Factors Affecting Caregiver Burden in Family Caregivers of Patients with Dementia

BACKGROUND: This study aimed to know the state of caregiver burden of family caregivers of patients with dementia and also to know factors associated with the severity of caregiver burden. METHODS: Family caregivers of dementia patients were enrolled from five general hospitals in Daegu Metropolitan city, Korea. The participants analyzed in the present study were 199 subjects who were 24 to 86 years old, living in Daegu or Kyungsang Bukdo province. Demographic features, general characteristics, characteristics of caring patients were collected and Burden Interview was done by a psychologist. Factors affecting caregiver burden were assessed using statistical analyses. RESULTS: The average severity of caregiver burden of family caregivers were between mild and moderate. Factors affecting the severity of caregiver burden were caregiving days per week among caregiver's characteristics and Neuropsychiatric Inventory and Geriatric Depression Scale among patient characteristics by multiple regression linear analysis. Caregiving cost shows marginal significance. Interestingly, cognitive scales like Mini-Mental State Examination, Clinical Dementia Rating, Physical and Instrumental Activities of Daily Living did not affect caregiver burden of family caregivers. CONCLUSIONS: This study showed that caregiving days per week of caregivers and neuropsychiatric symptoms including depressive mood of patients are closely related with caregiver burden of family caregivers of patients with dementia in Korea.

Comparison of Quality of Life in Patients With Epilepsy Taking Zonisamide or Topiramate: Before and After Treatment

BACKGROUND: Patients with epilepsy experience impairments in their quality of life (QOL). The objective of this study was to determine the influences of clinical factors on QOL and to compare QOL before and after antiepileptic drug (AED) treatment. METHODS: A cohort of 79 patients with epilepsy (43 male and 36 female) was recruited for this prospective study. The Quality of Life in Epilepsy (QOLIE)-31 survey was applied to evaluate QOL. The QOLIE-31 questionnaire was completed by the subjects before and 24 weeks after AED monotherapy (zonisamide or topiramate). The relationships of demographic, social, and clinical factors with QOL were evaluated. RESULTS: There was a negative correlation between seizure frequency and QOL in patients with epilepsy (p<0.05). The scores of patients without adverse effect were significantly higher for the seizure worry item of the QOLIE-31 questionnaire at 24 weeks compared to baseline (p<0.05). No other significant differences were found for any of the other QOLIE-31 items. CONCLUSIONS: Age, sex, seizure frequency, AED treatment, and AED adverse effects were significant clinical factors affecting QOL in patients with epilepsy. It is suggested that the physician should recognize these factors and manage them appropriately to improve the QOL of patients with epilepsy.

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

No abstract available.

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

No abstract available.

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.

Effects of Antiepileptic Drugs on Thyroid Hormones and Lipid Profiles / 대한간질학회지

PURPOSE: It is known that serum thyroid hormones and lipid profiles are affected by the different biotransformation pathways of antiepileptic drugs (AEDs). The aim of this study was to evaluate thyroid functions and lipids in epileptic patients taking AEDs. METHODS: We prospectively examined serum thyroid hormone concentrations and lipid profiles in 45 patients with epilepsy and compared them with 45 healthy age- and sex-matched controls. We measured serum free T4 (FT4), thyroid stimulating hormone (TSH), thyroid peroxidase antibody (TPO-ab), thyroid globulin antibody (TG-ab), total cholesterol, low density lipoprotein (LDL), high density lipoprotein (HDL) and triglycerides (TG). To analyze, the patient groups were divided into two groups:36 patients using hepatic enzyme inducing AEDs and 9 patients using non-enzyme-inducing AEDs. RESULTS: Mean age, sex and body mass index (BMI) were not different in both groups. The serum free T4 level of all patients was lower than that of the control group (p<0.05). The serum free T4 level of the patients taking hepatic enzyme inducing AEDs was lower than that of the control group. TSH, TPO-ab and TG-ab levels were not different in both groups. There was no correlation between free T4 levels and the duration of therapy in the patient group. For lipids, LDL, HDL, TG and total cholesterol, levels were not different in both groups. CONCLUSIONS: Hepatic enzyme inducing AEDs led to a decrease in free T4 levels but the TSH level remained normal. These findings seem to be not only due to liver enzyme induction but also hypothalamic interference of regulation of thyroid hormone production by the drugs. Lipid profiles were not significantly influenced by AEDs but further evaluation should be needed.

Ictal Vomiting Associated with Temporal Lobe Epilepsy of Dominant Hemisphere

Ictal vomiting is a rare clinical manifestation of temporal lobe epilepsy. This manifestation is considered a clinical lateralizing sign pointing toward seizure activity within the nondominant temporal lobe. However, we have experienced a case of ictal vomiting associated with temporal lobe epilepsy within the dominant hemisphere. The ictal vomiting occurred early in the course of the seizure when rhythmic discharges predominantly involved the left temporal lobe. This patient underwent a left anterior temporal lobectomy and experienced markedly reduced seizure frequency for more than 2 years.

