1. Def:
   1. – Most common & devastating fatal motor neuron disease among adults – characterized by degeneration & loss of motor neurons in the spinal cord, brainstem & brain, resulting in a variety of UMN & LMN clinical S&S
2. Motor Neuron Disorders-Subtypes Table 20.1 p. 820
   1. ALS
      1. – Degen of the corticospinal tracts – Degen of the neurons in the motor cortex & brainstem – Degen of the anterior horn cells in the spinal cord
   2. Primary Lateral Sclerosis
      1. Degen of UMNs
   3. Progressive Bulbar Palsy
      1. Degen of motor neurons of CN IX-CN XII
   4. Progressive Muscular Atrophy
      1. Loss or Chromatolysis of motor neurons of spinal cord & brainstem
3. Epidemiology
   1. – 30,000 individuals in US have ALS – 15 cases dx/day – prevalence > incidents: 4 – 10 cases per 100,000 – average age = mid to late 50s – men > women: 1.7:1; after age 65, diff is less pronounced – 5-10% = inherited (familial ALS [FALS]) – very large majority = no family hx (sporadic ALS [SALS]) – 70-80% = limb onset ALS –w/ initial involvement in the extremities – 20-30% = bulbar-onset ALS-w/ initial involvement in the bulbar muscles – bulbar onset ALS = middle-aged women – bulbar onset Sx: difficulty speaking, chewing, or swallowing
4. Etiology/Possible Mechanisms:
   1. Superoxide Dismutases (SODs)
      1. are a group of enzymes that eliminate O2 free radicals
      2. 3 isoforms of SOD in humans
         1. – Cytosolic copper-zinc superoxide dismutase (CuZnSOD) – Mitochondrial manganese superoxide dismutase (MnSOD) – Extracellular superoxide dismutate (ECSOD)
      3. – ~20% of pts w/adult-onset FALS have mutations in SOD1 (gene on chormosome 21 that encodes CuZnSOD) – When SOD enzyme activity decreases free radicals may accumulate causing damage – Suggested that mutant SOD-1 protein may have toxic properties that calls motor neurons to die (yet to be determined)
   2. Excess Glutamate
      1. excitatory NTs
      2. – Excess glutamate trigger the cascade of events leading to cell death – Increased levels in CSF, plasma & postmortem tissue has been reported
   3. Clumping of neurofilament proteins into spheroids in cell body & proximal axon
   4. Autoimmune rnx
      1. ie: serum factors toxic to anterior horn motor neurons & antibodies to Ca2+ channels
   5. Lack of Neurotrophic Factors
      1. Neurotrophic factors=important in motor neuron survival
   6. Other:
      1. – Exogenous or environmental factors – Apoptosis – Viral infections
5. Pathophysiology
   1. Characterized by: progressive degeneration & loss of motor neurons in the spinal cord, brainstem & motor cortex
   2. Healthy, intact surrounding axons can sprout & re-innervate the partially innervated muscles, in essence assuming the role of the degen motor neuron & preserving strength & fnx early in the disease; but the surviving motor units undergo enlargement. We innervation can compensate for the progressive degen until motor unit loss is ~50%
   3. Involved:
      1. – UMNs in cortex – corticospinal tracts – brainstem nuclei for CN V, VII, IX, X, & XII – anterior horn cells in the spinal cord – degeneration of Clarke's neurons & of the spinocerebellar tracts (reported)
   4. Spared:
      1. – Brainstem nuclei for CN: III, IV, VI (if is involved, occurs late in course) – Motor neurons of Onufrowicz nucleus (Onuf's nucleus)-located in the ventral margin of the anterior horn in the second sacral spinal level; control striated muscles in the pelvic floor, including anal & external urethral sprinters –Sensory syst & spinocerebellar tracts
   5. FALS vs SALS
      1. – Deg of spinocerebellar tracts: FALS > SALS (rare) – Posterior column degen: FALS > SALS (rare)
   6. ALS Progession:
      1. – Thought to spread in a contiguous manner, within spinal cord segments (cervical segments to cervical segments), before developing rostral or caudal Sxs – S&S spread locally within a region (bulabar, cervical, thoracic, lumbosacral) b4 moving to other regions – Caudal-to-Rostral spread w/in spinal cord & spread from cervical to bulbar region occurs FASTER than rostral-to-caudal spread w/in spinal cord
   7. Common Impairments
      1. LMN Patho
         1. – Muscle weakness – hyporeflexia – hypotonicity – atrophy – muscle cramps – fasciculations
      2. UMN Patho
         1. – Spasticity – pathological reflexes – hyperreflexia – muscle weakness
      3. Bulbar Patho
         1. – Dysphasia – dysarthria – sialorrhea – pseudobulbar affect
      4. Respiratory
         1. Exertional dyspnea, nocturnal respiratory difficulty, orthopnea, hypoventilation
      5. Other
         1. Dementia, Cognitive Impairments
      6. Rare Impairments
         1. Sensory impairments, bowel & bladder dysfnx, ocular palsy
