Wooooo!

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 (eg antiepileptics), 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

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

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 dopa decaboxylase 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

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

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

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)

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

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.

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.

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

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)