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Autoimmune conditions
Published in Ibrahim Natalwala, Ammar Natalwala, E Glucksman, MCQs in Neurology and Neurosurgery for Medical Students, 2022
Ibrahim Natalwala, Ammar Natalwala, E Glucksman
Small cell lung carcinoma can produce a range of paraneoplastic syndromes, of which LEMS would be the most likely in this case. This is due to the proximal muscle weakness that Angela is experiencing.2 LEMS is an autoimmune disorder in which antibodies are directed against the presynaptic voltage-gated calcium channels. This results in a failure to release enough acetylcholine at the neuromuscular junction. In MG, it is the postsynaptic acetylcholine receptors that are affected. Therefore, clinically patients with LEMS and MG both experience muscle weakness. The difference, is that in LEMS sustained muscle effort leads to improvement in weakness whereas in MG prolonged effort is very difficult as fatigue progressively worsens. Trunk and legs are most often affected in LEMS, autonomic dysfunction can occur and hyporeflexia is typical on neurological examination.3 Polymyositis is an inflammatory condition which predominantly causes pain and weakness in the proximal muscles. Patients will have a raised creatine kinase. Diagnosis is usually confirmed using electromyography and muscle biopsy. Mononeuritis multiplex would present with a painful, asymmetrical, asynchronous sensory and motor peripheral neuropathy involving isolated damage to at least two separate nerve areas.4
Selected topics
Published in Henry J. Woodford, Essential Geriatrics, 2022
Polymyositis causes a slowly progressive, symmetrical, proximal weakness. It is usually painless. It may be associated with dysphagia, polyarthritis or arthralgia. There can also be cardiopulmonary involvement causing pneumonitis, conduction defects, pericarditis or myocarditis. A number of people will have another underlying condition. Around 20% have a connective tissue disease (e.g. systemic lupus erythematosus or rheumatoid arthritis). It is more common in women than men. Dermatomyositis causes a rash in addition to muscular symptoms and has a more frequent association with malignancy. Gottron's papules are areas of macular erythematous scaling over the knuckles and extensor surfaces of the knees and elbows. It can also cause a heliotrope periorbital rash, or less commonly macular erythema over the anterior chest or shoulders. It may be associated with malignancy, especially in older people, most commonly ovarian, gastrointestinal, lung or breast cancer.69
Respiratory
Published in Kristen Davies, Shadaba Ahmed, Core Conditions for Medical and Surgical Finals, 2020
Polymyositis: Inflammatory muscle disorder that is either idiopathic or associated with other connective tissue diseases or malignancy. Features include proximal muscle weakness, Raynaud phenomenon and ILD. Investigations show a ↑ CK and positive anti-Jo1 antibodies. Dermatomyositis is a variant that involves the skin, manifesting in purple papules on the knuckles (Gottron) and a purple heliotrope rash around the eyes.
Clinical pearls and promising therapies in myositis
Published in Expert Review of Clinical Immunology, 2023
Caoilfhionn M. Connolly, Julie J. Paik
IMNM is a distinct subgroup of inflammatory myopathies, characterized by significant muscle involvement. In the past, these patients were most commonly diagnosed as ‘polymyositis’ given the absence of skin involvement. Now, there are two MSAs associated with IMNM including anti-signal recognition particle (anti-SRP) and anti-3-hydroxy 3-methylglutaryl-coA reductase (anti-HMGCR). Some IMNM patients do not have an identified MSA (also known as seronegative IMNM) [82]. Patients most often report myalgias and weakness [83,84], which can have an acute (<6 weeks) or subacute (<6 months) onset [82]. Disease can be rapidly progressive and difficult to treat due to poor response to immunosuppressive therapies [82]. Indeed, the acuity and severity of muscle involvement, as well as refractory nature to immunosuppression, can result in early debility and long-standing deficits. Weakness can often be severe at the time of presentation, and patients with IMNM often have dominant proximal lower extremity weakness and universally elevated CK levels [84–86].
Rare forms of inflammatory myopathies – part I, generalized forms
Published in Expert Review of Clinical Immunology, 2023
Claudio Galluzzo, Ilaria Chiapparoli, Ada Corrado, Francesco Paolo Cantatore, Carlo Salvarani, Nicolò Pipitone
Muscle biopsies from patients with myositis show a higher percentage (mean 1.2%) of cytochrome oxidase (COX) – negative muscle fibers compared with age-matched controls (mean 0.4%) [118]. The group of Pestronk described 10 patients with polymyositis who had an elevated (4–27%) rate of COX-negative muscle fibers [119]. Compared with the other patients with polymyositis diagnosed at the same institution, these patients were older, had less elevated CK levels, a slower progression of weakness, a marked weakness of knee extensors and tended to respond poorly to treatment. The authors suggested that polymyositis with COX-negative muscle fibers may be a condition at a cross between polymyositis and inclusion body myositis. However, because all patients also fulfilled the Griggs criteria for possible inclusion body myositis, it is unclear whether patients had a true variant of polymyositis or just IBM. Alternatively, patients may have had true polymyositis but a coexisting mitochondrial disorder.
Tumour necrosis factor inhibitor-induced myositis in a patient with ulcerative colitis
Published in Modern Rheumatology Case Reports, 2021
Akitsu Yoshida, Yasuhiro Katsumata, Shinya Hirahara, Masanori Hanaoka, Moeko Ochiai, Masaki Kobayashi, Masayoshi Harigai
This case report of TNF inhibitor-induced myositis in a patient with UC is the first in the English literature searched on PubMed to the best of these authors’ knowledge. As summarised in Table 2, for patients with rheumatic diseases and inflammatory bowel disease other than UC, 24 cases of TNF inhibitor-induced myositis have been reported [3–19]. However, in some of these reports, the diagnoses of myositis may not be definite because specific examinations, such as muscle biopsies and EMG, were not used in every case. In contrast, in the present case, a muscle biopsy, an EMG, and a MRI were performed, and their features were all compatible with polymyositis. Furthermore, the histologic findings of the present case satisfied the strict definition for polymyositis by the European Neuromuscular Centre international workshop [21]. However, the possibility of accidental complication of IIM cannot be totally excluded in this case because the myositis was treated with immunosuppressive therapy.