A confusing case of multiple sclerosis and central nervous system graft versus host disease

No abstract available.

Neuro-Behcet's Disease Presenting as Hypertrophic Pachymeningitis

A 25-year-old man presented with blurred vision and chronic headache. His brain MRI revealed bilateral frontal pachymeningeal enhancement with leptomeningeal enhancement. The patient had experienced recurrent oral ulcer and had anterior uveitis and papulopustules skin lesion. We diagnosed him with hypertrophic pachymeningitis (HP) associated with neuro-Behcet's disease (NBD). There have been few reports describing HP in patients with NBD. We report a case of NBD presenting as HP.

Involuntary Flexor Spasms Associated with Basal Ganglia Infarction

Flexor spasm caused by stroke has been rarely reported. We describe a 79-year-old man who suddenly presented with flexor spasm of left limb and neck muscles. His symptoms consisted of recurrent episodes of brief paroxysmal flexor contractions of left limb and neck muscles without alteration of consciousness. Brain Magnetic Resonance Imaging revealed acute infarction in right basal ganglia. The abnormal movement was not associated with any epileptiform discharges in the video-electroencephalography monitoring. It was controlled with carbamazepine and phenytoin.

Behavioral and Psychological Symptoms in Patients with Parkinson's Disease According to Cognitive Function

BACKGROUND: Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by motor and nonmotor symptoms such as cognitive impairment and neuropsychiatric symptoms. The aim of this study was to investigate the spectrum of neuropsychiatric symptoms in PD according to cognitive function. METHODS: One hundred twenty seven patients with PD were consecutively recruited. They had undergone an intensive interview with a neurologist and the Seoul Neuropsychological Screening Battery-dementia version, and were divided into three groups: 27 patients in PD with normal cognition (PDNC), 57 in PD with mild cognitive impairment (PDMCI), and 43 in PD with Dementia (PDD). Forty five healthy controls without memory complaints were also recruited. The caregivers of all participants administered the Caregiver-Administered Neuropsychiatric Inventory (CGA-NPI). RESULTS: There were significant differences in the CGA-NPI score (22.8+/-20.9 vs. 6.4+/-10.1 vs. 1.7+/-3.9 vs. 1.0+/-1.6, p<0.001), and the prevalence of neuropsychiatric symptoms (100% vs. 64.9% vs. 37.0% vs. 44.4%, p<0.001) among PDD, PDMCI, PDNC and control groups. Depression was the most common symptom (43.3%), followed by anxiety (31.5%), apathy (26.8%), and night-time behavior (26.8%) in all PD patients. Delusion, hallucination, and aberrant motor behavior were observed frequently in PDD, but were rare in PDMCI. CONCLUSIONS: Depression was the most common neuropsychiatric symptom in PD. The presence of delusion, hallucination, or aberrant motor behavior may suggest PDD. The neuropsychiatric symptoms were not prevalent in PD with normal cognition.

Cerebral Venous Thrombosis in the Inferior Sagittal Sinus

No abstract available.

Statin-associated Myasthenia Gravis

No abstract available.

Association Between Obstructive Sleep Apnea and Cardiovascular Diseases

BACKGROUND: Obstructive sleep apnea (OSA) contributes to the development of cardiovascular diseases. The aim of this study is to analyze the association between OSA and cardiovascular diseases in adult. METHODS: We recruited 718 consecutive subjects (611 male, 44.8+/-12.7 years old) diagnosed as OSA based on overnight polysomnography. The prevalence of hypertension, diabetes mellitus, myocardial infarction and stroke was defined and compared with that in general adult population (the Third Korea National Health and Nutritional Examination Survey, KNHANES III 2005). Correlation between the prevalence and OSA severity was analyzed after adjusting age, sex, body mass index, smoking, and alcohol consumption. RESULTS: Prevalence of vascular diseases was higher in OSA than general population. Prevalence of hypertension was 39.0% (13.0% from KNHANES III), diabetes 8.2% (5.2%), myocardial infarction 3.0% (0.6%) and stroke 7.2% (1.6%). Apnea-hypopnea index as the marker of OSA severity significantly correlated with hypertension (p=0.01). CONCLUSIONS: Cardiovascular diseases were more prevalent in OSA. Prevalence of hypertension increased proportionally to OSA severity.

