Long-Term Care Hospital and the Role of Neurologist

Korea health care system and public welfare system have been changed slowly and developed step by step. It makes efforts to promote the welfare of the people, but does not provide good benefits for geriatric patients. In 2008, it was incompletely set up the long term care system in Korea. And the Law on Long-Term Care Insurance for Elderly was officially announced in Nov. 2011 and the law for dementia, Dementia Management Act, was drafted in Feb. 2012. Though there were many laws, the concept of long-term care hospital and geriatric care facility is still obscure. In long-term care hospital, there are big expansion of the number of beds and institutions in Korea. Over a half of the patients in long-term care hospitals are suffered from mixed neurological diseases. Especially, dementia is the comorbid state with other chronic geriatric diseases. In view of the aging population, the role of neurologist will continue to grow. So neurologists need more special training for the needed skills and competencies, and the specific care settings program in long-term care hospital. In this review, the history and current state of long-term care hospital, related laws, and the role of neurologist will be discussed.

Endothelin 1 protects HN33 cells from serum deprivation-induced neuronal apoptosis through Ca2+-PKCalpha-ERK pathway

Endothelins (ETs), which were originally found to be potent vasoactive transmitters, were known to be implicated in nervous system, but the mode of mechanism remains unclear. ETs (ET-1, ET-2, and ET-3) were added to HN33 (mouse hippocampal neuron chi neuroblastoma) cells. Among the three types of ET, only ET-1 increased the intracellular calcium levels in a PLC dependent manner with the induction of ERK 1/2 activation. As the result of ET-1 exposure, the survival rate of HN33 cells and the PKCalpha translocation into the plasma membrane were increased. We suggest that ET-1 participated in the neuroprotective effect involving the calcium-PKCalpha-ERK1/2 pathway.

Hepatic Myelopathy Involving the Posterior Column

Hepatic myelopathy is a rare neurological complication of chronic liver disease, and is characterized by a progressing spastic paraparesis without any sensory loss. However, a few recent reports suggest that involvement of the sensory system is also possible in hepatic myelopathy. We present a patient with hepatic myelopathy, who had impaired proprioception and a delayed cortical response of somatosensory evoked potentials. This supports the hypothesis that hepatic myelopathy may involve the sensory system as well as the motor system.

A Case of Meningoencephalitis Associated with Relapsing Polychondritis

Relapsing polychondritis (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. We present a patient diagnosed with meningoencephalitis associated with RP whose initial symptoms included chronic headache with memory disturbance and disorientation. After treatment of meningoencephalitis, the patient had frequent bilateral auricular swelling, polyarthralgia, and sensory neural hearing loss. Although RP is an extremely rare disease, it should be considered as a possible cause of chronic meningoencephalitis.

Bitemporal Hemianopia Associated with Anti-Tuberculosis Medication

No abstract available.

Craniocervical Segmental Dystonia in the Spinocerebellar Ataxia Type 2

The spinocerebellar ataxia type 2 (SCA 2) is an autosomal dominant cerebellar ataxia that commonly presents with cerebellar ataxia, hyporeflexia, and slow saccades. Recent clinical series described movement disorder in the SCA 2 such as Parkinsonism or dystonia. Dystonia can be observed in and even be the presenting feature of the SCA 2. We report two patients with genetically confirmed SCA 2 displaying a slowly progressive syndrome combined with cerebellar ataxia and craniocervical segmental dystonia.

A Case of Dopamine Dysregulation Syndrome with Concurrent Parkinsonism Hyperpyrexia Syndrome

A few patients with Parkinson's disease (PD) crave large amounts of levodopa therapy far beyond those needed to alleviate their symptoms, even in the state of overdose complication. Such harmful pattern of compulsive dopaminergic drug use is called as dopamine dysregulation syndrome (DDS). We report a chronic stage PD patient presenting with high fever and altered mentality, who had the DDS as well as the Parkinsonism hyperpyrexia syndrome (PHS).

The Analysis of Treatment with Acyclovir and Prognostic Factors on Idiopathic Facial Mononeuropathy Using Sunnybrook Scale

BACKGROUND: Based on a recent study showing that the reactivation of HSV-1 may be one of the possible pathogenesis of idiopathic facial mononeuropathy, antiviral therapy has been suggested as a good treatment modality. However, the efficacy of antiviral therapy is still controversial. This argument may be caused by the absence of a reliable system of grading facial nerve paralysis. We performed this study to investigate the therapeutic effects of an antiviral agent and possible prognostic factors using the Sunnybrook scale. METHODS: Patients with idiopathic facial mononeuropathy were divided as two groups according to the treatment regimens (Acyclovir & Steroid vs. Steroid only). Clinical efficacy was evaluated using the House-Brackmann grading system and Sunnybrook scale. Electrodiagnostic studies including the blink reflex, facial nerve conduction study, and facial nerve excitability test were performed. The clinical improvement rate between the two groups and the usefulness of the electrodiagnostic studies as prognostic factors were analyzed. RESULTS: No significant differences of the clinical improvement rate was revealed with a 70% improvement rate for the Acyclovir treatment group and 84% for the steroid only group. Among electrodiagnostic studies, the compound muscle gamma action potential (CMAP) amplitude (=0.637, p=0.000) and nerve excitability =0.688, gamma test (p=0.000) on 2 weeks showed significant correlations to the final Sunnybrook score. There is good correlation between the two facial nerve grading systems. CONCLUSIONS: Acyclovir may not be effective in the recovery of idiopathic facial mononeuropathy. CMAP amplitude and nerve excitability tests on 2 weeks may provide valuable prognostic factors. The Sunnybrook scale may be useful as a facial grading system.

