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1.
Acta Diabetol ; 57(7): 891-898, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32124075

RESUMO

BACKGROUND: Studies by our group demonstrated brain-derived neurotrophic factor (BDNF) levels in blood and BDNF-Val66met-SNP as potential biomarkers in chemotherapy-induced peripheral neuropathy. Here, we evaluate symptoms of peripheral neuropathy (PN) and depression in patients with type II diabetes mellitus in search of an association with serum BDNF levels and the Val66Met-SNP. METHODS: In total, 90 patients enrolled in the study; 23 (25.6%) had known PN, as determined by nerve conduction studies (NCS-PN), and 67 (74.4%) were not diagnosed with PN (U-PN). PN symptoms were assessed and graded in these groups using the total neuropathy score (TNSr) and DN4 scales. Small nerve fiber testing of sensitivity thresholds to cold, warm and hot pain signals was performed using the Q-sense device. Depression was assessed using the PHQ9 questionnaire. BDNF protein levels and Val66Met-SNP were determined with ELISA and Sanger sequencing, respectively. RESULTS: NCS-PN patients showed lower serum BDNF levels alongside significantly higher TNSr, DN4 and PHQ9 scores and lower hot pain sensitivity thresholds as compared to U-PN patients. Patients with Met-BDNF-SNP showed increased TNSr scores and lower hot pain sensitivity thresholds as compared to patients with Val-BDNF-SNP. Depression showed a weaker correlation with sensitivity thresholds to hot pain signals as compared to TNSr and DN4 scores. CONCLUSIONS: Diminished peripheral BDNF resources and Met-BDNF-SNP genotype are associated with augmented symptoms of PN in patients with type II diabetes mellitus. Sensitivity thresholds to hot pain signals may be less influenced by depression and possibly more accurately detect PN symptoms in diabetic patients.


Assuntos
Fator Neurotrófico Derivado do Encéfalo/sangue , Fator Neurotrófico Derivado do Encéfalo/genética , Diabetes Mellitus Tipo 2/complicações , Neuropatias Diabéticas/sangue , Neuropatias Diabéticas/genética , Polimorfismo de Nucleotídeo Único , Adulto , Idoso , Substituição de Aminoácidos , Biomarcadores/sangue , Estudos de Casos e Controles , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/genética , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Masculino , Metionina/genética , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/sangue , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/genética , Valina/genética
2.
World Neurosurg ; 138: 231-241, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32169619

RESUMO

BACKGROUND: Filum terminale arteriovenous fistulas (FTAVFs) are rare, and the pathogenesis of these fistulas remains unclear. They may be either congenital or acquired in origin. The authors report 3 cases of FTAVFs in association with severe spinal canal stenosis. The authors also review literature of FTAVFs associated with spinal canal stenosis. CASE DESCRIPTION: All 3 cases harboring FTAVFs manifested with progressive myelopathy and bowel/bladder dysfunction following long history of back pain, sciatica, and/or intermittent claudication. The fistulas were located around or at the level of spinal canal stenosis and supplied by the anterior spinal and/or lateral sacral arteries with cranial drainage from the dilated vein of the filum terminale to the perimedullary veins. The first and third cases were treated concomitantly by performing instrumented fusion with decompressive laminectomy along with occlusion of the fistula with good results. The second case was unsuccessfully treated by endovascular treatment through the lateral sacral artery and denied further surgical treatment. CONCLUSIONS: Our 3 case reports may provide additional evidence supporting an acquired etiology of FTAVFs, probably secondary to the severe central canal stenosis. From our review, the level of the fistulas in most patients is correlated with the level of spinal canal stenosis. The authors preferred the concomitant surgical treatment by performing decompressive laminectomy and obliteration of the fistula in the same surgical session.


Assuntos
Fístula Arteriovenosa/complicações , Fístula Arteriovenosa/terapia , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/terapia , Estenose Espinal/complicações , Estenose Espinal/terapia , Idoso , Cauda Equina/patologia , Cauda Equina/cirurgia , Descompressão Cirúrgica/métodos , Embolização Terapêutica/métodos , Procedimentos Endovasculares/métodos , Feminino , Humanos , Laminectomia/métodos , Região Lombossacral , Masculino , Pessoa de Meia-Idade , Fusão Vertebral/métodos
3.
Brain ; 143(2): 480-490, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32040566

