2. Pathak, et al.: Ayurveda interventions in congenital muscular dystrophy
28 Journal of Ayurveda Case Reports - Volume 6, Issue 1, January-March 2023
legs from the supine position. The child was born with
full‑term, lower segment cesarean section (maternal
indication due to cephalopelvic disproportion),
and cried immediately after birth, with no perinatal
complications. Family history reveals mother had
hypothyroidism since 2015. She underwent surgery
for intestinal obstruction in May 2020 and expired
in September 2020 due to COVID‑19 infection. The
patient’s three years younger sister is also experiencing
the same problem as the patient have. Parents of the
child noticed muscle weakness, and delay in sitting and
standing at the 1st
year of age, and visited the hospital
in 2017, where after a thorough examination and muscle
biopsy (August 29, 2017), the child was diagnosed with
CMD (unclassifiable with available histochemical and
immunohistochemical stains). The patient was also
diagnosed with severe valvular pulmonary stenosis and
mild pulmonary regurgitation. Cardiac catheterization
with Tyshak balloon pulmonary dilation procedure
was done successfully with no complication at the age
of three years at the department of cardiology in a
multispecialty modern hospital in New Delhi.
CLINICAL FINDINGS
Onexamination,Nadi(~pulse)was74/min,Mutra(~urine)
frequency was 4–5 times in day and two times in
night. Mala (~excreta) was Nirama (~formed stool),
Shabda (~voice) was Spashta (~clear), Sparsha (~tactile
examination) was Ushna (~warm touch), Jivha (~tongue)
was Nirama (~clear), Drik (~eye and eyesight) was
Samanya (~normal), and Akruti (~body stature) was
Madhyama (~medium).
TIMELINE
The timeline of the case is depicted in Table 1.
DIAGNOSTIC ASSESSMENT
Details of the motor system examination including deep
tendon reflex[5]
are depicted in Table 2. Barthel index,[6]
muscle power grading with Medical Research Council
scale(MRCscale),[7]
SerumCreatinePhosphokinase(CPK),
and Serum Creatine Kinase‑MB (CK‑MB) which are
valuable noninvasive biomarker tools for monitoring
disease progression and therapeutic response in muscle
dystrophy was used as assessment tools before and after
the treatment.[8]
Liver Function Tests (LFT), Kidney
Function Tests (KFT), and Electrocardiogram (ECG)
were done before and after the treatment [Table 3]. The
patient was admitted on March 22, 2022, and internal
medications along with Panchakarma procedures (~five
therapeutic procedures) were started.
THERAPEUTIC INTERVENTIONS
After the admission of the patient, two times daily doses
of Bilwadi gutika (250 mg), Ashwagandha churna (2 g),
Pippali churna (250 mg), Pravala bhasma (250 mg), and
Shirisharishta (10 ml) were started. Udwartana (~therapeutic
massage using powders) with Kolakulathadi churna, Sarvanga
abhyanga (~oil application to the whole body) with
Table 1: Timeline of the case
Year Clinical event and intervention
April 24, 2015
The full‑term, lower segment cesarean section (maternal indication), cried immediately after birth, birth weight 2.5 kg
with no perinatal complications
April 27, 2015 Physiological jaundice; admitted for three days, treated and recovered
May 13, 2015 ECHO: Pulmonary stenosis, ventricular septal disease
February 27, 2017 Proximal muscle weakness noticed
March 22, 2017 CPK‑281 IU/L
April 5, 2017 Syrup osteocalcium 5 mL × 8 hourly, cholecalciferol granules 60,000 IU weekly for six weeks
June 6, 2017
Motor NCS: CMAPs were normal in all tested nerves with normal CVs and DLs
Sensory NCS: SNAPs were normal with normal CVs and DLs in all tested nerves
Needle EMG: Insertional activity was normal in tested muscles. No abnormal spontaneous activity was observed. The
recruitment pattern was normal in right vastus lateralis muscle with limited activity
