• Title/Summary/Keyword: Demyelinating

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A Case of Osmotic Demyelination Syndrome in a Patient with Severe Hyponatremia Complicated by Rhabdomyolysis (횡문근융해증이 합병된 중증 저나트륨혈증 환자에서 발생한 삼투성 탈수초 증후군 1예)

  • Lee, Da Young;Hong, Chang Woo;Lee, In Hee
    • Journal of Yeungnam Medical Science
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    • v.30 no.1
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    • pp.25-30
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    • 2013
  • Hyponatremia, the most common electrolyte disorder, has been rarely reported as causing rhabdomyolysis. Osmotic demyelination syndrome (ODS), a demyelinating disease of the central pons and/or other areas of the brain, is infrequently reported as associated with rapid correction of hyponatremia. This paper reports a case of ODS after correction of severe hyponatremia complicated by rhabdomyolysis. A 47-year-old female with a history of chronic alcoholism presented herself at the hospital with altered consciousness after three days of nausea and vomiting. She was on a thiazide diuretic for essential hypertension. Her blood tests upon her hospital admission showed hyponatremia ($Na^+$ 98 mEq/L), hypokalemia ($K^+$ 3.0 mEq/L), and elevation of her serum creatine phosphokinase (3,370 IU/L) with an increase in her serum myoglobin level 11,267 ng/mL). She was treated with intravenous fluid therapy that included isotonic and hypertonic salines along with potassium chloride. She became more alert, and her neurological condition gradually improved after the first five days of her therapy. On the ninth day after her admission, she developed progressive quadiaresis associated with dysarthria, dysphagia, and dystonia despite the resolution of her hyponatremia. Magnetic resonance imaging of her brain on 16th day revealed symmetrical areas of signal hyperintensity in her central pons, basal ganglia, and precentral gyrus in T2-weighted images, which are consistent with ODS. Her neurological symptoms steadily improved after six weeks with only supportive treatment and rehabilitation.

A case report of Guillain-Barre syndrome (Guiillain-Barre 증후군 환자의 치험 1례)

  • Kim, Ki-Hoon;Shin, Dong-Gil;Lee, Jin-Yong;Cho, Baek-Gun
    • The Journal of Pediatrics of Korean Medicine
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    • v.17 no.2
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    • pp.199-211
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    • 2003
  • Objective : This is clinical report about the Wei syndrome(?證)-patient diagnosed as Guillian-Barre syndrome. Guillain-Barre syndrome(GBS), what is called acute inflammatory polyneuritis, is a disorder in which the body's immune system attacks parts of peripheral nervous system. GBS is subclassified into acute inflammatory demyelinating polyneuropathy(AIDP), acute motor or motor-sensory axonal neuropathy(AMAN, AMSAN), and the other variants. The cause and mechanism of this syndrome are unknown yet. The typical Guillain-Barre syndrome could be diagnosed by the patient's syndroms and physical exams as the rapid onset of weakness, paralysis and loss of reflexes. The analysis of CSF and electrical test of nerve and muscle function can be performed to confirm the diagnosis. Most of the cases usually occur shortly after a viral infection. Method & Result : This is the clinical report about the one patient daignosed as Guillain-Barre syndrome. The patient, 9-year-old girl had the hemiparesis after upper respiratory infection. We characterized her as Wei syndrom(?證). The patient was treated by acupunture, indirect moxibustion, herb medication(通竅湯 加味方, 四物湯合檳蘇散 加味方) and had significant improvement in the Wei syndrome(?證). Conclusion : We report that we had good effects of oriental medical treatment on Guillain-Barre syndrome.

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Acute disseminated encephalomyelitis in children: differential diagnosis from multiple sclerosis on the basis of clinical course

  • Lee, Yun-Jin
    • Clinical and Experimental Pediatrics
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    • v.54 no.6
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    • pp.234-240
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    • 2011
  • Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

A Case Report of Korean Traditional Medical Therapy about Patient with Guillain-Barre Syndrome (Guillain-Barre Syndrome 환자의 한방 치험 1례)

  • Kim, Jong-Won;Kim, Min-Sang;Oh, Byeong-Yeol;Yu, Byeong-Chan;Jo, Hyun-Kyung;Kim, Yoon-Sik;Seol, In-Chan;Shim, Jae-Chul
    • The Journal of Internal Korean Medicine
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    • v.25 no.4
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    • pp.418-425
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    • 2004
  • The Guillain-Barre Syndrome(GBS), also called acute inflammatory demyelinating polyneuropathy and Landry's ascending paralysis, is an inflammatory disorder of the peripheral nerves - those outside the brain and spinal cord. It is characterized by the rapid onset of weakness and, often, paralysis of the legs, arms, breathing muscles and face. Finally, it leads to respiratory embarrassment and death. There is often a history of antecedent respiratory or gastrointestinal infection. Recently one patient was admitted with GBS. This patient was a 30 year-old man with quadriparesis, both facial palsy, numbness of hands and feet, and gait disturbance. After two weeks of oriental medicine and acupuncture treatment most symptoms improved. Therefore, this application of oriental medicine is reported with a plea for further investigation.

