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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 4  |  Issue : 1  |  Page : 16-21

Ayurvedic management in a chronic spinocerebellar ataxia and progressive improvement in scale for assessment and rating of ataxia score – An interrupted time series case report


Department of Kayachikitsa, Government Ayurveda College, Thiruvananthapuram, Kerala, India

Date of Submission26-Feb-2021
Date of Acceptance13-May-2021
Date of Web Publication17-Jun-2021

Correspondence Address:
Dr. Natesan K Pravith
Department of Kayachikitsa, Government Ayurveda College, Thiruvananthapuram, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jacr.jacr_20_21

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  Abstract 


Spinocerebellar Ataxias (SCAs) are a highly heterogenous group of disorders that often present as progressive incoordination of walking. Studies are underway globally to find a cure for this genetic disease. Although there are a few Ayurveda case studies reported in SCA, time series reporting of a case has not been done till date. In the present study, a case of SCA was admitted in the IPD, thrice in the period March 2019 to October 2020. The duration of therapy was around two months in each admission. Treatments that aim at arresting the progress of disease such as Nasya (~nasal therapy), Basti (~medicated enema), and Rasayana (~rejuvenative) and that strengthen musculoskeletal system such as Abhyanga (~external oleation) and Churna pinda sweda (~bolus fomentation) were done. The interrupted time series analysis was done with the help of Scale for Assessment and Rating of Ataxia (SARA) score on March 29, 2019, May 27, 2019, January 9, 2020, March 5, 2020, August 14, 2020, and October 13, 2020 and it showed progressive improvement with each admission.

Keywords: Abhyanga, Basti, Churna pinda sweda, Rasayana, spinocerebellar ataxia


How to cite this article:
Pravith NK, Krishna B. Ayurvedic management in a chronic spinocerebellar ataxia and progressive improvement in scale for assessment and rating of ataxia score – An interrupted time series case report. J Ayurveda Case Rep 2021;4:16-21

How to cite this URL:
Pravith NK, Krishna B. Ayurvedic management in a chronic spinocerebellar ataxia and progressive improvement in scale for assessment and rating of ataxia score – An interrupted time series case report. J Ayurveda Case Rep [serial online] 2021 [cited 2021 Jul 26];4:16-21. Available from: http://www.ayucare.org/text.asp?2021/4/1/16/318656




  Introduction Top


The Spinocerebellar Ataxias (SCAs) are a group of autosomal dominant disorders characterized by progressive ataxia due to degeneration of the cerebellum and its afferent and efferent pathways.[1] The core triad of SCAs includes gait ataxia and incoordination, nystagmus/visual problems, and dysarthria. Patients can present with additional features of pyramidal, extrapyramidal signs, ophthalmoplegia, and cognitive impairment in specific SCA.[2] With some exceptions, the onset of symptoms usually occurs after the age of 18 (“adult-onset”).[3] The main disease mechanisms of SCAs include toxic RNA gain-of-function, mitochondrial dysfunction, channelopathies, autophagy, and transcription dysregulation.[2] The different types of SCA are classified according to the mutated gene responsible. Slowly progressive ataxia accompanied by cerebellar degeneration is often genetic in origin.[4] SCA3 is the most common subtype worldwide. A recent systemic review shows that global prevalence of SCA is 3 in 100,000 placing it in the rare disease group.[2] Another study from India that retrospectively analyzed 3-year molecular data from central reference laboratory showed highest positivity for SCA12.[5] The prognosis of SCA varies with type. With a few exceptions (e.g., SCA6), SCAs are relentlessly progressive and fatal diseases. No drugs for SCAs have been approved by the Food and Drug Administration (FDA) or the European Medicines Agency. However, multiple studies support the efficacy of coordinative physical therapy.[6]

Several studies have explored the short and long-term effects of neuro-rehabilitation in the treatment of cerebellar ataxia and the results stressed the need for effective long-term strategies to continue to maintain functionality and to sustain the outcomes.[7],[8] There are just two reported Ayurvedic studies on SCA available in indexed journals.[9],[10] Both the studies did a pre and post-assessment of a single course IP treatment. Although these studies have shown evidence of improvement, there is no light on its effectiveness over a period of time, so a time series reporting is essential to look on to its long-term effect.

As per Ayurvedic concepts, SCA follows the Samprapti (~pathophysiology) of Dhatukshayaja vatavyadhi (~neuro-degenerative disorders). The predominant Dosha is Vata with its subtypes Prana, Udana, and Vyana causing Shoshana of Medas and Majja dhatu. The subtle function of Prana is the Dharana of Buddhi (~intellect), Hridaya (~heart), Indriya (~sense organs), and Chitta (~cognition).[11] Indriya perceives its Artha (~objects of perception) and the command is transferred to Atma (~soul) by Manas (~mind), and the resultant message is conveyed to Karmendriya (~responsible for motor functions) again through Manas. Buddhi is Atma guna. Hence, even though a direct inflict to Buddhi, Indriya, and Chitta does not happen, a Prana vayu dushti may cause an incoordination between these, so that the precision with which an Indriya perceives its Artha may be hampered. In ataxia, due to this incoordination between Buddhi, Hridaya, Indriyas, and Chitta; dysmetria and tremor like symptoms occur. Vak pravrutti (~phonation) is the main function of Udana vata and Vyana vata helps in all types of Gati (~movement) of Angas (~body parts) and it seems like Prana has a regulatory control over other Vayus including Udana and Vyana. Hence, the frequency and amplitude with which Udana and Vyana have to work are dependent mainly on the well-being of Prana vata. Head being the Sthana of Prana vata and in a condition like SCA where there is neuro-degenerative change in the brain, role of Prana vata is explicit.


