RESUMEN
Two scales have been developed and validated in English to evaluate the impact of tremor on daily life, namely Quality of life in Essential Tremor Questionnaire (QUEST) and Essential Tremor Embarrassment Assessment (ETEA). The psychometric properties of the French version of these two scales were assessed for 117 patients with head tremor. Both scales showed excellent acceptability, very good internal consistency (Cronbach's alpha coefficient>0.8) and reproducibility (Lin concordance coefficient>0.8), satisfactory external validity and satisfactory sensitivity to change. In conclusion, the French versions of QUEST and ETEA are comprehensive, valid and reliable instruments for assessing patients with head tremor.
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Temblor Esencial , Calidad de Vida , Humanos , Temblor Esencial/diagnóstico , Desconcierto , Temblor/diagnóstico , Temblor/etiología , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , PsicometríaRESUMEN
BACKGROUND AND PURPOSE: In patients with cervical dystonia, abobotulinumtoxinA solution for injection (ASI) has been shown to be similarly effective to freeze-dried abobotulinumtoxinA in reducing Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) total scores. In this secondary analysis, quality of life data as evaluated with the Cervical Dystonia Impact Profile (CDIP-58) are presented. METHODS: This was a double-blind, randomized, active and placebo-controlled study followed by an open-label extension (NCT01261611). In the double-blind phase, patients were randomized (3:3:1) to one cycle of ASI 500 U (n = 156), abobotulinumtoxinA 500 U (n = 159) or placebo (n = 54). Following the double-blind phase, all patients received open-label ASI for up to four cycles. RESULTS: The CDIP-58 total scores were significantly improved at week 4 of the double-blind phase in both the ASI 500 U and abobotulinumtoxinA 500 U groups versus placebo [least squares mean change from baseline of -9.5 (-11.51, -7.45) and -11.2 (-13.2, -9.26) vs. -0.9 (-4.04, 2.14), respectively; both P < 0.0001 vs. placebo]. All CDIP-58 domains contributed to this improvement and benefits were maintained across open-label treatment. Positive correlations were observed between CDIP-58 total score and all three TWSTRS domains (R = 0.42-0.62) and for all CDIP-58 subscales with the TWSTRS total score and domains (R = 0.23-0.60). CONCLUSIONS: Repeat ASI injections are similarly effective to abobotulinumtoxinA in improving patient-reported outcomes of health-related quality of life. Positive correlations were found between TWSTRS total and domain scores and CDIP-58 total and domain scores.
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Toxinas Botulínicas Tipo A/uso terapéutico , Fármacos Neuromusculares/uso terapéutico , Calidad de Vida , Tortícolis/tratamiento farmacológico , Adulto , Método Doble Ciego , Femenino , Humanos , Inyecciones Intramusculares , Masculino , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
Recovery of motor function after stroke occurs largely on the basis of a sustained capacity of the adult brain for plastic changes. This brain plasticity has been validated by functional imaging and electrophysiological studies. Various concepts of how to enhance beneficial plasticity and in turn improve functional recovery are emerging based on the concept of functional interhemispheric balance between the two motor cortices. Besides conventional rehabilitation interventions and the most recent neuropharmacological approaches, non-invasive brain stimulation (NIBS) has recently been proposed as an add-on method to promote motor function recovery after stroke. Several methods can be used based either on transcranial magnetic stimulation (repetitive mode: rTMS, TBS) via a coil, or small electric current via larges electrodes placed on the scalp, (transcranial direct current stimulation tDCS). Depending on the different electrophysiological parameters of stimulation used, NIBS can induce a transient modulation of the excitability of the stimulated motor cortex (facilitation or inhibition) via a probable LTP-LTD-like mechanism. Several small studies have shown feasible and positive treatment effects for most of these strategies and their potential clinical relevance to help restoring the disruption of interhemispheric imbalance after stroke. Results of these studies are encouraging but many questions remain unsolved: what are the optimal stimulation parameters? What is the best NIBS intervention? Which cortex, injured or intact, should be stimulated? What is the best window of intervention? Is there a special subgroup of stroke patients who could strongly benefit from these interventions? Finally is it possible to boost NIBS treatment effect by motor training of the paretic hand or by additional neuropharmacological interventions? There is clearly a need for large-scale, controlled, multicenter trials to answer these questions before proposing their routine use in the management of stroke patients.
