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1.
J Craniofac Surg ; 29(8): 2327-2333, 2018 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-30339599

RESUMEN

OBJECTIVE: Objective evaluation of the efficacy of tongue lip adhesion (TLA) in the management of Robin sequence (RS). STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral hospital. PATIENTS, PARTICIPANTS: The craniofacial database of Amsterdam UMC, Vrije Universiteit Amsterdam was searched to identify infants with RS who underwent tong lip adhesion (TLA). Forty-one RS infants who underwent TLA from 1993 to 2016 were identified. INTERVENTIONS: TLA. MAIN OUTCOME MEASURE: The outcome measures were pre- and postoperative polysomnography results, nutritional status, weight gain, age at operation, hospital stay length, extubation time after TLA, and complications. RESULTS: Forty-one RS patients were included who had TLA at an average age of 26.6 days. In 16 cases a pre- and postoperative polysomnography was performed. In 13 of these cases (81.3%) improvement was observed, in 2 (12.5%) the results were inconclusive, and in 1 (6.3%) no improvement was seen. Patients were extubated after a mean of 2.2 days.The mean hospital stay was 40.2 days. Reintervention was needed in 7 patients because of a wound dehiscence. The mean age of TLA release was 9.7 months. At discharge, 9 (22%) children still needed total nutritional support for persistent feeding difficulties. The average growth from birth to adhesion release was 4.6 kg. CONCLUSION: This cohort demonstrates that TLA is a successful procedure in children with RS in terms of respiratory, feeding, and growth outcome. Only minor complications were seen in our cohort.


Asunto(s)
Labio/cirugía , Síndrome de Pierre Robin/cirugía , Lengua/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Tiempo de Internación , Masculino , Polisomnografía , Estudios Retrospectivos , Adherencias Tisulares , Resultado del Tratamiento
2.
Clin Ther ; 40(9): 1467-1482, 2018 09.
Artículo en Inglés | MEDLINE | ID: mdl-28189366

RESUMEN

PURPOSE: The aim of the present study was to evaluate the efficacy of an oral formulation of Δ9-tetrahydrocannabinol (ECP002A) in patients with progressive multiple sclerosis (MS). METHODS: This accelerated proof-of-concept study consisted of 2 phases: a crossover challenge (dose-finding) phase and a 4-week, parallel, randomized, placebo-controlled treatment phase. Twenty-four patients with progressive MS and moderate spasticity were enrolled. During the treatment phase, biomarkers for efficacy and secondary pharmacodynamic effects were measured at baseline and after 2 and 4 weeks of treatment. Serum samples were collected to determine pharmacokinetic properties and perform population modeling. Safety and tolerability profiles were assessed based on adverse events and safety measurements. FINDINGS: Pain was significantly reduced when measured directly after administration of ECP002A in the clinic but not when measured in a daily diary. A similar pattern was observed in subjective muscle spasticity. Other clinical outcomes were not significantly different between active treatment and placebo. Cognitive testing indicated that there was no decline in cognition after 2 or 4 weeks of treatment attributable to ECP002A compared with placebo. Implications This study specifically underlines the added value of thorough investigation of pharmacokinetic and pharmacodynamic associations in the target population. Despite the complex interplay of psychoactive effects and analgesia, the current oral formulation of Δ9-tetrahydrocannabinol may play a role in the treatment of spasticity and pain associated with MS because it was well tolerated and had a stable pharmacokinetic profile.


Asunto(s)
Dronabinol/uso terapéutico , Esclerosis Múltiple Crónica Progresiva/tratamiento farmacológico , Espasticidad Muscular/tratamiento farmacológico , Neuralgia/tratamiento farmacológico , Administración Oral , Adulto , Anciano , Cognición/efectos de los fármacos , Estudios Cruzados , Dronabinol/farmacocinética , Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Múltiple Crónica Progresiva/complicaciones , Espasticidad Muscular/etiología , Neuralgia/etiología , Dimensión del Dolor , Prueba de Estudio Conceptual
3.
J Neurosurg Pediatr ; 18(2): 192-5, 2016 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27104630

