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  • About
  • The Global ETD Search service is a free service for researchers to find electronic theses and dissertations. This service is provided by the Networked Digital Library of Theses and Dissertations.
    Our metadata is collected from universities around the world. If you manage a university/consortium/country archive and want to be added, details can be found on the NDLTD website.
131

Neocortical Evoked Potentials: Effects of Environmental Enrichment and Electrical Stimulation

Seidlitz, Eric Paul 09 1900 (has links)
<p> Alterations in neural tissues associated with environmental variables have been studied for many years. Anatomical changes in the neocortex of rats in response to exposure to complex environments were observed and replicated in a number of studies both within and across species. These changes are not dependent on the age of the animal or on the duration of exposure, and have been demonstrated in structures outside of the cortex. Due to the undisputed involvement of both the neocortex and the hippocampus in learning and memory, researchers applied a widely used model system of a synaptic mechanism for learning, long-term potentiation (LTP), to the environmental enrichment paradigm and demonstrated significant enhancements in hippocampal field potentials in enriched rats. The present study examines whether the neocortex also showed evidence of plasticity in synaptic transmission. No effects for environmental enrichment were observed on the maximum amplitude of neocortical field responses evoked from the corpus callosum. To assess the plasticity of the chronic preparation used in the study, the animals were exposed to trains of pulses previously shown to induce electrical LTP in the cortex, but revealed only a slight, although significant, depression of the evoked response amplitude. An alteration in the stimulation parameters did not result in an enhanced response. Cortical depth measures suggested that the enriched environment was indeed sufficient to produce plastic changes in anatomy, if not in the efficacy of synaptic transmission. The importance of these findings in the neocortex leads us to question the validity of the LTP model of learning and memory.</p> / Thesis / Master of Science (MSc)
132

Elektrophysiologische Indikatoren für spezifische Prozesse der Vorbereitung

Ortner-Willnecker, Karin. January 1996 (has links)
Thesis (Doctoral)--Ludwig-Maximilians-Universität.
133

Ακουστικά προκλητά δυναμικά σε παιδιά με μαθησιακές δυσκολίες

Κασταμονίτη, Δήμητρα 19 January 2010 (has links)
Το αντικείμενο αυτής της εργασίας είναι η μελέτη των στελεχιαίων ακουστικών προκλητών δυναμικών σε παιδιά με μαθησιακές δυσκολίες. Στόχος είναι η εξέταση και η σύγκριση τυχόν διαφορών ως προς το χρόνο έλευσης των κυμάτων στον εγκέφαλο μετά το ερέθισμα click και ως προς το αν οι λανθάνοντες χρόνοι ήταν μέσα στα φυσιολογικά επίπεδα μεταξύ των παιδιών με μαθησιακές δυσκολίες και των φυσιολογικών παιδιών (χωρίς μαθησιακές δυσκολίες). Το δείγμα μας αποτελείται από 20 παιδιά ηλικίας από 7 εως 14 ετών. Τα δέκα από αυτά είναι παιδιά με μαθησιακές δυσκολίες επίσημα διαγνωσμένα από τον κρατικό φορέα Κ.Δ.Α.Υ. ενώ τα υπόλοιπα αποτελούν το συγκριτικό δείγμα της μελέτης. Τα κύματα που εξετάσαμε ήταν το I-II-III-IV-V καθώς και οι διαφορές τους III-I, V-III, V-I. Επιπλέον, εκτός από το χρόνο έλευσης αυτών των κυμάτων εξετάσαμε και το αν οι χρόνοι αυτοί ήταν μέσα στα φυσιολογικά πλαίσια. Συμπερασματικά αναφέρω, πως σύμφωνα με τον έλεγχο των μηδενικών υποθέσεων που έγινε με τη στατιστική δοκιμασία Student’s t-test, η μέση τιμή του χρόνου που απαιτείται για την έλευση των κυμάτων στα φυσιολογικά παιδιά είναι ίση με την αντίστοιχη μέση τιμή των παιδιών με μαθησιακές δυσκολίες.Επίσης, η μέση τιμή των χρονικών διαφορών έλευσης των κυμάτων στα φυσιολογικά παιδιά είναι ίση με την αντίστοιχη μέση τιμή των παιδιών με μαθησιακές δυσκολίες. Για κάθε ένα από τα κύματα I-V αλλά και για τις αντίστοιχες διαφορές τους ελέγχθηκαν μηδενικές υποθέσεις του τύπου «Η παρατήρηση παθολογικών λανθανόντων χρόνων είναι ανεξάρτητη της ύπαρξης μαθησιακών δυσκολιών». Από αυτή τη μελέτη βρέθηκε το εξής σημαντικό, πως το 50% των παιδιών μεμαθησιακές δυσκολίες παρουσίασαν εκτός φυσιολογικών ορίων χρόνους για το κύμα II στο αριστερό αυτί. Παρατηρήθηκε δηλ. στατιστικώς σημαντική διαφορά στο χρόνο έλευσης του κύματος II στο δεξί αυτί (R-tII) στα παιδιά με μαθησιακές δυσκολίες έναντι των υπολοίπων (p=0,026). Ο έλεγχος της μηδενικής υπόθεσης έγινε με τη στατιστική δοκιμασία Fisher’s exact test. Τα στελεχιαία ακουστικά προκλητά δυναμικά εγκεφαλικού στελέχους, όντας εύκολα στη χορήγηση, θα μπορούσαν να χρησιμοποιηθούν ως ένα άμεσο και εύκολο διαγνωστικό εργαλείο για τα παιδιά με μαθησιακές δυσκολίες, έτσι ώστε να αρχίσει η λογοθεραπευτική παρέμβαση και αποκατάσταση των προβλημάτων από μικρή ηλικία. / The object of this work is the study of auditory evoked potential in children with learning disorders. Our aim is to examine and compare any differences in the time of arrival of waves in the brain after the stimulus click and as to whether the latent period was within the normal levels among children with learning disabilities and normal children (without learning difficulties).
134

