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1.
Adv Exp Med Biol ; 1405: 421-455, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37452948

RESUMEN

Primary central nervous system germ cell tumors (CNS GCTs) are part of the GCTs in children and adults. This tumor entity presents with geographic variation, age, and sex predilection. There are two age peaks of incidence distribution at the first few months of life and in adolescence. CNS GCTs are heterogeneous in histopathological subtypes, locations, and tumor marker (AFP, ß-hCG) secretions. In the WHO CNS tumor classification, GCTS are classified as germinoma and nongerminomatous GCT (NGGCT) with different subtypes (including teratoma). Excluding mature teratoma, the remaining NGGCTs are malignant (NGMGCT). In teratoma, growing teratoma syndrome and teratoma with somatic-type malignancy should be highlighted. The common intracranial locations are pineal region, neurohypophysis (NH), bifocal pineal-NH, basal ganglia, and cerebral ventricle. Above 50% of intracranial GCTs (IGCTs) present obstructive hydrocephalus. Spinal tumors are rare. Age, locations, hydrocephalus, and serum/CSF titer of ß-hCG correlate with clinical manifestations. Delayed diagnosis is common in tumors arising in neurohypophysis, bifocal, and basal ganglia resulting in the increasing of physical dysfunction and hormonal deficits. Staging work-up includes CSF cytology for tumor cells and contrast-enhanced MRI of brain and spine for macroscopic metastasis before treatment commences. The therapeutic approach of CNS GCTs integrates locations, histopathology, staging, tumor marker level, and therapeutic classification. Treatment strategies include surgical biopsy/excision, chemotherapy, radiotherapy (single or combination). Secreting tumors with consistent imaging may not require histopathological diagnosis. Primary germinomas are highly radiosensitive and the therapeutic aim is to maintain high survival rate using optimal radiotherapy regimen with/without chemotherapy combination. Primary NGNGCTs are less radiosensitive. The therapeutic aim is to increase survival utilizing more intensive chemotherapy and radiotherapy. The negative prognostic factors are residue disease at the end of treatment and serum or CSF AFP level >1000 ng/mL at diagnosis. In refractory or recurrent NMGGCTs, besides high-dose chemotherapy, new therapy is necessary. Molecular profiling and analysis help for translational research. Survivors of pediatric brain tumors frequently experience cancer-related cognitive dysfunction, physical disability, pituitary hormone deficiency, and other CNS complications after cranial radiotherapy. Continuous surveillance and assessment may lead to improvements in treatment protocols, transdisciplinary interventions, after-treatment rehabilitation, and quality of life.


Asunto(s)
Neoplasias Encefálicas , Germinoma , Neoplasias de Células Germinales y Embrionarias , Neoplasias de la Médula Espinal , Neoplasias de la Columna Vertebral , Teratoma , Niño , Adulto , Adolescente , Humanos , alfa-Fetoproteínas/metabolismo , Calidad de Vida , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Neoplasias de Células Germinales y Embrionarias/terapia , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/patología , Germinoma/diagnóstico , Germinoma/patología , Germinoma/terapia , Teratoma/diagnóstico , Teratoma/terapia , Encéfalo/metabolismo , Estudios Retrospectivos
2.
Neuropediatrics ; 48(3): 190-193, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-28335042

RESUMEN

Acute sensory neuronopathy (SNN) is a rapidly developing peripheral nervous system disease that primarily affects sensory neurons in the dorsal root ganglion or trigeminal ganglion, leading to the impairment of sensory axons. SNN is notably uncommon in childhood; only three cases of childhood or adolescent SNN have been reported to date. Moreover, SSN has never been reported in association with enterovirus infection. Here, we report the case of a 3-year-old girl who was initially diagnosed with enterovirus infection based on the presentation of fevers, rashes on all extremities, and ulceration over the posterior pharynx. Nine days later, she presented with ataxic and wide-based gait and dysmetria affecting the extremities, with an absence of sensory nerve action potentials in the upper and lower limbs. The patient was diagnosed with acute SNN based on the criteria developed by Camdessanché et al in 2009. To our knowledge, this is the youngest case of SNN reported to date. In addition, this case reveals that enterovirus infection can be associated with acute SNN in children in rare cases. Accurate diagnosis relies on clinical suspicion, comprehensive knowledge of the patient's history, and careful characterization of abnormal findings in electrodiagnostic studies.