Dynamic Foot Pressure Measurement in Parkinson's Disease with Foot Scan System

BACKGROUND: Foot Scan System (RS scan international cooperation) measuring the plantar pressure distribution is simple and inexpensive tool for gait analysis. The analysis of plantar pressure distribution of the feet during gait maybe helps the assessment of motor symptoms or gait stability of Idiopathic Parkinson's disease (IPD). METHODS: We compared the parameters of foot scan system between 34 normal controls and 23 IPD patients before and after levodopa administration. Data of plantar force distribution and time of stance phase were collected using pressure-sensitive insoles as parts of the Foot Scan System. RESULTS:There were significant difference between normal controls and IPD patients in fore foot peak pressure/rear foot peak pressure ratio, time percentage of heel strike phase, mid stance phase, propulsion phase in stance phase. But in these parameters there were no significant difference between IPD patients before dopamine with after dopamine medication. CONCLUSIONS: Using the Foot Scan System (RS scan international cooperation) measuring the plantar pressure distribution, we showed that F/R ratio and time percentage of propulsion phase are reduced significantly in patients with IPD, which enables us to understand the features of gait in IPD patients more easily.

Chronic Inflammatory Demyelinating Polyneuropathy Developed during Interferon-beta Therapy in a Patient with Multiple Sclerosis

Preliminary studies have evaluated the effects of interferon beta formulations in the treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) because of pathogenic similarities between CIDP and multiple sclerosis (MS). However, the efficacy of Interferon, which has been widely used for relapsing-remitting MS, is controversial in CIDP. We report here a 31year old woman with relapsing-remitting type MS treated with IFN beta-1b over 2 years who developed overt CIDP. She responded favorably to steroids. This case suggests that IFN beta-1b treatment may not prevent development of CIDP.

Analysis of 10,811 Cases with Acute Ischemic Stroke from Korean Stroke Registry: Hospital-Based Multicenter Prospective Registration Study

BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.

Treatment with Repeated Lumbar Epidural Blood Patches for a Patient with Orthostatic Headache after Suboccpital Craniectomy

Orthostatic headache after suboccipital craniectomy has been rarely reported. A 32-year-old man underwent emergent suboccipital craniectomy due to acute bilateral cerebellar infarctions with massive brain edema. Although his neurological deterioration halted after the craniectomy, he still suffered from a severe disabling orthostatic headache. We performed two epidural blood patches at the levels of the lumbar and thoracic spine. He improved considerably a few days after the repetitive epidural blood patches. Epidural blood patches may be a useful treatment modality for post-craniectomy postural headaches.

Jerky Seesaw Nystagmus in Isolated Internuclear Ophthalmoplegia

The authors report jerky seesaw nystagmus, extorsional downbeating in the right eye and intorsional upbeating in the left eye, in a patient with right internuclear ophthalmoplegia (INO). This pattern of nystagmus may occur by disrupting pathways from contralateral posterior and anterior semicircular canals by a lesion in the medial longitudinal fasciculus. Depending on damage to the pathways from contralateral vertical canals, various patterns of dissociated torsional-vertical nystagmus may accompany INO.

Analysis of Dynamics of Gait in Parkinson's Disease with 3-Demensional Gait Analysis System

BACKGROUND: Gait disturbance is one of the most disabling symptoms of Parkinson's disease (PD). To better understand of the dynamics of gait in PD, we quantitatively evaluated the spatiotemporal and kinematic parameters of gait in PD patients and normal controls with 3-demensional gait analysis system. METHODS: The diagnosis of PD was based on UK Brain Bank criteria. Subjects with PD were recruited from Parkinson disease clinic of Korea University Medical Center. The severity was indicated by Hoehn and Yahr stage and Unified Parkinson disease rating scale (UPDRS). The gait analysis and assessment was performed in the off condition. RESULTS: Thirty PD patients and thirty normal controls were included. The statistical significant results of gait analysis between PD and control groups were as followings; reduced walking velocity, reduced stride length, increased double supporting time, reduced pelvic oblique movement with preserved pelvic tilting, reduced maximal flexion and extension of hip joint, reduced flexion angle of knee joint especially in terminal stance phase and swing phase, and reduced plantar flexion of ankle. But there were no significant difference between PD and control groups in cadence. And these parameters except cadence had significant correlation with clinical disability in PD patients. The cadence did not correlated significantly with clinical disability. CONCLUSIONS: The PD patients showed statistically gait cycle change with preserved cadence, reduced range of motion in hip, knee, and ankle joint with preserved ankle dorsiflexion in the profiles. These features were aggravated in advanced stage.

