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 Table of Contents  
Year : 2022  |  Volume : 5  |  Issue : 3  |  Page : 130-134

Ayurvedic management of Hirayama disease: A case report

Department of Kayachikitsa, All India Institute of Ayurveda, New Delhi, India

Date of Submission15-Dec-2021
Date of Acceptance27-Aug-2022
Date of Web Publication03-Oct-2022

Correspondence Address:
Dr. Pooja Sharma
Department of Kayachikitsa, All India Institute of Ayurveda, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jacr.jacr_108_21

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Hirayama disease (HD), also known as monomelic amyotrophy, is rare cervical myelopathy due to spinal cord compression by forward displacement of the posterior dural sac during neck flexion that manifests itself as asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. A 23-year-old man came to the outpatient department with a complaint of weakness of the right upper limb for the past 3 years which started in the right hand and forearm along with tremors in both hands. Considering the clinical history, physical examination, and electromyography, the condition was diagnosed as HD by an allopathic consultant two years ago. The symptoms can be included under Vata vyadhi. Some of the symptoms such as Asthibheda (~splitting type of pain in the bones), Sandhishoola (~pain in joints), Mamsa kshaya (~diminution of muscle mass), and Bala kshaya (~diminished strength) come under Asthimajjagata vata. Following the symptomatology and its resemblance with Asthimajjagata vata, the patient was treated with Snehapana (~internal oleation) with Ashwagandha ghrita followed by Virechana karma (~purgation therapy). Anuvasana basti with Panchatikta kshira along with Shalishastika pinda svedana was done after Virechana. Rasayana kalpa ksheera chikitsa was given after completion of Basti procedure. After 40 days of treatment, the patient showed significant improvement in muscle bulk. The magnetic resonance imaging shows no neural compression. This case shows that Ayurveda treatment may be helpful in pathologies like HD. Further, better results may be obtained if Ayurvedic therapies start at an earlier stage and continued for a longer duration.

Keywords: Asthimajjagata vata, Panchakarma, Pinda sveda, Rasayana chikitsa

How to cite this article:
Sharma P, Kajaria D. Ayurvedic management of Hirayama disease: A case report. J Ayurveda Case Rep 2022;5:130-4

How to cite this URL:
Sharma P, Kajaria D. Ayurvedic management of Hirayama disease: A case report. J Ayurveda Case Rep [serial online] 2022 [cited 2023 Mar 29];5:130-4. Available from: http://www.ayucare.org/text.asp?2022/5/3/130/357782

  Introduction Top

Hirayama disease (HD) is a juvenile nonprogressive amyotrophy disease. It is characterized by gradual onset of muscular dystrophy in the distal part of the upper limbs related to flexion movements of the cervical spine.[1] The pathogenic mechanism in HD is due to forward displacement of the posterior wall of the lower cervical dural canal when the neck is in flexion, which causes flattening of the lower cervical cord.[2],[3] It is significantly more common in males with the average age of onset as 15–25 years with insidious onset, gradually progressive unilateral muscular weakness, and atrophy affecting forearms and hands.[4] Amyotrophy is unilateral in most patients or asymmetrically bilateral in some patients. Only a few cases of HD with bilateral symmetric involvement have been reported in the literature, hence highlighting the importance of the need of imaging in such patients. Based on the presenting features, Asthimajjagata vata resembles HD. A few symptoms that can be seen in such manifestations are Sankocha (~contraction of body parts), Shosha (~wasting), Spandana (~pulsating sensation), and Bala kshaya (~diminished strength).[5] One such manifestation is visiting the outpatient department and was attempted to treat following the principles of Ayurveda.

  Patient Information Top

A 23-year-old male patient presented with the asymmetrical onset of slowly progressive weakness and atrophy that spontaneously started in the right hand and forearm over the past three years. After one year, it progressed to involve the left hand as well. He complained of pain in the right side of the neck, pain in bilateral knee joints, and tremulousness of both hands for the past one year that gradually progressed to involve both lower limbs during the next six months.