Impact of Obstructive Sleep Apnea on Carotid Intima-Media Thickness

BACKGROUND: Obstructive sleep apnea (OSA) is known to be an independent risk for cardiovascular diseases. The carotid intima.media thickness (IMT) is a surrogate marker of subclinical atherosclerosis. The aim of this study was to measure the carotid IMT in subjects with or without OSA and quantify its correlation with OSA severity. METHODS: Subjects were categorized into OSA and non-OSA groups based on polysomnography findings. The carotid IMT was considered a marker of atherosclerosis. The independent relationship between carotid IMT and OSA severity was analyzed using multiple linear regression. The presumed confounders were age, sex, body mass index, blood pressure, blood lipid and glucose profiles, smoking and alcohol drinking statuses, medications, being overweight, and having hypertension, diabetes mellitus, or hyperlipidemia. RESULTS: The carotid IMT was larger in OSA subjects (n=82, 0.620+/-0.096 mm [mean+/-SD]) than in non-OSA subjects (n=22, 0.551+/-0.078 mm; p=0.006), and was independently correlated with OSA severity (p<0.001). However, it was not affected by any of the other variables investigated. CONCLUSIONS: Subjects with OSA showed an increased IMT, with the thickness being correlated with the OSA severity. This finding suggests that accelerated atherosclerosis in OSA is the mechanism linking OSA and future cardiovascular events.

Plasma Total Homocysteine Levels are not Associated with Medial Temporal Lobe Atrophy, but with White Matter Changes in Alzheimer's Disease

BACKGROUND AND PURPOSE: Elevated plasma total homocysteine (tHcy) levels are reported to be associated with an increased risk of Alzheimer's disease (AD). However, the mechanism by which homocysteine contributes to the pathogenesis of AD is as yet unknown. The aim of this study was to elucidate the relationship between white matter changes (WMC) and medial temporal lobe atrophy (MTA) on brain magnetic resonance imaging (MRI), and plasma levels of tHcy in AD patients. METHODS: Seventy-two patients with a clinical diagnosis of probable AD were recruited to the study. Plasma tHcy levels, vascular risk factors, and WMC and MTA on brain MRI were evaluated in all patients. The AD patients were classified into two groups: those with no or minimal WMC (69.2+/-8.8 years, mean+/-SD, n=36) and those with moderate-to-severe WMC (74.6+/-4.6 years, n=36) on brain MRI. RESULTS: In a univariate logistic regression analysis, the risk of moderate-to-severe WMC in AD was significantly associated with increasing age, female gender, lower education level, hypertension, high plasma tHcy levels, and lower Mini-Mental State Examination (MMSE) score. Multivariate logistic regression analysis revealed only high plasma tHcy as the independent and significant risk factor for moderate-to-severe WMC [odds ratio (OR; adjusted for age, gender, education level, MMSE score, and hypertension comparing the top tertile - tHcy levels > or =12.9 micromol/L - with the bottom tertile - tHcy levels < or =9.4 micromol/L)=7.35; 95% confidence interval, confidence interval=1.36-39.84; p=0.02], and age as a borderline significant risk factor (OR=1.08, 95% CI=0.99-1.19, p=0.09) in AD patients. Plasma tHcy levels were not correlated significantly with either right or left MTA. CONCLUSIONS: Our results suggest that the vascular pathway mediates the association between elevated plasma tHcy levels and AD.

