Chp 20 ALS Flashcards
What is ALS
Amyotrophic Lateral Sclerosis - is a motor neuron degenrative disorder. Which means that it includes a heterogeneous spectrum of inherited and sporadic clinical disorders of the Upper and Lower motor neurons or a combo of both. comomonly refered to as Lou Gehrigs disease and is the most fatal among adults. This is disease is characterized by Spinal cord, brain stem, and brain resulting a variety of UMN and LMN.
Describe ALS. Prmary Lateral Sclerosis, Progressive Bulbar Palsy, Progressive Muscular Atrophy,
ALS- Degeneration of the corticospinal tracts, neurons in the motor cortex and brainstem and anterior horn cells in the spinal cord.
Primary Lateral Sclerosis- Primary degenration of UMN
Progressive bulbar Palsy- Degeneration of motor neurons of cranial IX to XII
Progressive Muscular atrophy- Loss of chromatolysis of motor neurons of the spinal cord and brainstem
Etiology what are the think theories believed to cause ALS remember exact cause is unkonwn
- Superoxide dismutases- (SODS) - Group of enezymes that elminate oxygen free radicals. On chorsome 21 the SOD-1 mutations
- Glutamate - Glutatmate an excitatory neurotransmitter excites cells so much that it can cause cell death. Excess can be found in the CSF if this is present
- Clumping of a neurofilament proteins into spheroids in the cell body and proximal axon is one of the histopathological characteristics of ALS
- Autoimmune response- For example serum factors toxic to anterior horn motor neurons in individuals with ALS have been reported and antibodies to calcium channels have been identified
- Lack of neurotrophic factors can lead to ALS . Due to the lack of the neuron growth cells the motor neurons start to die without the proper nourishment. This leads to this neurodegenrative disorder
- Exogenous ot enviromental factors, apoptosis (pre-programmed cell death) and viral infections
Pathophysiology - which cranial nerves can the ALS primarly affect and which are usally spared
ALS primarly affects- Brainstem for cranial nerves V(trigeminal), VII (Facial), IX (glossopharyengeal), x (vagus), and XII (hypoglossal) and anterior horn cells in the spinal cord.
Usally spared parts. Brainstem nuclei for nuclei external ocular movements III and IV and VI are usally sparred. Striated muscles of the plevic floor including external and anal sphincters.
The sensory systems and spinocerebellar tracts re also generally spared in ALS as well.
Impairmenrs with LMN Impairments seen with UMN impairments with bulbar pathology Respiratory impairments other impairments rare impairments indirect and composite impairments
(explain each one
LMN- Muscle weakness HYPOREFLEXIA, HYPOTONICITY, ATROPHY, MUSCLE CRAMPS, FASCICULATIONS - lower motor neuron root to spinal cord. Can lead to falls and balance issuses, foot drop
UMN- Spascity. Pathological Reflexes, HYPER reflexia, muscle weakness head drop leading forward weak neck extensors. claw hand
Impairments related to bulbar affect (think speech) dysphagia, dysarthia, sialorrhea, pseudobublar affect
Respiratory impairments- Exertional dyspnea, nocturnal respriatory difficulty, orthopena, hypoventilation,
other impairments- dementia, cognotive impairments
Rare impairments- Sensory impairments and bowel and bladder dysfunction usally doesn’t progress to this level
Indirect and Composite impairments- Fatique, weight loss, decreased ROM, tendon shortening, joint subluxation (probadly especially at the shoulder). adhesive capulitis, pain, balance and postural control, gait disturbances, decondtioning, depression, anxiety
Diagnosising ALS can be put into Suspected ALS, Possible ALS, Proable ALS, Definite ALS
What techiniques are typically used to D/X ALS
ALS is a combination of lab tests and these tests include EMG, nerve conduction velocity, nerve biopsy, neuroimaging techniques
Weakness/atrophy/hyperreflexia/spascity/ progression over time usually indications for ALS study
Suspected ALS - in only one Region like one region like left arm or left leg LMN >= 1 or UMN >/=1
Possible ALS- LMN + UMN 1 region
Proable ALS - LMN + UMN 1 region or UMN .>/=1 acute EMG >/= 2 limbs
Definite ALS - LMN + UMN 3 regions
Management of ALS what is the drug of choice
Riluzoe and its a glutamate inhibitor and it was approved by the FDA except it has vicious side effects and You can use palliative care just basically providing the patient with psychological, social, and spiritual problems is paramount.
