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CASE REPORT
Year : 2013  |  Volume : 2  |  Issue : 3  |  Page : 203-204

Rare yet treatable: Hypothyroid myopathy (Hoffman's syndrome)


1 Department of Neurology, Guntur Medical College, Guntur, Andhra Pradesh, India
2 Department of General Medicine, Guntur Medical College, Guntur, Andhra Pradesh, India

Date of Web Publication29-Aug-2013

Correspondence Address:
A Sridhar
FF-5 PG Hostel, Government General Hospital, Guntur - 522 001, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-8632.117190

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  Abstract 

Hypothyroid myopathy or Hoffman's syndrome is a rare manifestation of a common condition. It's diagnosis is also difficult. We present a case of Hoffman's disease with predominant proximal myopathy and myotonia.

Keywords: Hoffman′s disease, hypothyroid myopathy, myotonia


How to cite this article:
Sundarachari N V, Sridhar A, Lakshmi VP. Rare yet treatable: Hypothyroid myopathy (Hoffman's syndrome). J NTR Univ Health Sci 2013;2:203-4

How to cite this URL:
Sundarachari N V, Sridhar A, Lakshmi VP. Rare yet treatable: Hypothyroid myopathy (Hoffman's syndrome). J NTR Univ Health Sci [serial online] 2013 [cited 2019 May 20];2:203-4. Available from: http://www.jdrntruhs.org/text.asp?2013/2/3/203/117190


  Introduction Top


Hoffman Syndrome is a rare adult pseudohypertrophic myopathy associated with hypothyroidism, usually autoimmune thyroiditis. These patients present with focal or generalized muscle hypertrophy and other systemic features of hypothyroidism. [1] There have not been many cases reported in India. We report a typical case.


  Case Report Top


A 32-year-old male presented with stiffness of legs and weakness of proximal muscles since 1 year. The patient exhibited sparse hair, dry skin, and calf muscle hypertrophy [Figure 1]. Percussion myotonia [Figure 2] was present in all the limbs. There was delayed relaxation of deep tendon reflexes. Serum thyroid stimulating hormone (TSH) (429.51 μIU/mL) and creatine phosphokinase (CPK) levels (5,360 IU/mL) were elevated. Muscle biopsy showed no inflammation or muscular dystrophy. Nerve conduction velocities were normal. Electromyogram (EMG) showed no evidence of myotonia .
Figure 1: Calf muscle hypertrophy

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Figure 2: Percussion myotonia

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Complete blood counts, lipid profile, and liver and renal function tests were normal. Chest X-ray and electrocardiogram (ECG) were normal. Treatment with 200 μg thyroxin per day showed significant improvement in the patient. The follow-up values of TSH and CPK showed return to normal values [Table 1].
Table 1: Pre and Post Treatment Values of TSH and CPK

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  Discussion Top


Muscle hypertrophy as first presentation of hypothyroidism is quite rare. [2] Our case showed predominantly myopathy with minimal systemic manifestations of hypothyroidism. There are a few cases reported in India. [2],[3] Chopra et al., noticed hypertrophy of almost all muscles of body, especially calf and arm muscles. [4] In our case, there was predominance of arm muscle hypertrophy alone.

Hoffman's disease is a specific, rare form of hypothyroid myopathy, which causes proximal weakness and pseudohypertrophy of muscles, due to connective tissue deposition. [5] Possible mechanisms for myopathy seen in hypothyroidism include an autoimmune reaction affecting the muscle, infiltration by "myxedema", or a disorder of muscle membrane. It seems most likely that thyroid deficiency affects muscle cells directly. Muscular hypertrophy with muscle stiffness in hypothyroidism is seen in less than 10% of cases. [6],[7] A serous infiltration of muscle was mentioned only in occasional case reports and was not seen in the biopsy of patient reported here. Thyroxine is well known to have important effects on cell membranes. The serum enzymes may rise to high levels in hypothyroidism, presumably because thyroid deficiency permits leakage across muscle membranes and actual muscle necrosis. [8]

A very high suspicion of hypothyroid myopathy is necessary to rule out this cause of myopathy. Patients are often misdiagnosed with myotonia, polymyositis, or muscular dystrophies. Stiffness and myoedema on percussion are not features of Duchenne and Becker muscular dystrophy, sarcoidosis, and focal myositis. Histopathology can easily distinguish these disorders. Electric silence is a point in favor of myoedema. It mimicks myotonia on percussion, but myotonic discharges are not seen on EMG.

The key point to stress is the fact that this is a treatable condition and patients with aforementioned conditions should be screened with simple thyroid profile before making the diagnosis of a more treatment resistant condition. Moreover, two or more conditions can sometimes coexist emphasizing the need for thyroid profile.


  Acknowledgements Top


Departments of General Surgery and Pathology, Government General Hospital, Guntur.

 
  References Top

1.Mastaglia FL, Ojeda VJ, Sarnat HB, Kakulas BA. Myopathies associated with hypothyroidism: A review based upon 13 cases. Aust N Z J Med 1988;18:799-806.  Back to cited text no. 1
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2.Rudrajit P, Sinha PK, Mondal P, Banerjee AK. Hoffman syndrome as first manifestation of hypothyroidism: A rare case. JK Science 2011;13:190-2.  Back to cited text no. 2
    
3.Madhu SV, Jain R, Kant S, Prakash V, Kumar V. Myopathy presenting as a sole manifestation of hypothyroidism. J Assoc Physicians India 2010;58:569-70.  Back to cited text no. 3
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4.Chopra BK, Singh G, Puri A. Hoffman's syndrome (hypothyroid myopathy). J Assoc Physicians India 1972;20:387-90.  Back to cited text no. 4
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5.Qureshi W, Hassan G, Khan GQ, Kadri SM, Kak M, Ahmad M, et al. Hoffmann's syndrome: A case report. Ger Med Sci 2005;3:Doc05.  Back to cited text no. 5
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6.Deepak S, Harikrishnan, Jayakumar B. Hypothyroidism presenting as Hoffman's syndrome. J Indian Med Assoc 2004;102:41-2.  Back to cited text no. 6
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7.Udayakumar N, Rameshkumar AC, Srinivasan AV. Hoffmann syndrome: Presentation in hypothyroidism. J Postgrad Med 2005;51:332-3.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Fessel WJ. Myopathy of hypothyroidism. Ann Rheum Dis 1968;27:590-6.  Back to cited text no. 8
[PUBMED]    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]


This article has been cited by
1 Hoffmann Syndrome: An Unusual Cause of Proximal Muscle Hypertrophy
Anees Dudekula,Merwyn Fernandes,Ajit Mahale,Hitakshi Sharma
Research Journal of Radiology. 2016; 4(1): 1
[Pubmed] | [DOI]



 

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