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1.
J Contemp Dent Pract ; 22(6): 605-609, 2021 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-34393114

RESUMO

AIM AND OBJECTIVE: The present study compared the frictional forces of three types of self-ligating lingual appliances. MATERIALS AND METHODS: The lingual appliances (2D, Forestadent; Alias, Ormco; and Clippy L, Tomy International) consisted of a self-ligating bracket (second premolar) and two self-ligating tubes (first and second molars) bonded to a stainless steel jig and attached to a "drawing-friction tester." Full-size and non-full-size stainless steel archwires were tested, and the static and kinetic friction acting on six lingual appliance/wire combinations was estimated (n = 5). Three-dimensional micro-computed tomography (micro-CT) analysis of each premolar bracket was performed. The frictional forces were compared between the bracket/wire combinations using the Kruskal-Wallis and Mann-Whitney U tests. RESULTS: The Alias and Clippy L bracket/wire combinations had greater contact between the wire surfaces and bracket slots compared to the 2D bracket/wire combination. For all lingual appliances, the static and kinetic frictional forces were significantly higher for the full-size than non-full-size archwire. The 2D bracket, which had a wider outer wing, had less frictional force than the other appliances. The Alias, which had a narrower outer wing, had a significantly lower frictional force than the Clippy L. CONCLUSIONS: Frictional force was significantly higher for heavier full-size bracket/archwire combinations than for non-full-size archwires. The 2D bracket had lower frictional force due to its archwire-holding mechanism. The outer wing width may influence the frictional resistance. CLINICAL SIGNIFICANCE: The frictional forces of self-ligating lingual appliances vary, and bracket design and archwire size may influence the frictional performance.


Assuntos
Braquetes Ortodônticos , Fios Ortodônticos , Ligas Dentárias , Análise do Estresse Dentário , Fricção , Humanos , Teste de Materiais , Desenho de Aparelho Ortodôntico , Aço Inoxidável , Titânio , Microtomografia por Raio-X
2.
Brain Nerve ; 73(7): 819-828, 2021 Jul.
Artigo em Japonês | MEDLINE | ID: mdl-34234040

RESUMO

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune peripheral neuropathy with chronic progression over 2 months or more. In this review, we provide an overview of key clinical studies involved in the development of CIDP therapy, as well as a discussion of changes in the concept of treatment. Although a definitive therapy has not yet been established, international and Japanese clinical guidelines recommend three first-line treatment options for symptom improvement, namely corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, based on the results of clinical studies conducted before the early 2000s. Since 2010, several treatments for the prevention of CIDP relapse (maintenance therapy) have been developed and more recently, studies have focused on the optimization of each treatment. On the other hand, CIDP treatment is associated with several limitations, including a lack of biomarkers for prediction of disease progression, differences in response to treatment between CIDP subtypes, and difficulties in the selection of appropriate maintenance therapy. Several studies aimed at resolving these issues are currently being conducted. (Received 22 May, 2020; Accepted 12 January, 2021; Published 1 July, 2021).


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Corticosteroides , Humanos , Imunoglobulinas Intravenosas , Plasmaferese , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Recidiva , Resultado do Tratamento
3.
J Neurol ; 268(10): 3835-3844, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-33797627

