Does Injured Corticospinal Tract Recover after Rehabilitation Therapy in Patients with Hemiplegic Cerebral Palsy?

OBJECTIVE: To investigate whether injured corticospinal tract (CST) could recover after rehabilitative therapy in hemiplegic patients with cerebral palsy using diffusion tensor tractography (DTT) which is known to be useful in detecting microscopic white matter lesion in vivo. METHOD: Sixteen hemiplegic patients (mean age, 13.6 months) were enrolled. Diffuse tensor image (DTI) and gross motor functional measurement (GMFM) evaluation were performed before rehabilitative therapy and at follow-up. We measured the fractional anisotropy (FA), and apparent diffusion coefficient (ADC) of CST in the affected and unaffected hemisphere. To evaluate asymmetry of affected and unaffected CST, asymmetry index of fractional anisotropy (AA) and asymmetry index of apparent diffusion coefficient (AD) were measured. RESULTS: The FA value of the affected CST was lower than that of the unaffected CST and the ADC value was higher than that of the unaffected CST in initial and follow up DTT (p<0.001). Compared with the result of initial DTT, the results of follow up DTT demonstrated that the FA value of both CST was increased (p=0.000). A significant increase in AA was showed in all patients (p=0.000) and the increase of AA had significant correlation with the increase of FA of affected CST, but not with the unaffected CST (r=0.537, p=0.032). CONCLUSION: DTT would be a powerful modality not only for diagnosis and prognosis of cerebral palsy, but also assessment of microstructural change of CST after rehabilitative therapy.

The usefulness of diagnostic tests in children with language delay / 소아과

PURPOSE: To assess the usefulness of magnetic resonance imaging (MRI), karyotyping, brainstem auditory evoked potential (BAEP), electroencephalogram (EEG), tandem mass screening test, and newborn metabolic screening test in children with language delay for diagnosing underlying diseases. METHODS: From January 2000 to June 2007, a retrospective chart review was performed for 122 children with language delay who visited the Child Neurology Clinic at Yeungnam University Hospital and who underwent neuropsychologic tests and other diagnostic evaluations for underlying diseases. They were grouped into phenomenological diagnostic categories, and test results were analyzed according to the underlying diseases. RESULTS: Of 122 patients, 47 (38.5%) had mental retardation, 40 (32.8%) had developmental language disorders, 23 (18.9%) had borderline IQ, and 12 (9.8%) had autism spectrum disorder. In 26 (21.3%) cases, the causes or relevant clinical findings to explain language delay were found. Eight (10.4%) of 77 MRIs, 6 (8.0%) of 75 EEGs, and 4 (5%) of 80 BAEPs showed abnormal results. Results directly attributed to diagnosing underlying diseases were 2 hearing defects in BAEPs and 1 bilateral perisylvian cortical dysplasia in MRIs. No abnormal results were found in karyotyping, tandem mass screening tests, and newborn screening tests. CONCLUSION: Commonly used tests to diagnose the cause of language delay are not very effective and should only be used selectively, according to patient characteristics. However, despite the low diagnostic yields from these tests, because many patients show abnormal results, these tests are useful when conducted in complete evaluation.

Usefulness of DTI-based three dimensional corticospinal tractography in children with hemiplegic cerebral palsy / 소아과

PURPOSE: Magnetic resonance diffusion tensor imaging-based three-dimensional fiber tractography (DTI-FT) is a new method which demonstrates the orientation and integrity of white matter fibers in vivo. However, clinical application on children with cerebral palsy is still under investigation. We present various abnormal patterns of DTI-FT findings and accordance rate with clinical findings in children with hemiplegic cerebral palsy, to recognize the usefulness of DTI-FT. METHODS: The thirteen children with hemiplegic cerebral palsy evaluated at Yeungnam University hospital from March, 2003 to August, 2007 were enrolled in this study and underwent magnetic resonance DTI-FT of the corticospinal tracts. Two regions of interest (ROI) were applied and the termination criteria were fractional anisotropy > or =0.3, angle< or =70degrees. RESULTS: The patterns and distribution of abnormal DTI-based corticospinal tractographic findings were interruption(10 cases, 76.9%), reduction of fiber volume (8 cases, 61.5%), agenesis of corticospinal tract (3 cases, 23.1%), transcallosal fiber (2 cases, 15.4%) and, aberrant corticospinal tracts (4 cases, 30.8%). Abnormal DTI-based corticospinal tractographic findings were in accordance with the clinical findings of cerebral palsy in 84.6% of the enrolled patients. CONCLUSION: Our results suggest that DTI-FT would be a useful modality in the assessment of the corticospinal tract abnormalities in children with hemiplegic cerebral palsy.

