Diagnostic of immune-mediated neuropathies

2011 ◽  
Vol 152 (39) ◽  
pp. 1560-1568
Author(s):  
Péter Diószeghy

Separate discussion of immune-mediated neuropathies from other neuropathies is justified by the serious consequences of the natural course of these diseases, like disability and sometimes even life threatening conditions. On the other hand nowadays effective treatments already exist, and with timely and correct diagnosis an appropriately chosen treatment may result in significant improvement of quality of life, occasionally even complete recovery. These are rare diseases, and the increasing number of different variants makes it more difficult to recognize them. Their diagnosis is based on the precise knowledge of clinical signs and symptoms, and it is verified by the help of neurophysiologic and laboratory, first of all CSF examinations. Description of clinical features of the classic acute immune-mediated neuropathy, characterized by ascending paresis and demyelination is followed by a summary of characteristics of newly recognized axonal, regional and functional variants. Chronic immune-mediated demyelinating polyneuropathies are not diagnosed in due number even today. This paper does not only present the classic form but it also introduces the ever increasing special variants, like distal acquired demyelinating sensory neuropathy, Lewis-Sumner syndrome, multifocal motor neuropathy and paraproteinemic neuropathies. Vasculitic neuropathies can be divided into two groups: systemic and non-systemic ones. The first sign of a vasculitic neuropathy is a progressive, painful mononeuropathy; the classic clinical presentation is the mononeuritis multiplex. It is characterized by general signs like fever, loss of weight, fatigue. In systemic vasculitis organ specific symptoms are also present. From the paraneoplastic diseases the subacute sensory neuropathy and the sensory neuronopathy are members of the immune-mediated neuropathies, being most frequently associated with small cell lung cancer. Orv. Hetil., 2011, 152, 1560–1568.

2012 ◽  
Vol 7 (2) ◽  
pp. 124 ◽  
Author(s):  
Jean-Marc Léger ◽  
Eugen Gavriliuc ◽  
◽  

Multifocal motor neuropathy (MMN) is a rare, clinically well-defined condition within the spectrum of chronic, immune-mediated neuropathies. A typical patient history involves slowly or stepwise progressive, predominantly distal, asymmetrical limb weakness and muscle wasting, most frequently in the arm, that may have developed over a period of years. As a rare condition, MMN may present a diagnostic challenge for non-specialists and some patients may wait years for a correct diagnosis. Timely and accurate diagnosis is essential for patients with MMN. Unlike some motor neuropathies, MMN is treatable with intravenous immunoglobulin and untreated patients are likely to experience progressive muscle weakness that may result in serious functional impairment and impaired quality of life. The aim of this article is therefore to provide a guide for non-specialist neurologists to the clinical recognition and differential diagnosis of MMN.


Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1377
Author(s):  
Joanna Drat-Gzubicka ◽  
Anna Pyszora ◽  
Jacek Budzyński ◽  
David Currow ◽  
Małgorzata Krajnik

Neuropathic pain (NP) affects approximately 30% of patients with advanced cancer. The prevalence of neuropathic pain related to peripheral neuropathy (NP-RPN) in these patients is not known. The aim of the study was to evaluate NP-RPN prevalence in hospice patients and to find out whether the absence of this pain is sufficient to rule out peripheral neuropathy. The study included a total of 76 patients with advanced cancer who were cared for at inpatient hospices. All patients were asked about shooting or burning pain (of the feet and hands), were examined systematically for sensory deficits and had a nerve conduction study performed. NP-RPN was found in 29% of the patients. Electrophysiologically-diagnosed peripheral neuropathy was found in 79% of patients, and the diagnostic electrophysiological criteria for neuropathy were met by one half of the patients without NP-RPN. The severity of NP-RPN was correlated with the clinically assessed severity of sensory neuropathy and the Karnofsky score, but was not correlated with the intensity of the clinical signs of motor neuropathy. The presence of NP-RPN did not reflect greater prevalence of motor and sensory abnormalities in neurological and electrophysiological examinations. The absence of NP-RPN did not rule out polyneuropathy in hospice patients.