RESUMO

OBJECTIVES: To clarify whether serum neurofilament light chains (NfLs) serve as a biomarker of axonal damage in patients with chronic inflammatory demyelinating polyneuropathy (CIDP), especially in patients with anti-neurofascin 155 (NF155) antibodies. METHODS: The Simoa system was used to examine serum NfL levels from 58 patients with CIDP, including 13 anti-NF155 antibody-positive patients, and from 14 age- and sex-matched healthy individuals. Serum NfL levels were evaluated before and after treatment in eight patients with anti-NF155 antibodies. Clinical features, electrophysiological findings, and cerebrospinal fluid (CSF) protein levels, were evaluated. The pathological features of sural nerves from 40 patients were also examined. RESULTS: Serum NfL levels were significantly higher in patients with CIDP than in healthy individuals (median 29.63 vs. 7.71 pg/mL, p < 0.001) and were correlated with both modified Rankin Scale scores (r = 0.584, p < 0.001) and CSF protein levels (r = 0.432, p = 0.001). The NfL levels of anti-NF155 antibody-positive patients were higher than those of antibody-negative patients (p = 0.005). Serum NfL levels were negatively correlated with compound muscle action potential amplitudes of the tibial nerves (r = - 0.404, p = 0.004) and positively correlated with the degree of active axonal degeneration in the pathological findings (r = 0.485, p = 0.001). In the antibody-positive group, NfL levels and antibody titers decreased after treatment in all examined patients. CONCLUSION: Serum NfL correlated with pathological indices of axonal degeneration, and may serve as a biomarker that reflects active axonal damage of CIDP.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Biomarcadores , Humanos , Filamentos Intermediários , Nervo Sural
4.
J Neurol Sci ; 421: 117305, 2021 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-33540321

RESUMO

INTRODUCTION: Although polyneuropathy in patients with immunoglobulin light chain (AL) amyloidosis has been considered to be attributable to axonal degeneration resulting from amyloid deposition, patients with nerve conduction parameters indicating demyelination that mimics chronic inflammatory demyelinating polyneuropathy (CIDP) have also been reported anecdotally. METHODS: We evaluated the electrophysiological and pathological features of 8 consecutive patients with AL amyloidosis who were referred for sural nerve biopsy. RESULTS: Although findings of axonal neuropathy predominantly in the lower limbs were the cardinal feature, all patients showed one or more abnormalities of nerve conduction velocities or distal motor latencies. In particular, 2 of these patients fulfilled the definite electrophysiological for CIDP defined by the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS). On electron microscopic examination of sural nerve biopsy specimens, Schwann cells apposed to amyloid fibrils became atrophic in all patients, suggesting that amyloid deposits directly affect neighboring tissues. Additionally, detachment of the neurilemma from the outermost compacted myelin lamella was seen where amyloid fibrils were absent in 4 patients. Electrophysiological findings suggestive of demyelination were more conspicuous in these patients compared with the other patients. The detachment of the neurilemma from the outermost compacted myelin lamella was particularly conspicuous in patients who fulfilled the definite EFNS/PNS electrophysiological criteria for CIDP. CONCLUSION: Abnormalities of myelinated fibers unrelated to amyloid deposition may frequently occur in AL amyloidosis. Disjunction between myelin and the neurilemma may induce nerve conduction abnormalities suggestive of demyelination.


Assuntos
Amiloidose de Cadeia Leve de Imunoglobulina , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Humanos , Cadeias Leves de Imunoglobulina , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Condução Nervosa , Nervos Periféricos , Nervo Sural
5.
Microsc Res Tech ; 84(2): 171-179, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32869466

RESUMO

Surface pre-reacted glass-ionomer (S-PRG) technology allows for the release of multiple ions. This study was performed to investigate the remineralization of etched enamel after removal of the bracket using a novel paste containing S-PRG filler. Surfaces of polished enamel were etched with phosphoric acid and then subdivided into two regions. Bracket bonding resin was thinly applied to one region. After 24-hr immersion in artificial saliva, the bonding resin on the enamel surfaces was removed by grinding with tungsten carbide bur at low-speed, followed by polishing with one of four different polishing systems: (a) nonfluoridated paste (NF); (b) fluoridated paste (F); (c) S-PRG filler-containing paste (S-PRG); or (d) nonfluoridated plus S-PRG filler-containing paste (NF + S-PRG) (n = 15). The polished specimens were then immersed in an artificial saliva solution for 7 days. Nanoindentation testing of enamel surfaces was performed and their mechanical properties were compared. Representative specimens were examined with scanning electron microscope. In all specimens, the mechanical properties of the enamel surfaces were markedly degraded by acid etching. However, the mechanical properties of both regions (etched and resin-infiltrated enamels) showed recovery after polishing and 7-day immersion. Polishing with NF + S-PRG paste led to significant recovery of mechanical properties compared to polishing with NF or S-PRG paste alone, and remineralization was equivalent to that seen with F paste. Porous etched enamel surfaces were filled with a remineralization layer after each polishing procedure and 7-day immersion in all polishing groups. Polishing using NF + S-PRG paste can facilitate enamel remineralization after bracket removal.

