Korean Database of Cerebral Palsy: A Report on Characteristics of Cerebral Palsy in South Korea

OBJECTIVE: To introduce the Korean Database of Cerebral Palsy (KDCP) and to provide the first report on characteristics of subjects with cerebral palsy (CP). METHODS: The KDCP is a nationwide database of subjects with CP, which includes a total of 773 subjects. Characteristics such as demography, birth history, onset and type of CP, brain magnetic resonance imaging (MRI) findings, functional ability and accompanying impairments, were extracted and analyzed. RESULTS: Preterm delivery and low birth weight were found in 59.51% and 60.28% of subjects, respectively. Postnatally acquired CP was 15.3%. The distribution of CP was 87.32%, 5.17%, and 1.81% for spastic, dyskinetic, and ataxic types, respectively. Functional ability was the worst in dyskinetic CP, as compared to other types of CP. Speech-language disorder (43.9%), ophthalmologic impairment (32.9%), and intellectual disability (30.3%) were the three most common accompanying impairments. The number of accompanying impairments was elevated in subjects with preterm birth and low birth weight. Brain MRI showed normal findings, malformations, and non-malformations in 10.62%, 9.56%, and 77.35% of subjects, respectively. Subjects with normal MRI findings had better functional ability than subjects with other MRI findings. MRI findings of a non-malformation origin, such as periventricular leukomalacia, were more common in subjects with preterm birth and low birth weight. CONCLUSION: The KDCP and its first report are introduced in this report, wherein the KDCP established agreement on terminologies of CP. This study added information on the characteristics of subjects with CP in South Korea, which can now be compared to those of other countries and ethnicities.

Clinical Usefulness of Sonoelastography in Infants With Congenital Muscular Torticollis

OBJECTIVE: To evaluate the clinical usefulness of sonoelastography in infants with congenital muscular torticollis (CMT). METHODS: The medical records of 215 infants clinically diagnosed with CMT were retrospectively reviewed. Fifty-three infants met the inclusion criteria as follows: 1) infants diagnosed as CMT with a palpable neck mass before 3 months of age, 2) infants who were evaluated initially by both B-mode ultrasonography and sonoelastography, and 3) infants who had received physical therapy after being diagnosed with CMT. We checked the thickness of the sternocleidomastoid (SCM) muscles in B-mode ultrasonography, strain ratio of the SCM muscles in sonoelastography, and treatment duration. We evaluated the correlation between the treatment duration and the following factors: SCM muscle thickness, ratio of SCM muscle thickness on the affected to unaffected side (A/U ratio), and strain ratio. RESULTS: Both the thickness of the affected SCM muscle and the A/U ratio did not show significant correlation with the treatment duration (p=0.66, p=0.90). The strain ratio of the affected SCM muscle was significantly greater than that of the unaffected SCM muscle (p<0.001), and the strain ratio showed significant correlation with the treatment duration (p=0.001). CONCLUSION: Sonoelastography may be a useful adjunctive tool to B-mode ultrasonography for evaluating infants with CMT, especially when predicting their rehabilitation outcomes.

Effects of Repeated Steroid Injection at Subacromial Bursa With Different Interval

OBJECTIVE: To evaluate the effects of repeated steroid injection at subacromial bursa with different interval for patient with periarticular shoulder disorder. METHODS: Group A (n=10) received subacromial bursa injection only on their first visit, group C (n=10) received the injection on their first visit and one week later, and group B (n=10) received the injection on their first visit and two weeks later. All injections were done with a combination of 40 mg (1.0 mL) of triamcinolone and 5.0 mL 0.5% lidocaine (6 mL total). We examined the active range of motion (AROM) of the shoulder joint, visual analogue scale (VAS), and shoulder disability questionnaire (SDQ) at baseline at 1, 2, and 4 weeks after the initial injection. RESULTS: In VAS, comparing the changes in VAS between groups, group B showed significant improvements compared with group A or C at 4 weeks after the initial injection (p0.05). In AROM, comparing the changes in AROM of external rotation between groups, group B and C showed significant improvement compared with group A at 4 weeks after the initial injection (p<0.05). CONCLUSION: It may be more effective in pain relief for patients with periarticular disorder to receive subacromial bursa injections twice with 2-week interval, as opposed to once.

