A Case of Systemic Lupus Erythematosus Presenting with Amaurosis Fugax without Antiphospholipid Antibodies Syndrome / 영남의대학술지

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect many organ systems including the nervous system. The immune response in patients with SLE can cause inflammation and other damage that can cause significant injury to the arteries and tissues. A 48-year-old woman was admitted to the hospital because of transient monocular blindness. Magnetic resonance imaging and conventional angiography showed severe stenosis of the distal intracranial internal carotid artery. The patient was diagnosed as having SLE but the antiphospholipid antibodies were negative. Amaurosis fugax has not been previously reported as an initial manifestation of SLE in Korea. We report a patient with a retinal transient ischemic attack as the first manifestation of SLE.

A Case of Leptomeningeal Metastasis Associated with Cerebral Venous Thrombosis

A 59 year-old woman who had suffered daily headaches was diagnosed with leptomeningeal metastasis. Leptomeningeal metastasis is uncommon but devastating form of metastatic spread. Cerebral venous thrombosis is a disease sometimes associated with systemic cancer. Rarely, cerebral sinus occlusion accompanies leptomeningeal cancer. We report a patient who had cerebral sinus thrombosis and leptomeningeal metastasis.

A Case of ReVersible Basilar Artery Stenosis in Neuro-Behcet's Disease

The reversibility of basilar artery stenosis in neuro-Behcet's disease (NB) has been rarely reported. We report a patient with NB who developed brainstem infarction related to severe stenosis in basilar artery. Initial MRA showed severe stenosis in the basilar artery, which was improved on follow-up MRA after immunosuppressive treatment. This case shows that arterial stenosis in NB can be reversed with proper management.

Clinical and Electrophysiological Study on Guillain-Barre Syndrome / 영남의대학술지

BACKGROUND: Guillain-Barre syndrome is defined as a recognizable clinical entity that is characterized by rapidly evolving symmetric limb weakness, the loss of tendon reflexes, absent or mild sensory signs, and variable autonomic dysfunctions. This study evaluated the clinical and electrophysiological findings retrospectively. MATERIALS AND METHODS: Forty-five patients with Guillain-Barre syndrome, who were admitted to the Yeungnam University Hospital for six years from Jan. 1994 to Dec. 1999 were investigated. The correlation between the clinical manifestation and the electrophysiological study was evaluated. RESULTS: The male to female ratio was 1.8: 1 and there was a peak seasonal incidence in the winter. A preceding illness was noted in 66.7% of cases, and an upper respiratory tract infection was the most common one. The most common clinical manifestations were a loss of tendon reflex and ascending muscle weakness and paralysis. The cerebrospinal fluid examinations revealed, albuminocytologic dissociation in 33 cases (73.3%). Intravenous immunoglobulin therapy was performed in 29 cases (64.4%). The sequential electrophysiological abnormalities were most marked at 2 to 4 weeks after onset. At that time the most significant change was a decrease in the compound muscle action potential amplitude. These 45 patients with Guillain-Barre syndrome were subclassified using the clinical and electrophysiological data. CONCLUSION: The result in this study, concured with other research on the clinical and electrophysiological data of Guillain-Barre syndrome. However, an extensive and dynamic investigation is necessary to determine the reason for the peak seasonal incidence in winter.

Involvements of Oxidative Stress in beta-amyloid Peptide: induced Cytotoxicity in PC12 Cells

