Parkinson’s Week quiz time!

1. Wooooo! Parkinson’s Weekquiz time!

2. Give me 3 causes of gait abnormality: • Parkinson’s disease, Huntington’s disease, Parkinson plus syndromes: Multiple System Atrophy, Lewy Body Dementia, (each of these can produce a short shuffling Parkinson’s gait • Huntington’s is more commonly characterised by chorea (brief, purposeless jerky movements) • Multiple Sclerosis (tends to be ataxic) • Degenerative back, hip and knee joint diseases • Trauma (foot, knee, hip, back, head) • CVA, multi infarct dementia • Infective: encephalitis, meningitis, syphilis • Ataxia (ataxic gait) due to ethanol, drugs (egantiepileptics), B12 deficiency, renal failure, sensory ataxia due to spinal lesion or peripheral neuropathy (diabetes), hyper/hypo natremia, hypercalcaemia, cerebellar lesion eg following stroke. • Congenital brain abnormality

3. Parkinson’s Disease • List one protective and one risk factor for developing Parkinson’s Disease • Nicotine Genetics • What macroscopic change is evident in this disease? • Pallor of the substantianigra • State the pathophysiology behind the disease • Degen of dopaminergic neurons  ↓ striatal dopamine (putamen responsible for motor) Imbalance of Ach and Dopamine

4. Parkinson’s Disease • How is it diagnosed? • Clinical Diagnosis: Must include resting tremor, cogwheel rigidity, bradykinesia/ akinesia and impaired posture. Rule out other causes • What are Parkinson Plus Syndromes? • Disorders in which there is parkinsonism plus additional features and specific pathology • Describe how Levodopa and Carbidopa work • Carbidopa – peripheral dopadecaboxylase inhibitor. ↓ req. dose by 10x. Don’t penetrate BBB. Allows levodopa to diffuse across without being decarboxylated • Levodopa – Crosses BBB. Decarboxylated into exogenous dopamine floods the synapse

5. Multiple Sclerosis • What is optic neuritis • Inflammation of the optic nerve. Sx. due to loss of myelin sheath or, rarely, damage to the axon • What brain matter does MS effect? • White • What defines MS • Autoimmune demyelinating disorder characterised by distinct episodes of neurological deficits (separated in time) attributable to white matter lesions (separated in space) • symptoms must last for > 24 hr and occur as distinct episodes that are separated by a month or more

6. Multiple Sclerosis • What are the two main causes of death associated with MS • Uraemia and Bronchopneumonia • Describe the most common course of MS • Relapsing/remitting MS: discrete attacks that evolve over wks-mths. Complete recovery b/n events. If severe (ambulatory) 50%  • State some management options for MS • Education, rehab, social support, home support…. • Do steroids have a place in management? • Can reduce severity in relapse. No long-term diff

7. Alzheimer Disease • State the aetiological difference between delirium and dementia • Delirium – disturbed CNS functioning • Dementia – Structural CNS pathology • What is the DSM IV criteria for dementia • A1 – memory impairment • A2 - ≥1 cognitive disturbance (aphasia, apraxia, agnosia, executive functioning) • B – Disturbances significantly interfere with social and work functions • C – Gradual onset and continuing cognitive decline • D – Not due to a known organic cause (drugs, illness, CVA) • E – Not a delirium • F – Not due to another Axis 1 Disorder (eg depression)

8. Alzheimer Disease • How is AD diagnosed? • Diagnosis is made on clinical assessment and supported by investigation results. • State 3 causes of dementia • degenerative cerebral diseases, including • Alzheimer's disease (about 60%) • dementia of frontal type (up to 10%) • dementia with Lewy bodies (up to 10%) • vascular (15%) • alcohol excess (5%) • AIDS dementia • cerebral tumours • Cruetzfeldt-Jakob disease • Pick's disease • neurosyphilis

9. Alzheimer Disease • Which three secretases break down APP, and which is the troublesome one? • α, β and γ. β is the troublesome one • What two elements are involved in the pathogenesis of AD? • Aβ peptides which are insoluble and neurotoxic and lead to plaque formation and inflammation • Tau protein dysfunction  axon tangles • What changes in the brain are observed in AD? • Atrophy of the cerebrum, firstly the amygdala and hippocampus.

10. Huntington’s Disease • What inheritable genetic type is HD? So if both parents have one gene each for the disease, what chance do their kids have of developing it? • Autosomal dominant. 75% • What macroscopic changes are seen in HD? • Atrophy of the caudate nucleus and the putamen • How does this explain the Sx of HD? • Basal ganglia circuitry dampen (to modulate) motor output. Atrophy  ↑ motor output  chorea • Dementia probably due to atrophy of the cortex.

11. Huntington’s Disease • Please provide me with a 3D sketch of the caudate nucleus and the putamen, explaining their Function. • While you’re at it, do the same for the mammilary bodies • What protein is thought to be involved in the pathogenesis of HD? Function? • Huntingtin – unknown function • What Tx. is available for HD • None adequate. Psychosocial, dopamine blocking agents (SE), antidepressants/ anxiety drugs • Likely cause of death • Infection, suicide

12. Verbally go through a…. • Cranial Nerve Exam • I - XII • Upper an lower limb neuro exam • Position/Function Tests • Inspect • Tone • Strength/Power • Reflex • Co-ordination • Sensation (Soft, pain, vibration, proprioception)