scholarly journals Nerve Ultrasound as a Decisive Tool in Nonsystemic Vasculitic Neuropathy: A Case Report

2016 ◽  
Vol 8 (2) ◽  
pp. 108-114 ◽  
Author(s):  
Daniela Leupold ◽  
Ansgar Felbecker ◽  
Barbara Tettenborn ◽  
Thomas Hundsberger

Introduction: The additional value of peripheral nerve ultrasound in acquired immune-mediated neuropathies has recently been reported. Its impact in vasculitic neuropathy is yet to be defined. We report electrophysiological and nerve ultrasound studies in a patient with nonsystemic vasculitic neuropathy at first diagnosis and in response to immunosuppression. Case Report: A 44-year-old female presented with painful neuropathy and weakness of the intrinsic hand muscles. Electrodiagnostic studies revealed severe axonal neuropathy of the nerves of the left arm. On nerve ultrasound, massive and patchy swelling of these nerves was detected. Clinical, laboratory, and radiological evidence of nonneuromuscular involvement and systemic vasculitic diseases was absent. Hence, nonsystemic vasculitic neuropathy was diagnosed without the possibility of histological verification. After 6 months of systemic immunosuppression with steroids and cyclophosphamide, clinical symptoms improved in parallel with neurosonography. In contrast, electrophysiological studies remained pathological despite clinical improvement. Conclusions: Neurosonography studies in nonsystemic vasculitic neuropathy are rare but might be an ancillary technique to guide noninvasive diagnosis and therapeutic monitoring. Morphological analysis of nerves and changes in response to treatment could be well visualized. Additionally, neurosonography might be useful to target nerve biopsy.

2018 ◽  
Vol 89 (6) ◽  
pp. A7.2-A7
Author(s):  
Dev Nathani ◽  
Michael H Barnett ◽  
Judith Spies ◽  
John Pollard ◽  
Matthew C Kiernan

IntroductionNerve biopsy remains the gold standard to diagnose vasculitic neuropathy. Conversely, biopsy has imperfect sensitivity and entails risks associated with an invasive procedure. Methods to improve diagnostic accuracy remain important considerations given the severity of the disease, added with the risks associated with subsequent therapy, particularly in ill-defined cases.MethodsClinical, laboratory and neurophysiological parameters were analysed for all patients who subsequently underwent biopsy. Nerve and muscle biopsy reports were assigned pathologic categories of definite, probable, possible or absent vasculitis using standard guidelines. Correlations were assessed between pre-biopsy parameters and subsequent diagnosis of definite or probable vasculitic neuropathy (pathologically confirmed vasculitis).ResultsFrom a cohort of 207 patients who underwent nerve biopsy over 21 months, 70 were suspected of having vasculitic neuropathy prior to biopsy. Of the 70 patients, vasculitis was confirmed as definite (11.4%), probable (15.7%) or possible (10.0%) on neuropathological assessment. The most sensitive parameters for pathologically confirmed vasculitis were the presence of sensorimotor neuropathy (78%) and axonal neuropathy (67%) on nerve conduction studies (NCS) in the overall cohort. Pathologically absent vasculitis was most prevalent in patients with normal NCS (90%), chronic, symmetric symptoms (86%) and demyelinating findings on NCS (79%). The parameters with the strongest associations with pathologically confirmed vasculitis were positive autoantibody serology (50.0% vs 21.1%, p<0.01), anti-neutrophil cytoplasmic antibody (27.3% vs 6.4%, p<0.01), anti-myeloperoxidase antibody (22.7% vs 1.8%, p<0.005) and rheumatoid factor (22.7% vs 2.8%, p<0.005). In patients suspected to have vasculitis, 83.3% of anti-myeloperoxidase antibody positive patients had pathologically confirmed vasculitis. In patients not suspected to have vasculitis, acute symptoms had the strongest association with pathologically confirmed vasculitis (36.4% vs 10.5%, p<0.05).ConclusionSpecific characteristics of symptoms at the time of presentation, combined with the presence of autoantibodies and neurophysiological abnormalities, were predictive of a tissue diagnosis of vasculitic neuropathy.


Author(s):  
João Eugênio Loureiro Lopes ◽  
Helena Demuner Vallandro ◽  
Marina Dadalto Scarpati ◽  
Bruna Barcellos Chaia ◽  
Vitor Lorencini Belloti ◽  
...  