7.
J Oral Biosci ; 62(4): 299-305, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32882366

RESUMO

BACKGROUND: The periodontal ligament (PDL), which surrounds the tooth root, contains mesenchymal stem cells (MSCs) capable of differentiating into osteoblasts, cementoblasts, and fibroblasts under normal conditions. These MSCs are thought to have important roles in the repair and regeneration of injured periodontal tissues. However, since there is no useful marker for MSCs in the PDL, the characteristics and distributions of these cells remain unclear. Gli1, an essential hedgehog signaling transcription factor, functions in undifferentiated cells during embryogenesis. Previous studies have demonstrated that the dental epithelial and mesenchymal cells positive for Gli1 in developing teeth have stem cell properties, including the ability to form colonies and pluripotency. Therefore, the focus of this review is the stem cell properties of Gli1-positive cells in the PDL, with an emphasis on the differentiation ability of osteoblasts for the regeneration of periodontal tissues. HIGHLIGHT: Lineage tracing analysis identified Gli1-positive PDL cells as MSCs that contribute to the formation of periodontal tissues and can regenerate alveolar bone. CONCLUSION: Gli1 is a potential stem cell marker in the PDL. A more definitive understanding of the functions of Gli1-positive cells could be useful for the development of regenerative methods using the MSCs in the PDL.


Assuntos
Proteínas Hedgehog , Ligamento Periodontal , Cemento Dentário , Células-Tronco , Proteína GLI1 em Dedos de Zinco
8.
J Neuropathol Exp Neurol ; 79(12): 1303-1312, 2020 12 04.
Artigo em Inglês | MEDLINE | ID: mdl-32856086

RESUMO

To clarify the pathogenesis of anti-myelin-associated glycoprotein (MAG) antibody neuropathy associated with IgM monoclonal gammopathy (anti-MAG neuropathy), sural nerve biopsy specimens from 15 patients were investigated. Sodium channels, potassium channels, contactin-associated protein 1 (Caspr1), contactin 1, and neurofascin were evaluated by immunofluorescence in teased-fiber preparations. Immunoreactivity to the pan-sodium channel in both anti-MAG neuropathy patients and in normal controls was concentrated at the node of Ranvier unless there was demyelination, which was defined as the widening of the node of Ranvier. However, this immunoreactivity became weak or disappeared as demyelination progressed. In contrast, KCNQ2 immunostaining was nearly absent even in the absence of demyelination. The lengths of Caspr1, contactin 1, and pan-neurofascin immunostaining sites at the paranode were significantly increased compared with those of normal controls despite the absence of demyelination. The length of paranodal neurofascin staining correlated with the anti-MAG antibody titer, nerve conduction indices, the frequency of de/remyelination in teased-fiber preparations, and the frequency of widely spaced myelin (p < 0.05, p < 0.05, p < 0.01, and <0.05, respectively). These findings suggest that nodal and paranodal molecular alterations occur in early stages preceding the morphological changes associated with demyelination in anti-MAG neuropathy.