RESUMO

Ataxia, causing imbalance, dizziness and falls, is a leading cause of neurological disability. We have recently identified a biallelic intronic AAGGG repeat expansion in replication factor complex subunit 1 (RFC1) as the cause of cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) and a major cause of late onset ataxia. Here we describe the full spectrum of the disease phenotype in our first 100 genetically confirmed carriers of biallelic repeat expansions in RFC1 and identify the sensory neuropathy as a common feature in all cases to date. All patients were Caucasian and half were sporadic. Patients typically reported progressive unsteadiness starting in the sixth decade. A dry spasmodic cough was also frequently associated and often preceded by decades the onset of walking difficulty. Sensory symptoms, oscillopsia, dysautonomia and dysarthria were also variably associated. The disease seems to follow a pattern of spatial progression from the early involvement of sensory neurons, to the later appearance of vestibular and cerebellar dysfunction. Half of the patients needed walking aids after 10 years of disease duration and a quarter were wheelchair dependent after 15 years. Overall, two-thirds of cases had full CANVAS. Sensory neuropathy was the only manifestation in 15 patients. Sixteen patients additionally showed cerebellar involvement, and six showed vestibular involvement. The disease is very likely to be underdiagnosed. Repeat expansion in RFC1 should be considered in all cases of sensory ataxic neuropathy, particularly, but not only, if cerebellar dysfunction, vestibular involvement and cough coexist.


Assuntos
Ataxia/fisiopatologia , Ataxia Cerebelar/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Neuronite Vestibular/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Ataxia/complicações , Cerebelo/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Neurológico/efeitos adversos , Doenças do Sistema Nervoso Periférico/complicações , Reflexo Anormal/fisiologia , Transtornos das Sensações/etiologia , Transtornos das Sensações/fisiopatologia , Síndrome , Neuronite Vestibular/complicações
4.
World Neurosurg ; 138: 77-82, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32105875

RESUMO

BACKGROUND: Chronic abdominal pain is a debilitating condition known for its multifactorial nature. Outcomes with spinal cord stimulation (SCS) for abdominal pain syndromes are noticeably absent in the literature. To date, there have been no published reports of novel waveforms of SCS for management of chronic abdominal pain. We assessed the efficacy and durability of BurstDR SCS in reducing abdominal pain and analgesic consumption. CASE DESCRIPTION: We performed a retrospective review of medical records from 3 patients with different etiologies of abdominal pain (postherniorrhaphy pain syndrome, Crohn disease, abdominal neuropathy). All patients underwent thoracic laminectomy for BurstDR SCS paddle lead and pulse generator placement after a successful trial stimulation period. Data were collected with a telephone survey after a minimum duration of >24 months following implantation. Pain scores were measured using a numeric rating scale. Two of 3 patients were entirely pain-free and reported Patient Global Impression of Change scores of 7 after a minimum follow-up of >24 months. While the third patient continued to have chronic as well as episodic abdominal pain, he was able to discontinue all narcotic pain medications and experienced a 33% decrease in frequency and 60% decrease in severity of monthly pain exacerbations. He reported satisfaction and a Patient Global Impression of Change of 6. CONCLUSIONS: BurstDR SCS is a new programming modality, and long-term follow-up is necessary to determine its durability. Despite varying etiologies of abdominal pain, this series suggests BurstDR SCS sustained for >2 years might be effective as a treatment for abdominal pain syndromes. Future studies of SCS would benefit from standardized abdominal pain scores and high-powered studies using global patient registries.


Assuntos
Dor Abdominal/terapia , Estimulação da Medula Espinal/métodos , Doença Crônica , Doença de Crohn/complicações , Eletrodos Implantados , Feminino , Herniorrafia/efeitos adversos , Humanos , Nervos Intercostais/patologia , Laminectomia , Masculino , Pessoa de Meia-Idade , Entorpecentes/uso terapêutico , Medição da Dor , Dor Pós-Operatória/terapia , Satisfação do Paciente , Doenças do Sistema Nervoso Periférico/complicações , Resultado do Tratamento
5.
Diabetes Res Clin Pract ; 162: 108096, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32109517

