BACKGROUND: A newly-found mitochondrial toxin, 3-nitropropionic acid (3-NP), has been proved to induce apoptosis in the striatum. Although striatal lesions produced by 3-NP could develop through an excitotoxic mechanism, the exact relationship between apoptosis induction and excitotoxicity after 3-NP treatment is still not clear. We investigated the role of excitotoxicity and oxidative stress on apoptosis induction within the striatum following intra-peritoneal injection of 3-NP. METHODS: 3-NP was injected for 5 days intra-peritoneally in three month-old mice. One day after the last injection, animals were decapitated. To confirm the presence of apoptosis, we performed in-situ detection of DNA fragmentation by using TUNEL technique and agarose gel elctrophoresis after DNA extraction from striatum. To examine the effect of frontal cortex removal on 3-NP-indeced apoptosis, we removed left frontal cortex by aspiration. For excitotoxicity, NMDA-receptor antagonist-MK 801, non-NMDA antagonist-NBQX, and saline were injected intraperitoneally before 3-NP treatment To detect superoxide, we administered hydroethidium (HEt: 200 ul; 1mg/ml) into the jugular vein 2 days after 3-NP, and the density of oxidized HEt in samples were examined under flouscent microscope. We performed caspase staining to test immunoreactivity of caspase 3 in samples. RESULTS: The TUNEL positive cells were not observed in the striatum ipsilateral to the frontal cortex-removed side, but found in the contralateral striatum. Superoxide radicals measured by using HEt and caspase immunoreactivity were also significantly weaker in the striatum ipsilateral to the frontal cortex-removed side than the contralateral striatum. TUNEL staining revealed less apoptotic changes in the striatum of MK801-treated group than NBQX-or saline-treated groups. DNA laddering on agarose gel electrophoresis was observed in the striatum of NBQX- or saline-treated mice, but not found in MK 801-treated group. CONCLUSION: We demonstrated that removal of the corticostriatal glutamate pathway reduced superoxide production as well as apoptosis induced by 3-NP and NMDA receptor antogonist, but not non-NMDA antagonist, prevented 3-NP-induced apoptosis in the striatum. These results suggest that NMDA-mediated glutamatergic excitotokicity plays an important role in 3-NP related striatal damage.
Animals ; Apoptosis ; Caspase 3 ; DNA ; DNA Fragmentation ; Electrophoresis, Agar Gel ; Glutamic Acid ; In Situ Nick-End Labeling ; Injections, Intraperitoneal* ; Jugular Veins ; Mice* ; Mitochondria ; N-Methylaspartate ; Oxidative Stress ; Sepharose ; Superoxides

To get a better insight into the clinical differentiation between vertigo of cerebrovascular origin and of aural origin, we investigated radiologically proven stroke patients who presented with vertigo as an initial clinical manifestation. Of 154 stroke patients, 30 patients with vertigo (20%) had the relevant lesion, demonstrated with the initial computerized tomographic scan (13 patients) or the follow-up magnetic resonance imaging (MRI) study (17 patients) of the brain. Every lesion was in the vertebrobasilar arterial territory; 19 in the cerebellum, 8 in the pons, and 3 in the medulla oblongata. Although 12 of the 30 patients (40%) presented with vertigo in isolation at the onset of stroke, eight patients (27%) developed additional neurologic abnormalities from four hours to seven days later. Patients with isolated vertigo (13%) had the small lesion exclusively in the cerebellum of the PICA medial branch territory. The most frequent accompanying neurological sign was swaying in the cerebellar and medullary lesion, and dysarthria in the pontine lesion. The direction of nystagmus or swaying did not match the lesion side in some patients. Our findings suggest that cerebellar stroke may commonly manifest isolated vertigo or vertigo with swaying mimicking labyrinthine disorder, particularly at the onset of the disease. MRI study and tests for truncal ataxia and lateropulsion may be crucial for the detection of vertigo of cerebrovascular origin.
Adult ; Cerebrovascular Disorders/complications/*radiography ; Diagnosis, Differential ; Female ; Human ; Male ; Nervous System Diseases/etiology ; Nystagmus, Pathologic/etiology ; Prospective Studies ; Sensation Disorders/*diagnosis ; *Tomography, X-Ray Computed ; Vertigo/complications/*radiography