Alternating Hemiplegia of Childhood in Korea / 대한소아신경학회지

PURPOSE: Alternating hemiplegia of childhood(AHC) is a rare neurologic syndrome of unknown pathophysiology. The typical clinical features are characterized by recurrent hemiplegic attacks shifting from one side to the other, beginning before 18 months, dystonia, strabismus, nystagmus, epilepsy, and immediate resolution of all symptoms during sleep. This study is to access clinical features and outcome of treatment of AHC in Korea. METHODS: We sent standardized questionnaires by e-mail to 118 council members of the Korean Society of Child Neurology. We received it through e-mail from 7 centers and analyzed clinical characteristics, treatment, and outcomes. RESULTS: We collected 11 AHC patients(male 6, female 5). The mean age was 6.13+/-5.23 years. The mean age of onset of hemiplegia was 9.80+/-6.67 months. The mean frequency of episode was 10.73+/-11.44 per month and mean duration was 3.10+/-2.50 days. The manifestations were hemiplegia in 100%, dystonia in 81.8%, abnormal eye movements in 81.8%, developmental delay in 81.8%, seizure in 72.7%, autonomic phenomena in 54.5%, ataxia 45.5%, and episodes of quadriparesis in 45.5%. The onset age of seizure was 3-84 months. Psychomotor retardation was more severe in patients with seizures than in those without seizures. Magnetic resonance imaging of brain and cerebral angiography were normal in all patients. Interictal SPECT shows hypoperfusion in the frontal in 2 of 5 patients. Nine patients received flunarizine and antiepileptic drugs but two patients received antiepileptic drugs only. A favorable response was reported in 45.5%, which included excellent response to the combination therapy of flunarizine and high dose topiramate. CONCLUSIONS: Patients with seizures faired the poorer psychomotor retardation. The combination of flunarizine and high dose topiramate was very effective in AHC.

A Case of PEHO Syndrome / 대한소아신경학회지

Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy(PEHO) syndrome was first described in 1991 by Salonen. It is a rare, autosomal recessive, and severe neurodegenerative disease with onset in early infancy and guarded prognosis. The distinct clinical criteria for the PEHO syndrome are infantile hypotonia, convulsion, early arrest of mental development, poor or absent visual fixation with optic atrophy by 2 years of age and progressive brain atrophy, particularly of the cerebellum and the brainstem. Most of reported patients with PEHO syndrome are Finnish, and only very few patients in other countries including Japan, Canada and Netherlands have been described. I report a Korean girl with the distinct clinical features of PEHO syndrome and this child is the first Korean patient reported.

Comparison of Epileptic Seizures between Preterm and Term-born Epileptic Children with Periventricular Leukomalacia / 소아과

PURPOSE: This study compares the first epileptic seizures between preterm and term-born children with periventricular leukomalacia and epilepsy. METHODS: From 108 cases having lesions of high signal intensity around the ventricles in T2 weighted imaging of a brain magnetic resonance study, we selected 37 cases that showed epileptic seizures two times or more and divided them into the group of preterm-born (27 cases) and term- born children (10 cases). A retrospective study was made by comparing the two groups with regard to age, type of the first epileptic seizures, EEG findings and responsiveness to anticonvulsants. RESULTS: The age of the first epileptic seizure was 22.2+/-18.3 months in the preterm-born group and 26.9+/-21.1 months in the term-born group (P=0.505). As for the first epileptic seizure, 11 out of the 27 cases in the preterm-born group had infantile spasms. Out of the 10 cases in the term-born group, 7 had complex partial seizures. In the preterm group, hypsarrhythmias were found in 11 cases, focal epileptiform discharges in 6 cases. In term-born group, focal epileptiform discharges were found in 5 cases but no epileptiform discharge was found in 3 cases. Intractable epilepsies were diagnosed in 6 cases and all of them belonged to the preterm-born group. CONCLUSION: More severe epilepsies such as infantile spasm and intractable epilepsies seem to be more common in preterm-born epileptic children with PVL as well as more severely abnormal EEG finding compared to term-born epileptic children.

