BACKGROUND: Various neuronal and glial factors which participate in neural differentiation, including neural cell adhesion molecule (NCAM), are upregulated in pathogenesis of temporal lobe epilesy (TLE).This study aimed to investigate hte effect of (R-)-N6-phenylisopropyladenosine (RPIA), an adenosine A1 receptor agonist, on the morphological alteration of NCAM immunoreactivity (IR) in limbic system of Kainic acid (KA)-induced epileptic rats. METHODS: Experiment animals were divided into control group, KA treatment only (10 mg/kg. i.p.)group, and RPIA pretreatment (100 microgram/kg. i,p, 10 min prior to injection of KA) group. Animals were sacrificed at 24 hours and 1 week after KA treatment. Luxol fast blue-cresyl violet stain for histopathological observation, and NCAM immunohistochemistry to study alteration of NCAM IR in limbic system were performed. RESULTS: Neuronal loss in CA1 and CA3areas of hippocampus, piridorm cortex, basolateral amygdala nucleus and lateral dorsal thalamic nucleus were induced by KA unjection, and thoes were reduced by RPIA pretreatment. Inrease of NCAM-IR was observed in interneurons of all hippocampal areas. except CA2 area, pirform cortex and basolateral amygdala nucleus at 24 hours after KA injection. and increased NCAM-IR was observed in cell membrane and processes of neuroglia, dentate granule cells and pyramidal cells in CA1 area of hippocampus. and neurons in piriform cortex, amygdala and lateral dorsal thalamic nucleus 1 week after KA injection, but those changes were milder than those at 24 hours after KA injection. RPIA pretreatment significantly reduced KA-induced NCAM-IR in hippocampal CA3, CA1 area, piriform cortex, amtgdala and lateral dorsal thalamic nucleus. CONCLUSION: We suggest that decrease of NCAM immunoreactivity is associated with neuprotective effects of RPIA on limbic system against KA neurotoxiciy.
Amygdala ; Animals ; Cell Membrane ; Epilepsy, Temporal Lobe ; Hippocampus ; Immunohistochemistry ; Interneurons ; Kainic Acid ; Lateral Thalamic Nuclei ; Limbic System ; Neural Cell Adhesion Molecules ; Neuroglia ; Neurons ; Pyramidal Cells ; Rats ; Receptor, Adenosine A1 ; Temporal Lobe ; Viola

BACKGROUND AND OBJECT: Cheiro-oral syndrome (COS) is characterized by a sensory disturbance in the unilateral hand and ipsilateral mouth corner. It is usually due to a lesion in the parietal cortex, thatamocortical projections, thalamus, or rarely brain stem. However, the syndrome is relatively unknown and rarely mentioned in most neurological textbooks. We presented ten cases of COS with a review of the clinical symptoms and signs and the neuroradiological methods used to demonstrate the responsible site. METHODS AND RESULTS: We studied 10 patients with stroke who showed restricted sensory disturbance on the one hand and ispilateral mouth. The study forms consisted of clinical manifestaion, neurological examination, electrophysiological, and neuroradiologic studies. Computed tomography and/or magnetic resonance imaging identified lesion in the thalamus in 5, brain stem in 3, and corona radiata in 1 patient. But, the anatomical responsible site for one case was not founded. Infarction had occurred in nine cases and hemorrhage in one. Seven of the 10 patients showed sensory disturbances restricted to the perioral area, hands, fingers when they were first examined; the remaining patients complained more diffuse sensory disturbances at first, but it had become restricted to perioral and fingers, usually within 2-3 weeks. The durations of symptom varied from 5 days to more than 15 months and these symptoms were improved within 2-3 weeks to 4 months in treated patients. CONCLUSION: When the symptoms and signs of the COS were presented, especially if a history of migraine is lacking, neuroradiological methods such as CT or MRI should be undertaken to localize and diffentiate the nature of lesion.
Brain Stem ; Fingers ; Hand ; Hemorrhage ; Humans ; Infarction ; Magnetic Resonance Imaging ; Migraine Disorders ; Mouth ; Neurologic Examination ; Rabeprazole ; Stroke ; Thalamus