Melatonin Therapy for REM Sleep Behavior Disorder with Co-existing Moderate-to-Severe Sleep Apnea

BACKGROUND: REM sleep behavior disorder (RBD) commonly occurs in old-age group and shows very good response to clonazepam. However, clonazepam can aggravate or even precipitate sleep-disordered breathings (SDBs) that is highly prevalent among elderly people. Melatonin has been known to be effective in RBD treatment and is much less likely to increase the airway resistance. The aim is to document the efficacy of melatonin therapy in RBD with co-existing significant SDB. METHODS: We recruited consecutive patients who have both RBD and significant SDB (apnea-hypopnea index, AHI>15) confirmed by nocturnal polysomnography. Melatonin was started at the dose of 0.5 mg/night and increased according to the clinical response. Successful response is defined when symptoms are free or reduced in frequency more than 90% and any physical injury or violence should not be present. RESULTS: Eleven among 16 RBD patients were eligible and enrolled (eight male, median age 69 years, range 54-89). All had significant obstructive sleep-disordered breathings (mean AHI = 28.8, range 22.6-35.2). Melatonin was prescribed(dosage 2-8 mg/night, treatment duration, 7-35 months). Nine patients showed successful response (2-8 mg/night) but two showed unsatisfactory response to 6 mg or 8 mg/night. Two of nine patients with favorable response complained of mild sleepiness. CONCLUSIONS: Melatonin can be effective for controlling RBD with co-existing SDB. Effective dosage range is variable without intolerable adverse effect.

The Impact of Nocturnal Hypoxemia and Daytime Sleepiness on Cognitive Impairment in Patients with Obstructive Sleep Apnea Syndrome

BACKGROUND: Patients with obstructive sleep apnea syndrome (OSAS) have some deficits in attention and frontal functions. The pathophysiology of the cognitive dysfunction is still controversial. We investigated the cognitive performances of OSAS patients, and the relationship of cognitive functioning and nocturnal respiratory findings and daytime sleepiness. METHODS: Neuropsychological testing, the Beck Depression Inventory and the Epworth sleepiness scale (ESS) were administered to 35 patients with OSAS and to 31 normal controls. RESULTS: Compared to the controls, OSAS patients had significant impairment on the Korean mini mental state examination (K-MMSE), semantic and phonemic fluency, the time and number of correct on the Korean Stroop color word test, the Digit cancellation test and delayed response on the Seoul verbal learning test (SVLT). ESS was significantly correlated with K-MMSE, trail making A, the time and number of correct on the Korean Stroop color word test, delayed recall of SVLT and Rey figure test and number of fulfilled categories on the Wisconsin card sorting test in OSAS patients. The Apnea-hypopnea index (AHI) was significantly correlated with the copy of the Rey figure test. Arousal index, total time slept with oxygen saturation below 90% and the lowest oxygen saturation were not correlated with any neuropsychological parameters. CONCLUSIONS: Patients with OSAS have cognitive impairment concerning attention, executive function and retrieval of memory. The cognitive impairment may be attributed not to nocturnal hypoxemia, but to subjective daytime sleepiness.

The Primary Sjogren's Syndrome Involving Central and Peripheral Nervous System

Sjogren's syndrome(SS) is an autoimmune disease characterized by inflammation induced chronic dysfunction of the exocrine glands. Neurologic manifestations occurs in 30% of patients. We report a 55-year-old woman with primary SS, who presented with subacute aseptic meningitis combined with an unnoticed polyneuropathy. SS was confirmed by dry eyes and Schirmer's test, salivary scintigraphy and serum autoantibodies. We suggest SS as a cause of subacute aseptic meningitis in an elderly female patient with dry eyes and mouth.

Diffusion Tensor Imaging in Acute Ischemic Stroke: Usefulness of Fractional Anisotropy

BACKGROUND: Progressing stroke (PS) variably develops from initially the same size and severity, and is most frequently observed in lacunar infarctions. We investigated fractional anisotrophy (FA), mean diffusivity (MD) and infarct volume by using diffusion tensor imaging during the acute phase of ischemic stroke to determine whether these parameters are useful in characterizing and predicting PS. METHODS: In this study, 55 consecutive patients admitted within 24 hours of the onset of their first ischemic stroke were included. NIH stroke scale (NIHSS) and Canadian Neurological scale (CNS) were performed upon admission, twice a day, and at discharge. Modified Rankin scale and Barthel index were also evaluated. PS was defined as a 2-point drop in NIHSS and a 1-point drop in CNS from admission to day 3. A correlation analysis was performed between clinical scale scores and imaging parameters, and the distribution of those values was compared between the two groups with and without PS. RESULTS: Significant correlations were observed between clinical scale scores and infarct volumes. The FA ratio in 14 patients with PS was lower than the patients without PS (p=0.004). Other characteristics including infarct volume and MD ratio were not different. The FA ratio remained as an independent predictor of PS (OR, 1.055; p=0.011). CONCLUSIONS: In acute ischemic stroke within the first 24 hours, only infarct volume was correlated with clinical status. However, patients with PS showed lower FA values, which accounts for rapid and severe vasogenic edema involving the disruption of the cell membrane and axonal fibers. Moreover, FA may be a predictor of PS.

