RESUMO
BACKGROUND AND AIMS: To date, the correlation between sarcopenia, which exists before a stroke, and acute stroke outcome remains partially understood. This study aims to evaluate the skeletal muscle mass deficit using the bioelectrical impedance analysis in patients with acute ischemic stroke. METHODS: We enrolled 164 geriatric patients with acute ischemic stroke (108 males and 56 females) who underwent the bioelectrical impedance analysis. We evaluated clinical outcomes and their impact on patients with the skeletal muscle mass deficit determined using the skeletal muscle mass index. RESULTS: The skeletal muscle mass deficit was obtained using the bioelectrical impedance analysis in 101 patients. Patients with the skeletal muscle mass deficit determined by the skeletal muscle mass index exhibited severe neurological impairment and functional status on admission; moreover, they tended to display poor functional outcome and prolonged hospital stay. Logistic regression analysis revealed that the skeletal muscle mass deficit remained an independent poor outcome predictor. CONCLUSIONS: This study establishes the presence of the skeletal muscle mass deficit in over half patients with acute ischemic stroke. Furthermore, the skeletal muscle mass deficit correlates with neurological impairment owing to stroke with poorer functional prognosis.
Assuntos
Composição Corporal , Isquemia Encefálica/fisiopatologia , Músculo Esquelético/fisiopatologia , Sarcopenia/fisiopatologia , Acidente Vascular Cerebral/fisiopatologia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/complicações , Isquemia Encefálica/diagnóstico , Isquemia Encefálica/terapia , Impedância Elétrica , Feminino , Avaliação Geriátrica , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Força Muscular , Prognóstico , Recuperação de Função Fisiológica , Fatores de Risco , Sarcopenia/complicações , Sarcopenia/diagnóstico , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/terapia , Reabilitação do Acidente Vascular Cerebral , Fatores de TempoRESUMO
IgG4-related inflammatory pseudotumor is a feature of IgG4-related disease and develops in various organs. Intracranial IgG4-related inflammatory pseudotumor is rare, and data on the clinical course and response to treatment are insufficient in the literature. We herein report a patient with IgG4-related inflammatory pseudotumor who had magnetic resonance imaging findings similar to meningioma. Tumorectomy was discontinued because of the intraoperative rapid diagnosis, which revealed the infiltration of lymphocytes and plasma cells. She received oral prednisolone therapy for IgG4-related inflammatory pseudotumor, and the tumor size had significantly decreased after six months of treatment.
Assuntos
Granuloma de Células Plasmáticas , Neoplasias Meníngeas , Meningioma , Feminino , Humanos , Meningioma/diagnóstico por imagem , Imunoglobulina G , Granuloma de Células Plasmáticas/diagnóstico por imagem , Prednisolona/uso terapêutico , Diagnóstico Diferencial , Neoplasias Meníngeas/diagnóstico por imagemRESUMO
Objective Muscle atrophy is observed in a subset of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Its manifestation is associated with a poor functional prognosis and poor response to immunomodulatory therapies. We evaluated muscle atrophy in patients with CIDP using a bioelectrical impedance analysis (BIA). Methods We enrolled 12 patients with CIDP for a BIA of muscle atrophy. Of these 12 patients, 10 were diagnosed with typical CIDP, 1 with multifocal acquired demyelinating sensory and motor neuropathy, and 1 with distal acquired demyelinating symmetric neuropathy. All 12 patients underwent a series of assessments and evaluations, including a BIA and computed tomography (CT). A correlation was found between the skeletal muscle mass determined by the BIA and that found using CT of the muscles. Results The BIA provided values for each patient's skeletal muscle mass index (SMI) ranging from 4.1 to 8.1 kg/m2. Four of the patients with CIDP had SMI values below the threshold for sarcopenia. CT of the patients' muscles provided scores indicating grades of muscle atrophy in the upper and lower extremities. A comparison of the outcomes from these two measures showed a good correlation between their muscle atrophy ratings (p<0.05). Conclusion We found that a BIA and muscle CT provided muscle atrophy assessments of equivalent accuracy. Therefore, a BIA can be a simple alternative to muscle CT that is suitable for regular use in daily clinical practice as a reliable tool for assessing muscle atrophy in patients with CIDP.
Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Humanos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Impedância Elétrica , Atrofia Muscular/diagnóstico por imagem , Atrofia Muscular/etiologia , Tomografia Computadorizada por Raios X , Músculos , Músculo Esquelético/diagnóstico por imagemRESUMO
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic disorder that frequently affects the peripheral nervous system and consists of three distinct conditions: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously Wegener's granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome). The neuropathic features associated with this condition usually include mononeuritis multiplex, which reflects the locality of lesions. Findings suggestive of vasculitis are usually found in the epineurium and occur diffusely throughout the nerve trunk. Nerve fiber degeneration resulting from ischemia is sometimes focal or asymmetric and tends to become conspicuous at the middle portion of the nerve trunk. The attachment of neutrophils to endothelial cells in the epineurial vessels is frequently observed in patients with ANCA-associated vasculitis; neutrophils play an important role in vascular inflammation by binding of ANCA. The positivity rate of ANCA in EGPA is lower than that in MPA and GPA, and intravascular and tissue eosinophils appear to participate in neuropathy. Immunotherapy for ANCA-associated vasculitis involves the induction and maintenance of remission to prevent the relapse of the disease. A combination of glucocorticoids along with cyclophosphamide, rituximab, methotrexate, or mycophenolate mofetil is considered depending on the severity of the condition of the organ to induce remission. A combination of low-dose glucocorticoids and azathioprine, rituximab, methotrexate, or mycophenolate mofetil is recommended to maintain remission. The efficacy of anti-interleukin-5 therapy (i.e., mepolizumab) was demonstrated in the case of refractory or relapsing EGPA. Several other new agents, including avacopan, vilobelimab, and abatacept, are under development for the treatment of ANCA-associated vasculitis. Multidisciplinary approaches are required for the diagnosis and management of the disorder because of its systemic nature. Furthermore, active participation of neurologists is required because the associated neuropathic symptoms can significantly disrupt the day-to-day functioning and quality of life of patients with ANCA-associated vasculitis.
Assuntos
Polineuropatia Paraneoplásica , Autoanticorpos/imunologia , Síndrome de Guillain-Barré/imunologia , Humanos , Imagem Multimodal , Polineuropatia Paraneoplásica/diagnóstico , Polineuropatia Paraneoplásica/imunologia , Polineuropatia Paraneoplásica/terapia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologiaRESUMO
OBJECTIVE: Painful ophthalmoplegia includes nonspecific magnetic resonance imaging (MRI) manifestations and various clinical features including orbital pain and cranial nerve palsies. Treatment for painful ophthalmoplegia remains controversial. The aim of this report was to describe detailed clinical features, MRI findings, treatments, and prognosis of patients with painful ophthalmoplegia. Patients and Methods. We retrospectively investigated four cases of patients with painful ophthalmoplegia diagnosed using the International Classification of Headache Disorders, 3rd edition. RESULTS: All patients experienced unilateral orbital pain and oculomotor nerve palsy with diplopia but no vision loss. One of the four patients was diagnosed with Tolosa-Hunt syndrome based on the appearance of a granulomatous inflammation of the cavernous sinus on MRI. No specific lesions were detected on brain MRI for the other three patients; therefore, their headaches were attributed to ischaemic ocular motor nerve palsy. In all patients, a high-intensity ring appearance around the ipsilateral optic nerve was observed on MRI. Steroid therapy was administered to these patients, and good prognoses were anticipated. CONCLUSION: These results indicate that prednisolone is a useful treatment for painful ophthalmoplegia that displays ipsilateral hyperintense ring lesions around the optic nerve on MRI, regardless of the presence of granulomatous inflammation of the cavernous sinus.
