• Annals of Clinical Neurophysiology
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• v.15 no.2
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• pp.53-58
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• 2013

Background: The autophagy is the major route for lysosomal degradation of misfolded protein aggregates and oxidative cell components. We hypothesized that rapamycin (autophagy enhancer) would prolong the survival of motor neuron and suppress the disease progression in amyotrophic lateral sclerosis (ALS). Methods: A total of 24 transgenic mice harboring the human G93A mutated SOD1 gene were used. The clinical status involving rotarod test and survival, and biochemical study of ALS mice model were evaluated. Results: The onset of symptoms was significantly delayed in the rapamycin administration group compared with the control group. However, after the clinical symptom developed, the rapamycin exacerbated the disease progression and shortened the survival of ALS mice model, and apoptosis signals were up-regulated compared with control group. Conclusions: Even though further detailed studies on the relevancy between autophagy and ALS will be needed, our results revealed that the rapamycin administration was not effective for being novel promising therapeutic strategy in ALS transgenic mice and exacerbated the apoptosis.

• Annals of Clinical Neurophysiology
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• v.8 no.2
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• pp.203-205
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• 2006

There have been several reports about coexistence of myasthenia and other autoimmune disease. Psoriasis is a papulosquamous disease defined by erythematous plaques with a silvery scale and a T-cell-mediated autoimmune disease. We report a case of a 49-year-old man with generalized myasthenia gravis (MG) superimposed by psoriasis. MG was diagnosed by clinical symptoms, increased acetylcholine receptor antibody titer and repetitive nerve stimulation test. Psoriasis was diagnosed by clinical manifestations and specific skin biopsy findings. MG and psoriasis are both autoimmune diseases. The coexistence of MG and psoriasis suggest a close connection of pathogenesis.

• Annals of Clinical Neurophysiology
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• v.5 no.1
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• pp.11-15
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• 2003

Backgrounds: Kennedy disease is a X-linked recessive disease characterized by bulbar symptoms, proximal muscle weakness, and gynecomastia. Methods: We analyzed clinical symptoms and performed electrodiagnostic studies on 6 patients. Results: We found following features: 1) proximal muscle weakness 2) bulbar symptoms, as dysarthria, facial and tongue atrophy 3) hyporeflexia or areflexia 4) fasciculations, predominantly on face, and proximal upper extremities 5) decreased sensory nerve action potentials(SNAPs) 6) chronic neurogenic changes in needle EMG. Conclusions: Kennedy disease is characterized by degenerative process of anterior horn cell and dorsal root ganglion without upper motor neuron dysfunction. Increased triple nucleotide CAG repeats(>38) in androgen receptor gene of Xp21 will confirm early stage of this disease.

• The Journal of Internal Korean Medicine
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• v.40 no.5
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• pp.851-864
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• 2019

Multiple system atrophy is a neurodegenerative disease that causes diverse bodily dysfunctions (cerebellar, pyramidal, automatic, and urological, in any combination), as well as Parkinsonism. Patients with multiple system atrophy commonly display antecollis, a condition where the patient's head tilts forward more than 45 degrees. Despite its common occurrence in these patients, no current standardized therapies are effective for treating antecollis. In this study, Korean medicinal treatments, including Chuna manual therapy, pharmaco-acupuncture, bee venom acupuncture, acupuncture, herbal medicine, and moxibustion therapy, were administered to the patient over a 27-day period. After the treatment, assessments of the head position on the EPIS-PD scale (Part I) and at a standing position from the side (Part II) both revealed improvements. As the head flexion angle decreased, the patient's head posture improved, as determined by a decrease in angle from 80 degrees to 30 degrees in the upright, standing position. As a result, patients who previously were unable to walk without the support of walking frames could now roam freely and independently, with significant increases in both walking speed and distance. In essence, this study suggests that Korean medicine is an effective treatment for patients with multiple system atrophy who suffer from antecollis and gait disorders.

• The Journal of the Society of Stroke on Korean Medicine
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• v.23 no.1
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• pp.73-84
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• 2022

■Objective The purpose of this case report is to report the effect of Korean medicine treatment on a patient with pontine infarction complaining of discomfort due to aggravated voiding dysfunction after the onset of the stroke. ■Methods The patient was hospitalized for 44 days and treated with herbal medicine, acupuncture, electroacupuncture, moxibustion, and interferential current therapy(ICT). We checked the amount of self-voided volume and postvoid residual urine volume(PVR), and asked the patient to subjectively evaluate the degree of discomfort due to voiding dysfunction. ■Results After 44 days of hospitalization, the patient responded that the subjective discomfort due to residual urine remained 30% and delayed urine remained 50% compared to the day of admission. The maximum amount of PVR was 234 ml on the 3^rd day, and the minimum amount of PVR was checked on 25^th and 35^th day as 0 ml. ■Conclusions This study suggests that Korean medicine treatment may be effective in the treatment of voiding dysfunction after stroke.

• The Journal of Korean Medicine
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• v.44 no.3
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• pp.150-162
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• 2023

Meniere's disease is characterized by episodic vertigo, fluctuating hearing loss, tinnitus and ear fullness. The main pathological finding is endolymphatic hydrops, but the etiology of disease and effective treatment methods are still disputed. Recently, Cervical spine disorders(CSD) and Temporomandibular disorders(TMD) have been attracting attention as one of the causes of Meniere's disease. A 65-year-old female Meniere patient with musculoskeletal problems in the cervical and mandible area was treated by Korean medical therapies including pharmacoacupuncture treatment. After 5 weeks of treatment, there was no meaningful change in hearing level evaluated with pure tone audiometry, but the subjective symptoms of Meniere improved significantly. Numerical rating scale (NRS) decreased from 10 to 0 for hearing loss, 10 to 3 for tinnitus and 8 to 3 for ear fullness. Also NRS of cervicalgia was reduced from 5 to 0 after treatment. The result suggests that the Korean medical therapy including pharmacoapuncture targeting CSD and TMD could be safe and effective method for patients with Meniere's disease.