Headache

No abstract available.

Genetic Neurological Disorders in Childhood

No abstract available.

The Relationship between Underlying Diseases and the Patterns of Abnormal MR Image Findings in Childhood White Matter Diseases / 대한소아신경학회지

PURPOSE: In children disorders affecting brain white matter are not uncommon and MRI has high sensitivity to detect white matter lesions. We performed this study to find out the distribution of underlying diseases that show abnormal white matter image findings on brain MRI and the relationship between the underlying diseases and some particular patterns of MR imaging abnormalities. METHODS: Out of 1477 pediatric patients who visited the department of pediatrics of Yeungnam University Medical Hospital and took brain MRIs from January 1995 to June 2001, ninety five patients with white matter abnormalities on T2 MR images were evaluated retrospectively. This study excluded the patients with brain tumor, brain abscess, trauma, secondary white matter abnormalities due to hydrocephalus, cerebral infarct, and those whose main lesions were gray matter. RESULTS: The most common underlying disease was cerebral palsy(50 cases, 52.6%), followed by acute disseminated encephalitis(10 cases, 10.5%), intrauterine CMV infections (9 cases, 9.5%), inherited neurometabolic disorders(7 cases, 7.4%). In 12 patients(12.6%) with some neurological problems such as afebrile seizures and developmental delay, no underlying disease were found. The most common pattern of abnormal image findings was periventricular white matter lesions(45.3%) strongly related with cerebral palsy. The pattern of peritrigonal deep white matter lesions was found in 19 patients(20.0%), in 10 patients of whom no underlying diseases were found. The pattern of multiple round margined scattered lesions was found in all the 10 patients(10.5%) of acute disseminated encephalitis. CONCLUSION: There are diverse underlying diseases showing abnormal white matter image findings on MR in pediatric patients and particular patterns of abnormal image findings may serve to make a specific diagnosis possible. But not in a small group of children no diagnosis was established. Further studies regarding the underlying diseases affecting white matter lesions and more detailed knowledge of abnormal MR image findings are needed.

The Effect of Imipramine and Desmopressin for the Treatment of Enuresis in Attention Deficit Hyperactivity Disorder Children

PURPOSE: Attention Deficit Hyperactivity Disorder(ADHD) is one of the most common childhood psychiatric diseases and nocturnal enuresis is also a common involuntary disorder, whose symptoms can appear more than 2-3 times per month even after the age of 5. The incidence of nocturnal enuresis is believed to be increasing in ADHD patients. This study investigates the effectiveness of desmopressin and imipramine in the treatment of nocturnal enuresis as well as on ADHD. METHODS: ADHD children of over five years of age displaying nocturnal enuresis were divided into three groups : Group 1, the placebo group; Group 2, the imipramine group; and Group 3, the desmopressin group. They were given their respective treatment agents for 12 weeks and were asked to record their daily urination routine. Their progress was observed two and four weeks after treatment and psychiatric evaluation was performed two weeks after treatment. RESULTS: One week after the start of treatment, the frequency of enuresis in group 3 dropped markedly, showing a much faster treatment response than group 2. Although group 1 showed no significant change in the ADHD index after treatment, groups 2 and 3 showed better ADHD conditions along with enuresis. CONCLUSION: The ADHD scale improved in the two groups whose nocturnal enuresis was successfully treated with imipramine and desmopressin in our study. This may provide evidence for the possibility of one common factor in the cause of ADHD and nocturnal enuresis. More studies on this possibility are required.