Asunto(s)
Infecciones por Enterovirus/complicaciones , Infecciones por Enterovirus/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Enfermedades del Sistema Nervioso Periférico/etiología , Biomarcadores/metabolismo , Preescolar , Diagnóstico Diferencial , Electromiografía , Infecciones por Enterovirus/fisiopatología , Infecciones por Enterovirus/terapia , Femenino , Humanos , Conducción Nerviosa , Enfermedades del Sistema Nervioso Periférico/fisiopatología
3.
Arch Phys Med Rehabil ; 98(5): 947-956, 2017 05.
Artículo en Inglés | MEDLINE | ID: mdl-28209506

RESUMEN

OBJECTIVE: To compare the effectiveness of local steroid injection plus splinting with that of local steroid injection alone using clinical and electrophysiological parameters in patients with carpal tunnel syndrome (CTS). DESIGN: Randomized controlled study with 12 weeks of follow-up. SETTING: Tertiary care center. PARTICIPANTS: Volunteer sample of patients (N=52) diagnosed with CTS. INTERVENTIONS: Participants were randomly assigned to the steroid injection group (n=26) or the steroid injection-plus-splinting group (n=26). Patients of both groups received ultrasound-guided steroid injection with 1mL of 10mg (10mg/mL) triamcinolone acetonide (Shincort) and 1mL of 2% lidocaine hydrochloride (Xylocaine). Participants in the second group also wore a volar splint in the neutral position while sleeping and also during daytime whenever possible for the 12-week intervention period. MAIN OUTCOME MEASURES: Participants were evaluated before the treatment and at 6 and 12 weeks after the onset of treatment. The primary outcome measure was Boston Carpal Tunnel Questionnaire scores. The secondary outcome measures were as follows: scores on the visual analog scale for pain; electrophysiological parameters, including median nerve distal motor latency, sensory nerve conduction velocity (SNCV), and compound muscle action potential and sensory nerve action potential (SNAP) amplitudes; and patient's subjective impression of improvement. RESULTS: At 12-week follow-up, improvements in symptom severity and functional status scores on the Boston Carpal Tunnel Questionnaire as well as SNCV and SNAP amplitudes were greater in the group that received steroid injection combined with splinting than in the group that received steroid injection alone. The between-group difference was .48 points (95% confidence interval [CI], .09-.88 points; P=.032) in the Symptom Severity Scale score, .37 points (95% CI, .06-.67 points; P=.019) in the Functional Status Scale score, 3.38m/s (95% CI, 0.54-6.22m/s; P=.015) in the SNCV amplitude, and 3.21µV (95% CI, 0.00-6.46µV; P=.025) in the SNAP amplitude. CONCLUSIONS: In people with CTS, steroid injection combined with splinting resulted in modestly greater reduction of symptoms, superior functional recovery, and greater improvement in nerve function at 12-week follow-up as compared with steroid injection alone. However, these small differences are of unclear clinical significance.


Asunto(s)
Analgésicos/uso terapéutico , Síndrome del Túnel Carpiano/terapia , Lidocaína/uso terapéutico , Férulas (Fijadores) , Triamcinolona Acetonida/uso terapéutico , Adulto , Anciano , Analgésicos/administración & dosificación , Femenino , Humanos , Lidocaína/administración & dosificación , Masculino , Nervio Mediano/efectos de los fármacos , Persona de Mediana Edad , Conducción Nerviosa/efectos de los fármacos , Manejo del Dolor , Dimensión del Dolor , Índice de Severidad de la Enfermedad , Método Simple Ciego , Triamcinolona Acetonida/administración & dosificación , Ultrasonografía Intervencional
4.
Childs Nerv Syst ; 30(1): 13-7, 2014 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-23974968

RESUMEN

INTRODUCTION: Currently, there are only a few reported cases of symptomatic or asymptomatic subpial (intramedullary) spinal lipoma, and therefore no guidelines are available to indicate surgery. These lesions are infrequently associated with spina bifida. CASE REPORT: Herein, we provide our experience in the neurosurgical intervention of compressive myeloradiculopathy for encephalocraniocutaneous lipomatosis (ECCL). The patient initially presented with bilateral upper hand paralysis, then regained muscle power after surgery and during 1 year of follow-up. We discuss the neurosurgical indications and intervention, imaging studies, other associated symptoms, and the pathogenesis of ECCL in an infant.