August 29, 2017 Muscle biopsy report: CMD (unclassifiable with available histochemical and immunohistochemical stains)
November 15, 2017 Alpha‑glucosidase level: Normal
November 29, 2017 Pompe enzyme DBS: Normal level
April 1, 2018 Injection influenza vaccine given
December 18, 2018 Cardiac catheterization with Tyshak balloon pulmonary dilation procedure was done successfully with no complication
March 1, 2021 ABR: Bilateral good waves till 40 dB
March 22, 2022 Reported to our hospital for muscle weakness and other associated symptoms
ABR: Auditory brainstem response; DBS: Dried blood spot; EMG: Electromyography; DL: Distal latency; CVs: Conduction velocities;
SNAPs: Sensory nerve action potentials; NCS: Nerve conduction study; CMAPs: Compound muscle action potentials; CPK: Creatine phosphokinase;
ECHO: Echocardiogram; CMD: Congenital muscular dystrophy
[Downloaded free from http://www.ayucare.org on Tuesday, March 21, 2023, IP: 254.241.216.139]
3. Pathak, et al.: Ayurveda interventions in congenital muscular dystrophy
Journal of Ayurveda Case Reports - Volume 6, Issue 1, January-March 2023 29
Balashwagandhadi taila, and Sarvanga swedana (~whole body
fomentation with medicated vapors) with Dashamoola
kwatha and Yoga basti (~therapeutic enema) were used as
Panchakarma procedures [Table 4].
FOLLOW‑UP AND OUTCOME
Effects of therapy were assessed considering changes in
muscle power grading [Graph 1], Barthel index [Graph 2],
Sr. CPK, and Sr. CK‑MB [Table 3] before and after
treatment. At discharge after 17 days, the child started
balancing her body while standing and started lifting up
her legs from the supine position. The improvement was
seen in walking and climbing the stairs. Overall clinical
improvement was noted in the child.
DISCUSSION
Symptomsof CMDresembleMamsadhatukshaya(~depletion
of muscle tissues), Sphik griva shushyata (~muscle wasting in
hip and neck region),[9]
and Gatranam sadanam (~weakness
of body).[10]
Udwartana is useful in Sthirikaraṇaṃ anganam[11]
0
1
2
3
4
5
6
Elbow
Flexion
Elbow
Extension
Wrist
Flexion
Wrist
Extension
Knee
Flexion
Knee
Extension
Plantarflexion
Dorsiflexion
Before Treatment After Treatment
Graph 1: Muscle Power Grading (MRC scale). No muscle activation, 1:
Trace muscle activation, such as a twitch, without achieving full range of
motion; 2: Muscle activation with gravity eliminated, achieving full range
of motion; 3: Muscle activation against gravity, full range of motion;
4: Muscle activation against some resistance, full range of motion; 5:
Muscle activation against examiner’s full resistance, full range of motion)
Table 3: Patient assessment
Parameters March 23,
2022
April 7,
2022
Liver function test
Albumin (g/dL) 4.6 4.3
Total serum bilirubin (mg/dL) 0.2 0.2
Conjugated serum bilirubin (mg/dL) 0.1 0.1
Unconjugated serum bilirubin (mg/dL) 0.1 0.1
SGOT (IU/L) 37 27
SGPT (IU/L) 17 13
ALP (IU/L) 173 163
Kidney function test
Total protein (g/dL) 7.1 6.93
Blood urea (mg/dL) 14.16 15.33
Serum creatinine (mg/dL) 0.95 0.39
CPK (U/L) 283.0 197
CK‑MB (U/L) 27.90 25.10
ECG Within
normal limits
Within
normal limits
SGOT: Serum glutamic‑oxaloacetic transaminase; SGPT: Serum glutamic
pyruvic transaminase; CPK: Creatine phosphokinase; CK‑MB: Creatine
kinase‑MB; ECG: Electrocardiogram; ALP: Alkaline phosphatase
Table 2: Details of motor system examination
Left upper limb Right upper limb
Muscle tone Normotonic Normotonic
Left lower limb Right lower limb
Muscle tone Hypotonia Hypotonia
Deep reflexes Left upper limb Right upper limb
Biceps 2+ 2+
Triceps 2+ 2+
Supinator 2+ 2+
Left lower limb Right lower limb
Kneejerk 1+ 1+
Ankle jerk 1+ 1+
Superficial reflex Left Right
Planter reflex Negative Negative
Muscle bulk Left Right
Mid‑upper arm circumference 14.6 cm 14.6 cm
Mid‑thigh circumference 32 cm 32 cm
Upper limb Lower limb
Coordination test Finger to nose test‑Positive Romberg test‑Negative
Upper limb Lower limb
