Surgical Treatment in Cervical Myelopathy Combined with Flail Arm Syndrome / 계명의대학술지

Flail arm syndrome (FAS) is a variant of the amyotrophic lateral sclerosis also known as Lou Gehrig's disease. FAS is a kind of motor neuron disease that represents a bilateral proximal muscle wasting of upper extremities. Degenerative cervical spondylosis is a common cause of cervical myelopathy and radiculopathy. The coexistence of cervical spondylosis and motor neuron disease can cause difficulties in diagnosis and treatment. This case is a cervical spondylotic myelopathy associated with FAS who had undergone surgical treatment. After the operation, subjective symptoms of the patient was more aggravated and it may be owing to natural history of FAS. The surgical treatment must be made very carefully in cervical spondylotic myelopathy patient combined with motor neuron disease.

Prevalence of Anti-Ganglioside Antibodies and Their Clinical Correlates with Guillain-Barre Syndrome in Korea: A Nationwide Multicenter Study

BACKGROUND AND PURPOSE: No previous studies have investigated the relationship between various anti-ganglioside antibodies and the clinical characteristics of Guillain-Barre syndrome (GBS) in Korea. The aim of this study was to determine the prevalence and types of anti-ganglioside antibodies in Korean GBS patients, and to identify their clinical significance. METHODS: Serum was collected from patients during the acute phase of GBS at 20 university-based hospitals in Korea. The clinical and laboratory findings were reviewed and compared with the detected types of anti-ganglioside antibody. RESULTS: Among 119 patients, 60 were positive for immunoglobulin G (IgG) or immunoglobulin M antibodies against any type of ganglioside (50%). The most frequent type was IgG anti-GM1 antibody (47%), followed by IgG anti-GT1a (38%), IgG anti-GD1a (25%), and IgG anti-GQ1b (8%) antibodies. Anti-GM1-antibody positivity was strongly correlated with the presence of preceding gastrointestinal infection, absence of sensory symptoms or signs, and absence of cranial nerve involvement. Patients with anti-GD1a antibody were younger, predominantly male, and had more facial nerve involvement than the antibody-negative group. Anti-GT1a-antibody positivity was more frequently associated with bulbar weakness and was highly associated with ophthalmoplegia when coupled with the coexisting anti-GQ1b antibody. Despite the presence of clinical features of acute motor axonal neuropathy (AMAN), 68% of anti-GM1- or anti-GD1a-antibody-positive cases of GBS were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) by a single electrophysiological study. CONCLUSIONS: Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was correlated strongly with the various clinical manifestations. Nevertheless, without an anti-ganglioside antibody assay, in Korea AMAN is frequently misdiagnosed as AIDP by single electrophysiological studies.

Japanese-B Viral Encephalitis with a Biphasic Illness Pattern and Recovery after Intravenous Immunoglobulin Therapy / 대한임상신경생리학회지

Japanese-B viral encephalitis (JE) usually has a monophasic illness pattern. A 45-year-old woman in an altered mentality had improved over 1 month. About 1 week after the initial improvement, the patient became comatose with aggravated EEG and MRI findings. Assays of cerebrospinal fluid and serum were positive for the IgM antibody to Japanese-B virus. After intravenous immunoglobulin (IVIG) infusion, the patient recovered. We report a patient with JE who showed a biphasic illness pattern and recovered after IVIG therapy.

Erratum: Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

Development of Korean Neuropathic Pain Questionnaire for Neuropathic Pain Screening and Grading: A Pilot Study