BACKGROUND: Alzheimer's disease is a neurodegenerative disorder characterized by the extracellular deposition of beta-amyloid peptide(Abeta) in the brain, presumed to play a pathogenic role. However, the precise molecular mechanisms of its neurotoxicity are not fully understood. METHODS: Abeta-mediated cytotoxicity in neuronal cell lines (PC12, SH-SY5Y, IMR32, and U87) was measured by an MTT assay. NF-kappaB activation by Abetawas examined by a luciferase assay and apoptosis induced by Abetawas measured by cytoplasmic DNA fragmentations. RESULTS: Abetacytotoxicity in the tested cell lines was more prominent in the absence of serum than in the presence of serum in culture media. PC12 cells showed the highest sensitivity to Abetacytotoxicity among the cell lines. The Abeta(25-35) cytotoxicity in PC12 cells was increased in a dose-dependent manner. For convincing oxidative stress involved in Abetacytotoxicity, antioxidants such as DTT, GSH, vitamin C, or NAC were pretreated. GSH protected PC12 cells from Abetacytotoxicity, but DTT or NAC did not. Abeta (25-35) treatment to PC12 cells increased the NF-kappaB activity in a dose-dependent manner. Cytoplasmic DNA fragmentations, one of the apoptotic indicators, were increased at lower concentrations of Abeta(25-35) from 0.01 to 0.1 microM, however, dose-dependent increments of DNA fragmentations were not observed at higher concentrations from 1 to 10 microM. CONCLUSIONS: From these results, Abeta-induced cytotoxicity in PC12 cells might be mediated by oxidative stress.

A Case with Recurrent Tonic Spasms After Acute Cervical Cord Myelopathy

Paroxysmal sensorimotor phenomena of the spinal cord have been associated with contrast material injection, transverse myelitis, spinal cord injury and multiple sclerosis. We report a 48-year-old woman with recurrent tonic spasms, which occurred 1 month after acute cervical cord myelopathy. A tingling sensation beginning in the left leg was followed by painful tonic spasm and abnormal sensation of the upper and lower right limbs except the face. Consciousness, scalp EEG was normal and SPECT showed hot uptake in the cervical cord during spasms.

A Case with Recurrent Tonic Spasms After Acute Cervical Cord Myelopathy

Paroxysmal sensorimotor phenomena of the spinal cord have been associated with contrast material injection, transverse myelitis, spinal cord injury and multiple sclerosis. We report a 48-year-old woman with recurrent tonic spasms, which occurred 1 month after acute cervical cord myelopathy. A tingling sensation beginning in the left leg was followed by painful tonic spasm and abnormal sensation of the upper and lower right limbs except the face. Consciousness, scalp EEG was normal and SPECT showed hot uptake in the cervical cord during spasms.

Antithrombotic Therapy for Ischemic Stroke / 영남의대학술지

Ischemic stroke is among the principal causes of death and disability in the elderly. Although control of blood pressure, decreased cigarette smoking, and modified dietary habits are among important reasons for stroke decline, the use of antithrombotic therapy, rigorously prescribed. Several antiplatelet agents are approved to reduce the risk of recurrent stroke. Aspirin is the best-studied and most widely used antiplatelet agent for stroke prevention; it provides approximately 15% to 25% relatively risk reduction for secondary prevention of stroke or the major vascular death. Combining 2 antiplatelet agents with different mechanism of action was demonstrated to provide a substantial increase in efficacy in several studies. Anticoagulation should be considered first with potential cardiac sources of embolism. Heparin reduces development of erythrocyte-fibrin thrombi that form in regions of vascular stasis especially within the heart, in severely stenosed arteries sometimes engrafted on white thrombi, in acute arterial occlusion. Heparin should not be indiscriminately given to all acute brain ischemia patients, but may contribute to treatment of large artery occlusion and severe stenosis, cardiogenic embolism with a high acute recurrence risk, and dural sinus and cerebral venous thromobosis.

The Comparison of Sensitivities of Electrophysiological Parameters in Carpal Tunnel Syndrome