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Rehabilitación Neurológica/métodos , Recuperación de la Función , Rehabilitación de Accidente Cerebrovascular , Estimulación Transcraneal de Corriente Directa/métodos , Estimulación Magnética Transcraneal/métodos , Adulto , Encéfalo/fisiopatología , Humanos , Corteza Motora , Plasticidad NeuronalRESUMEN
OBJECTIVES: Transcranial magnetic stimulations (TMS) have been used for many years as a diagnostic tool to explore changes in cortical excitability, and more recently as a tool for therapeutic neuromodulation. We are interested in their applications following brain injury: stroke, traumatic and anoxic brain injury. DATA SYNTHESIS: Following brain injury, there is decreased cortical excitability and changes in interhemispheric interactions depending on the type, the severity, and the time-lapse between the injury and the treatment implemented. rTMS (repetitive TMS) is a therapeutic neuromodulation tool which restores the interhemispheric interactions following stroke by inhibiting the healthy cortex with frequencies ≤1Hz, or by exciting the lesioned cortex with frequencies between 3 and 50Hz. Results in motor recovery are promising and those in improving aphasia or visuospatial neglect are also encouraging. Finally, the use of TMS is mainly limited by the risk of seizure, and is therefore contraindicated for many patients. CONCLUSION: TMS is a useful non-invasive brain stimulation tool to diagnose the effects of brain injury, to study the mechanisms of recovery and a non-invasive neuromodulation promising tool to influence the post-lesional recovery.
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Lesiones Encefálicas/terapia , Estimulación Magnética Transcraneal , Afasia/terapia , Lesiones Encefálicas/complicaciones , Lesiones Encefálicas/diagnóstico , Lesiones Encefálicas/fisiopatología , Lesiones Encefálicas/rehabilitación , Contraindicaciones , Lesión Axonal Difusa/terapia , Humanos , Hipoxia Encefálica/terapia , Neuralgia/terapia , Plasticidad Neuronal , Trastornos de la Percepción/terapia , Recuperación de la Función , Convulsiones/etiología , Accidente Cerebrovascular/terapia , Estimulación Magnética Transcraneal/efectos adversosRESUMEN
The interhemispheric interactions between homologous wrist extensor and flexor muscles representations in the right and left primary cortex (M1) were studied using a paired-pulse transcranial magnetic stimulation in healthy subjects. The magnitude of interhemispheric inhibition (IHI) was studied in 9 right-handed subjects at short (10 ms, SIHI) and long (40 ms, LIHI) interstimulus intervals between the magnetic conditioning (CS) and test stimulus in the motor dominant to non-dominant cortex and vice versa, while the right or left hand was at rest or performing a unimanual sustained tonic contraction (holding a pen with the hand contralateral to the CS). A bidirectional powerful interhemispheric inhibition could be elicited at the short and long IHI phases (SIHI and LIHI) in wrist extensor and flexor muscles in most of the subjects at rest. SIHI but not LIHI was significantly bidirectionally reduced during unimanual contraction of the hand contralateral to the CS stimulation in comparison with rest. The amount of IHI after the stimulation of the "non-dominant" right hemisphere was not reduced in comparison with IHI after stimulation of the "dominant" left hemisphere whatever the active or resting condition. IHI directed to the wrist muscles had a similar level than IHI directed to digit muscles (FDI) at rest. Our data indicate that contralateral wrist muscles activity evokes a global, bidirectional reduction in IHI which was more pronounced for SIHI. These results provide additional evidence that changes in interhemispheric interactions between the M1s are involved in the control of unimanual movements including suppression of unwanted motor activity in the opposite limb during unilateral movements.
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Vías Eferentes/fisiología , Lateralidad Funcional/fisiología , Corteza Motora/fisiología , Músculo Esquelético/inervación , Inhibición Neural/fisiología , Muñeca/inervación , Adulto , Potenciales Evocados Motores/fisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Contracción Muscular/fisiología , Músculo Esquelético/fisiología , Estimulación Magnética Transcraneal/métodos , Muñeca/fisiología , Adulto JovenRESUMEN
OBJECTIVE: To investigate the contribution of group II spinal pathways in Parkinsonian upper limb rigidity and the modulation of spinal excitability of group I and group II pathways by L-DOPA and subthalamic nucleus-high-frequency stimulation (STN-HFS). METHODS: The effect of ulnar nerve electrical stimulation on Flexor Carpi Radialis Electromyogram (FCR EMG) was investigated in two groups of patients: patients receiving medication (MED group) and chronically surgically implanted patients (DBS group). Results were compared in patients ON and OFF treatment, and between patients and control subjects. RESULTS: The resulting long-lasting facilitation in FCR EMG had similar characteristics in all groups, and surface area was assessed in analysis windows corresponding to the parts supposed to be mediated by non-monosynaptic spinal pathways to FCR motoneurones, fed by hand muscle group I and group II afferents (Lourenço et al., 2006). In both the MED and DBS groups, the group I excitation was not altered but the group II excitation was particularly enhanced when OFF treatment, compared to controls, and both L-DOPA and STN-HFS restored the group II spinal excitation to normal level. CONCLUSION: Both L-DOPA and STN-HFS influence the metabolism of monoamines in the midbrain, and restore the descending neuromodulation on group II spinal reflex. SIGNIFICANCE: These results further support a group II contribution to the enhanced long latency response (LLR) to muscle stretch observed in wrist muscles of rigid Parkinson's disease (PD) patients.
