Multifocal motor neuropathy (MMN) is an uncommon, asymmetric motor neuropathy. As MMN is a treatable disorder, its differentiation from lower motor neuron disease is important. Evidence of conduction block (CB) or positive IgM anti-GM1 is considered one of important markers for the diagnosis. However, some patients with atypical MMN have no detectable CB or anti-GM1 antibody. We experienced a case of MMN with focal nerve enlargement on ultrasound. Ultrasound can be a valuable tool in supporting the diagnosis of MMN.
Diagnosis ; Humans ; Immunoglobulin M ; Motor Neuron Disease ; Peripheral Nervous System Diseases ; Ultrasonography

A 84-year-old woman presented with a two weeks history of dizziness, slurred speech and ataxia. The neurological exam showed spontaneous left beating nystagmus, horizontal gaze evoked nystagmus and limb ataxia. A few weeks earlier, she had an upper airway infection. Brain MRI revealed diffuse leptomeningeal enhancement in the both cerebellar hemisphere and brain single photon emission computed tomography (SPECT) showed hyperperfusion in both cerebellar hemisphere. Extensive laboratory studies with cerebrospinal fluid analysis did not reveal any etiologic factors. She was started on methylprednisolone (1 g/day for 7 days), gradually improved over the weeks. Post infectious cerebellar ataxia is a neurologic complication that occasionally follows systemic viral and bacterial infections. This case demonstrates that cerebellar abnormalities can be detected by brain magnetic resonance imaging and SPECT.
Aged, 80 and over ; Ataxia ; Bacterial Infections ; Brain ; Cerebellar Ataxia ; Cerebrospinal Fluid ; Dizziness ; Female ; Humans ; Magnetic Resonance Imaging ; Methylprednisolone ; Nystagmus, Pathologic ; Tomography, Emission-Computed, Single-Photon

We report a case of proximal internal carotid arterial spasm following pterional removal of pituitary adenoma. We discuss the possible mechanism of vasospasm associated with tumor resection.
Carotid Artery, Internal* ; Pituitary Neoplasms* ; Spasm

HPV E2 protein is known to act as a negative regulator of transcription and the disruption of E2 open reading frame by HPV integration can release suppression of E6 and E7 mRNA expression, resulting in uncontrolled cellular growth and malignant transformation by inactivating tumor suppressor gene products (p53, pRb). YY1 mutation of HPV URR has been suggested as one of indicator that explains development of cervical neoplasia by episomal type of HPV. To extend this hypothesis, we examined whether mutation(s) in specific sites of HPV URR is functionally related to the invasiveness of cervical neoplasia and the physical status of HPV DNA. The URR sequences were obtained by PCR amplification of HPV-16 genome from CIN and invasive cancer patients, cloned into pUC18 for sequencing, and into pBLCAT8+ for functional CAT assay. Our previous data classified HPV-infected patients into three groups: 3 cancer cases carrying episomal HPV DNA; 12 cancer cases carrying integrated HPV DNA; 12 CIN cases carrying episomal HPV DNA. The specific variants in HPV-16 URR were found in Korean women: GA transition at nt 7520 (100%, 27/27), AC transition at nt 7729 (70%; 19/27), and GA transition at nt 7841 (78%; 21/27). Selective mutations were observed at the YY1-binding sites of HPV-16 URR in the 3 patients with invasive cervical cancer, who having the episomal forms of HPV-16 DNA: AC transition at nt 7484 and GA transition at nt 7488 (YY1-binding site 2; from 7481 to 7489). Additionally, CT transition at nt 7785 (YY1-binding site 3; from 7781 to 7790) was found from 2 of 3 patients. No YY1 site mutations were detected in the 12 CIN patients and in the HPV-integrated invasive cancer patients. To determine whether these mutations have effect on the expression of HPV E6/E7 genes driven by URR, the transient transfection assay was employed using URR-CAT reporter plasmid. The relative activities of three URR mutants from episomal HPV-16 DNA of cervical cancers were 2- to 4-fold higher than that of HPV-16 URR prototype. In contrast, the URRs from integrated HPV-16 DNA in cervical cancer and from episomal HPV-16 DNA in CIN, where no mutation of the YY1-binding site was detected, showed similar levels of promoter activity to that of URR prototype.Our results support the hypothesis that the mutation at YY1 binding site is functionally related to the development of cervical neoplasia caused by episomal HPV-16 DNA in Korean cervical cancer patient. Thus, mutation in YY1 site of episomal HPV-16 URR may play a role of HPV integration in the progression of cervical cancer.
Animals ; Binding Sites ; Cats ; Clone Cells ; DNA* ; Female ; Genes, Tumor Suppressor ; Genome ; Human papillomavirus 16* ; Humans ; Open Reading Frames ; Plasmids ; Polymerase Chain Reaction ; RNA, Messenger ; Transfection ; Uterine Cervical Neoplasms

