• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.39-42
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• 2004

A 20-year-old man with bilateral medial medullary infarction showed transition of bowtie and upbeat nystagmus into hemi-seesaw nystagmus. Follow-up MRI revealed near complete resolution of the right medullary lesion. This transition of nystagmus suggests that the upbeat nystagmus was generated by bilateral lesions in the ascending pathways from both anterior semicircular canals (SCC), and that the hemi-seesaw nystagmus was caused by damage to the pathway from the left anterior SCC.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.43-47
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• 2004

We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache, diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealed tachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showed ptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increased serum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middle mediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biopsy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticosteroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite the chemotherapy for LMS.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.1-13
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• 2004

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that primarily affects motor neurons. The characteristic features of this devastating disorder are the simultaneous presence of upper and lower motor neuron (LMN) signs with progression from one region of the neuraxis to the next and eventual death, typically from respiratory compromise. Electrophysiological studies are an indispensible part of the ALS evaluation, especially serving as an extension of the clinical examination, and most useful in identifying LMN dysfunction. Not only may electrodiagnostic studies reveal characteristic changes in those regions clinically manifesting signs, but it also serves to disclose asymptomatic areas of involvement.

• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.61-63
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• 2004

The copy numbers of the CTG repeats are known to relate to the severity of clinical symptoms for myotonic dystrophy. The positive correlation between clinical manifestations and CTG repeat size has been demonstrated previously. A genetically confirmed myotonic dystrophy patient with 90 CTG repeat number had more severe clinical manifestation than brother with 120 CTG repeats, in adulthood.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.33-36
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• 2009

Bickerstaff's brainstem encephalitis (BBE) is an autoimmune central nervous system disorder. It can occur in more limited forms and may overlap with Guillain-Barr$\acute{e}$ syndrome (GBS). A 49-year-old female presented with rapidly progressive paralytic ileus, urinary retention, deep drowsiness, ophthalmoplegia, dysarthria, ataxia, quadriparesis and hyporeflexia after viral meningitis. She was diagnosed as BBE with GBS and treated with immunoglobulin. She was completely recovered after 1 month. It is a rare case of BBE overlapping with GBS presenting with severe paralytic ileus.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.24-27
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• 2009

Reversible posterior leukoencephalopathy syndrome (RPLS) is an acute encephalopathy, usually characterized by seizures, visual disorder, mental change and reversible posterior white and gray matter lesions on brain magnetic resonance imaging (MRI). Conditions commonly associated with RPLS include acute renal failure, eclampsia, hypertension, immunosuppresion state, and use of intravenous immunoglobulin or chemotherapy, and additional contributing factors. We report the case of RPLS associated with cyclosporine use in lupus nephritis. In this case, it was not clearly elucidated which was main cause of RPLS. We could think RPLS developed maybe due to combined effect of cyclosporine and lupus nephritis.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.28-32
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• 2009

True neurogenic thoracic outlet syndrome (NTOS) is an extremely rare condition, probably occurring with an incidence of one per million. It is often misdiagnosed as carpal tunnel syndrome or benign focal amyotrophy, and careful clinical examination, electrodiagnostic studies, and imaging studies are essential for the correct diagnosis. Here, we report a 23-year-old woman with slowly progressive weakness and atrophy of abductor pollicis brevis who were found to have NTOS caused by cervical rib.

• Annals of Clinical Neurophysiology
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• 제11권1호
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• pp.1-8
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• 2009

Orthostatic intolerance is defined as the development of various symptoms during standing that are relieved by recumbency. Postural tachycardia syndrome (POTS) is another nomenclature of orthostatic intolerance. POTS characterized by a heart rate increase ${\geq}30$ bpm from supine to standing or >120 bpm at standing without orthostatic hypotension. POTS is a heterogenous in presentation with various pathophysiologic mechanisms. Important mechanisms are hypovolemia, denervation, hyperadrenergic and deconditioning state. There are presented as lightheadness or dizziness, palpitations, presyncope, sense of weakness, tremulousness, shortness of breath. POTS are classified under 3 groups that are neuropathic, hyperadrenergic, and deconditioning POTS. Most patients can be improved from a pathophysiologically based regimen of management.

• Annals of Clinical Neurophysiology
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• 제15권1호
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• pp.30-33
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• 2013

• Annals of Clinical Neurophysiology
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• 제15권1호
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• pp.34-36
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• 2013