6- Amyotrophic Lateral Sclerosis Flashcards
- Definition & Diagnosis - Clinical Presentation - Prognosis - EDX Study - Dysphagia Rehabilitation: Risk Factor, Sign & Symptoms, Conservative & Interventional Management, Sialorrhea - Pulmonary Rehabilitation: Breathing, Cough & Secretion Management, Invasive (IV) & Non-Invasive Ventilation (NIV) - Motor of Rehabilitation - Psychology - Medication
Pathology of Amyotrophic Lateral Sclerosis (ALS) ๐๐
Amyotrophic Lateral Sclerosis (ALS)
Progressive neurodegenerative disorder characterized
Disease of UMN (Corticospinal & Corticobulbar tracts) and LMN (Anterior horn cells)
LMN โamyotrophicโ
Muscle denervation result in weakness, atrophy, and fasciculations
UMN โlateral sclerosisโ
Lateral corticospinal tracts sclerosis result in hyperreflexia and spasticity
Progressive bulbar palsy (Pure Bulbar Palsy)
- Dysarthria: Slurred speech, hoarseness, or decreased volume of speech
- Dysphagia: Aspiration or choking during a meal
Brachial amyotrophic diplegia (BAD)
- Upper limbs focus
Leg amyotrophic diplegia (LAD)
- Lower limbs focus
Progressive muscular atrophy (Full Body without Bulbar)
- Reduced finger dexterity and weakness or wasting of intrinsic hand muscles
- Wrist drop interfering with work performance
- Foot drop; patients may report a โslappingโ gait
- Tripping, stumbling when running
List 6 Amyotrophic Lateral Sclerosis-Plus Syndromes
ALS = Pyramidal .. think of other tracts
- Sensory loss
- Autonomic dysfunction
- Extrapyramidal signs
- Parkinsonism
- Ocular motility abnormalities
- Frontotemporal dementia (FTD)
What is the differential diagnosis of ALS? ๐๐ MOCK
- Multifocal motor neuropathy (most common)
- Cervical cord/foramen magnum lesions (tumor, syringomyelia, syringobulbia, spondylosis)
- Vitamin B12 myelopathy
- Multiple sclerosis
- Spinal muscular atrophy
- Myasthenia gravis
- Thyrotoxicosis
Neurology Secrets 6th Edition Chapter 6 MND pg92
23 yr old male presents with bilateral hand weakness with upper and lower motor neuron signs, leg weakness and bilateral spasticity. What is diagnosis? ๐๐
- ALS
- Transverse myelitis
Canada Q Bank
Amyotrophic lateral sclerosis (ALS): Pathology, Affected vs Spared ๐๐
Pathology
- Degeneration of the anterior horn cell & corticospinal tracts
Affected
- Bulbar & Pseudobulbar: Dysphagia, Dysarthria, Emotional Liability, Sialorrhea, Dysphonia
- Thoracic: Respiratory failure
- Limbs: Asymmetric weakness, Atrophy & fasciculations
Spared (Face, BBS, Sensory)
- Sensation
- Extraocular muscles
- Bowel and bladder
- +/- Cerebellar
Cuccurollo 4th Edition Chapter 5 EDX pg447-448 Table 5-61
List 4 Causes of Bulbar vs Pseudobulbar palsy ๐๐
Common Sing & Symptoms ๐
Bulbar Dysfunction
- DYSPHONIA & Difficulty breathing (airway obstruction)
- DYSARTHRIA & Slurred speech
- DYSPHAGIA
- Oral: Difficulty in chewing, Drooling of saliva
- Pharyngeal: Nasal regurgitation.
- Oesophageal
Difference
- Flaccid (LMN) vs spastic (UMN) tongue and jaw
- Hypo (LMN) vs Hyperflexia (UMN) Jaw Jerk
- Emotional liabelity (pseudobulbar)
Negative and positive factors affecting prognosis & Average survival. ๐๐ MOCK
Positive Factors
- Percutaneous endoscopic gastrostomy (PEG) tube
- Non-invasive ventilation (NIV)
- Riluzole
- Multidisciplinary Clinics
Negative Factors
- Female
- Old age of diagnosis
- Definite diagnosis of ALS
- Bulbar Onset
- Rapid progression
Braddom 6th Edition Chapter 40 MND Box 40.5
Prognosis & Mobility of ALS ๐
Average survival
- 3-5 years from onset
- 15 months from diagnosis
Prognosis
- 50% die within 3 years
- 30% live for 5 years
- 10% live for 10 years
Wheelchair
- by 12โ18 months
Cuccurollo 4th Eiditon Chapter 5 EDX pg446-447 Table 5-61
Braddom 6th Edition Chapter 40 Box 40.5
EMG Criteria for definitive ALS. ๐๐ MOCK
Clinically definite ALS
- Active denervation with reinnervation must be found in three of four body segments (craniobulbar, cervical, thoracic, and lumbosacral).