1) inform the family and patient about disease and the prognosis
2) symptom management of sialorrhea and pseudobublar affect
3) nutrition management and PEG decisions
4) management of respiratory insufficiency
Sialorrhea control
Alogrorithim pg. 827 If excess salvia is it at night usally not treated. During the day and the night then use anticholenergics such as glycopyrrolate and Pseudobulbar affect present use Luvox or SSRI’s. Then recheck every 3 months to see how symptoms are.
Management of dysphagia
A combined effort of both the speech pathologist and a nutritionist. Modify the diet and keep chin down when swallowing and maintaining adequate hydration
Management of Respiratory problems
1) pneumoccocal and influenza vacinations yearly
2) prevention of apsiration 3) effective oral and pulmonary secrection management (think of john when he helped faciliatate the stuff out of garys lungs and supplemental oxygen for patients with respiratory disease
Management of Dysarthia
speech lanuage pathologist not our field
Management of Muscle cramps, spasticity fasciculations and pain.
Management of Anxiety and Depression
Muscle cramps- tegretol, Aretorl and muscle stretching and adequate hydration
Spascity- baclofen
Fasiculations - avoid nicotine and caffeine my two favorites.
Mild pain- NSAIDS
Severe Pain- Hydrocodene via prescription
Anixety and Depression - Prozac and Zoloft
Review chart pg. 831 Framework for stages of ALS
Things a PT should look for in an Examination
Cognition- No ALS specific cognition test is available however, check the memory, launage, and abstract reasoning if something wrong is suspected. The Mini mental state
Psychosocial Function - Anxiety and depression are common refer to a psychologist
Pain - VAS
Muscle Performance- MMT, MVIC (Maximum voulantary muscle contraction,
Motor Function- Check for impariments in fine and gross motor control. Look for dexetrity in fingers.
Tone and Reflexes- Modified Ashworth and Deep tendon reflexes via the reflex hammer
Cranial Nerve Intergrity - Especially V, VII, IX, X, and XII ones that basically control the face muscles and mouth and throat.
Sensation- Close eyes let me know if you feel this on the left or on the right
Postural Aligment Control and Balance - POMA
Time up and go test, Berg Balance Scale
Gait- no ALS specific gait test or measures exist.
orthotics and AD’s
Respiratory Function- breathing patterns, chest effectivenss, breathing sounds, muscle function, and spriometers
Integument- look for any possible areas or things that can cause skin abrasions
Functional Status- FIM scale and mobility skills, Schwab and England Activites of Daily Living
Enviromental barriers and Fatique- Fatique Severtiy scale
Restoratvie, Compensatory, and Preventative interventions
Restorative- is directed toward re-mediating or improving impairments and functional limitations. In the later stages these types of interventions aim to restore problems from other systems such as edema, pneumonia, atelecltasis, and adhesive capsulitis.
Compensatory- is directed toward modifying activities, tasks or the environment to minimize functional limitations and disabilities
Preventative - is directed toward minimizing potential impairments, such as loss ROM, aerobic capacity or strength. Establishing an early prevention program may alter impairments and decrease physical disability temporarily, and may also increase the well being of the patient and prevent fatigue.
Dysarthia and Dysphaiga what can the PT do also look over chart on page 836.
For dysarthia and dysphagia although its primarily the SP job a PT can still teach proper head and neck positioning