Involuntary movements Mild tremors in bilateral hand while standing and then subsides No any involuntary movement
In deep reflexes (0: no response; 1+: A slight but definitely present response; 2+: A brisk response, 3+: A very brisk response; 4+: A tap elicits a
repeating reflex)
[Downloaded free from http://www.ayucare.org on Tuesday, March 21, 2023, IP: 254.241.216.139]
4. Pathak, et al.: Ayurveda interventions in congenital muscular dystrophy
30 Journal of Ayurveda Case Reports - Volume 6, Issue 1, January-March 2023
(~brings stability to body organs) and it is Rukshana
purvakarma (~preparatory procedure of desiccating
therapy). Udwartana with Kolakulathadi churna[12]
was selected
for improving body balance while standing, and also
as a preparatory procedure for Abhyanga (~therapeutic
massage) and Swedana (~sudation therapy). Abhyanga with
Balashwagandhadi taila[13]
is used to improve muscle strength.
It improves blood supply to the muscles, stimulates sensory
nerve endings of the skin, and reduces muscle fatigue
and pain.[14]
Swedana is Sthambhagna (~removes stiffness),
Gauravanigraha (~relief in heaviness), and Kapha‑Vata
nirodhaka.[15]
Swedana with Dashamoola kwatha was selected
for improving muscle spasm.[16]
Sthiradi niruha basti was
selected as it acts as Mamsabala pradaha (~enhances muscle
power)[17]
and the contents of Sthiradi niruha are having
Hridya (~beneficial for the heart) properties.[18]
Ashwagandha
is having Balya (~strength) and Rasayana (~rejuvenating)
properties,[19]
PippaliishavingDeepana(~enhancingmetabolic
fire), Vrishya (~aphrodisiac), and Rasayana (~rejuvenating)
properties.[20]
Pravala is also having Deepana, Vrishya, Balya
properties along with Vishaghana (~anti‑toxic) properties,[21]
it is also beneficial in cardiac disorders.[22]
Rasayana, Vrishya,
and Balya qualities of these drugs help in the improvement
of muscle strength.
Muscular dystrophies are typically associated with elevated
Serum creatine kinase[23]
(Sr. CPK and Sr. CK‑MB).
Higher values are associated with a greater burden on the
kidneys, developing acute renal failure, severe electrolyte
abnormalities, acid–base disturbances, and resulting in
significant morbidity.[24]
Excess of this act as endotoxin in
the body and can be considered Visha in Ayurveda. Drugs
such as Bilwadi gutika,[25]
Pravala, and Shirisharishta[26]
are
used due to their Vishaghana property. In Ayurveda, various
studies on muscular dystrophy used Vishaghana drugs, and
clinical improvement was observed.[27]
Improvement in muscle power was observed in the present
case infers improvement in activity of daily life. CPK
and CK‑MB values were insignificantly improved after
the treatment [Table 3], and LFT, KFT, and ECG were
within normal limits after treatment indicating the safety
of the internal medication and Panchakarma procedures.
At discharge after 17 days, the child started balancing their
body while standing and started lifting up their legs from
the supine position. This gives hope, where there are still
no curative treatment options for patients with congenital
muscular dystrophies,[28]
Ayurveda treatment modalities
can be explored for management.
CONCLUSION
Congenital muscular dystrophy can be managed on the
basis of Ayurvedic management. Improvement in the
activity of daily life and muscle power is seen in the present
case. Randomized controlled trials can be done to further
strengthen the evidence.
Declaration of patient consent
Authors certify that they have obtained the patient consent
form, where the caregiver has given her consent for
reporting the case along with the images and other clinical
information in the journal. The caregiver understands that
her name and initials will not be published and due efforts
will be made to conceal her identity, but anonymity cannot
be guaranteed.
Financial support and sponsorship
Nil.
