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Concurrence of Multifocal Motor Neuropathy and Hashimoto's Thyroiditis.

Suk Won AHN ; Su Hyun KIM ; Byung Su PARK ; Jeong In CHA ; Sung Min KIM ; Jung Joon SUNG ; Kwang Woo LEE

Journal of Clinical Neurology.2011;7(3):168-172. doi:10.3988/jcn.2011.7.3.168

BACKGROUND: Multifocal motor neuropathy (MMN) is an immune-mediated disorder that is characterized by slowly progressive and asymmetrical weakness, but its pathophysiological mechanism is uncertain. The hypothesis that MMN is an immunological disease has been supported by the proven therapeutic effects of intravenous immunoglobulin and the detection of antiganglioside antibodies in MMN patients. The coexistence of MMN with other immune diseases has been rarely reported. CASE REPORT: A 37-year-old woman visited our hospital complaining of weakness in both hands. The clinical manifestations coincided well with MMN: predominantly distal upper-limb weakness, asymmetric involvement, a progressive course, absence of sensory symptoms, absence of pyramidal signs, and sparing of the cranial muscles. The electrophysiological findings also supported a diagnosis of MMN, with motor nerve conduction block in the median, ulnar, and radial nerves, without sensory nerve involvement. The patient was simultaneously diagnosed as having Hashimoto's thyroiditis, which is a well-known immune-mediated disease. CONCLUSIONS: The concurrence of MMN and Hashimoto's thyroiditis in our patient is significant for understanding the immunological characteristics of the two diseases.
Adult ; Antibodies ; Female ; Hand ; Humans ; Immune System Diseases ; Immunoglobulins ; Muscles ; Neural Conduction ; Radial Nerve ; Thyroid Gland ; Thyroiditis

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Spontaneous Carotid Cavernous Fistula in a Case with Protein S Deficiency that Newly Developed Ophthalmoplegia after Embolization.

Chi Kyung KIM ; Je Young SHIN ; Jun Young CHANG ; Seung Hoon LEE

Journal of Clinical Neurology.2011;7(3):164-167. doi:10.3988/jcn.2011.7.3.164

BACKGROUND: Carotid cavernous fistula (CCF) is an abnormal communication between the carotid artery and the cavernous sinus. The pathogenesis of spontaneous CCF remains unclear, although sinus thrombosis is known to be a predisposing factor for dural arteriovenous fistula. Because spontaneous CCFs are mainly of the dural type, we considered that thrombogenic conditions, such as, protein S deficiency might be associated with CCF. CASE REPORT: A 42-year-old woman complained of conjunctival injection and retro-orbital pain that first appeared 1-month before visiting our hospital. She had no history of head trauma or intracranial surgery. Exophthalmos and chemosis were observed in her left eye, which also had lower visual acuity and higher intraocular pressure than the right eye. Magnetic resonance images and cerebral angiography revealed a left dural CCF. Her protein S was low, at 41% (normal range: 70-140%), but other hematologic values related to coagulation were normal. Her symptoms were relieved after initial transvenous coil embolization. However, a newly developed sixth-nerve palsy was detected 4 days after initial embolization. Follow-up angiography revealed a minimal shunt, and thus transvenous coil embolization was repeated. Two days later, the ophthalmoplegia started reducing, and 1-month later it had almost disappeared. CONCLUSIONS: To the best of our knowledge, this is the first report of spontaneous dural CCF in a Korean patient with concurrent protein S deficiency. Interestingly, transient sixth-nerve palsy developed after transvenous coil embolization in this patient. This additional symptom caused by the residual fistula was relieved after additional transarterial embolization.
Adult ; Angiography ; Carotid Arteries ; Cavernous Sinus ; Caves ; Central Nervous System Vascular Malformations ; Cerebral Angiography ; Craniocerebral Trauma ; Exophthalmos ; Eye ; Female ; Fistula ; Follow-Up Studies ; Humans ; Intraocular Pressure ; Magnetic Resonance Spectroscopy ; Ophthalmoplegia ; Paralysis ; Protein S ; Protein S Deficiency ; Sinus Thrombosis, Intracranial ; Visual Acuity

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Emergency Microsurgical Embolectomy for the Treatment of Acute Intracranial Artery Occlusion: Report of Two Cases.