Influence of Age on Auditory Gating

Smith, Ginny Marissa 08 June 2006 (has links) (PDF)
This study utilized paired tones at 1000 Hz with a 500 ms interpair interval and a 10 s interstimulus interval to assess sensory gating. Forty-two participants, ranging from 3-72 years of age were used to observe maturational changes in amplitude, latency, and suppression ratios of the P50 waveform. Previous research has shown that in normal adults the amplitude in response to the second of the paired tones is significantly suppressed compared to the amplitude in response to the first tone. The current study showed amplitude decreased with age to middle adulthood, where it increased slightly to later adulthood. Latencies decreased with age. Suppression ratios decreased from childhood to adolescence, with an increase from early adulthood to later adulthood. Sensory gating would appear to be a later developing aspect of human sensory physiology. Also similar to many other brain functions, sensory gating decreases in later adulthood.
135

Étude électrophysiologique des différents stades de traitement de l'information visuelle chez l'individu ayant subi un traumatisme craniocérébral

Lachapelle, Julie. January 2008 (has links)
No description available.
136

Potencial evocado auditivo de tronco encefálico com estímulo de fala em crianças com distúrbio fonológico / Speech-evoked auditory brainstem responses in children with phonological disorders

Goncalves, Isabela Crivellaro 28 August 2009 (has links)
INTRODUÇÃO: O Potencial Evocado Auditivo de Tronco Encefálico (PEATE) é um exame objetivo, que avalia a integridade da via auditiva no tronco encefálico e complementa os procedimentos audiológicos de rotina no diagnóstico de alterações auditivas. Atualmente, pesquisas têm sido desenvolvidas a fim de caracterizar os potenciais evocados auditivos obtidos com estímulos de fala, que são espectralmente e temporalmente mais complexos que o estímulo clique. OBJETIVO: caracterizar os achados dos PEATE com estímulo clique e de fala em crianças com diagnóstico de distúrbio fonológico. MÉTODOS: Foram avaliadas 36 crianças com limiares auditivos dentro da normalidade, na faixa etária de sete a 11 anos, por meio do PEATE com os estímulos clique e de fala, 18 com distúrbio fonológico (grupo estudo) e 18 em desenvolvimento típico (grupo controle). Na análise dos dados quantitativos, realizada por meio do teste T de Student, foram obtidos valores de média, mediana, desvio padrão, valor mínimo e máximo, e p-valor, quando da comparação entre os grupos. Na análise dos dados qualitativos, realizada por meio do Teste Exato de Fisher, comparou-se a ocorrência de resultados normais e alterados entre os grupos. Foram analisadas as relações entre os resultados do PEATE com estímulo de fala e o índice PCC-R (Percentage of consonants corrects Revised) no grupo estudo por meio da correlação de Pearson. RESULTADOS: No PEATE com estímulo clique, observou-se diferença estatisticamente significante entre os grupos para as latências absolutas das ondas I, III e V em ambas as orelhas, com latências maiores no grupo estudo, embora, na análise dos dados qualitativos, não tenham sido observadas diferenças entre os grupos com relação à ocorrência de resultados normais e alterados. No PEATE com estímulo de fala, verificou-se diferença estatisticamente significante entre os grupos para os valores de latência das