Episodic hyperhidrosis as the only clinical manifestation of neuromyotonia.
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Aged↗
Biomedical subjects
J Valls-Solé
Publications and source records attributed to J Valls-Solé.
Changes in the sympathetic skin response after thoracoscopic sympathectomy in patients with primary palmar hyperhidrosis.
OBJECTIVE: To investigate whether thoracic sympathectomy induced any change in the pattern of abnormalities or in the waveform of the sudomotor skin response (SSR) in patients with primary palmar hyperhidrosis (PPH). METHODS: We recorded the SSR to median nerve electrical stimuli before and after bilateral thoracoscopic sympathectomy in 27 patients with PPH. We analyzed the changes in amplitude, type of waveform and pattern of abnormality. RESULTS: All patients reported symptomatic improvement. The amplitude of the SSR decreased significantly in patients examined within 1 year after surgery, but was not different in patients examined after 1 year. The number of abnormally enhanced responses reduced after surgery, but there was no significant change in the number of patients with enhanced excitability recovery or with double-peak responses to single stimuli. There was a significant increase in the number of SSRs with a predominantly negative waveform after surgery. CONCLUSIONS: The persistence of SSR abnormalities after surgery suggests that the central nervous system dysfunction is not modified by sympathectomy. The change of the waveform to predominantly negative type after surgery could be the consequence of the decrease in the production of sweating. SIGNIFICANCE: Our results show the effects of sympathectomy on the SSR and on its abnormal patterns in patients with PPH.
Adolescent↗
Sensorimotor integration in patients with parkinsonian type multisystem atrophy.
Sensorimotor integration is an essential feature of the central nervous system that contributes to the accurate performance of motor tasks. Some patients with multiple system atrophy with parkinsonian features (MSAp) exhibit clinical signs compatible with an abnormal central nervous system excitability to somatosensory inputs, such as action myoclonus or enhanced cutaneo-muscular reflexes. To investigate further the site where such dysfunction in sensorimotor integration takes place, we examined the inhibitory effects of a cutaneous afferent volley at two different levels of the motor system in 10 MSAp patients and in 10 age-matched healthy volunteers. Electrical digital nerve stimuli were given as the conditioning stimulus for the motor evoked potentials (MEP) elicited by transcranial magnetic stimulation in hand muscles, and for the blink reflex responses obtained in the orbicularis oculi muscles by supraorbital nerve stimulation. Intervals for the conditioning were 20 to 50 ms for the MEP and 90 to 110 ms for the blink reflex. The MEP was significantly inhibited in test trials in healthy volunteers, reaching a mean of 32% of the baseline values at the ISI of 35 ms. Significant inhibition occurred also in the blink reflex, in which the R2 response was a mean of 12% of baseline values at the ISI of 100 ms. The inhibitory effects were abnormally reduced in 8 patients on the MEP, and in 7 patients on the blink reflex. There were significant group differences between patients and control subjects in the size of the conditioned MEP and blink reflex. These results suggest that sensorimotor integration is abnormal in patients with MSAp in at least two central nervous system sites: the sensorimotor cortex, and the brainstem reticular formation.
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Ballistic reactions under different motor sets.
In preparation for performing task specific ballistic movements, subjects may choose among different possibilities for setting up their motor apparatus, ranging from quiet resting to different types of muscle activation. In the study presented here, we investigated whether differences in the motor set modify either the reaction time or the kinematic characteristics of the movement. Subjects wearing surface EMG recording electrodes in the wrist extensor (WE) and wrist flexor (WF) muscles were requested to react to the presentation of a visual stimulus by performing a ballistic wrist extension movement of an amplitude of about 50 degrees in the following experimental conditions: resting quietly, which was considered as the control condition (CC); isometric contraction (IC), in which subjects were required to activate WE and WF muscles isometrically; rapid oscillations (RO), in which subjects were requested to make a fast oscillatory wrist movement; and slow oscillations (SO), in which subjects were maintaining a slow oscillatory motion of the wrist. To constrain the movement to the wrist joint and limit the action of postural muscles, the subject's forearm and hand were attached to joined non-resistive metallic platforms, allowing for free non-frictional displacement. In the EMG recordings, we measured the size of the EMG bursts in agonist and antagonist muscles, and the inter-burst intervals. In movement recordings, we measured movement onset latency and the velocity profile. Movement onset was delayed in SO with respect to all other conditions. Conversely, peak velocity was larger in all test conditions in comparison to CC. There were no differences in the size of the first EMG burst of the agonist muscle, but significant changes occurred in the subsequent bursts recorded in the agonist and antagonist muscles. Our study indicates that the motor program used to execute a ballistic voluntary movement is influenced by the conditions of the motor system. The configuration of the motor set should be specifically considered in the search for improving the speed of the reaction and the kinematics of ballistic movements.
