Cerebral palsy is a non-progressive cerebral sensori-motor defect, acquired either prenatally or in an early life and evolves over the first few years. Until recently many people believed that asphyxia at birth was a major cause of cerebral palsy and that the prevention of asphyxia at birth by improving perinatal care would reduce the number of cerebral palsy children. However the incidence of cerebral palsy in children has remained steady or ever risen slightly. The real cause of cerebral palsy is still unbaron to us.

This analysis was undertaken to determine the clinical features of cerebral palsy in Korea by the retrospective study of 98 children. Over a half of infants with cerebral palsy (64.2%) was recognized by parents before 1 year of corrected age, and their chief complaints were delayed developments or equinus foot deformities.

The most common type of cerebral palsy was spastic type (64.2%) which was followed by athetoid (10.5%), ataxia and hypotonia types (4.2% each). The mixed type was 19.4% Among 98 cerebral palsies, the preterm infants were 42.9% and the infants with low birth weight were 41.4%. The cerebral palsies with low birth weight and preterm infants were more likely to have spastic diplegia.

The most frequent abnormal primitive reflex was absent protective extension(78.3%). No significant associations of the type of cerebral palsy with primitive reflexes were found. An increased risk of cerebral palsy with increased maternal age was not observed in this study.

Of 55 MRI findings, no abnormalities were seen in 27.2%, periventricular leukomalacias in 34.5%, brain atrophies in 21.8%, cerebral infarcts in 10.9%, intracerebral hemorrhage in 3.6%, and delayed myelinations in 1.8%. The periventricular leukomalacias were associated with the preterm infants in 63.2%.

The authors studied 60 children (aged 4 months to 9 years) with cerebral palsy by means of magnetic resonance imaging (MRI) and single photon emission computed tomography (SPECT) of the brain. MRI showed normal in 15 cases (25.0%) and deep white matter pathology in 37 cases (61.7%), corpus callosum abnormality in 29 cases (48.3%). In addition, abnormal intensity of thalamus or basal ganglia and delayed myelination were found in minor cases. In all of the patients except for 1 case, SPECT showed the abnormal findings SPECT demonstrated hypoperfusion of thalamus in 58 cases (96.7%), cerebellum in 27 cases (45.0%), frontal and parietal lobe in 26 cases (43.3%), basal ganglia in 23 cases (38.3%) and temporal lobe in 22 cases (36.7%). There was no significant correlation between the severity of motor developmental impairment and the radiologic findings. The results of this study suggest that MRI of the brain provides the anatomical information such as deep white matter and corpus callosum pathologies resulted from the hypoxic-ischemic insult and SPECT of the brain is a very sensitive tool for the assessment of the functional abnormalities in cerebral palsy.

The purpose of this study was to investigate the prevalence of cerebral palsy among neonates with risk factors and the relationship between cerebral palsy and risk factors.

Among 6,220 neonates who were born in Yonsei University Hospital between January 1, 1990 and December 31, 1992; 699 neonates had more than one risk factors which were prematurity, low birth weight, perinatal asphyxia, and hyperbilirubinemia. This study comprised 361 neonates with risk factors who could be followed up to 1 year of age. The prevalence of cerebral palsy was 4.7% and the prevalence of delayed development was 2.5%. Among 264 cases with a prematurity, 15(5.7%) cases were diagnosed as cerebral palsy. Among 239 cases with a low birth weight, 12(5.0%) cases were diagnosed as cerebral palsy. Among 228 neonates with an APGAR score below 7 point in 1 minute, 16(7.0%) cases were diagnosed as cerebral palsy and among 79 neonates with an APGAR score below 7 point in 5 minute, 9(11.4%) cases were diagnosed as cerebral palsy.

Chemical neurolysis can be an extremely effective intervention for reducing spasticity. Phenol nerve block as a method of chemical neurolysis has been used over 40 years, nevertheless, many clinicians remain reluctant to perform this procedure. This is largely due to the fear of its side effects and complications such as excessive muscle weakness or increased spasticity of antagonistic muscles.

