Ultrahigh-frequency ultrasound of fascicles in the common fibular, superficial fibular, and sural nerves.

INTRODUCTION/AIMS: While ultrasound assessment of cross-sectional area and echogenicity has gained popularity as a biomarker for various neuropathies, there is a scarcity of data regarding fascicle count and density in neuropathies or even healthy controls. The aim of this study was to determine whether fascicles within select lower limb nerves (common fibular, superficial fibular, and sural nerves) can be counted in healthy individuals using ultrahigh-frequency ultrasound (UHFUS). METHODS: Twenty healthy volunteers underwent sonographic examination of the common fibular, superficial fibular, and sural nerves on each lower limb using UHFUS with a 48 MHz linear transducer. Fascicle counts and density in each examined nerve were determined by a single rater. RESULTS: The mean fascicle number for each of the measured nerves included the following: common fibular nerve 9.85 (SD 2.29), superficial fibular nerve 5.35 (SD 1.59), and sural nerve 6.73 (SD 1.91). Multivariate linear regression analysis revealed a significant association between cross-sectional area and fascicle count for all three nerves. In addition, there was a significant association seen in the common fibular nerve between fascicle density and height, weight, and body mass index. Age and sex did not predict fascicle count or density (all p > .13). DISCUSSION: UHFUS enabled the identification and counting of fascicles and fascicle density in the common fibular, superficial fibular, and sural nerves. Knowledge about normal values and normal peripheral nerve architecture is needed in order to further understand and identify pathological changes that may occur within each nerve in different disease states.

Development of a major histocompatibility complex class II conditional knockout mouse to study cell-specific and time-dependent adaptive immune responses in peripheral nerves.

INTRODUCTION/AIMS: The precise relationship between molecular mimicry and tissue-specific autoimmunity is unknown. Major histocompatibility complex (MHC) class II antigen presenting cell-CD4+ T-cell receptor complex interactions are necessary for adaptive immunity. This study aimed to determine the role of endoneurial endothelial cell MHC class II in autoimmune polyneuropathy. METHODS: Cryopreserved Guillain-Barré syndrome (GBS) patient sural nerve biopsies and sciatic nerves from the severe murine experimental autoimmune neuritis (sm-EAN) GBS model were studied. Cultured conditional ready MHC Class II antigen A-alpha chain (H2-Aa) embryonic stem cells were used to generate H2-Aaflox/+ C57BL/6 mice. Mice were backcrossed and intercrossed to the SJL background to generate H2-Aaflox/flox SJL mice, bred with hemizygous Tamoxifen-inducible von Willebrand factor Cre recombinase (vWF-iCre/+) SJL mice to generate H2-Aaflox/flox; vWF-iCre/+ mice to study microvascular endothelial cell adaptive immune responses. Sm-EAN was induced in Tamoxifen-treated H2-Aaflox/flox; vWF-iCre/+, H2-Aaflox/flox; +/+, H2-Aa+/+; vWF-iCre/+ and untreated H2-Aaflox/flox; vWF-iCre/+ adult female SJL mice. Neurobehavioral, electrophysiological and histopathological assessments were performed at predefined time points. RESULTS: Endoneurial endothelial cell MHC class II expression was observed in normal and inflamed human and mouse peripheral nerves. Tamoxifen-treated H2-Aaflox/flox; vWF-iCre/+ mice were resistant to sm-EAN despite extensive MHC class II expression in lymphoid and non-lymphoid tissues. DISCUSSION: A conditional MHC class II knockout mouse to study cell- and time-dependent adaptive immune responses in vivo was developed. Initial studies show microvascular endothelial cell MHC class II expression is necessary for peripheral nerve specific autoimmunity, as advocated by human in vitro adaptive immunity and ex vivo transplant rejection studies.

Granuloma, vasculitis, and demyelination in sarcoid neuropathy.