Evaluation of the Severity in Patients with Bronchial Asthma Using FEF25-75%

PURPOSE: Spirometry, because of its simplicity and reproducibility, has been used to evaluate pulmonary function in childhood asthma. We investigated whether FEF25-75% is the most sensitive parameter among other spirometric parameters in the diagnosis of pulmonary function and airway obstruction. METHODS: This study included 32 children with asthma. We recorded values of spirometry(FVC, FEV1, PEFR, and FEF25-75%) before and after symptomatic improvement and compared them. The values were recorded as a percent of the predicted normal value. RESULTS: The FEV1 value before and after symptomatic improvement was 71.7+/-15.6% and 83.8+/-12.8%, respectively; PEFR was 75.4+/-18.3% and 85.0+/-16.0%; and FEF25-75% was 60.8+/-17.9% and 81.5+/-16.3%. According to symptomatic improvement, FEF25-75% improved more than the other spirometric parameters. Of the 40 cases of spitometric measurement before symptomatic improvement, abnormal FEV1, PEFR, FEF25-75% values were observed in 25, 17 and 28 cases, respectively. There was statistically significant difference between FEF25-75% and PEFR(P=0.005), but not between FEF25-75% and FEV1(P=0.091). After symptomatic improvement, abnormal FEV1, PEFR, and FEF25-75% values were observed in 14, 12 and 9 cases, respectively. There was statistically significant difference between FEF25-75% and FEV1(P=0.044) but not between FEF25-75% and PEFR (P=0.097). The cases of normalized values with symptomatic improvement, 11 in FEV1, 7 in PEFR and 19 in FEF25-75%. CONCLUSION: FEF25-75% showed the most improvement rate before and after symptomatic improvement. FEF25-75% was helpful in the assessment of treatment effectiveness and reversible bronchial responsiveness. However, this study did not suggest that FEF25-75% is the most sensitive measurement of airway obstruction.

Overview of Epileptogenisis / 대한소아신경학회지

No abstract available.

A Case of Krabbe Disease / 대한소아신경학회지

Krabbe disease is an autosomal recessive neurodegenerative disorder that affects both the central and peripheral nerve system due to an enzymatic defect of galactocerebroside bata-galactosidase. The patient had typical clinical features of Krabbe disease, with irritability, hypertonicity, failure to thrive, and opisthotonic posturing. A brain MRI demonstrates profound white matter demyelination. The diagnosis of Krabbe disease is suspected on the basis of clinical pictures and confirmed by finding markedly reduced galactocerebroside bata-galactosidase activity in leukocyte or cultured fibroblast. Here we present the first reported case of Krabbe disease in Korea confirmed by decreased activity of galactocerebroside bata-galactosidase enzyme in leukocyte.

Action Mechanisms of Anticonvulsants / 대한소아신경학회지

No abstract available.

Health Assessment of Handicapped Chaildren in an Asylum

PURPOSE: To evaluate the health status of institutionalized handicapped children, a general health assessment was done in an asylum in Taegu in May 1998. METHODS: Body measurements, including height, weight, head circumference, skin fold thickness and mid-arm circumference, were done with physical examination on 53 children. Blood tests and chest radiography were done, and fat impedance was measured. RESULTS: Of the 53 children, height, weight and head circumference were below the third percentile in 37(69.8%), 35(66%) and 28(52.8%) children, respectively. Body mass index, skin fold thickness and mid-arm circumference were below the third percentiles in 77.3, 50.9% and 56.6% of the children, respectively. Although serum protein levels were within normal range, anemia was detected in 31 children(58.5%). Two children showed interstitial pneumonia on chest radiograms. And HBsAg was detected in three but their liver enzymes were within normal limits. CONCLUSION: Due to the poor nutritional status of handicapped children in the asylum, the prevalence of anemia was high. Evaluation of the nutritional status and hemoglobin measurernent must be included in the routine health assessment of these children.