The purpose of this study was to titrate the nerve block effects of phenol with different concentrations and different volumes of the phenol solution. Left tibial nerves of forty eight adult rabbits were injected with phenol solution of different concentrations(5%, 4%, 3%) and volumes(0.3 ml, 0.2 ml, 0.1 ml) into the epineural sheath. Nerve conduction study of the gastrocnemius muscle was performed before and after the nerve blocks(1day, 1week, 2weeks, 4weeks, 8weeks). The proportion of compound muscle action potential(CMAP) amplitudes and areas before and after the nerve blocks was used for the evaluation of nerve block effect.

There was a statistically significant difference in the proportion among three volumes of phenol solution(p＜0.05). However there was no statistical difference in the proportion among three concentrations of phenol solution(p＞0.05) although the higher concentration of phenol solution showed the tendency for smaller proportion. The area of histological degeneration appeared at 2 weeks following nerve block alongside the perineurium. The depth of degeneration area of nerve fascicle varied according to the distance from injection point.

These data suggest that the nerve block effect of phenol can be titrated more easily with the volume of phenol solution rather than the concentration until 8 weeks after the nerve block. The variation of the extent of degeneration with different volumes of phenol solution seems to be the mechanism for the titration.

Spasticity is common stroke in patients, and its management has been considered as one of the major problems in stroke rehabilitation. The goal of this study was to determine if transcutaneous electrical nerve stimulation(TENS) and acupuncture would reduce the muscle spasticity. To estimate the efficacy of electrical stimulation for the treatment of spasticity TENS(100 Hz, asymmetric bipolar pulse current) was applied to the skin over the extensor muscles of spastic limbs for 20 minutes, once a day in six stroke patients. In addition, acupuncture was also applied to the acupoints of extensor muscles of all extremities and face for 20 minutes twice a day to determine the efficacy of acupuncture for the treatment of spasticity in six stroke patients. As controls subjects, six stroke patients were examined without TENS or acupuncture treatment. In experimental groups, the efficacy of treatment was measured 1, 5, 10, 15 days and 20 days after treatment with either TENS or acupuncture using the spasticity measurement methods (modified Ashworth scale, ankle clonus score, and H/M ratio). Based on the results from the present study, we have concluded that the H/M ratios of affected spastic limbs were significantly higher than those of unaffected limbs (p＜0.05). TENS and acupuncture therapies lessened the spasticity of affected limbs of stroke patients when measured with the modified Ashworth scale however not with the H/M ratios nor with the ankle clonus scores.

Visual Perception in stroke patients is very important in the respect of prognosis and functional recovery. Therefore understanding and knowledge of the visual perceptual dysfuction should be the base for the training in activities of daily living.

The present study was designed to test the disturbance of visual perceptions, espescially for error in visual perception of verticality or horizontality of rod in hemiplegic patients.

In additions, we assessed the hemiplegic and control groups, for the balance index, the duration of disease onset, and the lateralization and localization of lesions by computerized tomography or magnetic resonance image respectively with analysis of an each factor.

The vertical average error angles of hemiplegic patients are greater than those of controls. The error angles of the left hemiplegic patients are greater than those of the right hemiplegia. Correlation between the vertical average error angles and the balance index has the reversed coefficient factors. Verticality recovers mostly in 2∼3 months of onset.

The neurobehavioral cognitive status examination(NCSE), a screening examination tool that assesses cognitive funtion in a brief but quantitative fashion, is used as an independent test of five major areas: language, constructions, memory, calculation and reasoning. The examination also separately assesses levels of consciousness, orientation, and attention. This instrument quickly identifies the intact areas of functioning, yet provides more detailed assessment in the areas of dysfunction. To determine whether the neurobehavioral cognitive status examination is a more sensitive instrument for the detection of cognitive impairment than the minimental state examination(MMSE), we performed these test simultaneously in 26 patients with stroke. The sensitivities were 88.5% for NCSE and 53.8% for MMSE respectively. The sensitivity of NCSE was derived from two features of its design: the use of independent tests to assess skills within five major areas of cognitive functioning, and the use of graded tasks within each of these cognitive domains.

