• Clinical Pain
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• 제18권1호
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• pp.48-51
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• 2019

Symmetrical proximal weakness and characteristic dermatologic manifestations are important in the diagnosis of dermatomyositis. We report a case of atypical presentation of dermatomyositis due to previous steroid use and also report steroid-induced myopathy which may occur from steroid administration during the course of treatment. A 77-year-old man, previous steroid user, showed rapidly progressing weakness after abruptly stopped medication. He has presented erythematous papule on face and anterior chest but no heliotrope rash and Gottron's papules were observed. Muscle enzyme (creatine kinase) concentration is increased, and needle electromyography shows increased spontaneous activity on proximal limb muscle. The muscle biopsy confirmed dermatomyositis. During the course of treatment, he revealed persistent weakness despite the continuous steroid use and stable creatine kinase level. Electrodiagnostic study suggests steroid-induced myopathy and after tapering steroid, proximal muscle strength improved. This case reports the effect of steroid use on dermatomyositis patients and a process of diagnosing coexisting steroid induced myopathy during treatment.

• Annals of Clinical Neurophysiology
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• 제5권1호
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• pp.11-15
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• 2003

Backgrounds: Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods: We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results: We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions: Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple nucleotide CAG repeats(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.

• Journal of Yeungnam Medical Science
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• 제28권2호
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• pp.173-179
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• 2011

Polymyositis is diffuse, inflammatory myopathy with proximal-muscle weakness due to lymphocyte infiltration to the muscle layer. The exact cause of the muscle weakness is unclear but may be related with an immunologic mechanism. Using high-dose steroid is the treatment of choice for polymyositis. It is difficult to distinguish steroid-resistant polymyositis from steroid myopathy, however, in the course of high-dose steroid therapy. These authors encountered a steroid myopathy patient during polymyositis treatment with high-dose steroid. A 57-year-old woman was diagnosed with polymyositis and was treated with high-dose steroid. Her condition was initially improved, but in the course of the treatment, her symptom was aggravated without increasing the muscle enzymes. Her muscle weakness was improved by reducing the steroid dosage.

• Journal of Genetic Medicine
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• 제10권2호
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• pp.94-98
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• 2013

Dystrophinopathy, caused by mutations in the DMD gene, presents with variable clinical phenotypes ranging from the severe Duchenne muscular dystrophy (DMD) to the milder Becker muscular dystrophy(BMD) forms. DMD is a recessive X-linked form of muscular dystrophy. Two-thirds of mothers of affected males are thought to be DMD carriers. Approximately 2.5-7.8% of female DMD carriers have muscle weakness and are categorized as manifesting DMD carriers. The symptoms of female carriers of DMD range from mild muscle weakness to severe gait problems. The most commonly presented symptom is mild proximal muscle weakness, which is often asymmetric and progressive, but shows variable clinical spectrum with BMD of more severe DMD-like phenotype. Atypical presentations in manifesting carriers are myalgia or cramps without limb weakness, isolated cardiomyopathy and camptocormia. Multiplex PCR and MLPA analysis are common techniques to identify mutations in the DMD gene. Relationship between X-chromosome inactivation and clinical severity is not clear. Female carriers of DMD are not less common, and they have an important role of birth of a male DMD.

• Annals of Clinical Neurophysiology
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• 제25권1호
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• pp.41-44
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• 2023

Overlap myositis (OM) associated with Sjögrens syndrome (SS) is a relatively uncommon disorder. It is more difficult to detect OM when a patient had not been previously diagnosed with SS. We report a case of OM associated SS that initially presented with dysphagia followed by proximal weakness. If proximal muscle weakness is preceded by dysphagia, the possibility of OM associated with connective-tissue disease should be considered.

• Annals of Clinical Neurophysiology
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• 제23권2호
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• pp.134-137
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• 2021

Primary hyperparathyroidism (PHP) is a disease in which excessive amounts of parathyroid hormone (PTH) are secreted and calcium levels in the blood increase. Hypercalcemia caused by PHP has a major influence on the peripheral nervous system and produces symptoms such as muscle cramps, paresthesia, and proximal muscle weakness. Here we report a rare case of sensory-dominant polyneuropathy caused by PHP, which improved after surgery.