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Antiparkinsonianos/uso terapéutico , Levodopa/uso terapéutico , Enfermedad de Parkinson/fisiopatología , Enfermedad de Parkinson/terapia , Reflejo/fisiología , Adulto , Anciano , Análisis de Varianza , Antiparkinsonianos/farmacología , Estimulación Encefálica Profunda , Electromiografía , Femenino , Humanos , Levodopa/farmacología , Masculino , Persona de Mediana Edad , Rigidez Muscular/terapia , Reflejo/efectos de los fármacosRESUMEN
Functional imaging has provided new evidence of the neurobiological impact of the treatment of aphasia, including speech therapy, through the alteration of the activated language neural network. In such a way, speech therapy has proved its impact. The role of each hemisphere is still very unclear. Some of the authors link the left-lateralisation of activations to the therapeutic improvement of language and the right-activated network to a maladaptative strategy, whereas others consider the latter as a useful compensatory network for speech disorders. Repetitive trans-cranial magnetic stimulation (rTMS), first used to determine cortical activity, is now used to directly interfere with cerebral activity. In the years to come, rTMS should be developed as an adjuvant therapy for aphasia.
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Afasia/patología , Afasia/terapia , Imagen por Resonancia Magnética , Logopedia , Estimulación Magnética Transcraneal , Afasia/fisiopatología , Corteza Cerebral/fisiopatología , Lateralidad Funcional/fisiología , Humanos , Procesamiento de Imagen Asistido por Computador , LenguajeRESUMEN
BACKGROUND: Patients suffering from Parkinson's disease (PD) describe painful sensations that could be related to neuropathic pain. Experimental data have indicated the involvement of basal ganglia and dopaminergic pathways in central nociceptive processing. AIM: The objective of this study was to assess and compare the effect of levodopa on the objective pain threshold in patients with PD and healthy subjects. METHODS: The objective pain threshold was assessed by the nociceptive flexion reflex (RIII) in 13 PD patients and 10 healthy subjects. Patients and healthy subjects were evaluated under two randomised conditions: with levodopa (ON) and without (OFF). RESULTS: Levodopa significantly increased the RIII threshold of PD patients (6.9 (1.2) mA in the OFF condition vs 8 (1.1) mA in the ON position; p = 0.02). RIII threshold was significantly lower in PD patients than in healthy subjects in the OFF condition (6.9 (1.2) mA vs 9.7 (3.4) mA; p = 0.02). RIII threshold did not change after levodopa administration in healthy subjects. CONCLUSION: These results provide evidence of a dopaminergic modulation of objective pain threshold in PD patients. In addition, the decrease in RIII threshold in PD patients, in the OFF condition, compared with controls, confirms the existence of an objective pain perception disturbance in PD.
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Levodopa/uso terapéutico , Umbral del Dolor/efectos de los fármacos , Dolor/tratamiento farmacológico , Dolor/etiología , Enfermedad de Parkinson/complicaciones , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Dolor/fisiopatologíaRESUMEN
For 15 years, 50 Japanese and European families with cortical myoclonic tremor and epilepsy were reported in the literature under various names. More recently, the acronym familial cortical myoclonic tremor with epilepsy (FCMTE) has been proposed for this new clinical entity based on both clinical and electrophysiological criteria: irregular postural myoclonic tremor of the distal limbs, familial history of epilepsy, autosomal dominant inheritance, and a rather benign outcome. The diagnosis is confirmed by electrophysiological features favoring cortical reflex myoclonus (enhanced C reflex at rest, giant somatosensory evoked potentials (SEPs), premyoclonus cortical spikes detected by the jerk-locked back-averaging method), and a good response to antiepileptic drugs. The genetic analysis of these families shows heterogeneity with a linkage to chromosome 8q24 for Japanese families, a linkage to chromosome 2p for Italian families, the exclusion of 8q24 locus for a Spanish family, and the exclusion of both loci for a Dutch family. The similarities of this syndrome with the group of myoclonic epilepsy suggest an abnormality of a gene encoding ion channels.