Dae Won KIM ; Sung Jo JANG ; Sung Don KANG

Journal of Clinical Neurology.2011;7(3):159-163. doi:10.3988/jcn.2011.7.3.159

BACKGROUND: The main treatment for acute arterial ischemic stroke is intravenous or intra-arterial thrombolysis within a particular time window. Endovascular mechanical embolectomy is another treatment option in the case of major artery occlusion. Endovascular mechanical embolectomy is a useful technique for restoring blood flow in patients with large-vessel occlusion, and especially in those who are contraindicated for thrombolytics or in whom thrombolytic therapy has failed. CASE REPORT: We report herein two cases of emergency microsurgical embolectomy for the treatment of acute middle cerebral artery and internal carotid artery occlusion as an alternative treatment for major artery occlusion. CONCLUSIONS: Emergency microsurgical mechanical embolectomy may be an alternative treatment option for restoring blood flow in selected patients with large-vessel acute ischemic stroke.
Arteries ; Carotid Artery, Internal ; Embolectomy ; Emergencies ; Humans ; Middle Cerebral Artery ; Stroke ; Thrombolytic Therapy

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Ipsilateral Tilt and Contralateral Sensory Change of Neck in Cortical Infarction.

Suk Yun KANG ; Hyeo Il MA ; Mi Jeong LEE ; Seok Beom KWON ; San JUNG ; Yun Joong KIM ; Sung Hee HWANG

Journal of Clinical Neurology.2011;7(3):156-158. doi:10.3988/jcn.2011.7.3.156

BACKGROUND: Numerous neck muscles are involved in neck movements, and so isolated neck weakness is extremely uncommon in cerebral infarction. CASE REPORT: We report herein the case of a 65-year-old woman with hypertension and acute cortical infarction, presenting with ipsilateral head tilt and contralateral sensory changes in the neck and shoulder area, which has never been described before. CONCLUSIONS: Transient neck weakness and sensory deficits can occur in acute cortical infarction. The motor representation of the neck muscles can be at the same level of the cortical sensory representation, near to the level of the trunk representation, which is in contrast to Penfield's findings. Several possible mechanisms for the ipsilateral tilt are described.
Aged ; Cerebral Infarction ; Female ; Head ; Humans ; Hypertension ; Infarction ; Magnetic Resonance Imaging ; Motor Cortex ; Neck ; Neck Muscles ; Shoulder

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Changes in Length of Stay for Neurological Geriatric Diseases in Korea between 2003 and 2007.

Hyeong Su KIM ; Kun Sei LEE ; Hee Joon BAE ; Im Seok KOH ; Soung Hoon CHANG ; Do Hui IM ; Jae Hyeok HEO

Journal of Clinical Neurology.2011;7(3):148-155. doi:10.3988/jcn.2011.7.3.148

BACKGROUND AND PURPOSE: The elderly population and the prevalence of stroke, dementia, and Parkinson's disease are increasing rapidly in Korea. The aim of this study was to establish the length of stay (LOS) for neurological geriatric diseases, and analyze this parameteraccording to healthcare institutions. METHODS: We used data from the Health Insurance Review and Assessment Service from 2003 to 2007. Nineteen neurological geriatric diseases were classified into four groups: dementia, cerebral hemorrhage, cerebral infarction, and Parkinson's disease. LOS was analyzed according to gender, age, insurance type, disease group, and type of healthcare institution. RESULTS: The LOS for neurological geriatric diseases lengthened from 5,550,193 days (10.8% of the total National Health Insurance admission days) in 2003 to 14,749,671 days (19.7%) in 2007. The mean LOS was 40.8 days in 2003, and lengthened to 71.2 days in 2007. After stratification by disease group, the mean LOS for long-term-care hospitals lengthened by 1.43 times (from 81.7 to 116.6 days) in the cerebral infarction group, 1.35 times (from 85.6 to 115.2 days) in the cerebral hemorrhage group, and 1.28 times (from 82.7 to 105.7 days) in the Parkinson's disease group. CONCLUSIONS: The LOS for neurological geriatric diseases has lengthened markedly, which isdue to an increasesin the number of hospitalized patients and the mean LOS, which have increased most rapidly in long-term-care hospitals. These results may be useful in developing geriatric health policies.
Aged ; Cerebral Hemorrhage ; Cerebral Infarction ; Delivery of Health Care ; Dementia ; Health Policy ; Humans ; Insurance ; Insurance, Health ; Korea ; Length of Stay ; National Health Programs ; Parkinson Disease ; Prevalence ; Stroke