BACKGROUND: Despite the suggestion that direct compression by granuloma and ischemia resulting from vasculitis can cause nerve fiber damage, the mechanisms underlying sarcoid neuropathy have not yet been fully clarified. METHODS: We examined the clinicopathological features of sarcoid neuropathy by focusing on electrophysiological and histopathological findings of sural nerve biopsy specimens. We included 18 patients with sarcoid neuropathy who had non-caseating epithelioid cell granuloma in their sural nerve biopsy specimens. RESULTS: Although electrophysiological findings suggestive of axonal neuropathy were observed, particularly in the lower limbs, all but three patients showed ≥1 abnormalities in nerve conduction velocity or distal motor latency. Additionally, a conduction block was observed in 11 of the 16 patients for whom waveforms were assessed; five of them fulfilled motor nerve conduction criteria strongly supportive of demyelination as defined in the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guideline for chronic inflammatory demyelinating polyneuropathy (CIDP). In most patients, sural nerve biopsy specimens revealed a mild to moderate degree of myelinated fiber loss. Fibrinoid necrosis was observed in one patient, and electron microscopy analysis revealed demyelinated axons close to granulomas in six patients. CONCLUSIONS: Patients with sarcoid neuropathy may meet the EAN/PNS electrophysiological criteria for CIDP due to the frequent presence of conduction blocks. Based on our results, in addition to the ischemic damage resulting from granulomatous inflammation, demyelination may play an important role in the mechanism underlying sarcoid neuropathy.

Mitochondrial DNA and Electron Transport Chain Protein Levels Are Altered in Peripheral Nerve Tissues from Donors with HIV Sensory Neuropathy: A Pilot Study.

Distal sensory polyneuropathy (DSP) and distal neuropathic pain (DNP) remain significant challenges for older people with HIV (PWH), necessitating enhanced clinical attention. HIV and certain antiretroviral therapies (ARTs) can compromise mitochondrial function and impact mitochondrial DNA (mtDNA) replication, which is linked to DSP in ART-treated PWH. This study investigated mtDNA, mitochondrial fission and fusion proteins, and mitochondrial electron transport chain protein changes in the dorsal root ganglions (DRGs) and sural nerves (SuNs) of 11 autopsied PWH. In antemortem standardized assessments, six had no or one sign of DSP, while five exhibited two or more DSP signs. Digital droplet polymerase chain reaction was used to measure mtDNA quantity and the common deletions in isolated DNA. We found lower mtDNA copy numbers in DSP+ donors. SuNs exhibited a higher proportion of mtDNA common deletion than DRGs in both groups. Mitochondrial electron transport chain (ETC) proteins were altered in the DRGs of DSP+ compared to DSP- donors, particularly Complex I. These findings suggest that reduced mtDNA quantity and increased common deletion abundance may contribute to DSP in PWH, indicating diminished mitochondrial activity in the sensory neurons. Accumulated ETC proteins in the DRG imply impaired mitochondrial transport to the sensory neuron's distal portion. Identifying molecules to safeguard mitochondrial integrity could aid in treating or preventing HIV-associated peripheral neuropathy.

The multiple roles of nerve biopsy in the diagnosis and prognosis of suspected immune neuropathies.

INTRODUCTION: The value of a sural nerve biopsy for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is controversial. Evidence-based recommendations for its implementation are lacking. We investigated factors leading to biopsy and analyzed biopsy outcomes and consequences, assessed the predictability of biopsy outcomes through clinical parameters to avoid unnecessary biopsies, and compared results with electrophysiological and clinical severity to determine their prognostic value. METHODS: 190 sural nerve biopsies were analyzed in two cohorts. One consisted of 163 biopsies and the second of 72 biopsies from the prospective Immune-mediated Neuropathies Biomaterial and Data registry (INHIBIT). Both have an intersection of 45 patients. 75 data sets from patients without biopsy were used. Analysis of nerve conduction studies, treatment, overall disability sum score (ODSS), biopsy outcomes, and diagnosis was performed. RESULTS: 51% of biopsied patients received the diagnosis CIDP (77% fulfilled EFNS/PNS criteria), 21% were not CIDP typical, and 27% were unspecific. Biopsied patients responded less frequently to immunotherapies at time of biopsy than non-biopsied patients (p = 0.003). Immunotherapy was initiated more frequently after biopsy (p < 0.001) and more often with intravenous immunoglobulins (p < 0.0001). 76% of all biopsied patients met the electrophysiological criteria for CIDP. Sensory nerve action potential amplitudes of 0 µV still provide 73% of histological diagnostic value. Histologic signs of degeneration predicted ODSS worsening after 1 year (p = 0.028) but disease severity did not correlate with histological damage severity. DISCUSSION: The main indication for nerve biopsy was the treatment of refractory cases of autoimmune neuropathies with the therapeutic consequence of treatment initiation or escalation. Sural biopsy also provided prognostic information. Even with extinguished sural SNAP, the biopsy can still have diagnostic value.