ondas V e A. Na análise dos dados qualitativos, quando comparada a ocorrência de resultados normais e alterados entre os grupos, observou-se diferença estatisticamente significante para as latências das ondas V e A. Não foram observadas correlações significantes entre os resultados do PEATE com estímulo de fala e o índice PCC-R no grupo de crianças com distúrbio fonológico. Conclusão: Crianças com distúrbio fonológico apresentam alterações no Potencial Evocado Auditivo de Tronco Encefálico com estímulo de fala, evidenciadas pelo atraso na condução do impulso elétrico quando comparadas a crianças em desenvolvimento típico, sugerindo a existência de comprometimentos na via auditiva nesta região / INTRODUCTION: The Auditory Brainstem Response (ABR) is an objective measure that evaluates the integrity of the brainstem auditory pathway and that supplements the routine audiological procedures in the diagnosis of hearing impairment. Nowadays, studies have been developed in order to characterize the auditory evoked potentials obtained through speech stimuli that are spectrally and temporally more complex than the click stimuli. AIM: To characterize the click- and the speech-evoked ABR in children with a diagnosis of phonological disorder. METHODS: Auditory Brainstem Responses to both a click and speech stimulus (syllable /da/) were recorded in 36 children with normal bilateral hearing, with ages ranging from seven to 11 years old - 18 children with typical development (Control Group - CG) and 18 children clinically diagnosed with a phonological disorder (Study Group - SG). The quantitative data analysis was performed through the unpaired T Student test. Values of mean, median, standard deviation, minimum and maximum, and p-value were obtained for the between group comparison. The qualitative data analysis was performed through the Fisher\'s Exact Test. The occurrence of normal and abnormal results was compared between groups. The relationship between the results of speech-evoked ABR and the PCC-R index (Percentage of consonants corrects Revised) of the SG was established by Pearson\'s correlation. RESULTS: In the click-evoked ABR, a statistically significant difference was observed on the between groups analysis for latency values of waves I, III and V in both ears, with higher latency values for the study group. However, no between group differences were observed in the qualitative data analysis regarding the occurrence of normal and abnormal results. In the speech-evoked ABR, a statistically significant difference was observed on the between groups analysis for the latency values of waves V and A. Similarly, a statistically significant difference between groups was observed on the qualitative data analysis of latencies of waves V and A when comparing the occurrence of normal and abnormal results. No significant correlations between the results of speechevoked ABR and the PCC-R index were observed in the group of children with phonological disorder. Conclusion: Children with phonological disorders present abnormal results in the speech-evoked ABR when compared to typically developing children. This was evidenced by the delay in conducting the electrical impulse through the brainstem, suggesting the existence of auditory pathway disorders in this region
137

Potenciais evocados auditivos e esclerose múltipla / Auditory evoked potentials and multiple sclerosis

