• Title/Summary/Keyword: slowly progressive

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Inclusion Body Myositis : Clinical Features and Electrophysiological Findings (봉입체 근염 : 임상과 전기생리학 소견)

  • Kim, Kwang-Kuk
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.16-22
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    • 2006
  • Sporadic inclusion body myositis (s-IBM) is an aquired slowly progressive inflammatory myopathy with unknown etiology. Although light microscopic abnormalities and characteristic histopathology on muscle biopsy distinguishes from other inflammatory myopathies, vacuolated muscle fibers, intracellular amyloid deposits or tubulofilaments in electromicroscopic findings are not definite in some patients. This review shows the prominently involved muscles in s-IBM and specific or nonspecific electrophysiologic manifestations from reported data for helping the diagnosis of definite-or probable-IBM patients. In lower limbs, the quadriceps is predominantly involved, as is iliopsoas, and tibialis anterior is common. In the upper limbs, the greatest weakness is in forearm finger flexors. Finger extensors, biceps and triceps also are moderately to prominently involved. The majority of patients demonstrate polyphasic MUAPs that are short in duration. An additional striking feature is the concomitant documentation of long-duration, large-amplitude, polyphasic MUAPs. In spite of the frequent mixed myopathic-neurogenic electromyographic findings of IBM, just like that of chronic myositis, asymmetric, slowly progressive weakness of flexor digitorum profundus or quadriceps femoris muscles after age of 50 is very necessary condition for the diagnosis of IBM.

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A Case of Wernicke's Encephalopathy Presenting as Acute Bilateral Wrist Drop (급성 양측 손목처짐으로 발현한 베르니케뇌병증 1예)

  • Kim, Do-Hyung;Oh, Sun-Young
    • Annals of Clinical Neurophysiology
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    • v.16 no.1
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    • pp.27-31
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    • 2014
  • Thiamine deficiency can cause peripheral polyneuropathy and Wernicke's encephalopathy. Wernicke's encephalopathy is characterized by ataxia, ophthalmoplegia, nystagmus, and confusion, and typically presents acute and rapidly progressive course, whereas peripheral neuropathy associated with thiamine deficiency manifests chronic and slowly progressive one. However, acute and rapidly progressive axonal polyneuropathy combined with Wernicke's encephalopathy is quite rare and unusual. Here, we describe a patient with Wernicke's encephalopathy who presented with acute bilateral axonal neuropathy.

Effect of Progressive Muscle Relaxation using Biofeedback on Perceived Stress, Stress Response, Immune Response and Climacteric Symptoms of Middle-Aged Women (바이오피드백을 이용한 점진적 근육이완이 중년여성의 지각된 스트레스, 스트레스반응, 면역반응 및 갱년기 증상에 미치는 효과)

  • 정인숙
    • Journal of Korean Academy of Nursing
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    • v.34 no.2
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    • pp.213-224
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    • 2004
  • Purpose: This study was aimed to evaluate the effect of progressive muscle relaxation training using biofeedback on perceived stress, stress response, immune response and climacteric symptoms, Method: This was a crossover, pre-post test design, The study subjects are 36 middle-aged women who were selected at 2 public health centers, The independent variable was Biofeedback training for 4 weeks, twice a week and home training for 4 weeks, Dependent variables were perceived stress, stress response, immune response, and climacteric symptoms measured with Hildtch's scale (1996), Result: Progressive muscle relaxation training using biofeedback was not effective in reducing perceived stress, but it was shown to be effective in reducing physiological stress responses such as pulse rate and EMG, Though blood pressure and skin conductance were repeatedly down, and skin temperature slowly increased, there were no statistically significant differences. Progressive muscle relaxation training using biofeedback was not effective in reducing serum cortisol, enhancing immune responses, or decreasing climacteric symptoms. Conclusion: The findings point to a pressing need for further, well-controlled and designed research with consideration in selection of subjects and instruments, frequency of measurements, the sampling method, and intervention modalities.

Three-dimensional imaging of progressive facial hemiatrophy (Parry-Romberg syndrome) with unusual conjunctival findings

  • Balan, Preethi;Gogineni, Subhas Babu;Shetty, Shishir Ram;D'souza, Deepa
    • Imaging Science in Dentistry
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    • v.41 no.4
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    • pp.183-187
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    • 2011
  • Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is an uncommon degenerative condition which is poorly defined. It is characterized by a slow and progressive atrophy affecting one side of the face. The onset usually occurs during the first two decades of life. Characteristically, the atrophy progresses slowly for several years, and then it becomes stable. Ophthalmic involvement is common, with progressive enophthalmos which is a frequent finding. Cutaneous pigmentation is common in such conditions, however its extension to the conjunctiva is rarely reported. We report a case of Parry Romberg syndrome with characteristic clinical and radiographic presentation accompanied with rare ocular findings. The clinical features, radiological findings, and differential diagnoses to be considered, and the available treatment options are discussed in this report.