RESUMO
PURPOSE: The purpose of this study is to increase awareness of the importance of considering neuromyelitis optica spectrum disorder (NMOSD) as a differential diagnosis for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). METHODS: We report two NMOSD patients demonstrating magnetic resonance imaging (MRI) abnormalities resembling those of CADASIL. RESULTS: Brain MRIs of both patients showed symmetrical hyperintense signals in the temporal poles and cerebral hemispheres on T2 weighted images. One case also involved the bilateral external capsule. The chief complaint of both patients was loss of visual acuity, and neurologic examination showed no other apparent neurological signs or symptoms. Anti-aquaporin-4 antibodies were detected on serological examination, and NMOSD was subsequently diagnosed. Visual acuity improved following intravenous methylprednisolone therapy. One patient refused further immunological treatment. Although she remained clinically stable, gradual radiographic deterioration was observed. This deterioration then stabilized after the patient commenced oral prednisolone therapy. The other patient was treated with prednisolone and azathioprine. She is clinically stable, but we have observed gradual radiographic deterioration over the past 5 years. CONCLUSION: MRI findings in patients with NMOSD may resemble those of CADASIL, namely symmetrical hyperintensities in the temporal poles, external capsules and cerebral hemispheres. NMOSD is a differential diagnosis for CADASIL, and testing for anti-AQP4 antibodies should be considered.
Assuntos
CADASIL , Neuromielite Óptica , Autoanticorpos , CADASIL/diagnóstico por imagem , CADASIL/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Humanos , Imageamento por Ressonância Magnética , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/tratamento farmacológicoRESUMO
Mowat-Wilson syndrome (MWS) is a rare genetic disorder characterized by intellectual disability, distinctive facial features, epilepsy, and multiple anomalies caused by heterozygous loss-of-function mutations in the zinc finger E-box-binding homeobox-2 gene (ZEB2). Treatment choice is very important as patients with MWS because patients sometimes develop drug-resistant epilepsy. Here, we report the case of a 45-year-old male patient with MWS who developed drug-resistant status epilepticus after a 26-years seizure-free period while taking multiple anti-seizure medications. He showed a characteristic magnetic resonance imaging finding with a focal lesion in his left thalamic pulvinar nucleus, a finding not previously reported in status epilepticus with MWS. We succeeded in controlling seizures in the patient after trying multiple new antiseizure drug combinations. These findings indicate that patients with MWS may develop drug-resistant status epilepticus with age, even after a long-term seizure-free period, which can be managed with anti-seizure medication. Therefore, careful monitoring of seizures is important for the treatment of people with MWS, even in patients who have not experienced seizures for a long time.
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/inervaçãoRESUMO
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/metabolismoRESUMO
We herein report a 33-year-old woman who was an asymptomatic hepatitis B virus (HBV) carrier and presented with distal muscle weakness in the legs and asymmetrical paresthesia in the distal extremities. A nerve biopsy specimen revealed fibrinoid necrosis associated with inflammatory infiltration in the perineural space, and deposition of hepatitis B core antigen and C4d complement was detected in the vascular endothelial cells as well as around the vessels. She was diagnosed with HBV-related vasculitic neuropathy and treated with intravenous immunoglobulin (IVIG). Her symptoms completely subsided after eight weeks. Vasculitic neuropathy rarely develops in the chronic inactive stages of HBV infection. This is the first report of an HBV-inactive carrier with vasculitic neuropathy successfully treated with IVIG.
Assuntos
Portador Sadio , Hepatite B/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Vasculite/etiologia , Adulto , Células Endoteliais/patologia , Feminino , Vírus da Hepatite B , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Doenças do Sistema Nervoso Periférico/virologia , Vasculite/virologiaRESUMO
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by reactivation of the JC virus under an immunosuppressed state. This condition carries a high risk of cryptococcal meningitis. We herein report a 65-year-old woman who simultaneously developed PML and cryptococcal meningitis and presented with bilateral sixth nerve palsy. She had been treated with methotrexate and infliximab for rheumatoid arthritis. Her symptoms improved with antifungal drug treatment and discontinuation of immunosuppression therapy. Although concurrent PML and cryptococcal meningitis is rare, it should be considered in immunosuppressed patients.