  Clinical Findings Top

On neurological examination, higher mental functions, cerebellar function, and function of other cranial nerves were found normal. Gait was normal. On motor examination, the patient had predominant right-hand thumb clawing with atrophy and weakness of the thenar, hypothenar, and forearm muscles of the right hand [Figure 1], [Figure 2], [Figure 3] and [Table 1]. Power testing was normal in all the muscle groups including both upper limbs and lower limbs except for proximal muscles of the right-hand power were 3 and lower limbs were normal [Table 2]. The assessment of muscle power was noted using knee hammer.[6] Superficial tendon reflexes (corneal, conjunctival, abdominal, and palatal reflexes), deep reflexes (jaw jerk, biceps jerk, triceps jerk, knee jerk, and ankle jerk), and abdominal reflexes were normal. There was no history of sensory involvement. The patient had no difficulty in walking, dysphagia, diplopia, and bowel or bladder involvement. Routine blood investigation reports were within normal limits.
Figure 1: Right-hand arm and forearm showing muscular atrophy as compared to the left hand

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Figure 2: Atrophied right forearm compared to the left hand

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Figure 3: Oblique aspect of the muscle to show the muscle circumference

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Table 1: Circumference of hands

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Table 2: Clinical assessment of muscle power using knee hammer

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  Timeline Top

The timeline of the management is depicted in [Table 3].
Table 3: Timeline of management

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  Diagnostic Assessment Top

Magnetic Resonance Imaging (MRI) cervical spine showed evidence of myelomalacia of hemicord on the right side at C5/C6 levels with anterior displacement of the cervical cord in flexion. On flexion of the neck, the spinal cord is displaced anteriorly with a reduction of anterior thecal space and engorgement of the vessels in the posterior epidural space. The Nerve Conduction Study (NCS) was normal but Electromyography (EMG) showed C7–C8 radiculopathy.

  Therapeutic Intervention Top

Bahya snehana (~external oleation) and Aabhyantara snehana (~internal oleation) are the line of treatment of Asthimajjagata vata vyadhi.[7] In this case, Snehapana (~internal oleation) followed by Virechana (~purgation therapy) was done after an initial course of Deepana (~enhancing metabolic fire) – Pachana (~digestion) [Table 3]. Deepana and Pachana were done with mixing of 3 gm each of Panchakola churna and Nagaramotha churna for four days with lukewarm water before the food twice daily followed by Snehapana with Ashwagandha ghrita. The dose for Snehapana was 50 ml on the 1st day which was increased gradually to 250 ml by the 5th day considering Koshtha of the patient. Features of adequate oleation (~Sneha siddhi) were observed during this period. This was followed by Abhyanga (~external oleation) with Ksheerbala taila and Svedana (~sudation) with Dashamoola kwatha for the next two days and Virechana (~purgation) with Trivrit avaleha (50 gm) and Katuki (Picrorhiza kurrooa Royle) churna (10 gm) with Anupana of Triphala kwatha (150 ml) followed by Samsarjana karma (~dietary regimens) for the next five days [Table 4].
Table 4: Samsarjana karma (specific dietary regimen)

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After Virechana, the procedure of Shalishastika pinda svedana (~sudation with a medicated cooked bolus of rice) was followed for the next eight days. Further, Yoga basti of Anuvasana (Bala Ashwgandhadi taila) and Panchatikta kshira basti was administered for eight days. Completion of these procedures, Rasayana kalpa chikitsa in the form of Kshirapaka processed with four herbs [Table 3] was followed.

  Follow-Up And Outcome Top

After the completion of 40 days of treatment, the patient was reexamined. There was no muscle wasting and no further progression of symptoms was noticed. Improvement in the muscle bulk and strength of the patient was noticed [Table 1] and [Table 2]. MRI cervical spine showed mild posterior bulges at C3–C4 to C6–C7 levels and the thecal sac with no significant neural compression. After the treatment, the EMG report was normal. Treatment was continued for two months altering a few medicines according to symptoms. A follow-up of the patient was taken after one month. There was no improvement in muscle bulk but the power of the right hand increased from 3/5 to 5/5 using knee hammer.

  Discussion Top

HD, a rare neurological disease, is characterized by insidious unilateral or bilateral muscular atrophy and weakness of the forearms and hands, without sensory or pyramidal signs.[8] As per Acharya Sushruta, Dosha ksheena should be treated with Brimhana[9] and for Brimhana various procedures are described in Ayurveda such as Snehana (~the administration of medicated Ghrita), Svedana (~one of the process applied in purification), Shalishastika pinda svedana (a unique bolus massage), Virechana (~therapeutic purgation), Basti (~medicated enema), and Rasayana (~rejuvenation). These procedures are more specifically needed in HD. There exist cervical compression and it can be compared with Sanga and atrophy with Mamsa kshaya. Sneha is beneficial not only to remove Vata anulomana but also to disintegrate the adhered morbid Doshas.[10] Sanga also can be avoided by means of Virechana due to its Srotosudhi properties.[11] By Snehapana and Virechana karma, Srotoshodhana is achieved which helps in bringing down various symptoms of Asthimajjagata vata. After Srotoshodhana and correction of Vata prakopa, proper Dhatu poshana will begin and gradually the atrophy may revert. After Srotomarga shudhi, Dhatu poshana will take place properly resulting in changes from atrophy of muscles to their original state. Similarly, for Brimhana chikitsa, Basti is considered to be the best therapy.