RESUMO

AIM: This study aimed to investigate the association of sarcopenia and muscle mass with both peripheral neuropathy and nerve function in type 2 diabetes mellitus. METHODS: A total of 1794 patients (937 men and 857 women) with type 2 diabetes, with a mean age of 60.22 years, were enrolled for a cross-sectional study; of these, 183 patients were enrolled for a follow-up study with a median follow-up of 2.7 years. All participants underwent nerve conduction studies and muscle mass index (ASM/HT2) measurements. The composite Z scores for the sensory nerve conduction velocity (SCV) and the motor nerve conduction velocity (MCV) were calculated. The changes in ASM/HT2, SCV, and MCV were calculated from the measurements nearly 2 years apart and classified into three groups: a decrease in ASM/HT2 of >3%, a minor change within ±3%, and an increase in ASM/HT2 of >3%. RESULTS: The ASM/HT2 of men was positively associated with the composite Z scores of MCV and SCV, and sarcopenia highly correlated with DPN after adjusting for confounding factors. The optimal cutoff point for ASM/HT2 that indicated DPN was 7.09 kg/m2. Furthermore, increases in ASM/HT2 independently predicted a greater benefit of MCV and SCV increment outcomes, whereas a minor change in ASM/HT2 only significantly associated with lower benefit in terms of SCV increment. However, this phenomenon was not observed in women. CONCLUSIONS: Sarcopenia and DPN exhibited a close association. The increased muscle mass improved the partial MCVs and SCVs. However, a sex-related discrepancy was observed in this phenomenon.


Assuntos
Diabetes Mellitus Tipo 2/complicações , Condução Nervosa/fisiologia , Doenças do Sistema Nervoso Periférico/complicações , Sarcopenia/complicações , Estudos Transversais , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Sarcopenia/fisiopatologia
6.
Internist (Berl) ; 61(3): 261-269, 2020 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-32072188

RESUMO

Vasculitic neuropathies result from inflammation of the vasa nervorum followed by ischemia and destruction of the peripheral nerve. The inflammation can be systemic or localized, i.e. non-systemic. Systemic vasculitis can be divided into primary and secondary forms. The latter is associated with, e.g. connective tissue diseases, infections, cancer or induced by certain drugs. Around two thirds of patients with systemic vasculitis develop vasculitic neuropathy presenting as characteristic painful, multifocal mononeuropathy of acute onset. The group of non-systemic neuropathies has grown in recent years with the addition of diabetic and non-diabetic lumbosacral radiculoplexus neuropathies, among others. Within the group of connective tissue diseases, other non-vasculitic neuropathies can occur as nerve-entrapment syndromes and sensory ataxic neuropathy. The aim of this article is to present a condensed overview of neuropathies associated with vasculitis and connective tissue diseases and to communicate characteristic clinical symptoms supporting rapid diagnostic and therapeutic procedures.


Assuntos
Doenças do Tecido Conjuntivo/complicações , Doenças do Sistema Nervoso Periférico/complicações , Polineuropatias/diagnóstico , Vasculite/complicações , Vasculite/diagnóstico , Humanos , Dor/diagnóstico , Dor/etiologia , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/terapia , Polineuropatias/terapia , Vasculite/terapia
7.
Neurology ; 94(12): e1314-e1319, 2020 03 24.
Artigo em Inglês | MEDLINE | ID: mdl-31992683

RESUMO

OBJECTIVE: To investigate the following among patients with phrenic neuropathy: (1) occurrences of water immersion activity-induced dyspnea; (2) clinical, electrophysiologic, sonographic, and pulmonary function test abnormalities; and (3) frequency of documented counseling regarding the risks of water immersion activities. METHODS: We identified all patients with test-confirmed phrenic neuropathy seen from January 1, 2000, to December 31, 2018, at Mayo Clinic. RESULTS: Of 535 patients with phrenic neuropathy, documentation of dyspnea with water activities was identified in 4% (22/535). The risks of water immersion were only documented in patients having experienced this problem. The majority had isolated phrenic neuritis or neuralgic amyotrophy syndrome (77.3%), mean age was 55 years (range 31-79), and most patients were men (81.9%). Patients had right-sided (45.5%) or bilateral (54.5%) phrenic neuropathy. None had isolated left phrenic involvement. Near-fatal drowning occurred in 18.2% (4/22), with persons needing assistance to be rescued from the water, following diving into water. Dyspnea with water immersion was the only symptom in 4.5% (1/22) and the presenting respiratory symptom in 36.4% (8/22). A range of electrophysiologic, sonographic, and pulmonary function test abnormalities including mild abnormalities were seen and not found to be significantly different from those in patients in whom water-induced dyspnea was not recorded. CONCLUSION: Respiratory distress with water immersion activities is a serious complication of phrenic neuropathies. Physician-documented counseling is lacking. Isolated phrenic neuritis, neuralgic amyotrophy, and right-sided and bilateral phrenic involvement are most commonly implicated, but the range of severity and testing abnormalities suggest that all patients with neuralgic amyotrophy or phrenic neuropathy should be warned especially about diving into water.