This study was designed to compare the percutaneous endoscopic gastrostomy(PEG) tube feeding with the nasogastric(NG) tube feeding for the patients with dysphagia after the stroke, and to find out the most optimal timing for the PEG tube feeding.

We monitored the nutritional parameters, the frequency and the timing of complications, and other risk factors in 54 stroke patients with dysphagia. In the group of patients with the nasogastric(NG) tube feeding, a reduction in nutritional parameters was greater than in the group of patients with PEG tube feeding. Especially the reduction in serum hemoglobin and albumin level was statistically significant. Thirteen cases of aspiration pneumonia who had frequent self removal of feeding tubes developed in the group with NG tube feeding. Most cases of aspiration pneumonia in the NG tube feeding group developed within the first 2 weeks. Complications from the PEG tube feeding group were three cases of upper gastrointestinal bleeding and three cases of local infection. There were no correlations between the duration of dysphagia and the location of brain lesions, the history of tracheostomy, the age, the initial mental status, or the artificial ventilation. But, there was a significant prolongation of duration of dysphagia in the group of patients who had a vocal cord palsy, an absence of gag reflex, a paralytic dysarthria and a prolonged intensive medical care.

We conclude that the PEG tube feeding is a safer and the more effective method to provide a long term enteral nutrition to patients with neurological dysphagia than the NG tube feeding. Since the most complications developed in the first 2 weeks, the PEG tube feeding should be applied within the initial 2nd to 3rd week for the stroke patients with dysphagia and aspiration risks. Further prospective study will be needed to decide an ideal timing of PEG tube feeding after an acute stroke.

As a result of significantly improved medical and surgical cares, traumatic brain injury has been significantly increasing. This calls for an imperative study of the perception ability and recovery in physiological and mental functions. With the assumption that comatous patients may respond to various auditory stimulations, authors studied somatic physiological responses searching for significant basic character of auditory stimulation using three different kinds of sound. Thirteen patients were stimulated with voices, classic musics and bird songs through the earphones. Pulse rates and respiration rates were checked simultaneously. The changes of pulse and respiration rates were not statistically significant. A trial to help recovery from a coma state, with Glasgow coma scale below 8, was not so significant clinically wiith these simple auditory stimulation. A further study of somatic physiological response to more complicated auditory stimulations or patients with clasgow coma scale above 8 would be necessary. In conclusion, for the comatous patients Glasgow coma scale below 8, family voices, classic musics and bird sounds did not change pulse rates and respiratory rates of statistical significance. In the comatous patients with Glasgow cama scale below 8, the trial to promote recovery by simple auditory stimulation seems to be unsuccessful clinically. More extensive study would be required.

The purpose of this study was to collect the informations on the current status and the needs of the physically disabled elderlies in the rural community along with the prevalence rate of these population. One study group for the study of disability status evaluation was 139 physically disabled elderlies living in the main island of Kangwha, and the other group for the study on prevalence of disability was 542 elderlies living in Songhae myun. The prevalence of physical disability was 6.5% in Songhae myun. The neurologic disorder was the most frequent diagnosis among the physically disabled elderlies followed by the musculoskeletal disorder. The time for the medical service delivery was delayed; 45.3% of the subjects received medical service more than one month after the onset of disability. Only 18.0% of the disabled elderlies received the public disability service, and 15.8% of the subjects was waiting for further service. The most common need from the subjects was the medical service, followed by the service from the institution such as a nursing home and the financial support. Registration rate of the disabled was very low(7.7%). There were only three physical therapists for the rehabilitation services in studies areas.

This study revealed that the public concepts for the disability and the rehabiltation service were inadequate. We hope that this basic data can be used for the planning of rehabilitation services in this community.