Avaliação crítica das técnicas cirúrgicas de correção do equino / Critical evaluation of the surgical techniques to correct the equinus deformity

RESUMO A deformidade em equino leva a diversos transtornos da marcha, ao causar alterações no apoio do pé e afetar regiões anatômicas mais distantes, como o joelho, quadril e tronco. Geralmente é secundária à retração, encurtamento ou espasticidade do tríceps sural, de modo que algumas intervenções cirúrgicas podem ser necessárias para corrigi-la. Trata-se de um dos procedimentos mais antigos da Ortopedia, antes realizado apenas no tendão calcâneo e que, ao longo do tempo, evoluiu com técnicas diferentes de acordo com o grau de deformidade, doença de base e perfil do paciente. Busca-se corrigir a deformidade, com a menor interferência possível na força muscular e, com isso, diminuir a incidência de complicações, como marcha agachada, arrastada e pé calcâneo. Do ponto de vista anatômico, o tríceps sural apresenta cinco regiões que podem ser abordadas cirurgicamente para correção do equino. Em virtude da complexidade do paciente com equino, os ortopedistas devem ter experiência com ao menos uma técnica em cada zona. Neste texto são abordadas e analisadas criticamente as técnicas mais importantes para correção do equino, principalmente de modo a evitar complicações. Foi realizada uma busca sobre técnicas cirúrgicas mais comuns de correção do equino em livros clássicos e identificação e consulta aos artigos originais. Em seguida, fez-se uma busca em bases de dados nos últimos dez anos.

ABSTRACT The equinus deformity causes changes in the foot contact and may affect more proximal anatomical regions, such as the knee, hip and trunk, potentially leading to gait disorders. The equinus is usually secondary to retraction, shortening and/or spasticity of the triceps surae, and it may require surgical correction. Surgery for the correction of equinus is one of the oldest procedures in Orthopedics, and it was initially performed only at the calcaneus tendon. The technique has evolved, so that it could be customized for each patient, depending on the degree of deformity, the underlying disease, and patient´s profile. The aim is to correct the deformity, with minimal interference in muscle strength, thus reducing the incidence of disabling complications such as crouch gait and calcaneus foot. We conducted a literature search for the most common surgical techniques to correct the equinus deformity using classic books and original articles. Further, we performed a database search for articles published in the last ten years. From the anatomical perspective, the triceps surae presents five anatomical regions that can be approached surgically for the equinus correction. Due to the complexity of the equinus, orthopedic surgeons should be experienced with at least one procedure at each region. In this text, we critically approach and analyze the most important techniques for correction of the equinus, mainly to avoid complications.

Hemangioma cavernoso de nervio periférico: presentación de un caso clínico / Cavernous hemangioma of the peripherical nerve: A case report

El hemangioma cavernoso, conocido también como hemangioma profundo, es una neoplasia benigna de los vasos sanguíneos, que se caracteriza por la presencia de un gran número de vasos normales y anormales sobre la piel u otros órganos internos. Su desarrollo de forma intraneural en nervio periférico es muy raro, con menos de 50 casos informados en la literatura. Presentamos un caso de un hemangioma cavernoso del nervio sural medial en una paciente con clínica de dolor severo y alodinia con resolución completa de la sintomatología tras su tratamiento mediante microcirugía (AU)

Cavernous hemangiomas, also known as deep hemangiomas are benign tumors of blood vessels, including normal and abnormal vascular structures, that develop in skin tissue and sometimes even in deep tissues. Its intraneural development in the peripheral nerve is very rare with less than 50 cases reported in the literature. We present a case of a cavernous hemangioma of the medial sural nerve in a patient with symptoms of severe pain and allodynia with complete resolution of symptoms with microsurgery (AU)

Carcinoma adenoide quístico parotídeo: soluciones estéticas y funcionales / Adenoid cystic carcinoma of the parotid: an aesthetic and functional solutions

El carcinoma adenoide quístico supone el 10-30% de las neoplasias malignas parotídeas, su tratamiento se basa en una parotidectomía que incluya el tumor con un adecuado margen de seguridad y la radioterapia postoperatoria dado que permite mejorar el control locorregional de la enfermedad. Revisamos un caso que permite exponer el manejo de las secuelas funcionales y estéticas derivadas de su tratamiento. Consideramos adecuada la reconstrucción inmediata del nervio facial cuando se encuentra clínicamente afecto o englobado por el tumor; así como suplir el defecto de volumen posparotidectomía con un colgajo local. Proponemos la anastomosis nerviosa con injerto de nervio sural de las ramas del nervio facial afectas y el relleno del defecto volumen posparotidectomía con un colgajo de fascia temporo-parietal (AU)

