Electrophysiological Changes in Lambert-Eaton Myasthenic Syndrome With Intravenous Immunoglobulin Therapy

No abstract available.

Two Cases of Cerebral Venous Thrombosis with High Signal Intensity of Intravascular Clots on Diffusion Weighted Image

High signal intensity on diffusion-weighted image (DWI) at the site of venous occlusion has previously been reported in cerebral venous thrombosis (CVT). The frequency and diagnostic value of these signal changes in CVT were unknown. Some authors suggest that the presence of high signal intensity on DWI in occluded veins might help diagnose CVT and suggest low rate of recanalization. We experienced two cases of CVT with high signal intensity at the site of intravascular clot on DWI.

Mild Encephalopathy with Reversible Lesion in the Splenium of the Corpus Callosum and Bilateral Frontal White Matter

A 59-year-old man visited an emergency room due to the sudden onset of severe dysarthria with a drowsy mental status. MRI demonstrated T2 prolongation and restricted diffusion involving the splenium of the corpus callosum and bilateral frontal white matter neurological signs and symptoms were mild, and the recovery was complete within a week. Follow-up MRI performed one month later revealed complete resolution of the lesions. The clinical and radiological courses were consistent with previously reported reversible isolated splenial lesions in mild encephalitis/encephalopathy except for the presence of frontal lesions. This case suggests that such reversible lesions can occur outside the splenium.

Inhibitory Effects of Valdecoxib on Sodium Currents in Sensory Neurons

BACKGROUND: Valdecoxib is a selective cyclooxygenase-2 (COX-2) inhibitor. It is effective in the treatment of rheumatoid arthritis, osteoarthritis, primary dysmenorrhea, and postoperative pain. Two kinds of sodium currents, tetrodotoxin-sensitive (TTX-S) and tetrodotoxin-resistant (TTX-R), are expressed in the dorsal root ganglia (DRG). Both sodium currents are implicated in the formation of normal and abnormal pain. METHODS: The effects of valdecoxib on sodium currents in rat DRG neurons were investigated using the whole-cell variation of the patch-clamp technique. RESULTS: Valdecoxib suppressed two types of sodium currents in a dose-dependent manner, without altering the activation and inactivation kinetics of either current type. It shifted the activation voltage toward a depolarizing direction and the steady-state inactivation voltage toward a hyperpolarizing direction, and suppressed resting channels to similar extents in both types of sodium currents. Valdecoxib slowed the recovery of both sodium currents from inactivation, and suppressed them in a frequency-dependent manner. CONCLUSIONS: The results suggest that valdecoxib may produce analgesic effects through the inhibition of sodium currents in sensory neurons as well as COX-2.

A Case of Reversible Peripheral Neuropathy Caused by Chronic Phenytoin Intoxication / 대한간질학회지

Peripheral neuropathy has been uncommon reported as one of neurologic adverse effects associated with phenytoin. A 53 year-old man presented with clinical and electrophysiologic signs of peripheral neuropathy after 13 years of phenytoin administration. Despite a modest dose of phenytoin (300 mg/day), blood level was 40 microg/ml. After discontinuing phenytoin about eight months, the peripheral neuropathy was improved. We present a case of reversible peripheral neuropathy caused by chronic phenytoin intoxication.

Isolated Weakness of Middle, Ring, and Little Fingers due to a Small Cortical Infarction in the Medial Precentral Gyrus

Small cortical strokes can produce predominant isolated weakness in a particular group of fingers: radial or ulnar. The traditional views are of point-to-point representations of each finger to neurons located in the precentral gyrus of the motor cortex such that the neurons of the radial fingers are located laterally and those of the ulnar fingers are located medially. We present a case of isolated weakness of middle, ring, and little fingers due to a small cortical infarction in the medial precentral gyrus.

