week 2 neuro summary.pdf

2.1 intro and clinical presentation autoimmune disease MS autoimmune disease autoimmunity classifications 1. systemic :entire body is affected ex: lupus, RA, dematomyositis 2. organ specific : organ targeted by immune system based on receptor sensitivity ex: thyroid- hashimoto or graves type 1 diabe...

2.1 intro and clinical presentation autoimmune disease MS autoimmune disease autoimmunity classifications 1. systemic :entire body is affected ex: lupus, RA, dematomyositis 2. organ specific : organ targeted by immune system based on receptor sensitivity ex: thyroid- hashimoto or graves type 1 diabetes-pancreas neuromuscular- myasthenia gravis myelin- MS and BS are target tissues pathophysiological differences GBS- PNS MS-CNS- brain and sc autoimmune pathophysiology autoimmunity: immune-mediated response of self antigens immune system begins to attack body by generation of auto antibodies normal circumstances: body has a natural autoimmunity and auto antibodies that exist as a way for the body to develop effective immune-related responses to pathogens. exaggeration of autoimmune response occurs if pt becomes susceptible. etiology (combo) genetics lifestyle environment diet stress normal aging microbiome: influencer of local and systemic inflammatory responses -according to rosenbaum and colleagues genetic allele: shown to provide the predisposition regardless of trigger -according to atassie and kosali molecular mimicry once external stresses are added to a normal system, body's immune system responds by releasing inflammatory cytokines to mediate problem=causes activation of selfreactive lymphocytes= destruction of the target tissues. immune system mistakes body for pathogenic molecules MS target tissue: myelin, oligodendrocytes and nerve cells of CNS *all autoimmune diseased originate from maladaptive factors in body but target tissue is diff -ppl w autoimmune disease are more prone to others if they dont mitigate prob why the fek do this gotta be so long clinical presentation myelin: meant to serve as insulator to nerves & aid in transmission of APs (bc of nodes of ranvier) forms a coating around nerve creating a tunnel so APs can flow w no slowdown symptoms: begin w visual disturbances ex: diplopia and nystagmus presentations extensions of plaques paresthesias bradykinesia fatigue abnormal tone tremors ataxia pain dysarthria generalized weakness forming damsons fingers around corpus callosum bc of break down of myelin and nervous tissue white plaques shown in T2-weighted MRI to diagnose MS -could be a distinguishing factor to rule in/out 4 phenotypes of MS 1. clinically isolated syndrome (CIS) isolated event of inflammatory demyelination that could progress to other forms if left unchecked no other clinical features shown only MRI can show can progress to 2. relapsing remitting (most common) discrete attacks followed by near baseline recovery 3. 4. secondary progressive worsening symptoms after relapse attack primary progressive continuous worsening of symptoms from 1st onset occurs w/out earlier relapses pts may have relapses attacks thru progression relapsing remitting for a little and then convert to secondary and get worse Summary Multiple Sclerosis manifests as an organ-specific autoimmune disease 4 Multiple Sclerosis Phenotypes 1. Clinically Isolated Syndrome 2. Relapsing Remitting 3. Secondary Progressive 4. Primary Progressive different presentations bc different lesions show up in new spots 2.2 medical management pharmacological interventions for acute exacerbation pharm management recommended for MS 1. methyprednisolone a corticosterioid drug w intention of decreasing inflammation w/in body and provides an immunosuppressive effect to decrease symptoms of attack administration: large doses intravenously for 3-5 days followed by a tapered oral administration many side effects! 1. plasmapheresis -provided after acute exacerbations utilized when pts do not respond well to corticosteroid treatment how its done healthcare professionals extract blood from pt and run it thru machine that separates red blood cells, white blood cells and plasma plasma portion is discarded and replaced w albumin substitution fluid disease modifying agents (DMA) attempt to decrease future disease attacks 4 themes of DMAs 1. need for early disease control to reduce i think we need to know these degree of disability 2. recognizing physical impairments that result from attacks and progression-one aspect of disability that needs to be mitigated 3. variable prognosis amongst pts 14 pharm DMAs approved by FDA 4. treatment adherence and any barriers to adherence-early one DMA is synthetic interferon drugs or beta interferons ex; avonex slows down immune response and prevent activated T cells from crossing BBB kappas and colleagues say deep delay in disability after administration primary progressive MS ocrevus monoclonal antibody that works to deplete B cells from circulating side effects of all these drugs flu like symptoms injection site discomfort rash cardiac toxicity (severe cases) anaphylaxis hepatotoxicity amenorrhea infertility ALL ARE FOR RELAPSING FORMS OF MS spasticity does not moderate symptoms can lead to lethargy and sleepiness 1. oral baclofen in decreasing spasticity consider: oral meds- no regulation of usually 1st to be prescribed and very effective initial administration is low w titration is based on results ???girl what effects on particular region of body pts that rely on spasticity to move can present w flaccidity instead bc of meds 2. tizanidine decreases ability for fxnal intervention 3. dantrolene sodium 4. diazepam (valium) 5. intrathecal baclofen (ITB) more invasive, less sedating pts have to demonstrate lack of efficacy w oral spasmodic and/or adverse effects and no contraindications to surgery administration: small pump inserted w a catheter into intrathecal space of sc to make direct contact with CSF.. pump then able to be titrated thru pts skin if more/less meds needed not local intervention may decrease muscle tone 6. botox used to assist w local spasticity doesnt affect body systemically lasts 