Asunto(s)
Vértebras Cervicales/patología , Oftalmopatías/diagnóstico , Oftalmopatías/cirugía , Lipomatosis/diagnóstico , Lipomatosis/cirugía , Síndromes Neurocutáneos/diagnóstico , Síndromes Neurocutáneos/cirugía , Procedimientos Neuroquirúrgicos/métodos , Femenino , Humanos , Recién Nacido
5.
Childs Nerv Syst ; 30(9): 1559-64, 2014 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24825575

RESUMEN

PURPOSE: The purpose of the study is to determine corticospinal organization using intraoperative neurophysiologic monitoring (IONM) during resective epilepsy surgery for patients with congenital hemiparesis and intractable epilepsy. METHODS: Ten patients, aged 3-17, with intractable epilepsy underwent resective surgery. Transcranial stimulation (TCS) was achieved using a pair of cork screws at Cz and C3/C4, respectively. A 1 × 4 stimulating electrode strip was placed on the presumed motor cortex of the affected hemisphere for direct cortical stimulation (DCS) after craniotomy. Multipulse TCS and DCS train stimulation was delivered, with simultaneous recordings from bilateral abductor pollicis brevis and abductor halluces, to determine the corticospinal projection pattern of the paretic limbs. RESULTS: The above mapping techniques revealed ipsilateral corticospinal projections from the contralesional hemisphere to target muscles in the paretic limbs in three patients, projections from both hemispheres to target muscles in three, and preserved crossed projections from the affected hemisphere in four. Nine patients were seizure free after surgery. Five had unchanged postoperative functional status, and three showed minimally improved use of the paretic hand. Two developed new motor deficits after surgery, which may have been due to a premotor syndrome in one patient, since it completely resolved within 2 weeks. The other experienced increased weakness of the paretic lower limb because a small part of the eloquent cortex was removed for better seizure control. CONCLUSIONS: Using IONM to define the corticospinal projection pattern is a valuable technique that can potentially replace preoperative fMRI and transcranial magnetic stimulation in resective epilepsy surgery, particularly for younger patients.


Asunto(s)
Epilepsia/cirugía , Monitoreo Intraoperatorio , Paresia/complicaciones , Tractos Piramidales/patología , Adolescente , Mapeo Encefálico , Niño , Preescolar , Craneotomía , Electroencefalografía , Epilepsia/complicaciones , Potenciales Evocados Motores/fisiología , Femenino , Lateralidad Funcional , Mano/inervación , Humanos , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Magnética , Masculino , Corteza Motora/fisiopatología , Paresia/congénito , Estimulación Magnética Transcraneal
6.
Childs Nerv Syst ; 30(11): 1885-95, 2014 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-25296550

RESUMEN

BACKGROUND: Focal cortical dysplasia (FCD) is a specific malformation of cortical development harboring intrinsic epileptogenicity, and most of the patients develop drug-resistant epilepsy in early childhood. The detrimental effects of early and frequent seizures on cognitive function in children are significant clinical issues. In this study, we evaluate the effects of early surgical intervention of FCD on epilepsy outcome and cognitive development. METHODS: From 2006 to 2013, 30 children younger than 18 years old underwent resective surgery for FCDs at Taipei Veterans General Hospital. The mean age at surgery was 10.0 years (range 1.7 to 17.6 years). There were 21 boys and 9 girls. In this retrospective clinical study, seizure outcome, cognitive function, and quality of life were evaluated. To evaluate the effects to outcomes on early interventions, the patients were categorized into four groups according to age of seizure onset, duration of seizure before surgery, and severity of cognitive deficits. RESULTS: Eleven of 22 (50 %) patients demonstrated developmental delay preoperatively. The Engel seizure outcome achievements were class I in 21 (70 %), class II in 2 (7 %), class III in 6 (20 %), and class IV in 1 (3 %) patients. The locations of FCDs resected were in the frontal lobe in 18 cases, temporal lobe in 7, parietal lobe in 2, and in bilobes including frontoparietal lobe in 2 and parieto-occipital lobes in 1. Eight cases that had FCDs involved in the rolandic cortex presented hemiparesis before surgical resection. Motor function in four of them improved after operation. The histopathological types of FCDs were type Ia in 1, type Ib in 7, type IIa in 7, type IIb in 12, and type III in 3 patients. FCDs were completely resected in 20 patients. Eighteen (90 %) of them were seizure free (p < 0.001) with three patients that received more than one surgery to accomplish complete resection. The patients who had early seizure onset, no significant cognitive function deficit, and early surgical intervention with complete resection in less than 2 years of seizure duration showed best outcomes on seizure control, cognitive function, and quality of life. CONCLUSION: Delay in cognitive development and poor quality of life is common in children treated for FCDs. Early surgical intervention and complete resection of the lesion help for a better seizure control, cognitive function development, and quality of life. FCDs involved eloquent cortex may not prohibit complete resection for better outcomes.


