Diabetic Neuropathy: Classification and Pathogenesis / 대한신경근육질환학회지

This article provides an overview for understanding the classification and pathogenesis of diabetic neuropathy. Diabetic neuropathies are prevalent disorder. The most common manifestation is distal symmetric polyneuropathy, but various patterns of neuropathy can occur. New information for the pathogenesis of diabetic neuropathy continues to emerge, which will lead to identifying new drug targets.

Dramatic unilateral decrease in uptake via the dopamine transporter: Imaging in a patient with hemiparkinsonism following the lacunar stroke in substantia nigra

@#Vascular parkinsonism (VaP) is typically defined as having predominant lower body involvement, postural instability, less prominent rest tremor and little or no response to treatment with levodopa. In this study, we report a patient with VaP with clear demonstration of a dramatic unilateral decrease of radiotracer uptake in a 18F-FP-CIT-PET study. A 62-year-old right-handed woman was referred to the neurology department due to rest tremor and rigidity in the right hand, which began after undergoing resection surgery for a left acoustic neuroma 7 years prior. Brain MRI, taken at 1 year after surgery showed an ischemic stroke lesion in the left medial pons and the left substantia nigra. 18F-FP-CIT-PET revealed a marked reduction of radiotracer uptake in left striatum compared to that of the right. We treated the patient with 100 mg of levodopa, 200 mg of entacarpone and 25 mg of carbidopa. There was an improvement in bradykinesia and tremor, but the symptoms persisted, and there was no deterioration during 6 months of observation. After acoustic neuroma surgery, ischemic complications are uncommon, and even a small lesion in the nigrostriatal pathway can cause a hemiparkinsonism. If a patient experience sudden onset hemiparkinsonism, they should be carefully examined for lesions in the nigrostriatal pathways. Under these conditions, the 18F-FP-CIT-PET scan can enable visualization of a unilateral decrease and is a useful tool for diagnosis and differentiation from idiopathic Parkinson’s disease

Roving Eye Movements in a Patient with Central Nervous System Infection

No abstract available.

Spontaneous Intracranial Hypotension Followed by Subdural Hemorrhage

No abstract available.

Relapsing Polychondritis Presented with Encephalitis Followed by Brain Atrophy

Relapsing polychondritis (RP) is a rare autoimmune disease that is characterized by inflammatory reaction of unknown etiology and destruction of cartilaginous structures. Characteristic symptoms of this disease include cartilage inflammation of the ear, nose, larynx, trachea, bronchi, joints, eyes, heart and skin. Concomitance with neurologic symptom is very rare in RP, and the detailed underlying mechanism of neurological involvement associated with RP is not fully understood. We herein described an unusual recurrent case of inflammatory brain lesions associated with RP, with attention to clinical manifestations, autoimmune disease involvement, and therapeutic effects.

Rapid Progression of Sporadic ALS in a Patient Carrying SOD1 p.Gly13Arg Mutation

Amyotrophic lateral sclerosis (ALS), the most common adult onset motor neuron disease, is pathologically characterized by progressive loss of the upper and lower motor neurons. Mutations in the Cu/Zn superoxide dismutase gene (SOD1) account for about 20% of familial ALS cases and a small percentage of sporadic ALS (SALS) cases, and have revealed a validated genotype-phenotype correlation. Herein, we report a p.Gly13Arg mutation in SOD1 exon 1 in a patient with SALS who presented with a rapidly progressive course, predominantly affecting the lower motor neurons. A 48-year-old man presented with progressive weakness and muscle atrophy of the left upper and lower limbs, followed by muscle fasciculation and cramping. The clinical features of the patient were clearly suggestive of ALS, and implied a sporadic form with rapid progression, predominantly affecting the lower motor neurons. Sequencing of the SOD1 gene by PCR revealed a missense mutation of G to C (c.37G>C) in exon 1, and amino acid substitution of glycine by arginine (p.Gly13Arg). This is the first case identifying the p.Gly13Arg mutation of SOD1 in the Korean population, and clinical assessments of this patient revealed a different phenotype compared with other cases.

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

No abstract available.