      7. Indirect & Composite impairments
         1. Fatigue, wt loss, cachexia, decreased ROM, tendon shortening, joint contracture, joint subluxation, adhesive capsulitis, pain, balance & postural control impairments, gait disturbances, deconditioning, depression, anxiety
6. Clinical Manifestations
   1. Depends on localization and extent of motor neuron loss, the degree and combination of LMN & UMN loss, pattern of onset and progression, body regions affected and stage of disease
   2. Onset-->progression:
      1. – @ onset, S&S=asymmetrical & focal – progression leads to increasing numbers & severity of impairments
   3. CARDINAL SIGN:
      1. Muscle Weakness: by LMN or UMN loss
         1. Initially: usually occurs in isolated muscles, most often distally & followed by progressive weakness & functional limitations
   4. Impairments Related to LMN Patho
      1. Muscle Weakness
         1. Cervical:
            1. May initially notice neck stiffness, feel "heavy-handed" after reading or writing, or may have difficulty stabilizing head with unanticipated movements; as weakness progresses, had begins to fall forward & in more advanced ages the neck becomes completely flexed with head dropped forward
         2. – Leads to decreased ROM: Predisposes patient to join subluxation, tendon shortening, join contractures & adhesive capsulitis – results in ambulation difficulties, deconditioning, & impaired postural control & balance – foot drop; false – patterns & progression of LD weakness: greater losses of muscle force and distal mm compared to proximal mm
      2. Fatigue
         1. – As motor neurons die, remaining neuron/sprouted neurons are overburdened. Week muscles must work at higher percentage of maximal strength to perform same activity = Hastings muscle fatigue – may be related to sleep disturbances, respiratory impairments, hypoxia & depression – in part is due to impaired contraction activation
      3. Atrophy
         1. b/c as m. fibers denervate, their vol decreases
      4. Fasiculations
         1. Thought to be related to hyper excitability of motor axons
      5. Other:
         1. – Hyporeflexia – flaccidity – muscle cramping: thought to be related to hyper excitability of motor neurons; can occur in uncommon sites
   5. Impairments Related to UMN Patho
      1. – Muscle weakness characterized by: spasticity, hyperreflexia, clonus & pathological reflexes (+ Babinski or Hoffman sign) – as the disease progresses, UMN signs may decrease – spasticity==>contractures & deformities, as well as cause dyssynergistic movement patterns, abnormal timing, loss of dexterity & fatigue
   6. Impairments Related to Bulbar Patho
      1. Initially:
         1. – May notice changes in voice, difficulty moving tongue, decreased ability to move lips, or open/closed mouth – may begin to notice drooling at night
      2. Generally
         1. Dysarthria
            1. –Dysarthria: owing to weakness of tongue & muscles of lip, jaw, larynx & pharynx w/initital sx: incld inability to project voice & problems with enunciation – Pharyngeal weakness: air in the mouth leaks into nose during enunciation, resulting in a nasal tone – with progression: speech becomes more difficult & unintelligible==>anarthric
         2. Dysphagia
            1. impaired chewing or swallowing
         3. Sialorrhea
            1. Excessive saliva & drooling -b/c of an absence of automatic, spontaneous swallowing to clear excessive saliva OR -b/c the lower facial muscles are too weak to close lips tightly to prevent leakage
      3. Spastic Bulbar Palsy
         1. where UMNs of bulbar muscles degenerate
         2. Spastic Dysarthria
            1. Voice sounds forced, as more effort is needed to move air through the upper airway
         3. Spastic Dysphagia
            1. Uncoordinated closure of the epiglottis, that may allow liquids or solids to pass to the larynx
         4. Pseudobulbar Affect
      4. Flaccid Bulbar Palsy
         1. where LMNs of bulbar muscles degenerate
         2. Flaccid Dysarthria
            1. The voice sounds hoarse or breathy
         3. Flaccid Dysphagia
            1. Liquids may regurgitate into the nose b/c of pharyngeal weakness & cough reflex may be weak or absent greatly increasing the risk of aspiration
   7. Respiratory Impairments
      1. are related to loss of respiratory mm strength & a decrease in vital capacity
         1. Vital Capacity:
            1. – Reduced to 50% of predicted ==> Respiratory Sx – Less than 25%-30% of predicted ==> significant risk of impeding respiratory failure or death
      2. Early S&S of respiratory mm weakness:
         1. – Fatigue – Dyspnea on exertion – Difficulty sleeping in supine – Frequent awakening at night – Recurrent sighing – Excessive daytime sleepiness – Morning headaches due to hypoxia
      3. As weakness progresses, the following is evident:
         1. – Truncated speech – Orthopnea – Dyspnea – Paradoxical breathing – Accessory muscle use – A weak cough
   8. Cognitive Impairments
      1. Cognitive impairments ranging from mild deficit to severe frontotemporal dementia (FTD)
      2. For Pts. w/ALS-associated FTD:
         1. – Cognitive decline – executive functioning impairments – difficulties with planning, organization & concept abstraction – personality & behavior changes
      3. For Pts. w/ALS w/o FTD:
         1. – Difficulties with verbal fluency – language comprehension difficulties – memory difficulties – abstract reasoning difficulties – generalized impairments and intellectual function
   9. Rare Impairments
      1. Usually spared in Pts. w/ALS but...
         1. Sensory Pathways
            1. May complain of vague, ill-defined sensory symptoms of paresthesia or focal pain in the limbs
         2. External ocular muscles
            1. – If degeneration occurs it does so late in the course of the disease – if maintained on ventilation for long periods of time, may develop inability to voluntarily close the eyes or Ophthalmoplegia, complete ocular paralysis
         3. Motor neurons controlling Anal & Vesicourethral Sphincter mm & mm of Pelvic Floor:
            1. Urinary Sx: Urgency, obstructive micturition, or both have been reported suggesting that supranuclear control over sympathetic, parasympathetic & somatic neurons may be abnormal in ALS
7. Dx
   1. The following exam procedures are used to support the ALS Dx & exclude other dx:
      1. – Laboratory studies – EMG – Nerve conduction velocity studies – Muscle and nerve biopsies – Neuroimaging studies
   2. Dx requires:
      1. The Presence of:
         1. 1. LMN signs by clinical, electrophysiological, or neural pathological exam 2. You in and signed by clinical examination 3. Progression of the disease within a region or to other regions the clinical examination of the of the medical hx
      2. The Absence of:
         1. 1. Electrophysiological & pathological evidence of other diseases that may explain the UMN & LMN signs 2. Neuroimaging evidence of other disease processes that may explain the observed clinical & electrophysiological signs
   3. EL ESCORIAL CRITERIA p. 824-825
      1. – Estab'd b/c of the variability in clinical findings in early stages of ALS & lack of absolute biological diagnostic markers – considered standard for the Dx of ALS for clinical practice, therapeutic trials & other research purposes
      2. Dx of ALS is Classified into:
         1. Clinically Definite
            1. – Defined as both UMN and & LMN the findings in at least 3 of 4 regions (bulbar, cervical, thoracic, or lumbosacral) OR UMN & LMN signs in the bulbar region & at least 2 spinal regions
         2. Clinically Probable
            1. – Defined as UMN & LMN signs the 2 regions, with at least 1 UMN finding rostral to the LMN findings
         3. Clinically Probable w/Laboratory Support
            1. – Defined as UMN & LMN clinical signs in one region only, or UMN signs alone present in 1 region & LMN signs defined by EMG criteria present in at least 2 regions
            2. EMG Criteria Include:
            3. – Signs of active denervation: fibrillation potentials & positive sharp waves – Signs of chronic denervation: large motor unit potentials (increased duration, increased proportion of polyphasic potentials, increased amplitude) – Unstable motor unit potentials
         4. Clinically Possible
            1. – Defined as UMN & LMN signs found together in only 1 region, or UMN signs found alone in 2 or more regions, or LMN signs found rostral to UMN science & the inability to establish a dx of clinically probable, laboratory-supported ALS
8. Disease Course
   1. – Disease course varies ranging from onset to death from several months to 20 years – Average duration = 27 – 43 months; median duration = 23 – 52 months – 5 year survival rates = 9 – 40% – 10 year survival rate = 8 – 16% – 50% survival probability after first symptoms = greater than 3 years unless using mechanical ventilation – Death occurs within 3 – 5 years after Dx (respiratory failure)
9. Prognosis
   1. AGE
      1. strongest relationship to prognosis
   2. Associated w/Better Prognosis:
      1. – Pts age 35-40 @ onset > than older Pts. – Limb-onset ALS > Bulbar-onset – Less severe involvement at the time of diagnosis – a longer interval between onset & diagnosis – no symptoms of dyspnea at onset – Pts. w/psychological well-being > Pts. w/psychological distress (mortality 6.8x greater)