Asunto(s)
Trastornos del Conocimiento/rehabilitación , Trastornos del Conocimiento/cirugía , Intervención Educativa Precoz , Epilepsia/cirugía , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Adolescente , Niño , Preescolar , Trastornos del Conocimiento/etiología , Trastornos del Conocimiento/psicología , Discapacidades del Desarrollo/cirugía , Epilepsia/etiología , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Malformaciones del Desarrollo Cortical/complicaciones , Malformaciones del Desarrollo Cortical/psicología , Pruebas Neuropsicológicas , Calidad de Vida , Estudios Retrospectivos
7.
Can J Anaesth ; 60(9): 902-6, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23807495

RESUMEN

PURPOSE: Sciatic neuropathy is a rare but serious complication of cardiac surgery. Neuropathic pain following nerve injury can be severely debilitating and largely resistant to treatment. We present a case of this complication where ultrasound-guided perineural steroid injection at the site of the sciatic nerve injury provided excellent pain relief and facilitated subsequent rehabilitation. CLINICAL FEATURES: A 17-yr-old boy developed bilateral sciatic neuropathy after a nine-hour cardiac surgical procedure in the supine position, resulting in debilitating dysesthesia refractory to neuropathic pain therapies and leading to severe functional limitation. With magnetic resonance imaging of the lower extremities, the location of the lesion was determined to be from the level of the superior gemellus to the level of the quadratus femoris. An ultrasound-guided injection of triamcinolone 20 mg and lidocaine 40 mg around both sciatic nerves at the level of the lesion was administered two months after the surgery, and the pain score (rated on a scale 0-10) at rest decreased from 9-10 to 1 two weeks after the injection. CONCLUSIONS: There are a limited number of reports in the literature on sciatic nerve injuries associated with cardiac surgery. This case illustrates the efficacy of ultrasound-guided steroid injection around sciatic nerves at the level of superior gemellus in treating our patient's neuropathic pain.


Asunto(s)
Lidocaína/administración & dosificación , Nervio Ciático/lesiones , Neuropatía Ciática/tratamiento farmacológico , Triamcinolona/administración & dosificación , Adolescente , Anestésicos Locales/administración & dosificación , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Procedimientos Quirúrgicos Cardíacos/métodos , Glucocorticoides/administración & dosificación , Humanos , Imagen por Resonancia Magnética , Masculino , Bloqueo Nervioso/métodos , Neuropatía Ciática/etiología , Ultrasonografía Intervencional/métodos
8.
Childs Nerv Syst ; 29(9): 1671-9, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24013337

RESUMEN

PURPOSE: This study was designed to investigate health-related quality of life (HRQL) in children and adolescents with spinal dysraphism and to determine the differences between the myelomeningocele (MMC) and lipomyelomeningocele (LMMC) groups. METHODS: Thirty-two patients (mean age, 13.2 years) with MMC and 28 patients (mean age, 11.3 years) with LMMC participated in this study. HRQL was measured using the Child Health Questionnaire-Parent Form 50 (CHQ-PF50). The CHQ-PF50 scores for the study participants were compared with those of a reference sample of healthy Taiwanese children. Outcomes for children with MMC were compared with those for children with LMMC. We also explored the relationships of the lesion level and associated medical problems with HRQL in these subjects. RESULTS: Patients with MMC had significantly lower scores on the physical and psychosocial aspects of CHQ-PF50 than did healthy children. Patients with LMMC had lower scores on some domains but had comparable psychosocial summary scores. Significant differences were noted between the MMC and LMMC groups for all subscales and both summary scores. Children with a higher lesion level had poor physical health (r = -0.52, P < 0.001) but not poor psychosocial health. The number of associated medical problems, indicating their severity, was related to both physical (r = -0.77, P < 0.001) and psychosocial health (r = -0.57, P < 0.001). CONCLUSIONS: Children and adolescents with MMC have reduced HRQL compared with those with LMMC. Children and adolescents with more associated medical problems have the greatest risk of diminished physical and psychosocial well-being.


Asunto(s)
Meningomielocele/complicaciones , Calidad de Vida , Disrafia Espinal/complicaciones , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Meningomielocele/psicología , Disrafia Espinal/psicología , Encuestas y Cuestionarios , Taiwán
9.
Childs Nerv Syst ; 29(2): 347-9, 2013 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-23129445

RESUMEN

INTRODUCTION: Refilling intrathecal baclofen (ITB) pumps can be difficult because many patients gain excessive weight after implantation due to their reduced expenditure of energy on muscle spasticity. METHODS: We report a case of a 12-year-old girl with spastic quadriplegia who gained 20 lbs after pump implantation. It was necessary to identify the access port of her pump by ultrasonography during drug refilling so as to avoid multiple needle punctures. RESULTS: The access port of the pump was readily visible by ultrasonography and stood out from other parts of the pump. CONCLUSION: Localisation of the access ports of ITB pumps by ultrasonography proved to be a feasible and easy technique for refilling the drug reservoir in patients with excessive weight gain and abundant subcutaneous fat after ITB therapy.