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Corteza Cerebral/fisiopatología , Epilepsias Mioclónicas/genética , Temblor/genética , Edad de Inicio , Anticonvulsivantes/uso terapéutico , Cromosomas Humanos Par 8/genética , Electroencefalografía , Epilepsias Mioclónicas/tratamiento farmacológico , Epilepsias Mioclónicas/epidemiología , Epilepsias Mioclónicas/fisiopatología , Europa (Continente)/epidemiología , Potenciales Evocados Somatosensoriales , Genes Dominantes , Heterogeneidad Genética , Humanos , Canales Iónicos/genética , Canales Iónicos/fisiología , Japón/epidemiología , Reflejo Anormal , Temblor/tratamiento farmacológico , Temblor/epidemiología , Temblor/fisiopatologíaRESUMEN
The aim of the study was to investigate the effect of chronic administration of paroxetine (selective serotonin reuptake inhibitor: SSRI) on motor cortex excitability in healthy subjects by means of transcranial magnetic stimulation (TMS), functional magnetic resonance imaging (fMRI) and behavioral motor tests. In a randomized, double-blind, crossover study, twenty-one right-handed subjects received 20 mg daily of either paroxetine or a placebo over a period of 30 days separated by a period of 3 months wash-out. The TMS study is presented here correlated with some results of the motor behavior study (finger tapping test) and the fMRI study (primary sensorimotor cortex (S1M1) volume of activation). TMS was used to test motor threshold (MT), motor evoked potential recruitment curve (RC), cortical silent period (CSP) and paired-pulse intracortical inhibition and facilitation (ICI, ICF). Chronic administration of paroxetine did not modulate ICI or CSP but induced a significant enhancement of mean ICF (ANOVA P=0.04), which significantly correlated with increase of speed in a finger tapping test (P=0.02). This suggests a modulation of cortical interneuronal excitatory pathways without changes in the excitability of cortical inhibitory GABAergic interneurons. A decrease of RC (ANOVA P=0.05) was also observed after 30 days intake of paroxetine in comparison with placebo and was associated with changes of fMRI activation intensity (left S1M1 hypoactivation, ), without changes of S1M1 activation volume. Finally, the different modulation of RC and ICF after chronic administration of paroxetine compared to single dose (opposite effects) emphasizes the different pharmacological action of the drug at cortical level depending on its acute or long-term administration.
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Corteza Motora/efectos de los fármacos , Paroxetina/farmacología , Inhibidores Selectivos de la Recaptación de Serotonina/farmacología , Adulto , Anciano , Estudios Cruzados , Relación Dosis-Respuesta a Droga , Método Doble Ciego , Campos Electromagnéticos , Electromiografía , Femenino , Dedos , Lateralidad Funcional/fisiología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Desempeño Psicomotor/efectos de los fármacos , Reclutamiento Neurofisiológico/efectos de los fármacosRESUMEN
Projections of group II afferents from intrinsic foot muscles to lower limb motoneurones were investigated in humans after electrical stimuli were applied to the tibial nerve (TN) at ankle level, using modulation of the quadriceps H reflex, on-going EMG of the quadriceps and peroneus brevis, and PSTHs of single quadriceps, biceps, semitendinosus, tibialis anterior, and peroneus brevis motor units. TN stimulation evoked late and high-threshold excitation in all leg and thigh muscles investigated. The mean latency of the late excitation was 13.5+/-0.4 ms longer than that of the heteronymous monosynaptic Ia excitation, and the more caudal the motor nucleus the longer the central delay of the late effect, suggesting mediation through interneurones located rostral to motoneurones. The electrical threshold and conduction velocity of the largest diameter fibres evoking the late excitation were estimated to be approximately 2 and 0.67 times, respectively, those of the fastest Ia afferents, i.e. consistent with a mediation by group II afferents. Stimulation of the skin areas innervated by TN did not evoke late excitations. Further support for mediation through group II afferents was provided by the findings that: 1. the latency of the TN-induced late and high-threshold excitation in Per brev units was more delayed by cooling the nerve than that of the excitation evoked by group I afferents, and 2. tizanidine intake (known to depress selectively transmission of group II effects) suppressed the TN-induced late and high-threshold excitation whereas the group I facilitation was not modified.