Introdução: A doença de Crohn é uma doença inflamatória intestinal (DII) que inicia um processo inflamatório crônico idiopático intestinal, levando a um quadro clínico variávelcujo principal sintoma é a diarreia crônica. O diagnóstico é feito a partir da combinação de dados clínicos, laboratoriais e exames endoscópicos. Após confirmado o diagnóstico, otratamento pode envolver classes medicamentosas como imunobiológicos. Em relação ao uso de imunobiológicos, como o adalimumabe, deve-se ter cautela pelo seu efeitoimunossupressor, que pode, em indivíduos susceptíveis, ativar focos latentes de infecção, como os de tuberculose (TB). Objetivo: Relatar um caso de tuberculose miliar como reação adversa ao uso de adalimumabe e conscientizar a comunidade médica quanto aos riscos da administração de terapia imunobiológica. Relato de caso: Paciente do sexo masculino, 31 anos, com diagnóstico de Doença de Crohn em acompanhamento ambulatorial com terapia imunobiológica com adalimumab, é admitido na emergência com quadro agudo de sintomas respiratórios e sistêmicos. Durante a investigação hospitalar, apesar de pesquisa do bacilo álcool- -ácido resistente (BAAR) no escarro ter apresentado três amostras negativas, a tomografia computadorizada (TC) de tórax apresentava padrão sugestivo de tuberculose miliar, além de um lavado broncoalveolar com pesquisa positiva para Mycobacterium tuberculosis. Confirmado o diagnóstico de TB miliar, o tratamento foi iniciado com  rifampicina, isoniazida, pirazinamida e etambutol. Conclusão: Levando em consideração a alta mortalidade e os impactos negativos da tuberculose miliar, é imprescindível que todos os pacientes com DII em uso de imunobiológicos sejam rastreados para infecção latente, através da radiografia de tórax e teste tuberculínico (PPD). Entretanto, mesmo com o rastreio regular e registro de vacinação prévia, a possibilidade de tuberculose miliar ainda não pode ser descartada em quadros clínicos inespecíficos neste perfil de pacientes.Palavras chave: Adalimumab, Tuberculose, Efeitos colaterais e reações adversas relacionados a medicamentosABSTRACTIntroduction: Crohn’s disease is an inflammatory bowel disease (IBD) that initiates a chronic idiopathic intestinal inflammatory process, leading to a variety of clinical symptoms with chronic diarrhea as the main one. Diagnosis is built from a combination of clinical, laboratory and endoscopic data. The treatment involves immunobiological drugs, such as adalimumab and due to its immunosuppressive effect, it must be administered with caution, because it isable to activate latent foci of infection, such as tuberculosis (TB), in susceptible individuals. Objective: To report a miliary tuberculosis case originated as an adverse reaction to the use of adalimumab and to make the medical community aware of the risks of administering immunobiological therapy. Case report: A 31-year-old male, diagnosed with Crohn’s Disease, undergoing immunobiological treatment with adalimumab, is admitted in the emergency department with acute respiratory and systemic symptoms. During hospitalization, despite the investigation of alcohol-acid resistant bacillus (BAAR) in sputum showing three different samples with negative results, the computerized tomography (CT) of the chest showed a pattern suggestive of miliary tuberculosis, in addition to a bronchoalveolar lavage with a positive test for Mycobacterium tuberculosis. After the diagnosis of miliary TB was confirmed, treatment with rifampicin, isoniazid, pyrazinamide and ethambutol was started. Conclusion: Considering the high mortality and negative impacts of miliary tuberculosis, it is essentialthat all patients with IBD on immunobiological treatment are screened for latent infection through chest radiograph and PPD. However, even with regular screening and registration of previous vaccinations, the possibility of miliary tuberculosis cannot be ruled out in this profile of patients with unspecific clinical conditions.Keywords: Adalimumab, Tuberculosis, Drug-related side effects and adverse reactions 


2016 ◽  
Vol 33 (S1) ◽  
pp. S333-S334
Author(s):  
J.M. Coll ◽  
G. Martínez-Alés ◽  
N. Salgado