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Polymorphism of the Glutamate Transporter Protein EAAT2 and Migraine Transformation into Chronic Daily Headache.

Hae Eun SHIN ; Soo Jeong HAN ; Kwang Soo LEE ; Jeong Wook PARK

Journal of Clinical Neurology.2011;7(3):143-147. doi:10.3988/jcn.2011.7.3.143

BACKGROUND AND PURPOSE: The progression of migraine into chronic daily headache involves multiple risk factors, but the main contributor is not known. Glutamate is the major excitatory neurotransmitter in central sensitization, which is an important process in the pathogenesis of migraine transformation. The glutamate transporter protein excitatory amino acid transporter 2 (EAAT2) is the primary modulator of glutamatergic neurotransmission, and genetic polymorphisms of its gene, EEAT2, have been identified. The aim of this study was to determine the effect of EAAT2 polymorphisms on migraine transformation into chronic daily headache. METHODS: We included 74 migraine patients with episodic attack (M-E) and 59 migraine patients with chronic daily headache (M-CDH). After amplifying EAAT2 by polymerase chain reaction, we assessed its genotype frequencies based on restriction fragment length polymorphisms. We reclassified all migraine patients into two groups according to their EAAT2 genotype, either with the A allele (n=62) or without it (n=71), and compared the clinical variables between the two groups. RESULTS: The genotype frequencies of EAAT2 polymorphisms did not differ between the M-E and M-CDH groups. Comparison between EEAT2 genotypes revealed that the frequency of analgesic usage was significantly higher among migraine patients with the A allele (12.9+/-1.6 days/month) than in those without the A allele (8.1+/-1.2 days/month; p=0.019). The other clinical variables of migraine did not differ between the two groups. CONCLUSIONS: The results suggest that EEAT2 polymorphism contributes to the tendency toward frequent analgesic usage in migraine patients. This implies a potential genetic influence on the progression of migraine into chronic daily headache through the development of medication-overuse headache.
Alleles ; Amino Acid Transport System X-AG ; Central Nervous System Sensitization ; Excitatory Amino Acid Transporter 2 ; Genotype ; Glutamic Acid ; Headache ; Headache Disorders ; Humans ; Migraine Disorders ; Neurotransmitter Agents ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Polymorphism, Restriction Fragment Length ; Risk Factors ; Synaptic Transmission

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Efficacy and Safety of Switching from Oral Cholinesterase Inhibitors to the Rivastigmine Transdermal Patch in Patients with Probable Alzheimer's Disease.

Hyun Jeong HAN ; Jeong Ju LEE ; Sun A PARK ; Hyun Young PARK ; Jeong Eun KIM ; Young Soo SHIM ; Dong Seok SHIM ; Eun Joo KIM ; Soo Jin YOON ; Seong Hye CHOI