Assuntos
Dispneia/etiologia , Doenças do Sistema Nervoso Periférico/complicações , Nervo Frênico/patologia , Esportes Aquáticos , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
8.
J Surg Oncol ; 121(1): 182-186, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31228351

RESUMO

This is a case report of a 68-year-old male with stage III right lower extremity lymphedema following right inguinal lymph node dissection and adjuvant chemoradiotherapy for Hodgkin's lymphoma. He developed peripheral neuropathy and radiation-induced right femoral artery thrombosis, treated with saphenous vein graft. He underwent three vascularized lymph node transfers (VLNTs) to the upper medial thigh, posterior calf, and ankle with placement of nanofibrillar collagen scaffolds. Three months after surgery, he had volume reduction, less neuropathic pain, and improved ambulation.


Assuntos
Linfedema/complicações , Linfedema/cirurgia , Doenças do Sistema Nervoso Periférico/complicações , Doenças Vasculares Periféricas/complicações , Idoso , Humanos , Linfedema/diagnóstico por imagem , Linfedema/patologia , Masculino , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/patologia , Doenças Vasculares Periféricas/diagnóstico por imagem , Doenças Vasculares Periféricas/patologia
9.
World Neurosurg ; 134: e1053-e1061, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31760186

RESUMO

OBJECTIVE: Lesions affecting the sciatic nerve (SN) can mimic lumbar radiculopathy. In patients presenting with sciatica, approximately 10% have a nondiscogenic etiology. Through neurological examination and imaging, it may be possible to confirm nondiscogenic sciatica (NDS). This study aims to present a series of 6 patients with infragluteal NDS, highlighting clinical and imaging aspects that may suggest this diagnosis. METHODS: This is a retrospective study of 6 patients treated for NDS from 2010 to 2018. The mean and median ages were 41.2 and 38.5 years, respectively, with all patients female. RESULTS: All patients presented with sciatic pain, tenderness to deep infragluteal palpation, and a positive Tinel's sign related to the SN. Four patients were referred for surgical treatment, whereas 1 underwent pharmacological therapy and 1 underwent incisional biopsy and radiotherapy. In our series, 6 different causes for NDS were diagnosed: 1 nontumorous cause: extrauterine endometriosis and 5 tumors: metastasis from rectal adenocarcinoma, low-grade sarcoma, schwannoma, high-grade sarcoma, and myxoma. CONCLUSIONS: Differentiating between discogenic and NDS can be challenging for clinicians. When patients present with sciatic pain, a Tinel's sign related to the SN elicited at the deep infragluteal region, tenderness to deep infragluteal palpation, occasionally with an SN motor deficit and imaging findings of the lumbar spine that do not justify a discogenic source, the cause should be considered nondiscogenic and they should be scheduled for magnetic resonance imaging of the gluteal and pelvic region.


Assuntos
Adenocarcinoma/secundário , Endometriose/diagnóstico por imagem , Mixoma/diagnóstico por imagem , Neurilemoma/diagnóstico por imagem , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Neoplasias Retais/patologia , Sarcoma/diagnóstico por imagem , Ciática/etiologia , Adenocarcinoma/complicações , Adenocarcinoma/diagnóstico por imagem , Adulto , Endometriose/complicações , Endometriose/patologia , Feminino , Humanos , Imagem por Ressonância Magnética , Mixoma/complicações , Mixoma/patologia , Neurilemoma/complicações , Neurilemoma/patologia , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/patologia , Neoplasias do Sistema Nervoso Periférico/complicações , Neoplasias do Sistema Nervoso Periférico/patologia , Exame Físico , Estudos Retrospectivos , Sarcoma/complicações , Sarcoma/patologia , Nervo Isquiático/diagnóstico por imagem , Nervo Isquiático/patologia , Adulto Jovem
10.
Reumatol. clín. (Barc.) ; 15(6): 368-369, nov.-dic. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-189661