BACKGROUND: Carpal tunnel syndrome (CTS) is the most common mononeuropathy encountered in clinical practice. When performing routine diagnostic nerve conduction studies (NCS) in patients with CTS, we sometimes happen to be confronted with patients who have no definite electrophysiological evidence of CTS. We therefore evaluated the diag-nostic sensitivities of several parameters in NCS for CTS patients. METHODS: We analyzed 187 patients (262 hands) who were diagnosed with CTS clinically and electrophysiologically for the past 4 years in this hospital. RESULTS: Significant abnormal findings of nerve conduction velocity (NCV) were noted in the palm to wrist (P-W) seg-ment( 98.5%), thumb-wrist (I-W) segment (97.3%), distoproximal ratio in the third finger (95.0%), and the terminal latency (71.4%) of the median nerve. There were no statistical differences in the abnormal NCVs between the male and female groups. The sensitivity of each compartment parameters were obtained in the female groups and statistical dif-ferences were found in the sensory NCV of the P-W segment and compound nerve action potential (CNAP) amplitude of the I-W segments in patients between the ages of 40 and 60. The sensory NCV of the P-W segment was especially more significant in this age group. There were statistical differences in the amplitude of the index finger to wrist (F-W) and P-W segments between the denervated electromyography group and normal group (p < 0.05). CONCLUSIONS: In clini-cally suspected CTS, the most sensitive tests were the sensory NCV of the P-W segment, I-W segment, and distoproxi-mal ratio in the third finger. Therefore, the sensory NCV of the P-W segment should be performed first. If the results of this test are unremarkable, then, the next tests should include the sensory NCV of the I-W segment and the distoproxi-mal ratio should be performed before ruling out the diagnosis of CTS.

A Case of Embolic Cerebral Infarction Following Disulfiram Poisoning

A 29 year old man without any noticeable past or familial history presented with left ACA and MCA territorial hemorrhagic infarction after consuming an overdose of disulfiram in a drunken state. This hemorrhagic infarction was thought to be caused by cardiac embolism, which developed following cardiac arrhythmia and cardiac injury induced by O form acceptor reductase associated with disulfiram poisoning. (J Korean Neurol Assoc 19(3):294~297, 2001)

Diffusion-Weighted MR Imaging in Animal Model with Acute Ischemic Brain Infarction: Evaluation of Reversible Brain Injury

PURPOSE: To determine whether the analysis of abnormally high signal intensities in ischemic tissue, as revealed by diffusion-weighted MR imaging (DWI) can be used to evaluate reversible brain lesions in a cat model of acute ischemia. MATERIALS AND METHODS: Ten cats were divided into two groups of five (Group I and Group II), and in all animals the middle cerebral artery was temporarily occluded. Group I underwent T2-DWI 30 minutes after occlusion, and Group II 120 minutes after occlusion. In both groups, DWI was performed one hour and 24 hours after reperfusion (at one hour, non-T2-weighted; at 24 hours, T2-weighted). Both occlusion and reperfusion were monitored by 99m TC-ECD brain perfusion SPECT. All animals were sacrificed 24 hours later and their brain tissue was stained with TTC. Signal intensity ratios (SIR, signifying average signal intensity within the region of interest divided by that in the contralateral, nonischemic, homologous region) of the two groups, as seen on DWI were compared. The percentage of hemispheric lesions occurring in the two groups was also compared. RESULTS: SIR after occlusion of the middle cerebral artery was 1.29 in Group I and 1.59 in Group II. Twenty-four hours after reperfusion, SIR in Group I was higher than in Group II (p<0.01). After occlusion and reperfusion, the percentage of hemispheric lesions in Group I was less than in Group II. For the latter, the percentage of these lesions revealed by TTC staining and T2-weighted imaging was 48% and 59%, respectively, findings distinctly different from those for Group I. In addition, in group I, infarction was revealed by neither TTC staining nor T2-weighted imaging (p<0.01). CONCLUSION: The use of DWI to evaluate signal intensity ratios can help determine whether or not brain injury after temporary cerebral ischemia is reversible.