• Korean Journal of Acupuncture
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• 제27권4호
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• pp.119-127
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• 2010

Objectives : Polymyositis is non-hereditary, autoimmune rheumatic disease, and one of the idiopathic inflammatory myopathy. It is characterized by progressive symmetrical proximal muscle weakness. Major clinical signs and symptoms of polymyositis are chronic non-suppurative inflammatation of skeletal muscle, elevation of muscle enzyme, and electromyographical changes. By use of Korean medicine treatments, muscle weakness and normalize muscle enzyme can be improved in case of polymyositis. Methods : A Patient, 32 years old, whom diagnosed with polymyositis on muscle biopsy and electromyographical changes, had medical treatments with Corticosteroids and Immunosuppressants, but no improvement was noted. She was treated with Korean Medicine, especially Ohaeng-Hwa Acupuncture(五行和 鍼法) and Ssanghwa-Tang(雙和湯) for three months period. Results : With Korean Medicine treatment of polymyositis for three months, most of her clinical signs and symptoms have been improved significantly, especially her muscle weakness and muscle enzyme level. Conclusion : This case has been reported to show that Korean medicine treatments have positive effects on polymyositis.

• Journal of Yeungnam Medical Science
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• 제27권1호
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• pp.42-46
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• 2010

Eosinophilic myositis is a rare idiopathic inflammatory muscle disease, and the patients with this malady present with diverse signs and symptoms such as muscle swelling, tenderness, pain, weakness, cutaneous lesions and eosinophilia. The etiology and pathogenesis of eosinophilic myositis remain elusive. Several drugs may occasionally initiate an immune mediated inflammatory myopathy, including eosinophilic myositis. We report here on a case a 17-year-old female patient who had taken anti-tuberculosis medicine for tuberculosis pleurisy. She presented with many clinical manifestations, including fever, skin rash, proximal muscle weakness, dyspnea, dysphagia and hypereosinophilia. She was diagnosed with eosinophilic myositis by the pathologic study. The muscle weakness progressed despite of stopping the anti-tuberculosis medicine, but the myositis promptly improved following the administration of glucocorticoid. Although drug induced myopathies may be uncommon, if a patient presents with muscular symptoms, then physicians have to consider the possibility of drug induced myopathies.

• Annals of Clinical Neurophysiology
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• 제4권1호
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• pp.7-11
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• 2002

Among drug-induced myopathy, steroids are probably the most common cause. The risk of steroid myopathy(SM) increases with the dose and duration of use. It is typically a proximal myopathy, preferentially affecting the hip girdle muscles. Motor and sensory nerve conduction studies are normal. The needle EMG is usually within the normal range or may be minimally abnormal. Occasionally, low-amplitude, short-duration MUAPs may be seen in the proximal muscles. Of note, abnormal spontaneous activity is not seen. This point is often very useful in differentiating polymyositis(PM) from SM. It is common for patients with PM to be treated with steroids, respond well, and then have the steroids tapered. If muscle weakness then returns, it may be very difficult to differentiate recurrent PM from SM on clinical grounds. The presence of abundant abnormal spontaneous activity strongly suggests PM rather than SM.

• 대한한의학회지
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• 제32권5호
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• pp.134-138
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• 2011

Glycyrrhetinic acid, which is a component of licorice, can cause hypermineralocorticoidism through the inhibition of $11{\beta}$-hydroxysteroid dehydrogenase. So, a high dose intake of licorice can lead to hypermineralocorticoidism with potassium loss and depression of the renin-angiotensin-aldosterone system. We report a 73-year-old man with muscle weakness of proximal lower limbs with hypokalemia (Serum $K^+$: 1.4 mEq/L) due to taking self-prescribed licorice without OMD's diagnosis. He boiled 60~100g licorice / day in water and drank it intermittently for 1 month due to arthralgia and swelling of both his knees. Patient's serum renin activity and aldosterone level were far beyond the normal range. He also had metabolic alkalosis with pH 7.552. After quitting the licorice, hypokalemia and muscle weakness of proximal lower limbs gradually improved within 1week.