The Effectiveness of Epidural Blood Patch in Patients with Syndrome of Cerebrospinal Fluid Hypovolemia

BACKGROUND: Therapy using a targeted epidural blood patch (EBP) has been linked to cerebrospinal fluid (CSF) hypovolemia syndrome empirically, when conservative therapies failed to relieve headaches. The aim of this study is to investigate the effectiveness of EBP, for those who are refractory to conservative therapy, as a treatment for syndrome of CSF hypovolemia. METHODS: Sixteen patients were studied, where six patients were treated with conservative therapy and 10 patients were treated with EBP. After the treatments, we evaluated the difference in the duration of headaches between the two groups. With the patients treated with EBP, we also evaluated the differences in the duration of headaches between those with or without pachymeningeal enhancement on a brain MRI, and those with or without CSF leakage site on RI cisternography. RESULTS: Mean duration (1.30 +/- 0.48 days) of headaches in those treated with EBP was significantly shorter than in those without EBP (p=0.022). There was no significant difference in duration of headaches between those with and without pachymeningeal enhancement in a brain MRI. There was also no significant difference in duration of headache between those with and without CSF leakage in RI cisternography. CONCLUSIONS: The EBP is an effective therapy for syndrome of CSF hypovolemia regardless of pachymeningeal enhancement in a brain MRI and CSF leakage in RI cisternography when conservative therapies fail to relieve headaches.

Focal Dystonia with Primary Medullary Hemorrhage

We report a patient with hand dystonia related with primary medullary hemorrhage. A 69-year-old man presented with dysarthria, right facial palsy, limb paresthesia, and limb ataxia of acute onset. Neurologic examination revealed abnormal tonic posturing of the right hand and wrist that persisted while at rest. Brain MRI showed acute hemorrhage at the right posteromedial medulla oblongata extending to the upper cervical cord. Ipsilateral limb dystonia might result from the interruption of the reticulospinal tract, sensory or olivocerebellar pathway.

A Case of Intractable Hiccup Controlled by Combination Drug Therapy with Levosulpiride, Omeprazole and Baclofen

No abstract available.

The Validity of the Korean Version of Global Deterioration Scale

BACKGROUND: Clinicians broadly accept the Global Deterioration Scale (GDS) as a staging measure for dementia. The aim of this study is to test the inter-rater reliability and concurrent validity of the Korean version of GDS. METHODS: Participants included 34 subjects without dementia and 41 patients with Alzheimer's disease, in addition to 37 patients with vascular dementia. A rater, using a semi-structured clinical interview, interviewed the participants and their informed collateral sources. To obtain the concurrent validity, all participants received the Korean Mini-Mental State Examination (K-MMSE), the Korean version of Syndrome Kurz Test (K-SKT) and a 15-items abbreviated Korean-Boston Naming Test (K-BNT) on the same day. We also tested the inter-rater reliability among the four raters using a videotape design. RESULTS: The agreements of the overall GDS ratings by the four raters were high (kappa, 0.93-1.0). GDS correlated significantly with K-MMSE, K-SKT, and the abbreviated K-BNT and Clinical Dementia Rating scale. CONCLUSION: The Korean version of GDS may be a reliable and valid tool to be used as a staging measure for Korean patients with Alzheimer's disease as well as vascular dementia.