Asunto(s)
Baclofeno/administración & dosificación , Bombas de Infusión Implantables , Infusión Espinal/métodos , Ultrasonografía Intervencional/métodos , Niño , Femenino , Humanos , Infusión Espinal/instrumentación , Cuadriplejía/diagnóstico por imagen , Cuadriplejía/tratamiento farmacológico
10.
Childs Nerv Syst ; 28(1): 159-63, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-21956785

RESUMEN

INTRODUCTION: Sciatic neuropathy is a rare but serious complication following surgery performed in the sitting position. METHODS: We report a case of a 12-year-old boy who developed bilateral sciatic neuropathy after a 9-h-long craniotomy while in the sitting position. RESULTS: Magnetic resonance imaging for the lower extremities revealed bilateral swelling of the sciatic nerves near the ischial tuberosities. CONCLUSION: A heightened awareness of sciatic nerve injury during prolonged surgery in the sitting position may make early diagnosis possible and allow prevention of further neurologic injury.


Asunto(s)
Craneotomía/efectos adversos , Craneotomía/métodos , Síndromes de Compresión Nerviosa/etiología , Complicaciones Posoperatorias/etiología , Neuropatía Ciática/etiología , Adolescente , Neoplasias Encefálicas/cirugía , Electromiografía , Humanos , Masculino , Síndromes de Compresión Nerviosa/patología , Glándula Pineal/cirugía , Pinealoma/cirugía , Complicaciones Posoperatorias/patología , Postura , Nervio Ciático/lesiones , Nervio Ciático/patología , Neuropatía Ciática/patología
11.
PLoS One ; 16(8): e0253338, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34351928

RESUMEN

OBJECTIVES: Many studies have investigated the surgical outcome and predictive factors of acoustic neuroma using different approaches. The present study focused on large tumors due to the greater likelihood of internal acoustic meatus involvement and the greater application of surgical intervention than radiosurgery. There have been no previous reports on outcomes of internal acoustic meatus tumor removal. We investigated the impact of the extent of internal acoustic meatus tumor removal using a translabyrinthine approach for large acoustic neuroma surgery and predictive factors of tumor control. METHODS: This retrospective study reviewed 104 patients with large cerebellopontine angle tumor >3 cm treated by translabyrinthine approach microsurgery. Predictive factors of postoperative facial palsy, tumor control, and extent of internal acoustic meatus tumor removal were assessed. RESULTS: The mean tumor size was 38.95 ± 6.83 mm. Postoperative facial function showed 76.9% acceptable function (House-Brackmann grade 1 or 2) six months after surgery. The extent of internal acoustic meatus tumor removal was a statistically significant predictor factor of poor postoperative facial function. Younger age, larger tumor size needing radiosurgery, and more extensive removal of tumor were associated with better tumor control. CONCLUSION: More extensive internal acoustic meatus tumor removal was associated with poor postoperative facial function and better tumor control.


Asunto(s)
Cara/fisiopatología , Neuroma Acústico , Recuperación de la Función , Adulto , Factores de Edad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neuroma Acústico/fisiopatología , Neuroma Acústico/cirugía , Estudios Retrospectivos
12.
Tzu Chi Med J ; 33(4): 395-398, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34760637

RESUMEN

OBJECTIVES: Intraoperative neurophysiological monitoring (IONM) has long been regarded as the "gold standard" when resecting a supratentorial glioma, as it facilitates the goals of maximal tumor resection and preservation of sensorimotor function. The purpose of the present study was to evaluate the ability of motor evoked potentials (MEPs) monitoring or subcortical mapping (SCM), alone or in combination, to predict postoperative functional outcomes in glioma surgery. MATERIALS AND METHODS: We retrospectively reviewed patients with supratentorial glioma that underwent craniotomy for tumor removal with IONM. Statistical analyses were used to evaluate whether the following criteria correlated with postoperative functional outcomes: Reduced amplitude (>50% reduction) or disappearance of MEPs (criterion 1), SCM with a stimulation intensity threshold less than 3 mA (criterion 2), the presence of both two phenomena (criterion 3), or either one of the two phenomena (criterion 4). RESULTS: Ninety-two patients were included in this study, of whom 15 sustained new postoperative deficits, 4 experienced improved functional status, and 73 were unchanged. Postoperative functional status correlated significantly with all four criteria, and especially with criterion 3 (r = 0.647, P = 0.000). Sensitivity of IONM was better if using criteria 2 and 4, but specificity was better if using criteria 1 and 3. Criterion 3 had the most favorable overall results. CONCLUSION: Using statistical methodology, our study indicates that concomitant interpretation of MEPs and SCM is the most accurate predictor of functional outcomes following supratentorial glioma surgery. However, accurate interpretations of the monitoring results by experienced neurophysiologists are essential.