Assuntos
Antifúngicos/uso terapêutico , Antirreumáticos/efeitos adversos , Artrite Reumatoide/tratamento farmacológico , Terapia de Imunossupressão/efeitos adversos , Infliximab/efeitos adversos , Leucoencefalopatia Multifocal Progressiva/tratamento farmacológico , Meningite Criptocócica/tratamento farmacológico , Metotrexato/efeitos adversos , Idoso , Antirreumáticos/uso terapêutico , Feminino , Humanos , Infliximab/uso terapêutico , Vírus JC/isolamento & purificação , Leucoencefalopatia Multifocal Progressiva/etiologia , Meningite Criptocócica/etiologia , Metotrexato/uso terapêutico , Resultado do TratamentoRESUMO
The impact of a polymer additive (polyvinylpyrrolidone, PVP) on hydrogen bonding in amorphous sugar matrices as well as on the glass transition temperature, T(g), were examined by temperature scanning Fourier transform infrared spectroscopy (TS-FTIR). An amorphous sugar matrix containing PVP was prepared by air-drying an aqueous solution of a sugar-PVP mixture. The hydrogen bonds in the sugar-PVP mixture (sugar-PVP and sugar-sugar hydrogen bonds) were analyzed from the IR peak positions corresponding to the stretching vibration of C==O groups of PVP and O--H groups of the sugar and the temperature dependence of the peak position of the O--H stretching vibration band. The addition of PVP to amorphous mono and disaccharides significantly lowered the extent of hydrogen bond formation while interactions between sugars and the PVP tended to prevent the disruption of hydrogen bonds due to increasing temperature, the magnitude of which was larger for larger oligomers. The T(g) value for the amorphous sugar was increased by the addition of PVP in many cases. As the size of sugar molecule became larger, the relative magnitude of the increased T(g) by PVP to the difference between the T(g) values for sugar alone and PVP alone became larger and then reached a certain level; it was slight in the case of glucose. Collectively, these results demonstrate that the magnitude of the impact of PVP on an amorphous sugar matrix strongly vary and are dependent on the types of sugar.
Assuntos
Carboidratos/química , Polímeros/química , Varredura Diferencial de Calorimetria , Cristalização , Ligação de Hidrogênio , Povidona/química , Espectrofotometria Infravermelho , Espectroscopia de Infravermelho com Transformada de Fourier , Temperatura , VibraçãoRESUMO
Aceruloplasminemia is an autosomal recessive inherited disorder caused by ceruloplasmin gene mutations. The loss of ferroxidase activity of ceruloplasmin due to gene mutations causes a disturbance in cellular iron transport. We herein describe a patient with aceruloplasminemia, who presented with diabetes mellitus that was treated by insulin injections, liver hemosiderosis treated by phlebotomy therapy, and neurological impairment. A genetic analysis of the ceruloplasmin gene revealed novel compound heterozygous mutations of c.1286_1290insTATAC in exon 7 and c.2185delC in exon 12. This abnormal compound heterozygote had typical clinical features similar to those observed in aceruloplasminemia patients with other gene mutations.