0
2
4
6
8
10
12
14
16
Feeding
Bathing
Grooming
Dressing
Bowels
Bladder
Toilet
Use
Transfers*
Mobility**
Stairs
Before Treatment After Treatment
Graph 2: Barthel index. *Transfers from bed to chair and back;
**Mobility on level surfaces
Table 4: Therapeutic interventions
Duration Internal medicine
March 22 to
April 7, 2022
Bilwadi gutika (250 mg) with Tulasi rasa twice a day
after meal
Ashwagandha churna (2 g) + Pippali churna (250 mg)
+ Pravala bhasma (250 mg) with honey twice a day
after meal
Shirisharishta (10 mL) with equal quantity of water
twice a day after meal
Panchakarma procedures
March
22nd
‑24th
,
2022
Udwartana with Kolakulathadi churna
March
25th
‑30th
, 2022
Sarvanga abhyanga with Balashwagandhadi taila and
Sarvanga swedana with Dashamoola kwatha
March 31‑April
7, 2022
Sarvanga abhyanga with Balashwagandhadi taila,
Sarvanga swedana with Dashamoola kwatha
Anuvasana basti with Balashwagandhadi taila on
March 31, April1st
, 3rd
, 5th
, and 7th
, 2022
Sthiradi niruha basti on April 2nd
, 4th
, and 6th
, 2022
[Downloaded free from http://www.ayucare.org on Tuesday, March 21, 2023, IP: 254.241.216.139]
5. Pathak, et al.: Ayurveda interventions in congenital muscular dystrophy
Journal of Ayurveda Case Reports - Volume 6, Issue 1, January-March 2023 31
Conflicts of interest
There are no conflicts of interest.
REFERENCES
1. Pasrija D, Tadi P. Congenital Muscular Dystrophy. StatPearls Treasure
Island (FL): StatPearls Publishing; 2022.
2. Salari N, Fatahi B, Valipour E, Kazeminia M, Fatahian R, Kiaei A,
et al. Global prevalence of duchenne and becker muscular dystrophy:
A systematic review and meta‑analysis. J Orthop Surg Res 2022;17:96.
3. Claeys KG. Congenital myopathies: An update. Dev Med Child Neurol
2020;62:297‑302.
4. Shastri A, editor. Vyadhi sammudeshiya. In: Sushruta Samhita of
Sushruta Sutrasthana. Ch. 24., Ver. 5. Varanasi: Chaukhambha Sanskrit
Sansthan; 2016. p. 130.
5. Walker HK. Deep tendon reflexes. In: Walker HK, Hall WD, Hurst JW,
editors. Clinical Methods: The History, Physical, and Laboratory
Examinations. 3rd
ed., Ch. 72. Boston: Butterworths; 1990.
6. Mahoney FI, Barthel DW. Functional evaluation: The Barthel index.
Md State Med J 1965;14:61‑5.
7. Naqvi U, Sherman AL. Muscle strength grading. In: StatPearls. Treasure
Island (FL): StatPearls Publishing; 2022.
8. Burch PM, Pogoryelova O, Goldstein R, Bennett D, Guglieri M,
Straub V, et al. Muscle‑derived proteins as serum biomarkers for
monitoring disease progression in three forms of muscular dystrophy.
J Neuromuscul Dis 2015;2:241‑55.
9. Shastri K, Chaturvedi G, editors. Kiyanta shiraseeya adhyaya. In:
Charaka Samhita of Charaka Sutrasthana. Ch. 17., Ver. 65, Varanasi:
Chaukhambha Bharti Academy; 2013. p. 348.
10. Shastri A, editor. Dosha‑dhatu‑mala‑kshaya‑vriddhi‑vijnaniya. In:
Sushruta Samhita of Sushruta, Sutrasthana. Ch. 15., Ver. 13, Varanasi:
Chaukhambha Sanskrit Sansthan; 2016. p. 76.
11. Gupta KA, editor. Dinacharya adhyaya. In: Astanga Hridaya of
Vaghbhat, Sutrasthana. Ch. 2., Ver. 15, Varanasi: Chaukhambha
Prakashan; 2019. p. 26.