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The Concepts of Montage in Somatosensory Evoked Potentials (체성감각 유발 전위에서 montage에 대한 개념)

  • Cha, Jae-Kwan;Kim, Seung-Hyun
    • Annals of Clinical Neurophysiology
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    • v.1 no.2
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    • pp.160-167
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    • 1999
  • Although somatosensory evoked potentials(SSEPs) have been utilized as the useful diagnostic tools in evaluating the wide variety of pathological conditions, such as focal lesions affecting the somatosensory pathways, demyelinating diseases, and detecting the clinically occult abnormality, their neural generators is still considerably uncertain. To appreciate the basis for uncertainties about the origins of SSEPs, consider criteria that must be met to establish a causal relationship between activity in a neural structure and a spine/ scalp-recorded potential. Electrode locations and channel derivations for SSEPs recordings are based on two principles:(1) the waveforms are best recorded from electrode sites on the body surface closest to the presumed generator sources along the somatosensory pathways, and(2) studies of the potential-field distribution of each waveform of interest dictate the best techniques to be used. In this article, authors will describe followings focused on ;(1) the concepts of near field potentials(NFPs) and far field potentials(FFPs) - the voltage of NFPs is highly dependent upon recording electrode position, FFPs are unlike NFPs in that they are widely distributed, their latencies and amplitudes are independent of recording electrode.(2) appropriate montage settings to detect the significant potentials in the median nerve and posterior tibial nerve SSEPs(3) neural generators of various potentials(P9, N13, P14, N18, N20, P37) and their clinical significance in interpretating the results of SSEPs. Especially, Characteristics of N18(longduration, small superimposed inflection) suggested that N18 is a complex wave with multiple generators including brainstem structures and thalamic nuclei. And N18 might be used as the parameter of braindeath. Precise understanding on these facts provide an adequate basis utilizing SSEPs for numerous clinical purposes.

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Expression of galectin-3 in the spinal cords of Lewis rats andNOD mice with experimental autoimmune encephalomyelitis (자기면역성뇌척수염 척수조직에서 galection-3의 발현)

  • Kim, Heechul;Joo, Hong-Gu;Moon, Changjong;Ahn, Meejung;Jee, Youngheun;Lim, Yoon-kyu;Koh, Chang-Sung;Shin, Taekyun
    • Korean Journal of Veterinary Research
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    • v.44 no.3
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    • pp.349-355
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    • 2004
  • The aim of this study was to evaluate the expression of galectin-3, one of beta-galactoside-binding proteins, in the experimental autoimmune encephalomyelitis(EAE) model of Lewis rats or non-obese diabetic (NOD) mice. Western blot analysis showed that galectin-3 was weakly expressed in the spinal cords of complete Freund's adjuvant(CFA) immunized control rats. In EAE, however, galectin-3 expression was significantly increased at the peak stage(days 14 post-immunization), while it was decreased slightly at the recovery stage(day 21 post-immunization). Immunohistochemical analysis showed that galectin-3 was detected in some macrophages in demyelinating lesions of NOD mice, while galectin-3 was immunoreacted in some inflammatory cells in the perivascular cuffing in rat EAE lesions. Collectively, it is postulated that the expression of galectin-3 is significantly increased in response to neuroimmunological stimulation in the central nervous system, whereas it is weak in normal rats and mice.

Diagnosis and treatment in Charcot-Marie-Tooth disease (샤르코-마리-투스 질환의 진단 및 치료)

  • Kim, Sang-Beom;Park, Kee Duk;Choi, Byung-Ok
    • Annals of Clinical Neurophysiology
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    • v.7 no.2
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    • pp.65-74
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    • 2005
  • Charcot-Marie-Tooth (CMT) disease was described by Charcot and Marie in France and, independently, by Tooth in England in 1886. CMT is the most common form of inherited motor and sensory neuropathy, and is a genetically heterogeneous disorder of the peripheral nervous system. Therefore, many genes have been identified as CMT-causative genes. Traditionally, subclassification of CMT have been divided into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). Recently, intermediate type (CMT-Int) with NCVs between CMT1 and CMT2 is considered as a CMT type. There are several related peripheral neuropathies, such as $D{\acute{e}}j{\acute{e}}rine$-Sottas neuropathy (DSN), congenital hypomyelination (CH), hereditary neuropathy with liability to pressure palsies (HNPP) and giant axonal neuropathy (GAN). Great advances have been made in understanding the molecular basis of CMT, and 17 distinct genetic causes of CMT have been identified. The number of newly discovered mutations and identified genetic loci is rapidly increasing, and this expanding list has proved challenging for physicians trying to keep up with the field. Identifying the genetic cause of inherited neuropathies is often important to determine at risk family members as well as diagnose the patient. In addition, the encouraging studies have been published on rational potential therapies for the CMT1A. Now, we develop a model of how the various genes may interact in the pathogenesis of CMT disorder.

