• Users Online: 32
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 15  |  Issue : 3  |  Page : 228-231

Hirayama disease: An ayurvedic approach


1 Department of Kayachikitsa, Danigond Post Graduation Centre, SDM Trust's Ayurvedic Medical College, Terdal, Karnataka, India
2 Department of Kayachikitsa - Rasayana Vajeekarana, KAHER's Shri BMK Ayurveda Mahavidyalaya Post Graduate Studies and Research Centre, Belagavi, Karnataka, India
3 Department of Kayachikitsa, SBG Ayurvedic Medical College and Hospital, Belagavi, Karnataka, India
4 Department of Kayachikitsa, SBSS Krishna Ayurvedic Medical College and Hospital, Sankeshwar, Karnataka, India

Date of Submission05-Oct-2020
Date of Decision03-Dec-2020
Date of Acceptance05-Dec-2020
Date of Web Publication25-Sep-2021

Correspondence Address:
Swarda Ravindra Uppin
Department of Kayachikitsa - Rasayana Vajeekarana, KAHER's Shri BMK Ayurveda Mahavidyalaya Post Graduate Studies and Research Centre, Shahapur, Belagavi, Karnataka
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/joa.joa_196_20

Rights and Permissions
  Abstract 


Introduction: Hirayama disease, also known as Monomelic amyotrophy or benign focal amyotrophy, is a rare motor neuron disease. It is reported to primarily affect males of southeast Asia, particularly India and Japan. Clinical findings and Intervention: A 27 years male patient approached Ayurveda OPD with a known case of Hirayama Disease from last 9 years. Considering this condition, the treatment was planned according to Ayurvedic Medicinal system which included courses of Nasya karma, Basti karma and Shamana aushadi. Outcome: Post treatment, the patient showed considerable clinical improvement, and arrest in disease progression was observed. Conclusion: The present condition is understood under the spectrum of Vata vyadhi and was diagnosed as a case of Viswachi. The treatment was focused upon pacifying vata Dosha and restoring strength and functional capacity.

Keywords: Ayurvedic approach, Hirayama disease, neuralgic amyotrophy, Viswachi


How to cite this article:
Nandigoudar SN, Uppin SR, Dalawai A, Bhagaje N. Hirayama disease: An ayurvedic approach. J Ayurveda 2021;15:228-31

How to cite this URL:
Nandigoudar SN, Uppin SR, Dalawai A, Bhagaje N. Hirayama disease: An ayurvedic approach. J Ayurveda [serial online] 2021 [cited 2021 Oct 21];15:228-31. Available from: http://www.journayu.in/text.asp?2021/15/3/228/326708




  Introduction Top


Hirayama disease, also known as monomelic amyotrophy or benign focal amyotrophy, is a rare motor neuron disease which was first described in 1959 in Japan by Hirayama et al.[1],[2] It is reported to primarily affect males of Southeast Asia, particularly India and Japan.[3] The symptoms usually appear during the second or third decade of life.[4] The characteristic features as originally described by Hirayama include:[5]

  1. Weakness and wasting predominantly in C7, C8, and T1 myotomes of unilateral upper limb or asymmetrically in bilateral upper limbs with sparing of brachioradialis
  2. Insidious onset in teens or early twenties
  3. Progression of 1 to 3 years followed by arrest of disease or relatively benign course
  4. Irregular-course tremors (minipolymyoclonus) in the fingers of the affected hand(s)
  5. Mild transient worsening of symptoms on exposure to cold
  6. Electromyography evidence of chronic denervation in the clinically or subclinically affected muscles
  7. The absence of substantial sensory loss/reflex abnormalities, cranial nerve, pyramidal tract in the lower limb, sphincter, or cerebellar deficits.


The disability originates with impaired functioning of the anterior horn cells of the lower cervical cord.[6] Researches reveal that the contributing factors are the forward displacement of the cervical dural sac and compressive flattening of the lower cervical cord during neck flexion.[7] Magnetic resonance imaging (MRI) cervical spine in flexion would reveal the cardinal features of Hirayama disease.[8]


  Case Report Top


Here, we report a case of Hirayama disease that approached and underwent Ayurveda management.

Personal history

A 27-year-old software engineer, with a history of keyboard typing and bike rides for long hours, had a relevant clinical history of frequent episodes of migraine and sinusitis, occasionally followed by pain at the cervical region since the age of 12 years, and approached the Ayurveda Outpatient Department, at KLE Ayurveda Hospital and Medical Research Centre, Belagavi, Karnataka.