12. Shastri K, Chaturvedi G, editors. Sutrasthana. In: Charaka Samhita
of Charaka. Ch. 3., Ver. 18, Varanasi: Chaukhambha Bharti Academy;
2013. p. 62.
13. Nishteswar K, Vidyanath R, editors. Tailaprakarana. In: Sahasrayogam
by Anonymous. Varanasi: Chowkhamba Sanskrit Series Office; 2014.
p. 117.
14. Nunes GS, Bender PU, de Menezes FS, Yamashitafuji I, Vargas VZ,
Wageck B. Massage therapy decreases pain and perceived fatigue after
long‑distance Ironman triathlon: A randomised trial. J Physiother
2016;62:83‑7.
15. Shastri K, Chaturvedi G, editors. Sutrasthana, swedadhyaya. In: Charaka
Samhita of Charaka. Ch. 14., Ver. 13. Varanasi: Chaukhambha Bharti
Academy; 2014. p. 283.
16. Shastri A, editor. Sutrasthana, dravya‑sangrahaniya. In: Sushruta
Samhita of Sushruta. Ch. 38., Ver. 71‑2. Varanasi: Chaukhambha
Sanskrit Sansthan; 2016. p. 189.
17. Shastri K, Chaturvedi G, editors. Siddhisthana, bastisutriyam siddhi. In:
Charaka Samhita of Charaka. Ch. 3., Ver. 36‑7, Varanasi: Chaukhambha
Bharti Academy; 2015. p. 999.
18. Sharma PV, editor. Priyanighantu, Haritakayadivarga. Ch. 1., Ver. 46‑7.
Varanasi: Chaukhamba Prakashan; 2018. p. 16.
19. Sitaram B, editor. Poorvkhanda guduchyadi varga. In: Bhavaprakasha by
Bhavamisra. Part I. Sec. 1. Ch. 6., Ver. 189‑90. Varanasi: Chaukhambha
Orientalia; 2020. p. 278.
20. Sitaram B, editor. Poorvkhanda haritakyadi varga. In: Bhavaprakasha
by Bhavamisra. Part I. Sec. 1. Ch. 6., Ver. 53‑8, Varanasi: Chaukhambha
Orientalia; 2020. p. 137‑8.
21. Mishra SN, Bhaisajaya ratnavali of Kaviraj Govind das sen. Sodhan
Maran Gunadi Prakaran. Ch. 3, Ver. 3/153‑156. Varanasi: Chaukambha
Subharati Prakashan; 2019. p. 53.
22. Shree Baidyanath Bhawan Limited. Prakarana 2. In: Ayurved
Saar‑Sangrah, Sodhan‑Maran Prakarana. Allahabad: Shree Baidyanath
Bhawan Limited; 2015. p. 140.
23. Rocha CT, Hoffman EP. Limb‑girdle and congenital muscular
dystrophies: Current diagnostics, management, and emerging
technologies. Curr Neurol Neurosci Rep 2010;10:267‑76.
24. Luckoor P, Salehi M, Kunadu A. Exceptionally high creatine
kinase (CK) levels in multicausal and complicated rhabdomyolysis:
A case report. Am J Case Rep 2017;18:746‑9.
25. Gupta KA, editor. Uttarsthana, Sarpavishapratishedh. In: Astanga
Hridaya of Vaghbhata. Ch. 36., Ver. 84‑5. Varanasi: Chaukhamba
Prakashan; 2019. p. 799.
26. Mishra SN, editor. Visharogadhikara. In: Bhaisajaya Ratnavali of
Kaviraj Govind Das Sen. Ch. 72., Ver. 71‑3. Varanasi: Chaukambha
Subharati Prakashan; 2014. p. 1106.
27. Pathak VK, Mahapatra AK, Rajagopala S. Ayurveda interventions in
the management of Duchenne muscular dystrophy – A review. Ann
Ayurvedic Med 2022;11:243‑55.
28. Kirschner J. Congenital muscular dystrophies. Handb Clin Neurol
2013;113:1377‑85.
[Downloaded free from http://www.ayucare.org on Tuesday, March 21, 2023, IP: 254.241.216.139]