BACKGROUND: The pain-screening questionnaire is a self-reported description of the intensity and nature of pain. This study aimed to develop the Korean Neuropathic Pain Questionnaire (KNPQ) and to assess its reliability and validity regarding the diagnosis of neuropathic pain. METHODS: Four screening tools and two rating scales were translated and modified to develop the preliminary KNPQ. Following a development phase and a pilot study, we generated the final 25-item version of the KNPQ. Each item was rated on a numerical scale of 0-10. The validation procedure was performed in 62 patients with neuropathic pain (21 with central pain and 41 with peripheral pain) and in 34 patients with nonneuropathic pain. The internal consistency between items was assessed to determine the reliability of the KNPQ, and its concurrent validity was determined by evaluating the relationship between the Visual Analogue Scale (VAS) and KNPQ scores. RESULTS: The KNPQ was not influenced by age, sex, or pain duration. The 25-item questionnaire demonstrated high internal consistency. The total score of the KNPQ was correlated with the global pain intensity on a VAS. These items were able to differentiate neuropathic pain from nonneuropathic pain with a sensitivity of 84% and a specificity of 44% (when using a cut-off point of 46). CONCLUSIONS: The newly developed KNPQ may be used for the initial screening of neuropathic pain patients. However, it cannot be used to differentiate central neuropathic pain from peripheral neuropathic pain.

Comparison between Clinical Disabilities and Electrophysiological Values in Charcot-Marie-Tooth 1A Patients with PMP22 Duplication

BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.

Clinical Features, Disability and Socioeconomic Status of Patients With Muscular Dystrophy in Korea

BACKGROUND: Since 2001, the Ministry of Health and Welfare in Korea has designated muscular dystrophy (MD) to be a rare and intractable disease, and has ensured that patients with this condition obtain support from the National Health Insurance Corporation for their medical expenditure. However, the health-related and socioeconomic status of Korean patients with MD has yet to be established. METHODS: We selected 441 patients with MD who received medical services at 17 neuromuscular centers during 2005. The medical records of selected patients were analyzed, and the subtype of MD was classified by its clinical course and diagnostic tests. A total of 95 patients or their family members participated in this health-related and socioeconomic status survey. RESULTS: Medical record analysis showed similar clinical and diagnostic characteristic data to those published previously in other countries: male predominance, being young at onset, and muscular weakness of the extremities as a predominant symptom in most patients. The diagnostic tests for MD were based on laboratory and electrophysiological studies. The most frequent form of MD among our cohort was Duchenne/Becker muscular dystrophy (42%). Our survey revealed the effect of the patients' profound disability on their activities of daily living. One-half of the patients were dissatisfied with the medical expenditure support service that was made available to them, and most patients suffered from a financial burden. The most important medical services to be developed in the future are expansion of the public health service or development of a rehabilitation hospital. CONCLUSIONS: This is the first multicenter-based epidemiologic study on the health-related and socioeconomic status of patients with MD in Korea. The findings indicate that medical coverage and public health service are currently inadequate and hence should be expanded in the future.

Ultrasonographic Findings of Mild and Very Mild Carpal Tunnel Syndrome

OBJECTIVE: To assess the clinical usefulness of ultrasonography for the diagnosis of mild and very mild carpal tunnel syndrome. METHOD: Ultrasonographic evaluation was performed in 39 hands of 29 patients with mild and very mild carpal tunnel syndrome according to Bland's neurophysiologic grading scale. Controls included 41 hands of 34 subjects without electrophysiologic evidence of carpal tunnel syndrome. Ultrasonographic findings were evaluated quantitatively with regard to the cross-sectional area, the flattening ratio, the swelling ratio of the median nerve, and palmar displacement of the flexor retinaculum. The analysis of differences between controls and patients group were done by independent t-test. Cut-off values using receiver operation characteristic, were calculated. RESULTS: Patients had significantly increased cross sectional area and flattening ratio of the median nerve at proximal carpal area, palmar displacement of the flexor retinaculum at proximal carpal area. A critical value of equal or higher to 8.5 mm(2) for cross-sectional area at the pisiform level showed a sensitivity 79.5%, a specificity of 95.1%, which had most diagnostic value compared with the value of cross-sectional area at the distal radius level, flattening ratio at the pisiform level and palmar displacement. CONCLUSION: Ultrasonography was useful in the diagnosis of mild and very mild carpal tunnel syndrome.

A Case of Acute Internal and External Ophthalmoplegia without Ataxia and Areflexia Associated with Anti-GQ1b Antibody

No abstract available.