RESUMEN

Selective dorsal rhizotomy (SDR) surgery is a well-established treatment for ambulatory children with bilateral spastic paresis and is performed to eliminate spasticity and improve walking. The objective of this case report is to describe sudden falls as a persistent complication of SDR. The authors report on 3 patients with bilateral spastic paresis, aged 12, 6, and 7 years at the time of surgery. The percentage of transected dorsal rootlets was around 40% at the L2-S1 levels. Sudden falls were reported with a frequency of several a day, continuing for years after SDR. The falls were often triggered by performing dual tasks as well as occurring in the transition from sitting to standing, during running, after strenuous exercise, or following a fright. Patients also had residual hyperesthesia and dysesthesia of the foot sole. The authors hypothesize that the sudden falls are caused by a muscle inhibition reflex of the muscles in the legs, as an abnormal reaction to a sensory stimulus that is perceived with increased intensity by a patient with hyperesthesia. A favorable effect of gabapentin medication supports this hypothesis.


Asunto(s)
Accidentes por Caídas , Espasticidad Muscular/diagnóstico , Espasticidad Muscular/cirugía , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Rizotomía/efectos adversos , Adolescente , Niño , Femenino , Humanos , Masculino , Rizotomía/tendencias , Adulto Joven
4.
Brain ; 137(Pt 2): 610-20, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24285642

RESUMEN

Insomnia is prevalent, severe and partially heritable. Unfortunately, its neuronal correlates remain enigmatic, hampering the development of mechanistic models and rational treatments. Consistently reported impairments concern fragmented sleep, hyper-arousal and executive dysfunction. Because fronto-striatal networks could well play a role in sleep, arousal regulation and executive functioning, the present series of studies used an executive task to evaluate fronto-striatal functioning in disturbed sleep. Patients with insomnia showed reduced recruitment of the head of the left caudate nucleus during executive functioning, which was not secondary to altered performance or baseline perfusion. Individual differences in caudate recruitment were associated with hyper-arousal severity. Seed-based functional connectivity analysis suggested that attenuated input from a projecting orbitofrontal area with reduced grey matter density contributes to altered caudate recruitment in patients with insomnia. Attenuated caudate recruitment persisted after successful treatment of insomnia, warranting evaluation as a potential vulnerability trait. A similar selective reduction in caudate recruitment could be elicited in participants without sleep complaints by slow-wave sleep fragmentation, providing a model to facilitate investigation of the causes and consequences of insomnia.


Asunto(s)
Núcleo Caudado/fisiopatología , Red Nerviosa/fisiopatología , Trastornos del Inicio y del Mantenimiento del Sueño/diagnóstico , Trastornos del Inicio y del Mantenimiento del Sueño/fisiopatología , Sueño/fisiología , Anciano , Nivel de Alerta/fisiología , Estudios Cruzados , Electroencefalografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Desempeño Psicomotor/fisiología , Vigilia/fisiología
5.
BMC Pediatr ; 13: 175, 2013 Oct 28.
Artículo en Inglés | MEDLINE | ID: mdl-24165282

RESUMEN

BACKGROUND: Dystonic cerebral palsy is primarily caused by damage to the basal ganglia and central cortex. The daily care of these patients can be difficult due to dystonic movements. Intrathecal baclofen treatment is a potential treatment option for dystonia and has become common practice. Despite this widespread adoption, high quality evidence on the effects of intrathecal baclofen treatment on daily activities is lacking and prospective data are needed to judge the usefulness and indications for dystonic cerebral palsy. The primary aim of this study is to provide level one clinical evidence for the effects of intrathecal baclofen treatment on the level of activities and participation in dystonic cerebral palsy patients. Furthermore, we hope to identify clinical characteristics that will predict a beneficial effect of intrathecal baclofen in an individual patient. METHODS/DESIGN: A double blind placebo-controlled multi-center randomized clinical trial will be performed in 30 children with dystonic cerebral palsy. Patients aged between 4 and 25 years old with a confirmed diagnosis of dystonic cerebral palsy, Gross Motor Functioning Classification System level IV or V, with lesions in the cerebral white matter, basal ganglia or central cortex and who are eligible for intrathecal baclofen treatment will be included. Group A will receive three months of continuous intrathecal baclofen treatment and group B will receive three months of placebo treatment, both via an implanted pump. After this three month period, all patients will receive intrathecal baclofen treatment, with a follow-up after nine months. The primary outcome measurement will be the effect on activities of and participation in daily life measured by Goal Attainment Scaling. Secondary outcome measurements on the level of body functions include dystonia, spasticity, pain, comfort and sleep-related breathing disorders. Side effects will be monitored and we will study whether patient characteristics influence outcome. DISCUSSION: The results of this study will provide data for evidence-based use of intrathecal baclofen in dystonic cerebral palsy.