Serum Leptin Levels in Children with Type I Diabetes Mellitus

PURPOSE: Leptin is a highly hydrophobic 16-kDa protein encoded by the ob gene which is expre- ssed in adipocytes and participates in the regulation of food intake and energy expenditure. The aims of the present study were to examine the comparison of leptin levels between type I diabetes mellitus (DM) patients who received exogenous insulin therapy and healthy children, and to find out correlating factors with leptin level in type I DM. METHODS: The study was conducted on 34 patients with type I DM and 29 healthy children. Body weight, height, leptin level and body fat percent were measured in both groups. The glycosylated hemoglobin value, C-peptide, insulin level and serum cholesterol were also measured in type I DM. RESULTS: There was no statistically significant difference in mean age, sex distribution and body mass index between the experimental groups, but body fat percent in type I DM was significantly lower than that of healthy children. The mean leptin levels in male (9.4+/-6.3ng/mL) and female (17.8+/-10.2ng/mL) type I DM were significantly higher than in male(6.3+/-5.7ng/mL) and female (10.2+/-0.9ng/mL) healthy children (male : P=0.045, female : P=0.005). Serum leptin levels were strongly correlated with the body fat percent (Pearson coefficient) (r=0.712). CONCLUSION: Serum leptin level in type I DM strongly correlated with the body fat percent. Despite lowered body fat percent in type I DM, the leptin level was significantly higher than values in healthy children. The mean leptin level in type I DM were significantly higher than value in healthy children, but body fat percent of type I DM was lower than value in healthy children who were adjusted for sex, age and body mass index.

Effects of Repeated Immobilization Stress on the Renal Peripheral Benzodiazepine Receptor in Rats

Primary small cell carcinoma of the salivary gland is a rare neoplasm that accounts for approximately 1.8% of all primary major salivary gland malignancies. Because of its rarity, it is difficult to diagnose small cell carcinoma of the parotid gland by fine needle aspiration cytology(FNAC). We experienced a case of primary small cell carcinoma of the parotid gland in a 72-year-old woman who presented with two palpable masses of the left infraauricular and ocular regions of two to three month's duration, respectively. Aspirate smears from the left infraauricular area were highly cellular on necrotic and lymphocytic background and showed individually dispersed cells or three-dimensional clusters of small cells. The tumor cells were round to oval with a very high nucleocytoplasmic ratio. Nuclei were about two times the size of lymphocytes and had uniformly dispersed but hyperchromatic to pyknotic chromatin. Nucleoli were occasionally visible but were generally inconspicuous. Numerous mitotic figures were detected. The clusters of these small tumor cells exhibited angular nuclear molding, irregular nuclear outlines, and occasionally rosette like arrangement. The tumor was confirmed by histology and immunohistochemistry.

Follow-up Study on Peripheral Neuropathy in Insulin-dependent Diabetes Mellitus in Childhood / 대한소아신경학회지

