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ROLE OF PANCHAKARMA
IN MUSCULAR
DYSTROPHY
Introduction
 Rare Inherited disease.
 Incidence : 1 : 10 000 (world wide).
 Characterized by Progressive muscle weakness and wasting .
 Gene mutation.
 Skeletal Muscles are affected. Later Fat and connective tissue
replaces the muscle fibre.
 Syn : Myodysrophy, Myodystrophia .
 Types : 30
 No cure ........ But can slow down the progression by
medications and other therapies.
 First historical account of Md. is appeared in 1830. by Sir
Charles Bell. After 1850 a French neurologist Guillaume
Duchenne gave a comperhensive account on Md.
 In 1864, Dr. Edward Meryon recognize the maternal
inheritence .
 In the late 1970 , genetic studies linked the Duchenne gene
to chromosome Xp21.
 In 1987 , dystrophin were discovered .
Historical overview
Muscular Dystrophy
 The word dystrophy is derived from the Greek word ‘dys’
means difficult and ‘troph’means nourish.
 Affects people of either Sex and of all age.
 It refers to a group of more than 30 genetic diseases
 In most of the cases the Muscles ( Vouluntary muscles) are
affecting but In some cases the invoulantary muscles and
other organs too are affected.
Conti..
 General Clinical symptoms -
1. Muscle weakness
2. Delayed development of motor muscle skills
3. Progressive muscle wasting
4. Difficulty using one or more muscle groups
5. Drooling
6. Eyelid drooping
7. Frequent falls
8. Lose of muscle strength, size
9. Waddling gait
10. Calf deformation and Respiratory difficulty
Contin...
 Early onset Symptoms-
1. Neonatal hypotonia
2. Generalised muscle weakness
3. Recurrent aspiration
4. Weak cry
5. Prominent head lag
6. Decreased Muscle bulk,Tendon reflex
7. Delayed motor milestone.
Types and classification
 Based on gene mutation-
DMD & BMD LGMD, CMD
 Involve mutations in the
dystrophin gene
 Involve mutations in several
genes
 X-linked inheritance  Autosomal recessive
Inheritance
• DEFECTS, in
Intracellular muscle cell protein
• DEFECTS, in
Extra-cellular Matrix
• In BMD
Dystrophin is partially functional or
its reduced expression is seen.
• In DMD
Dystrophin gene is missing
Duchenne’s Muscular Dystrophy
 Inheritance - X-Linked recessive
 Defective gene / Protein - Dystrophin
 Onset age - Before 5 years
 Clinical features -
Progressive weakness of gridle muscles,
Unable to walk after age 12,
Progressive Khyphoscoliosis and
Respiratory Failure in 2nd or 3rd decade of life.
 Other organ/ systems involved - Mental impairement
Cardiomyopathy,
Respiratory system
Pseudohypertrophy of calf muscles
Gower’s sign
Becker’s Muscular Dystrophy
 Inheritance - X-Linked
recessive
 Defective gene / Protein- Dystrophin
 Onset age - Early childhood to
adult
 Clinical features -
Progressive weakness of gridle muscles
Able to walk after age 15
Respiratory Failure in 2nd or 3rd decade of
life.
 Other organ/ systems involved- Cardio
Muscular distrophies
Limb Gridle Muscular Dystrophy
 Inheritance -
May be Autosomal dominant or Autosomal
recessive
 Defective gene / Protein - Several
( e.g Sarcoglycans, Dysferlin, Calpain-3 etc...)
 Onset age - Early childhood to early
adult
 Clinical features - Slow progressive
weakness of shoulder and hip gridle muscles.
 Other organ / systems involved -
Cardiomyopathy
Myotonic Muscular dystrophy
Features DM1 DM2
 Inheritance Autosomal dominant Autosomal dominant
 Defective gene Expansion CGT repeat Expansion CCGT
repeat
 Onset age Childhood to adult Maybe Infancy if mother
affected
 Clinical
features
Slowly progressive
weakness of face shoulder
gridle and foot dorsiflexion
Proximal muscle
weakness
 Other organ/
systems
involved-
Cardiac Conduction defects
Mental
impairment,Cataract,
Frontal baldness,
Gonadal Atrophy.