Asunto(s)
Baclofeno/uso terapéutico , Parálisis Cerebral/tratamiento farmacológico , Distonía/tratamiento farmacológico , Agonistas del GABA/uso terapéutico , Actividades Cotidianas , Adolescente , Adulto , Baclofeno/administración & dosificación , Encéfalo/efectos de los fármacos , Encéfalo/patología , Parálisis Cerebral/complicaciones , Niño , Preescolar , Método Doble Ciego , Distonía/etiología , Electromiografía , Estudios de Seguimiento , Agonistas del GABA/administración & dosificación , Reflejo H/efectos de los fármacos , Humanos , Bombas de Infusión Implantables , Infusión Espinal , Imagen por Resonancia Magnética , Manejo del Dolor , Proyectos de Investigación , Tamaño de la Muestra , Índice de Severidad de la Enfermedad , Apnea Central del Sueño/tratamiento farmacológico , Apnea Central del Sueño/etiología , Encuestas y Cuestionarios , Resultado del Tratamiento , Adulto Joven
6.
Dev Med Child Neurol ; 55(7): 610-6, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23557106

RESUMEN

AIM: The aim of this study was to evaluate the long-term effect of selective dorsal rhizotomy (SDR) on the gross motor function of ambulant children with spastic bilateral cerebral palsy (CP), compared with reference centiles. METHOD: The study used a prospective cohort design and participants comprised 29 children classified using the Gross Motor Function Classification System (GMFCS) in level I (n=7), II (n=4), or III (n=18; 18 males, 11 females; median age at time of surgery 6 y 4 mo; range 2 y 10 mo-12 y 1 mo), who were examined 5 years and 10 years after SDR. We used individual centiles based on Gross Motor Function Measure (GMFM-66) scores and age, corresponding to the GMFCS levels. Individual improvement or deterioration was defined as a change of more than 20 centiles. Side effects experienced and additional treatment received after SDR were also recorded. RESULTS: Five years after SDR, 10 out of 28 children showed improvement, and 10 years after SDR 6 out of 20 children had improved. Spinal side effects were noted in two children and hip subluxation in three. Additional treatments included subtalar arthrodesis (n=13), endorotational osteotomy of the tibia (n=5), and botulinum toxin treatment (n=13). INTERPRETATION: None of the children showed deterioration of gross motor function based on centile ranking. Five and 10 years after SDR, gross motor function in some children had improved more than would have been expected according to the reference centiles. This suggests, taking the limitations of this study into account, that the applied criteria for selection were adequate. However, the children still required additional treatment after SDR.


Asunto(s)
Parálisis Cerebral/cirugía , Destreza Motora/fisiología , Trastornos del Movimiento/cirugía , Rizotomía/métodos , Raíces Nerviosas Espinales/cirugía , Parálisis Cerebral/complicaciones , Parálisis Cerebral/tratamiento farmacológico , Niño , Preescolar , Electromiografía , Femenino , Humanos , Masculino , Trastornos del Movimiento/tratamiento farmacológico , Trastornos del Movimiento/etiología , Estudios Prospectivos , Reoperación/métodos , Rizotomía/efectos adversos , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento
7.
J Neurosurg Pediatr ; 7(5): 557-62, 2011 May.
Artículo en Inglés | MEDLINE | ID: mdl-21529199

