Neurodegenerative disorders Flashcards
What is the definition of dementia?
- impairment of intellect, reason and personality without impairment of consciousness
- often comes with emotional lability and memory dysfunction
What are the possible causes of dementia?
- Cerebrovascular disease
- HIV infection
- drugs/toxins
- metabolic disorders
- Vit def (Wernicke korsacoff)
- Paraneoplastic syndrome
What is the pathophysiology of Alzheimers?
- accumulation of amyloid which form amyloid plaques in extracellular matrix of cerebral cortex
- severe cortical atrophy
- narrowing gyri and widening of sulci
- dilatation of ventricular system
What is amyloid?
starch like protein which accumulates in extracellular matrix
What staining is used for amyloid?
congo red (stains red)
Where is it common for amyloid to pathologically accumulate?
- heart (causing HF)
- arterioles and capillaries in brain (predisposing to intracerebral haemorrhage)
Is Parkinson’s inherited?
mostly idiopathic but can have inherited predisposition and environmental triggers
What are the histologic markers of Parkinsons disease?
Lewy bodies
- rounded cytoplasmic inclusions with dense core and pale halo
- found in substantia nigra
Motor symptoms of Parkinsons disease?
Tremor Rigidity Bradykinesia Falls and impaired balance Freezing Muscle cramps Microphagia
Non-motor symptoms of Parkinson’s?
Increased Sebum production
Increased salivation
Anxiety, depression and dementia
What type of dementia is common in Parkinsons disease?
- Lewy body dementia
- can occur prior to motor symptoms
How is Huntingtons inherited?
Autosomal dominant
What is the pathophysiology of Huntingtons?
degeneration of putamen (striatum in particular)
caused by CAG trinucleotide repeat expansion of gene coding for Huntington protein
What are the features of Huntingtons?
associated with movement disorder and behavioural changes progressing to dementia
What is chronic traumatic encephalopathy?
- degenerative brain disease associated with history of repetitive head impacts
- cognitive impaired, neurobehavioral abnormalities also common (e.g. rage)