Assuntos
Autoanticorpos , Imunoglobulina M , Bainha de Mielina/patologia , Glicoproteína Associada a Mielina/imunologia , Paraproteinemias/patologia , Doenças do Sistema Nervoso Periférico/patologia , Nervo Sural/patologia , Idoso , Idoso de 80 Anos ou mais , Biópsia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Bainha de Mielina/metabolismo , Condução Nervosa , Paraproteinemias/imunologia , Paraproteinemias/metabolismo , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/metabolismo , Canais de Sódio/metabolismo , Nervo Sural/imunologia , Nervo Sural/metabolismo
9.
JMIR Res Protoc ; 9(4): e17117, 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-32234705

RESUMO

BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral neuropathy that is currently classified into several clinical subtypes, which are presumed to have different pathogenic mechanisms. Recently, studies identified a subgroup of patients with CIDP who were positive for IgG4 autoantibodies against paranodal proteins, such as neurofascin-155 and contactin-1, who respond poorly to first-line therapies for typical CIDP, including intravenous immunoglobulin therapy. OBJECTIVE: This study aims to evaluate the efficacy and safety of intravenous rituximab according to IgG4 autoantibody status in patients with refractory CIDP. METHODS: The Evaluation of the Efficacy and Safety of Rituximab in Refractory CIDP Patients with IgG4 Autoantibodies in the Exploratory Clinical (RECIPE) trial consists of 2 cohorts: a multicenter, placebo-controlled, randomized study cohort of 15 patients with IgG4 autoantibody-positive CIDP (rituximab:placebo = 2:1) and an open-label trial cohort of 10 patients with antibody-negative CIDP. The primary endpoint is improvement in functional outcome assessed using the adjusted Inflammatory Neuropathy Cause and Treatment Disability Scale score at 26, 38, or 52 weeks after the start of treatment with rituximab in patients with CIDP and anti-paranodal protein antibodies. Secondary outcome measures include grip strength, manual muscle testing sum scores, results of nerve conduction studies, and other functional scales. RESULTS: We plan to enroll 25 cases for the full analysis set. Recruitment is ongoing, with 14 patients enrolled as of January 2020. Enrollment will close in September 2020, and the study is planned to end in December 2021. CONCLUSIONS: This randomized controlled trial will determine if rituximab is safe and effective in patients with anti-paranodal antibodies. An open-label study will provide additional data on the effects of rituximab in patients with antibody-negative CIDP. The results of the RECIPE trial are expected to provide evidence for the positioning of rituximab as a pathogenesis-based therapeutic for refractory CIDP. TRIAL REGISTRATION: ClinicalTrials.gov NCT03864185, https://clinicaltrials.gov/ct2/show/NCT03864185 ; The Japan Registry of Clinical Trials jRCT2041180037, https://jrct.niph.go.jp/en-latest-detail/jRCT2041180037. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/17117.

10.
J Neurol Neurosurg Psychiatry ; 91(6): 650-659, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32245766

RESUMO

OBJECTIVE: To describe the pathological features of Guillain-Barré syndrome focusing on macrophage-associated myelin lesions. METHODS: Longitudinal sections of sural nerve biopsy specimens from 11 patients with acute inflammatory demyelinating polyneuropathy (AIDP) exhibiting macrophage-associated demyelinating lesions were examined using electron microscopy. A total of 1205 nodes of Ranvier were examined to determine the relationship of the macrophage-associated demyelinating lesions with the nodal regions. Additionally, immunohistochemical and immunofluorescent studies were performed to elucidate the sites of complement deposition. RESULTS: Overall, 252 macrophage-associated myelin lesions were identified in longitudinal sections. Of these, 40 lesions exhibited complete demyelination with no association with the lamellar structures of myelin. In 183 lesions, macrophage cytoplasm was located at internodes without association with the nodes of Ranvier or paranodes. In particular, these internodal lesions were more frequent in one patient (152 lesions). In the remaining 29 lesions, the involvement of nodal regions was obvious. Lesions involving nodal regions were more frequently observed than those involving internodes in four patients. Invasion of the macrophage cytoplasmic processes into the space between the paranodal myelin terminal loops and the axolemma from the nodes of Ranvier was observed in three of these patients. Immunostaining suggested complement deposition corresponding to putative initial macrophage-associated demyelinating lesions. CONCLUSIONS: The initial macrophage-associated demyelinating lesions appeared to be located at internodes and at nodal regions. The sites at which the macrophages initiated phagocytosis of myelin might be associated with the location of complement deposition in certain patients with AIDP.