10. Management
    1. Disease-Modifying Agents
       1. No Cure!
       2. Riluzole (Rilutec)
          1. – Approved by FDA for treatment of ALS – is a glutamate inhibitor – 50 mg tablet 2x/day
          2. Side Effects:
             1. – Liver toxicity (which requires discontinuation) – Asthenia – Nausea – Vomiting – Dizziness Effects of Riluzole=modest, extending survival for 2-3 mos
    2. Symptomatic Mgmt
       1. General Info:
          1. – Incurable but is a treatable disease – medical management = palliative – qualitative care = the active total care of patients whose disease is not responsive to curative treatment – medical management: prescription of anti-cramping & anti-spasticity agents, drying agents for sialorrhea, & antidepressants; recommendations & referrals for percutaneous endoscopic gastrostomy (PEG) tube & ventilatory support (noninvasive ventilation, tracheostomy); & discussion of advanced care directives
       2. Multidisciplinary Task Force
          1. was estab'd to develop recommendations for mgmt of ALS
          2. Examined the research & clinical evidence of 5 areas related to the care of individuals w/ALS:
             1. Informing the Pt. & family about dx prognosis
             2. Sx mgmt of Sialorrhea & Pseudobulbar Affect
             3. Nutrition Mgmt & PEG decisions
             4. Mgmt of respiratory insufficiency & ventilation decisions
             5. Advance Directives & Palliative care
       3. Mgmt of Sialorrhea & Pseudobulbar Affect
          1. Pharmacological Tx
             1. –For Sialorrhea: – anticholinergic medications that decrease saliva production:Robinul, Cogentin, Scopolamine, Artane For Associated Thick Mucous Production: – beta blockers: Propranolol, Toprol For Pseudobulbar Affect: – tricyclic antidepressant, Elavil, or SSRIs: Luvox, prescribed 2° to their dual action
          2. Nonpharmacological Tx:
             1. – Manually assisted coughing techniques – Use of suction or mechanical Insufflation-Exsufflation (MI-E) device **MI-E= designed to inflate the long with positive pressure & assist cough with negative pressure through the flip of a switch; peak expiratory "cost" flow within normal ranges achieved thereby assisting with clearance of secretion
       4. Mgmt of Dysphagia
          1. – Early, mild dysphasia = address by nutritionist or registered dietitian together with a speech language pathologist – nutrition status has been identified as a prognostic factor for survival & disease complication – the need for careful attention to nutrition & hydration status is emphasized
          2. Initial tx of dysphagia is directed toward:
             1. Dietary modifications: adapting foods & fluid consistencies for easier & safer swallowing
             2. Patient education regarding dietary strategies for maximizing calories & nutrition & maintaining adequate hydration
             3. Adaptations to promote swallowing such as tucking the chin down during swallowing or performing a clearing cough after each swallow
          3. PEG (percutaneous endoscopic gastrostomy)
             1. – May be recommended when the time required to consume a meal gradually increases owing to fatigue, increased difficulty chewing & frequent choking which may lead to accelerated weight loss – PEG is a type of gastronomy tube inserted via endoscopic surgery that creates a permanent opening into the stomach for the introduction of food – Should be offered to the patient & completed before vital capacity falls below 50%
       5. Mgmt of Respiratory Impairments
          1. Important mgmt Considerations:
             1. 1. Pneumococcal & yearly influenza vaccinations 2. Prevention of aspiration 3. Effective oral & pulmonary secretion mgmt
          2. Supplemental Oxygen
             1. – Use with caution because they can suppress respiratory drive, exacerbate hypoventilation, & ultimately lead to hypercarbia & respiratory arrest – typically recommended ONLY for indivds w/concomitant pulmonary disease or as a comfort measure for pts. who decline ventilatory support
          3. Positive-Pressure Noninvasive Ventilation (NIV)
             1. – Recommended when vital capacity decreases to 50% – shown to decrease symptoms of hypoventilation & increase arrival time by several months – when no longer can be tolerated or is no longer effective, decision must be made between invasion ventilation (IV) w/tracheostomy via surgical intervention or hospice care to address late-stage respiratory Sx
       6. Mgmt of Dysarthria
          1. – Primarily managed by a speech language pathologist – initially managed with intelligibility strategies: exaggerate articulation or decrease the rate of speech & environmental modifications: decreasing background noise