Facioscapulohumeral Muscular
Dystrophy
 Inheritance - Autosomal dominant
 Defective gene / Protein - DUX4 4q
 Onset age - Childhood to Adult
 Clinical features - Fascioscapulohumeral muscular
dystrophy is characterized by weakness of the facial, upper limb, and
shoulder girdle muscles. Weakness of the anterior tibial muscles
produces a footdrop gait, there is also scapular instability, marked
limitation in arm abduction, and the characteristic scapular winging.
 Other organ systems involved – Hearing loss and retinal
venous anomalies are common.
Oculopharyngeal Muscular
Dystrophy
 Inheritance - Autosomal dominant
 Defective gene / Protein- Expansion poly- A
RNA binding protein
 Onset age - 5th to 6th decade of life
 Clinical features - Slow progressive
weakness of extraoclular and limb muscles
along with progressive dysphagia, ptosis, and
proximal muscle weakness.
 When ptosis is severe, the patient tilts the head
back and contracts the frontalis muscle in order
to see.
Laboratory findings for
Diagnosis
 Serum CK levels (20 -100 times elevated, than normal)
 EMG reveals features typical of myopathy
 Muscle biopsy (necrotic and regenerating muscle fibres)
 Mutation Analysis (using Peripheral blood leukocytes.)
 Western Blot Analysis (using muscle biopsy specimen)
 Immunocytochemical staining of muscle with dystrophin
antibodies.
 Ultra sonography
For Carriers detection, dystrophin analysis, using
muscle biopsy is not reliable.
Treatment
 IN DMD
 Glucocorticoids
Prednisone in a dose of 0.75 mg/kg per day
 IN MYOTONIC
 Mexiletine, Phenytoin, Quinine,Procainamide , Carbamazepine
Assistive devices includes Braces, Canes,
Wheelchair,Walker, Ventilator.
 Gene therapy
 Physical therapy, Hydrotherapy (hot bath)
 Surgery
 Tendon release surgery ( eg at Achilles tendon, hip , knee)
Surgery also helps to correct the curvatures of spine.
Ayurvedic view
 Possible corelation -
 Dhatu kshaya
 Bijadosha vyadhi
 Mamsa Gata vata
 Snayugata Vata
 Line of Managenment –
 Srodhoshodhana
 Rukshana
 Avaranagna
 Brimhana chikitsa
Lakshana ( similarity with Md)-
Mamsa Kshaya Mamsa Gada vata Snayu gada vata
Indriya dourbalyam Gurvanga Bahya abhyantara
ayaama m
Ganda ,swik shushkatha Tudhyate (like beaten
with danda , Mushti
etc..)
Khalli
Sandhivedana Srama along with pain Kubhjatwam
Sarvaanga/ ekanga
rogam
Role of Panchakarma
 Deepana Pachana
Agni is considered as the whole and sole responsible for the
dhatupaka.Amapachana also
e.g Trikatu, Hinguvashtaka
 Langhana
 Rukshana with
e.g Udvarthana It provides the benefits like Sthirikarana anga ,
Dhanyamla dhara helps to remove the srotorodha
 Snehana bahya and abhyantara Snehana
 Abhyanga – Mahamasha th, Mahanarayana th,
Balashwagandhalakshadi. th
 Swedana
It relieves the pain due to the stiffness of muscles.
Shastika shali Pinda sweda, Bashpasweda, Nadisweda
 Shodananga snehapana
Amrutaprasha ghrita, Tikthaka ghrita Can be
used.
 Mridu Vamana
If the patient satisfies the conditons like Kaphagata
pitta, Utklesha kapha.It corrects the depletion of
medas.
Vamana with - Vacha, Madanaphala
 Mirdu Virechana
Helps to bring the Anulomana and Tridoshahara
 Bhrimhana Basthi
It should be in a kala or karma basti pattern.
Yapana basthi, Ksheera basthi, Using Mamsa rasa,
Madanaphala in basthi.
 Anuvasana Basthi
Tikta ghrita, Aswagandha ghrita, Chagalayadi ghritam.