RESUMEN

OBJECT: The primary aim of this prospective cohort study was to evaluate the short-term (1 year) and long-term (mean 6 years) effects of selective dorsal rhizotomy (SDR) on gross motor function and spasticity in ambulatory children with spastic diplegia. Secondary aims were to investigate side effects, additional treatment during follow-up (botulinum toxin type A injections or orthopedic surgery), and parental satisfaction. METHODS: Thirty-three children who had undergone SDR at a mean age of 6 years and 7 months (± 2 years) were included. There were 7 children at Gross Motor Function Classification System (GMFCS) Level I, 7 at Level II, and 19 at Level III. Gross motor function was assessed with the Gross Motor Function Measure-66 (GMFM-66). Spasticity was measured according to a modified Tardieu scale. Side effects, additional treatment, and parental satisfaction were recorded using a parental questionnaire and medical records. RESULTS: At 1-year follow-up, mean GMFM-66 scores improved significantly by 4.3 ± 4.1 points. Children at GMFCS Levels I and II showed significantly more improvement (7.2 points) on the GMFM-66 compared with children at GMFCS Level III (2.9 points). On long-term follow-up (mean 6 years ± 22 months), mean GMFM-66 scores improved significantly by 6.5 ± 5.9 points, without a difference between children at GMFCS Levels I and II and Level III. No relapse of spasticity was noted. Ten children (30%) needed orthopedic surgery and 13 children (39%) received botulinum toxin type A treatment after SDR. Twenty (91%) of the 22 parents who answered the questionnaire at long-term follow-up believed that their child's functioning had improved after SDR. CONCLUSIONS: Selective dorsal rhizotomy resulted in short- and long-term improvements in gross motor function, without relapse of spasticity. However, the majority of the children still needed additional surgery or botulinum toxin A treatment.


Asunto(s)
Parálisis Cerebral/cirugía , Paraparesia Espástica/cirugía , Rizotomía/métodos , Toxinas Botulínicas Tipo A/uso terapéutico , Parálisis Cerebral/fisiopatología , Niño , Preescolar , Estudios de Cohortes , Terapia Combinada , Evaluación de la Discapacidad , Femenino , Estudios de Seguimiento , Humanos , Masculino , Destreza Motora/fisiología , Examen Neurológico , Paraparesia Espástica/genética , Paraparesia Espástica/fisiopatología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/terapia , Estudios Prospectivos , Rango del Movimiento Articular/fisiología , Enfermedades de la Columna Vertebral/etiología
8.
Biol Psychiatry ; 68(10): 950-5, 2010 Nov 15.
Artículo en Inglés | MEDLINE | ID: mdl-20728874

RESUMEN

BACKGROUND: Chronic insomnia is a poorly understood disorder. Risk factors for developing chronic insomnia are largely unknown, yet disturbances in brain indexes of arousal seem to accompany the disorder. We here investigate whether insomnia patients and control participants differ with respect to brain responses to direct stimulation, i.e., cortical excitability. Transcranial magnetic stimulation (TMS) offers a method to directly investigate the excitability level of the human cerebral cortex in psychiatric and neurological disease. METHODS: We investigated cortical excitability in 16 insomnia patients and 14 carefully matched control participants using absolute and relative amplitudes of motor evoked potentials in response to single- and paired-pulse stimulation using TMS. RESULTS: Nonmedicated insomnia patients showed, first, an exaggerated absolute response to both suprathreshold single- and paired-pulse stimulation compared with control participants and second, a reduced relative response to paired-pulse stimulation at long interpulse intervals (i.e., a reduced intracortical facilitation). The abnormal excitability persisted despite sleep therapy that effectively improved sleep quality as well as behavioral and neuroimaging indexes of brain function. CONCLUSIONS: The results suggest that a subtly disturbed intracortical excitability characterizes patients with chronic insomnia: a relatively reduced intracortical facilitation in the context of a globally increased absolute excitability. The findings do not resemble TMS findings after sleep deprivation or in sleep apnea and thus seem specific to insomnia. They may offer diagnostic value and implications for assessment of risk to develop this common and disabling disorder.


Asunto(s)
Corteza Cerebral/fisiopatología , Terapia Combinada/métodos , Potenciales Evocados Motores/fisiología , Trastornos del Inicio y del Mantenimiento del Sueño/fisiopatología , Trastornos del Inicio y del Mantenimiento del Sueño/terapia , Estimulación Magnética Transcraneal/métodos , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad
9.
BMC Neurosci ; 10: 101, 2009 Aug 21.
Artículo en Inglés | MEDLINE | ID: mdl-19698093