Comparative Study between Visusal Analysis and Low Resolution Electromagnetic Tomography (LORETA) Method in the Localization of Epileptiform Discharges

BACKGROUND: LORETA (Low Resolution Electromagnetic Tomography) produces a three-dimensional current density mapping from digitized EEG (electroencephalography) data. A comparison between visual waveform analysis of scalp EEG and LORETA method may be helpful for the exact localization of epileptiform discharges. METHODS: Digital EEG data from 20 subjects showing epileptiform discharges that were localized in the left or right frontotemporal regions (F7, F8) were studied. Those EEG waves were recorded using international 10~20 system. Phase reversals in bipolar montage were converted to referential montage and waveforms were analyzed visually. Those spike waves were converted into three-dimensional potential density mapping by LORETA method and maximum negative potential density region was determined. RESULTS: The same localization in the frontotemporal region (F7, F8) between two methods was noted in 16 subjects (80.0%). The discordant localization by LORETA method was noted in 4 subjects (20.0%). In different localiza-tion subjects, their locations were frontopolar region (Fp1, Fp 2) in 2 subjects, lateral temporal region (T7, T8) in 1 and posterior temporal region (P7, P8) in 1 subject, respectively. CONCLUSIONS: Even though the localization of epileptiform discharges by visual waveform analysis is a traditional method, electrical potential density mapping by LORETA can be a useful adjunctive localization method. If detailed localization is needed, LORETA method should increase accuracy.

A Case of Cerebral Infarct in Combined Antiphospholipid Antibody and Ovarian Hyperstimulation Syndrome

Ovarian hyperstimulation syndrome is a serious complication of ovulation induction and has a diverse clinical spectrum from edema to thromboembolism. Antiphospholipid antibody syndrome, one of the well known hypercoagulable states, can be also manifested as an arterial or venous thrombosis and recurrent spontaneous abortion. Sometimes a patient with antiphospholipid antibodies might not notice a miscarriage and seek for assisted reproduction treatment, which harbors a chance of developing ovarian hyperstimulation syndrome. If this happens, the ovarian hyperstimulation syndrome can exacerbate the thrombotic complication of underlying antiphospholipid antibody syndrome, resulting in a catastrophic vascular event. The authors experienced a case of middle cerebral artery infarct, which developed during ovarian hyperstimulation syndrome in a 33-yr-old woman with a previous history of fetal loss. An elevated titer of anticardiolipin antibodies was noticed and persisted thereafter. The authors suggest screening tests for the presence of antiphospholipid antibodies before controlled ovarian hyperstimulation.

Performance Rate of Thrombolytic Therapy and Acute Prognosis of Hyperacute Ischemic Stroke

BACKGROUND: Tissue plasminogen activator (tPA) is known to be effective if given within 3 hours after the onset of ischemic stroke. However, no study has been performed regarding what proportion of patients with acute ischemic stroke are actually given tPA within this time window. We therefore tried to figure out the performance rate of thrombolytic therapy and acute prognosis in hyperacute ischemic stroke patients. METHODS: We prospectively registered all the acute ischemic stroke patients. Thrombolyic therapy was decided strictly according to the NINDS tPA protocol and clinical monitoring was done by the NIH Stroke Scale (NIHSS). If tPA was not given, the reason was recorded. RESULTS: Among 613 acute stroke patients during a period of 2.5 years, there were 146 (23.8%) hyperacute ischemic stroke patients within 3 hours after onset. Even in the 29 cases of in-hospital stroke, the rate of early detection and management was only 27.6% (8 cases). Among the hyperacute stroke population, 56 (38.4%) patients were treated with the thrombolytic agent and 27 patients showed positive responses. Thrombolytic therapy was not tried in the remaining 90 patients. The most common reason was expectation of favorable outcome without thrombolysis. But among these patients, subsequent neurological worsening was noticed in 9 patients, particularly in those with vertebrobasilar stroke. CONCLUSIONS: Urgent notice and management of ischemic stroke needs to be more emphasized not only to the general public, but also to medical personnel. More close observation is especially needed in vertebrobasilar stroke cases (J Korean Neurol Assoc 19(5):447~451, 2001)