Assuntos
Doenças Desmielinizantes/patologia , Síndrome de Guillain-Barré/patologia , Macrófagos/ultraestrutura , Bainha de Mielina/ultraestrutura , Neurônios/ultraestrutura , Idoso , Axônios/patologia , Axônios/ultraestrutura , Feminino , Humanos , Macrófagos/patologia , Masculino , Pessoa de Meia-Idade , Bainha de Mielina/patologia , Neurônios/patologia , Nós Neurofibrosos/patologia , Nós Neurofibrosos/ultraestrutura
11.
Neurology ; 94(16): e1726-e1737, 2020 04 21.
Artigo em Inglês | MEDLINE | ID: mdl-32217776

RESUMO

OBJECTIVE: To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS: We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients. RESULTS: Upper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group (p < 0.01). CONCLUSIONS: This study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA-positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA-negative patients.


Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/fisiopatologia , Debilidade Muscular/fisiopatologia , Nervos Periféricos/irrigação sanguínea , Doenças do Sistema Nervoso Periférico/fisiopatologia , Distúrbios Somatossensoriais/fisiopatologia , Idoso , Asma/etiologia , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/imunologia , Eletrodiagnóstico , Feminino , Humanos , Nefropatias/etiologia , Extremidade Inferior/inervação , Pneumopatias/etiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Mieloblastina/imunologia , Condução Nervosa , Otorrinolaringopatias/genética , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/patologia , Peroxidase/imunologia , Dermatopatias Vasculares/etiologia , Distúrbios Somatossensoriais/etiologia , Nervo Sural/patologia , Tomografia Computadorizada por Raios X , Extremidade Superior/inervação
12.
Dent Mater J ; 39(4): 593-600, 2020 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-32092724

RESUMO

We investigated the surface modification of orthodontic stainless steel wire using electrophoretic deposition (EPD) of bioactive glass (BG). BG coatings were characterized by spectrophotometry, three-dimensional (3D) focal laser scanning microscopy and scanning electron microscopy. The mechanical properties of the BG-coated wires were estimated nanoindentation, three-point bending and drawing friction tests. BG-coated specimens prepared at higher voltage showed higher values for both reflectance and L* compared to those prepared at lower voltage. Specimens coated at higher voltage had significantly lower surface roughness than those coated at lower voltage, and their BG layers had higher hardness and elastic modulus values. In the three-point bending test, BG-coated wires produced significantly lower elastic modulus than non-coated wires. Most BG-coated specimens produced similar frictional forces to those produced by non-coated specimens. The surface modification technique applying EPD and BG coating to orthodontic stainless steel wire could be used to develop new esthetical orthodontic wire.


Assuntos
Braquetes Ortodônticos , Fios Ortodônticos , Ligas Dentárias , Fricção , Vidro , Teste de Materiais , Aço Inoxidável , Propriedades de Superfície , Titânio
13.
Materials (Basel) ; 13(2)2020 Jan 19.
Artigo em Inglês | MEDLINE | ID: mdl-31963840