Author(s):  
Prassede Bravi ◽  
Patricia Tolaini

IntroductionGiant cell arteritis (GCA) is the most common systemic vasculitis in older individuals, characterized by granulomatosus inflammation of the wall of large and medium-sized arteries. The wide spectrum of arterial sites involved leads to ischemia of different organs resulting in a wide range of clinical signs and symptoms. Temporal artery is commonly involved (temporal arteritis). Unusual patterns of presentation, such as extraocular motility disorders and orbital swelling, may be early and transient manifestations of GCA and precede the permanent visual loss due to ischemic optic neuropathy.Case reportWe describe a patient with uncommon manifestations of GCA consisting of transient recurrent diplopia, ptosis, orbital swelling together with more typical clinical features of the disease such as musculoskeletal manifestations (polymyalgia rheumatica) and facial pain: all signs and symptoms promptly resolved under corticosteroid therapy without relapse.Conclusions A high level of suspicion of GCA in individuals over the age of 50 years is needed to prevent the development of severe complications. Clinicians should be aware of uncommon manifestations of the disease such as head–neck swelling and ophthalmoplegia: management guidelines have stated that prompt administration of adequate dose of corticosteroids as soon as ocular manifestations of GCA are noted may almost totally prevent blindness.


Hematology ◽  
2012 ◽  
Vol 2012 (1) ◽  
pp. 485-491 ◽  
Author(s):  
Peter Valent

Abstract Traditionally, cytopenias are classified as deficiency mediated, immune mediated, BM failure induced, renal, or idiopathic, with the latter including the so-called idiopathic cytopenias of undetermined significance (ICUS). Clinical findings, symptoms, blood counts, BM findings, and other laboratory parameters are usually sufficient to reveal the type and cause of a marked cytopenia. However, in patients with chronic mild cytopenia, it may be a challenge for the physician to establish a correct diagnosis. In such patients, laboratory features and findings often reflect a diagnostic interface, so that criteria that are otherwise robust may hardly be applicable or are not helpful. Even if the BM is examined, the diagnosis often remains uncertain in these patients. In addition, more than one potential cause of cytopenia may be present, especially in the elderly. A myelodysplastic syndrome (MDS) or another BM disorder, but also an overt autoimmune or other inflammatory disease, may develop during follow-up in these patients. A key problem is that in an early phase of MDS, most laboratory and clinical signs are “nonspecific.” One of the very few reliable peripheral blood parameters distinguishing between an early or “pre-phase” of MDS and most other causes of a mild cytopenia are the numbers of circulating colony-forming progenitor cells. In addition, flow cytometric and molecular investigations may sometimes assist in the delineation between clonal and reactive conditions underlying mild cytopenias. This review provides an overview of diagnostic approaches and algorithms for patients with mild unexplained cytopenia(s).


2019 ◽  
Vol 6 (4) ◽  
pp. 19
Author(s):  
Ashley Thomas ◽  
Nithya Krishnan ◽  
Nicole Vesely ◽  
Myint Thway ◽  
Rafik Jacob

Granulomatosis with polyangiitis (GPA) is an ANCA-associated, systemic vasculitis of small and medium-sized blood vessels. GPA causes inflammation and destruction to the vessel wall and eventual tissue and organ damage. It classically affects the tissues and vasculature of the sinuses, lungs, and kidneys. The organ damage results in epistaxis, cough, hemoptysis, shortness of breath and/or even kidney failure. Cutaneous manifestations are rare, but have been reported. We discuss the case of a 27-year-old African American female who presented with skin lesions on her bilateral forearms and hands, several years after diagnosis and treatment for GPA. The lesions were isolated papules, located on bilateral elbows, palms, and the lateral edge of the 3rd digit on her left hand. The lesions were intensely pruritic and non-painful. A skin biopsy of the lesions showed neutrophilic and granulomatous inflammation with nuclear debris, a largely non-specific histopathologic finding. An extensive autoimmune work up revealed elevated c-ANCA/PR3-ANCA levels supporting a diagnosis of cutaneous GPA. GPA is diagnosed by a combination of clinical signs and symptoms, serologic testing, and histology from biopsy of affected organs. Patient’s who meet the criteria should be tested for anti-neutrophil cytoplasmic antibody (ANCA), specifically c-ANCA/PR3-ANCA. A positive ANCA is supportive for GPA diagnosis. However, a negative ANCA does not rule out disease. Patients with cutaneous findings suggestive of GPA and positive c-ANCA/PR3-ANCA serologic testing should be closely followed up, which will lead to overall better prognosis, improved health outcomes and reduced patient and health care expenses.


Author(s):  
Abdulrahman Kamal Habash ◽  
Firas Walid Basoudan ◽  
Hassan Adnan Alwayel ◽  
Bassam Walid Alarfaj ◽  
Ali Naji Alwayel ◽  
...  