Adenoid cystic carcinoma represents 10-30% of all malignant neoplasms in the parotid gland. Treatment is a formal parotidectomy, which includes removing the tumour with an adequate margin and postoperative radiotherapy to improve the locoregional control of the disease. We report a case in order to present the management of the functional and aesthetic consequences obtained from its treatment. When the facial nerve is clinical affected or involved by the tumour, it requires resection and an immediate reconstruction. We suggest the sural nerve graft for the reconstruction of the affected facial branches and the temporo-parietal fascia flap to fill the volume left by the parotidectomy (AU)

Autosomal dominant HMSN with proximal involvement: new Brazilian cases / HMSN autossômica dominante com envolvimento proximal: novos casos brasileiros

We report four Brazilian siblings with Autosomal Dominant Hereditary Motor Sensory Neuropathy with Proximal Dominant Involvement (HMSN-P), a rare form of HMSN, that was characterized by proximal dominant muscle weakness and atrophy onset after the age of 30 years, fasciculation, arreflexia and sensory disturbances with autosomal dominant inheritance. Electrophysiological study and sural nerve biopsy were in the accordance with axonal sensory motor polyneuropathy and laboratorial analysis disclosed serum lipids and muscle enzymes abnormalities. Our report is the first done by a group outside Japan, where the disease initially seemed to be restricted and stressed the phenotypic variability from the original report.

Relatamos os casos de quatro irmãos brasileiros com Neuropatia Sensitivo Motora Hereditária com Envolvimento Proximal Dominante (HMSN-P), uma forma rara de HMSN caracterizada por fraqueza muscular de predomínio proximal e atrofia de instalação após os 30 anos, fasciculações, arreflexia, distúrbios sensitivos e padrão de herança autossômica dominante. Os estudos eletrofisiológicos e de biópsia do nervo sural confirmaram o diagnóstico de polineuropatia sensitivo-motora com padrão lesional axonal. Laboratorialmente foram constatadas anormalidades séricas no metabolismo lipídico e enzimas musculares. Nosso relato é o primeiro feito por um grupo fora do Japão, onde a doença parecia restrita até então e ressalta a variabilidade fenotípica apresentada nos casos Brasileiros.

Síndrome del túnel tarsiano bilateral por sinovitis. Aportación diagnóstica combinada de ecografía y electrofisiología / No disponible

No disponible

Depleción del ácido desoxirribonucleico mitocondrial y mutaciones de POLG en un paciente con neuropatía sensorial atáxica, disartria y oftalmoplejía / Mitochondrial DNA depletion and polg mutations in a patient with sensory ataxia, dysarthria and ophthalmoplegia

Fundamento y objetivo: Un amplio espectro de enfermedades mitocondriales está producido por mutaciones de POLG y se caracterizan por una alteración en la integridad del genoma mitocondrial. La oftalmoplejía progresiva externa suele ser el marcador clínico en los casos de deleciones múltiples, pero no lo es en aquellas enfermedades que cursan con depleción del ácido desoxirribonucleico mitocondrial (ADNmt). En este trabajo presentamos un paciente con la tríada clínica que define el síndrome de neuropatía sensorial atáxica, disartria y oftalmoplejía, las mutaciones del gen POLG y la presencia inusual de una marcada disminución en el contenido del ADNmt en el músculo esquelético.Paciente y método: El paciente presentó un cuadro clínico caracterizado por neuropatía sensorial atáxica, disartria y oftalmoplejía. El diagnóstico se realizó mediante estudios histológicos y análisis molecular del ADNmt y del gen POLG. Resultados: La biopsia del nervio sural detectó una pérdida intensa de las fibras nerviosas mielinizadas gruesas. El estudio molecular reveló mutaciones en el gen POLG, así como deleciones múltiples y una marcada depleción del genoma mitocondrial. Conclusiones: Los pacientes con síndromes atáxicos de origen mitocondrial presentan fenotipos mitocondriales moleculares diferentes, por lo que se aconseja la búsqueda de mutaciones del gen POLG en todos ellos, independientemente de la anomalía que presenten en el genoma mitocondrial (AU)