A Case of Nonaka Myopathy Confirmed by GNE Mutation

Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles (DMRV) is an autosomal recessively inherited neuromuscular disorder characterized by early adult-onset weakness of distal muscles, rimmed vacuoles in muscle biopsy, and mutations in the UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE) gene. The authors describe a patient with typical clinical features of NM confirmed by GNE mutation. Mutation analysis of the GNE gene revealed that the patient was a compound heterozygous for V572L and C13S mutations.

Comparison of the Protective Effect of Indole beta-carbolines and R-(-)-deprenyl Against Nitrogen Species-Induced Cell Death in Experimental Culture Model of Parkinson's Disease

BACKGROUND: The membrane permeability transition of mitochondria has been suggested to be involved in toxic and oxidative forms of cell injury. Mitochondrial dysfunction is considered to play a critical role in neurodegeneration in Parkinson's disease. Despite the suggestion that indole beta-carbolines may be neurotoxic, these compounds provide a protective effect against cytotoxicity of other neurotoxins. In addition, the effect of indole beta-carbolines on change in the mitochondrial membrane permeability due to reactive nitrogen species (RNS), which may lead to cell death, has not been clarified. METHODS: Differentiated PC12 cells were used as the experimental culture model for the investigation of neuronal cell injury, which occurs in Parkinson's disease. The effect of indole beta-carbolines (harmalol and harmine) on differentiated PC12 cells against toxicity of S-nitroso-N-acetyl-DL-penicillamine (SNAP) was determined by measuring the effect on the change in transmembrane potential, cytochrome c release, formation of ROS, GSH contents, caspase-3 activity and cell viability, and was compared to that of R-(-)-deprenyl. RESULTS: Specific inhibitors of caspases (z-LEHD.fmk, z-DQMD.fmk) and antioxidants (N-acetylcysteine, dithiothreitol, melatonin, carboxy-PTIO and uric acid) depressed cell death in PC12 cells due to SNAP. beta-Carbolines and R-(-)-deprenyl attenuated the SNAP-induced cell death and GSH depletion concentration dependently with a maximal inhibitory effect at 25-50 microM. The compounds inhibited the nuclear damage, decrease in mitochondrial transmembrane potential, cytochrome c release and formation of reactive oxygen species caused by SNAP in PC12 cells. beta-Carbolines and R-(-)-deprenyl attenuated the H2O2-induced cell death and depletion of GSH. CONCLUSIONS: The results suggest that indole beta-carbolines attenuate the SNAP-induced viability loss in PC12 cells by inhibition of change in the mitochondrial membrane permeability, which may be caused by free radicals. Indole beta-carbolines appear to exert a protective effect against the nitrogen species-mediated neuronal cell injury in Parkinson's disease comparable to R-(-)-deprenyl.

A Case of Brain Stem Neuro-Behcet's Disease Presenting as Paroxysmal Headache With Autonomic Features

Migraine without aura was significantly frequent in Beh et's disease patients. While, the relationship of TAC(trigeminal autonomic cephalalgia) and Beh et's disease was not known. And, it is thought that pathogenesis of proxysmal headache with autonomic features in neuroBehcet's disease as our case may be different from those of clust headache and TAC. A 45-year-old male admitted with left sided periobital poxysmal headache accompained by ipsilateral conjunctival injection and lacrimination with autonomic features. T2WMR image showed about 1.5x2.6x2.6 cm sized mass with high signal intensity surrounded by a rim of low signal intensity in pons. Enhanced MR image showed enhancing mass-like lesion inside the T2 high signal intensity. CSF study revealed neutrophilic pleocytosis with normal pressure, protein and glucose concentration. No malignant cell were seen. Antiphospholipid antibody and cryoglobuline was significantly increased. 3 days after hospitalvisit, oral ulcerations appeared in the patient. He was treated with high-dose steroid for a week and his headache and abnormal findings of CSF study became fully recovered. Two weeks later, the mass was nearly reduced in the pons on follow-up MR image. We suggest to include a careful interviewfor 2ndary TAC as the first neurologic symptom in the diagnostic work-up of Behet's disease.