RESUMO

Paciente masculino de 56 años con poliangitis microscópica y polineuropatía periférica simétrica de extremidades con p-ANCA positivo, patrón EMG de mononeuritis múltiple y biopsia cutánea con vasculitis inespecífica, quien presentaba flebectasia con plétora y trayectos tortuosos en el dorso de los dedos en ambas manos, paresia e hipoestesia digital en manos y pies con limitación funcional. Mediante la administración de prednisona, azatioprina y pulso de ciclofosfamida, mejoró rápidamente de los síntomas generales pero muy lentamente de la neuropatía. Después de 47 meses de evolución persiste discreta flebectasia digital en manos y ligera hipoestesia en manos y pies, con parámetros de laboratorio normales. La flebectasia, probablemente, fue el resultado de una disautonomía por vasculitis de la vasa nervorum y podría ser un signo a ratificar en casos semejantes


The patient was a 56-year-old man with microscopic polyangiitis and symmetrical peripheral polyneuropathy of the extremities who was positive for p-ANCA, EMG pattern of mononeuritis multiplex and skin biopsy showing the presence of nonspecific vasculitis. He had phlebectasia with plethora and tortuous vessels on dorsum of the fingers on both hands, paresis and hypoesthesia of fingers and toes with functional limitations. The administration of prednisone, azathioprine and a cyclophosphamide pulse achieved rapid improvement in the general symptoms, but the changes in the neuropathy occurred very slowly. After 47 months of treatment, he had mild phlebectasia in fingers and slight hypoesthesia in hands and feet, with normal laboratory tests. Phlebectasia was probably the result of an autonomic dysfunction due to vasculitis of the vasa nervorum and could be a sign to look for in similar cases


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Dedos/irrigação sanguínea , Poliangiite Microscópica/complicações , Doenças do Sistema Nervoso Periférico/complicações , Polineuropatias/complicações , Doenças Vasculares/etiologia , Veias/patologia , Dilatação Patológica/etiologia , Doenças Vasculares/patologia
11.
BMJ Case Rep ; 12(11)2019 Nov 26.
Artigo em Inglês | MEDLINE | ID: mdl-31776158

RESUMO

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/enzimologia , Mieloblastina , Doenças do Sistema Nervoso Periférico/complicações , Esclerodermia Difusa/complicações , Idoso , Humanos , Masculino
12.
Medicina (Kaunas) ; 55(11)2019 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-31731539

RESUMO

Diabetic foot ulcers (DFUs) are the fastest growing chronic complication of diabetes mellitus, with more than 400 million people diagnosed globally, and the condition is responsible for lower extremity amputation in 85% of people affected, leading to high-cost hospital care and increased mortality risk. Neuropathy and peripheral arterial disease trigger deformities or trauma, and aggravating factors such as infection and edema are the etiological factors for the development of DFUs. DFUs require identifying the etiology and assessing the co-morbidities to provide the correct therapeutic approach, essential to reducing lower-extremity amputation risk. This review focuses on the current treatment strategies for DFUs with a special emphasis on tissue engineering techniques and regenerative medicine that collectively target all components of chronic wound pathology.


Assuntos
Complicações do Diabetes/terapia , Pé Diabético/terapia , Desbridamento/métodos , Diabetes Mellitus/terapia , Pé Diabético/etiologia , Humanos , Terapia a Laser/métodos , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Dermatopatias/complicações
13.
BMC Musculoskelet Disord ; 20(1): 501, 2019 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-31666060

RESUMO

BACKGROUND: Charcot neuroarthropathy is a systemic disease that generates pathological changes in the musculoskeletal system, causing instability, dislocations, and deformities. Charcot neuroarthropathy of the knee, due to either diabetes mellitus or syringomyelia, is anecdotally reported with the epidemic of the diseases. However, idiopathic sensory peripheral neuropathy can inflict osteoarticular structures directly, inducing a dysfunctional Charcot neuroarthropathy. An early diagnosis and effective relief of the symptomatic deformity is essential for the treatment. CASE PRESENTATION: We report the case of a patient with idiopathic sensory peripheral neuropathy who presented with a swelling right knee, as well as distorted and painless gait disorder, diagnosed as Charcot neuroarthropathy of the knee. Partial weight bearing with a hinged knee brace was used to correct the abnormal alignment and gait posture, and bisphosphonates were prescribed to decrease pathological bone resorption. Although the alignment and Knee Society Score got a gradual deterioration, the combination of orthosis and pharmacy could alleviate the symptom to a certain extent. CONCLUSION: The diagnosis of Charcot neuroarthropathy of the knee is rare that requiring early diagnosis. The presence of features, including painlessness, numbness, and deformed arthropathy following chronic-onset algesthesia loss should be taken carefully.