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Pie/fisiología , Pierna/fisiología , Neuronas Motoras/fisiología , Músculo Esquelético/fisiología , Muslo/fisiología , Adulto , Vías Aferentes/fisiología , Anciano , Análisis de Varianza , Distribución de Chi-Cuadrado , Estimulación Eléctrica/métodos , Reflejo H/fisiología , Humanos , Persona de Mediana Edad , Tiempo de Reacción/fisiologíaRESUMEN
OBJECTIVE: To investigate the effect of tizanidine (an alpha(2) noradrenergic agonist) on transmission in the interneuronal pathway coactivated by group I and group II afferents in post-stroke patients with spastic hemiplegia. METHODS: Early and late facilitation of the quadriceps H reflex elicited in the common peroneal nerve--attributed to non-monosynaptic group I and group II excitation, respectively--was investigated in 14 spastic hemiplegic patients. All received a single dose of tizanidine (150 microg/kg) or placebo in randomised order at 10 day intervals. Repeated measurements were made at baseline (T0), 45-90 min, and 120 min after drug intake. Spasticity was assessed by modified Ashworth score in the quadriceps muscle and by a leg tone score calculated by the sum of the modified Ashworth score in five muscle groups. RESULTS: On the spastic side a decrease in late group II and, to a lesser extent, early group I common peroneal nerve induced quadriceps H reflex facilitation occurred with tizanidine (group II, mean (SEM) difference T0-T90: 34.3 (10.2)%, p<0.001; group I, T0-T120: 19.8 (9)%, p<0.05), but not with placebo (group II, difference T0-T90: 12.5 (8)%, NS; group I, T0-T120: 3.2 (7)%, NS). Tizanidine but not placebo decreased the quadriceps muscle and global lower limb Ashworth scores (2.9 (0.2) to 1.9 (0.3), p<0.001; and 12 (0.7) to 9.5 (0.8), p<0.0001, respectively). CONCLUSIONS: Enhancement of group II-group I facilitation of the quadriceps motor neurones on the spastic side of hemiplegic patients is modulated by alpha(2) noradrenergic agonists. This strengthens the view that late facilitation of quadriceps motor neurones is mediated by group II afferents and suggests that group II pathways may be involved in lower limb spasticity.
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Agonistas alfa-Adrenérgicos/farmacología , Clonidina/análogos & derivados , Clonidina/farmacología , Hemiplejía/tratamiento farmacológico , Hemiplejía/fisiopatología , Neuronas Motoras/efectos de los fármacos , Neuronas Motoras/fisiología , Conducción Nerviosa/efectos de los fármacos , Administración Oral , Agonistas alfa-Adrenérgicos/administración & dosificación , Adulto , Anciano , Clonidina/administración & dosificación , Femenino , Hemiplejía/etiología , Humanos , Pierna/inervación , Masculino , Persona de Mediana Edad , Placebos , Accidente Cerebrovascular/complicacionesRESUMEN
We report a case of cervical dystonia mimicking dropped-head syndrome (DHS) in a 57-year-old man treated for laryngeal carcinoma by radiotherapy (74.4 Gy) 3 months before. Cervical computerized tomographic scan and magnetic resonance imaging (MRI) did not find any muscle fat changes but found a high-intensity signal on T2 weighted images in the cervical spinal cord. Clinical and electromyographic findings were consistent with cervical dystonia. A trapezius biopsy was normal. Spontaneous remission of the dystonia was observed for 1 month whereas the laryngeal carcinoma progressed. The link between cervical dystonia and radiotherapy might be acute radiation-induced damage to the cervical spinal cord.
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Carcinoma de Células Escamosas/radioterapia , Neoplasias Laríngeas/radioterapia , Traumatismos por Radiación/etiología , Médula Espinal/efectos de la radiación , Tortícolis/etiología , Carcinoma de Células Escamosas/patología , Electromiografía/efectos de la radiación , Estudios de Seguimiento , Humanos , Neoplasias Laríngeas/patología , Masculino , Persona de Mediana Edad , Músculos del Cuello/inervación , Examen Neurológico/efectos de la radiación , Traumatismos por Radiación/diagnóstico , Remisión Espontánea , Tortícolis/diagnósticoRESUMEN
BACKGROUND: Intradermal injections of type A botulinum toxin have been reported to reduce excessive sweating in patients with primary palmar hyperhidrosis. Two preparations are commercially available in Europe: Botox (Allergan; 100 U per vial) and Dysport (Beaufour Ipsen Biotech; 500 U per vial), which are not bioequivalent. A few studies have tried to find an appropriate conversion factor between the two preparations in dystonic patients but results remain controversial. OBJECTIVES: To compare the efficacy of Botox and Dysport in palmar hyperhidrosis using a conversion factor of 1 : 4. METHODS: In a double-blind, randomized study, eight patients with severe primary palmar hyperhidrosis received in the same session intradermal injections of Dysport in one palm and Botox in the other, after regional median and ulnar nerve blocks. Quantification of sweat production was performed by Minor's iodine starch test at baseline, 1, 3 and 6 months after the treatment. Subjective assessment of sweat production was performed using a visual analogue scale. RESULTS: The mean +/- SD number of injection sites (28 +/- 1), mean volume of reconstituted solution injected (2.8 mL) and mean sweating area at baseline (BSA) were similar in each palm group. The mean +/- SD dose injected was 69.3 +/- 3.1 U for the Botox-treated palms and 283.7 +/- 11.3 U for