| Seung Min Kim | 6 Articles |
The pathogenesis of many strokes originates in the vessel wall. Despite this, most traditional imaging focuses on the vascular lumen. Vessel-wall magnetic resonance imaging (VWMRI) is useful for establishing the etiology of intracranial stenosis. It also provides information regarding atherosclerotic plaque composition and thus plaque vulnerability, which is an indication of its potential to cause a stroke. In this review we focus on the characteristics of VWMRI findings in various arteriopathies related to intracranial artery stenosis, and discuss the clinical implications of these findings.
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Background
Magnetic resonance (MR) images are useful for diagnosing myopathy. The purpose of this study was to determine the usefulness of lower-limb MR images in Korean patients with distal myopathy. Methods We reviewed medical records in the myopathy database from January 2002 to October 2016. We selected 21 patients from 91 unrelated families with distal myopathy: four with GNE myopathy, 11 with dysferlinopathy, and six with ADSSL1 myopathy. Results Ten (48%) of the 21 patients were men. The ages of the participants at symptom onset and imaging were 19.2 ± 9.5 and 30.4 ± 9.0 years (mean ± standard deviation), respectively. Their grade on the modified Gardner-Medwin and Walton grade was 3.3 ± 1.7. The strength grade of the knee extensors was not correlated with the Mercuri scale for the quadriceps (r = –0.247, p = 0.115). However, the Medical Research Council grades of the knee flexors, ankle dorsiflexors, and ankle plantar flexors were significantly correlated with the Mercuri scale ratings of the knee flexors (r = –0.497, p = 0.001), tibialis anterior (r = –0.727, p < 0.001), and ankle plantar flexors (r = –0.620, p < 0.001), respectively. T1-weighted MR images showed characteristic fatty replacement patterns that were consistent with the causative genes. Unsupervised hierarchical clustering of the Mercuri scale showed that the main factors contributing to the dichotomy were the causative gene and the clinical severity. Conclusions This study is the first to reveal the usefulness of lower-limb MR images in the differential diagnosis of distal myopathy in Korea.
Background
The Korean Society of Neuromuscular Disorders developed the guideline for the diagnosis of Charcot-Marie-Tooth disease (CMT). The guideline was intended to help readers select appropriate tests and practice systemized diagnostic approach in cases with suspected CMT in Korea. Methods: We developed this guideline of CMT through the de novo process using an evidence- based approach. After systematic review of the literature, eight statements were selected using the Appraisal of Guidelines for Research and Evaluation (AGREE) II process. Results: A total of 17 statements were proposed with the grading system and revised using the modified Delphi method. They were reviewed by external experts before receiving official endorsement from the Korean Society of Neuromuscular Disorders, and disseminated to doctors and other medical professionals for use in clinical practice in Korea. Conclusions: The guideline for proper diagnosis in CMT is presented here. This guideline will continue to be updated and revised periodically.
A 73-year-old woman presented with progressive, symmetric distal dominant sensory change followed by motor weakness accompanied with multiple skin lesions. Neurologic examinations and electrophysiologic study revealed demyelinating polyneuropathy. Diagnosis of leprous neuropathy was made with skin biopsy. This rare presentation of leprosy indicates leprosy can be considered as differential diagnosis in patients with clinically evident progressive polyneuropathy.
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