          2. Progresses:
             1. Mgmt=focus on decreasing patients dependent on speech as primary method of communication – Low-tech Devices: writing board or pad & pain; using alphabet board – High-tech Devices: computers with voice synthesizers or single switch, scanning computerized communication systems
          3. Palatal Lift Prosthesis
             1. – For Pts. w/ good articulation but you have a breathy voice quality or decrease loudness because of excessive hair loss through the nose – dental appliance designed to attach to the existing teeth & elevate the soft palate – allows the soft palate to close around the surrounding structures making verbal communication more understandable by reducing/eliminating hypernasal speech – also lowers the hard palate which reduces time that movement allowing speech to be less fatiguing
       7. Mgmt of Muscle Cramps, Spasticity, Fasciculations, & Pain
          1. For Muscle Cramps
             1. – Anticonvulsant meds: Dilantin, Carbamazepine (Atretol, Tegretol), if not relieved w/program of muscle stretching & adequate hydration & nutrition *side effects =GI upset & rash; Carbamazepine=sedation –Benzodiazepines: Valium, Klonopin, Ativan *side effects= sedation, dizziness, respiratory depression, & increased weakness
          2. For Spasticity
             1. – Benzodiazepines: esp Diazepam – baclofen – Zanaflex Side effects: weakness, fatigue, sedation & hypotension
          3. For Fasciculations
             1. – Avoid/minimize caffeine & nicotine – Ativan (decrease intensity)
          4. For Pain
             1. Mild Pain
             2. – Analgesics: acetaminophen or NSAIDs
             3. More Refractory Pain
             4. – Narcotics: codeine, hydrocodone, or methadone
             5. Terminal Stage of ALS
             6. – Morphine (provide analgesia, sedation & relief from respiratory distress)
       8. Mgmt of Anxiety & Depression
          1. Pharmacotherapy & psychological counseling
          2. For Depression
             1. – SSRI: Prozac, Zoloft side effects: agitation & insomnia
             2. If pt. has depression & insomnia or agitation, Tricyclic Antidepressant (Elavil or Tofranil)=preferred
          3. For Anxiety or combo of depression & insomnia
             1. Benzodiazepines: Librium, Clorozepate, Diazepam, Dalamane
          4. For affected respiratory status
             1. Nonbenzodiazepine Anxiolytic: Buspirone (BuSpar) is preferred
11. Framework for Rehab
    1. Early Stage of ALS
       1. – Variety of S&S recognized to Pt. as abnormal – Resultant impairments may or may not cause minor functional limitations & no disabilities will be present
    2. Middle Stage of ALS
       1. – Patient experiences increasing S&S & develops an increase in number of impairments or severity of impairments – Minimal to moderate functional limitations will be noted and as a result disabilities will develop
    3. Late Stage of ALS
       1. – Numerous & increasingly more severe impairments – patient becomes increasingly more functionally limited owing to lack of voluntary motor control & numerous disabilities into – becomes dependent in essentially all aspects of mobility & self-care & may require mechanical ventilation to address respiratory compromise if not already ventilated
    4. Within this framework, impairments, functional limitations & disabilities are managed through restorative, compensatory, or preventative PT interventions tailored to the stage of the disease
12. PT Exam
    1. Impairments may occur as:
       1. 1. Direct impairment – result of the pathology 2. Indirect impairment – sequelae to the pathology 3. Composite impairments – results of multiple underlying origins
    2. The test & measures frequently applicable to Pts. w/ALS include exam of:
       1. 1. Sensory function 2. Muscle performance 3. Coordination 4. Motor function 5. Gait 6. Functional status & activity level 7. Environment 8. Cognitive function
    3. Cognition
       1. – If dementia or cognitive impairments are suspected: executive functioning, language comprehension, memory & abstract reasoning should be examined – Mini-Mental State examination – Referral for neuropsychological eval
    4. Psychosocial Fnx
       1. Referral to a psychologist or psychiatrist for further eval as Depression & anxiety are common 1. Beck's Depression Inventory 2. The Center of Epidemiologic Study-Depression Scale 3. The Hospital Exciting and Depression Scale (HADS) 4. The State-Trait Anxiety Inventory
    5. Pain