Background and objetive: A broad spectrum of clinical disorders is produced by mutations in the DNA polymerase gamma mitochondrial (POLG) gene which are associated with altered mitochondrial DNA (mtDNA) integrity. The majority of disorders characterized by multiple mtDNA deletions present with progressive external ophthalmoplegia, though this feature is not usually found in syndromes caused by mtDNA depletion. We report on a patient having the clinical triad of sensory ataxic neuropathy, dysarthria and ophthalmoplegia (SANDO), POLG mutations and reduced muscle mtDNA content. Patient and methods: The patient presented with sensory ataxic neuropathy, dysarthria and ophthalmoplegia. Diagnosis was established by using histological and genetic procedures (nerve biopsy, mtDNA molecular analysis in skeletal muscle and mutation screening in the POLG gene). Results: Sural nerve biopsy showed marked loss of large myelinated fibers. Skeletal muscle analysis revealed multiple mtDNA deletions, a marked decrease in mtDNA copy number and pathogenic mutations in the POLG gene. Conclusions: POLG mutations must be considered in all patients with the cardinal findings of the SANDO phenotype, without taking into account the type of abnormalities encountered in the mitochondrial genome (AU)

Polineuropatia por triclorfon: registro de um caso com estudo eletrofisiológico e histopatológico do nervo sural / Polyneuropathy by trichlorfon: report of a case with electrophysiological and histopathological study of the sural nerve

É apresentado caso de paciente agricultor que, após uso inadequado de inseticida organofosforado (triclorfon), teve sinais e sintomas de intoxicaçäo aguda e, três meses após, desenvolveu quadro de polineuropatia sensitivo-motora. O exame eletroneuromiográfico revelou alteraçöes axonais e desmielinizantes difusas. O estudo do nervo sural, em cortes semi-finos, à ultramicroscopia e à técnica de fibras isoladas revelou degeneraçäo axonal e transformaçäo grnaular do axoplasma com perda dos neurofilamentos e neurotúbulos

Polineuropatia do paciente crítico: registro de caso / Critical illness polyneuropathy: case report

A polineuropia do paciente crítico é de natureza axonal, aguda e de predomínio motor, desenvolvendo-se em pacientes sépticos ou com falência de múltiplos órgãos que geralmente necessitam de ventilação mecânica em unidade de tratamento intensivo. Seu diagnóstico é corroborado por achados eletroneuromiográficos e, geralmente, a evolução é satisfatória e rápida. Relatamos o caso de uma paciente de 35 anos que, após internação em unidade de tratamento intensivo devido a septicemia e retirada de feto morto, desenvolveu tetraparesia com melhora importante em quatro meses. A eletroneuromiografia mostrou diminuição de amplitudes de potenciais sensitivos e motores, ondas positivas e fibrilações. A biópsia de nervo sural evidenciou neuropatia axono-mielínica em atividade. Estando esses achados condizentes com os da literatura, acreditamos tratar-se de caso de polineuropatia do paciente crítico.

Distrofia neuroaxonal infantil: relato de dois casos / Infantil neuroaxonal dystrophy: report of 2 cases

Descrevemos dois casos de distrofia neuroaxonal infantil ou doença de Seitelberger, que é doença rara, neurodegenerativa, com herança autossômica recessiva. O primeiro caso, sexo masculino, com 8 anos de idade, apresentava atraso do desenvolvimento psicomotor, ataxia e fraqueza muscular. Ao exame físico foi encontrado nistagmo horizontal e vertical com palidez do disco óptico, hipotonia e arreflexia profunda. O segundo caso, sexo masculino, com 1 ano e 6 meses de idade, apresentava atraso do desenvolvimento psicomotor e convulsões. No exame físico, apresentava atrofia de nervo óptico, hipertonia e hiperreflexia. A biópsia de nervo sural de ambos os pacientes mostrou aumento dos axônios, compátivel com distrofia neuroaxonal. As características clínicas pleomórficas, bem como os achados neurofisiológicos variáveis tornam difícil firmar o diagnóstico, o qual é ajudado pela confirmação anatomopatológica dos esferóides neuroaxomais.