IntroductionHyperprolactinemia can produce clinical symptoms affecting the patient's quality of life and therefore limiting therapeutic approaches to bipolar disorder.Case reportWe report a case of a 46-year-old woman, with a 10 year history of type I bipolar disorder and a microprolactinoma, who was admitted to a psychiatry inpatient unit due to a maniac episode. Current symptoms at the moment of admission included hyperthymia, verbiage, flight of ideas and insomnia. Menstrual changes and galactorrea had been present previously. Aripiprazole was introduced, reaching a dose of 30 mg/day, in addition to her usual treatment with lithium and gabapentin. Response to treatment was good and euthymia was reached within 10 days. Moreover, gabapentin was substituted by Valproic acid, and the patient was discharged once therapeutic levels were attained. Prolactin levels were measured at the moment of admission (128.75 ng/mL) and after 11 days of treatment (92.93 ng/mL).DiscussionChoosing an adequate antipsychotic agent can reduce the risk of iatrogenesis and thus enhance adherence to treatment and quality of life. Aripiprazole had previously shown a high potential at decreasing levels of prolactine. In this case, clinical practice supports scientific evidence.ConclusionsAripiprazole is an effective treatment for type I bipolar disorder. Especially, it can be a treatment of choice in patients suffering from symptoms related to high levels of prolactine, even using a high dosage.Disclosure of interestThe authors have not supplied their declaration of competing interest.


2019 ◽  
Vol 98 (4) ◽  
pp. 167-173

Introduction: Alveolar echinococcosis (AE) is a zoonosis caused by Echinococcus multilocularis. AE is primarily localised in the liver. Echinococcus multilocularis imitates tumour-like behaviour. It can metastasise through blood or lymphatic system to distant organs. Echinococcosis often remains asymptomatic due to its long incubation period and indistinct symptoms. Clinical symptoms are determined by the parasite’s location. Diagnosis of echinococcosis is based on medical history, clinical symptoms, laboratory tests, serology results, imaging methods and final histology findings. Surgical removal of the cyst with a safety margin, followed by chemotherapy is the therapeutic method of choice. Case report: We present a case report of alveolar echinococcosis in a thirty-year-old female patient in whom we surgically removed multiple liver foci of alveolar echinococcosis. The disease recurred after two years and required another surgical intervention. Conclusions: Alveolar echinococcosis is a disease with a high potential for a complete cure provided that it is diagnosed early and that the recommended therapeutic procedures are strictly adhered to.


2020 ◽  
Vol 30 (5) ◽  
pp. 82-84
Author(s):  
Ilja Skalskis

Hirschsprung disease (HD) is a developmental disorder characterized by the absence of ganglia in the distal colon, resulting in a functional obstruction. Incidence of total colonic aganglionosis (TCA) is 1 in 500 000 and it accounts for 5-10% of all cases of HD. HD should be suspected in patients with typical clinical symptoms and a high index of suspicion is appropriate for infants with a predisposing condition such as Down Syndrome (DS), or for those with a family history of HD. The treatment of choice for HD is surgical, such as Swenson, Soave, and Duhamel procedures. The goals are to resect the affected segment of the colon, bring the normal ganglionic bowel down close to the anus, and preserve internal anal sphincter function. We present a clinical case report of TCA in a child with Down syndrome (DS) and review of literature.


2021 ◽  
Vol 10 (6) ◽  
pp. 1154
Author(s):  
Eun Lee ◽  
Yun Young Lee

The prevalence of refractory Mycoplasma pneumoniae (MP) pneumonia is increasing. The present study aimed to identify the predictive factors of responses to treatment of MP pneumonia in children. A total of 149 children were diagnosed with MP pneumonia, of whom 56 were included in the good response group, 75 children in the slow response group, and 18 children in no response or progression group. Data on the clinical, laboratory, and radiologic features were retrospectively obtained through medical chart reviews. The severity of pneumonia, based on the extent of pneumonic lesions on chest x-ray (adjusted odds ratio (aOR), 10.573; 95% confidence intervals (CIs), 2.303−48.543), and lactate dehydrogenase (LDH) levels (aOR, 1.002; 95% CIs, 1.000–1.004) at the time of admission were associated with slow response to treatment of MP pneumonia. Pleural effusion (aOR, 5.127; 95% CIs, 1.404–18.727), respiratory virus co-infection (aOR, 4.354; 95% CIs, 1.374–13.800), and higher LDH levels (aOR, 1.005; 95% CIs, 1.002–1.007) as well as MP-specific IgM titer (aOR, 1.309; 95% CIs, 1.095–1.564) were associated with no response or progression of MP pneumonia. The area under the curve for the prediction of no or poor response in MP pneumonia using pleural effusion, respiratory virus co-infection, LDH levels, and MP-specific IgM titer at the time of admission was 0.8547. This study identified the predictive factors of responses to treatment of MP pneumonia in children, which would be helpful in establishing a therapeutic plan and predicting the clinical course of MP pneumonia in children.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1848.2-1849
Author(s):  
M. A. Mortada ◽  
H. Eitta ◽  
R. Elmallah ◽  
A. Radwan ◽  
A. Elsaman