3-4 months PT needed to improve length and ex: for this pt they use botox and it helped w his extension proprioceptive aspects of muscle in hopes to decrease ROM deficits 7. tendon lengthening last resort bc it comes w most challenges mostly used for improved QoL pain 1. neuropathic pain: burning type or pins & needles cymbalta® (selective serotonin and norepinephrine reuptake inhibitors (SSNRIs) lyrica® (Pregabalin) to treat pain 2. paroxysmal pain: characterized by erythema of skin and warmth (flushed) w attacks of extreme pain tegretol® (Carbamazepine) elavil® (Amitriptyline) dilantin® (Phenytoin) valium® (Diazepam) neurontin® (Gabapentin) 3. dysesthesias: noxious sensitivities to touch elavil® (Amitriptyline) tofranil® (Imipramine) norpramin® (Desipramine) flashcards fatigue 1. symmetrel (amantadine) 2. provigil (modafinil) 3. fatigue management/exercise: better than meds tremor and nausea 1. Tremor a. antihistamines b. propranolol (Beta Blocker) c. Klonopin® (Clonazepam – Anti deep brain stimulator may be surgically implanted to help manage side effects Anxiety) d. deep Brain Stimulators (DBS) 2. Nausea a. Antivert® (Meclizine) can be used for general nausea b. Zofran® (ondansetron) a,b prescribed c. Phenergan (promethazine) c not very effective cognitive and emotional deficits 1. Cognitive Deficits a. cognitive Retraining b. aricept® (Donepezil) c. namenda® (Memantine) for alzheimer’s 2. Emotional Deficits/Anxiety a. prozac® (Fluoxetine) b. paxil® (Paroxetine) c. zoloft® (Sertraline) treated w consultation of a speech and language pathologist cognitive retaining protocols often used anti anxiety meds depression bowel and bladder deficits 1. neurogenic bladder: unable to control urine output due to NS deficits a. spastic : cause incontinence and abnormal emptying -anticholinergic medications 2. flaccid - crede maneuver: manual downward pressure on abdomen - intermittent self-cathetarization ~4-5x a day 3. both (dyssenergic) - alpha adrenergic blockers - anti-spasmodics pts w MS often have difficulty w bowel impaction caused by constipation 4. bowel programs & dietary recommendations of increasing fiber intake are recommended to prevent stool impaction pts should have an organized toileting routine to improve QoL and social QoL Summary Medical Management of the disease Methylprednisolone Plasmapheresis Disease-Modifying Agents (DMAs) Medical Management of the symptoms Pharmacological agents Patient education 2.3 nutritional and alternative management-MS pharm interventions decrease adherence to medical protocols diet and nutrition minimal dietary advice for ppl w MS swank diet 1990 -low saturated fat daily no processed foods containing saturated fat or hydrogenated oils (cant exceed 15g per day & unsaturated fat at 20-50g/day) eat as many fruits and veggies as desired no red meat, including pork (for 1st year) then 3 ounces per week after that skinless poultry and white fish permissible no dark meat poultry and limited fatty fish dairy products w/out butter egg whites only cod liver oil w other supplements can eat whole grains and nuts dairy (may increase risk)- further research needed microbiome controlled fat intake was associated w decreases in the inflammatory process -research showing the role of leaky gut or increased intestinal permeability to pathological material into the bloodstream supporting fxns of gut and microbiome may help slow the diseases progress or symptomology the wahls protocol est. 2014 emphasizes importance of diet and nutrition & management of MS terry wahls, MD- diagnosed w MS in 2000 -wheelchair bound due to progression & given chemo meds (didnt help) then went into research institute of fxnal medicine (research of autoimmunity) feed and support the mitochondria inclusion of -paleo diet diet similar to ^^.. 9 cups of veggies and colorful fruits daily dairy free, gluten free, organic grass fed and wild caught went from wheel chair to riding 18 proteins and good fats: coconut, avocado and olive oil miles -exercise: stretching -electrical stimulation https://terrywahls.com/about/about-terry- -sleep, stress reduction and reduce toxic exposure wahls/ all of these factors improve QoL MS hope matthew embry- diagnosed in 1995 “living proof” documentary MS-direct him and his fam provide free evidence for those w MS and its all rooted in scientific evidence basis in diet, nutrition, exercise and other treatment like CCSVI vitamin and mineral supplementation epidemiology MS further from equator more likely to get MS (those in northern hemisphere)-lack of sun sunshine: only natural contributor to vitamin D vitamin D: hormone not vitamin decreased vitamin intake (sun or oral)= high risk of MS some say diary puts you at high risk while others say it helps antioxidants used to decrease oxidative stress reaction of body polyunsaturated fatty acids: low fat diet w antioxidant and polyunsaturated fatty acids supplementation led to significantly lower C-reactive protein -decreases rate of relaps and associated fatigue swank and wahls-condone intake of cod liver oil as a source of polyunsaturated fatty acids vitamin A research says that increased uptake of vitamin A vitamin D polyunsaturated fatty acids (PUFAs) vitamin B12 alpha-lipoic acid improves cognition and counter disability vitamin B12 based on immunomodulatory effects and need to form myelin sheath low B12 has been associated w RR MS alpha lipoid acid variabilities exist- more research needed alternative therapies and treatments meditation: good adjuvant to traditional interventions stem-cell therapy -research has shown to suppress MS for 4-5 yrs after stem cell tx in 70-80% of pts -procedures: good for young, ambulatory & have inflammatory or RR form of MS chronic cerebrospinal venous insufficiency (CCSVI) -no significant difference summary Multiple alternative treatments - Diet/Nutrition - Vitamins and Supplementation Alternative Interventions - Meditation - Stem Cell Therapy -CCSVI all methods need further research 2.4 examination-MS like all pts a thorough examination is required for pts w MS test and measures: cognition and psychosocial fxn outcome measures