Clinical and Laboratory Characteristics of Recurrent Optic Neuritis; Comparison with Monophasic Optic Neuritis

BACKGROUND: Although relapses are known to be common in optic neuritis, there are only a few follow-up studies concerning recurrent optic neuritis. The aim of this study is to characterize the difference between monophasic and recurrent optic neuritis by analyzing clinical and laboratory spectrums of index event. METHODS: We performed a partially retrospective and prospective cohort study of patients with optic neuritis. The patients with optic neuritis were included by review of their medical records and neuroimaging studies and then followed up for the relapses of optic neuritis. Excluded were those who showed any evidence of multiple sclerosis, and those with prior demyelinating attacks. RESULTS: Thirteen of 43 enrolled patients had a recurrent optic neuritis during a mean (SD) follow up period of 58.0 (21.2) months, yielding a 5-year cumulative rate of recurrence of 39.5 percent. The patients who had CSF pleocytosis were more likely to develop a recurrent attacks (P<0.05), but neither clinical findings nor the other laboratory results appeared to influence recurrence. CONCLUSIONS: We suggest that this disorder have a distinctive feature in terms of relapse and CSF pleocytosis compared with monophasic optic neuritis.

Loss of Striatal Dopamine Transporter Binding in Patients and a Family Member with Familial Parkinsonism with Parkin Gene Mutation

No abstract available.

The Effect and the Mechanism of Action of High Frequency Electrical Stimulation of Subthalamic Nucleus on Status Epilepticus Induced by Lithium-Pilocarpine of Rat / 대한간질학회지

OBJECTIVES: Deep brain stimulation (DBS) of subthalamic nuclei (STN) is one of the current modalities of refractory epilepsy, but its exact mechanism and route of action have not been elucidated yet. We investigated the effect of STN stimulation on the development and propagation of seizures in the rats with lithium-pilocarpine induced status epilepticus in its functional anatomy. METHODS: Both pilocarpine injection and high frequency stimulation on STN (HFSSTN) were provided to rats (STN group, n=12), but pilocarpine injection with no stimulation was done on the sham group (n=8). The latency to first discrete ictal discharges and the latency to status epilepticus (SE) were analyzed and the electrical stimulation lasted for 30, 60, 90, 120 minutes after its first discrete spikes. After stimulation, the rats were immediately decapitated for immunohistochemistry and histologic examination. RESULTS: Both the latency to first discrete ictal discharges and the latency to the onset of SE were delayed in the STN group than in the sham group. The latency to the first SE was also more delayed in the STN group (42.7+/-7.9 min) than in the sham group (p<0.05). Remarkably, there was marked Fos immunoreactivity (FIR) on the reticular thalamic nuclei in the STN group, but not in the sham group. CONCLUSIONS: Increased FIR in the reticular thalamic nuclei during HFSSTN suggested that the facilitation of the inhibitory thalamic output prevented generalized motor seizure behavior. We assume that HFSSTN has a pivotal role in the suppression or progression to SE, but cannot prevent seizure onset.

Antiepileptic and Neuroprotective Effect of Ketamine in Lithium-Pilocarpine Induced Status Epilepticus Rat Model / 대한간질학회지

PURPOSE: To examine the putative seizure-protective properties of ketamine in lithium-pilocarpine induced status epilepticus (LPSE). METHODS: Lithium chloride followed 24 h later by pilocarpine was administered for seizure induction. Ketamine (40 mg/kg) or phenytoin (50 mg/kg) was injected intraperitoneally 10 min or 60 min after the onset of continuous ictal discharge. Then the seizure behavior and EEG were observed and histological changes were compared through Nissl stain at 72 hours. RESULTS: The antiepileptic effect of ketamine, injected during the early stages of LPSE (10 min after the onset of continuous ictal discharge), was comparable to that of phenytoin. Ketamine was more effective than phenytoin in decreasing spike frequency, when administered on the plateau of LPSE (injection 60 min after onset of continuous ictal discharge electrographically). Anticonvulsant action of ketamine was confirmed by a less neuronal injury in hippocampus compared with control rats injected with phenytoin. CONCLUSIONS: In prolonged status epilepticus rat model, ketamine was effective as an antiepileptic, but phenytoin was not. Ketamine was also neuroprotective on the neuronal injury in the hippocampus. These results suggest that ketamine might be useful as an antiepileptic drug when standard antiepileptic drugs fail in the treatment of the refractory cases of status epilepticus.