Diagnostic Sensitivity of Several Muscles in Repetitive Nerve Stimulation Test for Myasthenia Gravis / 영남의대학술지

BACKGROUND: This study was undertaken to evaluate the diagnostic sensitivity of several muscles in repetitive nerve stimulation test (RNST) for myasthenia gravis (MG) patients. MATERIALS AND METHODS: The study population consisted of 39 MG patients classified by modified Ossermann's classification. Using Stalberg's method, RNST was systematically performed in facial (orbicularis oculi and nasalis) and upper extremity (flexor carpi ulnaris, abductor digiti quinti and anconeus) muscles. RESULTS: The significant electrodecremental response of RNST were noted in orbicularis oculi (58.9%), nasalis (51.3%), flexor carpi ulnaris (42%), anconeus (41%) and abductor digiti quinti muscles (27%). Among the 3 muscles of upper extremity (abductor digiti quinti, flexor carpi ulnaris and anconeus), the positive electrodecremental response of anconeus muscles was significantly higher than other two muscles (p<0.05) in type IIa, IIb and there were no statistical differences of the positive electrodecremental response between orbicularis oculi and nasalis muscles. The facial muscles showed more prominent decremental responses than upper extremity muscles in type I MG(p<0.05). In type IIa MG patients, there were no significant statistical differences between facial and upper extremity muscles but significant statistical differences among upper extremity muscles. In type IIb MG patients, there were no significant statistical differences in all tested muscles in spite of the increased positive electrodecremental response of RNST. CONCLUSION: On the basis of this study, RNST would be initially performed for the orbicularis or nasalis in type I MG and for the anconeus in type IIa or IIb MG.

Comparison of the Repetitive Nerve Stimulation Test(RNST) Findings Between in Upper and Lower Extremity Muscles in Myasthenia Gravis / 영남의대학술지

BACKGROUND AND PURPOSE: This study was undertaken to compare the sensitivity of the Repetitive Nerve Stimulation Test (RNST) between the upper and lower extremity muscles in myasthenia gravis(MG) patients. MATERIALS AND METHODS: The study population consisted of 20 normal persons(control group) and 10 MG patients(MG group). Using Stalberg's method. RNST was systemically performed in orbicularis oculi muscle. upper extremity muscles(flexor carpi ulnaris. abductor digiti quinti), and lower extremity muscles(tibialis anterior. extensor digitorum brevis. vastus medialis). RESULTS: There were statistical differences of decremental response(mean+/-SD) in orbicularis oculi and upper extremity muscles between the control and MG groups(p<0.05 or p<0.01). However, there was no statistical difference of decremental response(mean+/-SD) to RNST in lower extremity muscles between the control and MG groups. There were highersensitivity in orbicularis oculi and upper extremity muscles than lower extremity muscles. Although positive reponse were detected in the lower extremity muscles, the positive response rates of lower extremity muscles were lower than o.oculi and upper extremity muscles. CONCLUSIONS: When the response rates of RNST in facial and upper extremity muscles are normal, may not be required RNST in lower extremity muscles.

A Case of Leber's Hereditary Optic Nouropathy Showing 11778 Point Mutation of Mitochondrial DNA / 영남의대학술지

Leber's hereditary optic neuropathy(LHON) is an optic nerve disease that causes blindness and is associated with maternally inherited mitochondrial DNA(mt DNA) mutations. The most common mitochondrial DNA mutation among LHON patients is a point mutation at the nucleotide 11778 in the subunit 4 of complex I. In one 45-year old male LHON patient with bilateral optic neuropathy, we investigated the presence of a point mutation of mitochondrial DNA and identified a single guanine to adenine transition mutation in the mitochondrial DNA at nucleotide point 11778.

A Case of Becker's Type Congenital Myotonia / 영남의대학술지

Congenital myotonia is a hereditary disorder of the skeletal muscle. The most characteristic features of the disease are myotonia and variable muscular hypertrophy. Molecular biologic investigations have revealed that mutations in the gene of the human skeletal muscle chloride ion channel protein are a cause of the disease. The Becker's type congenial myotonia is clinically similar to the autosomal dominantly inherited congenital myotonia (Thomsen's disease). Both disorders are characterized electrophysiologically by increased excitability of muscle fibers, reflected in clinical myotonia. In general, Becker's type congenital myotonia is more severe than Thomsen's disease in muscular hypertrophy and weakness. The authors recently experienced a 25-year-old female patient who has no family-related disease history and who has conspicuous muscular hypertrophy and the stiffness with muscles which occurred from the age of 3 or 4. Clinically she showed the authors a percussion myotonia. On electrophysiological study, exercise and repetitive stimulation of the abductor digiti quinti muscle disclosed a decline in the compound muscle action potential. Biopsy of biceps muscle revealed enlargement of muscle fibers with marked nuclear internalization. After the oral taking the Mexiletine, the patient showed a favorable turn a little with her stiffness of muscles. So we authors are reporting one case of Becker's type congenital myotonia with review of literatures.

