• The Journal of Korean Oriental Chronic Disease
• /
• v.10 no.1
• /
• pp.53-61
• /
• 2005

Guillain-Barre syndrome (GBS) is a group of autoimmune syndromes consisting of demyelinating and acute axonal degenerating forms of the disease. Typically, Gullain-Barre syndrome can be diagnosed from the patient's symptoms and physical examination such as the rapid onset of weakness, paralysis and loss of reflexes. In most patients, resolution is complete or near complete. Treatment consists of supportive care, ventilatory management (in about one third of patients), and specific therapy with intravenous immunoglobulin or plasmapheresis. This clinical report is about suspected acute severe Guillain-Barre syndrome patient, 61-year-old man had quadriplegia, facial palsy, dysphasia, respiratory failure. After 5 weeks of East-West integrative medicine therapy - Conventional Conservative therapy(plasmaphresis and intravenous immunoglobulin) and Korean traditional medicine(Sasang medicine and acupuncture treatment) - most symptoms improved.

• Proceedings of the Korean Society of Applied Pharmacology
• /
• 2003.11a
• /
• pp.84-84
• /
• 2003

Lead has long been considered as a toxic environmental pollutant, which severely damages central nervous system. Lead can cause hypo- and de-myelination, and glial cells are closely related with myelination or demyelination. Matrix metalloproteinases (MMPs) are proteolytic enzymes that are involved in the remodelling of the extracellular matrix in a variety of physiological and pathological processes. MMPs also seem to be important in the pathogenesis of inflammatory demyelinating diseases of the central and peripheral nervous system. In this study, we investigated whether lead affects MMP-9 expression in rat primary glial cells. Treatment of 0.1-5 ${\mu}$M lead dose- and time-dependently increased MMP-9 expression in rat primary glial cells. The activity of MMPs was determined using zymography. Lead activated Erk(1/2) but neither of the other endogenous MAP kinases, p38 or JNK. Inhibition of Erk(1/2) activation by PD98059, a MEK inihibitor, prevented lead-induced expression of MMP-9. The results of the present study suggest that lead intoxication may adversely affect brain function at least in part by inducing MMP-9 expression through Erk(1/2) activation in primary glial cells.

• Journal of Genetic Medicine
• /
• v.10 no.2
• /
• pp.88-93
• /
• 2013

Alexander disease (ALXD) is a rare demyelinating disease of the white matter of the brain that is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. The overexpression of GFAP in astrocytes induces a failure in the developmental growth of the myelin sheath. The neurodegenerative destruction of the myelin sheath of the white matter is accompanied by an accumulation of abnormal deposits of Rosenthal fibers in astrocytes, which is the hallmark of ALXD. The disease can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. Early-onset disease is more severe, progresses rapidly, and results in a shorter life span than late-onset cases. Magnetic resonance imaging and genetic tests are mostly used for diagnostic purposes. Pathological tests of brain tissue for Rosenthal fibers are definitive diagnostic methods. Therapeutic strategies are being investigated. Ceftriaxone, which is an enhancer of glial glutamate transporter (GLT-1) expression, is currently in clinical trials for the treatment of patients with ALXD. To date, there are no clinically available treatments. The cause, pathology, pathophysiology, inheritance, clinical features, diagnosis, and treatment of ALXD will be reviewed comprehensively.

• Investigative Magnetic Resonance Imaging
• /
• v.18 no.2
• /
• pp.167-170
• /
• 2014

The medial longitudinal fasciculus (MLF) is myelinated composite tract, lying near the midline, ventral to periaqueductal grey matter that plays a key role in coordinating eye movements. A lesion of the MLF results in an ipsilateral adduction deficit and a contralateral abducting nystagmus, referred to as an internuclear ophthalmoparesis. The blended tract with adjacent white matter in pons and midbrain is indistinguishable on brain imaging such as CT and MRI. Until now, to the best of our knowledge, MLF is not delineated on in vivo MRI. We present a case showing the whole connecting courses of MLF lesion on MRI in a patient with inflammatory demyelinating disorder.

• Annals of Clinical Neurophysiology
• /
• v.7 no.2
• /
• pp.133-137
• /
• 2005

Underlying neuropathies combined with amyotrophic lateral sclerosis (ALS) cast doubt on the diagnosis of ALS when present. Abnormal sural nerve conductions were found in 3 patients with clinically definite ALS. Pathologically demyelinating, axonal, or vasculitic neuropathy was suggested respectively. High dose oral corticosteroid had no effect and clinical courses were deteriorating in all the patients. The causes of combined neuropathies were unclear. Possibility of direct consequence of ALS, concomitant neuropathies, or rare variants of ALS should be considered in these cases.