The whole treatment was divided into three steps in this case.

  1. Virechana karma
  2. Panchatikta ksheera basti along with Shalishastika pinda svedana
  3. Rasayana kalpa ksheera pana [Table 3].

Panchakarma is the comprehensive method of internal purification of the body through a multifaceted approach. Snehana, Svedana, and Basti with milk and Ghee processed with Tikta rasa are indicated in bone pathology.[12] Snehapana was given with Ashwagandha ghrita, it contains phospholipids and fatty acids[13] which may have promoted increased protein synthesis that are responsible for the increase in muscle bulk and strength.[14] Ghrita has Yogavahi (~enhancing bioavailability) and Samskar anuvarti (~pharmaceutical modification) properties,[15] which help in delivering the drug to the targeted site. More amyloid proteins are may be found in Ghrita preparation which is believed to interact with the liver protein synthesis activity and thus influence many modular proteins. Basti is called “Ardha chikitsa” (~half of the whole treatment) and some prefer it to say “Sampurna chikitsa” (~the whole treatment).[16] Acharya charaka specified that Asthivaha srotas diseases should be treated by using Tikta rasa pradhana aushadhi dravya (~bitter) along with Ghrita (~ghee) and Ksheera (~milk).[17]

Panchatikta ksheera basti was prepared with the help of Kwatha dravyas – (Guduchi [Tinospora cordifolia (Willd.) Miers.]), Nimba [Azadirachta indica A. Juss], Patola [Trichosanthes dioica Roxb], Vasa [Adhatoda vasica Nees], Kantakari [Solanum surattnse Burm. F], and Kalka dravya – [Putoyavanyadi kalka (30 gm), Guggulu tikta ghrita (90 ml), Kshira (240 ml), Madhu (60 gm), and Saindhava lavana (5gm)]). Kshira is the ingredient that can be used in the Basti preparation as per the condition and Dosha involvement. Thus, it acts as Shodhana as well as Snehana. Kshira basti relieves the Margavarodha and produces Brimhana effect. Guggulu tikta ghrita is mainly indicated in Asthi (~bone tissue) and Majjagata vikaras (~diseases of the bone marrow). It has Snigdha guna and Brimhana and Balya properties. Shalishastika pinda svedana provides nourishment to muscles and bones and it is also thought to help in opening up of blocks during nerve conduction and facilitates remyelination of nerves that could help in transmitting nerve impulses.[18]

Rasayana chikitsa (~rejuvenation) is a unique branch of Ayurveda. It improves nutritional status. The better qualities of Dhatus lead to a series of secondary attributes of Rasayana bestowing longevity, imparting strength, Ojabala, etc.[19] For this purpose, Rasayana drugs such as Ashwagandha (Withania somnifera Linn.),[20] Shatavari (Asparagus racemosus Willd.),[21] Yastimadhu (Glycyrrhiza glabra L.),[22] and Bala (Sida cordifolia L.)[23] were processed in the presence of milk and used. The quantity of herbal mixture was 6 gm on the 1st day, which was added with eight parts of milk and processed. The quantity of the herbal mixture was increased daily by 6 gm until it reached 48 gm. Then, the quantity was reduced by tapering sequentially on a daily basis until it reached 6 gm. The treatment proved satisfactory improvement in relieving symptoms such as improved power of the right hand and muscle bulk in both hands. The patient was followed for 1 month for their recurrence and relapse but there was no relapse and recurrence of such disease was found neither the disease progress. Symptomatic improvement is good enough to draw the attention of the scientific community.

  Conclusion Top

These combined Ayurvedic treatments of oral medications and Panchakarma procedures are helpful in treating the patient presented with HD. This approach may be considered in future HD treatment. Further, works are essential in creating evidence in such management.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2], [Table 3], [Table 4]


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