Action Potentials↗
The effects of a startle on awareness of action.
The execution of a ballistic movement within a reaction time task paradigm is significantly speeded up when an unexpected startling auditory stimulus (SAS) is delivered together with the imperative signal. Using Libet's clock, we investigated whether acceleration involves also the subjective appraisal of the time of task execution. In trials containing the SAS, reaction time shortened to 68.7% of control values. However, subjective judgment of task execution remained a similar time with respect to the imperative signal as in control trials. The dissociation between task execution and its subjective perception indicates the existence of separate circuits for action execution and action awareness.
Acoustic Stimulation↗
Abnormalities of prepulse inhibition do not depend on blink reflex excitability: a study in Parkinson's disease and Huntington's disease.
OBJECTIVE: Prepulse inhibition of the blink reflex is a robust phenomenon with an interesting physiology and a large potential for clinical applicability. In the study presented here we investigated whether the blink reflex inhibition by a prepulse (BRIP) is influenced by the blink reflex excitability recovery (BRER). METHODS: The study was undertaken in 20 patients with Parkinson's disease (PD), 20 patients with Huntington's disease (HD) and 20 healthy volunteers. BRER was determined by measuring the size of the response to a test supraorbital nerve stimulus as a percentage of the response to a conditioning stimulus at inter-stimuli intervals of 100-1000 ms. BRIP was determined as the percentage reduction induced in the response to a supraorbital nerve stimulus by either a low intensity auditory click or a weak third finger somatosensory stimulus, applied with a leading interval of 50-110 ms. RESULTS: There was a negative correlation between the percentage BRER and the percentage BRIP (Pearson's correlation coefficient of -0.37). BRER was enhanced in 14 PD patients (70%) and 6 HD patients (30%), while it was depressed in 10 HD patients (50%). BRIP was significantly reduced in 15 PD patients (75%) and 16 HD patients (80%). No significant correlation was found between abnormally enhanced BRER and abnormally reduced BRIP in all patients as a group (chi(2)=2.4;P=0.11). A weak correlation was found in PD patients (P=0.019) and no correlation was observed in HD patients (P=0.8). CONCLUSIONS: Our results indicate that an abnormally reduced BRIP was not always accompanied by an abnormally enhanced BRER in patients with HD. The two tests likely assess specific and distinct brainstem functions, and provide different types of information. While BRIP may be the result of a widespread integrative processing of sensory stimuli, BRER likely reflects the excitability of a chain of brainstem inter-neurons. SIGNIFICANCE: BRER and BRIP provide independent information on the state of functionally separate circuits that converge on trigemino-facial brainstem inter-neurons.
Acoustic Stimulation↗
Usefulness of neurophysiologic techniques in stereotactic subthalamic nucleus stimulation for advanced Parkinson's disease.
OBJECTIVE: The objectives of this study are to determine the impact of neurophysiologic guidance on subthalamic nucleus (STN) targeting and to assess its safety and effectiveness. METHODS: We have compared the initial theoretic anatomic target (TAT) of the STN with the final microrecording guided coordinates in 15 consecutive patients with bilaterally implanted electrodes in the STN. The clinical results and adverse effects are also reported. All comparisons were done through a paired Student's t test and Pearson's correlation test. RESULTS: Neurophysiological guidance changed the target coordinates in 26 of the procedures. The mean correction applied to the TAT in order to place the electrode in its definite location was 0.4 mm (+/-0.8, range 0-3; P=0.03) in the medial-lateral axis, 1.6 mm (+/-1.2, range 0-5; P=0.01) in the anterior-posterior plane and 0.8 mm (+/-0.8, range 0-3; P=0.26) in the vertical axis. The mean number of microrecording tracks employed to localize each STN was 2.8+/-1.8 (range 1-8) tracks. After surgery, the total UPDRS motor score in the off medication condition improved by 65.9%; UPDRS-II scores were reduced by 71.8% and Schwab and England scores improved by 45.3%. No intraoperative hemorrhages occurred in this series. CONCLUSIONS: Neurophysiological guidance is a safe and useful tool in order to improve and confirm target localization. The correction applied in the target resulted in a significant clinical improvement 6 months after surgery.