  Case Report Top


A 54-year-old unmarried male, belonging to a lower economic status, a non-smoker and a non-alcoholic with no known comorbidities presented to the OPD on March 29, 2019 with complaints of difficulty and unsteadiness in walking and impaired speech from his 13 years of age. He had insidious onset of imbalance while walking, i.e., swaying on either side when he was 13 years old. The imbalance gradually progressed and by five years after the onset of disease, he could walk only with support. Hence, he consulted in a government allopathic hospital and computed tomography scan was done, the reports of which are not available. He took allopathic medicines for around six years. However, the symptoms progressed. He developed tremors while attempting to do anything with his upper limbs. His speech became unintelligible. He had repeated falls forward and toward left side. Complaints of blurring of vision and double vision also developed. Nine years after the onset of disease, he again consulted in an allopathic hospital and MRI was done in which marked atrophy of cerebellar hemispheres and vermis, superior and middle cerebellar peduncle along with mild atrophy of brain stem was noted. He was clinically and radiologically diagnosed as SCA. He had no family history of such an illness. He again took allopathic medicines on and off for about 10 years. However, there was no improvement and the above symptoms worsened. For about 20 years, he was not on any medication. Hence, he decided to take Ayurvedic treatment. The patient was admitted in the IPD thrice during March 2019, January 2020, and August 2020. Ayurvedic diagnosis was made as Mastulunga kshaya janya vatavyadhi (~neuro-degenerative disorder) substantiated by MRI.

Clinical findings

In his initial presentation, the patient was moderately built and nourished with waist and hip measuring 79 cm and 86 cm, respectively. The patient had reduced sleep, moderate appetite, Visamagni (~unstable digestive functions), constipated bowel, and burning micturition with reduced frequency, clean tongue, and clear voice. His intelligence was normal and had a Vata-kapha prakriti with Madhyama sara (~medium purity of body tissue), Madhyama samhanana (~medium body built), Sama pramana (~equal body proportions), Madhyama satmya (~suitability or homologation), Madhyama satva (~psyche), Avara vyayamshakti (~least physical endurance), and Madhyama aharasakti and Jaranasakti (~medium food intake and digestive power). Skin, cardiovascular, respiratory, musculoskeletal, and genitourinary system examinations were normal.

Therapeutic interventions

Considering the Asadhya (~incurable) nature of the disease, a two-dimensional approach was planned to arrest the progress of disease and to improve the motor skills. The first course of inpatient treatment was given from March 29, 2019, to May 27, 2019 [Table 1]. Internally, Ashtavargam kashaya,[12] Gandharvahastadi kashaya,[12] with 5 g Vaiswanara churna[13] as Anupana and Chandanadi kashaya[14] for 17, 14, and 29 days, respectively, was given. Along with these, externally, he underwent Udwartana with Kolakulathadi churna,[15] Abhyanga with Bala taila,[16] Dhanyamladhara,[17] and Kayaseka and Yoga basti with Sahacharadi taila.[18] He was advised to take Brahma rasayana[19] (5 g morning) and Dasamoolahareethaki lehya[20] (5 g at bed time) internally for one month after discharge.
Table 1: First course of treatment

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The second course of treatment was from January 9, 2020 to March 04, 2020 [Table 2]. He was given Maharasnadi kashaya[21]and Guggulutiktaka ghrita[22] internally. Procedures such as Panchatiktaka ksheera basti[23] and Shashtika pinda sweda and Virechana with Gandharva eranda taila[24] were done. After the second course, he was advised to take Bhadradarvadi kashaya[25] (90 ml twice daily), Rasa taila[26] (10 drops with Kashaya), Rasnadasamoola ghrita[27] (5 g at bed time), and self-Abhyanga with Bala taila for one month.
Table 2: Second course of treatment

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The third course of treatment was from August 13, 2020, to October 13, 2020 [Table 3]. During this course, Pratimarsha nasya with Dhanwantara (101) avarti taila[28] and Kayaseka with Dhatryadi ghrita[29] were done. Rasayana therapy with Chitraka churna[30] and Ksheerabala taila[31] as Anupana was given finally. He was advised to continue Chitraka churna (2.5 g with 5 ml Ksheerabala taila at 6 am before food) and Abhyanga with Mahamasha taila[32] and Karpasasthyadi taila[33] for one month after discharge.
Table 3: Third course of treatment

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  Timeline Top


The patient was managed with repeated inpatient treatments. The timeline of improvement in SARA score with respect to admissions is represented graphically [Graph 1].