Follow-up Study on Peripheral Neuropathy in Insulin-dependent Diabetes Mellitus in Childhood / 대한소아신경학회지

PURPOSE: Peripheral neuropathy is the most common chronic complication of insulin- dependent diabetes mellitus, which is one of the most frequent endocrine metabolic disorders in children. However, there are very few long-term follow-up studies on the condition in publication and none in Korean literature. In order to find out the correlation between the severity of peripheral neuropathy and glycosylated hemoglobin level, an indicator of diabetes control, a follow-up study was conducted on cases of insulin- dependent diabetes mellitus. METHODS: The study was conducted on 37 patients with insulin-dependent diabetes mellitus (12 males, 25 females, aged 7-21 years) who were diagnosed and were being treated in general hospitals in the Taegu-Kyungpook area from 1994 to 1998. They were also subject to nerve conduction velocity test at least two times with an interval of one or more years. Nerve conduction velocity was measured by using type MEM-3102 apparatus of Nihon Kohden (on motor and sensory component of left ulnar nerve and median nerve, motor component of left and right peroneal, posterior tibial nerve, sensory component of left and right sural nerve) and medical history was taken, neurological examination was done and glycosylated hemoglobin levels were measured with each test. RESULTS: The total number of nerve conduction velocity tests was 89 and the results were divided into 5 groups [normal : 14 cases (15.7%); borderline: 35 cases (39.3%); mononeuropathy : 2 cases (2.2%); mild polyneuropathy : 22 cases (24.7%); severe polyneuropathy : 16 cases (18.0%)] by the number and distributions of nerves that were found abnormal. Ten of the 26 cases, belonging to the normal or borderline group in the first test, showed signs of peripheral neuropathy in later tests, and of the 11 cases with peripheral neuropathy in the first test, none improved to be included in the normal or borderline group in the follow-up study. The mean glycosylated hemoglobin level was 9.2+/-2.5% in normal group, 11.9+/-3.2% in borderline group, 13.0+/-0.3% in mononeuropathy group, 12.0+/-3.7% in mild polyneuropathy group and 14.8+/-4.0% in severe polyneuropathy group, and glycosylated hemoglobin level rose with the severity of the neuropathy (r=0.372, p< 0.01). In the 9 cases that had shown improvement of peripheral neuropathy, glycosylated hemoglobin levels decreased significantly by an average of 3.5+/-1.3% with each level of improvement (t-test, p<0.05), but in 20 cases with deteriorating nerve conduction velocity, there was no significant correlation with increase in glycosylated hemoglobin levels. CONCLUSION: Peripheral neuropathy complicating insulin-dependent diabetes mellitus can occur during diabetes treatment and does not improve easily once it occurs. In this study, the glycosylated hemoglobin level was shown to have a high degree of correlation with the occurrence and progression of neuropathy, which means that control of hyperglycemia is difficult and also that stricter control of blood glucose during early stages of diabetes is necessary to prevent neuropathy. Follow-up studies with bigger samples and longer time will be needed for a more detailed look.

