Electrophysiological spectrum of inclusion body myositis

Joy, Juan L.; Oh, Shin J.; Baysal, Ali İhsan

doi:10.1002/mus.880131010

Cited by 68 publications

(37 citation statements)

References 19 publications

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“…The semiquantitative assessment used in this study allows easy comparison with routine daily practice in which semiquantitative techniques are most frequently used. The present study confirms several findings reported previously on EMG abnormalities in myositis [10, 11,16,17,18,19,20,21]. Several conclusions can be drawn that add to the present knowledge on EMG in myositis, keeping the retrospective nature of the study in mind.…”

Section: Discussionsupporting

confidence: 81%

“…It has been suggested that a pattern of long- and short-duration MUPs is typical for IBM [11]. We found this pattern in a majority of patients with IBM and in a large group of patients with DM/PM.…”

Section: Discussionsupporting

confidence: 48%

“…On the other hand, IBM is thought to be characterized electromyographically by a mixture of myopathic and so-called neurogenic abnormalities, in combination with spontaneous activity [11, 12]. This presumed EMG characteristic of IBM can be used in clinical practice as an aid in differentiating between PM and IBM, a differential diagnosis that can be difficult to make, even histologically [13].…”

Section: Introductionmentioning

confidence: 99%

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Needle Electromyographic Findings in 98 Patients with Myositis

Blijham¹,

Hengstman

Hama-Amin³

et al. 2006

Eur Neurol

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Background/Aims: Little is known about the distribution of electromyographic (EMG) abnormalities in myositis even though this is relevant in daily practice. Methods: A retrospective semiquantitative analysis of needle EMG findings was performed in a group of 98 patients with myositis. The frequency, type, and distribution of abnormalities were studied. The influence of the use of corticosteroids and the stage of the disease were evaluated. Results: In most patients, a myopathic pattern with spontaneous activity was found, although several clinically relevant exceptions were noted. Long-duration motor unit potentials were found in all three diagnostic groups and were not associated with disease duration. In the lower extremity a distal to proximal gradient was present, adding to the diagnostic confusion with neurogenic diseases, and spontaneous activity was absent in a relatively large group although none of the patients in the acute stage of the disease had a normal EMG. The use of corticosteroids reduced the number of abnormal findings in dermatomyositis and polymyositis, but not in inclusion body myositis. Conclusion: A myopathic pattern with spontaneous activity was most frequently found, although several clinically relevant exceptions were noted. These results illustrate the spectrum of EMG findings in myositis, and may aid the clinician in the interpretation of the EMG in these patients.

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Section: Discussionsupporting

confidence: 81%

Section: Discussionsupporting

confidence: 48%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Needle Electromyographic Findings in 98 Patients with Myositis

Blijham¹,

Hengstman

Hama-Amin³

et al. 2006

Eur Neurol

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“…La afección del sensorio se examina clínicamente y con estudios electrofisiológicos y de neuroconducción, que evidencian la neuropatía axonal sensorial e incremento de la actividad polifásica motora (36,37). Los estudios de laboratorios pueden mostrar elevación de la CK sérica la cual puede estar normal o elevada hasta diez veces (38,39); los anticuerpos antinucleares pueden estar presentes en un 20% de los casos, pero usualmente están ausentes y un 20% de los pacientes pueden tener una gamapatía monoclonal indeterminada. La resonancia magnética puede mostrar afectación muscular focal, atrofia y definir los patrones de pérdida de unidades motoras después del traumatismo de nervios periféricos, por cual es importante para el diagnóstico clínico y seguimiento de respuesta al tratamiento (40)(41)(42)(43).…”

Section: Otros Desórdenes (Raros)unclassified

Miopatía por cuerpos de inclusión. Caso clínico y revisión de la literatura

et al. 2010

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ResumenLa miositis por cuerpos de inclusión (MCI) es una patología poco común que se presenta en individuos mayores de 50 años y con mayor frecuencia en hombres; sin embargo, los síntomas pueden empezar 20 años antes de su diagnóstico. Hasta un 30% de los casos de miopatías inflamatorias pueden ser de MCI y su prevalencia varía en los diferentes países y grupos étnicos. Clínicamente se manifiesta como debilidad lentamente progresiva proximal y distal. En este artículo se presenta un caso de una paciente de 78 años con antecedentes previos de diabetes tipo 2, hipertensión arterial, e hipotiroidismo, con cuadro de 18 meses de evolución consistente en debilidad de miembros inferiores, incapacidad para subir y bajar escaleras, limitación en la marcha, acompañado de disestesias. Se realizó la medición de la creatin quinasa (CK) y su valor inicial fue 7820, la electromiografía y neuroconducciones mostraron polineuropatia axonal motora en las cuatro extremidades y miopatía inflamatoria. Se trató con glucorticoides a dosis de 1 mg/kg y después de seis meses de tratamiento no se observó mejoría. Se decide tomar biopsia muscular que mostró miopatía por cuerpos de inclusión, lo que explicó la falla terapéutica inicial. Este caso es interesante desde el punto de vista clínico, ya que no es frecuente la elevación de la CK en los niveles que presentó la paciente y por las múltiples comorbilidades asociadas.Palabras clave: miositis, miopatía, cuerpos de inclusión. INCLUSION BODY MYOPATHY. CASE REPORT AND LITERATURE REVIEW AbstractInclusion body myositis (IBM) is an infrequent disease in people over 50 years of age, affecting more frequently males; however the symptoms may begin 20 years before its diagnosis. Up to 30% of all inflammatory myopathies can be IBM and its prevalence varies among different countries and ethnical groups. IBM manifest clinically by slowly progressive proximal and distal weakness. In this article a report of case is presented: A female patient 78 years old with previous history of type 2 diabetes mellitus, hypertension and hypothyroidism; with 18 months history of weakness in lower limbs, inability to climb stairs, limited to walk, associated with dysesthesias. The initial CK was 7820 mg/dl, the electromyography and nerve conductions reported a motor axonal polyneuropathy of four limbs and inflammatory myopathy. She was treated with steroids at doses of 1mg/kg without improvement, after 6 months of treatment. It was decided to take a muscle biopsy that showed an inclusion body myopathy, explaining * Correspondencia:

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“…One study reported an increased mean jitter of 83 s compared with an abnormality threshold of 60 s in all of seven IBM patients. 3 Another study reported abnormal jitter in 7 of 12 patients, 4 and a third reported increased jitter or blocking in 5 of 17 patients. 2 Jitter by itself does not cause muscle weakness, but impulse blocking does.…”

mentioning

confidence: 98%