Apolipoprotein E Polymorphism in Ischemic and Hemorrhagic Stroke / 대한해부학회지

There is an evidence for a role of apolipoprotein E (APOE) in atherosclerosis, coronary heart disease and carotid artery stenosis. Morbidity of carotid artery atherosclerosis is higher in persons carrying an epsilon4 allele, but the association of cerebrovascular disease and apoE genotype is controversial. We studied the association between APOE genotype and allele (epsilon2, epsilon3, epsilon4) frequency and stroke. We evaluated APOE genotype in 133 first -ever stroke patients and 111 healthy controls. We also estimated the risk factors of stroke such as hypertension, diabetes and lipid profiles in both groups. APOE genotypes were analyzed by polymerase chain reaction. APOE genotypes and allele distributions were not different in patients and controls. There is also no difference of APOE allele frequencies between ischemic small artery occlusive disease and hypertensive subcortical intracerebral hemorrhage. We concluded that the APOE -epsilon4 allele is not associated with stoke including ischemic and hemorrhagic stroke.

Clinical, Electrophysiological, and Genetic Analysis in a Family with Autosomal Dominant Nocturnal Frontal Lobe Epilepsy

BACKGROUND: Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is a distinct epilepsy syndrome and a genetically heterogeneous disorder linked to chromosomes 20q13.2, 15q24, and 1p21. Missense and insertion mutations in neuronal nicotine acetylcholine receptor 4 (CHRNA4) and 2 (CHRNB2) genes have been found in families with ADNFLE. METHODS: Clinical, EEG-Video monitoring, and neuropsychologic study in a family with ADNFLE were tested. For detect of mutation gene, polymerase chain reaction for CHRNA4 gene and CHRNB2 gene, single strand conformational polymorphism (SSCP) analysis and DNA sequencing were done. RESULTS: Among 15 living family members in three generations, nine had seizures. EEG-Video monitoring showed ictal epileptiform discharges genetically or regionally in frontal, frontocentral, frontotemporal, or temporal areas and less frequently no epileptiform discharges or non-specific generalized slowing. Two affected individuals demonstrated interictal temporal spikes, whereas the others were normal. Neuropsychological study showed mental retardation and decreased frontal executive function in five affected individuals. A cytosine to thymine exchange (755C>T; S252L) in exon 5 of the CHRNA4 gene was found on all affected individuals except in an individual who wasn 't tested, but this change was absent in those without epilepsy. CONCLUSIONS: This is the first study of genetically confirmed ADNFLE in a Korean family, who had mental retardation and various EEG abnormalities, ictally and interictally.

Effects of Deletions in the Regulatory Domain on the Stability and Enzymatic Characteristics of Tyrosine Hydroxylase

BACKGROUND: Various vectors have been developed and tried for the delivery of tyrosine hydroxylase (TH) in order to supplement dopamine, which is severely deficient in Parkinson's disease, however, none of the protocols tried have yielded fruitful results that can be applied directly to humans. One of the problems revealed from previous trials was a short duration of expression of the delivered gene, that is, tyrosine hydroxylase. METHODS: To extend the stability and to improve the enzymatic characteristics of the protein, part of the regulatory domain was deleted via PCR technique. The cDNA for regulatory domain-deleted THs (dTH) were sub-cloned into a retroviral vector and the resulting recom-binant retrovirus was used to infect NIH-3T3. After selection, expression levels of TH were determined by Western blot analysis and the enzymatic characteristics were examined. RESULTS: The deletion increased steady state expression level of TH protein by 7-fold for d19TH (TH with amino acids #2-19 are deleted) and 3-fold for d31TH (TH with amino acids #2-31 are deleted. The elevated expression level of d19TH is likely due to the enhanced stability of the protein as determined by a treatment of cycloheximide. The activity of d19TH was also increased approximately by 3-fold but no increase of the L-dopa production was observed. However, the production of L-dopa was dramatically increased when GTP cyclohydrolase I (GTPCH I) was co-transfected suggesting that the activity of d19TH is dependent on the presence of cofactor. d19TH seem to be free of feedback inhibition at low concentration of dopamine (10 nM~1 nM) but more sensitive to the inhibition at high concentration of dopamine (10 mM). CONCLUSIONS: The deletion of 18 amino acids on the regulatory domain increases the stability of the protein, reduces the activity, and frees it from the feedback inhibi-tion by the end product.

Effect of Ibuprofen on the Changes of Polyamine Level and Neuronal Cell Damage after Transient Global Ischemia in Gerbil