RESUMEN

BACKGROUND: Although a large body of knowledge about both brain structure and function has been gathered over the last decades, we still have a poor understanding of their exact relationship. Graph theory provides a method to study the relation between network structure and function, and its application to neuroscientific data is an emerging research field. We investigated topological changes in large-scale functional brain networks in patients with Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD) by means of graph theoretical analysis of resting-state EEG recordings. EEGs of 20 patients with mild to moderate AD, 15 FTLD patients, and 23 non-demented individuals were recorded in an eyes-closed resting-state. The synchronization likelihood (SL), a measure of functional connectivity, was calculated for each sensor pair in 0.5-4 Hz, 4-8 Hz, 8-10 Hz, 10-13 Hz, 13-30 Hz and 30-45 Hz frequency bands. The resulting connectivity matrices were converted to unweighted graphs, whose structure was characterized with several measures: mean clustering coefficient (local connectivity), characteristic path length (global connectivity) and degree correlation (network 'assortativity'). All results were normalized for network size and compared with random control networks. RESULTS: In AD, the clustering coefficient decreased in the lower alpha and beta bands (p < 0.001), and the characteristic path length decreased in the lower alpha and gamma bands (p < 0.05) compared to controls. In FTLD no significant differences with controls were found in these measures. The degree correlation decreased in both alpha bands in AD compared to controls (p < 0.05), but increased in the FTLD lower alpha band compared with controls (p < 0.01). CONCLUSION: With decreasing local and global connectivity parameters, the large-scale functional brain network organization in AD deviates from the optimal 'small-world' network structure towards a more 'random' type. This is associated with less efficient information exchange between brain areas, supporting the disconnection hypothesis of AD. Surprisingly, FTLD patients show changes in the opposite direction, towards a (perhaps excessively) more 'ordered' network structure, possibly reflecting a different underlying pathophysiological process.


Asunto(s)
Enfermedad de Alzheimer/fisiopatología , Demencia/fisiopatología , Modelos Neurológicos , Red Nerviosa/fisiopatología , Procesamiento de Señales Asistido por Computador , Adulto , Anciano , Encéfalo/fisiopatología , Electroencefalografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dinámicas no Lineales
10.
J Child Neurol ; 24(5): 625-7, 2009 May.
Artículo en Inglés | MEDLINE | ID: mdl-19151363

RESUMEN

Selective dorsal rhizotomy is an effective treatment for spasticity in children with cerebral palsy who have a spastic motor disorder. It is hypothesized that muscle shortening is related to spasticity; the lack of stretch of a muscle is thought to be the cause of muscle shortening. If this is true, the treatment for spasticity should prevent the occurrence of muscle shortening during growth. We present the case of 1 child with cerebral palsy and spastic diplegia, for whom the treatment with selective dorsal rhizotomy was successful in improving the walking abilities. She did, however, develop muscle shortening during growth. In conclusion, the development of muscle shortening during growth in children with cerebral palsy and spastic paresis cannot be prevented by treatment for the spasticity alone.


Asunto(s)
Parálisis Cerebral/complicaciones , Parálisis Cerebral/cirugía , Músculo Esquelético/crecimiento & desarrollo , Enfermedades Musculares/etiología , Raíces Nerviosas Espinales/cirugía , Niño , Desarrollo Infantil , Preescolar , Femenino , Humanos , Laminectomía , Vértebras Lumbares , Espasticidad Muscular/complicaciones , Espasticidad Muscular/cirugía , Músculo Esquelético/fisiopatología , Enfermedades Musculares/fisiopatología , Rizotomía , Resultado del Tratamiento
11.
J Sleep Res ; 17(3): 335-43, 2008 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-18844819

RESUMEN

Although complaints of impaired daytime functioning are essential to the diagnosis of primary insomnia, objective evidence for cognitive dysfunction has been hard to establish. A prerequisite for understanding the neurocognitive consequences of primary insomnia is to establish task paradigms that robustly differentiate insomniacs from well-sleeping subjects. We hypothesized that the decline in performance that typically occurs with an increasing cognitive demand would provide a more sensitive measure than performance on a single task version. The hypothesis was tested, first, by assessing the performance on two vigilance tasks with different cognitive demands in 25 elderly patients with primary insomnia and 13 healthy well-sleeping age-matched subjects. Secondly, we investigated the performance response to sleep therapy using a waiting-list controlled design. Sleep therapy consisted of a multi-component intervention including sleep restriction, cognitive behavioral therapy, bright-light therapy, structured physical activity and body temperature manipulations. The results show that insomniacs differed markedly from controls in their reaction times across tasks with different cognitive demands: patients responded faster on the 'simple' vigilance task, yet slower on the 'complex' vigilance task. Sleep therapy effectively restored normal performance: patients became significantly slower on the 'simple' task and faster on the 'complex' task, returning to the performance levels of control subjects. These findings indicate that the performance decline associated with increasing cognitive demands is possibly the first sensitive and robust measure of the neurocognitive sequelae of insomnia. We suggest that future studies on cognition in primary insomnia should apply a design that varies task demands.


