Annals of Indian Academy of Neurology
  Users Online: 1763 Home | About the Journal | InstructionsCurrent Issue | Back IssuesLogin      Print this page Email this page  Small font size Default font size Increase font size


 
Table of Contents
CASE REPORT
Year : 2015  |  Volume : 18  |  Issue : 1  |  Page : 115-116
 

Unilateral rubral tremors in Wilson's disease treated with dimercaprol


Department of Neurology, Topiwala National Medical College, Bai Yamunabai Laxman (B. Y. L.) Nair Charitable Hospital, Mumbai, Maharashtra, India

Date of Submission02-Jun-2014
Date of Decision13-Jul-2014
Date of Acceptance23-Sep-2014
Date of Web Publication10-Feb-2015

Correspondence Address:
Rahul T Chakor
Department of Neurology, C. T. Scan Building, Topiwala National Medical College, B. Y. L. Nair Ch Hospital, Mumbai Central, Mumbai - 400 008, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.144286

Rights and Permissions

 

   Abstract 

Tremors are reported as the most frequent neurological manifestation of Wilson's disease (WD) in some series. Postural tremors, rest tremors, action tremors and wing-beating (rubral) tremors are the different types of tremors seen in WD. We report a patient of WD with unilateral rubral tremors refractory to 1-year therapy with Penicillamine and anti-tremor medications. The tremors decreased considerably after adding chelation therapy with dimercaprol. Combination of Penicillamine and dimercaprol is an effective decoppering measure in rubral tremors of WD.


Keywords: Wilson′s disease, BAL, dimercaprol, chelation, rubral tremors


How to cite this article:
Chakor RT, Bharote H, Eklare N, Tamboli K. Unilateral rubral tremors in Wilson's disease treated with dimercaprol. Ann Indian Acad Neurol 2015;18:115-6

How to cite this URL:
Chakor RT, Bharote H, Eklare N, Tamboli K. Unilateral rubral tremors in Wilson's disease treated with dimercaprol. Ann Indian Acad Neurol [serial online] 2015 [cited 2019 Aug 22];18:115-6. Available from: http://www.annalsofian.org/text.asp?2015/18/1/115/144286



   Introduction Top


Tremors, dysarthria, dystonia,  Parkinsonism More Details and gait disturbances are the common neurological manifestations of Wilson's disease (WD). In some series tremors are reported as the most frequent neurological manifestation of WD [1] Postural tremors, rest tremors, action tremors and wing-beating (rubral) tremors are the different types of tremors seen in WD. [1],[2] We report a patient of WD with unilateral rubral tremors refractory to Penicillamine and anti-tremor medications. The tremors decreased considerably after chelation therapy with dimercaprol.


   Case Report Top


A 22-year-old right-handed man working in a motor rewinding company presented with tremor of right upper limb since 1 year. The tremor began distally in the right upper limb and progressed over 2 months to involve wrist and elbow. The tremors gradually increased in severity over the next few months and he was unable to work. Examination revealed a Kayser Fleischer (K-F) ring. There were high amplitude postural tremors with flexion extension at wrist. Tremor markedly exacerbated on outstretching of hands and action. In the shoulder abducted and elbow flexed posture wing-beating tremors with upward and downward movement of forearm were seen. The tremors disappeared completely at rest [see video 1 segment 1].

Serum ceruloplasmin levels were 5.16 mg/dl (10-20 mg/dl). Twenty-four-hour urinary copper levels were 756 μg. Magnetic resonance imaging scan of brain showed typical features of Wilson's disease [see [Figure 1]].
Figure 1: Axial T2 image of MRI Brain showing face of giant panda sign typical of Wilson's disease

Click here to view


He was started on chelation therapy with 250 mg of D-Penicillamine twice a day. Zinc was given in the dose of 200 mg three times a day. Penicillamine was gradually increased to 2 g/day in two divided doses administered in fasting and continued for 1 year. Compliance was closely monitored. Symptomatic anti-tremor therapy with clonazepam, primidone, trihexphenidyl was given. One year after chelation therapy with Penicillamine and symptomatic therapy the tremors persisted. Hence it was decided to give alternative chelation therapy. British antilewisite (BAL, 2, 3 dimercaprol) in dose of 5 mg/kg bolus dose followed by 2.5 mg/kg for 14 days was given intramuscularly along with penicillamine. One month later tremors reduced significantly and the patient was able to function normally [see video 1 segment 2].


   Discussion Top


Rubral tremors in WD can be refractory to chelation with penicillamine and symptomatic anti-tremor treatment. Our patient received penicillamine 2 g/day for 1 year. Tremors persisted after 1 year of penicillamine therapy and he could not function due to the tremors. Hence we decided to use alternative chelation therapy. Trientine, tetrathiomolybdate, dimercaprol are other chelation therapies for WD. [3] Trientine and tetrathiomolybdate are not freely available in India. Hence a combination of dimercaprol which is easily available and penicillamine was given. After a 2-week course of dimercaprol the tremors decreased significantly. Penicillamine in the dose of 1 g/day was continued after dimercaprol therapy. He has minimal tremors and is able to work at 3 months of follow-up after dimercaprol therapy.

Dimercaprol was designed by a team of workers in Oxford as an antidote to arsenical war gas lewisite. Cumings hypothesized that BAL might arrest the progress of WD. [3],[4] Therapy with dimercaprol is effective but the deep intramuscular injections are painful, may not be tolerated and with repeated courses the chelating effect decreases. Hence, oral-chelating agent penicillamine which is well tolerated and effective is favored. Adverse effects with penicillamine though uncommon are serious like SLE and nephropathy. Some patients continue to progress on penicillamine and in these patients a course with dimercaprol can induce remission of symptoms. Intensive therapy with dimercaprol has been used in severely dystonic patients of WD. Conventional chelation with penicillamine can then be continued. Thalamotomy and deep brain stimulation (DBS) have been tried in cases of refractory tremors in WD. A course of dimercaprol with penicillamine is much cheaper than and as effective as thalamotomy or DBS.


   Conclusions Top


Wing-beating tremors in WD may be refractory to Penicillamine therapy. Combination of Penicillamine and dimercaprol is effective in rubral tremors of WD.


   Acknowledgement Top


Dean, T. N. Medical College, B. Y. L. Nair Ch Hospital, Mumbai Central, Mumbai 400 008.

 
   References Top

1.
Prashanth LK, Taly AB, Sinha S, Arunodaya GR, Swamy HS. Wilson′s disease: Diagnostic errors and clinical implications. J Neurol Neurosurg Psychiatry 2004;75:907-9.  Back to cited text no. 1
    
2.
Machado A, Chien HF, Deguti MM, Cançado E, Azevedo RS, Scaff M, et al. Neurological manifestations in Wilson′s disease: Report of 119 cases. Mov Disord 2006;21:2192-6.  Back to cited text no. 2
    
3.
Walshe JM. The conquest of Wilson′s disease. Brain 2009;132: 2289-95.  Back to cited text no. 3
    
4.
Cumings JN. The effect of B.A.L. in hepatolenticular degeneration. Brain 1951;74:10-22.  Back to cited text no. 4
    


    Figures

  [Figure 1]



 

Top
Print this article  Email this article

    

 
   Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (480 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Conclusions
   Acknowledgement
    References
    Article Figures

 Article Access Statistics
    Viewed1935    
    Printed34    
    Emailed0    
    PDF Downloaded60    
    Comments [Add]    

Recommend this journal