Effects of Subacromial Bursa Injection With Corticosteroid and Hyaluronidase According to Dosage

OBJECTIVE: To evaluate effects of subacromial bursa injection with steroid according to dosage and to investigate whether hyaluronidase can reduce steroid dosage. METHODS: Thirty patients with periarticular shoulder disorder were assigned to receive subacromial bursa injection once a week for two consecutive weeks. Ten patients (group A) underwent subacromial bursa injection with triamcinolone 20 mg; another group of ten patients (group B) with hyaluronidase 1,500 IU and triamcinolone 20 mg; and the other ten patients (group C) with triamcinolone 40 mg. We examined the active range of motion (AROM) of the shoulder joint, visual analogue scale (VAS), and shoulder disability questionnaire (SDQ) at study entry and every week until 1 week after the 2nd injection. RESULTS: All groups showed statistically significant improvements in VAS after 1st and 2nd injections. When comparing the degree of improvement in VAS, there were statistically significant differences between groups C and A or B, but not between groups A and B. SDQ was statistically significantly improved only in groups B and C, as compared to pre-injection. There were statistically significant differences in improvement of SDQ after the 2nd injection between groups C and A or B. Statistically significant improvements in AROM were shown in abduction (groups B and C) and in flexion (group C only). CONCLUSION: Repeated high-dose (40 mg) steroid injection was more effective in terms of pain relief and functional improvements of shoulder joint than medium-dose (20 mg) steroid injection in periarticular disorder. Hyaluronidase seems to have little additive effect on subacromial bursa injection for reducing the dosage of steroid.

The Additive Effects of Hyaluronidase in Subacromial Bursa Injections Administered to Patients with Peri-Articular Shoulder Disorder

OBJECTIVE: To evaluate the additive effects of hyaluronidase combined with steroids in patients with peri-articular shoulder disorder. METHOD: Thirty patients with peri-articular shoulder disorder were given subacromial bursa injections once a week for three consecutive weeks. Fifteen patients (Group A) underwent subacromial bursa injections with hyaluronidase 1,500 IU, triamcinolone 40 mg and 0.5% lidocaine (total 6 ml). Another fifteen patients (Group B) underwent the same injections with triamcinolone 40 mg and 0.5% lidocaine (total 6 ml). We examined the active range of motion (AROM) in the shoulder, used a visual analogue scale (VAS) for measurement, and administered a shoulder disability questionnaire (SDQ) at the commencement of the study and then every week until one week after the third injection. RESULTS: There were no significant difference between group A and B before the injections took place (p>0.05). Statistically significant improvement was seen in the VAS, SDQ, and AROM of flexion, abduction, internal rotation at one week after the first and second injections compared with the parameters measured at previous visits in both groups (p0.05). Improvement in all parameters measured at one week after the third injection compared with the measurement values at one week after the second injection were not statistically significant in both groups (p>0.05). However, group A (the hyaluronidase group) showed significantly greater improvements than group B in terms of their SDQ and AROM of internal rotation scores one week after the three injections had taken place (p<0.05). CONCLUSION: Peri-articular shoulder disorder patients who underwent subacromial bursa injections using hyaluronidase and steroids showed greater functional improvements than those who were given only steroid injections.