• Journal of Korean Neurosurgical Society
• /
• v.29 no.12
• /
• pp.1682-1687
• /
• 2000

Progressive multifocal leukoencephalopathy(PML) is a fatal demyelinating disease that occurs in immunocompromised hosts. We report a case of PML that developed in patient with T cell lymphoma of head and neck. During chemotherapy for lymphoma, she was confused and had memory impairment. A magnetic resonance imaging of the brain revealed confluent signal change at white matter of the frontal lobe, insula, and anterior limb and genu of internal capsule. The lesion was confirmed with brain biopsy and the histopathological finding was compatible with PML.

• Korean Journal of Veterinary Research
• /
• v.53 no.3
• /
• pp.159-162
• /
• 2013

Galectin-3 is a ${\beta}$-galactoside-binding lectin that plays a role in neuroinflammation through cell migration, proliferation, and apoptosis. In the present study, regulation of galectin-3 was examined in the brain of mice infected with the Daniel strain of Theiler's murine encephalomyelitis virus (TMEV) at days 7 and 81 post-infection by immunohistochemistry. Immunohistochemistry revealed that galectin-3 was mainly localized in ionized calcium-binding adapter 1-positive macrophages/activated microglia, but not in Iba-1-positive ramified microglia. Galectin-3 was also weakly detected in some astrocytes in the same encephalitic lesions, but not in neurons and oligodendrocytes. Collectively, the present findings suggest that galectin-3, mainly produced by activated microglia/macrophages, may be involved in the pathogenesis of virus induced acute inflammation in the early stage as well as the chronic demyelinating lesions in Daniel strain of TMEV induced demyelination model.

• Annals of Clinical Neurophysiology
• /
• v.15 no.2
• /
• pp.68-70
• /
• 2013

Atopic myelitis (AM) is a relatively mild form of myelitis associated with allergic diathesis, and present with predominant sensory manifestations. Lhermitte's sign has been considered as a relatively non-specific clinical sign suggesting demyelinating lesion in cervical cord. Here we report a patient with recurrent AM who presented with isolated Lhermitte's sign, both in first and second attacks. This report suggests that either the diagnosis or recurrence of AM can be frequently underdiagnosed because of its predominant sensory manifestations.

• Journal of Oriental Medical Thermology
• /
• v.5 no.1
• /
• pp.14-20
• /
• 2006

Purpose: The purpose of this study is to report the effect of TKM to both lower extremities weakness after parturition who is diagnosed as Subacute inflammatory demyelinating peripheral neuropathy. Methods: The patient was a 31-year-old woman who couldn't walk at all by herself because of both lower extremities weakness after parturition. The patient was demonstrated as shin-yang-heo, and treated by Pal-mi-ji-hwang-hwan, Acupuncture treatment of Shin-jeong-gyeok, and Moxa treatment of Gwan-won. And the progress of symtoms was evaluated by observation of her walking status, cheking the change of VAS, and inspecting the DITI. Result: The patient could walk by herself. The feeling of both lower extremities weakness improved to VAS 2. The paraesthesia of the soles of feet improved to VAS 4. And the DITI state slightly got better. Conclusion: TKM is expected to have positive effect on puerperium disease especially the symptoms of pain, weakness, paraesthesia, etc.

• Journal of the Korean Child Neurology Society
• /
• v.24 no.3
• /
• pp.71-83
• /
• 2016

X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ATP binding cassette subfamily D member 1 (ABCD1), a gene that encodes peroxisomal membrane located on ABC half-transporter named adrenoleukodystrophy protein (ALDP). X-ALD is characterized by a highly variable clinical spectrum, including progressive cerebral type, adrenomyeloneuropathy, and addison-only phenotype. No genotype/phenotype correlation has been established. Thus, unidentified modifier genes and other co-factors are speculated to modulate the phenotypic variation and disease severity. Recent advanced sequencing methods and reprogramming technologies not only offer an affordable and applicable approach to investigate the pathophysiological mechanisms of adrenoleukodystrophy, but also provide means to develop therapy. A causal therapy of X-ALD is lacking. Lorenzo's oil therapy is recommended for asymptomatic boys, but the longest study found that the oil was not beneficial at all to symptomatic X-ALD patients. Hematopoietic stem cell therapy has a relevant chance of success when performed during this early stage of cerebral type X-ALD. Recently, it has been insisted that lentiviral-mediated gene therapy of hematopoietic stem cells can provide clinical benefits in X-ALD. This review describes current knowledge on the clinical presentation, pathogenesis, diagnosis and management of X- ALD.