13.
J Chin Med Assoc ; 82(2): 161-165, 2019 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-30839509

RESUMEN

BACKGROUND: The health-related quality of life (HRQoL) was affected in children and adolescents with scoliosis. However, there was lack of study to compare the HRQoL among patients with different types of scoliosis. We aimed to investigate whether the HRQoL differs among patients with idiopathic, congenital, neuromuscular, and syndromic scoliosis. METHODS: Children and adolescents with scoliosis were recruited from a single tertiary hospital. The HRQoL, as assessed by the child health questionnaire 50-item parent form, was compared with a reference health sample group using the effect size (ES). Intergroup differences related to scoliosis subtype and severity were explored. RESULTS: A total of 67 participants with scoliosis (24 idiopathic, 15 congenital, 15 neuromuscular, and 13 syndromic) were analyzed. The HRQoL in patients with neuromuscular scoliosis was affected the most, in both physical (ES range: 0.97-2.4) and psychosocial domains (ES range: 0.92-2.58). To a lesser extent, the physical (ES range: 0.99-1.13) and psychosocial (ES range: 0.8-1.18) domains were also affected in patients with syndromic scoliosis. The domains of family activities (ES = 1.1), role/social-emotional/behavioral (ES = 0.99), general health perception (ES = 0.94), and self-esteem (ES = 0.87) were affected in patients with idiopathic scoliosis. In contrast, only the general health perception domain (ES = 1.27) was affected in patients with congenital scoliosis. Scoliosis severity correlated with scores in the physical domains and some psychosocial domains, while treatment type correlated with scores in the physical domains only. Scoliosis subtype and severity both affected the physical and psychosocial domains, with a stronger impact for subtype. CONCLUSION: Differences in the HRQoL exist among scoliosis subtypes, with neuromuscular scoliosis being most affected. Although the scoliosis subtype and severity both affect the HRQoL, the subtype is more influential than severity.


Asunto(s)
Calidad de Vida , Escoliosis/psicología , Adolescente , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Masculino , Escoliosis/clasificación
14.
J Chin Med Assoc ; 82(6): 519-523, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30946116

RESUMEN

BACKGROUND: In the present study, we investigated whether blink synkinesis monitoring during microvascular decompression (MVD) is effective for predicting long-term outcomes in patients with hemifacial spasm (HFS). METHODS: This retrospective study included 69 patients who had undergone MVD for HFS at a tertiary hospital. All patients underwent intraoperative monitoring of blink synkinesis, lateral spread responses (LSRs), and facial nerve motor-evoked potentials (FNMEPs). Baseline signals were compared to those obtained following decompression with Teflon, and postoperative outcomes were recorded. RESULTS: A total of 65 patients were observed with complete relief of symptoms after 1 year after MVD, while 61 patients were observed with initial disappearance of blink synkinesis, 57 patients were observed with initial elimination of the LSR, and 45 patients with initial decreases in FNMEP amplitude (>50%). The highest sensitivity and accuracy values were observed for blink synkinesis. Chi-square tests comparing the sensitivity of the three methods revealed that FNMEP monitoring was associated with significantly lower sensitivity values than the remaining methods. Combined use of blink synkinesis and LSRs did not significantly increase sensitivity (61/65 vs 62/65) or accuracy (62/69 vs 63/69). CONCLUSION: Our results demonstrate that blink synkinesis monitoring is safe during MVD for HFS. Furthermore, blink synkinesis was associated with the highest sensitivity and predictive values among the three methods evaluated. These findings suggest that blink synkinesis can be regarded as the first choice for intraoperative monitoring during MVD. Concurrent use of blink synkinesis and LSR monitoring may maximize the ability to predict patient prognosis and determine the extent of decompression.