Spinal cord injury causes a decrease in bone mass, an osteopenia and an increased risk of fractures. In this condition, previous histomorphologic and biochemical reports have shown an uncoupling between bone formations and resorptions, however the exact sequence of events resulting in bone loss is still not fully understood. Since accurate and sensitive techniques have become available recently to assess bone metabolism, more informations are now available regarding the bone loss in paralysed or immobilized individuals. The purpose of this study is to clarify the changes of biochemical markers and bone densities. Ten complete and 10 incomplete spinal cord injury patients were enrolled for this study. The bone density of femur and lumbar vertebra, and the biochemical markers such as serum osteocalcin and urine deoxypyridinoline were measured. Results were analyzed by Mann-Whitney method and Pearson's correlation of SPSS PC program.

Comparing with normal values, in the spinal cord injury groups, the values of serum osteocalcin were elevated(p＞0.05), and also the values of urine deoxypyridinoline were significantly elevated(p＜0.05). The duration after spinal cord injury and the bone density of femur and lumbar vertebra showed a moderate negative correlation(Pearson's R: ⁣0.47, ⁣0.43, respectively)(p＜0.05).

In conclusion, the results of increased values of biochemical markers in bone metabolism support that the bone turn-over rate increases after the spinal cord injury.

The purpose of this study is to measure the skin epidural distance(SED) of L3∼4 interspace and to correlate SED with the individual constitutional data of patients such as body-weight, height, abdominal circumference and body mass index(BMI; kg/m2). In this study we examined 120 patients (45 men and 75 women) who had no pathological abnormality in L3∼4 interspace such as herniated disc, spondylolisthesis and spinal stenosis in MRI study, although majority of patients had pathology below this level. The SED was measured under fluoroscopic guide with loss of resistance technique, and we also measured individual constitutional data of patients.

Pearson's correlation analysis of the data showed that there were no correlations of statistical significance between SED and the height, abdominal circumference of the patient (p＞0.01). But there were statistically significant correlations between SED and body-weight as well as BMI (p＜0.0001). Linear regression analysis of these data showed significant correlations between SED and body weight(male r²=0.49, p＜0.0001, y=0.37x⁢18.1; female r²=0.31, p＜0.0001, y=0.18x⁢30.8), BMI(male r²=0.52, p＜0.0001, y=0.89x⁢23.1; female r²=0.35, p＜0.0001, y=0.41x⁢31.6).

Patellofemoral pain syndrome (PFS) is one of the most common types of anterior knee pain and many studies reported the patellofemoral malalignments (such as high patella, increased sulcus angle and increased congruence angle) as possible causes of the syndrone. In order to confirm this hypothesis, the difference of congruence, sulcus angles and patella height ratio in normal subjects and PFS patients was evaluated. The PFS subjects were selected according to the criterias of anterior knee pain, pain aggravation after the repeated knee flexion and extension motion. A combined knee injury was screened by MRI. We have compared our data to the previous studies of Merchant et al.(1974), Aglietti et al.(1983), and Dowd and Bently(1986). The radiographic studies were done on lateral view, Merchant view, and patella height was measured by Insall-Salvati method and Blackburn-Peel method. The sulcus angle and the congruence angle were measured on a Merchant view.

The results showed that the average patella height of normal group was 1.00⁑0.17 by the Insall test and the patella index was 1.02⁑0.23. There was no statistical difference between normal and patient group of which the patella height was 0.96⁑0.16 by Insall test with patella index of 1.04⁑0.17.

On Merchant view, the congruence angle was ⁣7⁑11 degrees, and the sulcus angle was 135.5⁑6 degrees in PFS patient group, and in normal group the congruence angle was ⁣2⁑14 degrees, and the sulcus angle was 136⁑5. A little statistical variation was noted in both group.

Our results were not much different from previous studies except for the Aglietti's study in congruence angle.

We concluded that the Insall test, patella index, congruence angle and sulcus angle could not help to idetify patellofemoral malalignment of knee joint in PFS patients.