Medical history

Previously diagnosed as a case of Hirayama disease, the patient presented with a history of gradual onset of weakness and tremors in his right hand since the age of 18 years. The condition progressed over the past 9 years to generate the same symptoms in the left hand and in addition, he developed wasting at the right tríceps muscle. Three months ago, while performing aerobics, he suddenly developed drooping of the right ring finger along with loss of sensation in the same finger. The symptoms were observed to aggravate on exposure to cold environment. In addition, occasional pain at the cervical region radiating to the left upper limb was reported. During his course of evaluation and treatment at contemporary health care, he underwent a series of investigations wherein his Vitamin B12 level was 123 pg/mL and Vitamin D level was 15.21 ng/mL, which were found to be low. Electromyogram study conducted in July 2016 showed low amplitude of bilateral median and ulnar nerves. MRI of the flexed cervical spine performed in July 2016 revealed that on sagittal sequences, the normal cervical lordosis seemed to be altered. Incidentally, Schmorl's impressions were noted along contiguous disc surface of the C7–T1 level. The posterior subarachnoid space at C2–C3 levels appeared reduced in comparison to the anterior space, in neural and flexion positions. In addition, on flexion images, the C5–C6 intervertebral disc and posterosuperior aspects of C6 vertebra were seen contacting the anterior cord silhouette, thus confirming focal cord T2 hyperintense signals at C5–C6 level and C4–C5 and C5–C6 posterior disc protrusions, causing minimal thecal compressions.

  • Thus, he was diagnosed as a case of Hirayama disease, and was advised to undergo surgery. The patient then approached Ayurveda consultation for conservative line of management.


Physical examination

On physical examination, he was conscious and well oriented to person, place, and time. In comparison to the left hand, more tremors were present in the right hand with reduced palmar grasp. There was reduced sensation at the right ring finger along with minipolymyoclonus. Wasting along with mild fasciculation was observed at the right tríceps muscle. Deep tendon reflexes were found to be diminished in the right upper limb.

Treatment

Based on his clinical and radiological findings, he was diagnosed as a case of Viswachi (neuralgic amyotrophy) and was put on Ayurveda line of treatment.

Classical ayurvedic therapeutic procedures were administered through nasal and rectal routes internally, and few external procedures were carried out in order to strengthen the spinal cord (nervous system). During the first course, Nasya Karma (Errhine therapy), that is, nasal instillation of medicated oil, was administered for a period of 7 days. For the initial 3 days, Shodhana nasya (to achieve purificatory effect) was administered using Shadbindu taila and for the next 4 days, Brinhana nasya was administered using Ksheerabala taila (101), whereas during the second course, Basti Karma (enema therapy), that is, medications through rectal route, was the focus of the treatment. External procedures such as Sarvanga abhyanga (whole-body oil massage), Prishtha basti (retaining medicated oil on back), Patra pinda sweda (hot sudation with herbal leaves), and Dashanga lepa (application of medicated paste) were performed for supportive symptomatic relief. The details of the treatment are summarized in [Table 1], [Table 2], [Table 3], [Table 4].
Table 1: The Treatment schedule for 7 days during Course 1:

Click here to view
Table 2: The Treatment schedule for 7 days during Course 2

Click here to view
Table 3: Enlisting the Oral medications during the entire course of treatment including follow-ups

Click here to view
Table 4: Enlisting the oral medications during the entire course of treatment including follow-ups

Click here to view


Progression

The patient is under monitoring through annual visits and regular phone call conversations. After 3 years of follow-up, the patient reported 30% relief with no aggravation of symptoms. He was asked to take the opinion of a neuro-physician who stated that there was no further progression of the disease.


  Discussion Top


Hirayama disease is a self-limiting condition diagnosed through flexion MRI. The condition is observed to exhibit with wide presentation in different individuals.[5] In Ayurveda, the present condition can be dealt under the spectrum of Vata vyadhi (disorders of nervous system) and was diagnosed as a case of Viswachi (neuralgic amyotrophy), wherein the pain evolves from the nape of the neck (manya) and radiates to either of the bilateral upper limbs. Assessing the chronicity of the condition, multiple courses of treatment were planned with the expectation of obtaining maximum relief.

During the first course, Nasya Karma (Errhine therapy), that is, nasal instillation of medicated oil, was administered as the prime management, as indicated in Viswachi to achieve target action. It has been well established that the mucus membrane of the nose is highly vascular and absorption through it is directly into the cerebrospinal fluid via inferior ophthalmic veins.[9] The networks of nasal blood and lymph vessels stimulate the nerve endings of the olfactory and trigeminal nerves and impulses are transmitted to the central nervous system (CNS). Researchers show that the blood–brain barrier is highly permeable for lipid substances[10] and thus, the medicated oil passes this barrier to exert its action on CNS and strengthens the nervous pathway.

As Viswachi is one among the Vatavyadhi, the second course of treatment was planned to pacify Vata Dosha, that is, Basti Karma (administration of medicated enema through rectal route). Recent advancements suggest the Gut Brain theory,[11] wherein the stimulation of gastrointestinal tract activates certain aspects of the CNS and thus exhibits its action. In addition, its ability to by-pass the first-pass effect[12] and absorb directly into the systemic circulation results in pacifying the Vata Dosha and strengthens majja dhatu (bone marrow), thereby reducing the symptoms such as tingling, pain, and weakness.

