Clinical, Neuroimaging and Neurophysiologic Evidences of Restless Legs Syndrome as a Disorder of Central Nervous System

Restless legs syndrome (RLS) is a sensorimotor neurological disorder in which the primary symptom is a compelling urge to move the legs, accompanied by unpleasant and disturbing sensations in the legs. Although pathophysiologic mechanism of RLS is still unclear, several evidences suggest that RLS is related to dysfunction in central nervous system involving brain and spinal cord. L-DOPA, as the precursor of dopamine, as well as dopamine agonists, plays an essential role in the treatment of RLS leading to the assumption of a key role of dopamine function in the pathophysiology of RLS. Patients with RLS have lower levels of dopamine in the substantia nigra and respond to iron administration. Iron, as a cofactor in dopamine production, plays a central role in the etiology of RLS. Functional neuroimaging studies using PET and SPECT support a central striatal D2 receptor abnormality in the pathophysiology of RLS. Functional MRI suggested a central generator of periodic limb movements during sleep (PLMs) in RLS. However, to date, we have no direct evidence of pathogenic mechanisms of RLS.OAIID:oai:osos.snu.ac.kr:snu2008-01/104/2014017262/3SEQ:3PERF_CD:SNU2008-01EVAL_ITEM_CD:104USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0FILENAME:rls is a disease of cns.pdfDEPT_NM:의학과CONFIRM:

A Case with Sleep Related Eating Disorder

Sleep-related eating disorders (SRED) comprise a spectrum of parasomnia and eating disorders, in which nocturnal arousals from sleep are followed by rapid and compulsory. Here we report a patient who showed unexpected and bizarre eating behavior with somnambulistic mental state during polysomnography.수면관련섭식장애(Sleep related eating disorders, SRED) 는 수면을 하던 도중 잠에서 깨어나 불수의적이거나 통제할 수 없는 방식으로 음식을 섭취하거나 음료수를 마시는 삽화가 반복하여 발생하는 질환이다. 삽화 도중 환자들은 대부분 각성 상태가 약간 저하되어 있거나 드문 경우 완전이 깨어 있는 경우도 있지만 대개 아침에 각성하였을 때 이러한 삽화를 기억하지 못한다.1,2,4,7 이러한 수면장애는 주로 20대에서 호발하며 몽유병 등 다른 사건 수면의 병력이나 만성질환을 가지고 있는 여자들에서 흔한 것으로 보고되고 있다. 진단에 수면다원검사가 필수요소는 아니지만 약 80%의 환자가 수면다원검사를 통해 진단된다. 저자들은 임상적으로 SRED가 의심이 되는 환자에서 수면다원검사를 통해 삽화가 기록된 1예를 경험하였기에 문헌 고찰과 함께 보고한다.OAIID:oai:osos.snu.ac.kr:snu2008-01/102/2014017262/9SEQ:9PERF_CD:SNU2008-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Aphasic Status Epilepticus：Two Cases

Background & Significance：Aphasic status epilepticus is an uncommon presentation of a simple partial status epilepticus characterized by prolonged speech impairment with clinical-EEG correlation in the absence of an altered level of consciousness. There has been no published report in Korea. This study reports two adult patients with prolonged aphasia and EEG abnormalities. Cases：Total three episodes of aphasic status epilepticus were documented in two patients. Patients were right-handed 58-year-old and 79-year-old women. The cause was intracranial plasma cell granuloma involving left temporal lobe and old left temporo-parietal hemorrhage respectively. Aphasic symptoms of these patients were characterized by difficult comprehension and jargon speech with clear consciousness. The duration of aphasia ranged 9-14 days. EEG showed continuous periodic lateralized sharp waves in one patient and discrete rhythmic theta to alpha activities in left temporo-parieto-occipital region in the other patient. Comments：Although rare in adults, aphasic status epilepticus should be considered in the differential diagnosis of aphasia.OAIID:oai:osos.snu.ac.kr:snu2000-01/102/2014017262/3SEQ:3PERF_CD:SNU2000-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