the Dysport-treated palms (1 : 4). At 1 month, Minor's test revealed significant decreases in mean sweating area for each preparation (Dysport palms: -78.6% vs. BSA, P = 0.0002; Botox palms: -56.6% vs. BSA, P = 0.003). The percentage of decrease was more pronounced in Dysport palms compared with Botox palms but the difference did not reach statistical significance. At 3 months, the decrease in sweating area remained significant for Dysport palms (-69.4% vs. BSA, P = 0.008) but not for Botox palms (-48.8% vs. BSA). Self-evaluation showed a similar amount of improvement in both palm groups at 1 and 3 months (77% and 75% for Dysport; 68% and 72% for Botox). Local side-effects were more frequent in Dysport palms (weakness of thumb-index pinch in four cases, lasting 8-30 days) than in Botox palms (weakness of thumb-index pinch in two cases, lasting 15-21 days). The mean duration of positive effect was similar: 17 weeks in Dysport (range 8-32) and 18 weeks in Botox palms (range 8-32). CONCLUSIONS: Using a conversion factor of 1 : 4, the efficacy of Botox and Dysport injections was similar. However, there was a trend towards a larger improvement after Dysport treatment but with a higher incidence of adverse effects.
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Toxinas Botulínicas Tipo A/uso terapéutico , Dermatosis de la Mano/terapia , Hiperhidrosis/terapia , Fármacos Neuromusculares/uso terapéutico , Adulto , Toxinas Botulínicas Tipo A/efectos adversos , Método Doble Ciego , Femenino , Humanos , Inyecciones Intradérmicas , Yodo , Masculino , Fármacos Neuromusculares/efectos adversos , Satisfacción del Paciente , Equivalencia Terapéutica , Resultado del TratamientoRESUMEN
Though intraoperative neurophysiology is essential to precisely define the definitive target, little is known regarding its predictive value in defining the most effective contact for chronic deep brain stimulation. In this retrospective study, we reviewed the correlation between intraoperative neurophysiology and contacts selected for chronic stimulation. Twenty consecutive patients implanted for subthalamic nucleus (STN) stimulation were reviewed. There was no significant correlation between the electrophysiologically defined STN and the most effective contact for chronic stimulation at 3 months or at 6 months. Furthermore, there was a discrepancy between the most effective contact for rigidity versus akinesia or tremor at 3 months. Interestingly, at 3 months, the same electrode contact was maximally efficient for rigidity, akinesia and tremor in only 13 of the 39 cases. This lack of correlation did not affect the global improvement.
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Terapia por Estimulación Eléctrica/métodos , Enfermedad de Parkinson/cirugía , Enfermedad de Parkinson/terapia , Núcleo Subtalámico/cirugía , Adulto , Anciano , Electrodos Implantados , Femenino , Humanos , Masculino , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Enfermedad de Parkinson/fisiopatología , Resultado del TratamientoRESUMEN
A potent heteronymous excitation of quadriceps motoneurones via common peroneal group II afferents has recently been demonstrated in normal subjects. The aim of this study was to investigate whether this group II excitation contributes to rigidity in Parkinson's disease. The early and late facilitations of the quadriceps H reflex elicited by a conditioning volley to the common peroneal nerve (CPN) at twice motor threshold, attributed to non-monosynaptic group I and group II excitations, respectively, were investigated. The comparison was drawn between results obtained in 20 "de novo" patients with Parkinson's disease (hemiparkinsonian, 17; bilateral, three) and 20 age-matched normal subjects. There was no statistically significant effect of "group" (patients/controls), "duration", "global severity" [Unified Parkinson's Disease Rating Scale (UPDRS)] or "side" (unilaterally versus bilaterally affected) factors on either group I or group II facilitations. To further the analysis, the factors of status (affected or non-affected limb), akinesia (lower limb akinesia score) and rigidity (lower limb rigidity score) were entered in a general linear model to explain the variations of the quadriceps H reflex facilitation. Rigidity was the only factor useful in predicting the value of the group II facilitation of the quadriceps H reflex (P < 0.007). Group I and group II facilitation was then compared between the rigid, non-rigid and control lower limbs [multivariate analysis of variance (MANOVA)]. Results are represented as mean +/- SEM (standard error of the mean). Group II facilitation was enhanced in the rigid lower limb of unilaterally affected patients (153.2 +/- 7% of control H reflex) compared with non-rigid lower limbs (124 +/- 4% of control H reflex; P < 0.007) or control lower limbs (126.1 +/- 4.1%; P < 0.01). There was no difference between the non-rigid lower limbs of the unilaterally affected patients and the control lower limbs, but a difference was observed between the rigid lower limbs of unilaterally less affected and bilaterally more affected patients (153.2 +/- 7% and 123.8 +/- 7.5% of control H reflex, respectively; P < 0.04). These results suggest a facilitation of the transmission in the interneuronal pathway activated by group II afferents in rigid lower limb of de novo hemiparkinsonian patients, probably resulting from a change in their descending monoaminergic inhibitory control.