Assuntos
Ceruloplasmina/deficiência , Hemossiderose/complicações , Hemossiderose/terapia , Distúrbios do Metabolismo do Ferro/complicações , Doenças Neurodegenerativas/complicações , Flebotomia/efeitos adversos , Idoso , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/tratamento farmacológico , Feminino , Humanos , Hipoglicemiantes/uso terapêutico , Mutação INDEL , Insulina/uso terapêuticoRESUMO
Styrene trimers migrate from polystyrene food container into foods. We evaluated the estrogenic activity of styrene trimers such as 2,4,6-triphenyl-1-hexene (ST-1), 1a-phenyl-4a-(1'-phenylethyl)tetralin (ST-2), 1a-phenyl-4e-(1'-phenylethyl)tetralin(ST-3), 1e-phenyl-4a-(1'-phenylethyl)tetralin (ST-4), and 1e-phenyl-4e-(1'-phenylethyl)tetralin (ST-5) using the reporter-gene assay with MVLN cells stably expressing the estrogen-stimulated reporter gene, and it was confirmed that ST-1, ST-3, and ST-4 had estrogen-like activity. On the other hand, ST-2 and ST-5 had anti-estrogen-like activity. We examined the estrogenic activity in vivo of ST-1, ST-3, and ST-4. The styrene trimers were administered to pregnant rats, and the effects on the offspring were examined. ST-1, ST-3, or ST-4 (0, 10, 100, 1000 microg/kg body wt/day) were subcutaneously injected into pregnant rats from gestational Day 11 through 17, and the male offspring were sacrificed on postnatal days (PND) 101-103. In the ST-4 treatment groups, the relative anogenital distance on PND 3 was significantly shortened. The relative testis weight was remarkably decreased in all styrene trimer treatment groups. Relative weights of the prostate and epididymides significantly decreased in the ST-4 treatment groups. The relative brain weight was markedly reduced in the ST-3 and ST-4 treatment groups. A significant decrease of the Sertoli cell count was observed in the ST-1 and ST-4 treatment groups. The serum follicle stimulating hormone level was remarkably reduced in all styrene trimer treatment groups. The luteinizing hormone level was significantly decreased and the testosterone level increased in the ST-1 and ST-4 groups. These results suggest that prenatal exposure to estrogenic styrene trimers at low levels obstructed genital organ development, and disrupted the endocrine systems of male rat offspring.
Assuntos
Genitália Masculina/efeitos dos fármacos , Exposição Materna , Estireno/farmacologia , Animais , Feminino , Masculino , Polímeros , Gravidez , Ratos , Ratos Sprague-Dawley , Estireno/químicaRESUMO
We herein report a case of peripheral neuropathy following exposure to large amounts of glyphosate-based herbicide. A 70-year-old man suffered from pain and purpura in the left sole following exposure to glyphosate-based herbicide. Pain and purpura spread to the opposite side and increased in severity. Mild weakness of the lower limbs was also observed. A sural nerve biopsy revealed the infiltration of lymphocytes around small vessels in the epineurium with numerous eosinophils, deposition of hemosiderins and focal axonal degeneration, compatible with findings of vasculitic neuropathy. Glyphosate-based herbicides should be recognized as a causative agent of vasculitic neuropathy.
Assuntos
Glicina/análogos & derivados , Herbicidas/toxicidade , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Vasculite/induzido quimicamente , Idoso , Glicina/toxicidade , Humanos , Masculino , Doenças do Sistema Nervoso Periférico/patologia , Vasculite/patologia , GlifosatoRESUMO
Temperature scanning Fourier transform infrared, TS-FTIR, spectroscopy of various amorphous sugar matrixes was conducted to investigate the relationship between the glass transition temperature, T(g), of an amorphous sugar matrix and the nature of the hydrogen bonds in the matrix. An amorphous sugar matrix was prepared by air-drying an aqueous solution of sugar, and the degree of formation of hydrogen bonds in the matrix was evaluated at different temperatures using the peak positions of the IR band corresponding to the O-H stretching vibration at around 3400 cm(-1). The T(g) value increased with increasing peak position of the O-H stretching vibration at T(g) and were correlated reasonably well with the magnitude of the peak shift by the temperature increase (from 25 degrees C) to the T(g) value. This demonstrates that the amorphous sugar matrix, in which the segments are fixed by fewer hydrogen bonds, has a higher thermal resistance. The glycosidic linkage largely contributes to the restriction of the segments, pyranose ring, rather than a hydrogen bond. As the degree of polymerization of pyranose rings increases, the degree of hydrogen bond formation needed to hold the matrix in a fixed position decreases. However, the magnitude of the restriction of pyranose rings by a glycosidic linkage changes depending on the type: the restrictions imposed by alpha-1,1 and -1,6 glycosidic linkages are the tightest and most flexible of all of the types of glycosidic linkages, respectively.