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Electrophysiological approach to the study of essential tremor in children and adolescents.
Surface electromyography and accelerometry provide essential information on the neurophysiological characteristics of essential tremor. There are many reports on neurophysiological features in adult-onset essential tremor, but to our knowledge there have been no similar investigations of essential tremor in children. We conducted a neurophysiological study of nine children, six males and three females, with definite essential tremor. They were subdivided into two groups according to age: a 'children's group', consisting of four patients aged from 7 to 12 years, and an 'adolescent group', consisting of five patients aged from 14 to 16 years. Finger tremor as opposed to hand tremor was studied. In children the mean tremor frequency was 5.3 Hz (SD 0.5) with arms extended, which increased to 8.2 Hz (SD 1.5) when we added a mass of 300 g. In adolescents the mean tremor frequency was 9.0 Hz (SD 1.4) with arms extended, and 7.2 Hz (SD 1.8) with added mass. We discuss several hypotheses to find an explanation for these results.
Adolescent↗
Role of brainstem-spinal projections in voluntary movement.
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Brain Stem↗
Long-term follow-up of diabetic polyneuropathy after simultaneous pancreas and kidney transplantation in type 1 diabetic patients.
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Action Potentials↗
Nociceptive quality of the laser-evoked blink reflex in humans.
Laser radiant-heat pulses selectively excite the free nerve endings in the superficial layers of the skin and activate mechano-thermal nociceptive afferents; when directed to the perioral or supraorbital skin, high-intensity laser pulses evoke a blink-like response in the orbicularis oculi muscle (the laser blink reflex, LBR). We investigated the functional properties (startle or nociceptive origin) of the LBR and sought to characterize its central pathways. Using high-intensity CO(2)-laser stimulation of the perioral or supraorbital regions and electromyographic (EMG) recordings from the orbicularis oculi muscles, we did five experiments in 20 healthy volunteers. First, to investigate whether the LBR is a startle response, we studied its habituation to expected rhythmic stimuli and to unexpected arrhythmic stimuli. To assess its possible nociceptive quality, we studied changes in the LBR and the R2 component of the electrical blink reflex after a lidocaine-induced supraorbital nerve block and after intramuscular injection of the opiate fentanyl and the opiate-antagonist naloxone. To characterize the central pathways for the LBR, we investigated the interaction between the LBR and the three components of the blink reflex (R1, R2, and R3) by delivering laser pulses to the perioral or supraorbital regions before or after electrical stimulation of the supraorbital nerve at various interstimulus intervals. Finally, to gain further information on the central LBR pathways, using two identical CO(2)-laser stimulators, we studied the LBR recovery curves with paired laser pulses delivered to adjacent forehead points at interstimulus intervals from 250 ms to 1.5 s. The LBR withstood relatively high-frequency rhythmic stimulations, and unexpected laser pulses failed to evoke larger responses. When lidocaine began to induce hypoalgesia (about 5 min after the injection), the LBR was abolished, whereas R2 was only partly suppressed 10 min after the injection. Fentanyl injection induced strong, naloxone-reversible, LBR suppression (the response decreased to 25.3% of predrug values at 10 min and to 4% at 20 min), whereas R2 remained appreciably unchanged. Whether directed to the perioral or supraorbital regions, preceding laser pulses strongly suppressed R2 and R3 though not R1. Conversely, preceding electrical stimuli to the supraorbital nerve suppressed the LBR. In response to paired stimuli, the LBR recovered significantly faster than R2. These findings indicate that the LBR is a nociceptive reflex, which shares part of the interneuron chain mediating the nonnociceptive R2 blink reflex, probably in the medullary reticular formation. The LBR may prove useful for studying the pathophysiology of orofacial pain syndromes.
Adult↗
Influence of gender on auditory startle responses.
The auditory startle reaction is considered a brainstem reflex in response to an unexpected loud stimulus. It may be abnormal in various neurological conditions. However, the influence of gender on physiological characteristics of auditory startle responses (ASRs) in humans has to date been studied only in orbicularis oculi muscle. We investigated 54 healthy adult subjects (27 males, 27 females). ASRs were elicited by binaural high-intensity auditory stimuli which differed randomly in tonal frequency and intensity (250 Hz-90 db; 500 Hz-105 dB; 750 Hz-110 db, 1000 Hz-110 dB nHL), presented through tubal insert phones. Reflex electromyographic activity was simultaneously recorded with surface electrodes from masseter, orbicularis oculi, sternocleidomastoid, biceps brachii, abductor pollicis brevis, rectus femoris, tibialis anterior, and soleus muscles. ASR probability was significantly lower, and ASR area under the curve was significantly smaller, in men versus women. Median onset latencies did not differ significantly, but tended to be shorter in the lower extremities of men despite greater body height. Habituation, measured as a reduction in response probability with repeated stimulation, was significant in all muscles except orbicularis oculi in both men and women. Our data provide evidence for a significant influence of gender on ASR characteristics. The observed differences are likely due to gender-specific variations of central processing in the brainstem centers involved in ASR generation, and should be taken into account when testing ASRs in health and disease.