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Neuronas Motoras/fisiología , Rigidez Muscular/fisiopatología , Músculo Esquelético/inervación , Enfermedad de Parkinson/fisiopatología , Nervio Peroneo/fisiopatología , Transmisión Sináptica , Adulto , Anciano , Condicionamiento Psicológico , Estimulación Eléctrica , Electromiografía , Femenino , Reflejo H , Humanos , Pierna , Masculino , Persona de Mediana Edad , Rigidez Muscular/etiología , Neuronas Aferentes/fisiología , Enfermedad de Parkinson/complicaciones , MusloRESUMEN
BACKGROUND: Familial adult myoclonic epilepsy (FAME) is defined by autosomal dominant inheritance, adult onset of myoclonus of the extremities, infrequent epileptic seizures, nonprogressive course, abnormality of polyspikes and waves on examination by EEG and photosensitivity, giant somatosensory evoked potentials, enhancement of C reflex, and premyoclonus spike detected by means of the jerk-locked averaging EEG method. These findings were also observed in patients with benign adult familial myoclonic epilepsy (BAFME) and patients with familial cortical tremor. FAME and BAFME have been described only in Japan. The genes responsible for FAME and BAFME were mapped in the same genetic interval in 8q22.3-q24.1 OBJECTIVE: To study clinical and genetic characteristics of a European family with FAME. METHODS: A four-generation European kindred presenting with FAME, including 18 members, is described. Clinical analysis was performed on 15 living subjects and electrophysiologic study on 5 patients. Linkage analysis was performed with fluorescent microsatellites encompassing the FAME/BAFME locus (8q23.3-q24.1). RESULTS: Ten living and three deceased relatives had the clinical characteristics of FAME. Mean age at onset of the 10 living patients was 41 years (range, 30-60 years). Eight of the 13 affected subjects had generalized tonic-clonic seizures. Electrophysiologic studies confirmed the diagnosis of FAME in the five patients studied. The pattern of inheritance was consistent with an autosomal dominant inheritance. The locus responsible for FAME/BAFME was excluded. CONCLUSION: Observation of a European family extends the occurrence of familial adult myoclonic epilepsy to non-Japanese patients. Exclusion of linkage of this family to the locus for familial adult myoclonic epilepsy/benign adult familial myoclonic epilepsy established the genetic heterogeneity of this disorder.
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Cromosomas Humanos Par 8/genética , Epilepsias Mioclónicas/genética , Ligamiento Genético/genética , Adulto , Anciano , Mapeo Cromosómico/estadística & datos numéricos , Electroencefalografía , Electromiografía , Electrofisiología , Epilepsias Mioclónicas/fisiopatología , Europa (Continente) , Femenino , Humanos , Masculino , Persona de Mediana Edad , LinajeRESUMEN
1. Group I projections from intrinsic plantar muscles to motoneurones (MNs) of human leg and thigh muscles were investigated. Changes in firing probability of single motor units (MUs) in the tibialis anterior (TA), peroneus brevis (Per brev), soleus (Sol), gastrocnemius medialis (GM), vastus lateralis (VL), semitendinosus (ST) and biceps (Bi) were studied after electrical stimuli applied to: (i) the tibial nerve (TN) at ankle level, (ii) the corresponding homonymous nerve, and (iii) the skin of the heel, to mimic the TN-induced cutaneous sensation. 2. Homonymous facilitation, attributable to monosynaptic Ia excitation, was found in all the sampled units. Early heteronymous excitation elicited by TN stimulation was found in many MUs. Later effects (3-5 ms central delay) were bigger and more frequently observed: excitation in most TA and Per brev MUs, and inhibition in most Sol, GM and Bi MUs and in many ST and VL MUs. The low threshold (approximately 0.5-0.6 x motor threshold) and the inability of a pure cutaneous stimulation to reproduce these effects (except the late excitation in TA MUs) indicate that they were due to stimulation of group I muscle afferents. 3. The early excitation was accepted to be monosynaptic when its central delay differed from that of the homonymous Ia excitation by less than 0.5 ms. Such a significant TN-induced monosynaptic Ia excitation was found in MUs belonging to all leg and thigh motor nuclei tested. Although its mean strength was relatively weak, it is argued that these monosynaptic connections might affect already depolarized MNs. 4. The late excitation found in TA and Per brev MUs is argued to be mediated through interneurones located rostral to MNs. 5. The late suppression, found in most Sol, GM and Bi MUs, and in many ST and VL MUs, was the dominant effect. It was accompanied by an inhibition of the Sol and quadriceps H reflexes at rest, and therefore reflects an inhibition directed to MNs. Its long latency is argued to reflect transmission by interneurones located rostral to MNs (the inhibitory counterpart of non-monosynaptic excitation). 6. The functional implications of these connections are discussed with respect to the requirements of the stance phase of human walking and running.
