Date post: | 20-Mar-2017 |
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Health & Medicine |
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MYOPATHIES
Dr Rashmi R PG scholar
Myopathy
• Disorders with structural changes or functional impairment in muscle• Unrelated to disorder of innervation or
neuromuscular junction• Worldwide incidence :
Muscular dystrophy- 63 per 1 millionInflammatory - 5-10 in 100,000
Clinical features
Muscle weakness• Hip girdle - Most common difficulty in getting up from squatting position• Hip abductor- Waddling gait• Upper girdle- difficulty in hanging clothes• Trunk - difficulty in turning and getting from
recumbent position• Neck muscle - neck pain , stiffness• Facial – Ptosis, Extra occular movements
Hypertrophy- more evident in calves, gluteis and deltoids• In Duchenne, Becker MD• Mild - Spinal muscular atrophy
Radiculopathies( neurogenic origin)
Myotonia- delayed relaxation after sustained contraction
Differential disorders
• LMN lesion- muscle weakness( chief symptom)• Differentiate between pure motor and
sensorimotor• Pure motor - muscles
Sensorimotor- nerves/roots
• Pure motor - proximal, synmetrical• Neuropathies - distal , synmetrical• Radiculopathies-pain, asynmetric loss of
semsorimotor function• Ant.horn cell - asynmetrical, proximal & distal
fasciculations, UMN signs• Mysthaenia gravis- External occular muscles,
swallowing No other LMN sign except fatigability and weakness
Investigations
• Serum CK(creatinine kinase)- elevated
• EMG
• CT/MRI
• Muscle biopsy
EMG in myopathies
• In acute myopathies- EMG must be conducted about 3 weeks from the onset of symptoms to ensure good senstivity• EMG done at two situations- at rest and voluntary
activity• Resting values also called spontaneous activity are of
3 types1. Fibrillations and positive waves2. High frequency discharges3. Myotonic discharges
MYOPATHY
ACQUIREDINHERITED
Inherited
• Dystrophies• Myotonic• Congenital• Distal• Metabolic• Mitochondrial
Muscular dystrophy• Genetically inherited primary muscle diseases• Progressive and unremitting muscular weakness
DUCHENNE’S OCULOPHARYNGEAL
LIMB GIRDLE
FACIOSCAPULOHUMERAL
BECKER’S
Duchenne MD
• X-linked recessive(Xp 21 myopathy)• Females are carriers, males symptomatic• Affects boys at age of 3-4 yrs• Calf pseudohypertrophy- early stage• Progressive weakness of girdle muscles• Death by age 20- respiratory failure• Cardiomegaly, reduced intelligence
Becker MD
• Similar to duchenne• Genetic defect same site• Slow progressive weakness• Cardiomegaly
Facioscapulohumeral MD
• Autosomal dominant • Males> females• 2nd-4th decade• Slowly progressive weakness of facial, scapular
and humeral muscles• Winging of scapula• Lack of facial mobility, incomplete eye closure,
transverse smile
Limb girdle MD
• Autosomal recessive• Affects both genders equally• Early childhood to adult• Slowly progressive• Starts in pelvic region and progresses
to arms and legs• Cardiomyopathy
Oculopharyngeal MD
• Autosomal dominant• 5th- 6th decade• Slow progressive weakness of extraoccular
eyelid, face and pharyngeal muscles• Ptosis• Difficulty in swallowing• Proximal limb involvement -later
Myotonic dystrophy
• Autosomal dominant, multisystem disease• Chronic, slowly progressive• Hatchet faced• Sternocleidomastoid, temporalis, masseter• Cardiomyopathy, CCF• EMG along with myopathic changes• Muscle biopsy- ring fibres with dystrophic
changes
Congenital myopathies
• At birth as floppy child• Weakness either static or improves as age
advances• skin and bones appearance• Cardiac, recurrent respiratory infection• Nemaline, Rod body, Zebra body
Distal myopathiesWelanderHand involvementSlowly progressive4th-6th decadeMiyoshiPosterior tibial involvementWasting of calf muscles