BACKGROUND: In brain ischemia, increased arachidonic acid metabolism can play important roles in neuronal dam-age. Ibuprofen was reported to have a protective role against neuronal damage in focal brain ischemia and reperfusion. The present study was designed to investigate whether ibuprofen can inhibit the global ischemia-induced neuronal dam-age and changes of polyamine (PA) level which is known to related to the neuronal damage, breakdown of blood brain barrier, and brain edema. METHODS: Male Mongolian gerbils were used in this study. Transient global ischemia was induced by occlusion of bilateral common carotid arteries for 3 min with microclips. Ibuprofen was administered imme-diately after ischemia. The animals were sacrificed one day after ischemia for PA measurement and sacrificed 5 days after ischemia for histological evaluation. Histological examination was performed by counting surviving neuronal cells in one mm of CA1 area in dorsal hippocampus. RESULTS: Cerebral cortex and hippocampal putrescine(PU) levels in vehicle-treated ischemic group significantly increased comparing to sham-operated animals and the increase of PU was attenuated by ibuprofen administration (50 mg/kg). Hippocampal spermine level decreased significantly after ischemia. Hippocampal neuronal cell damage in CA1 area was markedly observed in vehicle-treated animals compared to sham operated animals. Ibuprofen administration at the dose of 50 mg/kg significantly inhibited hippocampal CA1 neuronal damage compared to vehicle-treated animals. CONCLUSIONS: Ibuprofen attenuates PA response following transient glob-al ischemia and may have putative neuroprotective effect against neuronal damage induced by global ischemia.

Sudden Deafness and Anterior Inferior Cerebellar Artery Infarction

BACKGROUND AND OBJECTIVES : Acute ischemic stroke in the distribution of the anterior inferior cerebellar artery (AICA) is known to be associated with vertigo, nystagmus, facial weakness, and gait ataxia. There have been few reports on deafness associated with the AICA infarction. Furthermore, previous neurological reports have not emphasized the inner ear as a localization of sudden deafness. The aim of this study was to investigate the incidence of deafness associated with the AICA infarction and the sites predominantly involved in deafness. MATERIALS AND METHOD : During two years we prospectively identified 12 consecutive patients with unilateral AICA infarction diagnosed by brain MRI. Pure tone audiogram, speech discrimination testing, stapedial reflex testing, and auditory brainstem responses were performed to localize the site of lesion in the auditory pathways. Electronystagmography was also performed to evaluate the function of the vestibular system. RESULTS :The most common affected site on brain MRI was the middle cerebellar peduncle (11). Four patients had vertigo and/or acute auditory symptoms such as hearing loss or tinnitus as an isolated manifestation from 1day to 2 months prior to infarction. Audiological testings confirmed sensorineural hearing loss in 11 (92%) patients, predominantly involved the cochlea in 6 and retrocochlear in 1. The other 4 patients had a severe to profound hearing loss most likely of cochlear origin. Electronystagmography demonstrated no response to caloric stimulation in 10 (83%) patients. CONCLUSIONS : In our series, sudden deafness was an important sign for the diagnosis of the AICA infarction. Audiological examinations suggest that sudden deafness in AICA infarction is usually due to dysfunction of the cochlea, resulting from ischemia to the inner ear.

Three Cases of Anticonvulsant Hypersensitivity Syndrome Associated with Lamotrigine

Among the various side effects of anticonvulsant medication, the anticonvulsant hypersensitivity syndrome (AHS) is underrecognized. This condition developed frequently with aromatic anticonvulsants, but with new antiepileptic drugs as well. We experienced three lamotrigine-induced AHS cases with symptoms such as fever, rash, leukopenia, eosinophilia and lymphadenopathy, which subsided after withdrawal of lamotrigine.

Subdural Strip Electrode Studies in Temporal Lobe Epilepsy: Relation between Interhemispheric Propagation Time and Surgical Outcome

BACKGROUND: We evaluated whether the time required for a seizure to spread contralaterally, interhemispheric propagation time (IHSPT) could be related to post-surgical outcome in temporal lobectomy. METHODS: We performed a retrospective study of 28 patients. All patients had previously undergone a phase I presurgical evaluation including MRI and video-EEG monitoring with scalp and sphenoidal electrodes, which strongly suggested the diagnosis of mesial temporal lobe epilepsy, but proved inadequate to lateralize the epileptogenic zone. All patients performed the video-EEG monitoring with bilateral subdural strip electrodes on their basal and mesial temporal area and unilateral temporal lobectomy with a minimum of 1-year follow up postoperatively. IHSPT was divided into two categories, 0~5.0 seconds, 5.1 seconds or longer. RESULTS: Sixteen patients (57.1%) were seizure free and 12 suffered persistent seizures. A prolonged IHSPT (> 5.0sec) significantly correlated with a favorable surgical outcome (P = 0.05). Hippocampal atrophy on MRI significantly correlated with prolonged IHSPT (P<0.05). CONCLUSIONS: Our result suggests that hippocampal atrophy on MRI and IHSPT can predict the surgical outcome and may be used as the selection criteria of temporal lobectomy for patients with intractable temporal lobe epilepsy.

A Case of Recurrent Bickerstaff's Brainstem Encephalitis with an Overlap of Guillain-Barre Syndrome

Bickerstaff 's brainstem encephalitis (BBE) is characterized by acute ophthalmoplegia and ataxia. Some patients with BBE show mental changes, pyramidal signs and sensory disturbance. A forty-three year old woman was diagnosed as BBE in 1996. In 2000, she admitted again with complaints of ataxia and ophthalmoplegia. She was diagnosed as recurrent BBE and an overlap of Guillain-Barre syndrome (GBS). This is the first case of an overlap of BBE and GBS, as well as BBE in Korea.