• Journal of Environmental Health Sciences
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• v.24 no.3
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• pp.35-40
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• 1998

This study was conducted to examine the pathological changes of rat peripheral nervous system during exposure to tellurium known to be a demyelinating agent by using teasing nerve fiber method and quantitative light microscopic analysis by image analyzer. The pellet containing 1.2% of tellurium were fed for 3, 5, 7, 9, 13 days to male wistar rats (21 days old) and then neurologic symptom and the feature of nerve fiber myelination were studied. From this study, following results were obtained. In 3 days treated group, it showed various neurologic symptom and teased nerve fiber showed slight irregularity of the myeline sheath. In 5 days and 7 days treated groups, it showed the segmental demyetination in larger size fiber and widening of nodes of ranvier. In 9 days and 13 days treated groups, the remyelinated fibers were observed and it was generally small in size. We consequently suggest that teasing nerve fiber method and quantitative analysis of nerve fiber were useful pathologic screening method of neurotoxicity of the peripheral nervous system.

• Biomolecules & Therapeutics
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• v.22 no.2
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• pp.83-92
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• 2014

Fatty acids (FAs) are highly diverse in terms of carbon (C) chain-length and number of double bonds. FAs with C>20 are called very long-chain fatty acids (VLCFAs). VLCFAs are found not only as constituents of cellular lipids such as sphingolipids and glycerophospholipids but also as precursors of lipid mediators. Our understanding on the function of VLCFAs is growing in parallel with the identification of enzymes involved in VLCFA synthesis or degradation. A variety of inherited diseases, such as ichthyosis, macular degeneration, myopathy, mental retardation, and demyelination, are caused by mutations in the genes encoding VLCFA metabolizing enzymes. In this review, we describe mammalian VLCFAs by highlighting their tissue distribution and metabolic pathways, and we discuss responsible genes and enzymes with reference to their roles in pathophysiology.

• Annals of Clinical Neurophysiology
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• v.7 no.1
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• pp.37-42
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• 2005

Acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (Hurst's disease) are rare autoimmune demyelinating disorders, which show a monophasic illness with preceding infection. We report a 42-year-old woman presented with multiphasic and progressive neurologic deterioration without definite evidences of infection. She developed hypesthetic ataxia, followed by ipsilateral weakness after a weak, and finally encephalopathy after a month. In contrast to the first MRI showing a small longitudinal lesion, the next images revealed massive bilateral frontal lesions with hemorrhagic necrosis and biopsy unveiled inflammatory demyelination.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.54-57
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• 2017

Distal acquired demyelinating symmetric (DADS) neuropathy is a variant form of chronic inflammatory demyelinating polyradiculoneuropathy. A 54-year-old man presented with gait disturbance owing to weakness in both legs. Nerve conduction studies showed demyelinating sensorimotor polyneuropathy, and laboratory studies demonstrated anti-GM1 and anti-GD1b IgG antibodies, but no anti-myelin associated glycoprotein activity. We suggest that an antiganglioside antibodies assay needs to be applied when DADS neuropathy is suspected in order to improve the classification of dysimmune neuropathies.

• Journal of Veterinary Clinics
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• v.38 no.2
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• pp.89-93
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• 2021

2-Month-old, three related Perro de Presa Canario dogs were evaluated for similar neurological symptoms like circling, head pressing, depressed mental status, hypermetria, and vocalization. On magnetic resonance imaging (MRI) of the brain, there were large, bilaterally symmetrical lesions with involvement of thalamus, and brainstem that were T2- and FLAIR-hyperintense and T1-iso/hypointense. There was no inclusion of cerebellum. Single-voxel spectroscopy acquisition was located in the thalamus where abnormalities were found in MR images. The results of magnetic resonance spectroscopy (MRS) showed markedly decreased N-acetylaspartic acid value. Euthanasia was performed and lesions consistent with the canine spongy degeneration. Alteration in metabolites in the brain can be determined by MRS, which helps in diagnosing degeneration/leukodystrophy of the central nervous system in dogs.

• Kosin Medical Journal
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• v.33 no.2
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• pp.257-262
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• 2018

Guillain-$Barr{\acute{e}}$ syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.

• Journal of Genetic Medicine
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• v.15 no.2
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• pp.107-109
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• 2018

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is caused by the mutation in GJB1 gene, characterized by the transient central nervous system involvement and long standing peripheral polyneuropathy which does not fulfill the criteria of demyelination or axonopathy. We describe a 37-year-old man with progressive bilateral leg weakness since his early teen. He suffered transient right hemiparesis, followed by quadriparesis at 14 years of age. When we examined him at 37 years of age, he presented a distal muscle weakness on lower extremities with a sensory symptom. The nerve conduction study demonstrated a motor conduction velocity between 26 and 49 m/s. The whole exome sequencing revealed a novel variant c.136 G>A in GJB1. This report will raise awareness in this rare disease, which is frequently misdiagnosed early in its course.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.195-199
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• 2014

The X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disease by defects of ABCD1 gene on chromosome Xq28 leading to accumulation of saturated very long chain fatty acids (VLCFA), progressive demyelination and adrenal insufficiency. A 4-year-old boy was visited hospital with the chief compliant of hyperpigmentation beginning at 2-years old. Serum adrenocorticotropic hormone (ACTH) and cortisol concentration were compatible with adrenal insufficiency. The elevated plasmatic concentration of VLCFA and genotype analysis with sequencing of ABCD1 gene established the diagnosis of X-ALD. Brain MRI showed no abnormal high signal intensity on the white matter. Steroid replacement was started with good response. He initiated Lorenzo's oil with restriction of VLCFA by reducing the intake of fatty foods. The author highlight the importance of suspecting of X-ALD in the etiology of primary adrenal insufficiency as the first sign of the disease.

• Annals of Clinical Neurophysiology
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• v.15 no.1
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• pp.13-18
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• 2013

It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.

• Biomedical Science Letters
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• v.10 no.3
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• pp.285-291
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• 2004

To determine the magnetic resonance (MR) imaging findings and natural history of cerebral fat embolism in a cat model, and to correlate the MR imaging and histologic fmdings. Intemel carotid artery of 11 cats was injected with 0.1 ml of triolein. T2-weighted, T1-weighted and Gd-enhanced T1-weighted images were obtained serially for 2 hours, 1 days, 4 days, 1 week, 2 weeks and 3 weeks after embolization. Any abnormal signal intensity was evaluated. After MR imaging at 3 weeks, brain tissue was obtained for light microscopic (LM) examination using hematoxylin-eosin (HE) and Luxol fast blue staining, and for electron microscopic examination. The LM examination with HE staining revealed normal histological findings in the greater part of an embolized lesion. Cystic change was observed in the gray matter of 8 cats, while in the gray and white matter of 3 cats. At LM examination, Luxol fast blue, staining demonstrated demyelination around the cystic change occurring in the white matter, and EM examination of the embolized cortex revealed sporadic intracapillary fat vacuoles (n=11) and disruption of the blood-brain barrier (n=4). Most lesions were normal, however, and perivascular interstitial edema and cellular swelling were mild compared with the control side. The greater part of an embolized lesion showed reversible findings at MR and histological examination. Irreversible focal necrosis was, however, observed in gray and white matter at weeks 3.