       1. 1. VAS
    6. Joint Integrity, ROM, & Muscle Length
       1. Functional ROM, AROM, AAROM, PROM, muscle length & soft tissue flexibility & extensibility
    7. Muscle Performance
       1. 1. MMT 2. Isokinetic muscle strength testing 3. Handheld dynamometer 4. Maximum Voluntary Isometric Contraction (MVIC) – using a strain gauge tensiometer system – this method eliminates muscle length & velocity is factors in testing – the most direct techniques for investigating motor unit loss
    8. Motor Fnx
       1. – Impairments and dexterity, coordination of large movement patterns as well as gross & fine motor control may be evident owing to spasticity & muscle weakness – hand function & initiation, modification & control of movement patterns should be examined
    9. Tone & Reflexes
       1. 1. Modified Ashworth Scale 2. DTR & pathological reflexes (to distinguish between UMN & LMN involvement)
    10. Cranial Nerve Integrity
        1. – CNs commonly affected ALS: CN V, VII, IX, X & XII – determine extent of bulbar involvement – referral to speech language pathologist is recommended
    11. Sensation
    12. Postural Alignment, Control, & Balance
        1. Examine the following:
           1. 1. Static & dynamic postural alignment & body mechanics during self-care 2. Functional mobility skills 3. Functional activities 4. Work conditions & activities 5. Postural stability 6. Reactive control 7. Anticipatory control 8. Adaptive postural control
        2. 1. Tinetti Performance Oriented Mobility Assessment (POMA) –Tinetti Balance test: low scores=impaired bal==>moderately to strongly related to LE mm weakness & functional disabilities – reliable measure for individuals in the early early middle stages ALS 2. Berg Balance Scale 3. Timed Up and Go Test 4. Functional Reach Test
    13. Gait
    14. Respiratory Fnx
        1. Examine the following:
           1. 1. Respiratory symptoms & muscle function 2. Breathing pattern 3. Chest expansion 4. Respiratory sounds 5. Coughing effectiveness 6. Vital capacity or Forced vital capacity using a handheld's spirometer – may test aerobic capacity & cardiovascular pulmonary endurance during early stages using standardized protocols to evaluate & monitor responses to aerobic conditioning
    15. Integument
    16. Fnxal Status
        1. Examine:
           1. 1. BADLs & IADLs 2. Need for adaptive equipment
        2. Tools:
           1. 1. Functional Independence Measure (FIM) (fucntional status) 2. Schwab and England Activities of Daily Living Scale (p. 833)
    17. Environmental Barriers
    18. Fatigue
        1. 1. Fatigue Severity Scale
13. Disease-Specific & Quality of Life Measures
    1. Disease-Specific Measures
       1. 1. ALS Functional Rating Scale (p. 833) – examine the functional status of patients with ALS – valid & reliable for measuring decline in function of results from loss of muscular strength – ALSFRS-R: has internal consistency, construct validity & have retained the properties of the original scale 2. The Appel ALS Scale (AALS) 3. The ALS Severity Scale (ALSSS) 4. The Norris Scale
    2. Quality of Life Measures
       1. 1. SF-36 2. The Schedule for Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW) 3. The Sickness Impact Profile (SIP) 4. ALSAQ-40=an ALS-specific QOL measure (p. 834)
14. PT Interventions
    1. Types of Interventions:
       1. Restorative Intervention
          1. – Directed toward remediating or improving impairments & functional limitations – at best during the early & middle stages because disease progression is expected & permanent loss of function & disability is likely – restorative interventions in late stage are for the most part directed solely toward remediation of impairments that result from other systems pathology
       2. Compensatory Intervention
          1. – Directed toward modifying activities, tasks or the environment to minimize functional limitations & disabilities – in the early & middle stages of ALS, tasks or activities may be adapted to achieve function – in the later stages, increasing environmental adaptations will be necessary to maintain & promote function
       3. Preventative Intervention
          1. – In the early and early-middle stages, preventative intervention is directed toward minimizing potential impairments ( loss of ROM, aerobic capacity, or strength, preventing pneumonia or atelectasis, & functional limitations) – In the late-middle and late stages, pathology is more advanced & mobility becomes progressively restricted to maybe actually difficult/impossible to prevent impairments & functional limitations that are directly related to the nervous system pathology – the role of prevention is tertiary, in order to mitigate the effects of pathology that lead to impairments and other systems
