• Annals of Clinical Neurophysiology
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• 제17권2호
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• pp.73-75
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• 2015

A 69-year-old woman presented with a progressive limb weakness. Both clinical and neurophysiological findings were consistent with diagnosis of Guillain-$Barr{\acute{e}}$ syndrome (GBS). Two days after admission, the patient suffered from an acute coronary syndrome without stenosis at coronary arteriography. Echocardiography revealed left ventricular inferior wall and apical akinesia and decreased ejection fraction. A diagnosis of Takotsubo cardiomyopathy was then made. Left ventricular dysfunction and electrocardiography normalized within one month. Takotsubo cardiomyopathy can be developed as a complication of GBS.

• Annals of Clinical Neurophysiology
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• 제3권2호
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• pp.151-155
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• 2001

SCA 1 is an autosomal dominant disorder. The phenotypic manifestations of SCA 1 are not specific, and thus, the diagnosis of SCA 1 rests on molecular genetic testing. The number of CAG repeats ranges from 6-44 in normal alleles and from 39-81 repeats in disease-causing alleles(chromosomal locus 6p22-23). The main clinical features of SCA 1 are ataxia, dysarthria, ophthalmoparesis, extrapyramidal signs without retinal degeneration. A 24-year-old woman with suspected family history presented with progressive cerebellar ataxia, dysarthria, ptosis, titubation and general weakness. Brain MRI revealed a moderate cerebellar atrophy. A genomic polymerase chain reaction(PCR) analysis showed 66 repeats at the SCA 1 locus.

• Annals of Clinical Neurophysiology
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• 제24권2호
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• pp.73-78
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• 2022

Some cases of myasthenia gravis (MG) with abnormal spontaneous activity (ASA) in needle electromyography (EMG) have been reported, but the associated clinical characteristics remain to be fully elucidated. We report the case of a 36-year-old male with MG in whom ASA was observed. This study highlights that ASA may appear in needle EMG in patients with severe MG who predominantly have bulbar and/or respiratory involvement. Care is needed because this often accompanies myopathic features and can be misdiagnosed as myopathy.

• Annals of Clinical Neurophysiology
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• 제23권2호
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• pp.130-133
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• 2021

X-linked Charcot Marie Tooth disease type 1 (CMTX1) is a clinically heterogenous X-linked hereditary neuropathy caused by mutation of the gene encoding gap junction beta 1 protein (GJB1). Typical clinical manifestations of CMTX1 are progressive weakness or sensory disturbance due to peripheral neuropathy. However, there have been some CMTX1 cases with accompanying central nervous system (CNS) manifestations. We report the case of a genetically confirmed CMTX1 patient who presented recurrent transient CNS symptoms without any symptom or sign of peripheral nervous system involvement.

• Annals of Clinical Neurophysiology
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• 제25권1호
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• pp.50-53
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• 2023

Tram-track and doughnut-shaped enhancements of the optic nerve sheath in axial and coronal magnetic resonance imaging (MRI) views, respectively, play crucial roles in the diagnosis of optic nerve sheath meningioma (ONSM). However, this finding is not specific to ONSM since it can also be observed in optic perineuritis (OPN). Here we report a 42-year-old female with ONSM who presented with clinical and MRI findings similar to those of OPN.

• Annals of Clinical Neurophysiology
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• 제25권2호
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• pp.55-65
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• 2023

The ultimate role of ocular movements is to keep the image of an object within the fovea and thereby prevent image slippage on the retina. Accurate evaluations of eye movements provide very useful information for understanding the functions of the oculomotor system and determining abnormalities therein. Such evaluations also play an important role in enabling accurate diagnoses by identifying the location of lesions and discriminating from other diseases. There are various types of ocular movements, and this article focuses on saccades, fast eye movements, smooth pursuit, and slow eye movements, which are the most important types of eye movements used in evaluations performed in clinical practice.