 Nasya
It is having less importance but it can be used
in the Md associated with depression .
e.g Bhrimhana Nasya
Shamana medicines
 Kashayam –
Indukantham, Badradarvadi, Guduchyadi, Vidaryadi., Phalatrikadi
 Choornam, Vati, Guggulu –
Trikatu, Hinguvashtakam, Pippali, Ashwagandha, Chitrakadi Vati,
Trayodashanga Guggulu
 Ghritam
Nagabala, Bhrihat chagaladi, Shadpala
 Lehyam
Nayopayam, Narasimha, Kushmanda
 Arishtam
Balarishtam, Aswagandharishtam, Drakshasavam
Articles
 THERAPEUTIC EFFICACY OF
PANCHAKARMA IN MUSCULAR
DYSTROPHY- A CASE STUDY
(INTERNATIONAL AYURVEDIC MEDICAL JOURNAL)
A 12 yrs old male patient, c/o- Weakness in lower limbs, Difficulty
while walking, climbing stairs and running since 4 years.
Decreased muscle bulk around pelvic and thigh region and
increased muscle bulk in calf muscles since 3 years
Treatment given –
Total 4 sittings. 1 st sitting
1. SarvangAbhyanga (Mahanarayana oil) - 14
days 2. YapanaBasti (MamsaRasa) - 8 days
 Second sitting :
1. Tail Dhara (Dhanwantaram tailam) - 14 days
2. Yoga Vasti-Dashmoola Kwath Niruham Vasti
3. Anuvasana Vasti – Dhanwantaram tailam
 Third sitting:
1. Udvartana – 3 days followed by ShashtiShaliPinda Sweda for 14
days
2. Yoga Vasti –Yapana Vasti (Mamsa Rasa)
 Fourth sitting :
 1. Udvartana for 3 days followed by ShashtiShaaliPinda Sweda –
14 days 2. Yoga Vasti - Dashmoola Kwatha Niruham
3.Anuvasana Vasti - Dhanwantaram tailam
Muscle Bulk Thigh: 12.5 inch (B/T), 13 inch (A/T)
Calf muscles hypertrophy : 14.2 inch (B/T), 13.8 inch (A/T)
Reflex (knee jerk) Diminished (B/T) , (A/T) No change
Power (lower limb) Right
Left (B/T) (A/T) 4/5 NO CHANGES
Toe walking: Present (B/T), Not Present (A/T)
Serum CPK : 916 U/L (B/T)
256 U/L (A/T)
THANK
YOU

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Role of Panchakarma in Muscular Dystrophy

  • 1. ROLE OF PANCHAKARMA IN MUSCULAR DYSTROPHY
  • 2. Introduction  Rare Inherited disease.  Incidence : 1 : 10 000 (world wide).  Characterized by Progressive muscle weakness and wasting .  Gene mutation.  Skeletal Muscles are affected. Later Fat and connective tissue replaces the muscle fibre.  Syn : Myodysrophy, Myodystrophia .  Types : 30  No cure ........ But can slow down the progression by medications and other therapies.
  • 3.
  • 4.  First historical account of Md. is appeared in 1830. by Sir Charles Bell. After 1850 a French neurologist Guillaume Duchenne gave a comperhensive account on Md.  In 1864, Dr. Edward Meryon recognize the maternal inheritence .  In the late 1970 , genetic studies linked the Duchenne gene to chromosome Xp21.  In 1987 , dystrophin were discovered . Historical overview
  • 5. Muscular Dystrophy  The word dystrophy is derived from the Greek word ‘dys’ means difficult and ‘troph’means nourish.  Affects people of either Sex and of all age.  It refers to a group of more than 30 genetic diseases  In most of the cases the Muscles ( Vouluntary muscles) are affecting but In some cases the invoulantary muscles and other organs too are affected.
  • 6. Conti..  General Clinical symptoms - 1. Muscle weakness 2. Delayed development of motor muscle skills 3. Progressive muscle wasting 4. Difficulty using one or more muscle groups 5. Drooling 6. Eyelid drooping 7. Frequent falls 8. Lose of muscle strength, size 9. Waddling gait 10. Calf deformation and Respiratory difficulty
  • 7. Contin...  Early onset Symptoms- 1. Neonatal hypotonia 2. Generalised muscle weakness 3. Recurrent aspiration 4. Weak cry 5. Prominent head lag 6. Decreased Muscle bulk,Tendon reflex 7. Delayed motor milestone.