Neuropatia vasculítica na granulomatose de Wegener: relato de caso / Vasculitic neuropathy in Wegener granulomatosis: case report

O acometimento neurológico no curso da granulomatose de Wegener atinge 15-50 por cento dos pacientes, porém como sintomatologia inicial näo é comentado na literatura. Relatamos o caso de uma paciente com mononeurite múltipla secundária a granulomatose de Wegener, enfocando os aspectos de antecipaçäo do quadro neurológico em relaçäo aos sintomas sistêmicos, a contribuiçäo diagnóstica da biópsia do nervo sural e a boa evoluçäo do quadro neurológico

Biópsia do nervo sural na distrofia muscular miotônica / Sural nerve biopsy in myotonic dystrophy

Foram estudados 12 pacientes com distrofia muscular miotônica com o objetivo de verificar o comprometimento do sistema nervoso periférico. Todos apresentavam os sinais e sintomas principais da doença. Nenhum tinha outras causas que pudessem justificar uma polineuropatia. Todos foram submetidos a biópsia do nervo sural com contagem de fibras mielínicas e realizaçao de histograma. Dois nao apresentavam histograma bimodal. Os pacientes mostraram reduçao do número de fibras mielínicas. Concluímos que a polineuropatia pode fazer parte do quadro clínico polimorfo da distrofia muscular miotônica.

Polineuropatia hipoglicêmica: registro de caso com insulinoma / Hypoglycemic polyneuropathy: report of a case with insulinoma

Os autores registram o caso de jovem do sexo masculino que desenvolveu polineuropatia sensitivo-motora, com grande amiotrofia das mäos, no curso de crises de hipoglicemia, provocadas por provável insulinoma. A eletroneuromiografia revelou predominância de alteraçöes axonais. O nervo sural, estudado à microscopia óptica e eletrônica, mostrou perda axonal de fibras mielínicas de grande calibre e de fibras amielíncias. Consideraçöes sobre a etiopatogenia desta síndrome säo feitas, parecendo ser devida a hipoglicemia e näo a excesso de insulina

Neuropatia pela amiodarona: estudo clínico-patológico de dois casos / Amiodarone neuropathy: clinico-pathological study of 2 cases

Dois pacientes, uma mulher de 40 anos e um homem de 75 anos, apresentavam polineurite sensitivo-motora, cujo único antecedentes era o uso de amiodarona há 4 e 6 anos respectivamente. O ENMG revelou quadro neuropático tipo axonal. A biópsia do nervo sural mostrou rarefaçäo axonal, mielínica e amielínica, bem como inclusöes lamelares osmiofílicas nas células de Schwann e no endotélio venular. Com a retirada da amiodarona houve regressäo da polineurite. A semelhança do que foi descrito na neuropatia pelo maleato de perhexiline e pela cloroquina, a amiodarona constitui constitui importante fator de induçäo de neurolipidose medicamentosa

Polirradiculoneuropatia desmielinizante inflamatória crônica. Estudo de 18 pacientes / Chronic inflammatory demyelinating polyradiculoneuropathy. Study of 18 cases

Neste estudo prospectivo analisamos as características clínicas, evoluçäo e resposta terapêutica de 18 pacientes com a forma idiopática de polirradiculoneuropatia desmielinizante inflamatória crônica, que foram acompanhados por período que variou de 4 a 127 meses. O sexo masculino predominou sobre o feminino (1,25:1) e a idade de início dos sintomas variou de 6 a 85 anos. Observamos a preponderância da forma de evoluçäo progressiva (61,1 por cento) sobre a forma recidivante (38,9 por cento), bem como a baixa ocorrência de fatores predisponentes (16,7 por cento). Todos os pacientes apresentavam comprometimento sensitivo e motor, associado a hipo ou arreflexia, enquanto apenas três (16,7 por cento) apresentavam comprometimento de nervos cranianos. No exame do líquor, as taxas de proteínas estavam elevadas em 88,9 por cento dos pacientes, com média de 203,4 mg/dl. A eletroneuromiografia mostrou alteraçöes desmielinizantes em todos os pacientes, associadas a alteraçöes axonais em 94,4 por cento deles. Em todos os sete pacientes submetidos a biopsia de nervo sural encontramos alteraçöes compatíveis com desmielinizaçäo/remielinizaçäo. A análise com imunofluorescência, realizada em três pacientes foi normal em um e evidenciou depósito de anticorpos anti-CD3 em dois e anti-HLA-Dr em um. Optamos pela prednisona como tratamento inicial em todos os pacientes, sendo mantida posteriormente em doses reduzidas e em dias alternados em 72,2 por cento deles. Dois pacientes (11,1 por cento) estäo assintomáticos mesmo após retirada total da medicaçäo e introduzimos azatioprina, associada ou näo ao corticóide, nos quatro pacientes com má resposta à prednisona. Até a última avaliaçäo, 16 pacientes (88,9 por cento) evoluíram com melhora funcional.