For the prescription of exercise, the intensity is most important. And the most commonly used method for the regulation of exercise intensity is the target heart rate calculated by the results of graded exercise test. However, the heart rate method has many disadvantages such as variances in measurement, cessation of exercise to measure the rate, difficulties in application to patients on cardiac medications like beta-blockers. Rating of perceived exertion(RPE scale) has been widely used for the prescription of exercise intensity in the cardiac and the pulmonary rehabilitation. RPE scale can be superior to the heart rate method since the patient does not have to stop exercise to measure heart rates and also medications would not influence the program.

The purpose of this study was to find out if RPE scale would be useful method compared to other various parameters in normal subjects and cardiac patients for the prescription of exercise intensity.

In 250 normal healthy adults with normal graded exercise test results and 42 cardiac patients, RPE scale, heart rate, systolic blood pressure, diastolic blood pressure, expiratory volume, and respiratory rate were measured during the graded exercise test. Correlation analysis was done between these parameters.

In normal adults, heart rate, respiratory rate, double product(heart rate times systolic blood pressure), oxygen consumption to maximal oxygen consumption ratio(VO2max%) were significantly correlated with RPE scale. VO2max% showed the highest explanatory power of those parameters.

In cardiac patients, heart rate, respiratory rate, expiratory volume, VO2max%, double product were significantly correlated with RPE scale. Also, VO2max% showed the highest explanatory power. VO2max% at each RPE scale was slightly lower in cardiac patients than in normal adults but it was not statistically significant.

The results showed that RPE scale was highly correlated to VO2max% in both groups.

VO2max% at the same RPE scale was not statistically different in both groups.

We concluded that RPE scale is a useful method for the prescription of exercise intensity in both normal person and cardiac patients.

The changes of anterior horn cell excitability and conduction of the nervous system by the electrical stimulation of nerve have been reported in both vivo and vitro studies. Purpose of this study is to observe the neurophysiologic changes of nerves by 10 Hz electrical stimulation on polyneuropathic peripheral nerves. Subjects were 18 diabetic polyneuropathic patients diagnosed by the conduction studies. Electrophysiologic studies were performed in both right and left tibial nerves before and after conditioning of the right tibial nerve. Electrophysiologic studies included five tests which were the sural sensory and tibial motor conduction(abductor hallucis), F response(abductor hallucis), H reflex(gastrosoleus) and somatosensory evoked potential(ankle, SEP). Ten Hz rectangular electrical current was used for the conditioning stimulation. It was applied to the popliteal tibial nerve with the tolerable maximal intensity(10∼24 mA) for 5 minutes.

Following changes were statistically significant in statistics after the conditioning. Prolongation of F latency (p＜0.05), increases of F chronodispersion, duration and area(p＜0.05), prolongation of H latency(p＜0.05), increase of H amplitude(p＜0.05), decrease of P1 latency of SEP(p＜0.01) and increase of P1N1 amplitude of SEP(p＜0.01) were seen in both conditioned and unconditioned legs. Increase of F wave conduction time(FWCT) and decrease of F wave conduction velocity (FWCV) were seen in conditioned leg(p＜0.05).

Above findings suggest that certain electrical stimulation of polyneuropathic nerve may cause increase of the anterior horn cell excitability, fascilitation of the SEP conduction and slowness of alpha motor conduction to and from the spinal cord.

Thenar motor unit number estimation(MUNE) was performed in 49 normal subjects without known neuromuscular diseases using statistical method. The purposes of this study were to compare MUNE parameters between the dominant and non-dominant hands and to evaluate the changes according to age. Reproducibility was assessed in 20 subjects also. The results showed that thenar motor units number, maximum negative peak compound muscle action potential (CMAP) area and mean area of single motor unit potentials(SMUP) were not different between dominant and non-dominant sides, but maximum negative peak CMAP amplitude was larger in dominant than non-dominant side. With aging, the motor unit number, maximum negative peak CMAP amplitude and area decreased and the mean area of single motor unit potentials increased. There was a high reproducibility between the test and the retest values for motor unit number, maximum negative peak CMAP amplitude and area as well as mean area of single motor unit potentials.