Assuntos
Doença de Charcot-Marie-Tooth/diagnóstico por imagem , Doença de Charcot-Marie-Tooth/etiologia , Articulação do Joelho/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Adulto , Humanos , Masculino
14.
Postgrad Med ; 131(7): 546-549, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31483199

RESUMO

Objectives: To identify the cognitive and functional deficits in a well-characterized group of patients with vasculitis of the nervous system. Methods: Sixty-seven patients diagnosed with Central Nervous System (CNS) or Peripheral nervous System (PNS) vasculitis over a 14-year period were retrospectively identified. Data on clinical presentation, laboratory, radiographic and tissue biopsy investigations, and treatment were collated. Cognitive, functional and quality of life evaluation assessments were performed in 31 patients who agreed to participate and included Addenbrooke's Cognitive Examination-revised (ACE-R), Nottingham Extended Activities of Daily Living (NEADL) and EQ-5D-3L quality of life questionnaires. Results: CNS vasculitis patients exhibited cognitive impairment, with a mean ACE-R score of 74/100 (standard deviation (SD) 16). NEADL and EQ-5D-3L scores were in the impaired range at 41/66 (SD 21) and 57/81 (SD 22), respectively. Patients with just PNS vasculitis exhibited fewer cognitive deficits with ACE-R and NEADL scores of 87 (SD 8) and 46 (SD 16) respectively. EQ-5D-3L score was in the impaired range of 65 (SD 22). Conclusions: Vasculitis of the nervous system and, in particular, CNS vasculitis causes cognitive impairment and deficits in functional ability. Such patients should be targeted for cognitive rehabilitation.


Assuntos
Disfunção Cognitiva/psicologia , Doenças do Sistema Nervoso Periférico/psicologia , Vasculite do Sistema Nervoso Central/psicologia , Atividades Cotidianas , Adulto , Idoso , Idoso de 80 Anos ou mais , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/fisiopatologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/psicologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/fisiopatologia , Estudos de Coortes , Estudos Transversais , Feminino , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/fisiopatologia , Arterite de Células Gigantes/psicologia , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/fisiopatologia , Granulomatose com Poliangiite/psicologia , Nível de Saúde , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Qualidade de Vida , Estudos Retrospectivos , Vasculite/complicações , Vasculite/tratamento farmacológico , Vasculite/fisiopatologia , Vasculite/psicologia , Vasculite do Sistema Nervoso Central/complicações , Vasculite do Sistema Nervoso Central/tratamento farmacológico , Vasculite do Sistema Nervoso Central/fisiopatologia , Adulto Jovem
15.
Neurologist ; 24(5): 152-154, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31478999

RESUMO

INTRODUCTION: Glial fibrillary acidic protein (GFAP) immunoglobulin G is a recently discovered biomarker of an autoimmune central nervous system disorder characterized by a steroid-responsive meningoencephalomyelitis. CASE REPORT: A 63-year-old man with rheumatoid arthritis on etanercept presented with steroid-responsive subacute encephalopathy and foot drop. Brain and sural nerve biopsies demonstrated a T-cell perivascular infiltrate. Cerebrospinal fluid studies 18 months into the course of the illness demonstrated a GFAP antibody on mouse tissue immunofluorescence confirmed by cell-based assay. The patient was treated with steroids and cyclophosphamide leading to resolution of his symptoms. CONCLUSION: This case expands on the previously reported cases of GFAP immunoglobulin G autoimmunity by describing an associated inflammatory large fiber peripheral neuropathy.


Assuntos
Antirreumáticos/efeitos adversos , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Encefalopatias/imunologia , Etanercepte/efeitos adversos , Proteína Glial Fibrilar Ácida/imunologia , Autoimunidade , Encefalopatias/complicações , Humanos , Imunoglobulina G , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/imunologia
16.
Arq Neuropsiquiatr ; 77(7): 451-455, 2019 07 29.
Artigo em Inglês | MEDLINE | ID: mdl-31365635