Comparison of Usefulness of Laboratory Tests in Diagnosis of Myasthenia Gravis / 영남의대학술지

PURPOSE: This study was undertaken to evaluate the clinical usefulness of Tensilon test, repetitive nerve stimulation test(RNST), single fiber EMG(SFEMG) test and acetylcholine receptor antibody(AchR Ab) assay for making diagnosis of myasthenia gravis(MG). METHOD: These tests were performed in 21 MG patients which were classified into 11 ocular, 5 mild generalized, 4 moderate generalized, and 1 chronic severe MG. RESULT: The overall positivity of Tensilon test, SFEMG and AchR Ab was 95%, 87%, and 76% respectively. The overall positivity of RNST was 67%; 38% on flexor carpi ulnaris, 43% on adductor digiti quinti and 62% on orbicularis oculi muscles. The positivity of each test was higher in generalized MG group than in ocular MG group. But we could observe the statistically significant difference only in the RNST(p<0.05). CONCLUSION: Tensilon test showed the highest positivity in all MG groups. So we would like to recommend the Tensilon test for the diagnosis of MG at first, followed by RNST and AchR Ab assay, and SFEMG would be indicated to MG group which showed relatively low postivity in other tests.

A case of primary antiphospholipid antibody syndrome showing vegetation on the mitral valve through echocardiography / 영남의대학술지

Antiphospholipid antibody syndrome(APS) is a well-known clinical syndrome characterized by recurrent arterial or venous thromboses, recurrent fetal loss, thrombocytopenia, together with high titers of sustained anticardiolipin antibody(aCL) or lupus anticoagulant(LA). Although systemic lupus erythematosus(SLE) and APS may coexist, a high proportion of patients manifesting the APS do not suffer from classical lupus or other connective tissue disease. The patient has been defined as having a primary antiphospholipid antibody syndrome. We experienced one case of primary APS with recurrent fetal loss, recurrent cerebral infarctions, positive anticardiolipin antibody IgG and fluttering vegetation on the mitral valve, without other connective tissue diseases including SLE. Forty-three old female had 2 out of 11 criteria for the diagnosis of SLE, such as thrombocytopenia and positive antinuclear antibody, but did not meet whole criteria. The patient was treated with ticlopidine, and anticoagulant therapy was recommended.

Localization of Ulnar Neuropathy at the Elbow by Short Segment Stimulation

BACKGROUND AND OBJECTIVES: Local compression of the ulnar nerve occurs most commonly at the elbow and optimal surgical intervention should be directed at the specific site of involvement. This study is designed to localize the more discrete region by using the method of short segment stimulation in ulnar neuropathy at the elbow. METHODS: Thirty seven patients who were diagnosed as entrapment ulnar neuropathy at the elbow by routine nerve conduction studies were investigated. Latency changes and amplitude changes including conduction block were determined by stimulating the ulnar nerve at 2cm intervals across the elbow. Six of these patients had orthopedic surgery after undergoing short segment stimulation studies. RESULT: All patients had significant latency changes(> OR =0.7msec) in specific segments by short segment stimulation and 6 patients of them showed conduction block. The most frequently involved segments were between medial epicondyle and 2cm proximal(20 patients) and between medial epicondyle and 2cm distal(9 patients). Only two patients exhibited significant latency changes between 2 and 4cm distal to the medial epicondyle, suggesting cubital tunnel syndrome. Lesions, as identified by surgery, proved to be accurately predicted by preoperative short segment stimulation in 5 of 6 patients. CONCLUSION: Short segment stimulation studies are helpful in localizing more accurate involved segment in ulnar neuropathy at the elbow. And the most commonly involved site is within 2cm of the medial epicondyle suggesting tardy ulnar nerve palsy.

Distribution of Weakness at the Lower Extremity of Hemiparesis Patients / 영남의대학술지

The maximal voluntary strength of knee extension and flexion on both the right and left sides was measured in patients with hemiparesis of upper motor neuron type and in a group of normal subjects. Significant differences of maximal voluntary strength were found between male and female but the ratio of flexor to extensor strength did not vary significantly between the sides, between the exs in normal subjects. The maximal voluntary strength of uninvolved side were not reduced significantly but involved side reduced significantly in patients. The ratio of flexor to extensor strength in hemiparetic side was significantly less than the ratio for the normal subjects but not significant difference in uninvolved side of patients. According to the above results, the maximal voluntary strength of flexion was more reduced than that of the extension on lower extremity of hemiparesis patients. The strength ratio of flexion to extension was a useful parameter for guiding the rehabilitation of hemiparesis.