for cognition 1. (MECFIMS) important to consider: *scheduling -speed and working memory -learning and memory ask pt what type/time of day -executive fxn is best for them to avoid -visual spatial processing fatigue tests and measures: sensation and pain sensation outcome measures nottingham sensory assessment monofilament testing -both not validated but still used for info of sensation -word retrieval be cognizant of presentation and how long your evalv is visual analog scale pain mcgill pain questionnaire neuropathic pain scale brief pain inventory VAS and these 3 pain scales used to quantify pain and sensation *WE NEED to know monofilament test, VAS and Mcgill pain provides 2 specific domains focused on cognitive processing via: fatigue is a BIG factor when working w MS pts minimal examination of cognitive fxn in MS more for speech and language patho and not PT 2. mini-mental state examination (MMSE) comprised of a list of q’s and activities that pts fulfill w points devoted to each q’s comprised of things like: -spelling ‘world’ backwards -completing sentence w no ifs, ands or buts -drawing shapes that intersect results varying levels of cognitive impairment psychosocial fxn 1. MS quality of life scale pts may present w behavioral or depressive symptoms pseudobulbar affect emotional ability- occurs when an individuals are unable to control emotions w symptoms like laughing or crying uncontrollably presentations can also include: euphoria, depression, anxiety questionnaire tests and measures: neuro and msk neuro tests CN integrity: CN 2,3,5,4,6,9,12 for visual, gag reflex (9), dysphasia (12) -vestibular cochlear nerve bc vestib symptoms vision -for accommodation to light, blurred/double vision, saccades and nystagmus modified ashworth scales: tonal abnormalities DTR: babinski reflex vestibular screen; vestibular deficits msk tests ROM strength: MMT or dynamometry -may not be telling bc they can score well on MMT but have weakness during fxnal activities test and measures: fatigue and temperature fatigue modified fatigue impact scale (MFIS): used to measure how fatigue impacts cognitive, psychological and motor abilities in pts fatigue scale for motor and cognitive fxns visual analog scale temperature: pts have sensitivity to heat uhthoffs phenomenon: 60-80% of pts- increases in core body temp can trigger symptoms to worsen -disorder thought to occur due to decreases in conduction velocity of neuronal circuitry related to temp important: facilities of exercise or aquatic environment have to be a good temp so pts dont get too hot -cold can exacerbate symptoms pseudoexacerbation symptoms are transient and last less than 24 hrs measurement w thermometer recommended in cases where pseudo-exacerbation and exacerbation occur test and measures: balance outcome measures clinical test for sensory interaction and balance (CTSIB or M-CTSIB) berg balance score (BBS) tinetti POMA BESTest require testing to provide idea of fxn -helps get idea of fall risk in some outcome measures no outcome measures specific to MS test and measures: mobility gait and mobility time up and go temporal gait parameters 10MWT 6 min walk dynamic gait index (DGI) time 25 foot walk: very useful specifically for pts w MS MS walking scale (MSWS-12): self reported walking index- how much the individuals diagnosis has impacted walking ability t & m: general health and aerobic capacity general health health status questionnaire (SF-36): for ALL pts-info on general health -clinicians have difficult time distinguishing moderate and severe forms of MS w this aerobic capacity BORG rating and perceived exertion (RPE) dyspnea scale ❌ t & m: MS-specific expanded disability status scale (EDSS): gold standard in MS research graded based on 7 systems: pyramidal, cerebellar, brainstem, sensory, bowel and bladder, visual and mental -use 1/2 point scale- 0=no impairement to 10=death -ambulation focused on as primary indicator of disability MS quality of life-54 (MS-QOL 54): combine w SF-35 for MS -items ranked separately w subscales related to physical fxn: physical and emotional role limitations, pain, emotional, well being, energy, health perceptions, social fxn, cognitive fxn, health distress, QoL and sexual fxn MS QOL inventory (MSQLI): similar to ^^ goals come up diagnosis, prognosis then establish goals goals are specifically impacting disease itself encompass ABCDEF (what is this again??)- bc of ‘f’ we can write goals related to fxn can write goals for improving WB and alignment meaningful goals for our pts gait and transferability- more impactful for those in early stages impacts of disease dont write goals specific to decreasing relapses, etc QoL outcome measure goals body fxns/structures or impairment specific ROM pain dont write goals for decreasing spasticity activity based/fxnal goals should be emphasized include objective data: asses quality of motor fxn Summary Same general examination procedures MS-specific nuances Goals specific to Activity 2.5 Rehabilitation Management - MS Intervention Types Preventative minimizing complications delaying exacerbation (good prognosis of baseline (later, severe stages) AD after exacerbation) - All to help w/ ADL neural plasticity recovery of mvmt task modification - secondary prevention - ex: nutritionist Maintenance Restorative Compensatory decrease disability education (pt/ caregiver) assistive services not covered by insurance - tertiary prevention Early Stage Interventions/Impairments Relapsing Remitting - will not show limitations can affect instrumental ADLs, like social aspects compensatory strategies Preventative/Restorative Intervention CV exercise stretching strengthening education on energy conservation - know when overdoing things environmental modification middle stages compensatory strategies Preventative/Restorative mod independence bc mobility using AD consultations psychological support energy conservation activities - modify their sport late stages compensatory (improves QOL of pt/caregiver) Prevention WC cushion improve posture education on bed mob positioning to avoid contractures/atrophy pressure relieving AFOs/PRAFOs - decreases integumentary compromise lifting techniques Intervention for sensation Compensatory Restorative proprioceptive loading through jts weights w. resistance to improve body awareness feet home environment - rugs moved sensory brushing/pharmacological - heightened sensitivity at soles of vision - contrast/lighting to prevent falls in environment - bright colored tape eyepatch - diplopia do skin checks bc can lead to compounding integumentary dysfxn interventions for pain pain directly from MS Lhermitte’s sign - PT can’t directly impact - pharmacological agents/acupuncture/meditation pain due to MS symptoms use soft cervical collar for systemic body pain - pts will feel shocking pain if flex neck - ex: tight muscles/malaligned jts - postural reeducation - use swiss ball - stretching - improve flex, jt pain - aquatic therapy - calming - TENS pain following medication pain independent of MS - important to time therapy - referral interventions for exercise * exercise does not increase symptoms for people with MS usually* schedule & dosage important - avoid overheating or exacerbation current relapsing episode - no exercise until remission vitals - RPE, HR, BP stop when reach peak HR, dizziness, lightheadedness, or significant increase/decrease BP maintain diary to see sleep, how costly ADLs are distributed practice best, 3-5x a week alt days w. 60-85% HR peak per session recumbent bike water aerobics treadmills VR/gaming Interventions for strength progressive resistance bands weight training circuit training WEIGHTED VEST: activity based training closed chain exercises interventions for bowel and bladder control voiding times for improved QOL pelvic floor exercises interventions for flexibility - hold in end range for 30-60 seconds daily splinting spasticity Coordination & Balance: see ataxia/postural instability WB activities for trunk stab - reduces unintended mvmts manual over pressure - good for spasticity - Estim for strengthening of pelvic floor HEP PNF for coordination/stab - chop/lift/isotonic/reversal of antagonist/rhythmic stab - dont stretch too much bc can make worse - weather can make worse: heat packs help - proprioceptive feedback: WB dont do patterns emphasizing mvmts and not stability, can make it worse Interventions for gait: decreased balance, coordination, strength fxnal gait in all directions gait overground w/ assistance if can more assistanceL bodyweight supported treadmill Orthotics/AD compensatory avoid bracing until really need to encourage muscles to do work - AFOs: help w/ DF in swing phase, decreased genu recurvatum during stance, decrease PF spasticity later use power chair for energy conservation & participation in society summary combination of interventions preventative restorative compensatory maintenance interventions specific to symptoms and activity goal 2.6 GMS Intro Clinical Presentation GBS: n damage bc own body defenses, autoimmune disorder targeting peripheral n (myelin sheath mainly) Physiology/Presentation impair n. signal propagation for M & S LMN - weakness, poor muscle activation DEMYELINATION M&S - loss of reflexes - numbness, poor sensory feedback AIDP (acute inflammatory demyelinating polyneuropathy) most common myelin membrane on both motor & sensory peripheral n. attacked CN, Autonomic n., pain signals also involved AMAN (acute motor axonal neuropathy) axonal membranes of motor n. only AMSAN both motor & sensory peripheral n axons miller fisher cross reactive antibodies attack CN or BS paralysis of eye muscles muscle incoordination areflexia facial weakness dysphasia dysarthria respiratory failure CIDP (chronic inflammatory demyelinating polyneuropathy) slower disease course than AIDP motor impairments sensory dysfxn paresthesia kinda like MS but with peripheral nervous system in comparison of single acute attack in AIDP in GBS cauda equina & conus medullaris more apparent where peripheral n concentrated Clinical Presentation pathogenesis: progression of demyelination to axon damage tingling of feet/hands problems getting up from chair days later areflexia weakness distal sensory loss weakness to LE then UE sensory motor pattern ascends body in hours/days involve trunk intercostals neck muscles CN respiration problems b&b fxn areflexia, hyporeflexia, muscle flaccidity not initially atrophy history of GI virus more presentations fatigue - physiological: change in CNS or PNS by looking at loss of muscle force - CNS: change in neurotransmitter activity, mental or physical activity (hard to focus), loss of voluntary control - PNS: change in neuromuscular jxn, muscle activation by activity/mvmt - experienced fatigue: person’s perception/psychosocial impacts GBS thought to have high level of central fatigue minimize neural activations bc thought to damage muscle due to demyelination muscle perceives higher exertion -> central fatigue Summary GBS non progressive neuromuscular disease unclear etiology LMN impaired strength, sensation, ANS, participation when working w/ GBS watch out for peripheral fatigue bc can cause them to regress 2.7 GBS Medical Management Differential Diagnosis look at pattern of impairments - focal, multifocal, generalized subjective history to narrow down or use diagnostic tests - blood lab, CSF exam main presentations weakness in more than one limb symptoms progress in days/4 weeks helps identify what type progression is distal to prox can move to respiratory/CN involvement RULE OUT dont fall under GBS on a child w/ AIDP vs AMAN Nerve Conduction Velocity Tests increased conduction of velocity/ latency as time goes on CMAP = compounded muscle action potential - decrease w/ progression in both AIDP: less SNAP (sensory nerve axonal potential) SNAP not found in AMAN AIDP: 2 mV vs AMAN: 1 mV CMAP in AMAN way lower = more motor axon involvement Management of GBS PLEX plasma exchange removes cross reactive antibodies from BS to limit attack process - take blood, separate platelets, red cells, lymphocytes, granulocytes by centrifuge/filter - trash bad ones - red cells combined w/ fake plasma (colloid replacement) - put back takes hours 2-5 sessions over days