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A Clinical Case Report on Guillain-Barre Syndrome with diplopia (복시(複視)(Diplopia)를 호소하는 Guillain-Barre Syndrome(GBS) 환자 치험 1례 증례보고)

  • Kang, Sung-Wook;Moon, Mi-Hyun;Hong, Suk-Hoon;Hwang, Chung-Yeon;Kang, Jeong-Ran;Park, Joon-Young
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.18 no.3
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    • pp.102-107
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    • 2005
  • Guillain-Barre Syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy. It is disorder in which the body's immune system, attacks parts of the peripheral nervous system. It is characterized by the rapid onset of weakness, paralysis of the legs, arms, breathing muscles and face. But the analysis of CSF and electrical tests on nerve and muscle function can be performed to confirm the diagnosis. Most cases occur shortly after a viral infection. This is a clinical ease report on Cuillain-Barre Syndrome with diplopia. The patient, a 52-year-old man had a weakness in both legs and diplopia. His weakness and diplopia improved after oriental medical treatment, so this is reported as a potential treatment. Objective: This study was designed to evaluate the effects on oriental medicine therapy on Guillain-Barre Syndrome with diplopia. Methods & Result: The Clinical data was analyzed on a patient with Cuillain-Barre Syndrome whose main symptoms were diplopia. The patient was treated by acupuncture and oriental medicine. As a result, symptoms was improved remarkably. Conclusion: The patient showed weakness and diplopia. After acupuncture and oriental medicine treatment, weakness and diplopia was improved in 4weeks after visit to clinic. The study suggests that oriental medicine treatment is effective on Cuillain-Barre Syndrome.

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Report on the Guillain-Barre Syndrome with Autonomic Dysfunction(1 Case) (자율신경 기능 이상을 동반한 Guillain-Barre 증후군 1례)

  • Kim, Dong-Ung;Shin, Sun-Ho;Cho, Gwon-Il;Shin, Hak-Su;Han, Myung-A;Choi, Jin-Young;Choi, Woo-Jung;Yang, Jae-Hun;Chung, Yong-Jun;Kim, Kwan-Sick
    • The Journal of Internal Korean Medicine
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    • v.22 no.3
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    • pp.453-458
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    • 2001
  • Guillain-Barre syndrome, or acute inflammatory demyelinating Polyradiculoneuropathy, is frequently accompanied by cardiac and autonomic dysfunction. We report a patient who had tachycardia, orthostatic hypotension, hypertension, pronounced blood pressure fluctuations, abnormal sweating, constipation and urinary frequency as well as qudriparesis. We thought that the GBS was incurred by Damp-Heat, used Heat-Clearing and Dampness-Transforming decoction(Chongjoo-tang) in the early stage. In the later stage, fortifying the Spleen and Boosting Qi plus Supplementing the Kidney decoction(Palmultang+chongawon) was used to remove low back pain and boost recovery. The patient reached the nadir 14 days after onset. He became bed-bound and autonomic dysfunction was very severe. From 3rd week, abnormalities of autonomic function and paresis impoved gradually and he could walk above 5m without walker or equivalent support at the 5th week after onset.

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Surgical Management Options for Trigeminal Neuralgia

  • Lunsford, L. Dade;Niranjan, Ajay;Kondziolka, Douglas
    • Journal of Korean Neurosurgical Society
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    • v.41 no.6
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    • pp.359-366
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    • 2007
  • Trigeminal neuralgia is a condition associated with severe episodic lancinating facial pain subject to remissions and relapses. Trigeminal neuralgia is often associated with blood vessel cross compression of the root entry zone or more rarely with demyelinating diseases and occasionally with direct compression by neoplasms of the posterior fossa. If initial medical management fails to control pain or is associated with unacceptable side effects, a variety of surgical procedures offer the hope for long-lasting pain relief or even cure. For patients who are healthy without significant medical co-morbidities, direct microsurgical vascular decompression [MVD] offers treatment that is often definitive. Other surgical options are effective for elderly patients not suitable for MVD. Percutaneous retrogasserian glycerol rhizotomy is a minimally invasive technique that is based on anatomic definition of the trigeminal cistern followed by injection of anhydrous glycerol to produce a weak neurolytic effect on the post-ganglionic fibers. Other percutaneous management strategies include radiofrequency rhizotomy and balloon compression. More recently, stereotactic radiosurgery has been used as a truly minimally invasive strategy. It also is anatomically based using high resolution MRI to define the retrogasserian target. Radiosurgery provides effective symptomatic relief in the vast majority of patients, especially those who have never had prior surgical procedures. For younger patients, we recommend microvascular decompression. For patients with severe exacerbations of their pain and who need rapid response to treatment, we suggest glycerol rhizotomy. For other patients, gamma knife radiosurgery represents an effective management strategy with excellent preservation of existing facial sensation.