Acoustic Stimulation↗
Influence of age on auditory startle responses in humans.
The auditory startle reaction is considered a brainstem reflex in response to an unexpected loud stimulus. We investigated the influence of age on auditory startle responses (ASRs) in 54 adult healthy subjects separated in three age groups (below 30; 30 to 50; above 50 years). ASRs were elicited by auditory stimuli randomly presented through tubal insert phones. Reflex electromyographic activity was simultaneously recorded from eight facial, neck, and extremity muscles. ASR probability was lower in extremity muscles of younger versus older subjects, but did not differ among age groups in facial and neck muscles. Median ASR latencies were significantly shorter in all muscles of younger versus older subjects. Our data provide evidence of a significant influence of age on ASRs. The observed differences are likely due to age-specific variations of central processing in the brainstem centers involved in ASR generation.
Acoustic Stimulation↗
Small-vessel vasculitis surrounding a spared temporal artery: clinical and pathological findings in a series of twenty-eight patients.
OBJECTIVE: Occasionally, a temporal artery biopsy reveals small-vessel vasculitis (SVV) surrounding a spared temporal artery, the significance of which is unclear. We analyzed the final diagnosis in a series of patients with this condition and tried to identify histopathologic features with potential usefulness in predicting the ultimate diagnosis. METHODS: We performed a clinical and histopathologic review of 28 patients in whom SVV surrounding a spared temporal artery was the first histologic finding that led to the diagnosis of vasculitis. For comparison purposes, we analyzed the pattern of small vessel involvement in 30 patients with biopsy-proven giant cell arteritis (GCA). RESULTS: GCA was considered the most likely diagnosis in 12 patients, based on the absence of clinical evidence of additional organ involvement and normal findings on muscle biopsy and electrophysiologic study. Three patients had systemic necrotizing vasculitis (SNV), based on the demonstration of typical lesions on subsequent muscle, nerve, or kidney biopsy. After extensive evaluation, 4 patients remained unclassifiable. Nine patients were incompletely studied. Fibrinoid necrosis was significantly more frequent in patients with SNV (P = 0.0022), whereas involvement of vasa vasorum was more frequent in patients classified as having GCA (P = 0.022). No differences in the pattern of small vessel involvement were found in patients with SVV surrounding a spared temporal artery who were classified as having GCA compared with patients with biopsy-proven GCA. Granulocytes were observed at similar frequency in all conditions. CONCLUSION: SVV may be the only abnormal feature in a temporal artery biopsy and the only histologic evidence of vasculitis. The diagnosis of GCA can be reasonably established in most of these patients when there is no apparent evidence of additional organ involvement. However, when fibrinoid necrosis is observed or the temporal artery vasa vasorum are not involved, SNV must be extensively excluded.
Adult↗
The auditory startle reaction in parkinsonian disorders.
The auditory startle reaction to an unexpected loud stimulus is regarded as a brainstem reflex originating in the nucleus reticularis pontis caudalis and being distributed up the brainstem and down the spinal cord along slowly conducting pathways. Auditory startle responses (ASR) have been reported absent or reduced in progressive supranuclear palsy (PSP), and delayed in Parkinson's disease (PD), but normal in multiple-system atrophy (MSA). For the first time we studied ASR in patients fulfilling the clinical criteria of dementia with Lewy bodies (DLB) (n = 8), a neurodegenerative disorder characterized by cortical and subcortical depositions of Lewy bodies resulting in parkinsonism and progressive cognitive decline. For comparison, we also investigated patients with PD (n = 10), MSA (n = 7), PSP (n = 10), and age-matched healthy controls (n = 10). ASR were elicited by binaural high-intensity auditory stimuli. Surface electromyographic activity was simultaneously recorded from facial, upper, and lower extremity muscles. For each muscle, we assessed response probability and measured latency, amplitude, duration, and habituation rate. Patients with DLB had fewer and abnormally delayed ASR of low amplitude and short duration in extremity muscles compared to healthy controls. Furthermore, we confirm and extend previous findings of abnormal ASR in PSP and PD, and also demonstrate exaggerated ASR in extremity muscles of MSA patients. The different patterns of ASR abnormalities may reflect distinct types of brainstem dysfunction in DLB.
Acoustic Stimulation↗
Reciprocal changes of excitability between tibialis anterior and soleus during the sit-to-stand movement.
The excitability of spinal motoneurons is modified by central preparatory commands before muscle activation. In relatively complex long duration motor tasks such as the sit-to-stand (STS) movement, the central nervous system commands have to take into account the inputs from muscle, skin, and joint afferents during muscle contraction. We have investigated the changes occurring in tibialis anterior (TA) and soleus (SOL) motoneuronal excitability prior to and during the STS movement in normal subjects. Twelve healthy volunteers received the instruction to rise from a chair at the perception of an acoustic 'go' signal. Cortical transcranial magnetic stimuli (TMS) or peripheral nerve electrical stimuli (PNS) were used as test stimuli to elicit, respectively, the motor evoked potential (MEP) and the H reflex, at intervals of 50-1500 ms after the 'go' signal. Both the MEP and the H reflex were enhanced in the TA between 100 and 900 ms after the 'go' signal. At the same time there was inhibition of the H reflex but not of the MEP in the SOL. At the end of the STS movement, during quiet standing, the size of both the H reflex and the MEP of the TA were not different from those obtained in the sitting position. However, in SOL, the H reflex was smaller, and the MEP was larger, than at rest. Our observations suggest the participation of several mechanisms of control of motoneuronal excitability during the STS, ultimately leading to a dominant role of presynaptic inhibitory mechanisms in SOL during standing.
Adult↗
Enhanced gain of blink reflex responses to ipsilateral supraorbital nerve afferent inputs in patients with facial nerve palsy.
OBJECTIVES: Patients with peripheral facial palsy (PFP) may present with transient hyperkinetic movement disorders in the side contralateral to the paralysis. One possible cause of such enhanced motor activity is sensitization of reflex responses to afferent inputs from the unprotected cornea. We hypothesized that if this sensitization occurs, the size of the orbicularis oculi (OOc) responses induced by afferents from the ophthalmic branch of the paralyzed side would be larger than those induced by afferents from the contralateral side. METHODS: In 68 patients with complete PFP and in a group of 30 age-matched control subjects we recorded the response of the OOc muscle of one side to electrical stimulation of the supraorbital nerve of both sides, and calculated the ratio between R2c and R2 (R2c/R2). RESULTS: The mean R2c/R2 ratio was significantly larger in patients than in control subjects (unpaired t test, P<0.05). Larger R2c than R2 responses were observed in 23.1% of control subjects and in 80.9% of patients (chi(2)=13.3, P<0.01). CONCLUSIONS: Our results suggest that patients with PFP have an enhanced blink reflex gain to inputs from the paralyzed side compared to those of the non-paralyzed side. Sensitization of the blink reflex polysynaptic pathways to inputs carried by afferent fibers from the ophthalmic branch of the paralyzed side can play a role in inducing an abnormal facial motor behavior after PFP.
Adult↗
Examination of motor output pathways in patients with corticobasal ganglionic degeneration using transcranial magnetic stimulation.
The alien hand sign (AHS) is often encountered in patients with corticobasal ganglionic degeneration (CBGD), revealing a unilateral dysfunction of the motor system of unknown pathophysiology. We examined the possibility of an abnormal cortical representation of hand muscles in 10 patients with probable CBGD and a prominent AHS. Cortical maps were obtained from the responses to magnetic stimuli applied with a figure of eight coil at an intensity of 110% above motor threshold. For comparison, the same study was carried out in 10 normal volunteers, eight patients with Parkinson's disease and eight patients with Alzheimer's disease. AHS patients had a larger extension of the cortical map to stimulation of the hemisphere contralateral to the AHS in comparison with the ipsilateral hemisphere. Furthermore, in six patients, focal stimulation of the hemisphere ipsilateral to the AHS gave rise to ipsilateral responses, delayed by a mean of 7.7 +/- 2.2 ms with respect to those recorded in the same muscle to contralateral stimulation. None of the other patients or control subjects had ipsilateral responses. Our results indicate an enhanced excitability, or reduced inhibition, of the motor area of the hemisphere contralateral to the AHS. The delay of the ipsilateral responses is compatible with a disinhibited transcallosal input.
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