decreases time on ventilator, improve disability/motor scores complications: drop in BP, arrhythmias, relapse started before 7 days better outcome Intravenous Immunoglobulin (IVIg) less invasive, less side effects, cheaper targets immune cells attacking myelin adding healthy antibodies improves recovery, clinical outcomes but not mortality best within 2 weeks symptom onset DONT DO BEFORE PLEX bc then just remove good stuff not great for mild cases read general medical management Disease Course viral infection (GI 2 weeks before) cross reactive antibodies build up attacking myelin & PNS symptoms plateau w/in 4 weeks of onset months recover post plateau - better fxnal status rehab/compensatory schwann cells remyelinate which helps motor fxn partial recovery (2-4wks post plateau) - weakness, fatigue Prognosis & Outcomes fatality - 3-10% bc respiratory problems - 20-30% need mechanical vent disability - 75% - fatigue, paresthesias, pain/weakness ambulatory - 15% - cant walk indep Erasmus GBS Outcome SCore potential to walk again age, diarrhea, GBS disability score higher = higher chance of not walking indep at 6 months Poor prognostic indicators GI illness severeity of muscle weakness respiratory/mechanical vent CN involvement older age severity of nadir, prolonged time to reach nadir (higher chance of relapse) Summary GBS diagnosed based on progressive LE to UE weakness & areflexia high potential for survival & recovery of fxn & participation w/ residual impairments nadir = point of greatest disease severity 2.8 GBS REHAB PLAN OF CARE Evaluation: Relevant tests. MMT, CN Testing, Sensory, Functional Mobility Depending on function and goals BERG, Functional gait , Activities Specific balance, ten meter walk test, Six Meter Minute walk test, five times Sit to Stand Depending on Severity Presence and extent of the common impairments and functional limitations People recovering from GBS have a high chance of recovering strength and function, so outcome measures should allow for recovery and not have too much a ceiling effect Due to demyelination, do not test patients to fatigue or overwork weakness Quick notes: Someone with a grade 4, over the age of 60, has a 70% chance of walking in 6 months Someone who is over 60 and require ventilation has 50% chance of walking in 6 months Assessment of fatigue: Need to assess physiological and experienced fatigue Subjective and Objective outcome measures Objective: Check for muscle belly tenderness Gentle pressure with the palmar surface of hand and fingers to a relaxed muscle belly of a major muscle group. Patient rates 0-10 pain intensity For Physiological Fatigue: measuring force generated by muscle from electrical stimulation. Voluntary and involuntary contraction. Record decline of force which represents the fatigue Subjective: Patient reports perceived fatigue and impact on daily function and participation. Fatigue severity scale and modified impact scale are the two used measures. Rate level of agreement with various read statements ACUTE REHAB Individual is likely in hospital and need close monitoring and may require assistance. Assess vital capacity or other pulmonary measures Physical Therapy intervention is dependent on the severity. When able to increase endurance, patients should assume upright positions Educate and teach respiratory exercises Ensure range of motion. Range of motion commonly affected areas. Hip Flexors, ankle plantarflexors, knee flexors, shoulder and elbows Order of subacute rehabilitation Strength Training Often patients will be at a 1-2 grading scale in MMTs Training should be done with gravity eliminated positions with full ROM in mind Strength training can eventually be moved towards multi joint and multiplanar movements against gravity. Focus on submax strength for both fast twitch muscle fibers and eccentric muscle contractions. Durable Medical Equipment Ankle foot Orthosis. AFO For foot drop, and propulsion during gait 2/3 participants continue to use AFO at discharge 1/3 continue use 1 year after discharge 2.9 ALS Amyotropic Lateral Sclerosis Objectives: Presentation , pathophysiology ALS: Selective dysfunction and death of neurons in the motor pathway. Demyelination Angliosis of the corticospinal and corticobulbar tracts. UMN LMN - This can cause spastic and flaccid paralysis. Hypo to Hyperreflexia CAUSING: spasticity, hyperreflexia, general weakness, muscle atrophy, and paralysis Unknown reasons: Bladder and eye movements SPARED Sensory function is also spared. Patients can feel, but can’t move on their own CAUSES: Genetic mutation, Excitotoxicity 4 types: Classical, Progressive muscular atrophy, Progressive bulbar palsy, Primary Lateral Sclerosis Classical: both UMN LMN and involving Cranial Nerves Progressive muscular atrophy: LMN only Affects Limbs and trunk, little to no impact on cranial nerve nuclei Progressive Bulbar palsy: Musculature innervated by brainstem are impacted. Not limb and trunk Combined UMN LMN Primary Lateral Sclerosis: Motor tracts are impacted, UMN impairment Strong Link between ALS and Frontotemporal Dementia. 50% with ALS may also present with behavioral changes- degenerations of frontal and temporal lobes Leads to 10-15% having both of these Impacts behavior, language, and communication deficits in executive function, judgement, and decreased abilities for ADLs, and personality Clinical Signs muscle weakness general disuse atrophy, with sparing of the sensory system musculoskeletal pain somatosensory pain because of pressure while in one position drooping of head dragging feet, foot drop asymmetric tongue protrusion due to CN deficit impaired hand dexterity with intrinsic muscle atrophy muscle cramps with muscle fasciculations This indicates anterior horn cell damage Muscle atrophy Pseudobulbar signs like dysphasia, emotional liability, or dysarthria Central and Peripheral Fatigue Central is due to changes in neurotransmitter activity within the CNS and can be brought on by mental or physical activities Peripheral is due to changes at the neuromuscular junction or with muscle activation and is brought on by physical activity or movement