Neuronal Cell Death in the Contralateral Hippocampus after Unilateral Hippocampal Kainic Acid-induced Seizure in Rats / 대한간질학회지

BACKGROUND: The recurrent temporal lobe epilepsy induces contralateral cell damage and secondary epileptogenesis in the contralateral hippocampus of rats. This phenomenon is fairly constant and has been used as a model of human temporal lobe epilepsy. It is necessary to understand this patho-mechanism in order to prevent this cell damage. METHODS: We have investigated the patho-mechanism of secondary epileptogenesis by using the rat model injected with kainic acid (KA) into the unilateral hippocampus. KA model shows initial complex partial seizures originating from the limbic structures and following convulsive status epilepticus. Immunohistochemical staining for c-fos expression, TUNEL stain for apoptosis, and hematoxylin-eosin (H-E) stain for morphologic changes were used. RESULTS: In the injected hippocampus, transient activation of c-fos was expressed in the dentate gyrus and CA3 hippocampal area, which were shaded out within 24 hours after the onset of limbic seizure. The stained cell with normal appearance was not observed in the H-E stain after 72 hours due to diffuse cell death. In the contralateral hippocampus, transient expression of c-fos was observed in the dentate gyrus, hilus, CA3, and CA1 area. But the expression of c-fos in the CA3 and CA1 area was sustained to 24 hours. Cell loss was mild in the CA3 and hilus, and mild cell degeneration and shrinkage were observed in the CA1 area. Apoptotic body was expressed in the CA1 area at 72 hours after the onset of seizure. CONCLUSION: These results mean that the area of prolonged expression of c-fos is vulnerable to apoptosis. Also it suggests that the patho-mechanism of ipsilateral hippocampus is an acute cytotoxic edema, whereas the contralateral damage is an apoptosis.

A Case of Pericarotid Syndrome Associated with Malignant Lymphoma

Pericarotid syndrome is the combination of a postganglionic Horner's syndrome and ipsilateral head and facial pain, which is caused by diverse pathologic processes in and around the internal carotid artery. We report a case of peri-carotid syndrome which presented Horner's syndrome and ipsilateral periodic severe hemicrania associated with malig-nant lymphma lapping internal carotid artery. After surgical removal of the mass and chemotherapy, miosis, ptosis, and ipsilateral hemicrania improved.

A Case of Digoxin-induced Catatonic Stupor

We experienced a rare clinical manifestation of a digoxin induced catatonic stupor without other features of digoxin toxicity. This case suggests that the neurological manifestation of digoxin toxicity can occur without the usual side effects. Also, a serum digoxin level should be checked in any elderly patient presenting with abnormal cerebral func-tions, irrespective of whether or not the dose of digoxin has been changed. (J Korean Neurol Assoc 19(4):438~439, 2001)

Chromosomal Assay after In-vitro Irradiation of Lymphocytes in Ataxia Telangiectasia