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Pie/inervación , Neuronas Motoras/fisiología , Músculo Esquelético/inervación , Adulto , Anciano , Electromiografía , Pie/fisiología , Marcha/fisiología , Reflejo H/fisiología , Humanos , Persona de Mediana Edad , Músculo Esquelético/fisiología , Conducción Nerviosa/fisiología , Neuronas Aferentes/fisiología , Nervio Tibial/citología , Nervio Tibial/fisiologíaRESUMEN
OBJECTIVE: A potent heteronymous group II excitation of quadriceps motor neurons has been recently demonstrated in normal subjects. The present study was undertaken to investigate whether this heteronymous group II excitation also contributes to spasticity in hemiplegic patients. METHOD: The early and late facilitations of the quadriceps H reflex elicited by a conditioning volley to the common peroneal nerve at three times motor threshold, attributed to non-monosynaptic group I and group II excitations respectively, were investigated. The comparison was drawn between results obtained in 20 patients after stroke, with hemiplegia due to a vascular lesion in the territory of the middle cerebral artery, and 20 age and sex matched normal subjects. RESULTS: A significant increase in the group I as well as in the group II common peroneal nerve induced facilitation of the quadriceps H reflex was seen on the spastic side of the patients (group I: 159 (SEM 10)% of control H reflex; group II: 165 (SEM 8)%) compared with their unaffected side (group I: 126 (SEM 4)%; group II: 128 (SEM 5)%) (Wilcoxon signed rank test, p<0. 01), or to the right (group I: 132 (SEM 4)%; group II: 131 (SEM 5)%) or left (group I: 130 (SEM 3)%; group II: 135 (SEM 6)%) side of controls (Mann-Whitney U test, p<0.01). No significant correlation (Spearman rank test) was found between the degree of group I and group II induced facilitations on the spastic side of the patients and the degree of clinically assessed spasticity (Ashworth scale). CONCLUSION: These results reflect a facilitation of the transmission in the interneuronal pathway coactivated by group I and group II afferents, probably resulting from a change in their descending control in spastic hemiplegic patients.
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Hemiplejía/fisiopatología , Adulto , Vías Aferentes/fisiopatología , Anciano , Análisis de Varianza , Electromiografía , Femenino , Reflejo H/fisiología , Humanos , Masculino , Persona de Mediana Edad , Músculos/fisiopatologíaRESUMEN
We performed a prospective study in 21 patients to evaluate the cost of treatment of spasmodic torticollis (cervical dystonia) before and after botulinum toxin type A (BTA) treatment and to assess the impact of BTA treatment on quality of life. Data were recorded for the analysis over a period starting 8 months before and ending 7.2 +/- 0.2 months (mean +/- SEM) after the first injection of BTA. All patients received at least two BTA injections (2.9 +/- 0.2 injections per patient). We studied direct medical costs (drugs, outpatient and inpatient visits, diagnostic procedures, physiotherapy), clinical effects of BTA (clinical rating scale and patient's global assessment), quality of life (French version of the Nottingham Health Profile [NHP]), and adverse reactions. Costs associated with the treatment of spasmodic torticollis before the first BTA injection were 479 +/- 143 French Francs (FF)/patient/month (97 +/- 29 US $/pt/mo). During BTA treatment, costs were 1,126 +/- 147 FF/pt/mo (228 +/- 30 US $/pt/mo), including a mean cost of BTA of 771 +/- 131 FF/pt/mo (157 +/- 27 US $/pt/mo). Treatment with BTA significantly decreased clinical symptoms of spasmodic torticollis and improved the emotional, social, and pain-related domains of the quality of life assessment. Botulinum toxin type A treatment increases the cost of treating spasmodic torticollis but improves quality of life in terms of pain, social, and psychologic functioning in patients with spasmodic torticollis.