• Annals of Clinical Neurophysiology
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• v.19 no.1
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• pp.40-45
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• 2017

Background: Telbivudine is a nucleoside analogue used for the treatment of chronic hepatitis B, but it often develops mitochondrial toxicity leading to symptomatic myopathy. In this study, three patients with telbivudine induced myopathy were enrolled in order to investigate the nature and pathogenesis of mitochondrial toxicity caused by long-term use of telbivudine. Methods: Clinical features, laboratory findings, muscle pathology, and quantitation of mitochondrial DNA were studied in three patients. Results: Patients presented with progressive muscle weakness with high serum creatine kinase levels. Light microscopic findings of muscle pathology showed ragged red fibers that reacted strongly with succinate dehydrogenase stain, but negative for cytochrome c oxidase activities. Electron microscopy revealed abnormal mitochondrial accumulation with rod shaped inclusions. The quantitative peroxidase chain reaction showed a depletion of mitochondrial DNA in skeletal muscle of the patients. Conclusions: Nucleoside analogues including telbivudine are potent inhibitors of viral DNA polymerases. However, they are not specific for viral DNA and can disturb mitochondrial replication at the same time. All nucleotide analogues should be used with close clinical observation in order to avoid development of mitochondrial myopathy.

• Dementia and Neurocognitive Disorders
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• v.23 no.1
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• pp.11-21
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• 2024

Background and Purpose: Although dementia with Lewy bodies (DLB) is the second most common cause of neurodegenerative dementia, its clinical prevalence is low. We developed a short and easy-to-complete DLB screening questionnaire (DLBSQ) to raise diagnostic sensitivity in routine clinical settings. Methods: A total of 501 participants were retrospectively enrolled, including 71 controls, 184 patients without DLB, and 246 patients with probable DLB. All patients underwent clinical evaluation, including core features of DLB, the DLBSQ, brain magnetic resonance imaging, and detailed neuropsychological assessments. The diagnostic performance of the DLBSQ for probable DLB was investigated using a receiver operating characteristic curve analysis. Results: Total DLBSQ score was associated with visuospatial and frontal/executive dysfunction and the diagnosis of probable DLB. The area under the receiver operating characteristic curve for total DLBSQ score was 0.727. Youden's method revealed an optimal cutoff value of 3. The sensitivity and specificity of the DLBSQ were 68.7% and 62.4%, respectively. Its discriminating performance improved when cognitive test profiles were additionally considered (area under the curve: 0.822, sensitivity: 80.6%, and specificity: 70.4%). Conclusions: The DLBSQ might be a useful screening tool for DLB in routine clinical practice with good sensitivity and specificity.

• Annals of Clinical Neurophysiology
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• v.23 no.2
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• pp.108-116
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• 2021

Background: Nitrous oxide (N₂O) is used in surgery and dentistry for its anesthetic and analgesic effects. However, neurological and psychiatric manifestations of N₂O abuse have been increasingly reported among Korean adults. The aim of this study was to demonstrate laboratory findings of N₂O abuse in Korean patients. Methods: Patients diagnosed with N₂O-induced neuropathy or myelopathy from August 2018 to December 2019 were enrolled. Their clinical presentations and laboratory and imaging findings were analyzed. Results: Sensory changes and limb weakness were present in nine of the enrolled patients. The laboratory findings revealed that seven patients had high homocysteine levels and five had high methylmalonic acid levels in their blood. Nerve conductions studies indicated that axonal neuropathy was present in four cases and longer F-wave and Hoffman's-reflex latencies were present in two cases. Signal changes in cervical spine imaging occurred in five patients, while two had normal results. Conclusions: Chronic N₂O abuse can cause neurological damage or psychiatric problems. Because N₂O is illegal for recreational use in Korea, patients tend to hide their history of use. Even though the spinal imaging results were normal, clinicians should consider the possibility of N₂O use, and further electrophysiological tests should be applied for precise evaluations.

• Journal of clinical neurology
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• v.18 no.2
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• pp.207-213
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• 2022

Background and Purpose Neuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating disease of the central nervous system (CNS). We investigated the medical behaviors of experts in Korea when they are diagnosing and treating NMOSD. Methods An anonymous questionnaire on the diagnosis and treatment of NMOSD was distributed to experts in CNS demyelinating diseases. Results Most respondents used the 2015 diagnostic criteria for NMOSD and applied a cerebrospinal fluid examination, magnetic resonance imaging (MRI) of the brain and spine, and anti-aquaporin-4 antibody testing to all suspected cases of NMOSD. All respondents prescribed steroid pulse therapy as an first-line therapy in the acute phase of NMOSD, and 67% prescribed azathioprine for maintenance therapy in NMOSD. However, details regarding monitoring, the tapering period of oral steroids, second-line therapy use in refractory cases, management during pregnancy, and schedule of follow-up MRI differed according to the circumstances of individual patients. We analyzed the differences in response rates between two groups of respondents according to the annual number of NMOSD patients that they treated. The group that had been treating ≥10 NMOSD patients annually preferred rituximab more often as the second-line therapy (p=0.011) and had more experience with rituximab treatment (p=0.015) compared with the group that had been treating <10 NMOSD patients. Conclusions This study has revealed that NMOSD experts in Korea principally follow the available treatment guidelines. However, the differences in specific clinical practices applied to uncertain cases that have been revealed will need to be investigated further in order to formulate suitable recommendations.