Both can result in impaired muscle force degeneration ALS- central fatigue is correlated with depression, poor sleep qual, poor pulmonary fxn, and muscle weakness peripheral fatigue in ALS, decline in muscle force, larger motor units are less efficient and fatigue quicker 2.10 ALS Management Obj- diagnostic criteria and presentation medical management strategies progression and prognostic factors El Escorial Criteria- needs both UMN LMN degenerations along with progressive spread of symptoms Multipe levels of spinal cord, CNerves, or in motor cortex Diagnosis involves finding out absences of other findings that indicate other pathology strategy in ruling out things that mimic ALS like MS, myopathies, spinal muscular atrophy, polio, and even endocrine abnormalities Diagnosis of ALS less likely if there are changes in sensory ocular motion Definitive diagnosis may take 14 months due to bowel and bladder control Unusual presentation misinterpreted clinical findings low index of suspicion Electromyography Done in multiple regions to determin pattern of motor loss and spread upper and lower limbs tongue trunk Progression- often fast and relentless LMN impairment signs can begin, then be masked by UMN impairments LMN and UMN impairments can be inconsistent and there can be many variable patterns of spread making it difficult to diagnose and individualize treatment Team based approach is needed to address -nutrition -communication -swallowing -participation -ADLS -respiratory care -cognition -psychosocial needs -movement system limitations -end of life care -palliative care This can help in -longer survival times -less hospital visits -increase use in durable medical equipment -increase nutrition and pharmaceutic goals -increased quality of life Some things to consider DISEASE MODIFYING THERAPIES Riluzole- improves survival by 3 months in median duration Edaravone- slow the decline of early stage disability NUTRITION Less intake and increase energy expenditure by the body would require the patient to have a high calorie diet Feeding tube insertion is recommended if BMI is less than 18.5. Or if there is dysphasia, or decrease in functional vital capacity approaching or below 50% RESPIRATORY Airway clearing techniques Noninvasive ventilation for 65% vital capacity function Mechanical ventilation Less than 10% choose this because EXPENSIVE also decrease quality of life MEDICAL MANAGEMENT common symptoms pain, psudobulbar affect, mental health concerns, impaired speech and swallowing, sialorrhea, muscle spasms, impaired mobility AVRG survival rate 5-10 years Women faster decline Bulbar region onset and respiratory onset individuals have shorter life span Bulbar- 1-2years. 12-18 months if they use non-invasive ventilization 2.11 Rehab Management for ALS Objectives_ Design a rehab plan for ALS WHAT TO LOOK FOR ? PT assessment techniques Sesnsory testing Cranial testing Mobility Testing OUTCOME Measures Berg GOALS are NOT to improve functional gait assessment function activity specific balance Purpose is to maintain or confidence scale minimize decline 10 meter walk test 6 meter walk test 5 times sit to stand Severity? presence and extent of common impairments and functional limitations Check also for muscle fasciculations which would indicate that the disease has affected that motor level. Also Check for level of mental and physical fatigue. Scale measure 12 items on a 4 point scale Multidisciplinary care approach is inclusive to the roles of all these other professions COGNITIVE Behavioral screen assesses attention, concentration, track and monitor data, initiate a retrieve words Quationnaire for caregiver to complete, focused on observations of individuals mood and behavior ALS DEPRESSION inventory self report for mental health 12 statements with 4 possible answers PT Duty- respiratory mvmt chest expansion fatigue management mobility ' RESPIRATORY THERAPY Measure FVC Forced Vital Capacity ALS patients FVC>75% longer survival 65% is consideration point for non-invasive ventilation Less than 50% is consideration pt for tube feeding minimize risk for aspiration Implement incentive spirometers or chest physical therapy Patients can then be taught glossopharyngeal breathing, frog breathing, air stacking taught by speech therapists Learn to control muscles in mouth and pharynx including glottis to repeatedly gulp small amounts of air through larynx REHAB MANAGEMENT patients and caregivers need to be informed on maintain proper ROM decrease risk of pressure ulcers treat functional limitations gait training balance Is Exercise Worth It? Focus on the unaffected muscles Leads to neuro protective affects Slow down and delay disease progression Exercise = dendritic restructuring improve axonal transport ^ protein synthesis ^ signals at neuromusc junc alter electrophysiological properties and gene expression -Studies show lack of muscle activation leads to 3-5% loss of muscle strength daily -20% max muscle contraction can maintain current strength EQUIPMENT Equipment must be adaptable to the changes of ALS understand the uses of wheelchairs, power-chairs, lifts, adapted cars , hospital beds and bath chairs. 2.12 Interventions for Weakness - Strength Training Pathophysiology of Weakness - Results from - Neural Factors - Ability of CNS to recruit & activate motor units - Structural factors - Ability of the muscle fibers to create contraction - Heineman Size Principle - Small, slow motor units (Type S) - Fast, Fatiguable motor units (FF) - Neurologic Injury - Ability of CNS to drive motor neuron pool & regulate firing rate is disrupted - Strength training stimulates neural system by allowing adaptation, efficiency of firing, & improved motor unit recruitment - Strength increases within 4-6 weeks is due to this - Muscle hypertrophy does not occur until 6-12 weeks after initiation - Muscle weakness from disuse results in atrophy of Type 2 fibers (fast twitch) - Decreases ability to perform quick movement tasks Post Stroke Weakness - Shown to be directly related to sight & severity of injury - Distribution of weakness usually on paretic side, contralateral to