Gender Difference on Corpus Callosum in Korean Adults / 체질인류학회지

Corpus callsoum (CC) is the largest commissural fiber connecting the cerebral hemispheres. The gender difference in the size or the shape of CC is a long standing dispute. Some reported that adult female CC had more bulbous splenium and larger area considering brain size, but others failed to replicate this findings. There is no definite consensus on sexual dimorphism of CC yet, although extensive studies on sexual dimorphism has been expected to provide a clue to explain sociopsychological differences between male and female. This variable results are attributed to limited number of subjects, measured parameters, and method of measurement. We have employed comprehensive analytic parameters with large subjects to understand gender differences on CC of healthy Korean adults. We have analyzed the magnetic resonance image (MRI) in adults free from neurological disorders. The subjects were composed of 108 young people (3rd decade; male : 51, female : 57). Total area, its 5 sub-areas, linear parameters including height, length, and width and five specific angles of the CC were measured on the midsagittal MR images with NIH Image program (R) (Ver 1.6). The gender differences were observed in the area of splenium and length in the group. The male CC have larger splenium and longer length than female. The angle between neural axis and base of corpus callosum in female was significantly larger than that of male. This study reports not only gender difference of adult CC, but Korean adult standard morphometric data of CC research. These results might serve a useful basic data for various research in the fields of neuroanatomy, neuroradiology, and neuropsychiatry.

A Case of Severe Obesity-Hypoventilation Syndrome Treated by Bilevel Positive Airway Pressure Therapy

Obesity-Hypoventilation syndrome (OHS) is characterized by morbid obesity, hypoxia, and hypercapnea during wakefulness without parechymal lung disease or severe obstructive sleep apnea. A woman was admitted because of mental deterioration and diagnosed as OHS on the basis of obesity and hypoventilation, while awake, after ruling out other causes. By bilevel positive airway pressure (BiPAP) therapy, hypercapnea and hypoxia were resolved. We report that BiPAP can be an effective treatment for severe hypercapnea and hypoxia in OHS, which obviate the need for invasive endotracheal intubation.

Stroke Prevention

No abstract available.

New Approach in the Treatment of Epilepsy: Efficacy of New Antiepileptic Drugs

To consider current concepts of epilepsy further, the brief review begins with a discussion of what is epilepsy, discribes multifactorial nature of epileptic disorders, and ends with a presentation of current classifications. A combination of the standard antiepielptic drugs(AEDs) may be necessary to treat intractable seizures, but no studies have been done to indicate an optimal combination. The new AEDs provide alternative choices, but questions remain about the optimal timing and manner of administration. AEDs selection must individualized, no drug of choice can be named for all patients.

Central Pontine and EXtrapontine Myelinolysis

The knowledge about nutritional, toxic, and metabolic causes of dementia is particularly important, because they may be reversible. Central pontine myelinolysis(CPM) is one of these causes. CPM is a well known but rare metabolic disease of unknown etiology linked to overly aggressive correction of hyponatremia. We report a 74-year-old woman who developed disorientation, memory disturbance, and behavioral problem following intensive care unit management for pneumonia. Mini-mental status examination-Korean version(MMSE-K) study revealed severe cognitive dysfunction. Brain magnetic resonance imaging showed changes consistent with CPM and extrapontine myelinolysis. After supportive care, patient's clinical status was significantly improved. We suggest that a metabolic problem such as CPM should be considered in the diagnosis of acute or subacute cognitive deterioration in elderly patients.

The Effect of Unilateral Dopaminergic Deafferentation on Kainic acid Induced Seizures in the Rat

There has been quite a number of research regarding the role of dopamine in epilepsy and it has been still very controversial, In this study, the effect of dopaminergic deafferentation on kainic acid induced seizures was evaluated with behavioral study and molecular biologic method. We produced unilateral dopaminergic deafferentation via injection of 6-hydroxydopamine at the location of right substantia nigra in the Spague- Dawley rats (250-300gm) using the stereotaxic technique under pentobarbital anesthesia. Four weeks after this procedure, kainic acid (10m9/kg) was injected into the peritoneal cavity for induction of seizures. Observations of seizure pattern and mRNA expression of c-fos, dynorphin and enkephalin were obtained in the lesion group and were compared with those in the non-lesion group in terms of behavior characteristics and in situ hybridization histochemistry. In behavior study, the results demonstrated that more severe seizure patterns (rearing, falling, tonic-clonic seizure and status epilepticus) and more fast seizure evolution time(from onset to stage V) in the lesion group than those in the non-lesion group, which may indicated statistically significant (16.21+/-12.84 min. vs 35.88+/-16.55 min., p(0.05) The results of in situ hybridization revealed that the expression of c-fos, dynorphin and enkephalin mRNAs in certain areas of brain was higher in the lesion group than that in non-lesion group; c-fos in cerebral cortex, dynorphin in dentate gyrus, enkephalin in the entorhinal cortex and amygdala nuclei. Although the lesion was unilateral, the mRNA expression patterns appeared symmetrical shape. The characteristic behavior and molecular biologic study results are suggested that the dopamine may plays a important role in control of kainic acid induced seizures.