Oliveira, Caroline Rondina Salzano de 02 October 2008 (has links)
INTRODUÇÃO: A Esclerose Múltipla é uma doença desmielinizante e autoimune, na qual ocorre a destruição da bainha de mielina por autoanticorpos. Esta destruição causa uma diminuição na velocidade de condução do impulso nervoso alterando, assim, as funções cerebral e neural. Para ocorrer o funcionamento adequado do sistema auditivo, tanto na sua porção periférica quanto na central há necessidade que o mesmo esteja íntegro. OBJETIVO: Este estudo tem como objetivo verificar a ocorrência de alterações nos potenciais evocados auditivos de curta (PEATE), média (PEAML) e longa (P300) latências em adultos audiologicamente normais com diagnóstico de Esclerose Múltipla do tipo remitente recorrente ou surto remissivo. MÉTODO: Para o grupo controle foram avaliados 25 indivíduos com histórico de desenvolvimento neurológico normal e sem queixa de zumbido, sendo 19 do gênero feminino e seis do masculino, com idade variando entre 25 e 55 anos (média de 35,16 anos de idade). Para o grupo pesquisa foram avaliados 25 indivíduos com Esclerose Múltipla do tipo remitente-recorrente ou surto-remissivo, seis do gênero masculino e 19 do feminino, com idades entre 25 e 53 anos (média de 34,88 anos de idade). Todos os indivíduos participantes da pesquisa realizaram audiometria tonal e vocal, medidas de imitância acústica e os potenciais evocados auditivos de curta, média e longa latências. RESULTADOS: Os resultados demonstraram ocorrência de alterações nos potenciais evocados auditivos de tronco encefálico e de média latência e no potencial cognitivo, em indivíduos com Esclerose Múltipla. Com relação ao potencial evocado auditivo de tronco encefálico, verificou-se aumento de latência das ondas III e V e dos interpicos I-III e I-V, sugerindo alteração de tronco encefálico baixo. No que diz respeito ao potencial evocado auditivo de média latência observou-se atraso nas latências das ondas Na e Pa e, para o potencial cognitivo, evidenciou-se aumento da latência do P300. Conclusão: Indivíduos com Esclerose Múltipla do tipo remitente recorrente apresentam alterações eletrofisiológicas observadas nos resultados dos potenciais evocados auditivos de curta e média latências e no potencial cognitivo, sugerindo comprometimento em diferentes locais do sistema nervoso auditivo central / INTRODUCTION: The Multiple Sclerosis is a demyelinating disease and autoimmune, which is the destruction of the myelin sheath of a selfantibodies. This destruction causes a decrease in impulse driving speed of nervous changing thus the brain and neural functions. To place the proper functioning of the auditory system, both in its peripheral portion as the central need that it is full. PURPOSE: This study aims to determine the occurrency of changes in auditory evoked potentials of short (PEATE), middle (PEAML) and long (P300) latencies in adults audiologically normal diagnosed with Multiple Sclerosis remitting type of applicant or remissive outbreak. METHOD: For the control group were evaluated 25 individuals with a history of normal neurological development and without complaint from tinnitus, and 19 females and six males, with ages ranging between 25 and 55 years (average of 35.16 years of age) . To search the group were evaluated 25 individuals with Multiple Sclerosis-remitting type of applicant or flareremissive, six males and 19 females, aged between 25 and 53 years (average of 34.88 years of age). All individuals participating in the search conducted tone audiometry and speech, acoustic immitance measures brainstem auditory evoked potential, middle latency response and cognitive potential RESULTS: The results showed occurrencies of changes in brainstem auditory evoked potential and middle latency and cognitive potential in individuals with Multiple Sclerosis. Regarding the potential of brain stem auditory evoked, there was an increase in latency wave III and V and interpeaks I-III and IV, suggesting change in brainstem down. Regarding the auditory evoked potential of average latency there was delay in latencies of Na and Pa waves, and the potential for cognitive, showed an increase of latency of P300. Conclusion: Individuals with multiple sclerosis relapsing remitting type of applicant presents electrophysiological changes seen in the results of sound evoked short and middle latency and the potential cognitive suggesting impairment from different parts of the central auditory nervous system
138

Potenciais evocados auditivos e esclerose múltipla / Auditory evoked potentials and multiple sclerosis

