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

Orbital infarction syndrome is a rare complication of neurosurgical procedures. The authors recently experienced two patients suffered from acute proptosis, ophthalmoplegia, and blindness developed immediately after surgery for ruptured anterior communicating artery aneurysms. Both patients underwent standard frontotemporal craniotomies to clip their aneurysms. Retinal and choroidal nonperfusion, and ophthalmoplegia, which suggested hypoperfusion of the ophthalmic artery and its branches, consistent with the orbital infarction. We report two cases of orbital infarction syndrome and discuss possible mechanism with literature review.
Aneurysm ; Blindness ; Choroid ; Craniotomy ; Exophthalmos ; Humans ; Infarction* ; Intracranial Aneurysm* ; Neurosurgical Procedures ; Ophthalmic Artery ; Ophthalmoplegia ; Orbit* ; Retinaldehyde

Pneumocephalus is commonly seen after craniofacial injury. The pathogenesis of pneumocephalus has been debated as to whether it was caused by ball valve effect or combined episodic increased pressure within the nasopharynx on coughing. Discontinuous exchange of air and cerebrospinal fluid due to “inverted bottle” effect is assumed to be the cause of it. Delayed tension pneumocephalus is not common, but it requires an active management in order to prevent serious complication. We represent a clinical case of a 57-year-old male patient who fell down from 3 m height, complicated by tension pneumocephalus on 5 months after trauma. We recommend a surgical intervention, but the patient did not want that so we observe the patient. The patient was underwent seizure and meningitis after 7 months after trauma, he came on emergency room on stupor mentality. Tension pneumocephalus may result in a neurologic disturbance due to continued air entrainment and it significantly the likelihood of intracranial infection caused by continued open channel. Tension pneumocephalus threat a life, so need a neurosurgical emergency surgical intervention.
Cerebrospinal Fluid ; Cerebrospinal Fluid Leak ; Cough ; Craniocerebral Trauma ; Emergencies ; Emergency Service, Hospital ; Humans ; Male ; Meningitis ; Middle Aged ; Nasopharynx ; Pneumocephalus* ; Seizures ; Stupor

OBJECTIVE: Extracranial-intracranial arterial bypass (EIAB) has proved to be useful in selected patients with artherosclerotic cerebral ischemia and moyamoya disease. But neurological deterioration (ND) after EIAB has occasionally been reported in spite of successful EIAB. We have performed EIAB in 150 patients with artherosclerotic cerebral ischemia and moyamoya disease during the recent 8 years. We analyzed the patients who exhibited ND after successful EIAB was performed for a selected group of patients with artherosclerotic cerebral ischemia and moyamoya disease. METHODS: Among 150 patients, the cause of the hemodynamic ischemia was atherosclerotic in 90 and moyamoya disease in 60. Eighteen patients experienced ND after successful EIAB. There were 14 patients with temporary neurologic deficit and 5 patients had a permanent deficit. We divided these 18 patients into two groups. Group 1 revealed relative hyperperfusion of a chronically hypoperfused area of the brain after successful EIAB. Group 2 showed hypoperfusion of the brain by the change of the flow pattern after successful EIAB. RESULTS: Of the 18 patients who experienced ND after successful EIAB, 8 patients belonged to group 1 and 10 patients belonged to group 2. We divided group II into four subgroups according to angiographic flow patterns. The first subgroup (2 patients) showed delayed filling of one division out of two divisions of the middle cerebral artery. The second subgroup (3 patients) showed collision between the orthograde flow and the retrograde flow from the grafted vessel, which resulted in more profound hypoperfusion. The third subgroup (2 patients) exhibited a complete occlusion of the preoperative stenotic artery. The fourth subgroup (3 patients) included the cases with marginal hypoperfusion in the periphery of the perfused region from the grafted extracranial artery. CONCLUSION: EIAB is a reliable, reasonably safe method for establishing new pathways of collateral circulation to the brain. However, this operation can have potential complications according to the relative hyperperfusion or hypoperfusion that's due to the altered flow pattern after the bypass.
Arteries ; Brain ; Brain Ischemia ; Cerebral Revascularization ; Collateral Circulation ; Glycosaminoglycans ; Hemodynamics ; Humans ; Ischemia ; Middle Cerebral Artery ; Moyamoya Disease ; Neurologic Manifestations ; Transplants

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: 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 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