- At least two muscles with different innervations should be abnormal
- LMN and UMN signs in three of four body segments
Clinically probable ALS
- Evidence of LMN and UMN signs in at least two regions
- PLUS UMN signs above the LMN signs.
Cuccurollo 4th Edition Chapter 5 EDX pg448 Table 5-61
Braddom 6th Edition Chapter 40 MND pg831 Box 40.5
ALS. Why they are considered at higher risk for malnutirion?
What are the Signs of Dysphagia? ๐
Risk Factor for Malnutirion
- Bulbar muscle dysfunction due to motor neuron involvement in the brainstem
- Fatigue and slow eating due to bulbar weakness โ Increased time required to eat
- Difficulty transferring food to the mouth due to arm weakness.
- Depression
- Hypermetabolic state due to increased respiratory effort
Bulbar Involvement
- Dysarthria: Slurred speech
- Dysphonia: Hoarseness
- Dysphagia
- Oral Phase: Difficulty Chewing, Swallowing
- Pharyngeal Phase: Choking & Aspiration even with saliva
- Esophageal Phase: GERD, Vomiting
- Complications: Malnutrition, Aspiration pnuemonia & Weight loss
DeLisa 5th Edition Chapter 28 MND pg731
Braddom 6th Edition Chapter 40 MND pg836
Feeding Management in MND. ๐๐
FOOT
- Diet Type: Using thickener for liquids and soften moist solid food
- Diet Route: PEG tube with high caloric supplements
COMPENSATORY STRATIGIES
-
Low Risk Feeding Strategies
- Sit up at 90 degrees
- Small amounts
- Donโt mix solids and liquids (i.e., mixed consistencies)
- Supervision
- Remain upright 3o minute
- Focus on eating and eliminate any distraction (Tv)
-
Compensatory Techniques
- Chin tuck
- Head turn to weaker side
- Head tilt to stronger side
-
Swallowing Maneuvers
- Double, dry, effortful swallow
- Supraglottic & Supra-Supraglottic swallowing
-
Clearing Techniques
- Liquid mouth wash
- Manual removal by fingers
Dr. Maitham Note
Risks / Complications of PEG tube placement ๐๐
๐ก Overall risk of complications for PEG tube placement in patients with ALS is 6% to 10%
- Tube displacement
- Pneuomoperitoneum
- Hemoperitoneum
- Peritonitis
- Gastrointestinal bleeding
- Gastrocolic fistula
- Death
โMany patients decline a PEG tube because they are uncomfortable with the idea. This can be due to lacked information about the role and potential benefits of a PEG tube or mistaken by their swallowing feeling adequate. This often leads to a delay in the discussion, at which point the risk of placing the PEG increasesโ
Braddom 6th Edition Chapter 40 MND pg837
How to monitor feeding in ALS? ๐๐
When do you refer ALS patient for PEG insertion? ๐๐
MONITOR DYSPHAGIA
- Malnutrition
- Reduced caloric intake
- Weight loss more than 10% of bodyweight
- Dehydration
- Repeated aspiration pneumonia
- Choking
- Repeated aspiration pneumonia
EVERY 3 MONTHS
- Bodyweight measurement
- FVC >50%
PEG TUBE
- Once FVC <50%
- Moderate to high risk (operate vs palliative care)
Braddom 6th Edition Chapter 40 MND pg834 Box 40.4
DeLisa 5th Edition Chapter 28 MND pg732 Figure 28-7
Impact of Sialorrhea on Quality of Life, Managment & their S/E ๐๐
SIALORRHEA
- Anterior: Socially disabling & Oral infections
- Posterior: Choking and aspiration pneumonia
NON-PHARMA
- Suction machine
- Towel and verbal cueing
PHARMA
- Amitriptyline 25-50mg 3 times daily (Anticholinergic)
- Atropine drops 0.25 to 0.75 mg 3 times daily (Anticholinergic)
- Transdermal scopolamine (Anticholinergic)
- Botulinum toxin type A given every 3 months at a dose of 7 to 22.5 units into each parotid gland โ jaw dislocation
INTERVENSION
- Radiation
- Surgical Excision
Braddom 6th Edition Chapter 6 MND pg837
Why ALS have comporomized respiratory function? ๐๐
BREATHING
- Bulbar Dysfunction โUpper Dysfunctionโ
- Sialorrhea
- Dysphaiga leading to aspiration Pneumonia
- Weak upper respiratory muscles leading to collpase of upper airways
- Respiratory Dysfunction โLower Dysfunctionโ
- Poor intercostal muscles leading to Inadequate ventilation โ Atelectasis
COUGH
- Difficulty generating a strong cough
- Inability to take a deep breath
- Weak exhalation muscles
CLERANCE
- Inadequate mucus clearance
- Thick mucus due to anticholingeric agents, decreased intake of liquids and weakened cough
Dr. Maitham