Asunto(s)
Atención , Regulación de la Temperatura Corporal , Terapia Cognitivo-Conductual , Ejercicio Físico , Reconocimiento Visual de Modelos , Fototerapia , Desempeño Psicomotor , Tiempo de Reacción , Privación de Sueño/psicología , Trastornos del Inicio y del Mantenimiento del Sueño/psicología , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pruebas Neuropsicológicas/estadística & datos numéricos , Polisomnografía , Psicometría , Valores de Referencia , Trastornos del Inicio y del Mantenimiento del Sueño/terapia
12.
J Child Neurol ; 23(7): 818-22, 2008 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-18658081

RESUMEN

Selective dorsal rhizotomy at the lumbar level is a neurosurgical procedure, which reduces spasticity in the legs. Its effect has mainly been studied in children with spastic cerebral palsy. Little is known about the outcome of selective dorsal rhizotomy in patients with neurodegenerative disorders. We report the clinical course after selective dorsal rhizotomy in 2 patients with progressive spasticity. Leg spasticity was effectively and persistently reduced in both patients, facilitating care and improving sitting comfort. However, spasticity of the arms and other motor disturbances, such as spontaneous extension spasms and the ataxia, increased gradually in time. Selective dorsal rhizotomy leads to a disappearance of leg spasticity in patients with a neurodegenerative disease. Other motor signs are not influenced and may increase due to the progressive nature of the underlying disease.


Asunto(s)
Espasticidad Muscular/cirugía , Enfermedades Neurodegenerativas/complicaciones , Rizotomía/métodos , Adolescente , Atrofia/complicaciones , Ganglios Basales/patología , Cerebelo/patología , Niño , Preescolar , Humanos , Pierna/inervación , Pierna/fisiopatología , Vértebras Lumbares , Masculino , Espasticidad Muscular/complicaciones , Espasticidad Muscular/fisiopatología , Enfermedades Neurodegenerativas/fisiopatología , Enfermedades Neurodegenerativas/cirugía , Raíces Nerviosas Espinales/cirugía , Resultado del Tratamiento
13.
Clin Neurophysiol ; 119(8): 1732-1738, 2008 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-18490193

RESUMEN

OBJECTIVE: To investigate the presence of EEG abnormalities in frontotemporal lobar degeneration (FTLD) in comparison with Alzheimer's disease (AD) and non-demented individuals with subjective memory complaints (SMC), using an elaborated visual EEG rating scale; furthermore, to investigate whether assessment of resting-state functional connectivity of the EEG is superior to visual evaluation in distinguishing between FTLD, AD and non-demented controls. METHODS: EEGs of 15 patients with FTLD, 20 with AD and 23 individuals with SMC were visually compared using the Grand Total EEG (GTE) score. The synchronization likelihood (SL) as a measure of functional connectivity between different EEG channels was calculated for the 0.5-4Hz, 4-8Hz, 8-10Hz, 10-13Hz, 13-30Hz and 30-45Hz frequency bands. Patients had mild to moderate dementia. RESULTS: In AD, as expected, the GTE revealed significant differences from FTLD and SMC, indicating more EEG slowing and loss of reactivity. Patients with FTLD, however, could not be discriminated from individuals with SMC by the GTE score. Analysis of resting-state functional connectivity showed decreased SL in AD compared to both FTLD and SMC in the lower and higher alpha frequency band and decreased SL in AD compared to SMC in the beta frequency band, whereas no differences between FTLD and AD or SMC were found. CONCLUSIONS: In patients with mild to moderate FTLD both the visually rated EEG and EEG measures of resting-state functional connectivity are normal. SIGNIFICANCE: Although widespread neuronal degeneration takes place in frontotemporal lobar degeneration, this is not reflected in the EEG during the mild to moderate stages of the disease. An abnormal EEG in a mildly demented subject favours a diagnosis of AD.