• Annals of Clinical Neurophysiology
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• 제15권2호
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• pp.42-47
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• 2013

Background: Diffusion-weighted image (DWI) might be useful to predict the prognosis of acute hypoxic encephalopathy. The aim of our study was to test whether the early change and extent of DWI abnormalities can be an indicator of the clinical outcome of hypoxic encephalopathy. Methods: Forty-four patients who were diagnosed as hypoxic encephalopathy due to the cardiorespiratory arrest were retrospectively identified. Clinical variables were determined, and the DWI abnormalities were counted by four areas: cortex, subcortical white matter, cerebellum and deep grey matter, and were divided into three groups by the extent of lesions. Prognosis was classified as 'poor' (Glasgow coma scale (GSC) at 30 days after arrest <9 or death) and 'good' (GSC at 30 days after arrest ${\geq}9$). Results: GCS at day 3 (p<0.001), presence of seizure (p=0.01), and presence of lesion (p<0.001) were significantly different in prognosis, but statistically there is no association with the extent of lesions and prognosis (p=0.26). Conclusions: Presence of early DWI changes could predict the clinical outcome of hypoxic encephalopathy after cardiorespiratory arrest.

• Annals of Clinical Neurophysiology
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• 제21권2호
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• pp.87-93
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• 2019

Background: The standardized autonomic function test has become widely available. However, there are no reference data for this test for the Korean population. This study explored reference data for sudomotor and cardiovagal function tests for the Korean population. Methods: The sweat volume by quantitative sudomotor axon reflex test, heart-rate response to deep breathing (HRdb), expiration:inspiration (E:I) ratio, and Valsalva ratio (VR) were measured in 297 healthy Korean volunteers aged from 20 to 69 years. Multivariate regression analysis was performed to evaluate the effects of age, sex, and body mass index on these variables. The 2.5th, 5th, 10th, 90th, 95th, and 97.5th percentile values were obtained for each investigation. Results: The sweat volume was higher in males than in females. The HRdb and E:I ratio were negatively correlated with age, and were higher in males than in females. The VR was negatively correlated with age, but it was not correlated with sex. Conclusions: This study has provided data on the reference ranges for sudomotor and cardiovagal function tests in healthy Korean adults.

• Annals of Clinical Neurophysiology
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• 제15권1호
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• pp.7-12
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• 2013

Background: Local steroid injection is used to treat carpal tunnel syndrome (CTS). The aim of this study was to evaluate the clinical and electrophysiological effects of local steroid injection in patients with CTS over a 3-months period. Methods: Twenty-one patients (35 hands) with clinical and electrophysiological evidence of CTS were treated by injection of triamcinolone 40 mg to the carpal tunnel. Visual analog scale (VAS), Boston Carpal Tunnel Questionnaire (BCTQ), rates of paresthesia, night awakening, and electrophysiological studies were used as outcomes. Clinical and electrophysiological assessments were performed before, 1 and 3 months after treatment. Results: Prior to treatment, 86% of patients complained of night awakening. At 1 and 3 months after injection, only 17% and 29% of the patients, respectively, had night awakening (p<0.001). All patients complained of paresthesia before the treatment. This symptom disappeared in 60% and 31% of the patients after 1 and 3 months, respectively (p<0.001). Compared to baseline, both BCTQ and VAS show significant improvement during the 3 months of the study (p<0.005). Although significant improvements in clinical parameters were shown, electrophysiological parameters were not significantly improved at 1 and 3 months. Conclusions: Local corticosteroid injection for the treatment of CTS provides significant improvement in symptoms for 3 months. On the other hand, no significant improvement was observed in electrophysiological parameters.

• Annals of Clinical Neurophysiology
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• 제3권1호
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• pp.43-46
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• 2001

Acute pandysautonomic neuropathy(APN) is an uncommon clinical entitiy involving vasomotor, sudomotor, pupilomotor, secretomotor and other autonomic systems. Both sympathetic or parasympathetic fibers are involved with relative preservation of somatic sensory and motor function. Although APN shares several clinical features with GBS, it is not clear whether APN is a subvariety of GBS. We report two young patients with APN. Patient 1 was a 18-year-old girl with recurrent fainting spells. Patient 2 was a 23-year-old man sufferring from unexplained nausea and vomiting. Both had a history of previous upper respiratory infection. They presented with gastroparesis, anhydrosis and orthostatic hypotension. Mild numbness and tingling sense was present, but motor power was intact. Neurologic examination showed bilateral tonic pupil, decreased pain and vibration sense, and absent tendon reflexes. Nerve conduction study indicated diffuse sensorimotor polyneuropathy. Nerve biopsy in patient 2 revealed axonal degeneration. After conservative management, gastrointestinal symptoms were improved in patient 2, however, patient 1 suffered from the symptoms lasting more than several months. These cases suggest that post-infectious dysautonomic symptoms in young patient may indicate the diagnosis of APN. Although the natural course is generally benign, accurate diagnosis and proper management may be mandatory for the better clinical outcome.