Background:Musculoskeletal Ultrasonography (MSUS) is now a widely used tool for monitoring of rheumatoid arthritis (RA). Although there are many proposed sets of composite scores, a fixed set of joints may not be an ideal tool to assess a disease like RA, which affects many joints and tendons in different presentations. In previous study (1) U9 score was proven to be correlated with disease activity parameters.Objectives:To determine whether US assessment using U9 score is useful for monitoring response to treatment for RA or not?Methods:A prospective, multicenter study were conducted in period from July 2019 to December 2019. All recruited RA patients were subjected to: Disease activity assessment by clinical disease activity indices (CDAI and DAS28 ESR). Functional status assessment by (HAQ) and ultrasonographic assessment using U9 score which include 8 joints (bilateral wrists,2ndMCP,3RDMCP and knees) plus most clinically affected joint or tendon (one joint or one tendon). Most clinically affected joints from 48 joints. Any affected tendons could be choosing. All targeted joints were evaluated according to EULAR guidlines and by EULAR/ OMERACT combined score (0-3). Targeted tendons were scored (0-3).All patients received their treatment (biologic and non biologic DMARDs) according to the decision of the treating physicians. No specific therapy is needed. CDAI and DAS28 ESR, HAQ and U9 score were repeated after 3 months to detect the response to change after receiving the therapy.Results:One hundred and forty patients (23.6% were male) with mean age 39.26±11.30 were recruited from 4 tertiary referral university hospitals.There was a significant difference (<0.001) between the first and second visits as regards clinical, laboratory and ultrasonographic parameters. DAS 28 decreased form (5.29±1.21) to (3.95±0.99), ESR decreased from (42.12±15.24) to (26.84±12.32), HAQ2 improved from (0.652±0.350) to (0.510±0.237) and U9 total US score decreased from (13.56±5.18) to (8.02±4.28).There was significant correlation between U9 ultrasonographic score and clinical parameters at both visits (table 1).Table 1.correlation between U9 ultrasonographic score and clinical parameters.U9 at 1stvisitU9 at 2ndvisitDAS-28Pearson Correlation(P value)0.806<0.0010.790<0.001CDAIPearson Correlation(P value)0.787<0.0010.773<0.001HAQPearson Correlation(P value)0.431<0.0010.317<0.001We found that the most suitable cut-off value of U9 score to predict high disease activity was 11.5 (sensitivity 85.7% and specificity 80.6%), cut off value for moderate disease activity was 5.5(sensitivity 83.2% and specificity 88%) and cut off value for low disease activity was 3.5 (sensitivity of 83.3% and specificity 57.1%). These results are summarized in the following table:Conclusion:U9 ultrasonographic score is very useful method for evaluating the monitoring the response of treatment.References:[1]Mortada, et al. Annals of the Rheumatic Diseases 2019;78:1009.Disclosure of Interests:None declared


2020 ◽  
Vol 36 (1) ◽  
Author(s):  
Victoria Blackabey ◽  
Olivia Kenyon ◽  
Rishi Talwar

Abstract Background Sinonasal melanoma is a rare head and neck tumour. It is associated with a poor prognosis, high rates of loco-regional recurrence and distant metastasis. Treatment of the disease is therefore complicated, and because of limited data regarding the cancer, management is frequently tailored to the individual patient. We describe an unusual presentation of sinonasal melanoma with relevant histology, radiology and clinical photography. Case presentation The case report describes the presentation of a 64-year-old man to the Ear, Nose and Throat department with progressive right-sided hearing loss. A thorough history highlighted other clinical symptoms including unilateral nasal obstruction and epistaxis. Clinical examination showed a right middle ear effusion with a polypoidal lesion in the right nasal cavity. Relevant imaging demonstrated a destructive process that required further assessment. An endoscopic sinus procedure was performed to obtain histological diagnosis as well as providing symptomatic relief. Histology confirmed malignant mucosal melanoma. The patient underwent maxillectomy and orbital exenteration (due to further progression of disease) at a tertiary centre with a plan for subsequent immunotherapy. This however has been delayed due to further surgery to excise a metastatic lesion to the right femur. Conclusions This case report highlights the importance of a thorough clinical history and examination. An unusual presentation of a sinonasal tumour can easily be missed leading to a significant delay in treatment. The case report also describes the use of functional endoscopic sinus surgery in order to obtain histological diagnosis and to debulk the tumour, providing symptomatic relief. The current literature regarding management will be discussed as well as current developments guiding future treatment.


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