External procedures such as Sarvanga abhyanga (whole-body oil massage), Prishtha basti (retaining medicated oil on back), Patra pinda sweda (hot sudation with herbal leaves), and Dashanga lepa (application of medicated paste) contain drugs that are fat and water soluble. These medications when rubbed over the skin surface enhance the micro-circulation (blood plasma), thereby strengthening the local tissues and aiding in relieving symptoms.

The herbo-mineral preparations when administered orally help in nerve stimulation, prevent neuronal degeneration, re-establish micro-circulation, relieve muscle stiffness, and improve neuro-muscular fine co-ordination. Ashwagandha (Withania somnifera) in particular is proven to contain phytosteroidal properties that aid in preventing the myelin sheath discontinuation.[13] Further, Rajata bhasma and Abhraka bhasma (nano particles of calcined argentum and mica) are excellent brain nutrients in preventing spinal and cerebral atrophy.


  Conclusion Top


The case presented above was observed to progress for a period of 9 years. Although the radiological findings showed slight variation in the presentation of typical features, the clinical features suggested it as more of Hirayama disease. The patient showed good clinical improvement with stabilization of the condition on receiving Ayurveda line of management. Hirayama disease is a less common disorder and thus, patients approaching Ayurveda center are still rare. In this case, we have made an effort to frame a treatment protocol to manage the condition through Ayurveda.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.





 
  References Top

1.
Gourie-Devi M. Monomelic Amyotrophy of upper or lower limbs. In: Wisen AA, Shaw PJ, editors. Handbook of Clinical Neurology. BV: Elsevier; 2007. p. 207-27.  Back to cited text no. 1
    
2.
Pal PK, Atchayaram N, Goel G, Beulah E. Central motor conduction in brachial monomelic amyotrophy. Neurol India 2008;56:438-43.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Narayana Gowda BS, Mohan Kumar J, Basim PK. Hirayama's disease – A rare case report with review of literature. J Orthop Case Rep 2013;3:11-4.  Back to cited text no. 3
    
4.
Alkan G, Akgol G, Gulkesen A, Kaya A. Hirayama Disease (monomelic amytrophy). Medicine Science International Medical Journal 2017;6:560-1.  Back to cited text no. 4
    
5.
Panchal M, Sharma C, Sharma AK. Hirayama Disease: Study of clinical, electrophysiological & radiological characteristics at tertiary care centre from north west India. Indian Journal of Medical Specialities. Elsevier 9(2018):20-2.  Back to cited text no. 5
    
6.
Polo A, Curro' Dossi M, Fiaschi A, Zanette GP, Rizzuto N. Peripheral and segmental spinal abnormalities of median and ulnar somatosensory evoked potentials in Hirayama's disease. J Neurol Neurosurg Psychiatry 2003;74:627-32.  Back to cited text no. 6
    
7.
Hirayama K, Tokumaru Y. Cervical dural sac and spinal cord in juvenile muscular atrophy of distal upper extremity. Neurology 2000;54:1922-6.  Back to cited text no. 7
    
8.
Mahajan SK, Kaushik M, Raina R, Sharath Babu NM, Raghav S. Hirayama Disease - A Variant of Motor Neuron Disease and Role of Flexion MRI in Diagnosis. Online J Health Allied Scs 2013;12:16.  Back to cited text no. 8
    
9.
Khurana P, Pareek T, Saroch V, Pareek RK. Role of kukkutanda swedna and nasya in the management of Ardita – A pilot study. Int J Ayurvedic Herb Med 2014;4:1602-7.  Back to cited text no. 9
    
10.
Das B, Ganesh RM, Mishra PK, Bhuyan G. A study on Apabahuka (frozen shoulder) and its management by Laghumasha taila nasya, AYU Oct-Dec 2010;31:488-94.  Back to cited text no. 10
    
11.
Shukla GD. Pharmacodynamic understanding of Basti A contemporary approach. Int J Pharm Biol Arch 2012;3:89.  Back to cited text no. 11
    
12.
Biradar S, Grampurohit PL, Wasedar VS. Ayurveda exploration of pharmacokinetics of sneha basti - review article, IAMJ march 2018;6:607-10.  Back to cited text no. 12
    
13.
Manjunath MJ, Muralidhara, Standardized extract of Withania somnifera (Ashwagandha) markedly offsets rotenone-induced locomotor deficits, oxidative impairments and neurotoxicity in Drosophilia melanogaster. J Food Sci Technol 52, 1971-1981 (2015).  Back to cited text no. 13
    



 
 
    Tables

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



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Tables

 Article Access Statistics
    Viewed95    
    Printed0    
    Emailed0    
    PDF Downloaded26    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]