Journal of Clinical Neurology.2011;7(3):137-142. doi:10.3988/jcn.2011.7.3.137

BACKGROUND AND PURPOSE: The goal of this study was to estimate the efficacy and safety of the rivastigmine transdermal patch in patients with probable Alzheimer's disease (AD) who cannot tolerate or do not respond to oral cholinesterase inhibitors (ChEIs). METHODS: A 24-week, prospective, open-label, single-arm, multicenter study was conducted from June 2009 to June 2010 in patients with probable AD. The enrolled patients had either a poor response or a decline in global function after treatment with oral ChEIs, or they were not able to tolerate treatment with oral ChEIs due to adverse events such as nausea or vomiting. A poor response was defined as a decrease of at least 2 points on the Korean version of the Mini-Mental State Examination (K-MMSE) within the previous 6 months (the decline in global function was determined by the investigator or caregiver). The efficacy of treatment was assessed using a follow-up Clinical Global Impression of Change (CGIC) assessment and K-MMSE conducted after 24 weeks, and safety was measured by the occurrence of adverse events and patient disposition. RESULTS: In total, 164 patients aged 74.7+/-7.52 years (mean+/-SD) and with 5.12+/-3.64 years of education were included. The study was completed by 70% of the patients (n=116), with 12.2% discontinuing due to adverse events. The most frequently reported adverse events (11%) were skin lesions, such as erythema or itching, followed by gastrointestinal problems (1.2%). Either an improvement or no decline in CGIC scores was reported for 82% of the patients. CONCLUSIONS: The immediate switching of patients from an oral ChEI to the rivastigmine transdermal patch without a washout period was safe and well tolerated by the probable-AD patients in this study.
Aged ; Alzheimer Disease ; Cholinesterase Inhibitors ; Cholinesterases ; Erythema ; Follow-Up Studies ; Humans ; Nausea ; Phenylcarbamates ; Prospective Studies ; Pruritus ; Research Personnel ; Skin ; Transdermal Patch ; Vomiting ; Rivastigmine

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Psychiatric Symptoms and Quality of Life in Patients with Drug-Refractory Epilepsy Receiving Adjunctive Levetiracetam Therapy.

Jang Joon LEE ; Hyun Seok SONG ; Yang Ha HWANG ; Ho Won LEE ; Chung Kyu SUH ; Sung Pa PARK

Journal of Clinical Neurology.2011;7(3):128-136. doi:10.3988/jcn.2011.7.3.128

BACKGROUND AND PURPOSE: Levetiracetam (LEV) is a new antiepileptic drug that has been found to be effective as an adjunctive therapy for uncontrolled partial seizures. However, the results of several studies suggested that LEV has negative psychotropic effects, including irritability, aggressiveness, suicidality, and mood disorders. We investigated the impact of adjunctive LEV on psychiatric symptoms and quality of life (QOL) in patients with drug-refractory epilepsy (DRE) and determined the risk factors provoking psychiatric adverse events. METHODS: A 24-week, prospective, open-label study was conducted. At enrollment, we interviewed patients and reviewed their medical charts to collect demographic and clinical information. They were asked to complete self-report health questionnaires designed to measure various psychiatric symptoms and QOL at enrollment and 24 weeks later. RESULTS: Seventy-one patients were included in the study, 12 patients (16.9%) of whom discontinued LEV therapy due to serious adverse events including suicidality. The risk factor for premature withdrawal was a previous history of psychiatric diseases (odds ratio 4.59; 95% confidence interval, 1.22-17.32). LEV intake resulted in significant improvements in Beck Anxiety Inventory score (p<0.01) and some domains of the Symptom Checklist-90-Revised, such as somatization (p<0.05), obsessive-compulsiveness (p<0.05), depression (p<0.05), and anxiety (p<0.05). These improvements were not related to the occurrence of seizure freedom. The Quality of Life in Epilepsy Inventory-31 overall score and subscale scores, such as seizure worry (p<0.01), overall QOL (p<0.05), emotional well-being (p<0.05), energy-fatigue (p<0.05), and social function (p<0.05), also improved. CONCLUSIONS: Adjunctive LEV in patients with DRE is likely to improve psychiatric symptoms and QOL. Clinicians should be well aware of the psychiatric histories of patients to prevent them from developing serious adverse events related to LEV.
Anxiety ; Depression ; Epilepsy ; Freedom ; Humans ; Mood Disorders ; Piracetam ; Prospective Studies ; Quality of Life ; Risk Factors ; Seizures ; Suicide ; Surveys and Questionnaires

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New Insights into Neuromyelitis Optica.