RESUMO

In current orthodontic practice, miniscrew implants (MSIs) for anchorage and bone fixation plates (BFPs) for surgical orthodontic treatment are commonly used. MSIs and BFPs that are made of bioabsorbable material would avoid the need for removal surgery. We investigated the mechanical, degradation and osseointegration properties and the bone-implant interface strength of the AZ31 bioabsorbable magnesium alloy to assess its suitability for MSIs and BFPs. The mechanical properties of a Ti alloy (TiA), AZ31 Mg alloy (MgA), pure Mg and poly-L-lactic acid (PLA) were investigated using a nanoindentation test. Also, pH changes in the solution and degradation rates were determined using immersion tests. Three-dimensional, high-resolution, micro-computed tomography (CT) of implants in the rat femur was performed. Biomechanical push-out testing was conducted to calculate the maximum shear strength of the bone-implant interface. Scanning electron microscopy (SEM), histological analysis and an evaluation of systemic inflammation were performed. MgA has mechanical properties similar to those of bone, and is suitable for implants. The degradation rate of MgA was significantly lower than that of Mg. MgA achieved a significantly higher bone-implant bond strength than TiA. Micro-CT revealed no significant differences in bone density or bone-implant contact between TiA and MgA. In conclusion, the AZ31 Mg alloy is suitable for both MSIs and BFPs.

14.
Intern Med ; 59(3): 435-438, 2020 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-31588080

RESUMO

We herein report the case of a 67-year-old man who presented with the acute onset of limb weakness. Brain magnetic resonance imaging revealed multiple abnormal-signal-intensity lesions. Steroids were administered, and the patient initially responded. Nerve conduction testing findings were consistent with demyelinating polyneuropathy. A sural nerve biopsy specimen revealed fascicles with extensive onion-bulb formation. Although skin and sural nerve biopsies showed no atypical cellular infiltration, the histopathological diagnosis of intravascular large B-cell lymphoma was obtained by a brain biopsy. The neuropathy in this patient may be attributed to a demyelinating process independent of ischemic damage by lymphoma.


Assuntos
Doenças Desmielinizantes/etiologia , Doenças Desmielinizantes/fisiopatologia , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Debilidade Muscular/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Esteroides/uso terapêutico , Idoso , Humanos , Linfoma Difuso de Grandes Células B/fisiopatologia , Imageamento por Ressonância Magnética , Masculino , Debilidade Muscular/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Resultado do Tratamento
20.
J Neurol Neurosurg Psychiatry ; 90(9): 988-996, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31227562

RESUMO

OBJECTIVE: To evaluate the clinical and pathological correlations characterising each clinical subtype of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: We assessed 106 consecutive patients who had CIDP fulfilling the European Federation of Neurological Societies and Peripheral Nerve Society criteria and had been referred for sural nerve biopsy. Patients with anti-neurofascin 155, anti-contactin 1 and anti-LM1 antibodies were excluded. RESULTS: 55 patients were classified as having typical CIDP. Regarding atypical CIDP, the multifocal acquired demyelinating sensory and motor (MADSAM) (n=15), distal acquired demyelinating symmetric (DADS) (n=16) and pure sensory (n=15) forms were major subtypes, while the pure motor (n=4) and focal (n=1) forms were rare. Nerve conduction studies revealed that distal motor latencies and F-wave latencies were markedly prolonged in the typical CIDP group but relatively preserved in the MADSAM group. Motor conduction velocity was conspicuously slowed in the DADS group, and distal motor latencies were markedly prolonged in the pure sensory group. Sural nerve biopsy specimens from patients with MADSAM, DADS and pure sensory type tended to show extreme variation in myelinated fibre density among fascicles due to focal myelinated fibre loss or onion-bulb formation, whereas patients with typical CIDP tended to show mild fascicular variation. Epineurial lymphocytic infiltration was conspicuous in cases with marked fascicular variation in myelinated fibre density. CONCLUSIONS: Preferential involvement of distal and proximal segments and uniform pathological features in typical CIDP indicate a role of humoral factors at sites where the blood-nerve barrier is deficient. By contrast, focal lesions in MADSAM, DADS and pure sensory forms may share neuropathic mechanisms primarily affecting the nerve trunk.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Biópsia , Eletrofisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Condução Nervosa/fisiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/classificação , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Nervo Sural/patologia
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