Oral manifestations of oropharyngeal infections in immunocompromised individuals pose a unique challenge for medical and dental specialists, as clinical signs and symptoms could be minor, making correct diagnosis and treatment difficult. Effective infection control and oral symptom management are critical, and they can be done by the careful use of topical and systemic medicines, as well as proper oral hygiene. According to current guidelines, children should receive an oral examination before starting cancer treatment.  Before the commencement of cancer treatment–related oral issues, a pretreatment examination helps develop a dentist-child relationship. Intensive mouth care is critical because it reduces the chance of developing moderate/severe mucositis while also reducing the risk of septicemia and oral infections. Almost all of the therapy's oral side effects are caused by stomatotoxicity, either direct or indirect. The purpose of therapy for this patient group is to keep the oral mucosa healthy, avoid secondary infection, relieve pain, and maintain nutritional intake. In this narrative review we will be looking at evaluation, prevention and management of dental complications in immunosuppressed children.


2017 ◽  
Vol 158 (48) ◽  
pp. 1903-1907
Author(s):  
Eszter Szabó ◽  
Lídia Balogh ◽  
Attila Szabó ◽  
Ildikó Szatmári

Abstract: Inherited errors of metabolism are rare genetic disorders characterized by diverse clinical and biochemical phenotypes. The complexity of signs and symptoms often presents a challenge for both clinicians and laboratory specialists. In many cases, prevention of permanent neurological symptoms or death in patients presenting these disorders is dependent on early diagnosis and introduction of appropriate therapy. For professionals it is indispensable to be familiar with the major clinical signs of inborn errors of metabolism and with the necessary and available laboratory studies to achieve an early diagnosis. The review tries to give a way of approach, diagnostic algorithm of laboratory measurements for the correct diagnosis in inherited errors of metabolism. The combination of biochemical and clinical signs, results of special metabolic investigations represent a portentous challenge in general practice. For the correct diagnosis of an inherited error of metabolism, the teamwork between clinicians and laboratory specialists is indispensable. Orv Hetil. 2017; 158(48): 1903–1907.


2016 ◽  
Vol 12 (3) ◽  
Author(s):  
Ann Tammelin

Swedish nursing homes are obliged to have a management system for systematic quality work including self-monitoring of which surveillance of infections is one part. The Department of Infection Control in Stockholm County Council has provided a simple system for infection surveillance to the nursing homes in Stockholm County since 2002. A form is filled in by registered nurses in the nursing homes at each episode of infection among the residents. A bacterial infection is defined by antibiotic prescribing and a viral infection by clinical signs and symptoms. Yearly reports of numbers of infections in each nursing home and calculated normalized figures for incidence, i.e. infections per 100 residents per year, as well as proportion of residents with urinary catheter are delivered to the medically responsible nurses in each municipality by the Department of Infection Control. Number of included residents has varied from 4,531 in 2005 to 8,157 in 2014 with a peak of 10,051 in 2009. The yearly incidences during 2005 - 2014 (cases per 100 residents) were: Urinary tract infection (UTI) 7.9-16.0, Pneumonia 3.7-5.3, Infection of chronic ulcer 3.4–6.8, Other infection in skin or soft tissue 1.4–2.9, Clostridium difficile-infection 0.2–0.7, Influenza 0–0.4 and Viral gastroenteritis 1.2–3.7. About 1 % of the residents have a suprapubic urinary catheter, 6–7 % have an indwelling urinary catheter. Knowledge about the incidence of UTI has contributed to the decrease of this infection both in residents with and without urinary catheter.


Healthcare ◽  
2021 ◽  
Vol 9 (5) ◽  
pp. 498
Author(s):  
Mark Reinwald ◽  
Peter Markus Deckert ◽  
Oliver Ritter ◽  
Henrike Andresen ◽  
Andreas G. Schreyer ◽  
...  

(1) Background: Healthcare workers (HCWs) are prone to intensified exposure to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection in the ongoing pandemic. We prospectively analyzed the prevalence of antibodies against SARS-CoV-2 in HCWs at baseline and follow up with regard to clinical signs and symptoms in two university hospitals in Brandenburg, Germany. (2) Methods: Screening for anti-SARS-CoV-2 IgA and IgG antibodies was offered to HCWs at baseline and follow up two months thereafter in two hospitals of Brandenburg Medical School during the first wave of the COVID-19 pandemic in Germany in an ongoing observational cohort study. Medical history and signs and symptoms were recorded by questionnaires and analyzed. (3) Results: Baseline seroprevalence of anti-SARS-CoV-2 IgA was 11.7% and increased to 15% at follow up, whereas IgG seropositivity was 2.1% at baseline and 2.2% at follow up. The rate of asymptomatic seropositive cases was 39.5%. Symptoms were not associated with general seropositivity for anti-SARS-CoV-2; however, class switch from IgA to IgG was associated with increased symptom burden. (4) Conclusions: The seroprevalence of antibodies against SARS-CoV-2 was low in HCWs but higher compared to population data and increased over time. Screening for antibodies detected a significant proportion of seropositive participants cases without symptoms.


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