  • 8. Types and classification  Based on gene mutation- DMD & BMD LGMD, CMD  Involve mutations in the dystrophin gene  Involve mutations in several genes  X-linked inheritance  Autosomal recessive Inheritance • DEFECTS, in Intracellular muscle cell protein • DEFECTS, in Extra-cellular Matrix • In BMD Dystrophin is partially functional or its reduced expression is seen. • In DMD Dystrophin gene is missing
  • 9. Duchenne’s Muscular Dystrophy  Inheritance - X-Linked recessive  Defective gene / Protein - Dystrophin  Onset age - Before 5 years  Clinical features - Progressive weakness of gridle muscles, Unable to walk after age 12, Progressive Khyphoscoliosis and Respiratory Failure in 2nd or 3rd decade of life.  Other organ/ systems involved - Mental impairement Cardiomyopathy, Respiratory system
  • 10.
  • 11. Pseudohypertrophy of calf muscles Gower’s sign
  • 12. Becker’s Muscular Dystrophy  Inheritance - X-Linked recessive  Defective gene / Protein- Dystrophin  Onset age - Early childhood to adult  Clinical features - Progressive weakness of gridle muscles Able to walk after age 15 Respiratory Failure in 2nd or 3rd decade of life.  Other organ/ systems involved- Cardio
  • 14. Limb Gridle Muscular Dystrophy  Inheritance - May be Autosomal dominant or Autosomal recessive  Defective gene / Protein - Several ( e.g Sarcoglycans, Dysferlin, Calpain-3 etc...)  Onset age - Early childhood to early adult  Clinical features - Slow progressive weakness of shoulder and hip gridle muscles.  Other organ / systems involved - Cardiomyopathy
  • 15.
  • 16. Myotonic Muscular dystrophy Features DM1 DM2  Inheritance Autosomal dominant Autosomal dominant  Defective gene Expansion CGT repeat Expansion CCGT repeat  Onset age Childhood to adult Maybe Infancy if mother affected  Clinical features Slowly progressive weakness of face shoulder gridle and foot dorsiflexion Proximal muscle weakness  Other organ/ systems involved- Cardiac Conduction defects Mental impairment,Cataract, Frontal baldness, Gonadal Atrophy.
  • 17.
  • 18. Facioscapulohumeral Muscular Dystrophy  Inheritance - Autosomal dominant  Defective gene / Protein - DUX4 4q  Onset age - Childhood to Adult  Clinical features - Fascioscapulohumeral muscular dystrophy is characterized by weakness of the facial, upper limb, and shoulder girdle muscles. Weakness of the anterior tibial muscles produces a footdrop gait, there is also scapular instability, marked limitation in arm abduction, and the characteristic scapular winging.  Other organ systems involved – Hearing loss and retinal venous anomalies are common.
  • 19.
  • 20. Oculopharyngeal Muscular Dystrophy  Inheritance - Autosomal dominant  Defective gene / Protein- Expansion poly- A RNA binding protein  Onset age - 5th to 6th decade of life  Clinical features - Slow progressive weakness of extraoclular and limb muscles along with progressive dysphagia, ptosis, and proximal muscle weakness.  When ptosis is severe, the patient tilts the head back and contracts the frontalis muscle in order to see.
  • 21.
  • 22. Laboratory findings for Diagnosis  Serum CK levels (20 -100 times elevated, than normal)  EMG reveals features typical of myopathy  Muscle biopsy (necrotic and regenerating muscle fibres)  Mutation Analysis (using Peripheral blood leukocytes.)  Western Blot Analysis (using muscle biopsy specimen)  Immunocytochemical staining of muscle with dystrophin antibodies.  Ultra sonography For Carriers detection, dystrophin analysis, using muscle biopsy is not reliable.
  • 23. Treatment  IN DMD  Glucocorticoids Prednisone in a dose of 0.75 mg/kg per day  IN MYOTONIC  Mexiletine, Phenytoin, Quinine,Procainamide , Carbamazepine Assistive devices includes Braces, Canes, Wheelchair,Walker, Ventilator.  Gene therapy  Physical therapy, Hydrotherapy (hot bath)
  • 24.  Surgery  Tendon release surgery ( eg at Achilles tendon, hip , knee) Surgery also helps to correct the curvatures of spine.
