Chronic Neuro 2 Flashcards
Define dementia
A severe loss of memory and other cognitive abilities which leads to impaired daily function, regardless of the underlying cause
Dementia differentials (13)
Alzheimer’s Frontotemporal dementia, Lewy Body Dementia Vascular Dementia Wernicke-Korsakoff’s Hydrocephalus Bilateral Subdural Haematomas HIV, syphilis, CJD Frontal mass, limbic encephalitis Hypoglycaemic episodes
5 A’s of dementia
Amnesia - Remembering Anomia - Naming Apraxia - Doing Agnosia - Recognising Aphasia -Speaking
Pathophysiology of AD
APP breakdown by and γ secretase → abnormal product resistant to degradation → beta Amyloid. This accumulates outside the cell to form amyloid plaques which interferes with neural communication and causes dementia. Also intracellularly causes the phosphorylation of tau causing it to dissociate from the MF and accumulate into neurofibrillary tangles which causes neuronal apoptosis and dementia
What performs normal breakdown of APP
Alpha and γ secretase → normal degradation product
RF of dementia (8)
Vascular RF: DM, BP, dyslipidaemia, diet Females Trauma Age Down's syndrome
Ix for Alzheimers
Mainly clinical diagnosis
Can look in CSF for leaked tau protein and low amyloid due to it being in plaques
Can do brain imaging too but only really useful in advanced disease
What are the 10 questions of the AMT
Age? Time to nearest hour? Addres for recall at the end of the test Year? Name of this place? Identification of two people? Date of birth? Year of First World War? Name of current monarch? Address recall correct?
What is vascular dementia caused by?
Neuronal infarcts so basically loads of mini strokes.
What is the difference between vascular dementia and AD? (4)
Focal neurology
Sudden onset
Stepwise deterioration
CV RF
What does the pathophysiology of Pick’s disease involve
Tau but not beta amyloid plaques
What is a Pick body
Hyperphosphyorlated tau protein
What is a hyperphosphorylated Tau protein known as
Pick body
Difference in epidemiology of Picks disease and AD
Hyperphosphyorlated tau protein
Also typically affects people younger than in other dementias
±FHx (although most are sporadic)
Death within 5-10yrs
Which lobes does Pick’s disease affect
Fronto-temporal
Symptoms of Pick’s disease (6)
Personality change Disinhibition Overeating, preference for sweet foods Emotional blunting Lack of hygiene Relative preservation of memory
Wernicke’s encephalopathy triad
ACE
Ataxia
Confusion
Eye signs
Wernicke’s encephalopathy signs (2)
Wernicke’s encephalopathy triad
What eye signs are present in Wernicke’s encephalopathy (4)
ophthalmoplegia, nystagmus, diplopia, ptosis
Why do alcoholics get WKS
generally have a poor diet. Alcohol also prevents vitamin B-1 absorption and storage.
What is thiamine involved in (4)
Metabolism of carbohydrates, releasing energy.
Production of neurotransmitters including glutamic acid and GABA.
Lipid metabolism, necessary formyelin production.
Amino acid modification. Probably linked to the production of taurine, of great cardiac importance.
What is thiamine’s role (2)
Thiamine acts as an essentialcoenzyme in the TCA cycle and pentose phosphate shunt.
Ix for Wenicke’s encephalopathy (5)
Bloods –serum albumin and vitamin B1
ECG
CT
Neuropsychology
Mx for Wernicke’s encephalopathy
IV Pabrinex immediately
4 differences between Wernicke’s encephalopathy and Korsakoff’s psychosis
W: Acute Confusion Cerebellar and eye signs Reversible
K: Chronic Alert Amnesia and confabulation Irreversible
Motor symptoms of Huntingtons (4)
Chorea
Athetosis - writhing movements of hands
Ataxia
Dysphagia
Cognitive symptoms of Huntingtons (4)
Lack of concentration
Depression
Dementia
Personality changes, aggression
Prognosis of Huntington’s
10-15 years from onset of symptoms
What sign will you illicit in the cranial nerve exam of a Huntington’s patient
Unable to protrude tongue
Where is the pathology in Huntington’s
in the striatum, there is atrophy of mainly the caudate, but also putamen
Pattern of inheritance of Huntington’s disease
Autosomal dominant
Mutation in which gene and nucleotide causes Huntington’s
mutation of the Huntingtin gene (HTT)
Expansion of CAG nucleotide from less than 35 to over 40
Ix for Huntingtons
CAG repeat testing