Although it is well known that the respiratory failure is a major cause of death in most patients with chronic neuromuscular disease, predominant respiratory dysfunction without severe involvement of limb muscles is an unusual complication of mitochondrial myopathy in adult age. We experienced two cases of mitochondrial myopathy with severe involvement of respiratory function and only mild involvement of limb muscles. One is a 16 year old female and another is a 22 year old male. The diagnosis is based on morphologic characteristics of "ragged red fibers" under the light microscope and abnormal mitochondrias on the electron microscope in the muscle biopsy.
Adolescent ; Adult ; Electromyography ; Female ; Human ; *Mitochondria, Muscle/ultrastructure ; Muscular Diseases/*complications ; Respiration, Artificial ; Respiratory Insufficiency/*etiology/therapy

The myoclonic epilepsies of infancy and early childhood pose the most difficult problems in the diagnosis and classification of epilepsies because they are often confused with the Lennox-Gastaut syndrome sharing a number of common features. However, their correct differentiation is easily justifiable because some of the myoclonic epilepsies of early childhood have better prognosis than the Lennox-Gastaut syndrome. We experienced and treated a 4-year-old boy who had normal intellectual function but frequent myoclonic and generalized clinic-tonic-clinic seizures, which were successfully controlled by anti-epileptic drugs. Hence we report a case with brief review of literatures.
Child, Preschool ; Classification ; Diagnosis ; Epilepsies, Myoclonic* ; Epilepsy ; Humans ; Male ; Prognosis ; Seizures

We evaluated and compared procedural memory and auditory P300 event-related potential in age-matched normal controls (n = 15) and drug-naive patients with Parkinson's disease (n = 16). We used Gollin's incomplete picture test for visual procedural memory function and Tower of Hanoi puzzle for visuomotor procedural memory function. The mean latency of P300 was significantly prolonged in the Parkinsonian group than in the controls. In the neuropsychology test, the patients group revealed selective impairment of visuomotor procedural memory against preserved visual procedural memory. In the patients group, the latency of P300 was inversely correlated with performance of visuomotor procedural memory. These results suggest that prolonged auditory P300 event-related potential show the dysfunction of visuomotor procedural memory in the basal ganglia, which appears to be more selectively impaired than visual procedural memory in drug-naive patients with Parkinson's disease.
*Event-Related Potentials, P300 ; *Evoked Potentials, Auditory ; Female ; Human ; Male ; Memory/*physiology ; Middle Age ; Neuropsychological Tests ; Parkinson Disease/*physiopathology/*psychology ; Reaction Time

Objective: Mild cognitive impairment (MCI) is considered a pre-stage of dementia. This study aims to develop a cognitive intervention treatment program (CITP) as a non-pharmacological therapy, apply this program into MCI patients, and examine patients’ changes in cognitive function. Methods: Among 16 patients with MCI, 10 patients were randomly assigned to the experimental group and 6 patients were assigned into the control group. The patients assigned to the experimental group participated in the CITP once a week for a period of 15 weeks.The control group were suggested to live a normal daily life without CITP given. After 15 weeks (3 months), pre- and post-investigations, such as cognitive function test, emotional test, brain oxygen saturation test, were conducted and compared for each group. Results: The cognitive function scores and the brain oxygen saturation levels taken during the Verbal Fluency Test showed a sta-tistically significant difference between those of experimental and the control groups. To be specific, while the cognitive function score improved in the experimental group, there was a decline in the control group. There was no statistically significant difference in emo-tional changes between two groups. Looking at the changes within each group, the overall cognitive function score of the experimen-tal group was significantly increased, but no pre- and post-significantly changes were observed in brain oxygen saturation activation. On the other hand, the control group showed a statistically significant decline in the attention criteria of the cognitive functional ar-eas, and no statistically significant changed in brain activation. Conclusion The result from this study has given some promising views on maintaining and improving the deteriorating cognitive function in patients with MCI. Conducting CITP on patients is expected to strengthen the neural network. Eventually, there would be a less deterioration of cognitive function and less progression of MCI into dementia.