Obturator Nerve Block Using Ultrasound-guided Intraneural Alcohol Injection in Patient with Hip Adductor Spasticity

OBJECTIVE: To investigate the effects of obturator nerve blocks with ultrasound guided intraneural alcohol injection. METHOD: Nine quadriplegic patients suffering hip adductor spasticity were included in this study. The obturator nerve was identified at just below inguinal ligament area on anteromedial surface of upper leg at supine position. An 23 G needle was inserted into anterior branch of obturator nerve under real time ultrasonography. The 50% alcohol solution was injected 3~5 ml at a time within the epineurium of the anterior branch of obturator nerve until the expanding nerve was visualized. We examed modified Ashworth scale (MAS) of hip adductor and passive range of movement of hip abduction at supine position at study entry, 1, 4, and 12 weeks after ultrasound guided intraneural injection. RESULTS: There were statistically significant improvement was seen in MAS of hip adductors and hip abduction angle at 1, 4, and 12 weeks after ultrasound guided intraneural injection, compared with parameters measured at previous injection (p<0.05). CONCLUSION: Ultrasound guided intraneural alcohol injection into anterior branch of obturator nerve for treatment of hip adductor spasticity in patients with quadriplegia is an effective and safe procedure for relieving localized spasticity of the hip adductors.

Effects of the Balance Control Training in Chronic Hemiplegic Stroke Patients

OBJECTIVE: To investigate the training effects on balance and gait ability using balance control trainer combined with partial weight-bearing system in chronic hemiplegic stroke patients. METHOD: A prospective crossover clinical trial was designed. The subjects consisted of 16 chronic hemiplegic stroke patients. All patients had a stroke more than six months. In addition to conventional physical therapy (PT), 8 patients in group A were trained with the balance control trainer for 30 min/day, 5 day/week, for first 2 weeks and then received only conventional physical therapy for 2 weeks. The other 8 patients in group B received only conventional PT for first 2 weeks and then were trained with the balance control trainer for 30 min/day, 5 day/week, for next 2 weeks, with additional conventional PT. We evaluated with clinical tests including functional ambulation categories (FAC), Berg balance scale (BBS), 6 min walking distance (6mWT), timed up and go (TUG), Korean-modified barthel Index (K-MBI) and muscle strengthening of knee extensor (MMTknee) before training, 2 weeks and 4 weeks after training in those patients. RESULTS: After training, subjects in experimental period (2weeks period of conventional PT+Balance control trainer in group A&B) showed more improvement than those in control period (2 weeks period of only conventional PT in group A&B) in FAC, BBS, 6mWT, TUG, K-MBI (p<0.05). CONCLUSION: We think the balance control trainer combined with the partial weight-bearing system can be a useful tool for improving balance and gait ability in chronic hemiplegic stroke patients.

Subacromial Bursa Injection of Hyaluronate with Steroid in Patients with Peri-articular Shoulder Disorders

OBJECTIVE: To investigate the additive effect of sono-guided subacromial bursa injection of hyaluronate with steroid in patients with peri-articular shoulder disorders. METHOD: This prospective randomized controlled trial involved 26 patients who had shoulder pain. Group A, consisting of 13 patients, was treated with a sono-guided subacromial bursa injection containing a mixture of 0.5% lidocaine (5 ml) and triamcinolone 40 mg (1 ml), followed by injection with sodium hyaluronate (2 ml) once a week for 3 weeks. The other 13 patients (Group B) were treated with a sono-guided subacromial bursa injection containing a mixture of 0.5% lidocaine (5 ml) and triamcinolone 40 mg (1 ml) once a week for 3 weeks. The effects were assessed using a visual analogue scale (VAS) of shoulder pain, active range of motion (AROM), shoulder function assessment scale (SFA), shoulder disability questionnaire (SDQ) at study entry and every week from first injection until 4 weeks after the 1st injection (= 2 weeks after 3rd injection). RESULTS: (1) Demographic features and all parameters measured before injection did not show a significant difference between the 2 groups. (2) Statistically significant improvements were shown in VAS, SFA, SDQ during the 1st, 2nd, and 4th week after the first injection in both groups (p<0.05). (3) SFA showed significant improvement at 1 week after injection only in group A (p<0.05). (4) AROM of internal rotation showed significant improvement at week 4 after the 1st injection only in group A (p<0.05). CONCLUSION: Subacromial bursa injection of hyaluronate with steroid in patients with peri-articular shoulder disorders has additive effects on functional improvement of the affected shoulder, including the AROM of internal rotation.