Reduced Gray Matter Volume in Subjective Cognitive Decline: A Voxel-Based Morphometric Study

BACKGROUND AND PURPOSE: Subjective cognitive decline has been proposed as a potential indicator of the preclinical state of Alzheimer's disease (AD). The results of the studies of cortical atrophy on brain MRIs in subjects with subjective cognitive decline are inconsistent across the literatures. We investigated whether subjects with subjective cognitive decline had less gray matter volume compared to controls without subjective cognitive decline as per brain MRI. METHODS: Thirty-six subjects with subjective cognitive decline and thirty-three controls without subjective cognitive decline were recruited retrospectively from among the patients who had visited the department of neurology at Inha University Hospital between January 2008 and December 2010. All subjects had undergone a brain MRI scan including 3D T1-weighted spoiled gradient recalled echo imaging. We used voxel-based morphometry (VBM) to examine gray matter volumes between the two groups, after controlling for age, sex, education, and total intracranial volumes (TIV). RESULTS: There were no significant differences in age, gender, education, and TIV between the two groups. In comparison to controls without subjective cognitive decline, subjects with subjective cognitive decline showed gray matter atrophy in the left superior and medial frontal gyri, left superior and inferior parietal lobules, and right precuneus and insular in the VBM analysis. CONCLUSIONS: Individuals with subjective cognitive decline encountered in clinical settings have greater similarity to an AD gray matter atrophy pattern compared with cognitively normal individuals without subjective cognitive decline.

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.

Driving in Patients with Dementia: A CREDOS (Clinical Research Center for Dementia of South Korea) Study

BACKGROUND: There are few studies about driving and dementia in Korea. The purpose of this study is to investigate the real condition of automobile driving in patients with dementia and the characteristics of those who continue to drive after diagnosis of dementia. METHODS: A total of 4,377 patients including 3,792 with Alzheimer's disease and 585 with vascular dementia were recruited from multiple nationwide hospitals. Clinical evaluations and neuropsychological tests were done in them according to the protocol of the Clinical Research Center for Dementia of South Korea study. Caregivers replied which patients drove an automobile, gave up driving, or has never driven. The same evaluations of them were followed after one year. RESULTS: There were 272 (6.2%) drivers, 321 (7.3%) ex-drivers, and 3,784 (86.5%) non-drivers with dementia. Drivers with dementia were younger and had higher Korean Mini-Mental State Examination (K-MMSE) (21.5+/-4.3 vs 19.8+/-5.1 vs 17.4+/-5.2, p<0.001) and lower Clinical Dementia Rating scale-Sum of Boxes (CDR-SB) scores (4.08+/-2.15 vs 5.80+/-3.41 vs 5.83+/-3.39, p<0.001) compared with ex-drivers and non-drivers with dementia. The drivers and ex-drivers with dementia had higher educational level and a higher proportion of male compared with non-drivers with dementia (77.6% vs 80.1% vs 23.3%, p<0.001). About 54.6% of the drivers with dementia continued to drive at one year after diagnosis of dementia. They had higher K-MMSE (23.2+/-3.6 vs 20.8+/-4.5, p=0.003) and lower CDR-SB scores (3.30+/-1.47 vs 4.40+/-1.85, p=0.001) at baseline compared with those who quitted driving after baseline. CONCLUSIONS: Male patients who were relatively young and had high educational level and mild dementia had a tendency to drive an automobile at that time of diagnosis of dementia. About half of them continued to drive after diagnosis of dementia.

Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings

Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. When encountering a patient who presents with this symptomatic profile, possible diagnoses include upper limb onset ALS (UL-ALS), and FAS. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. The aim of this study was to compare clinical and electromyographic findings from patients diagnosed with FAS with those from patients diagnosed with UL-ALS. To accomplish this, 18 patients with FAS and 56 patients with UL-ALS were examined. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. The presence of upper motor neuron signs and lower motor neuron involvement evidenced through electromyography showed no significant between-group differences.

Diffusion Tensor Imaging Changes Correlate with Clinical Progression in Vascular Mild Cognitive Impairment and Vascular Dementia of Subcortical Type

BACKGROUND: Cerebral small vessel disease (SVD) induces vascular cognitive impairment (VCI) such as subcortical vascular dementia (SVaD) and subcortical vascular mild cognitive impairment (svMCI). We compared MRI parameters between SVaD and svMCI and determined which MRI parameters best correlated with cognitive function and disability on cross-sectional and longitudinal analyses within them. METHODS: Twenty-four patients with SVaD and twelve with svMCI were recruited. They underwent multimodal MRIs including fluid-attenuated inversion recovery lesion load, lacunar infarct number, and fractional anisotropy (FA) and mean diffusivity (MD) from diffusion tensor imaging (DTI), neuropsychological testing, Sum of Boxes of Clinical Dementia Rating Scale (CDR-SB), Barthel Index, and the Korean version of a Geriatric Depression Scale (GDS-K). Seventeen patients were retested after 20 months for a brain MRI and clinical evaluation. RESULTS: There were significant differences in average MD and peak height of MD histograms within normal-appearing brain tissue (NABT) between SVaD and svMCI patients. In the cross-sectional analysis, average MD within NABT significantly correlated with the composite neuropsychology score (r=-0.80, p<0.001), the composite executive function score (r=-0.67, p< 0.001), and the CDR-SB (r=0.54, p=0.001), and the Barthel Index correlated with peak heights of the MD histograms (r=0.37, p=0.03) in NABT. Changes of CDR-SB was associated with changes of average MD within WMH (r=0.57, p=0.02), and changes of GDS-K was associated with changes of WMH volume (r=0.51, p=0.04) on a longitudinal scale. CONCLUSIONS: DTI parameters in NABT correlated with cognitive impairment and disability in VCI associated with SVD. Clinical progression of SVD was associated with some increment of WML volume and ultrastructural changes in WMH.