Rett's syndrome(RS) is a progressive neurodegenerative disorder characterized by exclusive occurrence in females, autistic behavior, dementia, gait ataxia, loss of purposeful use of the hands with stereotypic hand movement, and seizures. Initially RS was considered to be very rare; however, recent reports suggest that the prevalence is considerably higher and occurrence is world-wide. Because the pathophysiological process remains unknown, the diagnosis of RS is based mainly on its characteristic clinical features and course. We experienced two cases of RS which, to our knowledge, are the first reported in Korea. It is quite possible that many patients with RS not yet being diagnosed in Korea.
Child ; Child Development/physiology ; Female ; Haloperidol/therapeutic use ; Human ; Korea/epidemiology ; Rett Syndrome/drug therapy/*epidemiology/physiopathology ; Sex Factors

BACKGROUND AND OBJECTIVES: Stroke onset is known to vary by several factors. Although it has been known that stroke may develop most frequently in the morning, its association with the type of activity has quite rarely been described. METHODS: We prospectively investigated by interview the time of and the activity during or before the onset of stroke in patients with acute cerebral infarction from Aug. 1995 to Mar. 1996. The activities were subdivided into basal metabolic rate state, sedentary, light, moderate, and heavy movements based on the caloric expenditure. RESULTS: One hundred-twenty five patients were enrolled. The time of day when ischemic stroke most frequently occurred was from 8:00 AM to noon. The type of activity was significantly associated with stroke onset in that it developed most commonly during and just after sleep or resting. The relationship between the onset of stroke and such patterns of onset time and the activity was found only in the atherothrombotic infarction, but not in the other stroke types. CONCLUSION: We demonstrated that stroke has clear diurnal variation. Our observations also suggested that the activity may be significantly associated with stroke onset. These findings may be useful for better understanding of the pathogenesis and prevention of ischemic stroke.
Basal Metabolism ; Cerebral Infarction* ; Health Expenditures ; Humans ; Infarction ; Prospective Studies ; Stroke

Hemintaxia-hypesthesia in thalamic stroke has been rarely reported. We experienced six patients who has hemiataxia-hypesthesia with or without transientweakness due to thalamic stroke. Upon reviewing the pattern of sensory deficits and the presence of weakness m our cases and those in previously reported thalamic stroke patients with hemiataxiahypesthesia with or without transient weakness, hemiataxia-hypesthesia has a localizing value of the thalamic lesion, particularly in case of presenting with pain sensory loss and of no weakness.
Humans ; Stroke*

C-reactive protein(CRP) of the serurn and CSF vras quantitatively measured in patients who were diagnosed as tuberculous meningitis(19 cases), aseptic meningitis(44 cases) and control group(23 cases) in order to evaluate the diagnostic value and the possible dynarnics of CRP between serum and CSF. Following results were obtained. 1. Significant difference(p<0.005) OF CRP was observed in the initial CSF of the patients with tuberculous meningitis and aseptic meningitis. When the CRP of CSF was set above 0.4rng /dl, a sensitivity of 0 79 apd a specificity of 0.90 were obtained. 2. When the CRP in the initial serurn as compared, significant difference(p<0.0210) was also observed between the patients with tuberculous rneningitis and aseptic meningitis. However, serurn CRP were not diagnostic because of significant overlap between the tuberculous and the aseptic meningitis. 3. On follow up measurement of CRP, patients with tuberculous meningitis showed signihcant decrease in both serurn and CSF while patients with aseptic meningitis showed significant decrease only in the serum. 4. In both groups of patients with tuberculous meningitis and aseptic meningitis, CRP values in the CSF were closely correlated to those of the serum(Pearson's r=0.25326 p<0. 035, Pearson's r=0.4520 p<0.0000 respectively) Considering that the content of protein was also significantly(p<0.000) elevated in the patients with tuberculous meningitis and that the CRP ratio(Qcrp) was also significantly(p<0.0035) elevated in comparison to the controls, the increased CRP in the CSF of tuberculous meningitis is probably due to an increased simple diffusion through the blood-CSF barrier resulted from increased serurn CRP and through impairrnent of blood-CSF barrier.
Diffusion ; Follow-Up Studies ; Humans ; Meningitis, Aseptic ; Sensitivity and Specificity ; Tuberculosis, Meningeal