Parkinson's and MND

Question 1

Discuss the aetiology of Parkinsonism

Answer 1

Collection of symptoms and signs that follow damage to the basal ganglia

Classified as either idiopathic (no known cause)–> this is what we know as Parkinson’s disease or secondary (occurring due to a known cause i.e. Wilson’s disease, exposure to toxins, atherosclerotic ischaemic damage to basal ganglia (stroke), drug induced, neoplasm)

Question 2

Briefly define Parkinson’s disease

Answer 2

PD is a condition ‘associated with progressive loss of dopaminergic neurons in the substantia nigra, as well as with more widespread neuronal changes that cause complex and variable motor and non-motor symptoms’

Question 3

Is Parkinson’s disease a common condition?

Answer 3

Fairly common

1% of population over 50 years are affected (in in 3000/4000 people in AUS)

Question 4

Discuss the typical histological changes seen in the CNS of Parkinson’s disease patients

Answer 4

• Loss of pigment neurons
• Gliosis of SN + other BG
• Presence of lewy bodies

Question 5

Where in the CNS do these changes occur?

Answer 5

Substantia Nigra

Question 6

What is the key biochemical anomaly encountered in PD?

Answer 6

Depletion of dopamine (thought to be responsible for dysfunction of the extrapyramidal system)

Question 7

List and briefly define the clinical features of PD

i.e. signs and symptoms

Answer 7

• akinesia (difficulty initiating motor pattern/lack of movement)
• bradykinesia
• rigidity (led pipe/cogwheel (addition of tremor))
• resting tremor
• impaired automatic postural responses to position and movement
• decreased mm power
• muscle easily fatigued
• myerson’s sign
  Later:
• festinating gait
• monotonous voice
• micrographia (small writing because can’t do big movements and its easier to control
• parkinsonian posture (flexed at hips and hunched
• depression
• dementia

Question 8

Describe the Parkinsonian gait

Answer 8

festinating - pt. walks stooped forward with short suffering steps which is hard to initiate and terminate

Question 9

Describe the Parkinsonian Posture

Answer 9

stooped posture, bent at knees, hips and neck, arms held close to sides, with flexed elbows and wrists with hands outstretched

Question 10

Which of the clinical manifestations of Parkinson’s disease are Primary and which are Secondary?

Answer 10

Primary:

• akinesia
• bradykinesia
• resting tremor
• led pipe rigidity

Secondary:

• cogwheel rigidity (as its a combination of resting tremor and led pipe)
• gait
• micrographia
• depression

Question 11

Discuss the clinical course of PD

Answer 11

onset in ppl >50 years, slow progression
2/3rds disables <5 years
80% disabled after 10 years

Question 12

How is PD treated?

Answer 12

Drugs - levodopa (but has bad side effects)
Surgery
Exercise therapy
Emotional support

Question 13

Discuss the pathological changes encountered in the CNS in patients with MND

Answer 13

UMN loss is characterised by

• atrophy of corticospinal tracts
• pallor of corticospinal tracts
• precentral gyrus atrophy

LMN loss is characterised by

• mm atrophy
• anterior (motor) spinal cord root:
  
  • discolouration of grey matter
  • atrophy of grey matter

scarring of corticospinal tracts, loss of ventral horn and scar tissue

Question 14

List the ssx indicative of UMN lesion/disease

Answer 14

weakness - distal movements i.e. hip flexion and shoulder abduction
spasticity
increased reflexes with clonus
extensor plantar response (babinski)

Question 15

List the ssx indicative of LMN lesion/disease

Answer 15

weakness of individual mm or group of mm supplied by the same nerve
mm wasting
loss of tendon reflexes
hypotonia

Question 16

Briefly define amyotrophic lateral sclerosis

Answer 16

most common form of MND, usually affecting the LMN first

A: atrophy (lack of mm nutrition)
L: lateral tract scarring
S: sclerosis

Question 17

list and write notes on the early clinical manifestations of ALS

Answer 17

LMN: (UL)

• reflexes preserved +/- mm cramps
• mm weakness: progressive distal>proximal
• mm atrophy: prominent early, UL>LL
• fasiculations

Question 18

list and write notes on the late clinical manifestations of ALS

Answer 18

UMN: (LL)

• spasticity (LL>UL)
• hyperreflexia
• clonus and extensor plantar response
• normal abdo reflex, cremesteric and bowel/bladder function

Question 19

Describe the classic triad of clinical manifestations of ALS

Answer 19

atrophic weakness (hands/forearms)
slight spasticity (legs)
generalised hyperreflexia (in spite of amyotrophy)

Question 20

Define progressive bulbar palsy

Answer 20

Progressive paralysis of the medulla oblongata (affecting lower medullary nuclei)

Question 21

Write brief notes on the clinical manifestations of PBP

Answer 21

• affects lower medullary nuclei –> CNS control of Jaw, tongue, pharynx, face and larynx
• rapidly progressing, observe widespread weakness, wasting and hyperactive reflexes, fasciculations and emotional liability

Question 22

Define Progressive Muscular Atrophy

Answer 22

LMN leading to progressive loss

Question 23

Brief notes on the clinical manifestations of PMA

Answer 23

slowly advancing and widespread but only affecting LMN

observe: mm atrophy, weakness and fasiculations

Question 24

How is ALS diagnosed

Answer 24

clinical presentation

Question 25

what is the prognosis of ALS

Answer 25

3-5 years max