• Annals of Clinical Neurophysiology
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• 제6권1호
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• pp.48-51
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• 2004

The occurrence of polyneuropathy in association with monoclonal gammopathy of undetermined significance (MGUS) is quite common. However, reports of MGUS associated cranial neuropathies are rare. A 63 year-old women was presented with diplopia and swallowing difficulty. Neurological examination showed limitation of abduction of right eye, right peripheral facial palsy, decreased hearing and gag reflex, left side deviation of uvula, and decreased DTR. Sensorimotor polyneuropathy were observed with elctrophysiological studies. Protein and immunoelectrophoresis revealed IgG ${\kappa}$monoclonal gammopathy. She was treated with intravenous immunoglobulin and steroid, and her symptoms and signs were improved. This case suggested that she had sensorimotor polyneuropathy and multiple cranial neuropathies associated with IgG ${\kappa}$MGUS.

• Journal of Yeungnam Medical Science
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• 제33권1호
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• pp.1-7
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• 2016

The two distinctive clinical features of varicella-zoster virus (VZV) are varicella (chickenpox) by primary infection and zoster (singles) by the reactivation of latent infection. In addition to the two typical clinical symptoms mentioned above, diverse clinical manifestations have been reported as a result of VZV reactivation, including chronic radicular pain without rash, visual loss, facial palsy, dysphagia, sore throat, odynophagia, otalgia, hearing loss, dizziness, headache, hemiplegia, etc. Most of these symptoms are derived from neuropathy and vasculopathy of affected nerves and arteries. Diagnosis of VZV disease can be difficult if there is no appearance of a skin rash during development of atypical symptoms. In addition to natural infection, vaccination and anti-viral agent treatment have influenced the changes of epidemics and clinical presentations of varicella and zoster. In this article, diverse clinical manifestations caused by VZV reactivation, particular without skin rash, are reviewed.

• Investigative Magnetic Resonance Imaging
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• 제23권2호
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• pp.172-174
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• 2019

Upon review, it is noted that recurrent painful ophthalmoplegic neuropathy (RPON) is a rare neurological syndrome characterized by recurrent unilateral headaches and painful ophthalmoplegia of the ipsilateral oculomotor nerve. As seen on brain MRI, thickening and enhancement of the oculomotor cranial nerve can be observed in these cases. We experienced a case of RPON in an adult patient who showed thickening and enhancement of the oculomotor nerve on gadolinium-enhanced 3D-FLAIR image. The authors report a case of RPON with a review of the literature.

• Journal of Oral Medicine and Pain
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• 제41권4호
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• pp.200-204
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• 2016

Numb chin syndrome, or mental neuropathy is a rare sensory neuropathy characterized by abnormal sensation such as hypoesthesia, paresthesia, or dysesthesia in the chin and lower lip innervated by the mental nerve. Sensory neuropathy of mental nerve is somewhat familiar to dentists because it can occur following nerve damage by trauma or dental treatment such as implant surgery or third molar extraction. It can also result from dental causes including abscess or osteomyelitis. However, it can be the first sign of the systemic disease or malignancy if it is not related to dental causes. In this study, we present the case of a patient who present with hypoesthesia and pain in chin area without other symptoms and is later diagnosed with diffuse large B-cell lymphoma.

• Journal of Cerebrovascular and Endovascular Neurosurgery
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• 제20권4호
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• pp.241-247
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• 2018

Treatment of paraclinoid aneurysms weather by surgery, or endovascular embolization has a risk of visual loss due to optic neuropathy, or diplopia due to cranial nerve palsies. Visual complications occur immediately after the clipping, whereas they can occur variable time after endovascular coiling. Recently, endovascular coiling for paraclinoid aneurysm is regarded as a safe and feasible treatment. But it still has risks of acute thromboembolic complication, or cranial nerve palsies. A 45-year-old woman was referred from local hospital to our hospital due to ruptured large ICA dorsal wall aneurysm. A total of 12 coils (195 cm) were used for obliteration of aneurysm. Postoperative diffusion weighted image showed no abnormal signal intensity lesion and magnetic resonance angiography demonstrated no sign of vasospasm, or vessel narrowing. But, she complained visual problem 23 days after coil embolization. Ophthalmologist confirmed the left optic disc atrophy on fundoscopy. Although steroid was started, but monocular blindness did not recover completely. The endovascular embolization of paraclinoid aneurysm, especially projecting superiorly with large irregular shape, has the risk of progressive visual loss because of the proximity to optic nerve.

• Annals of Clinical Neurophysiology
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• 제24권2호
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• pp.90-92
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• 2022

AGel amyloidosis is an autosomal dominantly inherited disease caused by a GSN mutation, and affected patients typically present with the clinical triad of corneal lattice dystrophy, progressive cranial neuropathy, and cutis laxa. We report a Korean family with AGel amyloidosis with predominant manifestations of facial and bulbar muscle weakness. Whole-exome sequencing revealed a common missense mutation (p.Asp214Tyr) in GSN. This case strongly suggests that AGel amyloidosis should be considered when a patient presents with progressive facial and bulbar palsies.