EEG Patterns of High dose Pilocarpine-Induced Status Epilepticus in Rats

Background : We studied EEG changes during pilocarpine-induced status epilepticus(SE), a widely used model whose EEG characteristics have not been fully described previously. Methods : Male Sprague-Dawley rats weighing 250-350 grams were used as subjects. SE was induced 5-7 days after placement of chronic epidural electrodes, using 360-380 mg/Kg pilocarpine IP. Rats were observed with continuous EEG recording following pilocarpine injection until end of the SE episode. Results : SE occurred in 10/12 rats studied. SE began with a series of discrete seizures 11.1±3.93 minutes after pilocarpine injection. 5.2±2.71 seizures occurred over 10.9±4.62 minutes, until the EEG converted to a waxing and waning pattern, during which the amplitude and frequency of epileptiform activity increased. After 1.4±1.82 minutes, a pattern of continuous high amplitude rapid spiking was established. Continuous spiking continued for 3.4±0.48 hours with a very gradual decline in amplitude and frequency, until periodic epileptiform discharges(PEDs) began to occur. The EEG consisted primarily of PEDs for another 7.4±3.09 hours, until electrographic generalized seizures began to occur. These continued for 5.8±4.82 hours until death. Duration of SE was 17.0±5.88 hours. Flat periods were a prominent feature during all EEG patterns in this model. Conclusion : EEG features distinctive in pilocarpine SE(but not unique to it) include flat periods during all patterns and resumption of continuous spiking episodes after the onset of PEDs. The sequence of discrete seizures to waxing and waning to continuous spiking to PEDs was identical to that which has been described in humans and other animal models.OAIID:oai:osos.snu.ac.kr:snu2000-01/102/2014017262/1SEQ:1PERF_CD:SNU2000-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Two Patients with Epilepsy Induced by Complex Thinking

Reflex epilepsies are distinct but not clearly understood clinical entity. Various cerebral activities induced by simple stimulation including visual, auditory, somatosensory stimulation, as well as diverse functional tasks such as reading, calculation, complex thinking are believed to be seizure-inducing factors. We experienced two patients whose seizures were readily precipitated by complex, strenuous thinking. Both patients was teen-aged boy at the onset of seizure(13, and 15 years of age each) with normal physical and mental growth. Although first seizure was precipitated by watching TV and playing puzzles in each patient, initial diagnosis was idiopathic generalized epilepsy, possibly juvenile myoclonic epilepsy(JME). For the first few years, seizures were infrequent but mostly precipitated by the tasks needs concentration such as playing computer games, decision-making, mathematics, reading, or during the examination. EEG revealed various thinking process including reading hard books, drawing complex figure, complex calculation induced epileptic discharges even if it usually needs certain period of concentration. Phenytoin, valproic acid, clonazepam, vigabatrin, and lamotrigine sometimes abated their seizures but none of these made them seizure-free. Complex reflex epilepsy induced by thinking was proposed to be a separate type of epilepsy or a variant of JME. Age, sex, stereotypic seizure-inducing factors, clinical course, and refractory epilepsies in these patients highly suggested this type of epilepsy as a variant of JME but its refractoriness and unique provocation still needs more speculation.OAIID:oai:osos.snu.ac.kr:snu2000-01/102/2014017262/4SEQ:4PERF_CD:SNU2000-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Unilateral Restless Legs Syndrome

하지불안증후군은 하지에 움직이고 싶은 충동이 있고 불편하거나 기분 나쁜 느낌이 동반되며, 이러한 충동이나 감각 증상이 쉬거나 앉거나 눕는 것처럼 활동이 적을 때 발생하거나 악화된다. 이러한 증상은 움직이면 부분적으로 혹은 완전하게 사라지며, 낮보다는 밤에 악화되거나 밤에만 나타나는 것을 특징이다. 하지불안증후군의 증상은 대개 양측 하지에서 비슷하게 나타나지만 전체 환자의 29%는 한쪽에서 증상이 더 심하게 나타난다. 하지만 지금까지 편측 하지에 증상이 국한된 경우는 세 건의 증례가 있었다.3 저자들은 하지불안증후군의 진단기준에 맞고, 이차적 원인이 없으며 편측 상하지에 국한된 특발하지불안 증후군의 증례를 경험하였기에 보고한다.OAIID:oai:osos.snu.ac.kr:snu2012-01/102/2014017262/14SEQ:14PERF_CD:SNU2012-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:YEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Basic elelctrophysiology of the electroencephalography

Although neuroimaging techniques and other diagnostic procedures has been developed, electroencephalography (EEG) is still very important for the evaluation of various brain diseases and functional studies of human brain. EEG is formed mainly by spatial and temporal summations of postsynaptic potentials generated from a large population of pyramidal cells that can be considered as a collection of oscillating dipoles. EEG shows continuous rhythmic oscillation depending on sleep-waking state. Alpha rhythms are generated in cortical areas acting as epicenters with local spread, although the precise cellular mechanism is still unknown. Its been known that neurons in the nucleus reticular thalami are the pacemakers of sleep spindle. Alterations in the circuit of the reticular nuclei-thalamocortical relay neuron-cortical neuron are responsible for generalized spike and wave complexes. At the intracellular level, large paroxysmal depolarizing shifts produce focal epileptic spikes. Slow waves of EEG appear to be related to thalamocortical and/or corticothalamic deafferentation. The interpretation of routine EEG requires a well training from a qualified EEG teacher and reading adequate amount of EEG under supervision. Frequent misinterpretations of routine EEG have been observed in both local clinics and general hospitals. The most common findings of normal routine EEG misinterpreted as abnormal are normal variants and artifacts of various sources. There are considerable variations of normal EEG rhythms and pseudoepileptiform discharges. Eyeball movements produce prominent or subtle EEG changes over the frontal regions that are sometimes hard to be differentiated from abnormal slow waves over that region. Systematic approach was described for a good interpretation of routine EEG.OAIID:oai:osos.snu.ac.kr:snu2003-01/102/2014017262/3SEQ:3PERF_CD:SNU2003-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Comparison of sleep quality and polysomnographic findings in patients with RLS according to the presence of periodic limb movments during sleep

