Myopathy: A Closer Look .

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Myopathy. Definitionneuromuscular issue in which the essential side effect is muscle shortcoming because of brokenness of muscle fiber.** Definition by the National Institute of Neurological Disorders and Stroke. Let\' Start With Basics!. . Muscle Anatomy: terrible and minute. MUSCLE FIBER. Capacity of Muscle.
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Myopathy: A Closer Look Sofiya Prilik, MD Physical Medicine and Rehabilitation

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Myopathy Definition neuromuscular disarranges in which the essential side effect is muscle shortcoming because of brokenness of muscle fiber.* * Definition by the National Institute of Neurological Disorders and Stroke

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Let\' Start With Basics!

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Muscle Anatomy: gross and tiny

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Function of Muscle

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Motor Unit An engine unit is comprised of an engine neuron and all the muscle cells it empowers. Engine units change in size. Little engine units are utilized for exact, little developments; substantial engine units are utilized for gross developments. The quantity of cells inside an engine unit decides the level of development when the engine unit is animated. Muscle tone is kept up by nonconcurrent incitement of irregular engine units.

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Normal Muscle

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Abnormal Muscle

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Myopathy: indications Muscle Weakness Proximal Muscles>distal muscles Fatigue Difficulty ascending from a seat, floor, tub Difficulty with stairs Difficulty with overhead assignments Respiratory muscles Bulbar shortcoming discourse, gulping, oculomotor, facial

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Myopathy: side effects Pain Mostly with incendiary and metabolic High serum CK level Aching, dull, cramping Patients will state: "sore", "hurt", "fit" No deadness or paresthesias

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Physical Exam: Full exam is imperative ! Perception – search for muscle decay, deformations Strength testing – manual muscle test ROM testing Functional testing Stand up from a seat Walk Step up on a low stool Don\'t overlook REFLEXES and SENSATION

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Inflammatory Myopathies Polymyositis Dermatomyositis Inclusion body myositis Viral Muscular dystrophies X-connected Limb-girdle(ar/d) Congenital Fasioscapulohumeral (advertisement) Scapuloperoneal (promotion) Distal (Welander) (advertisement/r) Myotonic Syndromes Myotonic dystrophy (promotion) Inherited Schwarz-Jampel Drug-prompted Congenital myopathies Central center illness Nemaline myopathy Myotubular Fiber-sort lopsidedness Metabolic myopathies Glycogenoses Mitochondrial Periodic loss of motion Endocrine myopathies Thyroid Parathyroid Adrenal/steroid Pituitary Drug-initiated/dangerous Myopathic Disorders

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Myopathy: sorts Muscular dystrophies Inherited Abnormal muscle proteins Progressive course and early onset Congenital Slowly dynamic or non-dynamic Distinct finding on muscle biopsy

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Metabolic Defect in intracellular vitality generation Inflammatory Acquired Caused by resistant or irresistible process Almost dependably are related with lifted Creatinine Kinase level in serum. Atrophic Drug-initiated (Colchicine, AZT, ETOH, Statins (1/10,000 every year) Endocrine (steroid) CK is regularly ordinary

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Myotonic Congenital or grown-up Cardiopulmonary trade off

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Epidemiology Worldwide rate of all inheritable myopathies is around 14% Overall frequency of strong dystrophy is around 63 for each 1 million. Overall occurrence of incendiary myopathies is around 5–10 for every 100,000 individuals. More normal in ladies Corticosteroid myopathy is the most well-known endocrine myopathy and endocrine issue are more basic in ladies Overall occurrence of metabolic myopathies is obscure.

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Diagnosis Case: 59 year-old male with history of smoking, who was determined to have extreme COPD/emphysema 2.5 years prior. From that point forward, he had a few hospitalizations because of exacerbating SOB and beneficial hack. He was treated with high dosages of IV corticosteroids took after by moderate oral steroid decreases. After the last hospitalzation 4 months prior, he has been kept up on a Prednisone 5 mg day by day. Regularly, the patient is free with exchanges, ambulation and ADL\'s. His strolling resilience is around 1-2 squares, constrained by SOB. 2 weeks back, patient introduced to his PMD c/o dynamic useful decrease in strolling resistance, and particular trouble with exchanges and stairs. Exam uncovered a thin male, with O2 immersion of 93% on RA. No obvious respiratory pain was noted. No cushinoid components were seen. Germane positives included obviously clear decay in the proximal muscles gatherings of both UE and LE. Quality testing was inside ordinary breaking points. Quiet experienced issues standing up from a sitting position. He was not able perform squats. Labs – WBC 11.8, Glu 120, generally ordinary. CK - ordinary NCS/EMG - typical

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DIAGNOSIS Steroid instigated myopathy.

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STEROID INDUCED MYOPATHY Insidious sickness handle shortcoming of proximal muscles of the upper and lower appendages and neck flexors. Initially depicted by Cushing in 1932 An overabundance of either endogenous or exogenous corticosteroids is accepted to bring about the condition. Perpetual or intense (less normal) Catabolic impact on muscle – gluconeogenesis from aminoacids

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STEROID INDUCED MYOPATHY Fluorinated steroids are involved Dexamethasone Triamcinolone Also observed with non-fluorinated ones Prednisone Inhaled steroids

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Pathophysiology diminished protein amalgamation expanded protein corruption adjustments in starch digestion system mitochondrial changes electrolyte unsettling influences diminished sarcolemmal volatility

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Epidimiology For a given dosage of steroid, ladies give off an impression of being twice as likely as men to create muscle shortcoming Worldwide frequency or prevalance is obscure

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Diagnostic reviews Labs Routine Labs Special labs Creatinine Kinase – typical Urine Creatinine – expanded No myoglobinuria or rhabdomyalysis Muscle biopsy sort IIB strands are generally influenced No irritation, putrefaction or recovery

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DIAGNOSTIC STUDIES Electrodiagnostic concentrates Normal nerve conduction examines (NCS) Electromyography can be ordinary (EMG tests sort I filaments, while SM for the most part influences IIB) DON\'T FORGET: A constantly or basically sick patient, can have other co-sullen conditions, that may affect NCS or EMG

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TREATMENT Steroid treatment alteration Pain control Prevention of contractures Avoid practice to the point of weariness Aerobic practice ROM Moderate resistance practice Assistive gadgets Other: ventilation, percutaneous enteric sustain

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MYOPATHY RELATED TO CRITICAL ILLNESS Common in patients even after a concise period in the emergency unit. Evaluated to be around 25%. Picked up acknowledgment in the most recent decade Often misdiagnosed or missed Can happen in conjunction with polyneuropathy Associated with delayed ventilation and troublesome weaning

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Differential Diagnosis Motor neuron infection ALS Late onset spinal strong decay Post-polio disorder Neuromuscular intersection issue Myasthenia Gravis Lambert-Eaton myasthenic disorder Motor neuropathy Myelopathy/spinal stenosis Parkinson\'s

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What is PPS? Started January 1, 2002 Inpatient Rehab Facility Prospective Payment System (IRF-PPS) is the repayment program for Medicare Part-A patients in light of their particular impedance + level of working upon affirmation 21 general Rehab Impairment Categories (RIC), 85 particular Impairment Group Codes (IGC), Admission FIM scores and some of the time Age, decide the Case Mix Group (CMG) The CMG decides the one-time settled repayment sum per quiet per remain at an IRF and produces an Average Length of Stay (ALOS) in view of national standards ~ 70% Rusk populace are MCR Part-A beneficiaries

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What is the 60% Rule? To qualify as an IRF, a supplier must convey escalated restoration administrations to a populace of inpatients, presently 60% of whom, fall into at least one of 13 particular weakness classes, (the "CMS 13"), from the aggregate 21 Rehab Impairment Categories (RICs)

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PPS : 21 IGCs Specific ICD-9-CM codes for comorbs that give extra repayment 3 Tiers (B,C,D) – High to low levels of extra repayment 60% Rule : 13 Qualifying IGCs Specific ICD-9-CM codes for etiologies and comorbs that qualify cases in the decision No effect on repayment Compliance keeps up office\'s status as an IRF PPS versus CMS 60% Rule

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Active Comorbidities "Conditions bringing about useful shortages that will be tended to or checked amid the inpatient recovery stay": Medical conditions requiring counsels, testing as well as pharmaceuticals Conditions influencing ADLs Conditions or inconveniences that influence recovery treatment course or plan of care

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How Can Health Care Providers Contribute? Acquaint yourselves with ordinarily observed comorbid conditions, including, yet not constrained to, qualifiers in the 60% lead and PPS reimbursable comorbidities Identify patients who have shortfalls characteristic of myopathy and talk about deficiencies with the recovery doctors Clearly report the ebb and flow shortages, dole out precise engine and intellectual FIM scores to speak to the patient\'s actual practical levels of help and weight of care while in recovery Clearly archive any remaining shortages from past ailments/occasions that are as yet being tended to in treatment sessions, including "settling" conditions

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Specificity in Documentation – Importance of Communication amongst Therapists and MDs Rehab-assigned Medical Records Coders can just dole out ICD-9-CM Codes for conditions incorporated into doctor documentation If treatments or nursing alone give documentation - conditions won\'t be coded Accurate coding adds to both qualifying cases in the 60% Rule and extra repayment for PPS

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60% run Qualifying ICD-9-CM Codes and Verbiage for Myopathy 359.0 - Congenital Hereditary Muscular Dystrophy 359.1 - Hereditary Progressive Muscular Dystrophy 359.2 - Myotonic Disorders 359.3 - Familial Periodic Paralysis 359.4 - Toxic Myopathy 359.5 - Myopathy in Endocrine Diseases Classified Elsewhere 359.6 -

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