• Annals of Clinical Neurophysiology
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• v.6 no.1
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• pp.57-60
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• 2004

The sixty two-year-old woman was admitted with facial diplegia and ataxic gait. Neurological examination revealed areflexia and sensory ataxia with decreased sensation of position and vibration in both lower extremities. Electrophysiologic study suggest motor dominant demyelinating polyneuropathy and bilateral facial neuropathy. CSF study revealed no cells and increased proteins. After intravenous immunoglobulin therapy, sensory ataxia and electrophysiological study had markedly improved for 3 months.

• Journal of Korean Neurosurgical Society
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• v.53 no.4
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• pp.245-248
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• 2013

Guillain-Barr$\acute{e}$ syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy. In typical cases, the first symptoms of GBS are pain, numbness, paresthesia, weakness in the limbs. Autonomic involvement is common and causes urinary retention and ileus. Much of these symptoms overlap with those of lumbar spinal stenosis. Therefore, correct diagnosis of GBS in a patient with symptomatic lumbar spinal stenosis or in a patient with atypical manifestations of GBS can be difficult, especially early in the course of GBS. Here, we report on a case of atypical GBS in a 74-year-old previously healthy patient with lumbar spinal stenosis and discuss the differential diagnosis of the GBS and lumbar spinal stenosis.

• Annals of Clinical Neurophysiology
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• v.10 no.1
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• pp.70-73
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• 2008

By definition, the time to reach nadir in Guillain-Barre syndrome (GBS) is within four weeks. This is in contrast to the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which progress for at least two months. However, CIDP can take a relapsing and remitting form and could mimic treatment related fluctuations of GBS (GBS-TRFs) especially during the early phase of disease. We report a patient with CIDP who initially presented with a rapidly progressive limb weakness mimicking GBS, but finally showed good recovery after long term corticosteroid therapy.

• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.31-34
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• 2015

We report a case with squamous cell lung cancer with concomitant Guillain-Barre syndrome (GBS) as a paraneoplastic syndrome. A 67-year-old patient who was previously diagnosed as metastatic squamous cell lung cancer developed mild symmetrical weakness, paresthesia and sensory ataxia. Nerve conduction study showed sensorimotor polyneuropathy. Analysis of cerebrospinal fluid showed high tilter for monospecific anti-GD1b IgG antibody without onconeuronal antibodies. After treatment with intravenous immunoglobulin, the patient's symptoms improved.

• Annals of Clinical Neurophysiology
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• v.7 no.1
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• pp.31-33
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• 2005

Segmental zoster paresis (SZP) is a clinically rare complication of herpes zoster. But it has not been reported that acute cerebral motor cortical infarction coincidentally occurred in SZP. A 86-year-old woman was admitted due to pain, tingling sensation, and weakness of left arm. She had an acute onset of pain and tingling sensation in left arm at first day, shoulder weakness at second day, and multifocal vesicles at fourth day. Deep tendon reflexes of left arm were decreased than right. Electromyography showed an axonal polyneuropathy at superior trunk level of left brachial plexus. Median and ulnar sensory evoked potential tests were normal. Brain MRI showed a high signal in right primary motor cortex on diffusion weighted image. We report a case of acute cerebral motor cortical infarction coincidentally occurred in SZP.

• Annals of Clinical Neurophysiology
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• v.7 no.1
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• pp.28-30
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• 2005

Bilateral brachial neuritis is clinically uncommon and accidentally involvement of bilateral phrenic nerves is rarely reported. We experienced a 26 year old man who developed subacute onset of asymmetric bilateral shoulder and arm weakness. The weakness slowly aggravated and finally suffered from dyspnea due to bilateral phrenic nerve palsy. Cervical spine MRI and CSF study showed no abnormality. Viral markers and other serological test showed no specific finding. Electromyographic study showed bilateral brachial axonal polyneuropathy with cervical and upper thoracic polyradiculopathy. And bilateral phrenic nerve conduction study showed no resopnse. He showed no improvement for 10 months after treatment and managed with continuous artificial ventilation. We report a case of idiopathic bilateral brachial neuritis accidentally involving bilateral phrenic nerves.

• 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 The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.42-47
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• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• Korean Journal of Pharmacognosy
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• v.39 no.2
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• pp.75-79
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• 2008

Nerve growth factor (NGF) is a protein plays a major role in the development and maintenance of central and peripheral nervous system. Recent data suggest that reduced availability of NGF may play a significant role in the pathogenesis of diabetic $polyneuropathy.^{1)}$ In our previous study, steroidal saponin from Liriope platyphylla showed neurotrophic effect by stimulation of NGF synthesis and activation of tyrosine kinase $signaling.^{2)}$ In this study, we examined the neurotrophic effect of Liriope platyphylla (LP); which was from Mylyang(MYL) and Cheongyang(CHE), and Ophiopogon japonicus (CHI) on in vitro and in vivo model for the comparison of their NGF induction. We quantitatively analyzed spicatoside A in the LP and CHI by HPLC. And we investigated the correlation between the contents of spicatoside A and NGF induction efficacy on PC 12 cells and mouse serum. These results suggest that spicatoside A may enhance NGF induction in animal model.

• Annals of Clinical Neurophysiology
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• v.8 no.1
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• pp.48-52
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• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.