RESUMO

METHODS: Sensory neuronopathies (SN) are a group of peripheral nerve disorders characterized by multifocal non-length-dependent sensory deficits and sensory ataxia. Its recognition is essential not only for proper management but also to guide the etiological investigation. The uncommon SN clinical picture and its rarity set the conditions for the misdiagnosis and the diagnostic delay, especially in non-paraneoplastic SN. Therefore, our objectives were to characterize the diagnostic odyssey for non-paraneoplastic SN patients, as well as to identify possible associated factors. We consecutively enrolled 48 non-paraneoplastic SN patients followed in a tertiary neuromuscular clinic at the University of Campinas (Brazil). All patients were instructed to retrieve their previous medical records, and we collected the data regarding demographics, disease onset, previous incorrect diagnoses made and the recommended treatments. RESULTS: There were 34 women, with a mean age at the diagnosis of 45.9 ± 12.2 years, and 28/48 (58%) of the patients were idiopathic. Negative sensory symptoms were the heralding symptoms in 25/48 (52%); these were asymmetric in 36/48 (75%) and followed a chronic course in 35/48 (73%). On average, it took 5.4 ± 5.3 years for SN to be diagnosed; patients had an average of 3.4 ± 1.5 incorrect diagnoses. A disease onset before the age of 40 was associated to shorter diagnosis delay (3.7 ± 3.4 vs. 7.8 ± 6.7 years, p = 0.01). CONCLUSIONS: These results suggest that diagnostic delay and misdiagnosis are frequent in non-paraneoplastic SN patients. As in other rare conditions, increased awareness in all the healthcare system levels is paramount to ensure accurate diagnosis and to improve care of these patients.


Assuntos
Doenças do Sistema Nervoso Periférico/diagnóstico , Adulto , Idoso , Brasil , Diagnóstico Tardio , Erros de Diagnóstico/classificação , Feminino , Marcha Atáxica/etiologia , Gânglios Sensitivos/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/complicações
17.
Intern Med ; 58(23): 3433-3439, 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31391398

RESUMO

We herein report a 28-year-old woman with type 1 diabetes with an asymptomatic pontine lesion and diabetic amyotrophy. She had suffered from diabetes from 10 years old. Treatment in a hospital reduced the hemoglobin A1c level from 14.2% to 7.2% for approximately 2 months. She suffered from acute-onset pain and weakness of the lower limb muscles without central nervous system manifestations. Magnetic resonance imaging showed high-intensity lesions at the brainstem and lower limb muscles on T2-weighted images. These findings and symptoms gradually resolved. Rapid treatment of poor glycemic control might increase the risk of asymptomatic pontine lesions and diabetic amyotrophy.


Assuntos
Encefalopatias/etiologia , Diabetes Mellitus Tipo 1/dietoterapia , Neuropatias Diabéticas/dietoterapia , Ponte , Adulto , Glicemia/metabolismo , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/tratamento farmacológico , Neuropatias Diabéticas/complicações , Neuropatias Diabéticas/tratamento farmacológico , Feminino , Hemoglobina A Glicada/metabolismo , Humanos , Hipoglicemiantes/uso terapêutico , Insulina/uso terapêutico , Plexo Lombossacral , Imagem por Ressonância Magnética , Debilidade Muscular/etiologia , Dor/complicações , Dor/dietoterapia , Dor/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/complicações , Doenças do Sistema Nervoso Periférico/dietoterapia , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Resultado do Tratamento
18.
Diabetes Metab Syndr ; 13(2): 1523-1528, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31336516

RESUMO

AIMS: The aim was to see the frequency of CAN in type 2 diabetes mellitus patients with peripheral neuropathy, and its association with peripheral nerve conduction abnormalities. METHODS: A cross-sectional study at BIRDEM was conducted in 62 patients with type 2 diabetes mellitus having electrophysiologically diagnosed peripheral neuropathy. CAN was detected by four clinical tests - heart rate response to deep breathing and valsalva maneuver, blood pressure response to standing and sustained handgrip. RESULT: The study showed that all patients had CAN - 14.52% had early, 26.67% had definitive and 59.68% had severe CAN. Patients with severe CAN had significantly reduced nerve conduction velocity and amplitude of peripheral nerves (sural 4.36 ±â€¯12.77 vs 9.65 ±â€¯17.77 m/s, p = 0.009; 2.23 ±â€¯1.89 vs 3.01 ±â€¯2.76 mV, p = 0.001; peroneal 7 ±â€¯4.23 vs 8.53 ±â€¯5.99 mV, p = 0.047; tibial 0.008 ±â€¯0.03 vs 0.026 ±â€¯0.05 mV, p = 0.009) and higher serum triglyceride levels (221.17 ±â€¯120.61 vs 197.76 ±â€¯68.43 mg/dl, p = 0.033). CONCLUSION: Diabetic patients with peripheral neuropathy have CAN, the severity of which increases with worsening neuropathy.