Caroline Rondina Salzano de Oliveira 02 October 2008 (has links)
INTRODUÇÃO: A Esclerose Múltipla é uma doença desmielinizante e autoimune, na qual ocorre a destruição da bainha de mielina por autoanticorpos. Esta destruição causa uma diminuição na velocidade de condução do impulso nervoso alterando, assim, as funções cerebral e neural. Para ocorrer o funcionamento adequado do sistema auditivo, tanto na sua porção periférica quanto na central há necessidade que o mesmo esteja íntegro. OBJETIVO: Este estudo tem como objetivo verificar a ocorrência de alterações nos potenciais evocados auditivos de curta (PEATE), média (PEAML) e longa (P300) latências em adultos audiologicamente normais com diagnóstico de Esclerose Múltipla do tipo remitente recorrente ou surto remissivo. MÉTODO: Para o grupo controle foram avaliados 25 indivíduos com histórico de desenvolvimento neurológico normal e sem queixa de zumbido, sendo 19 do gênero feminino e seis do masculino, com idade variando entre 25 e 55 anos (média de 35,16 anos de idade). Para o grupo pesquisa foram avaliados 25 indivíduos com Esclerose Múltipla do tipo remitente-recorrente ou surto-remissivo, seis do gênero masculino e 19 do feminino, com idades entre 25 e 53 anos (média de 34,88 anos de idade). Todos os indivíduos participantes da pesquisa realizaram audiometria tonal e vocal, medidas de imitância acústica e os potenciais evocados auditivos de curta, média e longa latências. RESULTADOS: Os resultados demonstraram ocorrência de alterações nos potenciais evocados auditivos de tronco encefálico e de média latência e no potencial cognitivo, em indivíduos com Esclerose Múltipla. Com relação ao potencial evocado auditivo de tronco encefálico, verificou-se aumento de latência das ondas III e V e dos interpicos I-III e I-V, sugerindo alteração de tronco encefálico baixo. No que diz respeito ao potencial evocado auditivo de média latência observou-se atraso nas latências das ondas Na e Pa e, para o potencial cognitivo, evidenciou-se aumento da latência do P300. Conclusão: Indivíduos com Esclerose Múltipla do tipo remitente recorrente apresentam alterações eletrofisiológicas observadas nos resultados dos potenciais evocados auditivos de curta e média latências e no potencial cognitivo, sugerindo comprometimento em diferentes locais do sistema nervoso auditivo central / INTRODUCTION: The Multiple Sclerosis is a demyelinating disease and autoimmune, which is the destruction of the myelin sheath of a selfantibodies. This destruction causes a decrease in impulse driving speed of nervous changing thus the brain and neural functions. To place the proper functioning of the auditory system, both in its peripheral portion as the central need that it is full. PURPOSE: This study aims to determine the occurrency of changes in auditory evoked potentials of short (PEATE), middle (PEAML) and long (P300) latencies in adults audiologically normal diagnosed with Multiple Sclerosis remitting type of applicant or remissive outbreak. METHOD: For the control group were evaluated 25 individuals with a history of normal neurological development and without complaint from tinnitus, and 19 females and six males, with ages ranging between 25 and 55 years (average of 35.16 years of age) . To search the group were evaluated 25 individuals with Multiple Sclerosis-remitting type of applicant or flareremissive, six males and 19 females, aged between 25 and 53 years (average of 34.88 years of age). All individuals participating in the search conducted tone audiometry and speech, acoustic immitance measures brainstem auditory evoked potential, middle latency response and cognitive potential RESULTS: The results showed occurrencies of changes in brainstem auditory evoked potential and middle latency and cognitive potential in individuals with Multiple Sclerosis. Regarding the potential of brain stem auditory evoked, there was an increase in latency wave III and V and interpeaks I-III and IV, suggesting change in brainstem down. Regarding the auditory evoked potential of average latency there was delay in latencies of Na and Pa waves, and the potential for cognitive, showed an increase of latency of P300. Conclusion: Individuals with multiple sclerosis relapsing remitting type of applicant presents electrophysiological changes seen in the results of sound evoked short and middle latency and the potential cognitive suggesting impairment from different parts of the central auditory nervous system
139

Cervikální spondylogenní myelopatie: Chirurgická strategie a vývoj klinických projevů / Cervical spondylotic myelopathy: development of clinical symptoms and surgical management

Štěpánek, David January 2014 (has links)
Based on contemporary theoretical knowledge in this prospective study we outline the relationship between a chosen surgical approach (anterior or posterior approach) as it relates to the localization of spinal cord lesion (anterior or posterior spinal pathways) assessed by the use of evoked potentials (SEP, MEP) and the effect of this approach on the postoperative state of patients with cervical spondylotic myelopathy. Furthermore we evaluate clinical outcome of these patients according to several aspects of their MRI and X-ray findings. The study, from 2006 to 2010, comprised 65 patients with clinical signs of cervical myelopathy. These patients had been indicated for surgery, which subsequently was performed by using either the front (anterior - a) or back (posterior - p) approach. The patients were assessed using Nurick and mJOA scores before surgery, then at 12 months, and finally 24 months after surgery. In addition, they were preoperatively examined with a battery of evoked potentials (EP) - somatosensory evoked potential (SEP) and motor evoked potential (MEP) tests. Based on EP, principal spinal cord disability was determined: A - anterior (maximum changes in MEP), P - posterior - maximum change in SEP). The entire group was, on the basis of EP partitioning and the surgical approach used, divided...
140