Effect of Botulinum Toxin Type A on Morphology of Salivary Glands in Patients with Cerebral Palsy

OBJECTIVE: To investigate the effect of botulinum toxin type A (BTXA) on drooling and the morphologic change of the salivary gland in patients with cerebral palsy. METHOD: Eight cerebral palsy patients suffering from severe drooling participated in this study. BTXA was injected into both submandibular and parotid glands under intravenous sedation and with ultrasound guidance (1 unit/gland/kg: maximum 100 units) in an outpatient or inpatient procedure. The severity of drooling was measured before injection and 3 weeks after injection using the Teacher Drooling Scale, the Drooling Score-severity, frequency and the Visual Analog Scale. To investigate the morphologic change of the salivary glands, the size of salivary glands were measured before injection and 3 weeks after injection using computed tomography of the neck. The measurement values were analyzed by Wilcoxon signed rank test. RESULTS: Statistically significant improvements were shown in all three parameters for assessing the severity of drooling after BTXA injections (p<0.05). Size of the salivary glands were significantly decreased at 3 weeks after BTXA injection (p<0.05). CONCLUSION: Salivary gland injection with BTXA could be a useful treatment method to reduce drooling in patients with cerebral palsy and decreased size of salivary glands may partially explain the mechanism.

Effects of Botulinum Toxin A Injection into Salivary Glands of Patients with Brain Lesion Suffering from Posterior Drooling

OBJECTIVE: The aim of the study was to evaluate the effectiveness of ultrasouond-guided salivary gland injection of botulinum toxin A (BTX-A) for posterior drooling. METHOD: 11 patients with brain lesion (9 cerebral palsy, 1 hypoxic ischemic encephalopathy and 1 mental retardation) with posterior drooling (an initial PDAS score greater than 2) and related pulmonary problems were recruited. Drooling severity was measured at baseline, 4 weeks, 3 months and 6 months after botulinum toxin A injection, by using Teacher Drooling Scale (TDS), Visual Analogue Scales (VAS), Drooling Score System (DSS)-severity, frequency and Posterior Drooling/Aspiration System (PDAS). RESULTS: The TDS, DSS-severity, DSS-frequency, VAS, PDAS were significantly reduced at 4 weeks and 3 months after BTX-A injection into salivary glands compared to pre-injection (p<0.05). However, there were no significant changes at 6 months compared to pre-injection level. CONCLUSION: BTX-A injection into salivary glands may improve anterior drooling in patients with brain lesions. Furthermore BTX-A injection into salivary glands may also decrease the posterior drooling which might related to respiratory symptoms in aspiration pneumonia.

Leg Length Discrepancy Induced by Arterio-Venous Fistula Due to Repetitive Vascular Punctures: A case report

The causes of leg length discrepancy in childhood include neurologic, musculoskeletal, tumor and vascular diseases. It may contribute secondary complications such as gait disturbance, scoliosis, hip joint arthritis or cosmetic problem etc. Acquired arteriovenous fistula is a rare cause of leg length discrepancy. Multiple vascular punctures in the neonates, especially in prematures, can result in iatrogenic arteriovenous fistula formation. We report two cases of arteriovenous fistula secondary to vascular punctures, diagnosed by color doppler ultrasonography and CT angiography. The lesions were explored and treated surgically.

Detection of Saliva Aspiration Using Salivagram in Bedridden Patients with Brain Lesion

OBJECTIVE: To investigate the aspiration of saliva itself in bedridden patients with brain lesion using the response of radionuclide salivagram, and its association with patient characteristics and clinical factors. METHOD: Thirty two patients (21 men and 11 women) in bedridden state with brain lesion were performed the radionuclide salivagram. (99m)Tc sulfur colloid (1.0 mCi in a drop of saline) was instilled into patients' tongue with supine position. The sequential images were obtained at first 5 minutes and 10 minutes interval for 1 hour, and evaluated the presence of saliva aspiration as the entrance of tracer into major airways or lung parenchyma. The characteristics of patients and the states of cooperation, drooling, tracheostomy, and method of feed were also assessed. RESULTS: Seven out of 32 subjects exhibited positive response of saliva aspiration by radionuclide salivagram. Men, uncooperative, and anterior drooling was significantly associated with positive finding of salivagram (p<0.05). CONCLUSION: In bedridden patients with brain lesion, it seems that radionuclide salivagram may be one of methods for detection of the aspiration of saliva itself.