Assuntos
Oligossacarídeos/química , Temperatura , Ligação de Hidrogênio , Transição de Fase , Sensibilidade e Especificidade , Espectroscopia de Infravermelho com Transformada de Fourier/métodosRESUMO
OBJECTIVE: To examine intraepidermal nerve fibre densities (IENFDs) in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change (POEMS) syndrome. METHODS: The IENFDs of 11 patients with POEMS syndrome were estimated. We determined whether IENFD was associated with patient clinical features or the estimated number of nerve fibres on complete cross-sections of biopsied sural nerves. RESULTS: IENFD was significantly reduced (9.7±4.4fibres/mm) compared with normal controls (p<0.05), although the individual values varied from 1.4 to 14.4fibres/mm. The presence of glucose intolerance was significantly associated with a reduction of IENFD (p<0.05). The number of unmyelinated fibres was preserved at the sural nerve level and was not correlated with IENFD. In contrast, the number of myelinated fibres was correlated with IENFD (p<0.05). CONCLUSIONS: Some of the patients presented with a severe IENFD reduction. Because the number of unmyelinated fibres was well preserved at the level of the sural nerve biopsy, this severe reduction may indicate involvement at the most distal nerve terminals of unmyelinated fibres. Although the reduction of IENFD becomes evident as polyneuropathy becomes severe, the effects of glucose intolerance should also be considered in patients with moderate to severe reductions.
Assuntos
Epiderme/inervação , Fibras Nervosas Amielínicas/patologia , Síndrome POEMS/complicações , Síndrome POEMS/patologia , Nervo Sural/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Colágeno Tipo IV/metabolismo , Epiderme/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Ubiquitina Tiolesterase/metabolismoAssuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/imunologia , Interleucina-5/imunologia , Mononeuropatias/imunologia , Polineuropatias/imunologia , Idoso , Biópsia , Síndrome de Churg-Strauss/patologia , Síndrome de Churg-Strauss/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mononeuropatias/patologia , Mononeuropatias/fisiopatologia , Mieloblastina/imunologia , Fibras Nervosas Mielinizadas/patologia , Peroxidase/imunologia , Polineuropatias/patologia , Polineuropatias/fisiopatologia , Estudos Retrospectivos , Nervo Sural/patologiaRESUMO
To elucidate the significance of uncompacted myelin lamellae (UML) and ion channel disruption at the nodes of Ranvier in the polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, we evaluated sural nerve biopsy specimens from 33 patients with POEMS syndrome and from 7 control patients. Uncompacted myelin lamellae distribution was assessed by electron microscopy and immunofluorescence microscopy. In the POEMS patient biopsies, UML were seen more frequently in small versus large myelinated fibers. Paranodes and Schmidt-Lanterman incisures, where normal physiologic UM is located, were frequently associated with UM. Widening of the nodes of Ranvier (i.e. segmental demyelination) was not associated with UML. There was axonal hollowing with neurofilament condensation at Schmidt-Lanterman incisures with abnormal UML, suggesting axonal damage at those sites in the POEMS patient biopsies. Myelin sheath irregularity was conspicuous in large myelinated fibers and was associated with abnormally widened bizarrely shaped Schmidt-Lanterman incisures. Indirect immunofluorescent studies revealed abnormalities of sodium (pan sodium) and potassium (KCNQ2) channels, even at nonwidened nodes of Ranvier. Thus, UML was not apparently associated with segmental demyelination but seemed to be associated with axonal damage. These observations suggest that nodal ion channel disruption may be associated with functional deficits in POEMS syndrome patient nerves.