Paroxysmal choreoathetosis(dyskinesia) is classified into two subtypes: paroxysmal kinesigenic choreoathetosis(PKC) and paroxysmal dystonic choreoathetosis(PDC). PDC consist of attacks of dystonia and /or choreoathetosis during which the patients are dysarthric or anarthric, have irregular clonic movement and dystonic posturing of extremities, and these not precipitated by sudden movement but rather by alocohol, coffee and stress. Attacks are longer (2ninute-4hours), but less frequently(3-4times/day) than PKC, they are not responsive to anticonvulsants(eg, phenytoin, carbamazepine) but controlled by clonazepam.This disease is rare, in the reported families, the transmission was clearly autosomal dominant with high penetrance.
Coffee ; Dystonia ; Extremities ; Humans ; Penetrance ; Phenytoin

Sparganosis of the central nervous system is an uncommon parasitic zoonosis caused by the migrating larva of the genus Spirometra mansonoides. The patient, a 53-year-old woman, complained of focal involuntary movement involving the right face and headache. Three months after first admission, paraparesis developed. Serial MRI revealed cerebral and spinal lesions and micro-ELISA test disclosed positive reaction to sparganus antigen.
Central Nervous System ; Dyskinesias ; Female ; Headache ; Humans ; Larva ; Magnetic Resonance Imaging ; Middle Aged ; Paraparesis ; Sparganosis* ; Spinal Cord* ; Spirometra

Hereditary progressive dystonia develope in the first decade of ?life and is characterized by marked diurnal fluctuation of symptoms(dystonic postures and movements), i.e.symptoms aggrevated towards evening and alleviated after sleep. These symptoms and signs responded dramatically to levodopa. With the high incidence of familial cases this disorder is considers to be hereditary, but inheritance is not clear yet. Segawa regarded this disorder as a dystonia different from Parkinson's disease and other types of dystonic movement disorders, thereafter it has been reported under name of hereditary progressive dystonia with marked diurnal fluctuation.
Dystonia* ; Incidence ; Levodopa ; Movement Disorders ; Parkinson Disease ; Posture ; Wills

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Hereditary spastic ataxia is a familial neurological disorder which exhibit the features of a progressive combined pyramidal tract and cerebellar deficiency. The main features are progressive gait disturbance, incoordination, nystagmus, visual impairment, hyperreflexia, extensor plantar response, peripheral neuropathy, and pes cavus. A 27-year-old male patient with spastic ataxic gait was evaluated. He showed characteristic features of hereditary spastic ataxia. There were another twelve affected members in four generations of his family which may be inherited by autosomal dominant pattern. One of them is reported with review of the literature on familial spastic ataxia.
Adult ; Ataxia* ; Family Characteristics ; Foot Deformities ; Gait ; Humans ; Male ; Muscle Spasticity* ; Nervous System Diseases ; Peripheral Nervous System Diseases ; Pyramidal Tracts ; Reflex, Abnormal ; Reflex, Babinski ; Vision Disorders

The spontaneous hematomyelia is an uncommon event and its predisposing conditions are vascular malformation, syringomyelia, pregnancy and delivery, angioma, hemophilia, anticoagulant therapy, etc. We have recently experienced the patient with spontaneous onset and resolving hematomyelia in the cervical spinal cord. A 30-year-old male patient with non-traumatic spinal shock was evaluated. On MRI, a hematomyelia along cervical spi-nal cord was revealed. A suspicious AV malformation was noticed at C3-4 level. Fol-low-up MRIs showed spontaneous resolution of the hematoma.
Adult ; Hemangioma ; Hematoma ; Hemophilia A ; Humans ; Magnetic Resonance Imaging ; Male ; Pregnancy ; Shock ; Spinal Cord ; Spinal Cord Vascular Diseases* ; Syringomyelia ; Vascular Malformations

We treated a 66 year-old-male patient with non-traumatic spontaneous E. coli meningitis, whose cerebrospinal fluid showed turbid, and from which E. coli was cultured, and who had urinary tract infection and fatty liver disease which were thought to be predisposing factors to the meningitis. Gram-negative bacillary meningitis is a very rare condition after the neonatal period, and may be complicated by penetrating cranial injuries, neurosurgical interventions or such debilitating diseases as diabetes, liver cirrhosis, urinary tract infection, malignancy and alcholism, etc. It has a high mortality rate in spite of using various antibiotics.
Adult* ; Anti-Bacterial Agents ; Causality ; Cerebrospinal Fluid ; Fatty Liver ; Humans ; Liver Cirrhosis ; Meningitis* ; Mortality ; Urinary Tract Infections