Effects of the Constraint-Induced Movement Therapy on Cortical Reorganization in Patients with Hemiplegic Cerebral Palsy

OBJECTIVE: To investigate the effects of modified constraint- induced movement therapy (CIMT) on motor function and cortical activation in children with hemiplegic cerebral palsy (CP). METHOD: Five children with hemiplegic cerebral palsy were studied with Jebsen hand function test and quality of upper extremity skills test (QUEST), dynamic electromyography (EMG), and single photon emission computed tomography (SPECT) at rest before and after the CIMT period. Children were treated with a 4-week protocol of modified CIMT, consisting of twice-weekly 2-hour sessions of structured activities and a home program for non-treatment days. Children wore orthoses on their less affected upper extremities for 6 hours per day, during which time they were engaged in play, functional activities and 2-hour protocol of motor tasks. RESULTS: Improvements in upper-extremity function were found in Jebsen hand function test and QUEST. Increased muscle activities in elbow extensors were observed in dynamic EMG during affected hand grip. In right hemiplegic patients, regional cerebral perfusion increased in right Brodmann area (BA) 3 and lentiform nucleus. In left hemiplegic patients, regional cerebral perfusion increased in right BA 6, 9, 21, left BA 19 and left cerebellum, but decreased in left BA 11. CONCLUSION: Modified CIMT appears to change local cerebral perfusion in areas known to participate in movement planning and execution. These changes might be a sign of cortical activation after CIMT in the children with hemiplegic cerebral palsy. Results of this study suggest that modified CIMT may be an effective way of treating children with hemiplegic CP.

The Discharge Destinations of Geriatric Stroke Patients Admitted in the University Hospitals

BACKGROUND: The purpose of this study is to investigate discharge destinations and outcomes of geriatric stroke patients in university hospitals, and to examine their correlates and variables. METHODS: A total of 24 subjects, who were admitted between January and June 2006 in two university hospitals in Daegu, were enrolled in this prospective study. Data on demographic factors, socioeconomic factors, clinical, functional status and discharge destinations of patients were documented. Pearson's chi square test and t-test were used for statistic analysis. RESULTS: Ten patients were discharged to the community(their own home: 7, offspring's home: 3) and 14 patients were discharged to other hospitals(rehabilitation hospitals or nursing home:12, oriental medicine hospitals: 2) Analysis revealed that discharge destinations had significant correlations with severity of cognitive function, severity of speech disturbance, admission FIM scores and discharge FIM scores(p<0.05). CONCLUSIONS: Large part(75%) of geriatric stroke patients could not return to pre-stroke living situations. Their discharge destinations were associated with cognitive function, speech disturbance, admission and discharge FIM scores. Recognizing these correlations could be helpful for appropriate intervention to geriatric stroke patients during admission for more comprehensive rehabilitational management.

A case of Dyke-Davidoff-Masson syndrome in Korea / 소아과

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

Leigh Syndrome : Two cases report

Leigh syndrome is a subacute necrotizing encephalomyelopathy of infancy or early childhood. The clinical presentation can be highly variable. The classical presentations are central hypotonia, developmental regression or arrest, ataxia, ophthalmoplegia, and abnormal respiratory pattern. Diagnosis is usually confirmed by radiologic evidence of focal, bilateral and usually symmetric lesions of the both gray and white matter in the brain and the spinal cord. We experienced 2 cases of Leigh syndrome in a brother and sister.The first case, 4 year-old boy, was misdiagnosed as a cerebral palsy initially, but after acute infection, he revealed developmental regression and abnormal movement. His disease was confirmed by typical magnetic resonance imaging findings. The second case, 1 year-old girl with nystagmus, showed bilateral symmetric high signal intensity in globus pallidus on T2WI. We reported these cases with a brief review of the related literature.