Asunto(s)
Parpadeo/fisiología , Espasmo Hemifacial/cirugía , Cirugía para Descompresión Microvascular/métodos , Monitoreo Intraoperatorio , Sincinesia/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Potenciales Evocados Motores/fisiología , Nervio Facial/fisiología , Femenino , Espasmo Hemifacial/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
15.
J Chin Med Assoc ; 71(11): 571-5, 2008 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-19015055

RESUMEN

BACKGROUND: Long-term piano training might induce some biochemical and structural adaptations in the intrinsic muscles of the hand or change the motor strategy of the nervous system. The main purpose of this study was to analyze whether the intrinsic muscles of the hands of pianists and sedentary controls differ in electromyographic characteristics at different strengths. METHODS: Fifteen college piano students and 15 sedentary controls were asked to sit on an examination bench and perform first dorsal interosseous muscle contractions for 1 minute. The motor unit potentials were recorded during various percentages of maximal voluntary muscle contraction (MVC) by automatic decomposition electromyography. RESULTS: The pianists demonstrated a significantly higher firing rate, shorter duration, and higher amplitude of motor unit potentials during minimal muscle contractions than the sedentary controls. But when comparing all the parameters at other degrees of contractions, the pianists were found to have significantly higher firing rate only at 25% and 50% of MVC, and higher amplitude at maximal contraction than the control group. The amplitude at maximal control contraction was higher in pianists than in controls. CONCLUSION: These results imply that high-frequency and highly efficient muscle fibers are recruited in pianists when minimal muscle contractions are performed, which also indicate that by using smaller motor units, pianists may delicately control their fine motor performance.


Asunto(s)
Electromiografía , Ejercicio Físico , Música , Adolescente , Femenino , Mano/fisiología , Humanos , Masculino , Adulto Joven
16.
J Chin Med Assoc ; 71(10): 502-8, 2008 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-18955184

RESUMEN

BACKGROUND: Spinal dysraphism is a common birth defect that causes different kinds of secondary impairments, including joint deformities, reduced mobility, and bowel/bladder dysfunction. Due to the diversity in terminology, cultural/ethnic differences, and medical policies, prior study results cannot be generalized to all populations. Therefore, we performed this study to define the characteristics of patients in Taiwan with spinal dysraphism. METHODS: Patients diagnosed with a myelomeningocele or lipomyelomeningocele were identified from the database of our spinal dysraphism multidisciplinary clinic. A cross-sectional study was conducted by telephone interview and retrospective chart review. Clinical characteristics, such as neurologic level, orthopedic deformities, assistive device use, and level of ambulation, were collected. Spearman's correlation (r) tests were performed between ambulation or neurologic level and other variables. RESULTS: Seventy-eight subjects were included in the current study. Subjects with myelomeningoceles had more severe neurologic involvement, poorer ambulation outcome, and higher rates of orthopedic deformities, assistive device use, lower hand function, and bowel/bladder dysfunction. The correlation test revealed that the level of ambulation was negatively influenced by a higher neurologic level, a history of shunt placement, and various orthopedic deformities. Neurologic level also had widespread influence on history of shunt placement, orthopedic deformities, assistive device use, the need for additional assistive devices, aggressiveness of assistive devices, and bowel/bladder dysfunction. CONCLUSION: For patients with spinal dysraphism, the neurologic level is the most important prognostic factor for many other clinical characteristics, including ambulation status.


Asunto(s)
Disrafia Espinal/fisiopatología , Adolescente , Adulto , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Meningomielocele/fisiopatología , Pronóstico , Estudios Retrospectivos , Disrafia Espinal/complicaciones , Caminata
17.
J Neurosurg Spine ; 29(4): 456-460, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30004314

RESUMEN

OBJECTIVE: The aim of this study was to investigate the feasibility of using subdural strip electrodes, placed just rostral to the surgical field, to record sensory evoked potentials (SEPs) from the lumbosacral sensory nerves and define the most inferior functional portion of the conus medullaris during detethering surgery for spinal dysraphism and/or tethered cord syndrome (TCS). METHODS: Six patients, 2 boys and 4 girls, aged 0.5 to 16 years, were enrolled in this study. One patient had lipomyelomeningocele-related, 4 had myelomeningocele-related, and 1 had diastematomyelia and lipomyelomeningocele-related TCS. In addition to the routine preparations that are needed for performing functional mapping and monitoring during surgery for spinal dysraphism and TCS, the patients had a 1 × 4 strip of electrodes placed rostral to the surgical field, where it was secured by a surgeon after opening the dura. With the patient under total intravenous anesthesia, the sensory nerves and conus medullaris were stimulated with a concentric bipolar electrode over the surgical field while SEPs were recorded with the strip electrodes to identify any possible sensory roots with remaining function and the most inferior functional portion of the conus medullaris. RESULTS: The SEP amplitudes that were recorded with the subdural strip electrodes ranged from 4 to 400 µV, and the responses to sensory nerve stimulation were frequently much larger than were those to conus stimulation. Use of the SEP recordings for sensory mapping along with the routine mapping and monitoring techniques allowed detethering to be completed such that none of the patients sustained any new functional deficit after surgery. CONCLUSIONS: Recording SEPs from the functional sensory nerves and conus medullaris through subdural strip electrodes proved to be a feasible and valuable tool during detethering surgery in young patients. This approach may help surgeons achieve maximal detethering while preserving important sensory functions, consequently retaining the patient's quality of life.