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.

Gene-Smoking Interaction on Ischemic Stroke: Case-Control Study of Glutathione S-Transferase Polymorphism / 대한뇌졸중학회지

BACKGROUND: Smoking is a major risk factor of stroke, but not all smokers develop stroke. This individual difference could be explained by the variation of detoxification capacity. We investigated the relationship of smoking with the genetic polymorphism of a detoxification enzyme (glutathione S-transferase: GST). METHODS: This study was conducted as a case-control study. Conventional risk factors for stroke and 3 genetic polymorphisms of GST (GSTM1, GSTT1, and GSTP1) were studied in both 290 acute ischemic stroke patients and 290 age and sex matched controls. Smoking status was determined by urinary cotinine level. The effect of interaction of GST polymorphisms and smoking on stroke risk was investigated. RESULTS: Stroke patients had higher cotinine level compared to that of control (P<0.01). There was little difference between the patient group and control group with regard to the GST polymorphism alone, but significant interaction was noticed between the GST polymorphism and the smoking status. When we stratified the group according to the smoking status by cotinine level, stroke was significantly more frequent in GSTM1 null type and GSTT1, GSTP1 wild type of the high cotinine level group (OR and 95% CI: 2.115, 1.219-3.670; 2.620, 1.480-4.638; 2.212, 1.343-3.644 respectively). CONCLUSION: GST polymorphisms interact with the smoking and confer an increased risk of ischemic stroke, indicating that genetic polymorphism of GST might reveal smokers who are more susceptible to the ischemic stroke.

The Effect of Cognitive Training in Patients with Mild Cognitive Impairment and Early Alzheimer's Disease: A Preliminary Study

BACKGROUND AND PURPOSE: The objective of this study was to determine the benefits of cognitive training in patients with amnestic mild cognitive impairment (aMCI) and those with early Alzheimer's disease (AD). METHODS: Eleven patients with aMCI and nine with early AD (stage 4 on the Global Deterioration Scale) participated in this study. Six participants with aMCI and six with AD were allocated to the cognitive training group, while five participants with aMCI and three with AD were allocated to a wait-list control group. Multicomponent cognitive training was administered in 18 weekly, individual sessions. Outcome measures were undertaken at baseline, and at 2 weeks and 3 months of follow-up. RESULTS: In the trained MCI group, there were significant improvements in the delayed-recall scores on the Seoul Verbal Learning Test at both the 2-week and 3-month follow-ups compared with baseline (baseline, 1.6+/-1.5; 2 weeks, 4.4+/-1.5, p=0.04; 3 months, 4.6+/-2.3, p=0.04). The phonemic fluency scores (1.0+/-0.8 vs. 5.0+/-1.8, p=0.07) and Korean Mini-Mental State Examination scores (18.8+/-0.5 vs. 23.8+/-2.2, p=0.07) also showed a tendency toward improvement at the 2-week follow-up compared to baseline in the trained AD group. CONCLUSIONS: This study provides evidence of the effectiveness of cognitive training in aMCI and early AD. The efficacy of cognitive training programs remains to be verified in studies with larger samples and a randomized design.

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.

Prognostic Factors of Thrombolytic Therapy in Ischemic Stroke

BACKGROUND: The intravenous thrombolysis is a well established treatment of acute ischemic stroke. However, baseline prognostic factors were poorly identified by previous studies. METHODS: From January 2001 to May 2006, prospective data of 121 patients treated with intravenous tissue plasminogen activator (tPA) were collected. The clinical, radiologic, transcranial Dopper (TCD) and laboratory finding were evaluated. Clinical assessment was done by National Institutes of Health Stroke Scale (NIHSS) for one week, and by modified Rankin Scale (mRS) at baseline for three months. Early improvement was defined as the complete resolution of the neurological deficit or an improvement of > or =4 points by NIHSS within 24 hours, and good outcome as mRS score of 33% of total MCA territory) were significantly associated with early improvement. Good outcome was associated with younger age, lower levels of baseline NIHSS score, mean blood pressure, fasting glucose, lipoprotein (a), and normal CT finding. Multivariate analysis revealed age <66 years and no HMCAS as independent predictors of early improvement. Thrombolysis in brain ischemia grade by TCD monitoring significantly correlated with NIHSS score for 24 hours. CONCLUSIONS: These results suggest that younger age and normal CT findings are important prognostic factors of acute thrombolytic therapy.