A Case of Asymptomatic Inclusion Body Myositis

Bello, Rey; Bertorini, Tulio; Ganta, Kartheek; Mays, William

doi:10.1097/cnd.0000000000000142

Cited by 5 publications

(6 citation statements)

References 11 publications

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“…In most cases we saw myofiber atrophy with subsequent invasion of connective and adipose tissue. The results are in accordance with observations made by others [12,17,27,36,37]. In a few cases hypertrophic myofibers in IBM patients were also reported [12,27].…”

Section: Discussionsupporting

confidence: 93%

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Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series

et al. 2018

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Sporadic inclusion body myositis (s-IBM) is a progressive, skeletal muscle disease with poor prognosis. However, establishing the final diagnosis is difficult because of the lack of clear biomarkers in the blood serum and very slow development of clinical symptoms. Moreover, most other organs function normally without any disturbance. Here, in patients with this untreatable disease, we have underlined the importance of immunohistochemical and ultrastructural assessment of skeletal muscle in patients diagnosed with s-IBM. The goal of this study was to identify the distribution of specific antigens and to determine morphological features in order to localize pathological protein aggregates, rimmed vacuoles, and loss of myofibrils, which are key elements in the diagnosis of s-IBM. All studied patients were between 48 and 83 years of age and were hospitalized in the Department of Rheumatology and Internal Medicine between 2011 and 2016. Anamneses revealed an accelerated progression of muscle atrophy, weakness of limb muscles, and difficulties with climbing stairs. Based on histopathology and transmission electron microscopy examination, inflammatory infiltrations consisting of mononuclear cells, severe atrophy and focal necrosis of myofibers, splitting of myofilaments, myelinoid bodies and rimmed vacuoles were observed. Primary antibodies directed against CD3, CD8, CD68, cN1A, beta-amyloid, Tau protein and apolipoprotein B made it possible to identify types of cells within infiltrations as well as the protein deposits within myofibers. Using a combination of immunohistochemistry and electron microscopy methods, we were able to establish the correct final diagnosis and to implement a specific treatment to inhibit disease progression.

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Section: Discussionsupporting

confidence: 93%

“…As is well known, serum CK and LDH levels increase during skeletal muscle damage and degeneration. Therefore, a muscle biopsy in every case where IBM is suspected is essential for a definitive diagnosis even if there are no features of muscle weakness according to the MRC scale [13,26,35,36].…”

Section: Discussionmentioning

confidence: 99%

Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series

et al. 2018

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“…HyperCKemia with or without minimal symptoms constituted the second most common subgroup among atypical IBM patients. Although only two cases of IBM presenting with asymptomatic hyperCKemia were previously reported, 30,31 this group is probably underestimated. It is noteworthy that the concept of prodromal disease is increasingly recognized in other neurological disorders 32 ; such view serves earlier interventions in therapeutic trials.…”

Section: Discussionmentioning

confidence: 92%

“…Although only two cases of IBM presenting with asymptomatic hyperCKemia were previously reported, 30,31 this group is probably underestimated. It is noteworthy that the concept of prodromal disease is increasingly recognized in other neurological disorders 32 cases have been reported.…”

Section: Discussionmentioning

confidence: 92%

Atypical presentations of inclusion body myositis: Clinical characteristics and long‐term outcomes

2022

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Introductions/Aims Inclusion body myositis (IBM) typically presents with progressive weakness preferentially involving finger flexors and quadriceps. Atypical presentations have been less commonly reported. Here, we aim to describe the clinical characteristics and long‐term outcomes of IBM patients with atypical presentations. Methods We retrospectively searched the Mayo Clinic medical records to identify IBM patients with atypical disease onset, seen between 2015 and 2020. Results We identified 357 IBM patients, of whom 50 (14%) had an atypical presentation. Thirty‐eight patients were diagnosed with IBM because they fulfilled one of the European Neuromuscular Center diagnostic categories at a later stage, 10 had all IBM histopathological features, and 2 were diagnosed on the basis of clinical and laboratory data. The most common presentation was dysphagia (50%), followed by asymptomatic hyperCKemia (24%; CK, creatine kinase), then foot drop (12%). 6% of patients presented with proximal arm weakness, 4% with axial weakness and 4% with facial diplegia. Median time from symptom onset to diagnosis was 9 y. Median age at diagnosis was 70.5 y. 16% of patients needed a walking aid. When tested, 86.5% of patients had impaired swallowing and 56% had elevated cytosolic nucleotidase‐1A antibodies. Only 1/26 patients who received immunotherapy had minimal improvement. Upon follow‐up, most patients had generalization of their weakness with a decline in their strength summated score of 0.082/mo. Discussion A significant proportion of IBM patients may have an atypical presentation. Recognition of such heterogeneity could improve early diagnosis, prevent unnecessary immunotherapy, and provide insight for future diagnostic criteria development and clinical trials.

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“…Asymptomatic presentation of sIBM is rare. 6 Electromyography (EMG) can be used to exclude typical causes of neurogenic disorders and to classify the disease into the group of muscle damage; however, it cannot accurately extract its cause (myositis, toxic or dystrophic myopathic processes). 5 Therefore, muscle biopsy with histopathologic, immunohistochemical and microscopical examination remains the best method for diagnosing sIBM.…”

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confidence: 99%

Challenges in diagnosis and treatment of sporadic inclusion-body myositis

et al. 2018

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Sporadic inclusion body myositis (sIBM) is a rare yet increasingly prevalent disease and the most common cause of inflammatory myopathy in people over the age of 50. The exact cause of the disorder is unknown. In sIBM 2 processes, first autoimmune and the other degenerative, parallelly occur in the muscle cells. The inflammation aspect is characterized by the cloning of T cells that appear to be driven by specific antigens to invade muscle fibers. The degeneration aspect is characterized by the appearance of holes in the muscle cell vacuoles, deposits of abnormal proteins within the cells and in filamentous inclusions. The disease has a major impact on patients' motor functionality and their quality of life. The treatment of sIBM still remains a major challenge. Early diagnosis of sIBM (already at the histopathology stage), when one still cannot observe fully developed clinical symptoms, may stop help to the progression of the disease.

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A Case of Asymptomatic Inclusion Body Myositis

Abstract: Asymptomatic hyperCKemia should be investigated and followed closely for definitive diagnosis and possible treatable causes.

Cited by 5 publications

References 11 publications

Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series

Immunohistochemical and ultrastructural analysis of sporadic inclusion body myositis: a case series

Atypical presentations of inclusion body myositis: Clinical characteristics and long‐term outcomes

Challenges in diagnosis and treatment of sporadic inclusion-body myositis

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