Activation Pattern of the Somatosensory Cortex in Subcortical Stroke Patients with Clinical Sensory Recovery of Hand

OBJECTIVE: To investigate the activation pattern of somatosensory cortex in subcortical stroke patients underlying recovered somatosensory capacity of hand, using functional MRI (fMRI). METHOD: Four patients with subcortical cerebral hemorrhage or infarction and five normal healthy volunteers were investigated. Sensory task was given on the palm of hand by brushing as a frequency of 1 Hz. In fMRI study, ten slices were obtained using the Echo Planar Imaging technique, data was statistically analyzed using SPM-99 software. RESULTS: During the tactile stimulation of affected hand, contralateral primary somatosensory cortex was activated in all the patients. In the two patients with full recovery of tactile sense, cortical activation for paretic hand was stronger than in nonparetic hand. On the other hand, the other two patients with incomplete recovery showed that cortical activation for nonparetic hand was stronger than in paretic hand and even normal control. CONCLUSION: Our result suggested that functional consequences of the somatosensory cortical area were not limited to the ipsilateral hemisphere to the lesion, but affect the contralateral, nonlesioned hemisphere, in subcortical stroke patients with recovered somatosensory capacity.

Treatment of Idiopathic Cervical Dystonia with Phenol Block: A case report

Cervical dystonia is the most common form of local dystonia encountered in a movement disorders clinic. Rotatocollis is involuntary contraction of sternocleidomastoid and contralateral splenius muscles resulting in twisting of the head clockwise or counterclockwise on the axial plane. We injected phenol solution into the affeced muscle or nerve branch in two patients with rotatocollis. There was significant improvement in neck movement and position. Phenol block may offer an easy and inexpensive alternatives for patients with cervical dystonia.

Diffusion Tensor MRI on Evaluation of Microstructural Abnormality Compatible with the Motor Weakness in Children with Brain Injury

We present four pediatric cases examined by diffusion tensor MRI (DTI) to investigate microstructural abnormalities of brain related to their neurologic disability and motor weakness, following absence of any remarkable lesion on the motor pathway in conventional MRI. DTI is a relatively novel MR technique that enables investigation of the orientation and integrity of white matter fiber tracts of the brain in vivo. Case 1, 2 and 3 had suffered from cerebral palsy and case 4 had developed left hemiparesis after a traumatic brain injury. Conventional brain MRI presented no specific abnormality in case 1 and 2, a focal encephalomalacia in the left anterior thalamus in case 3, and contusional hemorrhages in the left temporal and parietal lobes in case 4. But DTI disclosed microstructural abnormalities on descending motor pathway including the posterior limb of the internal capsule or the cerebral peduncle of the midbrain in all cases.

Evidence of Microstructural Abnormality on Descending Motor Pathway in Cerebral Plasied Children with Periventricular Leukomalacia: Diffusion Tensor MRI Study

OBJECTIVE: Using diffusion tensor MRI (DTI), to investigate the microstructural abnormality of corticospinal tract in the cerebral palsied children with periventricular leukomalacia on conventional MRI and to recognize the clinical usefulness of DTI. METHOD: Seven patients were studied. DTI was peformed using 1.5T MR scanner (Vision Plus, Siemens, Erlangen, Germany) and fractional anisotropies of corona radiata, posterior limbs of internal capsules, and cerebral peduncles of midbrain in both hemisphere were calculated. RESULTS: The fractional anisotropy was significantly decreased in all corona radiata, posterior limbs of internal capsules, cerebral peduncles of midbrain in six patients except one hemiplegic patient, compared with that of control group. In in the hemiplegic patient, the fractional anisotropy was reduced only in affected hemisphere. In addition, the fractional anisotropy tended to be also increased as gross motor function measure (GMFM) score was increased. CONCLUSION: We believe that DTI may be efficient in evaluating microstructural abnormality on the motor pathway of brain and helpful in providing prognosis of clinical findings in cerebral palsied children with periventricular leukomalacia.