BACKGROUND: Hypersensitivity to both cell-killing and chromosome-damaging effects of ionizing radiation is a consistent feature of cells from individuals with ataxia-telangiectasia (AT). This radiobiological behavior of AT cells is a component of genetic instability and may contribute to cancer risk. Also, heterozygotes for AT-mutated (ATM) genes have no clinical expressions of AT, but may become cancer prone with a moderate increase in in-vitro radiosensitivity. METHODS: We performed a chromosomal analysis on lymphocytes from 3 AT patients, 5 obligate AT carriers (siblings and parents of the patients), and 5 normal controls. RESULTS: Increases in chromosomal breakages after irradiation with 1 gray/min in cells from AT patients ranged from 0.65 to 0.83 rearrangements per metaphase, while in the carriers and controls the levels of breakage were between 0 and 0.15 per metaphase cells (P<0.05). CONCLUSIONS: These results are consistent with previously reported chromosomal radiosensitivity in AT patients. However, carriers do not show moderate radiosensitivity due to various technical factors such as the dose or distance of radiation. Although this research has some limitations due to the small numbers of patients, carriers and controls, this method may be an easy and useful diagnostic tool for AT patients in Korea. (J Korean Neurol Assoc 19(5):509~513, 2001)

A Case of Myasthenia Gravis after Allogeneic Bone Marrow Transplantation

A 38-year-old woman, affected by chronic myeloid leukemia, received a BMT from his HLA identical brother. A mild acute graft-versus-host disease (GVHD) developed during the first month after the BMT. A typical clinical and electrophysiological feature of myasthenia gravis (MG) developed 3 months after the BMT requiring medication with pyridostigmine and steroids. Laboratory findings including acetylcholine receptor antibody and other autoantibodies were negative. MG is a well-characterized autoimmune disease which, on rare occasions, is also diagnosed as chronic GVHD after BMT. We report a first case of MG during an acute GVHD period. Since the patient had a myasthenic symptom during an acute GVHD period and no evidence of antibody mediated autoimmunity, this is likely to be an immune complication of acute GVHD. (J Korean Neurol Assoc 19(1):60~61, 2001

Immunosuppressants induced reversible posterior dominant emcephalopathy-A capillary leak syndrome

BACKGROUNDS: Recently, the cases with transient neurologic deficit and brain MRI abnormalities similar to hypertensive encephalopathy or eclampsia were reported in the organ transplanted patients, during the administration of cyclosporine with the hypothesis as "capillary lack syndrome". But in addition to cyclosporine, other immunosuppressive agents as cytabine and ant-lymphocyte globulin(ALG) may induce similar transient neurologic manifestations such headache, seizure, altered mental state and change of signal intensities in brain MRI. METHODS AND CASES: 11 patients who had suffered form hematologic disorder were included in the study. The patients were presented with a reversible transient neurologic manifestations and brain MRI abnormalities during administration of various immunosuppressant,. We analysed neourologic symptoms and signs, anatomic localizations and laboratory findings. RESULTS: The underlying hematologic disorder were severe aplastic anemia acute lymphocyte leukemia, multiple myeloma, chronic myelocytic leukemia and acute myelocytic leukemia. The cyclosporine was prescribed in six. ALG in three, idarubicin in two and prednisolone in one patient. The accompanied neurologic symptoms and sign were seizure(11/11), visual disturbance or cortical blindness(5/11) and mental status change(3/11). All the symptoms were spontaneously improved by conservative management. The insidious increase on blood pressure, hepato-renal impairment, sepsis, and hepatopathy were noted in some cases just before and after the neurologic manifestation. The ESR was elevated in all examined cases and the cholesterol level was normal. Serum cyclosporine was elevated in 2/6 cases. They showed typical MRI findings which were high signal intensity an T2WI and iso- to low signal intensity on TIWI with blunding of cortical sulci and with no enhancement. The involved lesions were the parieto-occipital area, frontal lobe, temporal lobe, and basal ganglia in series. One patient showed a petechial hemorrhage in the occipital area on both CT and MRI. In six cases, follow-up MRI showed nearly complete resolution of the lesions correlated with the clinical symptoms. CONCLUSION: We can't explain the exact mechanisms of the neurologic complication of immunosuppressants, but the characterstics of transient neurologic deficits and the corresponding reversible brain image are similar to those of hypertensive encephalopathy or eclampsia. It is suggested that the mechanism of clinical syndrome maybe a capiliary leak due to the cytokine-induced vasculopathy but future studies are warranted.