brain lesion\ - Still weakness on ipsilateral side - Usually found in distal extremities - Primarily caused by decreased number of motor units, increased motor unit innervation ratio, impaired firing frequency, muscle fiber changes - Study done by Sawtelle - Significant different b/w paretic & non-paretic limbs - Strength does not equate to muscle activation directly Weakness Post Acquired Brain Injury - Can follow various patterns depending on injury itself - Focal Injury (Bleeds in 1 hemisphere) - Display weakness characteristics similar to stroke, contralateral - If brainstem is involved, or multiple cortical lesions, or diffuse axonal injury - Presents bilaterally - Direct mechanisms include central neural activation deficits - Indirect include disuse atrophy Evidence of Strength Training - MS, Parkinsons, ALS, Stroke, Brain Injury, Cerebral Palsy - Exercise programs of 6-8 weeks have been shown to improve neural recruitment & efficiency - Bone mineral density has been shown to occur w/ weighted strengthening activities - Implementing can occur in all stages of disease, w/ proper monitoring - Efficacy of strength training for improving functional outcomes is less robust - Unless within task itself General Approaches to Strength Training - Facilitation - Can assist in guiding patient to achieve the desired response, building strength - Multiple key inputs in order to achieve spatial summation of neurons - Temporal summation of neuron by utilizing quick stretch throughout range during PNF - Neuromuscular Electrical Stimulation (NMES) - Provides electrically induced contractions - Potential for strength training, or reeducation - EXAMPLES - Central based severe weakness, stimulus directly to muscle - Voluntary activation weakness, muscle reeducation - Electromyographic Biofeedback - Provides feedback in sync w/ activities themselves - Small handheld device sued by patients - Gives audible beep if not within desired contractile range - Does not impart stimulus at all - Proprioceptive Neuromuscular Facilitation (PNF) - Requires resistance - Open/Closed Chain Activities - Typically used in healthy population, can be used in neural (Depends) - Progressive Resistive Exercise - Typically used in healthy population, can be used in neural (Depends) - Isokinetic Exercise - Helps build strength within a full ROM - Task-Specific Training - Allows patient to complete activity based tasks & strengthen Principles of Strength Training - 4 Principles - Overload Principle - States that a muscle must be progressively challenged for strengthening to occur - 60-70% of 1 rep max or maximal load lifted only once, is needed to increase muscle strength - Takes 80-100% 1 rep max & 6-12 reps to build strength in healthy individuals - Specificity of Training - States that training effects are directly related to type of training demands on muscle - If muscle working concentrically for a task - Encourage concentric strengthening to improve task - Cross Training - Promotes constriction of various types of contractile muscle demands while performing different tasks - Strengthen concentrically, isometrically, essentrically - Reversibility - Concept that if strength training does not continue, effects may be reversed Strength Training Prescription - Exercise till fatigued for neuro patents - Stop when they begin to compensate during activity or exercise - Pay attention to vitals as well - Modify based on patient presentation & severity of symptoms Guidelines for Strength Training 2.13 Interventions for Limited ROM Contractures - 2 Types that limit PROM - Muscle Contracture - Physiologic shortening of muscle tissue - Joint Contracture - Capsular tightness to joint itself Pathophysiology of Muscle Shortening - Passive Resistance occurs from 3 aspects - Inertia of limb - ThickSOmething?? - Increased stiffness when not moving, decreased stiffness when moved - Stretch Reflex - Associated w/ spasticity in neuropathologic people - Occurs due to lower threshold of alpha motor neuron activation - Type 1 afferent from muscle spindles have lower threshold to high velocity, low amplitude stretches - Results in motor neuron being closer to depolarization - Viscoelastic Properties - Contractile or Non-Contractile elements - Contractile - Refer to muscle, tendons, attachments - Non-Contractile - Refer to ligaments, joint capsule, bones - Tendons & Ligaments comprised of collagen w/ prolonged immobility - Structures become tight, limiting PROM - Prolonged or cyclic stretching is beneficial to improve PROM Immobilization - Possibly self immobilized due to - Impaired Functional Mobility - Impaired Strength - Impaired Motor Control - Slings - Not good for someone who suffered stroke - Muscles & joint stiffness, muscle shortening & contractures would occur Heterotopic Ossification - Bone formation within soft tissue outside of normal bone/joint structures \ - Causes decrease in PROM & pain - Happens in about 50% of patients w/ spinal cord injury Interventions - Slow Stretch - Sustained (30-90 sec hold) - Avoid high velocity stretches - Splinting - Provides low load stretch for prolonged period of time - Usually wear it at night for as long as they can - Dynamic Splints - Allow for modification of the stretch - Static Splints - Serial Casting - Removes cast, positions another cast w/ greater ROM on patient - Skin irritation & break down our adverse effects that are common - Joint Mobilizations - Rely on the arthrokinematics of the joint - Heat - Used to provide relaxing sensation of the muscle & overlying fascia - Helps vasodilation & blood flow to the area - Surgical Release - If they’ve already had a contracture - Tendon lengthening is often performed 2.14 Interventions for Involuntary Contractions & Movement Dyskinesias - Abnormal movement disorders arising from basal ganglia - Tremor - Characterized by reciprocally innervated muscles - Resting Tremor usually seen in PD, seen at rest - Action Tremor, seen w/ any active voluntary movement - Intention Tremor, seen w/ attempting to localize an extremity for goal directed behavior - Essential Tremor, occurs during rest & w/ voluntary