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CASE REPORT
Year : 2012  |  Volume : 2  |  Issue : 4  |  Page : 135-136

Hoffman's syndrome


Department of Medicine, Osmania Medical College, Osmania General Hospital, Hyderabad, Andhra Pradesh, India

Date of Web Publication19-Sep-2014

Correspondence Address:
Dr. Ravala Siddeswari
Department of Medicine, Osmania Medical College, Osmania General Hospital, Hyderabad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2249-4855.141135

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  Abstract 

Hoffman's syndrome is characterized by hypothyroidism with muscle stiffness and pseudo hypertrophy We herewith present a case report of 28 years male with cramps of both lower limbs associated swelling of both calf muscles. Investigations revealed hypothyroidism with elevated TSH and TPO antibodies with focal hypertrophy and hyperplasia of calf muscles on biopsy with normal nerve conduction studies.

Keywords: Hashimotto′s thyroiditis, Hoffman′s syndrome, hypothyroidism, pseudo hypertrophy


How to cite this article:
Siddeswari R. Hoffman's syndrome. J Acad Med Sci 2012;2:135-6

How to cite this URL:
Siddeswari R. Hoffman's syndrome. J Acad Med Sci [serial online] 2012 [cited 2019 May 21];2:135-6. Available from: http://www.e-jams.org/text.asp?2012/2/4/135/141135


  Introduction Top


Hoffman's syndrome (Kocher Debre Semelaigne syndrome) first described in 1897 is characterized by the presence of hypothyroidism with muscle stiffness and pseudo hypertrophy [1],[2],[3] muscular symptoms are common complaints. Increased muscle volume and slowness of contraction constitute the muscular symptoms. The entire musculature is affected to some extent, but the most obvious enlargement in tongue arms and legs. [4] Hashimotto's disease is the most common cause of hypothyroidism in areas of the world in which dietary iodine is sufficient. The mean incidence in women is in the order of 3.5 cases/1000 people/year and in men is 0.8 case/1000 people/year. Autoantibodies to the thyroid-stimulating hormone (TSH) receptor that act as TSH antagonists may be the cause of some cases of the atrophic form of Hashimotto's disease In some patients with Hashimotto's disease may have autoantibodies to thyroglobulin, thyroperoxidase (TPO), and the TSH receptor. Hashimotto's disease is common and may be increasing in frequency. [5]


  Case Report Top


A 28-year-old male patient presented with progressive swelling of calf muscles since 6 months h/o of easy fatigability and difficulty getting up now patient taking help of family members for getting up from the sitting position on clinical examination findings include puffiness of face dry skin pallor sparse hair, bradycardia (52/min), and hypertrophied calf musclesas shown in [Figure 1]a and b. Muscle firm consistency and nontender. On clinical examination, nervous system and cardiovascular system are unremarkable.
Figure 1: (a) Psuedo hypertrophy of calf muscles, (b) Hypertrophied calf muscles

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  Investigations Top


Complete Blood Picture

Hb - 8 g% nomocytic, normochromic anemia, red blood cell - 3.2 cu/mm, white blood cell - 6500 cu/mm. Platelets - 1.7 lakh, clotting time and bleeding time - normal, prothrombin time international normalized ratio - 1.2, complete urine analysis - normal, renal function test with serum electrolytes: Blood urea - 22 mg/dL, serum creatinine - 0.8 mg/dL, serum potassium - 4.2 meq/dL serum sodium - 142 meq/dL, serum calcium- 8.2 mg/dL, serum phosphorus - 3 mg/dL. Serum total proteins - 7.8 g/dL, serum albumin - 4.5 g/dL. Liver functional tests - normal, thyroid profile: TSH - >150 mIU/mL, free T3 - 0.01 ng/mL, free T4 - 0.1 ng/dL.

Anti-microsomal antibodies (TPO) - 113 U/mL (>50 u/mL), thyroid ultrasonography: Hashimotto's thyroiditis.

Ultrasonography abdomen - normal study, X-ray chest pulmonary artery view - normal, electrocardiogram - sinus bradycardia, low voltage complexes, two-dimensional echocardiogram-minimal pericardial effusion, muscle biopsy-focal hypertrophy and hyperplasia of muscles, nerve conduction studies (NCS)-normal.


  Discussion Top


Primary hypothyroidism accounts for 95% of the cases of thyroid insufficiency Hashimotto thyroiditis is the main etiology. Hashimotto's thyroiditis characterized by gradual thyroid failure because of autoimmune destruction of the thyroid gland. This disorder is most prevalent between 45 and 65 years of age and is more common in women than in men, with a female predominance of 10:1-20:1. It is an autoimmune chronic thyroiditis characterized by high levels of antiperoxidase antibodies (anti-TPO) and anti-thyroglobulin, as documented in our case. Anti-thyroglobulin is present in 80-90% of the cases and anti-TPO may be present in 90-100% of the cases. [6]

Hypothyroidism can affect all organs these manifestations are independent of the underlying disorder, but are function degree of hormone deficiency symptoms of the muscular system include stiffness, aching of muscles worsened by cold temperature delayed muscle contraction and relaxation are slowness of movement delayed tendon reflexes muscle enlarged or reduced due to interstitial myxedema muscle mass slightly increased muscle tends to firm rarely profound increase muscle mass with slowness of muscular activity may be predominate manifestation (Kocher Debre Semelaigne syndrome, Hoffmann's syndrome) myoclonus may present. [5]

Electromyogram may be normal or may exhibit disordered discharge hyperirritability and poly-phasic action potentials. Histopathological examination include pale and swollen muscle with the loss of normal striations separated by mucinous deposits and type 1 muscle fibers are predominant [5] in our case NCS are normal.

Microscopic examination of the thyroid gland revealed widespread infiltration of the parenchyma by a mononuclear inflammatory infiltrate containing small lymphocytes, plasma cells, and well-developed germinal centers. The thyroid follicles are atrophic and are lined in many areas by epithelial cells distinguished by the presence of abundant eosinophilic, granular cytoplasm, termed Hürthle, or oxyphil, cells. [6] Suggestive of Hashimotto's thyroiditis.

We are herewith submitting a case report of 25-year-old male with Hoffman's syndrome whose symptoms are improved with thyroxin treatment and his TSH levels 10 mIU/mL after 3 months. Patient is on regular follow-up.

 
  References Top

1.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. 1
    
2.Klein I, Parker M, Shebert R, Ayyar DR, Levey GS. Hypothyroidism presenting as muscle stiffness and pseudohypertrophy: Hoffmann's syndrome. Am J Med 1981;70:891-4.  Back to cited text no. 2
[PUBMED]    
3.Omer S. Hoffmann's syndrome (Hypothyroid myopathy). J Assoc Physicians India 1964;12:353-8.  Back to cited text no. 3
[PUBMED]    
4.Mastropasqua M, Spagna G, Baldini V, Tedesco I, Paggi A. Hoffman's syndrome: Muscle stiffness, pseudohypertrophy and hypothyroidism. Horm Res 2003;59:105-8.  Back to cited text no. 4
    
5.Larsen PR, Davies TF, Hay ID. The thyroid gland. In: Williams Textbook of Endocrinology. 9 th ed. Philadelphia: W.B. Saunders Company; 1998. p. 401-6.  Back to cited text no. 5
    
6.Kumar V, Mitchell R, Abbas AK, Fausto N. Thyroid. In: Robins Basic Pathology. 8 th ed. 2007. p. 758-71.  Back to cited text no. 6
    


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Abstract
Introduction
Case Report
Investigations
Discussion
References
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