PURPOSE: Peripheral neuropathy is the most common chronic complication of insulin- dependent diabetes mellitus, which is one of the most frequent endocrine metabolic disorders in children. However, there are very few long-term follow-up studies on the condition in publication and none in Korean literature. In order to find out the correlation between the severity of peripheral neuropathy and glycosylated hemoglobin level, an indicator of diabetes control, a follow-up study was conducted on cases of insulin- dependent diabetes mellitus. METHODS: The study was conducted on 37 patients with insulin-dependent diabetes mellitus (12 males, 25 females, aged 7-21 years) who were diagnosed and were being treated in general hospitals in the Taegu-Kyungpook area from 1994 to 1998. They were also subject to nerve conduction velocity test at least two times with an interval of one or more years. Nerve conduction velocity was measured by using type MEM-3102 apparatus of Nihon Kohden (on motor and sensory component of left ulnar nerve and median nerve, motor component of left and right peroneal, posterior tibial nerve, sensory component of left and right sural nerve) and medical history was taken, neurological examination was done and glycosylated hemoglobin levels were measured with each test. RESULTS: The total number of nerve conduction velocity tests was 89 and the results were divided into 5 groups [normal : 14 cases (15.7%); borderline: 35 cases (39.3%); mononeuropathy : 2 cases (2.2%); mild polyneuropathy : 22 cases (24.7%); severe polyneuropathy : 16 cases (18.0%)] by the number and distributions of nerves that were found abnormal. Ten of the 26 cases, belonging to the normal or borderline group in the first test, showed signs of peripheral neuropathy in later tests, and of the 11 cases with peripheral neuropathy in the first test, none improved to be included in the normal or borderline group in the follow-up study. The mean glycosylated hemoglobin level was 9.2+/-2.5% in normal group, 11.9+/-3.2% in borderline group, 13.0+/-0.3% in mononeuropathy group, 12.0+/-3.7% in mild polyneuropathy group and 14.8+/-4.0% in severe polyneuropathy group, and glycosylated hemoglobin level rose with the severity of the neuropathy (r=0.372, p< 0.01). In the 9 cases that had shown improvement of peripheral neuropathy, glycosylated hemoglobin levels decreased significantly by an average of 3.5+/-1.3% with each level of improvement (t-test, p<0.05), but in 20 cases with deteriorating nerve conduction velocity, there was no significant correlation with increase in glycosylated hemoglobin levels. CONCLUSION: Peripheral neuropathy complicating insulin-dependent diabetes mellitus can occur during diabetes treatment and does not improve easily once it occurs. In this study, the glycosylated hemoglobin level was shown to have a high degree of correlation with the occurrence and progression of neuropathy, which means that control of hyperglycemia is difficult and also that stricter control of blood glucose during early stages of diabetes is necessary to prevent neuropathy. Follow-up studies with bigger samples and longer time will be needed for a more detailed look.

Medical Treatment of Infantile Hypertrophic Pyloric Stenosis Using Intravenous Atropine Sulfate

Infantile hypertrophic pyloric stenosis(IHPS) is one of the most common causes of nonbilious vomiting in early infancy, and is caused by hypertrophied pyloric muscle but its exact etiology and pathogenesis are still unknown. Fredet-Ramstedt pyloromyotomy has been accepted as the treatment of choice for IHPS. Atropine is a cholinergic blocking agent with potent antimuscarinic activity that decreases peristaltic contractions by relaxing smooth muscles. We treated a case of IHPS in a 33-day-old male infant by administering atropine sulfate intravenously. One day after atropine sulfate administration, he did not vomit any more. Ultrasonograms of the pyloric canal which were done on eight days and three weeks after atropine treatment revealed no limitation in the passage of gastric content, and no changes in the muscle thickness and length of the pyloric canal. Thereafter, he did not show up at follow ups, we received his mother's answer through phone that he did not suffer from vomiting and he was growing well at 5 months of age.

Follow up of Infants with Congenital Hypothyroidism Who were Detected by Newborn Screening Test

PURPOSE: Developmental delay in congenital hypothyroidism can be prevented by early detection and treatment. We conducted a follow-up study to assess the growth and development of infants with congenital hypothyroidism who were detected by newborn screening test and received thyroid hormone therapy. METHODS: Nineteen hypothyroid infants were detected by newborn screening test from April 1995 to May 1997. Measurements of free T4 and TSH levels by radioimmunoassay were used as a screening test, and serum T4, T3, free T4, TSH, TBG levels were measured and radioisotope scan of thyroid gland were done for final diagnosis. Height and weight measurements, thyroid function test, and Bayley Scales of Infant Development(BSID II) were done during follow up. RESULTS: Male to female ratio was 2.2 : 1. The etiologies of congenital hypothyroidism were dyshormonogenesis(7 cases), hypoplasia(6 cases), ectopic gland(5 cases), and aplasia(1 case). All but one infant revealed normal physical growth. Mild developmental delay was detected in five cases by BSID-II about ten months after thyroxine replacement. Two cases revealed mild delay in Mental Developmental Index and 2 cases showed mild delay in Psychomotor Developmental Index, only one case was delayed in both indices. There was a negative correlation between PDI and age of starting treatment(r=-0.505, P<0.05), and a positive correlation between free T4 at the time of diagnosis and MDI(r=0.472, P<0.05). CONCLUSION: Early diagnosis and treatment were important for normal growth and development of hypothyroid infants, and Bayley developmental scale can be used for long term follow up.