Acquired
• Infective• Inflammatory• Endocrine & metabolic• Drug induced & toxic
Infective
Viruses • Influenza, coxsackie produce acute myositis• Swelling and pain in muscles with weakness• Complications- myoglobinuria & renal failure• Acute , self limiting
Bacteria• Staphylococci - multiple abscess• Rx- Antibiotics Drainage of pus
Parasitic• Cysticercus cellulosae• Trichenella spiralis• Pain and hypertrophy of muscles• Rx- Albendazole 15-25 mg for 1-3 weeks
Inflammatory
• Dermatomyositis• Polymyositis• Inclusion body myositis• Cancer assosciated myositis
Dermatomyositis
• Proximal muscle weakness• Cutaneous rashes over knuckles, eyelids & anterior
part of chest• Gottron’s papules- pathognomonic• Skin calcinosis- late stage
Polymyositis
• Proximal muscle inflammation, synmetrical• Immune mediated disease of skeletal muscle• May occur in assosciation with SLE• Dysphagia may be present• Rx- steroids + immunosupressants+ physiotherapy
Inclusion body myositis
• Older males affected• Slow progression• Asynmetrical• Distal and proximal• Wrist drop and finger drop
Endocrine and metabolic
• Thyroid and parathyroid disorders• Osteoprosis• Osteomalacia• Renal insufficiency
Drug induced and toxic
• Corticosteroids• Statins• Anti- epileptic agents• Toxins
Management of myopathies
4 S APPROACH• Strength therapies- Physiotherapy and exercise• Supportive care- Respiratory problems in limb
girdle MD• Symptomatic care- cardiac involvement in
myotonic dystrophy• pSychological support
AYURVEDIC VIEW
Mamsa dhatu• Dasavidha pranayatani• Matruja bhava• Parthivam in nature• Gunas – sthula, sthira, guru, khara, kathina,
slakshna• First solid tissue formed from rasa- rakta dhatus• Functions- sarira pushti
medo pushti sarira lepa ojokara
Role of vata in myopathy
• Dynamic factor for gati• Akunchana prasarana of mamsa peshis• Vyana vayu- controller of voluntary movements• Circulation of rasadi dhatus• Integration of motor and sensory functions
VATA MAMSA DHATU GATI
Ayurvedic concepts
• Adibala pravritta rogas• Mamsa pradoshaja vikara• Snayu sira kandara upadhatu pradoshaja• Mamsa kshaya• Mamsa gata vata• Mamsa dhatu avarana
Srotas involvement
• Mamsavaha Kshaya- Angaglani, ganda, sphik sushkata
• Rasavaha - gourava, srotorodha, sada, krishangata, sosha
• Raktavaha - sopha
Nidana
• Lack of proper garbhavriddhikara bhavas(Beeja kshetra guna …….)
• Improper nutrition of mother during garbhakala( 5th month- mamsa sonitha upachaya)
• Mamsavaha sroto dushti karana(Abhisyandini bhojyaani………..)
NIDANAS
VAYUMAMSA DHATU
KSHAYA SROTORODHA
SAADASANGA
STAMBHAKARSYA
RASADIDHATU
PARINAMA
BEEJA
AHARA
KAPHA
AGNIMANDYA
KSHETRA
SNAYU
Preventive aspect
• Nidana parivarjana• Masanumasika garbhini charya( 5th month)• Ksheera sarpi• Shashtika odana with payas• Protect agni-to cause doshapaka & prevent
dhatupaka• Ojaskara, rasayana
Aim at…
• Vata shamana
• Kapha vardhaka
• Amapachana, deepana
• Mamsa dhatu pushtikara
Chikitsa sootra• Mamsa dhatu avrita chikitsa“Swedaabhyanga rasah ksheeram sneho mamsavrite hitam”
SWEDANAM
MAMSARASA
KSHEERAMSNEHANAM
ABHYANGAM
• Rajayakshma chikitsa“Balino bahudoshasya snigdha swinnasya sodhanamUrdhwa adho yakshminah kuriyaat sneham yat naa karshanam”
• After proper sodhana - annapanaBrmhanam DeepanamHrudya & laghuVathagna
• Mamsarasa -“Mamsam mamsena vardhyate”“Balam hyalam doshaharam param tat cha balapradam”
Formulations • Kashayam - Dashamoola , Panchkola,Vidaryadi
• Choornam- Shaddharanam, Vaiswanaram
• Ghritam- Shatpala, Indukantham,Dasamoola siddha ghrita with payas/ mamsarasa
• Lehyam- Aswagandhadi, Chyawanaprasam
• Rasayana- Lasuna, Brahma, Dasamoola
Treatment procedures
• Abhyangam• Swedanam• Snigdha virechanam• Shashtika pinda swedam• Ksheera vasthi
THANK YOU