Introduction: Amyloidosis features protein deposition in the organs and tissues and has been associated with carpal tunnel syndrome CTS when it occurs in the wrist. The objective is to describe a case series of patients undergoing surgery for CTS associated with amyloidosis. Methods: The study included 12 patients who underwent surgery to treat CTS in whom amyloidosis was proven by biopsy; the follow-up period was 5 years. The patients were evaluated by clinical tests, electroneuromyography, radiological images, and biopsy. Results: All patients presented with musculoskeletal complaints, severe symptoms of median nerve compression, and changes on neurophysiological tests. Surgery, synovectomy, and biopsy were performed.
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The diagnosis of Graves' orbitopathy is usually straightforward. However, orbital diseases that mimick some clinical signs of Graves' orbitopathy may cause diagnostic confusion, particularly when associated to some form of thyroid dysfunction.
This report describes the rare occurrence of localized inferior rectus muscle amyloidosis in a patient with autoimmune hypothyroidism, who was misdiagnosed as Graves' orbitopathy. A year-old man complained of painless progressive proptosis on the left side and intermittent vertical diplopia for 6 months. The diagnosis of Graves' orbitopathy was entertained after magnetic resonance imaging revealing a markedly enlarged, tendon-sparing inferior rectus enlargement on the left side, and an autoimmune hypothyroidism was disclosed on systemic medical workup.
After no clinical improvement with treatment, the patient was referred to an ophthalmologist and further investigation was performed.
The presence of calcification in the inferior rectus muscle on computed tomography, associated with the clinical findings led to a diagnostic biopsy, which revealed amyloid deposition. This report emphasizes that a careful evaluation of atypical forms of Graves' orbitopathy may be crucial and should include, yet with rare occurrence, amyloidosis in its differential diagnosis. Figure 1. Photographs at presentation showing left lower eyelid bag swelling, left proptosis and extraocular….
Figure 2. Coronal T1-weighted gadolinium-enhanced magnetic resonance imaging of the orbits showing marked hyperintense enlarged…. Figure 3. Computed tomography scans of the orbits show enlarged left inferior rectus muscle with….
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Full-text links Cite Favorites. Abstract in English , Portuguese. Figures Figure 1. Photographs at presentation showing left…. Coronal T1-weighted gadolinium-enhanced magnetic resonance…. Computed tomography scans of the…. Histopathological study of the inferior…. See this image and copyright information in PMC. Similar articles Hard metal lung disease: a case series. Mizutani RF, et al. J Bras Pneumol. Chronic polyarthritis as the first manifestation of childhood systemic polyarteritis nodosa.
Novak GV, et al. Einstein Sao Paulo. Mesquita ET, et al. Arq Bras Cardiol. Epub Jun Performance of apparent diffusion coefficient of medial and lateral rectus muscles in Graves' orbitopathy. Abdel Razek AA, et al. Neuroradiol J. Epub Apr 5. The cerebrospinal fluid in multiple sclerosis: far beyond the bands. Domingues RB, et al.
Show more similar articles See all similar articles. Turck N, et al. Dis Markers. The pathophysiology of thyroid eye disease.
J Neuroophthalmol. Am J Ophthalmol. Am J Med Sci. Unilateral proptosis: the role of medical history. Br J Ophthalmol. Sao Paulo: Publication types Case Reports Actions. Biopsy Actions. Humans Actions. Immunoglobulin Light-chain Amyloidosis Actions. Male Actions. Middle Aged Actions. Tomography, X-Ray Computed Actions.
Supplementary concepts Hypothyroidism, Autoimmune Actions. Copy Download.
Identificou-se que raramente ocorre em pacientes abaixo dos 70 anos. Diante disso propomos um fluxograma norteador para o manejo destes pacientes Figuras 2 e 3. TTR: transtirretina. A medida da espessura do septo interventricular pode sugerir o tipo de amiloidose presente no paciente, sendo frequentemente maior nos casos de amiloidose por TTR do que a forma AL, podendo em muitos casos ser superior a 20 mm. Trends in prevalence and outcome of heart failure with preserved ejection fraction.
Isolated Primary Amyloidosis of the Inferior Rectus Muscle Mimicking Graves' Orbitopathy
Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis AL : a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, April Am J Hematol. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol. MAG is also an author of several references cited in this monograph. DR has been reimbursed by Millennium Pharmaceuticals, Inc and Johnson and Johnson, the manufacturers of bortezomib, for attending several conferences, for speaking at educational meetings, and for consulting work.
Mueller OS. Kyle RA. Amyloidosis: a convoluted story. Br J Haematol. Rev Bras Reumatol. Significance of SAA1. Merline G, Bellotti V.