Neuro scenario 1 (MND) Flashcards
Give the pathology for ALS
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the death of pyramidal neurons in the motor cortex (upper motor neurons) and motor neurons in the brain stem and central spinal cord (lower motor neurons).
The mechanism that causes ALS is still not clear but respiratory failure is the most common cause of death. ALS is inherited in 5% to 10% of people. For the rest, the cause isn’t known and is referred to as sporadic ALS. However, evidence from scientific studies suggests that both genetics and environment play a role in the development of ALS. The average survival time after diagnosis is 3-5 years.
ALS is characterized by muscle spasticity and progressive weakening of the muscles. Degeneration of UMNs is responsible for the spasticity and modest weakness. Degeneration of LMNs causes more disabling weakness. As motor neurons stop working, muscles begin to atrophy.
ALS often starts in the hands, feet or limbs, and then spreads to other parts of your body, eventually affecting chewing, swallowing, speaking and breathing
In about 25% of cases, ALS begins with brainstem symptoms (dysarthria, difficulty swallowing) followed by extremity weakness. Frontotemporal dementia develops in a significant proportion of ALS patients
What techniques are available for diagnosing ALS?
ALS diagnosis requires:
evidence of UMN degeneration - spasticity, atrophy
evidence of LMN degeneration - weakness
progressions of S&S - muscle cramping, fatigue
- Electromyogram (EMG)
the electrical activity of the muscle is monitored and tells us when it is contracting and resting. helps to determine if pt has another neuromuscular condition causing the same symptoms. used to confirm LMN degeneration - MRI
uses radio waves and magnetic fields to produce images of the spinal cord and brain. not used often but can provide info that supports the diagnosis of ALS - Blood and urine tests
it allows them to rule out other hypotheses. looking for the marker of ALS - 8OH2’dG - Muscle biopsy
helps to distinguish between muscle disease and ALS. a small bit of muscle is removed via a biopsy needle and is analysed in the lab. used as a last resort for diagnosis
What movements are available at the pelvic girdle?
Anterior tilt - lumbar extension, flexion at hips (sticking your butt out)
Posterior tilt - lumbar flexion, extension at hips (thrusting)
Right lateral rotation - lift left leg off floor so lumbar spine is flexed, right hip slightly abducted
Left lateral rotation - lift right leg off floor so lumbar spine is flexed, left hip slightly abducted
Right and left transverse rotation
What subjective questions could be asked?
When was pt diagnosed?
Health of wife - do they have external help?
Accomodation and modifications
PMH - THREADS and falls history
Full drug list - timings, doses and side effects
Contact with other MDT - psychologist for anxiety
Yellow flags - anxiety, depression, motivation
What would you assess objectively?
- cognition, vision, hearing, speech, swallow
- co-ordination: finger-nose, heel-shin, dysdiadochokinesia
- proprioception: joint positioning, mirroring
- sitting balance
- observe ly-sit and sit-stand
- observe transfers
- tone: PROM and assess end feel
What recommendations can be given to combat fatigue?
- be flexible, don’t force yourself to finish a task
- prioritise
- keep a fatigue log, helps to find patterns and determine which activities fatigue
- track sleep pattern
- sit rather than stand where possible
- dry off using a towelling robe
- use an electric razor and toothbrush
Clinically reason treatment for this pt
There is no cure for MND. Treatment should be aimed at maintaining QoL.
ask pt what he wants
- standing program
movement and weight-bearing maintains ROM, use an ankle weight to stop ankle turning in. neuroplasticity = stand when making a cup of tea
- posture education
lie down so gravity pulls spine down. keep blankets off feet so arent being pushed down into plantar (Achilles reflex causes plantar flexion). when sitting, roll shoulders back to reduce kyphotic posture (pretend to bring shoulder blades together)
- core exercises
aims to increase core stability and strength to help with transfers
- external rotation of hips in supine
- sitting on a gym ball
lie to sits - teach him to use momentum to get sat up and raise the bed so is easier for him and carers
- grip strength
assess prior to treatment, use a rugby shaped stress ball and squeeze 5 times, then place down and pick up with the opposite hand to improve co-ordination and proprioception.
What is hyperreflexia a sign of?
Hyperreflexia is a sign of upper motor neurone damage and is associated with spasticity and a positive Babinski sign
Spasticity = Spasticity, as seen in clonus, is caused by damaged nerves among the brain, spinal cord, and muscles.
Babinski sign = big toe bends up and back to the top of the foot and the other toes fan out.
Normal adult Plantar Reflex presents as a downward flexion.
Note to remember clonus sign also:
Clonus is a type of neurological condition that creates involuntary muscle contractions. This results in uncontrollable, rhythmic, shaking movements. Indicates UMN lesion. Support ankle and quickly dorsiflex. Hold for a few seconds and release, looking for the tremors.
Give some strengthening and ROM exercises for the pelvis and hips.
strengthening:
- sit in chair with arm and back support
- lift knee up in air (marching)
- kick leg straight out, making it slow and controlled
- tap toes up and down, keep heel on floor
- push foot down into gym ball, gives proprioceptive input and intrinsic feedback
ROM:
- sit in chair with good back support
- bring knee up towards chest
- bring ankle up too and hold for 10 seconds
- cross ankle over knee and push down on knee
- put leg on another chair and push down on knee
- to progress, put dressing gown tie around foot and pull back firmly
What outcome measures could be used for this pt?
Incentive spirometry Pt reported outcome measures Pt specific functional scale - choose 5 activities and rate 0-10 Timed ly-sit Timed transfers Timed standing
Briefly describe the anatomy of the shoulder girdle and what movements can occur there
It is a bone ring, incomplete posteriorly formed by:
- scapulae
- clavicles
- manubrium
It connects the upper limb to the axial skeleton and forms the following joints:
- sternoclavicular joint
- acromioclavicular & glenohumeral joints
- scapulothoracic joint (physiological joint)
Movements available:
- protraction: move scapula forward, away from the spine
- retraction: pulls scapula back towards spine & together
- elevation: shoulder girdle moves up, shrugging
- depression: shoulder girdle moves down
- upward rotation: rotating scapula away from ribs, arms adduct
- downward rotation: rotating scapula towards the ribs, arms behind back
How would the neurologist’s assessment affect your treatment?
Neurologist will assess what level of degeneration pt is at.
This will guide treatment by knowing whether spasticity or muscle weakness is the bigger issue
UMN - spasticity, so target ROM
LMN - weakness, so target strengtheing treatments
The assessment can tell you how far progressed the disease is.
How would you assess standing balance and what outcome measures can you use?
- vertical posture/ head alignment, tip of the nose to the sternum, is head leaning to one side
- level of shoulders
- angle of clavicles
- iliac crests, compare angle of ASIS and PSIS - usually 15degrees
- line of appleton - ear to the humeral head
- arm length - compare fingertips
- space between trunk and arms
- knees: apex of patellas level, varus/valgus
- level of medial malleolus
- is head positioned anteriorly/posteriorly
- are shoulder protracted/retracted
- cervical lordosis/ thoracic kyphosis/ lumbar lordosis
- posterior: same acromia, scapula spines & inferior angles, how far away medial borders from the spine.
OMs:
- timed standing unsupported
- trunk control test (scores of 0,12,25)
- roll to weak side
- roll to strong side
- balance in sitting position
- sit up from lying down