OBJECTIVE: The objectives of the present study were to investigate the annual detection rate of patients with Moyamoya disease (MMD) and to describe the prevalence and epidemiological features of the Moyamoya patients in Korea. MATERIALS AND METHODS: The authors analyzed the epidemiological data of Korean patients taken from the National Health Insurance Corporation in Korea among Moyamoya patients who were treated from 2004 until 2008. RESULTS: Based on 2004 data, 2,539 MMD patients were treated in Korea and the prevalence rate was 5.2 per 100,000 people. There were 2,987 in 2005, 3,429 in 2006, 4,051 in 2007, and 4,517 cases in 2008, and the prevalence rates per 100.000 people were 6.3, 7.0, 8.6, and 9.1, for those respective years. This represents an annual increase of 15% of new cases during this period. In 2008, 466 people were newly diagnosed with MMD, representing an incidence rate of 1 per 100,000 persons. The gender ratio was 1,547 men (34%) and 2,970 women (66%). Women had a higher incidence rate than men (1.94 times). There were two age peaks: teenagers and those in their forties. CONCLUSION: The present study shows that the number of Moyamoya patients in Korea is increasing. This increase could partly be explained by a recent increase in newly diagnosed cases, suggesting that a more careful consideration of the disease and better diagnostic techniques should be promoted among clinicians.
Adolescent ; Female ; Humans ; Incidence ; Korea ; Male ; Moyamoya Disease ; National Health Programs ; Prevalence

OBJECTIVE: In recent years, CyberKnife has emerged as an important treatment modality in the management of pituitary adenomas. Treatment results after performing CyberKnife and the complications of this procedure are reviewed. METHODS: Twenty-six patients with pituitary adenomas received stereotactic radiosurgery with the CyberKnife (CKRS). The follow-up periods ranged from 7 months to 47 months (mean+/-SD : 30+/-12.7 months). The patients consisted of 17 with non-functioning adenomas, 3 with prolactinomas and 6 with acromegaly. The change in the tumor volume, visual acuity, hormonal function, and complications by this therapy were analyzed in each case. RESULTS: The tumor control rate was 92.3%. Hormonal function was improved in all of the 9 (100%) functioning adenomas. Hormonal normalization was observed in 4 of the 9 (44%) patients with a mean duration of 16 months. In two patients (7.6%), visual acuity worsened due to cystic enlargement of the tumor after CKRS. No other complications were observed. CONCLUSION: CyberKnife is considered safe and effective in selected patients with pituitary adenomas. However, longer follow-up is required for a more complete assessment of late toxicity and treatment efficacy.
Acromegaly ; Adenoma ; Follow-Up Studies ; Humans ; Pituitary Neoplasms ; Prolactinoma ; Radiosurgery ; Treatment Outcome ; Tumor Burden ; Visual Acuity

OBJECTIVE: The authors evaluated the effect of direct bypass (superficial temporal artery-middle cerebral artery bypass) in the prevention of rebleeding episodes in patients suffering from hemorrhagic moyamoya disease by comparing this method with indirect bypass. METHODS: Fifteen patients who had hemorrhagic moyamoya without aneurysm comprised the study group. The mean age of patients was 44.4 years and follow up period ranged from 0.8 to 7.1 years (mean; 3.61 years). Revascularization surgery was performed in 21 sides in 15 patients. Direct bypass was performed in 17 sides and indirect bypass in the other 4 sides. RESULTS: During the follow-up period after the revascularization surgery, three sides (14.3%) of the 21 sides presented with rebleeding episode, one of 17 sides (mean follow-up periods; 2.94 years) treated with direct method and 2 of 4 sides (mean follow-up periods; 6.45 years) treated with indirect method. Kaplan-Meier analysis of rebleedingfree survival showed quite different between direct and indirect method but statistically insignificant (p=0.0541). CONCLUSION: Direct bypass may reduce the risk of hemorrhage more effectively than indirect bypass. However, direct bypass cannot always prevent rebleeding.
Aneurysm ; Cerebral Arteries ; Follow-Up Studies ; Hemorrhage* ; Humans ; Kaplan-Meier Estimate ; Moyamoya Disease*