    2. Must Consider when Developing a POC:
       1. 1. Rate of disease progression 2. Extent & area of involvement 3. Stage of disease 4. Respiratory & bulbar factors that may impact participation 5. Timing of intervention 6. Patient acceptance & motivation 7. Life-support choices 8. Availability of psychosocial support 9. Resources
    3. ALS Disease Stages & Common Intervention Strategies: Framework for Rehab Table 20.3 p. 836
       1. Early Stage
          1. Common Impairments & Functional Limitation
             1. 1. Mild to moderate weakness in specific muscle groups 2. Difficulty with ADLs & mobility toward end of the stage
          2. Interventions:
             1. Restorative/Preventative
             2. 1. Strengthening exercises 2. Endurance exercises 3. AROM, AAROM, stretching exercises
             3. Compensatory
             4. 1. Determine potential need for adaptive/Assistive devices 2. Determine potential need for ergonomic modifications of home/workplace 3. Energy conservation 4. Educate patient about disease process, energy conservation & support groups
       2. Middle Stage
          1. Common Impairments & Functional Limitation
             1. 1. Progressive decrease and mobility throughout stage 2. w/c needed for long distances; increased w/c used toward end of stage 3. Severe muscle weakness and some group; mild to moderate weakness and other groups 4. Progressive decrease in ADL skills throughout stage 5. Pain
          2. Interventions:
             1. Preventative
             2. 1. AROM, AAROM, & PROM, stretching exercises 2. Strengthening exercises (early middle) 3. Endurance exercises (early middle) 4. Determine need for pressure relieving devices (mattress)
             3. Compensatory
             4. 1. Support weak muscles (assistive & supported devices, adaptive equipment, slings, orthosis) 2. Modifications to workplace/home 3. w/c prescription 4. Educational caregivers regarding functional training
       3. Late Stage
          1. Common Impairments & Functional Limitation
             1. 1. w/c dependent or restricted to bed 2. Complete dependence with ADLs 3. Severe weakness of UE, LE, neck & truck muscles 4. Dysarthria, dysphagia 5. Respiratory compromise 6. Pain
          2. Interventions:
             1. Preventative
             2. 1. PROM 2. Pulmonary care 3. Hospital bed & pressure relieving devices 4. Skin care, hygiene
             3. Compensatory
             4. 1. Caregiver education regarding transfers, positioning, turning, skin care 2. Mechanical lift
    4. Cervical Muscle Weakness
       1. Progressive cervical extensor weakness
       2. For mild to moderate cervical weakness
          1. A soft foam collar may be worn during specific activities
       3. For moderate to severe weakness
          1. A semi rigid or rigid collar is prescribed
       4. For combo Cervical & Upper thoracic weakness:
          1. Cervical-thoracic orthosis or Sterno-occipital mandibular Immobilizer (SOMI)
       5. For Severe or Intractable neck weakness
          1. Referral to orthotist (custom-made device)
       6. Other interventions:
          1. 1. Taking frequent rest periods 2. Supportive seating such as high-back chairs or recliners 3. Tilt in space or reclining wheelchairs 4. Elevating reading material 5. Education about good arm support for prolonged sitting 6. Proper use of head rest when writing in car 8. Ergonomic changes for work centers
    5. Dysarthria & Dysphagia
       1. 1. Address the patient's head & trunk control & position in sitting 2. Reinforce the use of strategies for eating & swallowing & use of prescribed communication devices with the patient 3. Educate patient, family & caregiver because patient is at risk for aspiration
    6. UE Muscle Weakness
       1. 1. Adaptive equipment use 2. For shoulder subluxation: sling 3. To prevent contractures on proved patient's function (ability to grasp): splinting of wrist or hand
    7. Shoulder Pain
       1. 1. ROM exercises 2. Passive stretching 3. Joint mobilizations 4. Education about proper joint support & protection
    8. Respiratory Muscle Weakness
       1. Educate & show patient & caregivers how to: 1. How to balance activity & rest & educate about energy conservation techniques 2. Educate about signs & symptoms of aspiration 3. Positioning to avoid aspiration (upper cervical spine flexion during eating) 4. Causes & signs of respiratory infection 5. Strategies for managing oral secretions or choking episodes (Heimlich maneuver) 6. Breathing exercises & positioning to optimize ventilation/perfusion matching 7. Airway clearance techniques 8. Use of manually assisted coughing techniques are mechanical insufflation-exsufflation