Asunto(s)
Mapeo Encefálico , Demencia/patología , Demencia/fisiopatología , Electroencefalografía , Descanso/fisiología , Adulto , Anciano , Enfermedad de Alzheimer/patología , Enfermedad de Alzheimer/fisiopatología , Análisis de Varianza , Electrodos , Electroencefalografía/clasificación , Femenino , Humanos , Masculino , Trastornos de la Memoria/fisiopatología , Persona de Mediana Edad , Vías Nerviosas/patología , Vías Nerviosas/fisiopatología
14.
Neurosurgery ; 59(4): 858-67; discussion 867-9, 2006 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-17038950

RESUMEN

OBJECTIVE: A systematic follow-up of infants with an obstetric brachial plexus lesion of C5 and C6 or the superior trunk showing satisfactory spontaneous recovery of shoulder and arm function except for voluntary shoulder exorotation, who underwent an accessory to suprascapular nerve transfer to improve active shoulder exorotation, to evaluate for functional recovery, and to understand why other superior trunk functions spontaneously recover in contrast with exorotation. METHODS: In 54 children, an accessory to suprascapular nerve transfer was performed as a separate procedure at a mean age of 21.7 months. Follow-up examinations were conducted before and at 4, 8, 12, 24, and 36 months after operation and included scoring of shoulder exorotation and abduction. Intraoperative reactivity of spinatus muscles and additional needle electromyographic responses were registered after electrostimulation of suprascapular nerves. Histological examination of suprascapular nerves was performed. Trophy of spinatus muscles was followed by magnetic resonance imaging scanning. The influence of perinatal variables and results of ancillary investigations on outcome were evaluated. RESULTS: Exorotation improved from 70 degrees to functional levels exceeding 0 degrees, except in two patients. Abduction improved in 27 patients, with results of 90 degrees or more in 49 patients. Electromyography at 4 months did not show signs of denervation in 39 out of 40 patients. Intraoperative electrostimulation of suprascapular nerves elicited spinatus muscle reaction in 44 out of 48 patients. Histology of suprascapular nerves was normal. Preoperative magnetic resonance imaging scans showed only minor wasting of spinatus muscles in contrast with major wasting after successful operations. CONCLUSION: An accessory to suprascapular nerve transfer is effective to restore active exorotation when performed as the primary or a separate secondary procedure in children older than 10 months of age. Contradictory spontaneous recovery of other superior trunk functions and integrity of suprascapular nerves, as well as absence of spinatus muscle wasting direct to central nervous changes are possible main causes for the lack of exorotation.


Asunto(s)
Nervio Accesorio/trasplante , Traumatismos del Nacimiento/complicaciones , Neuropatías del Plexo Braquial/fisiopatología , Neuropatías del Plexo Braquial/cirugía , Articulación del Hombro/inervación , Articulación del Hombro/fisiopatología , Hombro/fisiopatología , Neuropatías del Plexo Braquial/diagnóstico , Neuropatías del Plexo Braquial/etiología , Niño , Preescolar , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Músculo Esquelético/patología , Rango del Movimiento Articular , Recuperación de la Función , Rotación , Hombro/patología
15.
Neurosurgery ; 57(5): 930-40; discussion 930-40, 2005 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-16284563

RESUMEN

OBJECTIVE: Preoperative, reliable detection by ancillary investigations of spinal nerve root avulsions in infants with severe obstetric brachial plexus lesions to avoid ineffective operative repair from deceivingly intact but actually avulsed nerve roots. METHODS: Ten infants were selected with an infrequent, severe dominant C7 lesion, primarily because of the anatomically distinct supraclavicular course of this spinal nerve. Three-dimensional constructive interference in steady-state magnetic resonance imaging (3D CISS MRI) studies under mild sedation were performed and evaluated for detection of avulsed nerve roots by two experienced neuroradiologists. Preoperative electrodiagnostics (electromyography and somatosensory evoked potentials) as well as intraoperative somatosensory potentials and muscle contractions after electrostimulation were recorded. Preoperative and intraoperative ancillary investigations were correlated with intraoperative findings in eight patients and clinical status in two children who recovered spontaneously. RESULTS: Despite two minor motion artifacts, the quality of the 3D CISS MRI studies was good. In 8 of 10 patients, prediction of root continuity was consistent with operative or clinical findings, and 2 remained doubtful. Preoperative and intraoperative electrodiagnostics tended not to correlate with intraoperative findings in this small, selected group. CONCLUSION: 3D CISS MRI provides good images of anterior and posterior spinal roots in infants with obstetric brachial plexus lesions. Images seem to allow accurate prediction of root avulsion in the majority of patients. In this study, electrodiagnostics were of limited value.


