• Title/Summary/Keyword: myopathy

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Application of Supercharge End-to-Side (SETS) Obturator to Femoral Nerve Transfer in Electrical Injury-Induced Neuropathy to Improve Knee Extension

  • Katie Pei-Hsuan Wu;Li-Ching Lin;Johnny Chuieng-Yi Lu
    • Archives of Plastic Surgery
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    • v.49 no.6
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    • pp.769-772
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    • 2022
  • Femoral nerve injuries are devastating injuries that lead to paralysis of the quadriceps muscles, weakening knee extension to prohibit ambulation. We report a devastating case of electrical injury-induced femoral neuropathy, where no apparent site of nerve disruption can be identified, thus inhibiting the traditional choices of nerve reconstruction such as nerve repair, grafting, or transfer. Concomitant spinal cord injury resulted in spastic myopathy of the antagonist muscles that further restricted knee extension. Our strategy was to perform (1) supercharge end-to-side technique (SETS) to augment the function of target muscles and (2) fractional tendon lengthening to release the spastic muscles. Dramatic postoperative improvement in passive and active range of motion highlights the effectiveness of this strategy to manage partial femoral nerve injuries.

Tc-99m ECD Brain SPECT in MELAS Syndrome and Mitochondrial Myopathy: Comparison with MR findings (MELAS 증후군과 미토콘드리아 근육병에서의 Tc-99m ECD 뇌단일 광전자방출 전산화단층촬영 소견: 자기공명영상과의 비교)

  • Park, Sang-Joon;Ryu, Young-Hoon;Jeon, Tae-Joo;Kim, Jai-Keun;Nam, Ji-Eun;Yoon, Pyeong-Ho;Yoon, Choon-Sik;Lee, Jong-Doo
    • The Korean Journal of Nuclear Medicine
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    • v.32 no.6
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    • pp.490-496
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    • 1998
  • Purpose: We evaluated brain perfusion SPECT findings of MELAS syndrome and mitochondrial myopathy in correlation with MR imaging in search of specific imaging features. Materials and Methods: Subjects were five patients (four females and one male; age range, 1 to 25 year) who presented with repeated stroke-like episodes, seizures or developmental delay or asymptomatic but had elevated lactic acid in CSF and serum. Conventional non-contrast MR imaging and Tc-99m-ethyl cysteinate dimer (ECD) brain perfusion SPECT were Performed and imaging features were analyzed. Results: MRI demonstrated increased T2 signal intensities in the affected areas of gray and white matters mainly in the parietal (4/5) and occipital lobes (4/5) and in the basal ganglia (1/5), which were not restricted to a specific vascular territory. SPECT demonstrated decreased perfusion in the corresponding regions of MRI lesions. In addition, there were perfusion defects in parietal (1 patient), temporal (2), and frontal (1) lobes and basal ganglia (1) and thalami (2). In a patient with mitochondrial myopathy who had normal MRI, decreased perfusion was noted in left parietal area and bilateral thalami. Conclusion: Tc-99m ECD SPECT imaging in patients with MELAS syndrome and mitochondrial myopathy showed hypoperfusion of parieto-occipital cortex, basal ganglia, thalamus and temporal cortex, which were not restricted to a specific vascular territory. There were no specific imaging features on SPECT. The significance of abnormal perfusion on SPECT without corresponding MR abnormalities needs to be evaluated further in larger number of patients.

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Overlap Syndromes in Polymyositis and Dermatomyositis (다발근육염과 피부근육염에서 관찰된 중첩증후군)

  • Park, Kyung Seok;Kim, Nam-Hee;Hong, Yoon-Ho;Sung, Jung-Joon;Nam, Hyunwoo;Park, Seong-Ho;Lee, Kwang-Woo
    • Annals of Clinical Neurophysiology
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    • v.9 no.1
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    • pp.11-15
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    • 2007
  • Background: The term "overlap syndromes" designates a group of diseases in which polymyositis (PM) or dermatomyositis (DM) is associated with some other disorders of connective tissues. The aim of this study was to delineate the clinical features, laboratory findings, and outcome of treatment of "overlap syndromes" Methods: We analyzed the medical records of 16 patients (PM in 10, DM in 6) with well documented "overlap syndromes" between 1997 and 2004. The diagnosis was made when the criteria for two different disorders were fulfilled. Results: All patients were female. Age of onset ranged from 14 to 52 years (mean 29.8 years) with peak incidence in the third and fourth decades. Systemic lupus erythematosus (SLE) was associated in 10, systemic sclerosis in 7, and rheumatoid arthritis in 3 patients. Four of the patients had two different connective tissue diseases simultaneously. The characteristic clinical features were muscle weakness, arthralgia, Raynaud's phenomenon, and myalgia. In laboratory tests, creatine kinase (CK), lactic dehydrogenase (LDH), and transaminases were usually abnormal. Positive antinuclear antibody (ANA), rheumatoid factor (RF), and cryoglobulin were found in 100%, 69%, and 67% of the patients, respectively. Needle electromyography (EMG) showed abnormal findings compatible with myopathy in 15 patients. The pathology of muscle biopsy from 14 patients revealed findings compatible with inflammatory myopathy. Glucocorticoids were administered to 15 patients. The muscle strength improved in all the treated patients, which was well correlated with repeat CK level and EMG findings. Conclusions: The presence of autoantibodies such as ANA, RF, and cryoglobulin in patients with PM or DM highly suggests the possibility of an overlap syndromes. These syndromes reveal a strong female predominance. The myositis associated with them usually shows a good response to glucocorticoids treatment.

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White striping degree assessment using computer vision system and consumer acceptance test

  • Kato, Talita;Mastelini, Saulo Martiello;Campos, Gabriel Fillipe Centini;Barbon, Ana Paula Ayub da Costa;Prudencio, Sandra Helena;Shimokomaki, Massami;Soares, Adriana Lourenco;Barbon, Sylvio Jr.
    • Asian-Australasian Journal of Animal Sciences
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    • v.32 no.7
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    • pp.1015-1026
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    • 2019
  • Objective: The objective of this study was to evaluate three different degrees of white striping (WS) addressing their automatic assessment and customer acceptance. The WS classification was performed based on a computer vision system (CVS), exploring different machine learning (ML) algorithms and the most important image features. Moreover, it was verified by consumer acceptance and purchase intent. Methods: The samples for image analysis were classified by trained specialists, according to severity degrees regarding visual and firmness aspects. Samples were obtained with a digital camera, and 25 features were extracted from these images. ML algorithms were applied aiming to induce a model capable of classifying the samples into three severity degrees. In addition, two sensory analyses were performed: 75 samples properly grilled were used for the first sensory test, and 9 photos for the second. All tests were performed using a 10-cm hybrid hedonic scale (acceptance test) and a 5-point scale (purchase intention). Results: The information gain metric ranked 13 attributes. However, just one type of image feature was not enough to describe the phenomenon. The classification models support vector machine, fuzzy-W, and random forest showed the best results with similar general accuracy (86.4%). The worst performance was obtained by multilayer perceptron (70.9%) with the high error rate in normal (NORM) sample predictions. The sensory analysis of acceptance verified that WS myopathy negatively affects the texture of the broiler breast fillets when grilled and the appearance attribute of the raw samples, which influenced the purchase intention scores of raw samples. Conclusion: The proposed system has proved to be adequate (fast and accurate) for the classification of WS samples. The sensory analysis of acceptance showed that WS myopathy negatively affects the tenderness of the broiler breast fillets when grilled, while the appearance attribute of the raw samples eventually influenced purchase intentions.

Treatment of Eyelid Ptosis due to Kearns-Sayre Syndrome Using Frontalis Suspension

  • Weitgasser, Laurenz;Wechselberger, Gottfried;Ensat, Florian;Kaplan, Rene;Hladik, Michaela
    • Archives of Plastic Surgery
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    • v.42 no.2
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    • pp.214-217
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    • 2015
  • Blepharoptosis is a common indication for surgery in plastic surgery units, yet its possible underlying pathology frequently remains unidentified. A 52-year-old man with a 20-year history of progressive bilateral ptosis (right>left) presented with recurrent ptosis of both eyes; he had undergone an operation on the levator aponeurosis 12 years prior. Due to the suspicion of an underlying disease, he was evaluated further. Chronic progressive external ophthalmoplegia in transition to the more severe syndromic variant Kearns-Sayre syndrome, a mitochondrial disorder causing myopathy, was diagnosed. The patient was treated with coenzyme Q10, and he underwent ptosis surgery on both eyes. This case illustrates a potentially multi-systemic disease that was diagnosed by a further evaluation of a common symptom, in this case worsening blepharoptosis. Awareness of myopathic symptoms is necessary to prevent overlooking serious yet improvable conditions.

The Contents of Tumor Necrosis Factor-${\alpha}$ and Interleukin-6 in Right Auricular Tissue (우심이 조직내의 Tumor necrosis Factor-${\alpha}$와 Interleukin-6의 함량)

  • 김송명;신현우;박성달;이재성
    • Journal of Chest Surgery
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    • v.33 no.1
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    • pp.1-6
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    • 2000
  • Background: TNF-$\alpha$ plays a major role in producing left ventricular dysfunction cardio-myopathy pulmonary edema and inhibits the compensatory mechanism of congestive heart failure. IL-6 is an acute reactant of immune reaction and also known to control immune reaction but its function in the myocyte was not clearly investigated. Author's performed this experiment to investigate the contents of TNF-$\alpha$ and IL-6 on the assumption that TNF-$\alpha$ and IL-6 may reside in nonfailing heart that has gone cardiac surgery and play some role in cardiac function. Material and Method : Right auricular tissues were sampled from 12 patients who had undergone total corrective surgery for both congenital and acquired heart diseases from January 1998 to June 1998 in Kosin Universcfy Gospel hospital. The quantitive analysis of TNF-$\alpha$ and IL-6 were assessed by ELISA method in right auricular tissue. Hemodynamic values about the pressure of ventricle atrium aorta pulmonary artery and cardiac index pulmonary and systemic vascular resistance and cardiac output were measured by echocardiography and cardiac catheterization and biochemical analyses of LDH & AST were done before operation. statistical analysis was by Paired Student t-test. Patients were divided into children(under 15 years olds) and adults groups and the data was compared beween two groups. Conclusion: Mild pulmonary hypertension and increased pulmonary vascular resistance were existed in both group. The contents of tissue TNF-$\alpha$ IL-6 in each group were independent of each data.

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Myopathy in Clinically Healthy Cultured Abalone Haliotis discus hannai (양식 참전복(Haliotis discus hannai)의 비병증 예의 근육변성)

  • Kim, Bo Seong;Yi, Sung Joo;Hwang, Se Myung;Lee, Mu Kun;Park, Young Jin;Song, Kyo Jung;Huh, Min Do
    • Korean Journal of Fisheries and Aquatic Sciences
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    • v.47 no.2
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    • pp.174-178
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    • 2014
  • In this study, 6-10 clinically healthy farmed abalone, Haliotis discus hannai were obtained from Jeollanam-Do Wando Soan-myeon, Bogil-myeon, and Nohwa-eup monthly for 1 year 7 months and were examined histopathologically. As a result, the adductor muscle had severe cellular swelling and myonecrosis. These lesions were stained with hematoxylin and eosin and Giemsa. The lesions were not caused by infection of bacteria or parasites. We investigated the relationship between lesion frequency and water temperature and discovered that increased water temperature was associated with increasing lesion frequency. As water temperature is related to growth rate, increased growth rate was closely related to increased lesion frequency. We considered that these lesions could be a useful factor in measuring shellfish health.

Central Core Disease : Clinical Characteristics of Family Members Manifested by Autosomal Dominant Pattern through Three Generations (중심핵병 : 삼대에 걸쳐 상염색체 우성 양식으로 발현된 가계 환자들의 임상적 특징)

  • Park, Kee Hyung;Shin, Dong Jin;Kim, Seung Hyun
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.23-28
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    • 2006
  • Background: central core disease is one of the non-progressive benign congenital myopathies characterized by the presence of cores in muscle fibers, which was originally described by Shy and Magee (1956). We describe clinical charcteristics of central core disease in a Korean family manifested by autosomal dominant pattern through three generations. Methods: Clinical, serologic, and electrophysiologic profiles were evaluated in eleven members among 22 family members through three generations. Results: Six family members were symptomatic and five were non-symptomatic. Instead of proximal muscle weakness, musculoskeletal manifestations including non-specific joint pain and stiff sense were the most frequent symptoms. Muscle biopsy performed in two symptomatic patients revealed that type I fiber showed central halo, which is charactreristics of central core disease. No remarkable findings were present in serologic study including CPK level and electromyographic findings suggesting myopathic pattern were only present in two patients among 11 symptomatic group. Conclusions: In evaluating non-specific musculoskeletal complaints from the familial members showing genetic trait, central core disease should be considered to one of the possible diagnosis.

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Identification of a Mitochondrial DNA Mutation in Paraffin-Embedded Muscle Tissues (파라핀조직을 이용한 미토콘드리아 DNA 돌연변이 확인)

  • 김상호;유석호
    • Journal of Life Science
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    • v.14 no.2
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    • pp.296-300
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    • 2004
  • We investigated feasibility of using the formalin-fixed and paraffin-embedded tissue to study mitochondrial mutations in the case that fresh or frozen tissue, or blood samples are not available. Four paraffin blocks of muscle biopsies in Korean MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) patients were chosen. Total DNA was extracted from these blocks for PCR/RFLP analysis, and sequencing was performed to study the most common mutation, A to G transition at nucleotide position 3243 underlying MELAS in the mitochondrial tRN $A^{Leu(UUR)}$ gene. We could identify the A to G mutation at nt.3243 in three MELAS patients. Our results show that the mitochondrial genome of our paraffin blocks is presumably in good condition. Our results are in accordance with the previous findings by other investigators that PCR allows molecular genetic analysis of paraffin-embedded tissues stored in most histopathology laboratories.s.

Differential Diagnosis of Hirschsprung's Disease (Hirschsprung's Disease의 감별 진단)

  • Yoo, Soo-Young
    • Advances in pediatric surgery
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    • v.8 no.1
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    • pp.54-61
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    • 2002
  • Hirschsprung's disease (HD) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of meconium within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium ileus, meconium plug syndrome, distal ileal atresia and low imperforate anus often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, intestinal neuronal dysplasia (IND), hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions.

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