Objectives: Although it has been reported that periodic limb movements during sleep (PLMS) is present up to 80% in patients with restless legs syndrome (RLS), it is unclear that PLMS could influence on sleep quality and excessive daytime sleepiness (EDS) in patient with RLS. To investigate influence of PLMS on sleep quality and EDS, we analyzed sleep quality and EDS in patient with RLS. Methods: Fifty five RLS pa\-tients were included in this study. RLS was diagnosed according to International Restless Legs Syndrome Study Group criteria. Patients were classified into two groups according to the presence of PLMS. Group I (n=32) consisted of RLS patients without PLMS. Group II (n=23) consisted of RLS patients with PLMS. The severity of RLS symptom was assessed using International RLS Study Group Rating Scale (IRLS). Sub\-jective sleep quality was assessed by Pittsburgh Sleep Quality Index (PSQI), Athens Insomnia Scale (AIS), and Epworth Sleepiness Scale (ESS). Serum ferritin level and polysomnographic parameters were also com\-pared between both groups. Results: Mean age was significantly higher in RLS patients with PLMS (p<0.05). IRLS, PSQI, ESS and AIS were not significantly different between groups. Among sleep parame\-ters on polysomnography only PLM related variables were significantly higher in RLS patients with PLMS. Conclusions: Our study suggests that presence of PLMS in patient with RLS has no significant negative effect on sleep quality and excessive daytime sleepiness.OAIID:oai:osos.snu.ac.kr:snu2011-01/102/2014017262/6SEQ:6PERF_CD:SNU2011-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Transient Hyperorality during Automotor Seizure in a Patient with Right Temporal Lobe Epilepsy

Klüver-Bucy Syndrome (KBS) is consisting of hyperorality, emotional blunting, hypersexuality, altered dietary habits, visual and auditory agnosia. It has been reported in variable neurological diseases. However, only a few cases reported in epilepsy. We report a patient with unilateral temporal lobe epilepsy who presents transient hyperorality during seizure. A 46-year-old man has complex partial seizures which were abdominal aura followed by hyperorality and hand automatisms. Hyperorality was characterized by putting patients hand or seizure button into his mouth. Brain MRI demonstrated right hippocampal sclerosis. The interictal and ictal SPECT suggested right temporal lobe dysfunction, and PET showed bitemporal hypometabolism. Rhythmic ictal activities were arising from right temporal region when patient presented hyperorality. We speculate that transient hyperorality in this patient could be a symptom of KBS. This case suggests that transient KBS can be occurred in a unilateral temporal lobe epilepsy when ictal discharges cause bitemporal dysfunction during temporal lobe seizureOAIID:oai:osos.snu.ac.kr:snu2008-01/102/2014017262/3SEQ:3PERF_CD:SNU2008-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM:

Laryngeal Stridor in a Patient with Multiple System Atrophy

Sleep-related breathing disorders have been extensively reported as a frequent and major problem in patients with multiple system atrophy (MSA). Laryngeal stridor and obstructive sleep apnea are the most common sleep disordered breathing in MSA. We report laryngeal stridor in a patient with multiple system atrophy. A 56-year-old man presented with abnormal breathing sound different from snoring during sleep noticed by his bed partner. He is unaware of his nocturnal stridor. Polysomnography showed obstructive sleep apnea and laryngeal stridor. Laryngoscopy during wakefulness detects normal movement of vocal cord. CPAP (continuous positive airway pressure) eliminated laryngeal stridor and obstructive sleep apnea with pressures of 9 cmH2O.OAIID:oai:osos.snu.ac.kr:snu2007-01/102/2014017262/8SEQ:8PERF_CD:SNU2007-01EVAL_ITEM_CD:102USER_ID:2014017262ADJUST_YN:NEMP_ID:A079623DEPT_CD:801CITE_RATE:0DEPT_NM:의학과SCOPUS_YN:NCONFIRM: