RESUMO

Our aim was to study motor unit number index (MUNIX) in myopathic disorders. We studied 11 patients with myopathy, and healthy controls. We obtained MUNIX, compound muscle action potential (CMAP), motor unit size index (MUSIX) and alpha (α, power exponent from MUNIX equation) measurements from three different muscles. MUNIX and CMAP were significantly lower in one muscle. This MUNIX decrease may not be related to motor neuron loss, but rather to muscle fiber atrophy. MUSIX and α did not change and may be useful in determining whether the MUNIX decrease is indeed due to motor unit loss.

Assuntos

RESUMO

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement. CASE REPORT: We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained. CONCLUSIONS: Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie's pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.

Assuntos

RESUMO

INTRODUCTION: In this study we aimed to determine the contribution of the E2 (reference electrode) to the compound muscle action potential (CMAP) amplitude during fibular motor recording to the tibialis anterior (TA) when E2 is placed over routine referential vs. alternative sites. METHODS: The CMAP was obtained from 10 healthy subjects, using the active electrode (E1) over sites routinely used as E2 for the TA, whereas the E2 was over the contralateral knee. The same procedure was performed with the E1 over alternative E2 sites. RESULTS: Significant electrical signal was captured over routine E2 placement sites. Among the tested alternative E2 sites, the ipsilateral patella (especially its medial aspect) was the most electrically silent. DISCUSSION: Using alternative E2 sites with near isoelectric recordings can optimize near-field potential measurement in the fibular motor recording to the TA and represents a more accurate way of measuring nerve and muscle function. Muscle Nerve 59:249-253, 2019.

Assuntos

RESUMO

INTRODUCTION: Our objective was to determine the utility of motor unit number index (MUNIX) and neurophysiological index (NI) as surrogate biomarkers of disease progression in limbs without clinical signs of lower motor neuron (LMN) involvement from patients with slowly progressive amyotrophic lateral sclerosis (ALS). METHODS: Patients with slowly progressive ALS and at least 1 clinically unaffected limb were prospectively enrolled. Clinical signs of LMN loss and results from hand-held dynamometer (HHD), revised ALS Functional Rating Scale (ALSFRS-R), mean-MUNIX (from 3 different muscles), and NI were longitudinally recorded. RESULTS: Eighteen patients with 43 presymptomatic muscles were evaluated. Twenty-seven muscles remained clinically unaffected during study, with stable ALSFRS-R subscores and HHD measures. However, a significant decline in mean-MUNIX and NI was detected. DISCUSSION: Mean-MUNIX and NI were more sensitive than clinical measures at detecting LMN loss in presymptomatic limbs from patients with slowly progressive ALS. Therefore, these electrophysiological biomarkers should be included in early study phases as meaningful outcome measures. Muscle Nerve 58: 204-212, 2018.

Assuntos

RESUMO

INTRODUCTION: Reproducibility is an important aspect of any method intended to be a marker of disease progression. In this study we investigated approaches for improving motor unit number index (MUNIX) reproducibility. METHODS: We used the intraclass correlation coefficient (ICC) and the coefficient of variation (CV) to study reproducibility in healthy subjects. We tested reproducibility between test and retest of a single MUNIX from 3 different muscles (S-MUNIX) and also of the mean of a set of 3 measurements from these same muscles (M-MUNIX). RESULTS: M-MUNIX was more reproducible than S-MUNIX. The CV showed a greater improvement than the ICC in all 3 muscles. CONCLUSIONS: M-MUNIX may be a valuable approach for following motor unit loss, because it is more replicable than MUNIX. This may be especially relevant in amyotrophic lateral sclerosis patients, in whom MUNIX variability is higher than in healthy individuals. Muscle Nerve, 2016 Muscle Nerve 55: 635-638, 2017.

Assuntos

RESUMO

Peripheral neuropathy is frequent in spinocerebellar ataxia type 2 (SCA2), but the pattern and characteristics of nerve involvement are still an unsettled issue. This study aimed to evaluate the prevalence, extent, and distribution of nerve involvement in SCA2 patients through neurophysiological studies. Thirty-one SCA2 patients and 20 control subjects were enrolled in this study. All subjects were prospectively evaluated through electromyography, including nerve conduction, needle electromyography in proximal and distal muscles of the upper and lower limbs, and sural radial amplitude ratio (SRAR). We aimed to differentiate distal axonopathy from diffuse nerve commitment, characterizing neuronopathy. Nerve involvement was observed in 83.6 % (26 individuals) of SCA2 patients. Among these, 19 had diffuse sensory abnormalities on nerve conduction predominantly on the upper limbs, with diffuse chronic denervation on needle electromyography and elevated SRAR values. Four individuals had only diffuse sensory involvement, and 2 had only motor involvement on needle evaluation and normal nerve conduction. These were interpreted as neuronopathy due to the diffuse distribution of the involvement. One individual had distal sensory axonopathy, with lower limb predominance. In this study, we found neuronopathy as the main pattern of nerve involvement in SCA2 patients and that motor involvement is a frequent feature. This information brings new insights into the understanding of the pathophysiology of nerve involvement in SCA2 and sets some key points about the phenotype, which is relevant to guide the genetic/molecular diagnosis.

Assuntos

RESUMO

INTRODUCTION: Machado-Joseph disease is defined as an autosomal dominant ataxic disorder caused by degeneration of the cerebellum and its connections and is associated with a broad range of clinical symptoms. The involvement of the vestibular system is responsible for several symptoms and signs observed in the individuals affected by the disease. We measured cervical and ocular vestibular evoked myogenic potentials in a sample of Machado-Joseph disease patients in order to assess functional pathways involved. METHODS: Bilateral measures of cervical and ocular vestibular evoked myogenic potentials (cVEMP and oVEMP) were obtained from 14 symptomatic patients with genetically proven Machado-Joseph disease and compared with those from a control group of 20 healthy subjects. RESULTS: Thirteen (93%) patients showed at least one abnormal test result; oVEMP and cVEMP responses were absent in 17/28 (61%) and 11/28 (39%) measures, respectively; and prolonged latency of cVEMP was found in 3/28 (11%) measures. Of the 13 patients with abnormal responses, 9/13 (69%) patients showed discordant abnormal responses: four with absent oVEMP and present cVEMP, two with absent cVEMP and present oVEMP, and three showed unilateral prolonged cVEMP latencies. CONCLUSION: Both otolith-related vestibulocollic and vestibulo-ocular pathways are severely affected in Machado-Joseph disease patients evaluated by VEMPs.

Assuntos

RESUMO

A 23-year-old woman presented to our hospital with 9 months history of progressive ataxia, visual loss since childhood due to retinitis pigmentosa and primary amenorrhea. On examination, there were also sparse scalp hair, very long and curled upwards eyelashes and short stature. Oliver-McFarlane syndrome was suspected. Brain MRI disclosed cerebellar atrophy and hyperintense signal in corticospinal tracts on FLAIR and T2-weighted images. Therefore, brain imaging must be thoroughly investigated in patients with suspected Oliver-McFarlane syndrome, in order to determinate whether cerebellar atrophy and hyperintense signal in corticospinal tracts are part of this neurological condition.

Assuntos

RESUMO

INTRODUCTION: The Perception Sensory Threshold (ST) for sinusoidal current stimuli at 5, 250, and 2,000 Hz is commonly used in the assessment of peripheral nerve fibers (C, Aδ, and Aβ, respectively). However, the neuroselectivity of these frequencies is far from consensus. In addition, Reaction Time (RT) measurements suggest that 2,000 Hz stimuli excite Aβ-fibers, 250 Hz Aβ- or Aδ-fibers, as well as 5 Hz Aβ-, Aδ- or C-fibers. Therefore, we suppose that the sinusoidal current neuroselectivity may be better observed if ST and RT parameters are jointly evaluated. In addition, we have investigated whether there are other sets of frequencies that could be used. METHODS: Thus this work investigates ST and RT for stimuli with frequency ranging from 1 to 3,000 Hz, on 28 healthy subjects aged from 19 to 44 years old (27.1±5.49). ST and RT dissimilarity among different frequencies was evaluated applying bi-dimensional Fisher Quadratic Discriminant. RESULTS: The lowest classification error (3.6%) was obtained for 1, 250, and 3,000 Hz. Error for 5, 250, and 2,000Hz was 16.7%. Stimulation frequency at 1 Hz evoked more sensations related to C-fibers (53% of reports) than to Aβ-fibers (36%). However, this behavior did not repeat itself at 5 Hz (only 21% of perceptions were related to C-fibers against 64% to Aβ-fibers). Sensations related to Aβ-fibers prevailed for the highest frequencies presented to the subjects (2,000 Hz - 82% and 3,000 Hz - 93%). Mean RT values showed a decreasing trend with frequency. CONCLUSION: These results suggest that frequencies 1, 250, and 3,000 Hz are more neuroselective than 5, 250, and 2,000 Hz for the evaluation of peripheral sensitive fibers. Furthermore, they show RT usefulness.

RESUMO

OBJECTIVE: Neuropathy is a well-recognized feature in spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD), but the pattern of neuropathy is still a matter of debate. This study aimed to evaluate peripheral nerve involvement in MJD patients. Neurophysiological and clinical data were analyzed to distinguish neuronopathy from length-dependent distal axonopathy. METHODS: In the present study we evaluated 26 patients with clinical and molecular-proven MJD and investigated their peripheral nerve involvement. Neurophysiological and clinical data were compared and correlated aiming to distinguish neuronopathy from distal axonopathy. RESULTS: The neurophysiological evaluation showed that 42.3% of the patients had polyneuropathy. Among these patients, 81.8% presented neuronopathy. CONCLUSION: We concluded that neuronopathy is the most common form of peripheral nerve involvement in MJD patients.

Assuntos

RESUMO

INTRODUCTION AND HYPOTHESIS: Although still a matter of debate, stress urinary incontinence (SUI) may be accompanied by damage to urethral and pelvic floor innervations, thus promoting dysfunctions of the urethral support and sphincteric closure mechanisms. The aim of this study was to analyze the pelvic floor and urethral innervations through pelvic electrophysiological tests to identify whether neurological alterations interfere with urinary continence and urethral functional activity. METHODS: This prospective study included 52 women, 33 with clinically and urodynamically proven SUI and 19 continent volunteers matched for age, height, parity, and number of vaginal deliveries by the propensity score method. The patients were divided according to the severity of urinary loss evaluated by measuring abdominal leak point pressure (ALPP). Pudendal nerve terminal motor latency (PNTML), pudendal somatosensory evoked potential (SSEP) latencies, urethral and clitoral sensory thresholds, and urethroanal reflex latency were tested. RESULTS: SUI and control subjects did not differ in PNTML, SSEP latency, and clitoral sensory thresholds. However, reduced responsiveness to urethral electrosensitivity and prolonged urethroanal reflex latency were detected in most incontinent patients. In addition, urethral electrosensitivity was altered in suspected intrinsic sphincteric dysfunction. CONCLUSIONS: Urethral afferent pathways can be altered in women with SUI and may play an important role in evoking intrinsic sphincteric dysfunction.

Assuntos

RESUMO

OBJECTIVE: To evaluate a group of spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD) (SCA3/MJD) patients and assess whether there is an association between neuropathy and serum ferritin levels and restless legs syndrome (RLS). METHODS: Twenty-six SCA3/MJD patients underwent electromyography studies to check for neuropathy. Their serum ferritin levels were measured as well. These findings were evaluated based on the presence or not of RLS and its severity. RESULTS: The proportion of neuropathy in the RLS group was not significantly higher compared to the non-RLS group (23 vs. 15%, Fisher's exact test, p = 1.000). Furthermore, no association was found between RLS and ferritin levels. CONCLUSION: We found no correlation between neuropathy or ferritin levels and RLS in SCA3/MJD patients.

Assuntos

RESUMO

PURPOSE: We evaluated the somatic and autonomic innervation of the pelvic floor and rhabdosphincter before and after nerve sparing radical retropubic prostatectomy using neurophysiological tests and correlated findings with clinical parameters and urinary continence. MATERIALS AND METHODS: From February 2003 to October 2005, 46 patients with prostate cancer were enrolled in a controlled, prospective study. Patients were evaluated before and 6 months after nerve sparing radical retropubic prostatectomy using the UCLA-PCI urinary function domain and neurophysiological tests, including somatosensory evoked potential, and the pudendo-urethral, pudendo-anal and urethro-anal reflexes. Clinical parameters and urinary continence were correlated with afferent and efferent innervation of the membranous urethra and pelvic floor. We used strict criteria to define urinary continence as complete dryness with no leakage at all, not requiring any pads or diapers and with a UCLA-PCI score of 500. Patients with a sporadic drop of leakage, requiring up to 1 pad daily, were defined as having occasional urinary leakage. RESULTS: Two patients were excluded from study due to urethral stricture postoperatively. We evaluated 44 patients within 6 months after surgery. The pudendo-anal and pudendo-urethral reflexes were unchanged postoperatively (p = 0.93 and 0.09, respectively), demonstrating that afferent and efferent pudendal innervation to this pelvic region was not affected by the surgery. Autonomic afferent denervation of the membranous urethral mucosa was found in 34 patients (77.3%), as demonstrated by a postoperative increase in the urethro-anal reflex sensory threshold and urethro-anal reflex latency (p <0.001 and 0.0007, respectively). Six of the 44 patients used pads. One patient with more severe leakage required 3 pads daily and 23 showed urinary leakage, including 5 who needed 1 pad per day and 18 who did not wear pads. Afferent autonomic denervation at the membranous urethral mucosa was found in 91.7% of patients with urinary leakage. Of 10 patients with preserved urethro-anal reflex latency 80% were continent. CONCLUSIONS: Sensory and motor pudendal innervation to this specific pelvic region did not change after nerve sparing radical retropubic prostatectomy. Significant autonomic afferent denervation of the membranous urethral mucosa was present in most patients postoperatively. Impaired membranous urethral sensitivity seemed to be associated with urinary incontinence, particularly in patients with occasional urinary leakage. Damage to the afferent autonomic innervation may have a role in the continence mechanism after nerve sparing radical retropubic prostatectomy.

Assuntos

RESUMO

OBJECTIVE: Reproducibility testing of nerve conduction studies and F-wave latencies in a group of healthy volunteers. METHODS: A total of 32 healthy volunteers underwent sensory and motor nerve conduction studies of the ulnar and tibial nerves, including F-waves, elicited by 32 stimuli, repeated in two different days by the same examiner. RESULTS: The F-wave latencies showed less variability than the other parameters of conduction studies, and, among them, the F-wave mean latency of the tibial nerve has been the one with the higher reproducibility. CONCLUSIONS: F-wave latencies were the parameters with best reproducibility, followed by conduction velocities and amplitudes. SIGNIFICANCE: Our data showed that the F-wave mean latency is a parameter with a high reliability, considering the reproducibility, for sequential studies.

Assuntos

RESUMO

We report a 30-year-old man with moving ear syndrome caused by focal myoclonic jerks of the right temporal muscle. This focal myoclonus would disappear while the patient was sleeping, swallowing, or speaking. He was treated with botulinum toxin type A with a favorable outcome. Previous reports of this condition and possible therapeutic approaches are discussed.

Assuntos

RESUMO

Fibras finas (FF) aferentes primárias mediam a percepção de sensações relacionadas à dor e à temperatura, e as fibras grossas (FG) ao tato. O objetivo deste trabalho é avaliar percepções relacionadas às FF (picada, pontada, agulhada, queimação) e às FG (aperto, pressão, vibração e movimento)evocadas por estimulação elétrica senoidal com 5 Hz e 2 kHz, respectivamente. O dedo indicador da mão esquerda de 150 voluntários, sem doença neurológica conhecida e medicação, foram estimulados nessas frequências com intensidades decorrente igual ao limiar de sensibilidade (LS) e 1,5 vezes LS.O voluntário escolheu das 8 palavras, 3 que mais se aproximavam à sua percepção do estímulo. Outro grupo de 60 voluntários foi utilizado como controle, sendo que as 3 palavras foram escolhidas sem nenhuma estimulação. O valor de “1” foi atribuído à palavra escolhida, e “0” à não selecionada. As sensações foram agrupadas utilizando-se o vizinho mais próximo (single linkage) e a correlação entre variáveis binárias(tetrachloric correlation). Os resultados indicam uma tendência a sensações de fibras grossas sem estimulação, sendo que 5 Hz evoca principalmente as sensações de fibras finas e 2 kHz as de fibras grossas. A exceção foi a sensação de FF queimação, a qual se mostrou inconsistente em ambos os grupos.

Thin fibers mediate the perception related to temperature and painful sensations, and thick fibers the perception related to touch. This work tries to evaluate the perceptions related to thin (prick, pang, twinge and burning) and thick fibers (squeeze, pressure, movement and vibration) evoked by sinusoidal stimulation with 5 Hz and2 kHz, respectively. The left index fingers of 150 volunteers, without known neurological problems or under medication, were stimulatedat these frequencies and at current intensities equal to the sensory threshold and 1.5 times the sensory threshold. The volunteers chose three words out of eight that better represented the sensation felt during the stimulus. Another group of 60 volunteers was used as control, for which the three words were chosen with no stimulation. The value “1” was attributed to each sensation chosen and “0” forthe others. The sensations were clustered using the single linkage nearest neighbor method and the tetrachloric correlation between binary variables. The results showed a tendency to thick fiber sensations with 2 kHz or no stimulation, while 5 Hz evoked thin fiber sensations. The exception was the FF sensation of burning, which did not fit into any group.

Assuntos

RESUMO

OBJECTIVE: To analyze the relationship between perceptions and electrical senoidal current stimulation (ESCS). METHOD: The study population comprise 100 healthy volunteers. ESCS of 5 Hz and 2 kHz were applied to the left index finger at one and 1.5 sensory threshold. Following each stimulus train a list of eight words (four related to thin fiber sensations and four related to thick fiber sensations) was presented to the subjects who were asked to choose the three words closer to the experienced sensation. Each chosen word was given a score 1; final results were obtained by the sum of the scores for the words related to thin and thick fiber systems for each situation. RESULTS: For 5 Hz ESCS at one and 1.5 sensory threshold thin fibers had significantly higher scores than thick fibers; for 2 kHz ESCS, thick fibers had significantly higher scores. CONCLUSION: These results show that there is a relation between different sensations and ESCS of different frequencies.

Assuntos

RESUMO

OBJETIVO: Estudar a relação entre as sensações evocadas por estimulação elétrica por corrente senoidal (ESCS). MÉTODO: 100 voluntários normais foram estudados. ESCS a 5 Hz e 2 kHz foram aplicadas no dedo indicador esquerdo com uma e 1,5 vezes o limiar sensorial. Listas de oito palavras (4 relacionadas a fibras grossas, 4 a fibras finas) foram apresentadas após cada estimulação e foi solicitado que o sujeito escolhesse as 3 palavras que mais se aproximassem das sensações experimentadas. Às palavras escolhidas foi dado o escore 1. Os resultados finais para análise foram obtidos da soma dos escores para as palavras relacionadas aos diferentes sistemas de fibras. RESULTADOS: Para ESCS a 5 Hz sensações relacionadas a fibras finas foram significantemente mais escolhidas, já para estimulações a 2 kHz sensações relacionadas a fibras grossas foram significantemente mais escolhidas. CONCLUSÃO: Estes resultados mostram um relação entre diferentes percepções e diferentes freqüências de correntes elétricas senoidais.

Assuntos

RESUMO

Reference values using the instrument Neurometer were determined and compared with those suggested by the manufacturer; the sensory thresholds for the different frequencies in different body segments were determined; the influence of age, level of education, gender and the reutilization of the electrodes in the different sites were also evaluated. Sensory threshold was determined in 101 normal volunteers (68 women), ages ranged from 17 to 87 years old. The procedure was fully automated with the sensory threshold being determined by the method of limits followed by the forced choice method. Analysis of the results revealed that: there was a significant difference between the values obtained for the index fingers and the halux; no difference was detected between both index fingers; gender and level of instruction had no effect on the results, age had a small influence after stimulation with 2000Hz and 250Hz; three reutilizations of the electrodes did not affect the results on the index fingers, however at the halux, the third utilization deteriorated the results from the 2000Hz stimulation. Except for the 2000Hz stimulation, significant differences were suggested between the values reported here and those provided by the manufacturer.

Assuntos