Clinical and Genetic Characteristics in Patients of Charcot-Marie-Tooth type 2A with Mitofusin 2 (MFN2) Mutations

BACKGROUND: Mitofusin 2 (MFN2) is a membrane protein and is an essential component of mitochondrial fusion machinery. Mitochondrial fusion is essential for various biological functions in mammalian cells. Thus mutations in MFN2 are the underlying cause of Charcot-Marie-Tooth neuropathy type 2A (CMT2A). However, there has been no reports investigating the MFN2 genes in Korean CMT patients. Therefore, we investigated to find the clinical and genetic characteristics in Korean patients with the MFN2 gene mutation. METHODS: We examined the mutations of the MFN2 gene in 137 Korean CMT families. According to criteria from the European CMT consortium, CMT2 was 45 families. Mutations were confirmed by both strands sequencing. Nerve conduction studies were carried out in CMT patients having each mutation. RESULTS: Eight pathogenic mutations were found in 10 families. Six mutations (Leu92Pro, Gly127Asp, His165Arg, Ser263Pro, Arg364Trp, Met376Thr) were determined to be novel, and those were not detected in the 100 healthy controls. A de novo missense mutation was found in three CMT families (30%). The frequency of the MFN2 mutation was 22.2%, which was higher than those found in the Cx32 mutation. In CMT2A, the frequencies with early age at onset (<10 years) and flat feet were 46.2%. CONCLUSIONS: We found MFN2 mutations in patients with sporadic or dominantly inherited CMT. In the majority of cases with CMT type 2, the axonal neuropathy, may be due to MFN2 mutations.

A Case of Diabetic Uremic Encephalopathy with Symmetric MR signal Changes in the Basal Ganglia

Acute changes of the bilateral basal ganglia shown in the brain MRI a diabetic uremic patient have been rarely reported. We report a 52-year-old diabetic uremic patient who had neurological disturbances. At admission, axial diffusion-weighted images and T2-weighted images showed high signal intensities in the bilateral basal ganglia, and axial T1-weighted images visualized low signal intensities in same area. The underlying mechanism may be associated with metabolic as well as vascular factors.

Non-convulsive Complex Partial Status Epilepticus as a Manifestation of Non-Ketotic Hyperglycemia

Status complex partial seizure caused by non-ketotic hyperglycemia (NKH) has been known to be rare. A 42-year-old diabetic man was admitted because of abnormal behavior. Video-EEG monitoring revealed an ictal EEG correlate of this behavior. Brain MRI showed an abnormal signal intensity in the right temporal cortex. Seizures were controlled by lowering glucose levels and a follow-up MRI showed resolution of the abnormal signal. We report a case of non-convulsive status epilepticus as a complex partial seizure type by NKH.

A Case of ReVersible MRI and SPECT Abnormalities in the Neocortex of the Temporal and Parietal Lobes after a Generalized Seizure

We report a case with reVersible temporal and parietal neocortical abnormalities detected by MRI and SPECT following a brief seizure. Post ictal MRI abnormalities may indicate an underlying structural abnormality, but may also occur in non-lesional epilepsy and represent a transient physiologic change induced by ictal activity.

Two Cases of Transient Focal Lesion in the Splenium of the Corpus Callosum after Aggravated Seizures

Transient focal lesions in the splenium of the corpus callosum have been rarely reported in epileptic patients receiving antiepileptic drugs. We examined two patients with epilepsy whose epileptic seizure frequency has drastically increased. The brain MRI findings of these patients showed a transient focal lesion in the splenium of the corpus callosum. Follow up MRI taken 15 days and 6 days later after controlling their seizures demonstrated complete resolution of the lesion, without specific treatment for the lesion.

A Case of Viral Encephalitis as a Presenting Symptom of Histiocytic Necrotizing Lymphadenitis: Kikuchi's Disease

No abstract available.