• Journal of Korean Neurosurgical Society
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• 제59권6호
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• pp.570-576
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• 2016

Objective : Elevated cell counts and protein levels in cerebrospinal fluid (CSF) result from disease activity in patients with leptomeningeal carcinomatosis (LMC). Previous studies evaluated the use of CSF profiles to monitor a treatment response or predict prognosis. CSF profiles vary, however, according to the sampling site and the patient's systemic condition. We compared lumbar and ventricular CSF profiles collected before intraventricular chemotherapy for LMC and evaluated the association of these profiles with patients' systemic factors and LMC disease activity. Methods : CSF profiles were retrospectively collected from 228 patients who underwent Ommaya reservoir insertion for intraventricular chemotherapy after a diagnosis of LMC. Lumbar samples taken via lumbar puncture were used for the diagnosis, and ventricular samples were obtained later at the time of Ommaya reservoir insertion. LMC disease activity was defined as the presence of LMC-related symptoms such as increased intracranial pressure, hydrocephalus, cranial neuropathy, and cauda equina syndrome. Results : Cell counts (median : 8 vs. 1 cells/mL) and protein levels (median : 68 vs. 17 mg/dL) significantly higher in lumbar CSF than in ventricular CSF (p<0.001). Among the evaluated systemic factors, concomitant brain metastasis and previous radiation were significantly correlated with higher protein levels in the lumbar CSF (p=0.01 and <0.001, respectively). Among the LMC disease activity, patients presenting with hydrocephalus or cauda equina syndrome showed higher lumbar CSF protein level compared with that in patients without those symptoms (p=0.049 and p<0.001, respectively). The lumbar CSF cell count was significantly lower in patients with cranial neuropathy (p=0.046). The ventricular CSF cell counts and protein levels showed no correlation with LMC symptoms. Carcinoembryonic antigen (CEA), which was measured from ventricular CSF after the diagnosis in 109 patients, showed a significant association with the presence of hydrocephalus (p=0.01). Conclusion : The protein level in lumbar CSF indicated the localized disease activity of hydrocephalus and cauda equina syndrome. In the ventricular CSF, only the CEA level reflected the presence of hydrocephalus. We suggest using more specific biomarkers for the evaluation of ventricular CSF to monitor disease activity and treatment response.

• Journal of Korean Neurosurgical Society
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• 제30권11호
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• pp.1308-1313
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• 2001

Object : The goals of radiosurgery include preservation of neurological function and prevention of tumor growth. We document the results of gamma-knife radio-surgery for vestibular schwannoma. Method & Object : Eighty-two patients underwent stereotactic radiosurgery for an vestibular schwannoma from October, 1994 to December, 2000. Sixty-five of these patients were followed up for radiological and clinical evaluation. As pregamma-knife modality, surgical resection were done in 23 patients,and V-P shunt in 2 patients. Initial symptoms were headache(n=45), dizziness(n=16), tinnitus(n=17). While normal facial function(House-Brackmann grade 1) was present in 48 patients(73.8%), other patients showed grade 2 function in 8, grade 3 function in 7,and grade 4 function in 2. The Gardner/Robertson scale was used to code hearing function. Male to female ratio was 1:3. Mean tumor volume was $7.98cm^3$. Mean dose delivered to the tumor margin was 14.2Gy,and mean maximal dose was 28.3Gy. Results : Mean follow-up duration of 19.9 months. Thirty-five showed decrease(53.8%) in size, 19 patients(29.2%) stationary, 3(4.6%) initial decrease follow up increase, 5(7.6%) initial increase follow up decrease,and 59 patients (90.8%) were well controlled. Two patients experienced transient facial neuropathy, one transient trigeminal neuropathy, and one transient hearing deterioration. After gamma-knife radiosurgery, ventriculoperitoneal shunt was done in 4 patients. Conclusions : Gamma-knife radiosurgery can be used to treat postoperative residual tumors as well as in patients with concomitant medical problems in patients with preserved hearing function. Gamma-knife radiosurgery is safe and effective method to treat small, medium sized(less than 3cm in extracanalicular diameter), intracanalicular vestibular schwannoma, associated with low rate of cranial neuropathy.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제38권3호
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• pp.177-183
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• 2012

Herpes zoster is a viral infection caused by the reactivation of the varicella zoster virus, an infection most commonly affecting the thoracolumbar trunk. Herpes Zoster Infection (HZI) may affect the cranial nerves, most frequently the trigeminal. HZI of the trigeminal nerve distribution network manifests as multiple, painful vesicular eruptions of the skin and mucosa which are innervated by the infected nerves. Oral vesicles usually appear after the skin manifestations. The vesicles rupture and coalesce, leaving mucosal erosions without subsequent scarring in most cases. The worst complication of HZI is post-herpetic neuralgia; other complications include facial scarring, motor nerve palsy and optic neuropathy. Osteonecrosis with spontaneous exfoliation of the teeth is an uncommon complication associated with HZI of the trigeminal nerve. We report several cases of osteomyelitis appearing on the mandible, caused by HZI, and triggering osteonecrosis or spontaneous tooth exfoliation.

• Radiation Oncology Journal
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• 제35권3호
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• pp.268-273
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• 2017

Purpose: We performed this retrospective study to investigate the outcomes of patients with hemangioma of the cavernous sinus after fractionated radiotherapy. Materials and Methods: We analyzed 10 patients with hemangioma of the cavernous sinus who were treated with conventional radiotherapy between January 2000 and December 2016. The median patient age was 54 years (range, 31-65 years), and 8 patients (80.0%) were female. The mean hemangioma volume was $34.1cm^3$ (range, $6.8-83.2cm^3$), and fractionated radiation was administered to a total dose of 50-54 Gy with a daily dose of 2 Gy. Results: The median follow-up period was 6.8 years (range, 2.2-8.8 years). At last follow-up, the volume of the tumor had decreased in all patients. The average tumor volume reduction rate from the initial volume was 72.9% (range, 18.9-95.3%). All 10 of the cranial neuropathies observed before radiation therapy had improved, with complete symptomatic remission in 9 cases (90%) and partial remission in 1 case (10%). No new acute neurologic impairments were reported after radiotherapy. One probable compressive optic neuropathy was observed at 1 year after radiotherapy. Conclusion: Fractionated radiotherapy achieves both symptomatic and radiologic improvements. It is a well-tolerated treatment modality for hemangiomas of the cavernous sinus.