Parkinson's and Motor Neuron Disease Flashcards
Discuss the aetiology of parkinsonism
- Idiopathic Parkinsonism - Known as “Parkinson’s DISEASE”
- Secondary Parkinsonism - Referred to as “Parkinson’s Syndrome”
- Postencephalitic parkinsonism
- Atherosclerotic ischaemic damage to the basal ganglia
- Drug induced : phenothiazines, reserpine
- Exposure to toxins
- Shy-Drager syndrome
- Wilson’s Disease
Briefly define the term Parkinson’s Disease
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
Is parkinson’s disease a common condition?
Most common neurological disorder.
1% of individuals >60 yrs.
Prevalence: 120/100,000
List and briefly define the clinical features of Parkinson’s disease.
- Symptoms
- Signs
6 Cardinal clinical features of parkinsonism:
- Tremor at rest Flexed posture of neck, trunk & limbs - Rigidity - Loss of postural reflexes - Bradykinesia, Akinesia - Freezing phenomenon
- Clinical picture = combo of:
- Akinesia
- bradykinesia
- rigidity
- resting tremor
- impaired autonomic postural responses to position or movement.
- decreased muscular power
- muscles are easily fatigued
- later stages:
- depression
- dementia - progressive changes:
- festination gait (stooped + shuffle, hard to start and stop)
- voice drops in amplitude + monotonous
- micrographia (writing)
- parkinsonian posture:- stooped,
- bent knees and hip and neck,
- arms held close to sides flexed at elbows and wrists,
- hands outstretched, extended thumbs, flexed MCP joints
Which of the clinical manifestations of Parkinson’s disease are primary i.e. due to the histopathological changes, and which are secondary i.e. due to a combination of primary clinical manifestations?
Primary:
- Akinesia
- bradykinesia
- rigidity
- resting tremor
- impaired autonomic postural responses to position or movement.
- decreased muscular power
- muscles are easily fatigued
Secondary:
- depression
- dementia
- festination gait
- voice drops in amplitude + monotonous
- micrographia (writing)
- parkinsonian posture:
Describe the Parkinsonian Gait and Posture.
Parkinsonian Gait:
- festination gait (stooped + shuffle, hard to start and stop)
Parkinsonian Posture:
- stooped,
- bent knees and hip and neck,
- arms held close to sides flexed at elbows and wrists,- hands outstretched, extended thumbs, flexed MCP joints
Discuss the clinical course of Parkinson’s disease.
Clinical Course
- Usual onset is 50 years of age and older.
- Slow progression.
- Two thirds of patients are disabled within 5 years.
- 80% of patients are disabled after 10 years.
How is Parkinson’s disease treated?
- Drugs e.g. Levodopa -
- Surgery
- Emotional support
- Exercise
Discuss the pathological changes encountered in the CNS in patients with MND.
Upper motor neuron (UMN) loss is characterised by:
- atrophy of cortiscospinal tracts
- pallor of cortiscospinal tracts
- sometimes: precentral gyrus atrophy
Lower motor neuron (LMN) loss is characterised by:
- muscle atrophy
- discolouration of grey matter Of the anterior (motor) roots of the spinal cord
- atrophy of the gray matter
Note different combo’s of changes are seen in different types of MND
List the symptoms and signs which are indicative of upper motor neuron (UMN) disease.
- Weakness
- distal movements
- Hip flexion
- Shoulder abduction
- spasticity
- increased tendon reflexes
- extensor plantar response
List the symptoms and signs which are indicative of lower motor neuron (LMN) disease.
LMN Weakness fasciculation muscle wasting loss of tendon reflexes hypotonia
Briefly define the term Amyotrophic Lateral Sclerosis (ALS)
ALS is a specific disease that causes the death of neurons which control voluntary muscles characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size
a. List and write notes on the early clinical manifestations of ALS.
b. List and write notes on the late clinical manifestations of ALS
Early: Mainly LMN ssx Muscle weakness - progressive - affects distal structures more than proximal structures eg small hand mm Muscle atrophy - prominent - Early - affects upper limbs early, lower later - extraocular muscles are usually preserved Fasciculations - in atrophying mm Reflexes - deep tendon reflexes preserved in early stage (esp upper limb) +/- Muscle cramps
Late: Add UMN ssx Spasticity (esp lower extremities) Hyperreflexia (esp lower extremities) Clonus Extensor plantar response Normal: - superficial abdominal reflexes - bladder function - Cremasteric - bowel function
Describe the classic triad of clinical manifestations in ALS.
atrophic weakness of hands/forearms
slight spasticity of legs
generalised hyperreflexia - in spite of amyotrophy
a. Define the term Progressive Bulbar Palsy.
b. Write brief notes on the clinical manifestations of Progressive Bulbar Palsy.
PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts. This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII)
Both UMN and LMN are affected Affects muscles of the: - Jaw - Tongue - Pharynx - Face - Larynx - NOT extraocular mm