The Influence of Education on Knowledge of Their Disorder and Quality of Life in Patients with Epilepsy / 대한간질학회지

PURPOSE: The education of the patients is an effective component of comprehensive care, especially in patients with epilepsy. We developed an epilepsy educational program designed to improve the knowledge and understanding of the natural clinical course, the treatment, and prognosis of epilepsy. We evaluated the efficacy of this educational program to epilepsy patient's knowledge of their disorder and quality of life. METHODS: We studied 224 patients with epilepsy from Dongsan Epilepsy Center. All patients completed the Knowledge and Quality of Life in Epilepsy-31 (QOLIE-31) questionnaire before a one-day educational program was completed. Six months after the educational program was given, patients completed the same questionnaire. The results of the two surveys were compared. RESULTS: In the initial questionnaire, about 60% of the patients thought that epilepsy is not related with head trauma. About 40% of the patients thought that epilepsy is a genetic disorder. Over 80% of the patients thought that behavior changes are not related to epilepsy. Over 90% of the patients had correct responses about taking antiepileptic drugs. However, only 60% of the patients had their ideas regarding proper social activity correct. After completing the educational course, the results of the follow-up questionnaires were markedly improved concerning the patients' knowledge of epilepsy. In contrast, the quality of life measures changed a little. CONCLUSION: An epilepsy educational program for patients improves their knowledge of epilepsy. This study was performed with only one education workshop, which is not sufficient to improve the quality of life measures. A well-regulated, systematic educational program is needed to efficiently improve the lives of epileptic patients.

Spinal Cord Infarction with Anterior Chest Pain

We report a man who presented anterior chest pain with mild weakness of both hands while swimming. Initially, he was admitted to the department of internal medicine for evaluation and management of suspected myocardial infarction. Cardiac evaluations including coronary angiogram showed the patient to be normal. Spinal MRI showed a hyperintense signal on the sagittal T2-weighted images at the cervical spinal cord (C6-7). When a patient with chest pain shows weakness in both arms, not only cardiac evaluation but also spine work up is needed.

Effect of Antiepileptic Drugs on Bone Mineral Density and Bone Metabolism in Epileptic Patients

BACKGROUND: Bone mineral density (BMD) is influenced by genetic, hormonal, and environmental factors. Long-term antiepileptic drug (AED) use also causes osteopenia or osteoporosis that have been most extensively described in institutionalized patients. But, the mechanism of these abnormalities is unclear. The objective of this study is to determine the effect of AED on bone density and to explain the pathophysiologic mechanisms by analyzing bone related factors. METHODS: We prospectively examined BMD by dual-energy X-ray absorptiometry in 45 patients with epilepsy. We measured the serum calcium, phosphorus, protein, alkaline phosphatase (ALP), bone specific ALP, vitamin D and osteocalcin to analyze the factors that influence bone metabolism. RESULTS: BMD was significantly lower in the patient group than in the control group (p<0.05). 13% of patients had osteopenia and 3% of patients had osteoporosis. The level of bone specific ALP was higher in the patient group, but the level of vitamin D was not different, implying that BMD is decreased by the direct effect of antiepileptic drugs. There was a weak negative correlation and marginal significance between BMD and the duration of therapy in the patient group (r=-0.407, p<0.05). CONCLUSIONS: Long-term antiepileptic drug therapy in patients who have seizures causes significant bone loss in the lumbar spine even in the absence of vitamin D deficiency. In addition, the degree of bone mineral density was weakly related with the therapeutic duration of antiepileptic drugs. The regular evaluation of BMD in patients with long-term antiepileptic drugs might be helpful to prevent decreases in BMD.

A Familial Outbreak of Food-borne Botulism

We experienced 3 cases of food-borne botulism within a family. They presented with progressive ptosis, dysphonia, dysarthria and limb weakness, which had started about 12 hours after ingestion of vacuum-packed sausage. Two cases progressed to respiratory failure. Botulinum toxin A was detected from the stool sample of one case. Conservative treatment with mechanical ventilation was done and they recovered from respiratory failure over a 30-70 days period. This is the first case report of food-borne botulism in Korea.

A Case of Viral Encephalitis with Disproportionate Retrograde Amnesia

A 44-year-old man showed recurrent paroxysmal amnesic attacks following viral encephalitis which, despite antiepileptic treatment, developed into status epilepticus. Interestingly, the amnesic attacks mainly consisted of amnesia for retrograde events. After recovery from status, he showed a persistent amnesia which was characterized as disproportionate retrograde amnesia for the past 20 years. We attribute the amnesic attacks in the acute stage to a transient epileptic amnesia and the profound retrograde amnesia in the chronic stage to status- or infection-related focal brain damage.