  • 25. Ayurvedic view  Possible corelation -  Dhatu kshaya  Bijadosha vyadhi  Mamsa Gata vata  Snayugata Vata  Line of Managenment –  Srodhoshodhana  Rukshana  Avaranagna  Brimhana chikitsa
  • 26. Lakshana ( similarity with Md)- Mamsa Kshaya Mamsa Gada vata Snayu gada vata Indriya dourbalyam Gurvanga Bahya abhyantara ayaama m Ganda ,swik shushkatha Tudhyate (like beaten with danda , Mushti etc..) Khalli Sandhivedana Srama along with pain Kubhjatwam Sarvaanga/ ekanga rogam
  • 27. Role of Panchakarma  Deepana Pachana Agni is considered as the whole and sole responsible for the dhatupaka.Amapachana also e.g Trikatu, Hinguvashtaka  Langhana  Rukshana with e.g Udvarthana It provides the benefits like Sthirikarana anga , Dhanyamla dhara helps to remove the srotorodha  Snehana bahya and abhyantara Snehana  Abhyanga – Mahamasha th, Mahanarayana th, Balashwagandhalakshadi. th  Swedana It relieves the pain due to the stiffness of muscles. Shastika shali Pinda sweda, Bashpasweda, Nadisweda
  • 28.  Shodananga snehapana Amrutaprasha ghrita, Tikthaka ghrita Can be used.  Mridu Vamana If the patient satisfies the conditons like Kaphagata pitta, Utklesha kapha.It corrects the depletion of medas. Vamana with - Vacha, Madanaphala  Mirdu Virechana Helps to bring the Anulomana and Tridoshahara
  • 29.  Bhrimhana Basthi It should be in a kala or karma basti pattern. Yapana basthi, Ksheera basthi, Using Mamsa rasa, Madanaphala in basthi.  Anuvasana Basthi Tikta ghrita, Aswagandha ghrita, Chagalayadi ghritam.
  • 30.  Nasya It is having less importance but it can be used in the Md associated with depression . e.g Bhrimhana Nasya
  • 31. Shamana medicines  Kashayam – Indukantham, Badradarvadi, Guduchyadi, Vidaryadi., Phalatrikadi  Choornam, Vati, Guggulu – Trikatu, Hinguvashtakam, Pippali, Ashwagandha, Chitrakadi Vati, Trayodashanga Guggulu  Ghritam Nagabala, Bhrihat chagaladi, Shadpala  Lehyam Nayopayam, Narasimha, Kushmanda  Arishtam Balarishtam, Aswagandharishtam, Drakshasavam
  • 32. Articles  THERAPEUTIC EFFICACY OF PANCHAKARMA IN MUSCULAR DYSTROPHY- A CASE STUDY (INTERNATIONAL AYURVEDIC MEDICAL JOURNAL) A 12 yrs old male patient, c/o- Weakness in lower limbs, Difficulty while walking, climbing stairs and running since 4 years. Decreased muscle bulk around pelvic and thigh region and increased muscle bulk in calf muscles since 3 years Treatment given – Total 4 sittings. 1 st sitting 1. SarvangAbhyanga (Mahanarayana oil) - 14 days 2. YapanaBasti (MamsaRasa) - 8 days
  • 33.  Second sitting : 1. Tail Dhara (Dhanwantaram tailam) - 14 days 2. Yoga Vasti-Dashmoola Kwath Niruham Vasti 3. Anuvasana Vasti – Dhanwantaram tailam  Third sitting: 1. Udvartana – 3 days followed by ShashtiShaliPinda Sweda for 14 days 2. Yoga Vasti –Yapana Vasti (Mamsa Rasa)  Fourth sitting :  1. Udvartana for 3 days followed by ShashtiShaaliPinda Sweda – 14 days 2. Yoga Vasti - Dashmoola Kwatha Niruham 3.Anuvasana Vasti - Dhanwantaram tailam
  • 34. Muscle Bulk Thigh: 12.5 inch (B/T), 13 inch (A/T) Calf muscles hypertrophy : 14.2 inch (B/T), 13.8 inch (A/T) Reflex (knee jerk) Diminished (B/T) , (A/T) No change Power (lower limb) Right Left (B/T) (A/T) 4/5 NO CHANGES Toe walking: Present (B/T), Not Present (A/T) Serum CPK : 916 U/L (B/T) 256 U/L (A/T)

Notas del editor

  1. with later involvement of lower extremity muscles in about 20% of cases.
  2. apaharyheal,