    9. LE Muscle Weakness & Gait Impairments
       1. 1. Ankle orthosis 2. AFOs= good for patients who have medial lateral instability of the ankle with quadriceps weakness 3. Hinged AFO's = allow dorsiflexion; appropriate for pt w/ adequate the extensor strength with mild ankle strength loss 4. Wheeled walkers 5. Crutches rarely ever used, if so=Lofstrand (Canadian) crutches
    10. ADLs
        1. Common Types of Adaptive Equipment Table 20.5 p. 838
    11. Decreased Mobility
        1. 1. Placing a firm cushion 2 – 3 inches thicker under but not in chair or elevating the chair by placing the legs and prefabricated blocks (for difficulty with sit-->stand or car transfers) 2. Self powered lifting cushions (need adequate trunk control & balance) 3. Powered seat lift recliner chairs 4. Transfer boards 5. Transfer belts 6. Swivel cushions 7. Hydraulic or mechanical lift 8. Electric hospital bed 9. Home modifications & automobile adaptations 10. Chair glides or stairway lifts 11. Wheelchair use for mobility: manual-->powered
    12. Muscle Cramps & Spasticity
        1. 1. Massage & stretching program 2. Cold (decree spasticity) 3. Slow prolonged stretches 4. PROM exercises 5. Postural & positioning techniques 6. Splinting (prevent contractures)
    13. Psychosocial Issues
        1. PT must be able to recognize/differentiate: – recognize the patient's ability to cope & adapt, his/her psychological reactions, level of acceptance & willingness & ability to integrate therapeutic recommendations – differentiate between normal reactionary grief to losses or a change in physical function & the presence of clinical anxiety & depression & referrer patient to appropriate healthcare team member
15. Exercise & ALS
    1. – When prescribed appropriately, exercise may be beneficial, especially in the early stages of the disease – may not improve the strength of muscles are ready weakened by ALS, certainly not those below grade 3 – Gen. AROM & stretching of affected joints, resistive strengthening exercises of unaffected muscles with low to moderate weights, & aerobic activities may be prescribed – when designing strengthening exercise program, PT must take into consideration the balance between overuse fatigue & disuse atrophy – the type & intensity of the exercise program should be carefully monitored & ingested by the PT in order to prevent excessive fatigue, while at the same time promoting optimal use of intact muscle groups – patient should be advised not to reach extreme fatigue and keep track of symptoms of overuse: inability to perform daily activities following exercise due to exhaustion of pain, increase fasciculations, or increased muscle cramping – may be advised to exercise for several brief periods throughout the day, with sufficient rest and between
    2. Disuse Atrophy
       1. – With insufficient activity, disuse atrophy develops and muscle contractions are less than 20% of the total tension in muscle is capable of producing – As contractile proteins are lost, the muscle weakness progresses at a rate of 3%/day
    3. Overuse Fatigue
       1. – ALS pts= abnormal physiological & metabolic responses to single bouts of exercise. Oxygen consumption during submaximal exercise was increase in individuals with ALS compared to controls, & VO2Max & work capacity were decreased – Availability of metabolic substrates of plasma for energy production is affected – a weak or denervated muscle is more susceptible to overwork damage because it is already functioning close to its maximal limits – prescribed exercise training at moderate to low intensities
16. Patient/Client-Related Instruction
    1. – Assist the individual & his/her family & caregiver's account to terms with the impact of the disease – provide psychological support & opportunities for expression of feelings, frustrations & concerns
    2. The scope of education can include but not limited to:
       1. i. Provide accurate, factual information about the disease process and clinical manifestations and their significance in terms of management ii. Instruct patient, family members and caregivers regarding intervention that can be carried out independently iii. Devise the patient about methods to promote general health iv. Counseling regarding care and life decisions v. Referring patients to support groups for psychological counseling vi. Providing information on health and available social and support services