OBJECTIVE: Hemifacial spasm has characteristic and specific electrophysiological finding, lateral spread response(LSR). We study the correlation between change of lateral spread response during microvascular decompression(MVD) and clinical outcome after MVD. METHODS: Sixty two patients with hemifacial spasm who were treated with microvascular decompression from March 2000 to February 2003 were included in this study. The monitoring of intraoperative facial electromyography(EMG) and brain stem auditory evoked potential were performed. RESULTS: In 28 (44.7%) patients, there was persistence of lateral spread response after vascular decompression in root exit zone of facial nerve. Among these 28 patients, 9 had mild hemifacial spasm at discharge. Three out of 34 patients who had intraoperative disappearance of lateral spread response after MVD had mild hemifacial spasm. But Both groups, disappearance of LSR (Group I), and persistence (Group II) had only 2 patients with mild hemifacial spasm, and 5 patients at 3 months, respectively. CONCLUSION: Although intraoperative EMG monitoring is very useful in assessing the efficacy of MVD, the clinical outcome of MVD in patient with hemifacial spasm does not always correlate with EMG finding. The prognostic value of intraoperative LSR monitoring in the long-term results is questionable.
Decompression ; Electromyography ; Evoked Potentials, Auditory, Brain Stem ; Facial Nerve ; Hemifacial Spasm* ; Humans ; Microvascular Decompression Surgery

OBJECTIVE: Patients undergoing intracranial operations often suffer from post-operative epidural hematoma (EDH). The incidence and risk factors for with the occurrence of EDH after intracranial operations are not well described previously. The objective of this study was to identify the risk factors and the incidence of post-operative EDH adjacent and regional to the craniotomy. METHODS: This was a retrospective study of 23 (2.4%) patients, between January 2005 and December 2011, who underwent epidural hematoma evacuation after primary intracranial during this period, 941 intracranial operations were performed. The control group (46 patients) and hematoma group (23 patients) were categorized on the basis of having undergone the same pre-operative diagnosis and treatment within 3 months of their operations. The ages of the hematoma and control group were individually matched to similar ages within 10 years of each other to minimize bias of age. RESULTS: Univariate analysis showed that the significant pre-operative and intra-operative factors associated with post-operative EDH were a pre-operative Glasgow Coma Scale (GCS) scored <8 (crude odds ratio 8.295), prothrombin ratio >1.0 (p=0.014), prothrombin time (PT) >11.3 sec (p=0.008), intra-operative blood loss >650 mL (p=0.003) and craniotomy size >7,420 mm2 (p=0.023). In multivariate analysis, intra-operative blood loss exceeding 650 mL (median of total patients) placed a patient at significantly increased risk for post-operative EDH. CONCLUSION: Recognizing the limitations of the study, large intra-operative blood loss, wide craniotomy area, prolonged PT and a pre-operative GCS <8 are presented implicated with an increased risk of post-operative EDH after intracranial surgery.
Bias (Epidemiology) ; Craniotomy ; Glasgow Coma Scale ; Hematoma ; Hematoma, Epidural, Cranial ; Humans ; Incidence ; Multivariate Analysis ; Odds Ratio ; Prothrombin ; Prothrombin Time ; Retrospective Studies ; Risk Factors

OBJECTIVES: The purpose of this study is to evaluate the radiological charactersitics related to the formation of peritumoral edema in meningiomas. METHODS: Fifty patients with meningioma were examined by magnetic resonance images and cerebral angiography. The predictive factors associated peritumoral edema, such as, tumor size, peritumoral rim (cerebrospinal fluid cleft), shape of tumor margin, signal intensity of tumor in T2WI, and pial blood supply were evaluated. RESULTS: Tumor size, peritumoral rim and pial blood supply correlated with peritumoral edema on univariate analyses. But in multivariate analyses, pial blood supply was statistically significant as a factor for peritumoral edema in meningioma. CONCLUSION: In our results, pial blood supply is significant contributing factor for peritumoral edema in meningioma.
Cerebral Angiography ; Edema* ; Humans ; Meningioma* ; Multivariate Analysis