or memantine are used in people with frontotemporal dementia
What is Creutzfeldt-Jakob disease (CJD)?
- Rapidly progressive neurological condition caused by prion proteins
- These proteins induce the formation of amyloid folds resulting in tightly packed beta-pleated sheets resistant to proteases
Features
- Dementia (rapid onset)
- Myoclonus
What are the investigation findings in CJD?
- CSF is usually normal
- EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
- MRI: hyperintense signals in the basal ganglia and thalamus
What are the features of sporadic CJD?
- Acounts for 85% of cases
- 10-15% of cases are familial
- Mean age of onset is 65 years
What are the features of new variant CJD?
- Younger patients (average age of onset = 25 years)
- Psychological symptoms such as anxiety, withdrawal and dysphonia are the most common presenting features
- ‘Prion protein’ is encoded on chromosome 20 - it’s role is not yet understood
- Methionine homozygosity at codon 129 of the prion protein is a risk factor for developing CJD - all patients who have so far died have had this
- Median survival = 13 months
What other prion diseases exist?
- Kuru
- Fatal familial insomnia
- Gerstmann Straussler-Scheinker disease
What is HIV related dementia?
- Before HAART, people with HIV would go on to develop dementia, but this is now uncommon
- However, many people who have HIV still experience a milder type of cognitive impairment such as problems with their memory and thinking - sometimes called HIV-associated neurocognitive disorder (HAND)
What problems does HAND cause for people with it?
People with HAND may experience difficulties with:
- Concentration
- Memory
- Planning
- Organising
- Decision making
Symptoms usually stay stable over time rather than progressing to dementia - the person may also have problems with their mood such as depression or irritability
How should people with HIV be monitored in relation to HAND?
- If a person has had a recent diagnosis of HIV they should have their cognitive and emotional wellbeing assessed within 3 months
- Should then be re-assessed at least once a year
How is learning disability defined?
- IQ <70
- Loss of adaptive social functioning
- Onset before the age of 18
What is NOT learning disability?
- People who develop an impairment after the age of 18
* People who suffer brain injury in accidents after the age of 18
What is mild LD?
- IQ 50-69 - language fair, reasonable level of independence
What is moderate LD?
- IQ 35-49 - generally better receptive than expressive language
What is severe LD?
- IQ 20-34 - increased sensory and motor deficits, 50% will have epilepsy