Estudos das lesões de vias medulares através dos potenciais evocados somatossensitivos (PESS) e motores (PEM) em pacientes com mucopolissacaridoses (MPS)

Silva, Daniel de Souza e January 2013 (has links)
Made available in DSpace on 2014-08-26T17:31:43Z (GMT). No. of bitstreams: 2 license.txt: 1748 bytes, checksum: 8a4605be74aa9ea9d79846c1fba20a33 (MD5) 69589.pdf: 12544721 bytes, checksum: e242b76c9cdf8b6c04d9e749f41a5d1f (MD5) / Fundação Oswaldo Cruz. Instituto Fernandes Figueira. Departamento de Ensino. Programa de Pós-Graduação em Saúde da Criança e da Mulher. Rio de Janeiro, RJ, Brasil. / As mucopolisacaridoses (MPS) são doenças geneticamente determinadas, causadas pela deficiência de enzimas lisossômicas, o que leva ao acúmulo de glicosaminoglicanos (GAGs) universalmente. As mielopatias cervicais ao nível da junção craniocervical são uma complicação frequente, grave e multifatorial das MPS. Este trabalho teve como principal objetivo o estudo das vias sensitivas e motoras centrais (cordão posterior e trato córtico-espinhal) em topografia medular cervical nos pacientes com mucopolissacaridoses (MPS), utilizando os potenciais evocados somatossensitivos e motores. Como objetivos específicos, a avaliação dos pacientes com MPS em fases distintas da doença, a demonstração da existência de comprometimento da condução nervosa nos trajetos das vias medulares na topografia já mencionada e o destaque do papel dos testes neurofisiológicos (funcionais) no diagnóstico, acompanhamento e na tomada de decisões terapêuticas nos pacientes com MPS. Foi realizado um estudo transversal, de uma série de casos, tendo como metodologia a revisão de prontuários, no Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Foram incluídos os pacientes com o diagnóstico de MPS, sem restrições quanto à idade, aos subtipos da doença ou ao fato de estarem ou não sob tratamento específico e excluídos aqueles pacientes com lesões graves do sistema nervoso central que impedissem a realização dos testes propostos. Os dados colhidos referiram-se à idade dos pacientes, o tipo de MPS, os achados do exame neurológico, os resultados dos estudos de imagem (ressonância magnética) da coluna cervical e os parâmetros neurofisiológicos encontrados nos potenciais evocados somatossensitivos (PESS) e motores (PEM), que incluiram as latências das ondas (N9, N13 e N20 nos PESS de membros superiores e N21 e P37 nos PESS de membros inferiores), os tempos de condução central (N20 – N13 e P37 – N21) e o tempo de condução motora central (nos PEM de membros superiores e inferiores). Foram analisados resultados de cinquenta e oito exames de potenciais evocados somatossensitivos e motores, realizados em vinte e oito pacientes com MPS. Todos os exames foram anormais, com alterações mais graves nos exames de PEM. / Mucopolysaccharidosis (MPS) are inherited metabolic diseases caused by deficiency of lysosomal enzymes leading to progressive accumulation of glycosaminoglycans (GAGs) in nearly all cell types, tissues and organs. Cervical myelopathy at the cranio-cervical junction is a frequent, severe and multifactorial complication of MPS. The first objective of the present study is the evaluation os the function of central neural pathways (brain stem and spinal cord posterior columns and corticospinal tract in patients with MPS using somatosensory evoked potentials (SEP) and motor evoked potentials (MEP). The secondary objectives are the evaluation of the patients in different periods of disease, the diagnostic of the lesions in cervical spinal cord and the remark of the role of these neurophysiological tests in diagnostic, follow-up and therapeutic decisions. This cross-sectional study included all patients with MPS of Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira, despite age, type of disease, enzyme replacement therapy or not, except the patients with severe central nervous system lesions in whom the neurophysiological evaluation became impossible. Data included the age of patients, type of disease, the findings in neurological examination, magnetic resonance imaging of cervical spinal cord and the neurophysiological parameters [the latencies of N9, N13 and N20 waveforms, central conduction time (upper limbs SEP), the latencies of N21 and P37 waveforms, central conduction time (lower limbs SEP), central motor conduction time (upper and lower limbs MEP)].Twenty-eight patients were enrolled and fifty-eigth tests (SEP and MEP, upper and lower limbs) were performed in these patients. All tests were abnormal with most severe functional impairment in MEP tests.

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