movement - Dystonia - Patient moves into posture & stays there brief or extended period of time Extra Infö Pathophysiology of Dyskinesia - Role of basal ganglia is to produce smooth coordinated movements - Basal Ganglia Pathways - Direct Pathway (Review from Neuro) - Indirect Pathway (Review from Neuro) Related Diagnoses - Huntington’s Disease - Genetic abnormality caused by expansion of a gene - Age of onset around 30-40 yrs old - Lifespan after is 15-20 yrs - Indirect pathway is affected - Chorea type movements, gait impaired - Parkinson’s Disease - Damage to direct pathway, excessive slowing of movement - Bradykinesia, Cogwheel rigidity, postural instability, poor balance/coordination, depression, cognitive deficits - Resting Tremor in about 80-90% - Meds shown to reduce tremor in about 50% - Cerebral Palsy - Congenital disorder, develops in a developing fetus - Movement disorder a non progressive in nature, accompanied by other system disturbances - Also suffer from epilepsy - Stroke - Possibly present w/ hemichorea, hemiballismus, dystonia, Parkinsonism - Depends on location of infarct Outcome Measures - Proper clinical decision making is needed to choose which outcome measure to use for specific abnormal movement patterns Interventions - Patient/Caregiver Education & Respite - Medication - Botox used for focal dystonia, tremors, ticks - Botox temporarily blocks acetylcholine receptors - Repeat injections after 3-4 months - Deep Brain Stimulators (DBS) - Used if meds/botox does not work - Implanted into basal ganglia, pulse generator delivers stimulation - Whole Body Vibration - Patient steps on force vibration plate, helps mitigate tremor & spasticity - Provide input to muscle spindles to biased information about muscle length, reducing movement error - Orthoses - Resistance Training/Weights - Transcranial alternating or Direct Current - Positioned on body, shown to reduce tremors by up to 50% in people w/ PD - Sensorimotor training for dystonia - Practicing novel skill or utilizing mirror during mirror therapy - TENS/Biofeedback - Taping/Immobilization - Helps decrease unwanted movements - Compensatory Techniques 2.15 Interventions for Ataxia & Incoordination Normal Motor Circuitry Definitions - Coordination - Cooperation of 2 opposing muscle groups, agonist & Antagonist - Related to multi-joint muscles & inter-limb muscle groups for functional activity - Postural control during volitional movement - Ataxia - Occurs when lack of or absence of sensory input from periphery to cerebellum or higher centers - Inability to perform coordinated skilled volitional movements Circuitry - Peripheral system carries out motor plan developed by CNS - Provides feedback for movement controlled by cerebrum & cerebellum - Cerebellum provides adaptive feed forward control loop - Relearning of coordinated movement guided by cerebrum instead of cerebellum - Tasks often fractured when relearning w/ inability to intrinsically adapt to demands of task - Cerebellar Involvement Patients - Are unable to elicit anticipatory feed forward control of movements - Can not adapt to small changes of conditions - Requires much more practice & reps Clinical Presentation - Depends on location of lesion occurs - Present w/ dysmetria, decomposition of movement, disdyadokokinesia, asthenia, intention tremor, hypermetria, rebound phenomenon - Rebound Phenomenon - Test where patient is asked to move arm against resistance, resistance moved & arm should continue & recoil back to starting position - Positive sign if does not go back Functional Anatomy Presentation - Cerebellum = “Little Brain” - Anterior Lobe - Posterior Lobe - Flocculonodular Lobe - Functional Areas - Vestibulocerebellum - Lesions result in decreased balance, particularly w/ upright axial position - Decreased coordination of eye movements w/ movement of head - Spinocerebellum - Lesions result in deficits w/ postural coordination, hypotonia, dysmetria - Unable to correct timing of finger during movement of limb - Cerebrocerebellum - Lesion results in decreased timing of movements & decreased fluidity - Decomposition of movement General Management - Physical Therapy - Treatment based on signs & symptoms - Compensatory Interventions - Should try to be removed as patient progresses - Goal of Postural control - WEightbearing - Small Movements First - Progress by decreasing visual input - Vision should be used in the beginning - Pharmacological Intervention - Aminopyridines - Acetazolamine - Idebenone - Specific for individuals w/ Friedrichs Ataxia - Disease predominated by lack of mitochondrial protein, results in increased iron in body - Zinc - Shown to have promise in improving functional mobilities Equipment during Exercise - Weighted Vests - Shown to improve motor coordination, balance, & functional ability - Weighted utensils/devices - Helps improve dexterity - “Exergames” - Video games that assist individuals in physiotherapy type movements - Motivates them to perform certain activities at home - Cycle Ergometry - Assists in modulation of agonist/antagonist muscles Interventions - Balance & Gait Focus - Quadruped helpful due to weight bearing component on all 4 limbs - Can increase rigor of task, asymmetrical arm lifting - Progress to half-kneeling to increase DoF - Proprioceptive Neuromuscular Facilitation (PNF) - Rhythmic initiation to begin, then stabilization to improve postural control - Frenkel Coordination Exercises - Use vision as principal source of feedback at first, to adapt to movements to environment - Normal progression of exercises, similar to 1st intervention - Cawthorne-Cooksey Exercises - May be provided to patients w/ vestibular deficits - Purpose is to allow patient to compensate for abnormal signals - Distal Support during Functional Tasks - Decreases DoF the patient has to control class notes 1/16/2024 - week 2 Wahls Protocol for MS- Diet and Nutrition Daily intake of veggies in Wahls protocol- 9 cups What foods to avoid in Wahls for MS- Red Meat Vitamin for counteracting disability in MS- B12 Leaky gut/intestinal permeability in MS management- Slows down the progression of MS