Asunto(s)
Plexo Braquial/patología , Radiculopatía/diagnóstico , Raíces Nerviosas Espinales/patología , Electromiografía/métodos , Humanos , Imagenología Tridimensional/métodos , Lactante , Imagen por Resonancia Magnética/métodos , Radiculopatía/fisiopatología , Radiculopatía/cirugía , Estudios Retrospectivos , Tomografía Computarizada por Rayos X/métodos
16.
J Clin Neurophysiol ; 22(3): 216-21, 2005 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-15933495

RESUMEN

The reported relationships between nerve conduction studies (NCS) and outcome measures in carpal tunnel syndrome (CTS) are weak to moderate. However, selection of patients may have confounded nonrandomized studies. NCS have potentially great value in selecting patients for a specific treatment and in objectively assessing the efficacy of treatments in CTS, especially if they correlate significantly with clinical outcome measures. To investigate the relationship between clinical outcome measures for the severity of complaints and NCS in patients treated for CTS, data were obtained from a multicenter randomized controlled trial on the efficacy of splinting versus surgery for CTS. At baseline and 12 months after randomization, clinical outcome measures were assessed and NCS were performed. In total, 138 patients completed the questionnaires and underwent repeated NCS. Relationships were analyzed with Spearman rank correlation coefficients and Pearson correlation coefficients. All NCS parameters showed highly significant improvement compared with baseline (P < 0.001). Modest correlations (< 0.4) were found between the neurophysiologic and clinical outcome measures after 12 months, and between the changes in these different categories of outcome measures. This study confirms that the parameters of NCS improve significantly after treatment for CTS, but the modest correlations between neurophysiologic and clinical outcome measures do not support that NCS are routinely performed in clinical practice to evaluate treatment effects. However, studies investigating the effects of treatment for CTS should incorporate both clinical outcome measures and NCS, because they are complementary. Furthermore, NCS can provide additional information to the clinician when treatment effects are unsatisfactory.


Asunto(s)
Síndrome del Túnel Carpiano/fisiopatología , Síndrome del Túnel Carpiano/terapia , Estimulación Eléctrica/métodos , Conducción Nerviosa/fisiología , Evaluación de Resultado en la Atención de Salud , Adulto , Electromiografía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Nervio Mediano/fisiopatología , Nervio Mediano/efectos de la radiación , Persona de Mediana Edad , Conducción Nerviosa/efectos de la radiación , Examen Neurológico , Tiempo de Reacción/fisiología , Tiempo de Reacción/efectos de la radiación , Recuperación de la Función , Sensibilidad y Especificidad , Umbral Sensorial , Índice de Severidad de la Enfermedad , Estadística como Asunto , Encuestas y Cuestionarios
17.
Clin Neurophysiol ; 114(1): 50-5, 2003 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-12495763

RESUMEN

OBJECTIVE: To investigate whether epileptic seizure activity can be distinguished from non-epileptic background activity in the neonatal electroenceplalogram (EEG), using synchronization likelihood as a measure of synchronization between EEG channels. METHODS: Forty-two 21s EEG epochs and two complete EEGs from 21 different neonatal patients in a 12-channel bipolar recording were studied (AD-conversion 16bit; sample frequency 200Hz; filter setting 0.5-30Hz). For EEG of each patient, we selected one epoch with epileptic discharges and one without. Synchronization was calculated in all epochs. In two complete EEGs, synchronization was calculated and correlated with a visual scoring of the EEG. RESULTS: Synchronization likelihood was higher in all the epochs with epileptic seizures as compared to the epochs without epileptic activity (P<0.01). When synchronization likelihood exceeded 0.11, the sensitivity for the presence of epileptic activity was 0.85 (95% confidence limits [CL(95)]=0.69-1) and the specificity was 0.75 (CL(95)=0.56-0.94).Analysis of EEG score and synchronization likelihood of two complete EEGs revealed a high correlation between the occurrence of epileptic seizures and elevated synchronization likelihood (Spearman r=0.707, P<0.001). CONCLUSIONS: The results of this study demonstrate that synchronization likelihood is a potential tool in the automatic monitoring of high-risk infants for epileptic activity on neonatal wards.


Asunto(s)
Sincronización Cortical , Electroencefalografía , Epilepsia/fisiopatología , Humanos , Recién Nacido , Convulsiones/fisiopatología
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