Woojun KIM ; Su Hyun KIM ; Ho Jin KIM

Journal of Clinical Neurology.2011;7(3):115-127. doi:10.3988/jcn.2011.7.3.115

Neuromyelitis optica (NMO) is an idiopathic inflammatory disorder of the central nervous system (CNS) that preferentially affects the optic nerves and spinal cord. In Asia, NMO has long been considered a subtype of multiple sclerosis (MS). However, recent clinical, pathological, immunological, and imaging studies have suggested that NMO is distinct from MS. This reconsideration of NMO was initially prompted by the discovery of a specific antibody for NMO (NMO-IgG) in 2004. NMO-IgG is an autoantibody that targets aquaporin-4 (AQP4), the most abundant water channel in the CNS; hence, it was named anti-AQP4 antibody. Since it demonstrated reasonable sensitivity and high specificity, anti-AQP4 antibody was incorporated into new diagnostic criteria for NMO.The spectrum of NMO is now known to be wider than was previously recognized and includes a proportion of patients with recurrent, isolated, longitudinally extensive myelitis or optic neuritis, and longitudinally extensive myelitis or optic neuritis associated with systemic autoimmune disease or with brain lesions typical of NMO. In this context, a new concept of "NMO spectrum disorders" was recently introduced. Furthermore, seropositivity for NMO-IgG predicts future relapses and is recognized as a prognostic marker for NMO spectrum disorders. Humoral immune mechanisms, including the activation of B-cells and the complement pathway, are considered to play important roles in NMO pathogenesis. This notion is supported by recent studies showing the potential pathogenic role of NMO-IgG as an initiator of NMO lesions. However, a demonstration of the involvement of NMO-IgG by the development of active immunization and passive transfer in animal models is still needed. This review focuses on the new concepts of NMO based on its pathophysiology and clinical characteristics. Potential management strategies for NMO in light of its pathomechanism are also discussed.
Asia ; Autoimmune Diseases ; B-Lymphocytes ; Brain ; Central Nervous System ; Complement System Proteins ; Humans ; Light ; Models, Animal ; Multiple Sclerosis ; Myelitis ; Neuromyelitis Optica ; Optic Nerve ; Optic Neuritis ; Recurrence ; Sensitivity and Specificity ; Spinal Cord ; Vaccination ; Water

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Disability-Adjusted Life Years Analysis: Implications for Stroke Research.

Keun Sik HONG

Journal of Clinical Neurology.2011;7(3):109-114. doi:10.3988/jcn.2011.7.3.109

Stroke is a prototype disorder that disables as well as kills people. The disability-adjusted life years (DALY) metric developed by the World Health Organization to measure the global burden of disease integrates healthy life years lost due to both premature mortality and living with disability. Accordingly, it is well suited to stroke research. The DALY has previously been applied only to large but relatively crude population-level data analyses, but now it is possible to calculate the DALY lost in individual stroke patients. Measuring each patient's stroke outcome with DALY lost has expanded its application to the analysis of treatment effect in acute stroke trials, delineating the poststroke complication impact, the differential weighting of discrete vascular events, and estimating a more refined stroke burden in a specific population. The DALY metric has several advantages over conventional stroke outcome measures: 1) Since the DALY measures the burden of diverse health conditions with a common metric of life years lost, stroke burden and benefits of stroke interventions can be directly compared to other health conditions and their treatments. 2) Quantifying stroke burden or interventional benefits as the life years lost or gained makes the DALY metric more intuitively accessible for public and health system planners. 3) As a continuous, equal-interval scale, the DALY analysis might be statistically more powerful than either binary or ordinal rank outcome analyses in detecting the treatment effects of clinical trials. 4) While currently employed stroke outcome measures take one-time snapshots of disability or mortality and implicitly indicate long-term health impact, the DALY explicitly indicates the burdens of living with disability for an individual's remaining life.
Humans ; Mortality, Premature ; Outcome Assessment (Health Care) ; Statistics as Topic ; Stroke ; World Health Organization

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