  Follow up and outcome Top


The patient was assessed on the Scale for Assessment and Rating of Ataxia (SARA) on the day of admission and discharge which showed progressive improvement with each admission [Table 4].
Table 4: Assessment before and after treatment

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  Discussion Top


Considering the incurability and chronicity of the disease, long-term management of the patient is inevitable. There are only a few therapeutic options available, as there is no known cure for this disease in modern medicine. Many drugs are under research and those proven is not easily available and are quite expensive.[6] Although neuro-rehabilitation is known to be effective in SCA,[34] owing to patient's economic status, he did not opt for it. The most prominent available alternative therapy in India, for a neuro-degenerative disease like SCA, is Ayurveda. There are two reported Ayurveda case studies available in indexed journals.[9],[10] Both the studies did a pre and post assessment of a single course IP treatment. The first case report made their assessment on the basis of SARA score, and substantial recovery was reported by the authors of the said study through Ayurvedic management. The second study showed an objective improvement in balance after one month of Ayurvedic therapy where the objective testing of balance was done using dynamic posturography. While before treatment, all the patients had one or more abnormal balance indices, following treatment, 40% of patients showed normal balance indices.[10] The two studies have proven the effectiveness of Ayurvedic management in SCA, but none of them have prospectively followed the patient to assess the long-term benefit of the treatment. Hence, this kind of a time series case reporting is imperative in the current scenario.

Mastulunga kshaya (~brain atrophy) is taken as the cause of ataxia as per Ayurveda. The cumulative principle of treatment adopted was Rukshana, Snehana, and Rasayana. Rukshana was done through Udwartana; Snehana through procedures such as Abhyanga, Nasya and Rasayana through Basti and Chitraka rasayana. Head being the site, Prana vata has an inevitable role. Hence, treatments that target Prana vata are important in the management. Yoga basti is one such treatment that alleviates all Vata from Apana to Prana. Basti contains a homogenous emulsion made of oil, honey, and bolus of herbs. Here, the oil taken was Sahacharadi taila which is indicated in Akshepaka vyadhi (~movement disorders). The second discharge was done in March (Greeshma ritu), so in order to sustain the results, Basti was modified as Ksheera basti. Brain is considered to be a form of Majjadhara kala in Ayurveda.[35] Unsteadiness and blurred vision are described in Majja-pradoshaja vikaras (~diseases produced in vitiated Majja dhatu).[36] In Majjagata diseases, Tiktadi and Madhura drugs are indicated.[37] Hence, Panchatiktaka ksheera basti was selected. Nasya is another treatment that acts at the site as nostrils are the gateway to access brain. The oil used was Dhanwantara (101) avarti taila which is indicated for all Vata disorders and disorders of Marma (~vital organs).

In general, neuro-degenerative disorders are compiled into the spectrum of Vata vyadhi. Therefore, treatments for muscle strengthening such as Abhyanga, Churna pinda sweda, Kayaseka, and Shashtika pinda sweda were done. The oil chosen for all these treatments were generally Vata alleviating and Brimhana (~nourishing). Churna pinda sweda leave channels open for accepting further nourishing treatments offered. Kayaseka and Shashtika pinda sweda clears minute circulatory pathways and thus nourishes muscles and peripheral nerves. At the end, Rasayana therapy in the form of Chitraka churna and Ksheerabala taila as Anupana (~adjuvant) was used. Chitraka churna is Pachana (~digestive) and thereby aids Brimhana. Ksheerabala taila is Jeevana (~vitaliser), Swarya (~good for speech), and Brimhana.[27] Through this treatment protocol, we could bring the initial SARA scoring of 30 to 11½ at the time of last discharge. During the first course, SARA score decreased from 30 to 24. When he came for the second admission after seven months, his SARA score increased to 26, however, it did not go to the initial score. Similarly, there was an increase in the score when he came for the third admission, but not as high as that of the second admission score. Although there was improvement in the score, it was not fully sustained till the next admission, in spite of being on continuous follow-up with internal medications. Hence, it suggests the need of frequent combined internal and external treatments to sustain the results.


  Conclusion Top


Spinocerebellar ataxia can be managed with satisfactory outcome with Ayurvedic medicine and Panchakarma procedures. To sustain the obtained results, repeated combined internal and external treatments in an interval of 4–5 months are required. Such repeated hospitalizations are financially debilitating, so new government policies and schemes aiming to provide palliative care should be implemented in this regard. These findings may prove helpful for bringing discussions and development of new strategies and protocol in spinocerebellar ataxia and other similar conditions.

Acknowledgment

I wish to extend my deep sense of gratitude and profound thanks to my HOD Dr. Ambika K, Professor, Dept.of Kayachikitsa, Govt. Ayurveda College, Thiruvananthapuram and other faculties of the department for their keen interest and inspiring guidance to bring this article into fruition.

Declaration of patient consent

Authors certify that they have obtained patient consent form, where the patient/caregiver has given his/her consent for reporting the case along with the images and other clinical information in the journal. The patient/caregiver understands that his/her name and initials will not be published and due efforts will be made to conceal his/her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Introduction
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