Assuntos
Doenças do Sistema Nervoso Autônomo/etiologia , Diabetes Mellitus Tipo 2/fisiopatologia , Cardiomiopatias Diabéticas/etiologia , Doenças do Sistema Nervoso Periférico/complicações , Adolescente , Adulto , Idoso , Doenças do Sistema Nervoso Autônomo/metabolismo , Doenças do Sistema Nervoso Autônomo/patologia , Biomarcadores/análise , Glicemia/análise , Estudos Transversais , Cardiomiopatias Diabéticas/metabolismo , Cardiomiopatias Diabéticas/patologia , Feminino , Seguimentos , Hemoglobina A Glicada/análise , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Adulto Jovem
19.
J Clin Neurosci ; 68: 128-133, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31326286

RESUMO

Subacute myelo-optico neuropathy (SMON) patients typically suffer from sequelae that cause sleep disturbances. We sought to examine the prevalence of sleep problems among SMON patients. We conducted a questionnaire-based survey concerning sleep problems among 106 SMON patients, and 110 age- and gender-matched control participants. The prevalence of subjective insomnia (6 ≤ Athens Insomnia Scale score) was 89.6% among SMON patients, which was significantly higher than among control participants 54.4%. Sleep quality measured with the Pittsburgh Sleep Quality Index (PSQI) revealed that the prevalence of poor sleepers (6 ≤ PSQI score) was higher among SMON patients than control participants (75.6% vs 39.6%, respectively). Subscale analyses of rapid eye movement sleep behavior disorder screening questionnaire revealed that scores on two items ("dreams match nocturnal behavior" and "limb movements") were significantly higher among SMON patients than control participants. In addition, daytime sleepiness scores were significantly higher among SMON patients than control participants (4 ≤ Epworth Sleepiness Scale scores: 54.0% vs 29.0%, respectively). The current study revealed that most SMON patients suffer from insomnia with dissatisfactory sleep quality, likely due to their long-term physical sequelae. Moreover, SMON patients showed higher rates of daytime sleepiness and sleep medication intake, which could be related to reduced activity during the day, as well as insomnia.


Assuntos
Clioquinol/efeitos adversos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/complicações , Distúrbios do Início e da Manutenção do Sono/etiologia , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Distúrbios do Início e da Manutenção do Sono/epidemiologia , Inquéritos e Questionários
20.
Int Rev Neurobiol ; 145: 127-176, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31208522

RESUMO

Peripheral neuropathy is a common and debilitating complication of diabetes and prediabetes. Recent clinical studies have identified an association between the development of neuropathy and dyslipidemia in prediabetic and diabetic patients. Despite the prevalence of this complication, studies identifying molecular mechanisms that underlie neuropathy progression in prediabetes or diabetes are limited. However, dysfunctional mitochondrial pathways in hereditary neuropathy provide feasible molecular targets for assessing mitochondrial dysfunction in neuropathy associated with prediabetes or diabetes. Recent studies suggest that elevated levels of dietary saturated fatty acids (SFAs) associated with dyslipidemia impair mitochondrial dynamics in sensory neurons by inducing mitochondrial depolarization, compromising mitochondrial bioenergetics, and impairing axonal mitochondrial transport. This causes lower neuronal ATP and apoptosis. Conversely, monounsaturated fatty acids (MUFAs) restore nerve and sensory mitochondrial function. Understanding the mitochondrial pathways that contribute to neuropathy progression in prediabetes and diabetes may provide therapeutic targets for the treatment of this debilitating complication.


Assuntos
Complicações do Diabetes/fisiopatologia , Diabetes Mellitus/fisiopatologia , Transtornos Heredodegenerativos do Sistema Nervoso/fisiopatologia , Dinâmica Mitocondrial/fisiologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Animais , Dislipidemias/complicações , Dislipidemias/fisiopatologia , Gânglios Espinais/metabolismo , Transtornos Heredodegenerativos do Sistema Nervoso/complicações , Humanos , Hiperglicemia/complicações , Hiperglicemia/fisiopatologia , Doenças do Sistema Nervoso Periférico/complicações
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