Asunto(s)
Electrodos , Meningomielocele/cirugía , Defectos del Tubo Neural/cirugía , Médula Espinal/cirugía , Adolescente , Adulto , Cauda Equina/cirugía , Niño , Preescolar , Potenciales Evocados Somatosensoriales/fisiología , Femenino , Humanos , Lactante , Masculino , Procedimientos Neuroquirúrgicos/métodos , Proyectos Piloto
18.
J Electromyogr Kinesiol ; 43: 21-27, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30179730

RESUMEN

Clinicians frequently incorporate unstable sitting devices into training plans for improving proximal postural muscle control; however, the effect of unstable sitting conditions on postural adjustments during dynamic activities has not been fully explored. The aim of this study was to characterize early postural adjustments (EPAs) and anticipatory postural adjustments (APAs) under stable and unstable sitting conditions. Using a cross-sectional laboratory study design, 13 healthy college student volunteers used their dominant hand to reach forward and push a target under stable and unstable sitting conditions; subjects sat on an air-filled rubber cushion for the unstable condition. EPAs and APAs were quantified by recording muscle activation of the trunk and lower extremity muscles using electromyography (EMG). The center of pressure (COP) was measured using a force plate. The resulting EMG integral of the ipsilateral gastrocnemius muscle was larger during the EPA phase and smaller during the APA phase under unstable conditions (p = 0.014 and p = 0.041, respectively). COP amplitude in the anterior-posterior direction, path length, and velocity, was larger during the APA phase (p = 0.035, p = 0.023, and 0.023, respectively). This suggests greater distal muscle activation during EPAs in unstable sitting conditions, specifically in the ipsilateral gastrocnemius muscle. In addition, APAs adjusted by reducing the activity of the ipsilateral gastrocnemius muscle and increasing the anterior-posterior shift in the COP to compensate for the expected additional perturbation due to an unstable surface.


Asunto(s)
Adaptación Fisiológica/fisiología , Anticipación Psicológica/fisiología , Equilibrio Postural/fisiología , Desempeño Psicomotor/fisiología , Sedestación , Adolescente , Adulto , Estudios Transversales , Electromiografía/métodos , Femenino , Voluntarios Sanos , Humanos , Masculino , Músculo Esquelético/fisiología , Postura/fisiología , Presión , Torso/fisiología , Adulto Joven
20.
J Mot Behav ; 39(4): 317-24, 2007 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-17664173

RESUMEN

The authors examined whether the intended task goal of the subsequent action affects the reaching patterns before the intended goal in 17 children with cerebral palsy (CP). The authors hypothesized that when the children with CP used their less affected hand, they would not be affected by the task goals and that their reaching pattern would deviate from those of 17 age-matched children without CP and 20 young healthy adult participants. All participants were instructed to reach and grasp a tennis ball and then to either fit or throw it. Kinematic variables (movement time, straightness ratio, peak velocity, percentage of time to peak velocity, and number of movement units) were used as outcome measures. Children with CP used slower, less straight, less forceful, and jerkier reaching patterns to approach the object than did children without CP and adults. Unlike in children and adults without CP, the intended goal of the subsequent action did not affect the reaching pattern before the intended goal in children with CP. Children with CP may therefore have difficulty in anticipatory planning and thus must segment the integrative action plan into sequential submovements.


Asunto(s)
Parálisis Cerebral/fisiopatología , Intención , Movimiento/fisiología , Fuerza Muscular/fisiología , Desempeño Psicomotor/fisiología , Adaptación Fisiológica , Adulto , Factores de Edad , Estudios de Casos y Controles , Preescolar , Femenino , Objetivos , Humanos , Masculino , Análisis por Apareamiento , Motivación , Valores de Referencia , Análisis y Desempeño de Tareas
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