Week 2 Neuro Summary PDF

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 C...

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