The statistical estimate of the motor unit number is a reliable method and easily available in clinical settings. The results of this study are expected to be used as a baseline data for the future.

The study of blink reflexes was carried out to demonstrate the correlations, if there were, between the stage of diffuse axonal injury(DAI) and the abnormality of blink reflexes. The blink reflex was recorded in 20 healthy adult subjects and 22 patients with DAI who were classified according to Adams' classification(DAI I; 7, DAI II; 9 and DAI III; 6). The latencies and amplitudes of R1 and R2 in patients with DAI were compared with those of healthy subjects.

The results were as follows; 1) In 20 subjects of patient group, the latencies of R1 were all within a normal range. In 2 subjects, the difference in latencies between the two sides was above 1.4 msec. 2) In 15 subjects, R2 was absent or delayed, and reduced in the size of amplitude in all. Nine were affected bilaterally, and 4 were unilaterally. 3) Seventy one percent of patients in each stage represented abnormal findings. 4) There were no correlations between the DAI stage and the blink reflex.

This study demonstrated that the polysynaptic R2 was more profoundly suppressed than the oligosynaptic R1 in a diffuse axonal injury because of a loss or decrease of suprapontine facillitation, which influenced the trigeminal spinal complex and the interneuron of lateral reticular formation.

Recent studies for the diabetic polyneuropathy have quantified as well as compared the clinical and the electrophysiological findings. Thirty-one diabetic patients were examined with a conventional nerve conduction study, a late response, a somatosensory evoked potential (SEP), a sympathetic skin response (SSR), a R-R interval, and a needle electromyography (EMG) after the clinical examination. The purposes of this study were to evaluate the correlation of clinical features and electrophysiological findings and to provide reliable criteria for the diagnosis of diabetic polyneuropathy. Diabetic polyneuropathy was diagnosed when a nerve conduction study revealed abnormal findings in two or more peripheral nerves. The following parameters were highly correlated with a diabetic polyneuropathy; ankle jerks (Spearman's r=0.92), H reflexes (Spearman's r=0.92), F waves by the tibial nerve stimulation (Spearman's r=0.88), F waves by the peroneal nerve stimulations (Spearman's r=0.84), and amplitudes of a sensory nerve action potential (SNAP) in the sural nerve (Spearman's r=0.79), SEPs by the tibial nerve stimulation (Spearman's r=0.79). Motor nerve conduction of the upper limbs, R-R interval ratios, and blood pressure changes had no significant correlations with a diabetic polyneuropathy.

We concluded that these clinical and electrophysiological findings with significantly high correlation values would be good parameters for the diagnosis of diabetic polyneuropathy.

In a previous study, the authors have suggested that there is a central mechanism in pain modulation of TENS, and interferential current therapy(ICT) by using the tail-flick reflex and measuring the cerebrospinal fluid Ղ-endorphin of the rat. To confirm this central opiate-mediated pain modulation, we examined the reversal of a delayed tail-flick reflex latency and the cerebrospinal fluid Ղ-endorphin level by the naloxone administration. We measured the latencies of fictive tail-flick reflex before, immediately after TENS, ICT, and 15 minutes after naloxone administration. Cerebrospinal fluid Ղ-endorphin was also measured quantitatively by radioimmunoassay after TENS, ICT and naloxone administration.

The results revealed that in the group with TENS application, the latency of tail flick reflex after naloxone administration(31.54⁑5.08) was reversed to the level before the TENS application(34.76⁑6.09 msec) compared to the level after the TENS application(were 42.28⁑10.14 msec). With ICT application, the latency of tail flick reflex after naloxone administration(32.46⁑5.52msec) was also reversed to the level before the ICT (33.39⁑4.72 msec) application compared to the level immediately after the ICT application(46.87⁑10.14 msec). The Ղ-endorphin levels in cerebrospinal fluid were significantly decreased in the groups of naloxone administration, both with the TENS(14.86⁑3.92 pmol/l) and the ICT applications(18.04⁑3.93 pmol/l) compared to the control group(52.05⁑14.12 pmol/l).