Chronic progressive external ophthalmoplegia in a Saudi patient with a mutation in the POLG gene successfully managed with bilateral frontalis sling

  • Algahtani, Hussein;Shirah, Bader;Alsaggaf, Khalid;Al-Qahtani, Mohammad H.;Abdulkareem, Angham Abdulrahman;Naseer, Muhammad Imran;Abuzinadah, Ahmad R.
    • Journal of Genetic Medicine
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    • v.18 no.2
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    • pp.121-126
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    • 2021
  • Chronic progressive external ophthalmoplegia (CPEO) is a complex slowly progressive mitochondrial disorder characterized by extraocular muscle weakness with or without multisystem involvement. The mainstay of therapy in a patient with CPEO is supportive. However, in moderate cases, surgery might be indicated including surgeries for ptosis and strabismus. In this article, we report a Saudi patient with CPEO due to compound heterozygous variants in the DNA polymerase gamma (POLG) gene c.2246T>C p.(Phe749Ser) and c.1735C>T p.(Arg579Trp), which are classified as pathogenic. Proper diagnosis with genetic testing confirmation is important to guide the management and counsel the patient about the prognosis and the management options. The patient was successfully managed with bilateral frontalis sling and illustrates the importance of surgical intervention to improve vision and cosmetic appearance in patients with CPEO. We emphasize the importance of multidisciplinary care in the management of cases of mitochondriopathy, especially CPEO.

Multifocal Motor Neuropathy (다초점성 운동신경병증)

  • Lee, Dong-Kuck
    • Annals of Clinical Neurophysiology
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    • v.4 no.2
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    • pp.98-107
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    • 2002
  • Multifocal motor neuropathy (MMN) is a chronic immune-mediated peripheral myelinopathy. The major clinical features include slowly progressive, painless, and asymmetric weakness, usually of distal limb muscle. Early in the course of the disease, weakness is not necessarily associated with muscle atrophy, owing to the initial primary involvement of peripheral myelin. Chronic progressive weakness is often associated with some degree of concurrent axonal loss and subsequent muscle atrophy. Sensory symptoms are usually mild or absent, and involvement of cranial and respiratory muscles is rare. The findings of multifocal motor conduction block, abnormal temporal dispersion, and focal conduction slowing at segments not at risk for common entrapment or compression injury, associated with normal sensory conduction studies along the same segments, are the hallmark electrophysiologic features of MMN. The slow progression and absence of upper motor neuron signs are the major clinical points that separate MMN from amyotrophic lateral sclerosis. The role of GM1 antibodies, found in high titers in 22~84% of MMN patients, remains uncertain. The contention that MMN is an autoimmune disorder is largely based on the often dramatic improvement in symptoms following the administration of intravenuos immunoglobulin or cyclophosphamide.

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A Case of Multiple Symmetrical Lipomatosis Causing Upper Airway Obstruction (상기도 폐쇄를 일으킨 다발성 대칭성 지방종증 1예)

  • Kim, Jeong-Kyu;Kim, Kyung-Chan
    • Tuberculosis and Respiratory Diseases
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    • v.70 no.1
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    • pp.63-68
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    • 2011
  • Multiple symmetrical lipomatosis (MSL), also called Madelung's disease, is a rare disorder of unknown etiology and characterized by abnormal accumulation of large subcutaneous fatty masses in neck, shoulder, and upper trunk. MSL has known to predominantly affect middle-aged men with a history of alcoholism. Although the clinical course of MSL is considered to be slowly progressive, in advanced stage, fatty masses in the neck may compress the upper aerodigestive tract, resulting in dyspnea and dysphagia. The treatment of MSL is surgical resection, but radical excision is very difficult and recurrence after surgery is frequent. We report the case of 55-year-old man with long lasting MSL, which caused severe airway obstruction. This patient was admitted with progressive dyspnea and massive accumulation of fat around the vocal cord that was detected on a neck CT scan. This abnormal fatty infiltration in supraglottic region caused upper airway obstruction.

Progressive Image Transmission by Hierarchical Images of arbitrary Ratio (배율가변형 계층구성을 이용한 영상의 단계적 전송)

  • 정기용;이채욱;김신환
    • The Journal of Korean Institute of Communications and Information Sciences
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    • v.17 no.6
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    • pp.621-628
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    • 1992
  • This paper proposes a progressive Image transmission method with a variable magnification hierarchical structure for image processing system. As Introduced in the literature, the progressive image transmission method, uses a fixed magnification rates of either 4 or 1/4. Thus, a sudden in-crease In resolution Is obtained due to a sudden Increase in information. By adapting a variable magnification hlerarchical structure In this research, a gradual increase in resolution Is possible by slowly inrireasing information between hierarchical levels. The simulation results show that a 5.7dB SNR improvemr'nt Is obtained with an Improved compression rate by 0.7 Ult /pel compare to the LP method. It also gives about 1 dB SNR improvement compare to the PCS method at intermediate hierarchical levels.

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