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