We confirmed that the central pain modulation mechanism of TENS and ICT was through the reversal of delayed tail-flick reflex latency and elevated CSF β-endorphin level after naloxone administration

Glycogen Storage Disease Type II is caused by the deficiency of acid maltase resulting in lysosomal accumulation of glycogen. There are two major clinical syndromes, a severe generalized and invariable fatal disease of infancy, and a myopathy starting in juvenile or adult life.

The clinical and laboratory findings of a patient with Glycogen Storage Disease Type II are presented. The patient, a 17-year-old male, experienced slowly progressive weakness of muscle of the pelvis shoulder girdles and trunk. Muscle biopsy showed vacuolar myopathy and electromyograph showed features of myopathy with fibrillation potentials, positive sharp waves, myotonic discharges, without clinical myotonia at rest, and polyphasic potentials on volition.

Clinical features, histopathologic and electrophysiologic findings of this disease and differential diagnosis were reviewed.

Electrophysiologic monitoring during surgery for the spinal cord tumor is necessary for identification of the nerve root, prevention of the nerve injury, and prediction of postoperative prognosis. In other countries, intraoperative electrophysiologic monitorings are commonly done in various cases such as selective posterior rhizotomy, scoliosis, sponlylolisthesis, lipomeningocele, and spinal cord tumor, but it is not a common procedure in Korea except for the selective posterior rhizotomy.

We report 3 cases of electrophysiologic monitoring during sugery for the spinal cord tumor at lower thoracic level. Using multichannel EMG machine, we recorded free-run EMG, somatosensory evoked potential(SSEP) of tibial nerve, and compound motor unit action potential (CMAP) of various regions such as abdomen, lower extremity, and anus, stimulating nerve roots at the lower thoracic level.

We identified CMAP from rectus abdominis muscles only in the first case, but in the second case, we identified CMAP from tibialis anterior and gastrocnemius muscles which avoided the injury to lumbosacral roots. In the third case, SSEP improved immediately after we removed the mass at lower thoracic level.

We concluded that intraoperative electrophysiologic monitoring combined with recording CMAP is a useful procedure to minimize neural tissue damage during surgery for the spinal cord tumor at lower thoracic level.

Palatal tremor is a rare disorder characterized by involuntary rhythmic movements of the soft palate. Palatal tremor is devided into symptomatic palatal tremor(SPT) and essential palatal tremor(EPT) on the basis of clinical features. SPT is associated with brain stem or cerebellar disease, whereas the EPT has no known etiology. Reverberant neural activity in the region of the brain stem or cerebellum within the Guillain-Mollaret triangle is believed to underlie SPT. We present a case of SPT associated with cerebellar lesion. Electromyographic recording from the levator veli palatini muscle and voice spectrogram analysis showed abnormal bursting activity time locked to the palatal movements. Botulinum toxin was injected into the levator veli palatini muscle to reduce the voice tremor. The pathology and management of this condition is briefly discussed.

Myositis ossificans progressiva, a rare autosomal dominant disorder, is characterized by progressive heterotopic ossification of muscle and connective tissue associated with pain and disability.

I have experienced a 15-year-old woman with multiple contracture and deformity in both lower extremities. Clinical features and laboratory findings including electrodiagnostic findings were compatible with myositis ossificans progressiva. I report this case with the review of literature.

The Proteus syndrome is a recently described congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the musculoskeletal system, primarily by hemihypertrophy, macrodactyly, exostoses and kyphoscoliosis, and the skin and the subcutaneous tissue, primarily by pigmented nevi and subcutaneous tumors. These findings are diagnostic features of Proteus syndrome. We report typical manifestations of Proteus syndrome in a 12-year-old boy with brief review of literature.