Guillain-Barré syndrome: diagnosis and treatment guidelines

GuillainBarr syndrome is an acute, rapidly progressive immune-mediated disease of the peripheral nervous system, combining several variants and subtypes with various clinical, pathophysiological and electrophysiological signs. GuillainBarr syndrome usually develops 13 weeks after the viral or bacterial infection, which acts as the trigger triggering autoimmune mechanisms, leading to demyelination and axonal damage. The disease is getting more acute due to the emergence of a new coronavirus infection. Behind the diseases dipathophysiology there is the activation of cellular and humoral immunity with the production of autoantibodies to specific gangliosides and glycolipids and the formation of circulating immune complexes that attack peripheral nerves and roots (the phenomenon of molecular mimicry). The examination of patients requires an integrated approach, including, along with clinical and anamnestic data, the results of laboratory and neurophysiological examination. The treatment of patients with GuillainBarr syndrome is carried out in an intensive care unit and includes both pathogenetic therapy and nonspecific measures aimed to correct dysfunctions of vital organs, prevent complications and provide symptomatic therapy. Currently, the main direction of pathogenetic therapy of this disease is the use of high-dose intravenous immunotherapy with human normal immunoglobulin preparations or high-volume therapeutic plasmapheresis. Taking into account the absence of data about differences in the effectiveness of these methods, the choice of direction is determined taking into account the contraindications and possible development of adverse events, as well as the capabilities of the medical institution (2 tables, bibliography: 15 refs)

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Bilateral dysthyroid compressive optic neuropathy responsive to teprotumumab

European Journal of Ophthalmology ◽

10.1177/1120672121991042 ◽

2021 ◽

pp. 112067212199104

Author(s):

Catherine J Hwang ◽

Erin E Nichols ◽

Brian H Chon ◽

Julian D Perry

Keyword(s):

Side Effects ◽

Optic Neuropathy ◽

Surgical Decompression ◽

Thyroid Eye Disease ◽

Eye Disease ◽

High Dose ◽

Compressive Optic Neuropathy ◽

Traditional Management ◽

Immune Mediated ◽

Orbital Radiation

Thyroid eye disease is an auto-immune mediated orbitopathy which can cause dysthyroid compressive optic neuropathy. Traditional management of active thyroid eye disease includes temporizing high-dose steroids, orbital radiation and surgical decompression, which each possess significant limitations and/or side effects. Teprotumumab is an IGF-IR inhibitor recently FDA-approved for active thyroid eye disease. The authors report reversal of bilateral dysthyroid compressive optic neuropathy managed medically utilizing teprotumumab.

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The underrepresentation of female participants in studies assessing the impact of high-dose exercise on cardiovascular outcomes: a scoping review

European Journal of Preventive Cardiology ◽

10.1093/eurjpc/zwab061.367 ◽

2021 ◽

Vol 28 (Supplement_1) ◽

Author(s):

S Pallikadavath ◽

R Patel ◽

CL Kemp ◽

M Hafejee ◽

N Peckham ◽

...

Keyword(s):

Scoping Review ◽

High Volume ◽

Cardiovascular Outcomes ◽

High Dose ◽

Funding Sources ◽

Review Protocol ◽

The Mean ◽

The Impact ◽

Over Time ◽

Funding Acknowledgements

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Cardiovascular adaptations as a result of exercise conducted at high-intensity and high-volume are often termed the ‘Athlete’s heart’. Studies have shown that these cardiovascular adaptations vary between sexes. It is important that both sexes are well represented in this literature. However, many studies assessing the impact of high-dose exercise on cardiovascular outcomes under-recruit female participants. Purpose This scoping review aimed to evaluate the representation of females in studies assessing the impact of high-dose exercise on cardiovascular outcomes and demonstrate how this has changed over time. Methods The scoping review protocol as outlined by Arksey and O’Malley was used. OVID and EMBASE databases were searched and studies independently reviewed by two reviewers. Studies must have investigated the effects of high-dose exercise on cardiovascular outcomes. To assess how the recruitment of females has changed over time, two methods were used. One, the median study date was used to categorise studies into two groups. Two, studies were divided into deciles to form ten equal groups over the study period. Mean percentage of female recruitment and percentage of studies that failed to include females were calculated. Results Overall, 250 studies were included. Over half the studies (50.8%, n = 127) did not include female participants. Only 3.2% (n = 8) did not include male participants. Overall, mean percentage recruitment was 18.2%. The mean percentage of recruitment was 14.5% before 2011 and 21.8% after 2011. The most recent decile of studies demonstrated the highest mean percentage of female recruitment (29.3%) and lowest number of studies that did not include female participants (26.9%). Conclusion Female participants are significantly underrepresented in studies assessing cardiovascular outcomes caused by high-dose exercise. The most recent studies show that female recruitment may be improving, however, this still falls significantly short for equal representation. Risk factors, progression and management of cardiovascular diseases vary between sexes, hence, translating findings from male dominated data is not appropriate. Future investigators should aim to establish barriers and strategies to optimise fair recruitment. Mean percentage females recruited per study (%) Percentage studies that do not include women (%) Overall (n = 250) 18.2 50.8 (n = 127) Studies before 2011 (n = 121) 14.5 59.5 (n = 72) Studies after 2011 (n = 129) 21.8 42.6 (n = 55) Table 1: Female recruitment characteristics. The year 2011 (median study year) was chosen as this divides all included studies into two equal groups.

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Nonepileptic Myoclonus in COVID-19: Case Report

The Neurohospitalist ◽

10.1177/19418744211004315 ◽

2021 ◽

pp. 194187442110043

Author(s):

Henly Hewan ◽

Annie Yang ◽

Aparna Vaddiparti ◽

Benison Keung

Keyword(s):

Case Report ◽

Critically Ill ◽

Recovery Phase ◽

Critically Ill Patient ◽

High Dose ◽

The Novel ◽

Neurological Manifestations ◽

Immune Mediated ◽

Novel Coronavirus ◽

Post Infectious

In late 2019, the novel coronavirus, SARS-CoV-2, and the disease it causes, COVID-19, was identified. Since then many different neurological manifestations of COVID-19 have been well reported. Movement abnormalities have been rarely described. We report here a critically ill patient with COVID-19 who developed generalized myoclonus during the recovery phase of the infection. Myoclonus was associated with cyclical fevers and decreased alertness. Movements were refractory to conventional anti-epileptic therapies. There was concern that myoclonus could be part of a post-infectious immune-mediated syndrome. The patient improved fully with a 4-day course of high-dose steroids. Our experience highlights a rare, generalized myoclonus syndrome associated with COVID-19 that may be immune-mediated and is responsive to treatment.

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Immunomodulating functions of recombinant ovine interferon tau: potential for therapy in mulitple sclerosis and autoimmune disorders

Multiple Sclerosis Journal ◽

10.1177/135245859800400204 ◽

1998 ◽

Vol 4 (2) ◽

pp. 63-69 ◽

Cited By ~ 10

Author(s):

O A Khan ◽

H Jiang ◽

P S Subramaniam ◽

H M Johnson ◽

S S Dhib-Jalbut

Keyword(s):

Therapeutic Option ◽

High Dose ◽

Interferon Tau ◽

Unique Type ◽

Immune Mediated ◽

High Concentrations ◽

Ifn Treatment ◽

High Doses ◽

Mulitple Sclerosis

The interferons (IFN) are a family of complex proteins possessing antiviral, antiproliferative, and immunomodulatory activities. Two type 1 recombinant human IFN have been recently approved for the treatment of multiple sclerosis (MS). However, use of high dose type 1 IFN treatment in MS patients has been limited by dose-related toxicity. Ovine IFNt is a unique type 1 interferon discovered for its role in the animal reproductive cycle. It differs from other type 1 IFNs in that it is remarkably less toxic even at high concentrations, is able to cross species barriers, and is not inducible by viral infection. Ovine IFNt has been shown to be very effective in the treatment of animal models of MS. In this study, we examined the toxicity of OvIFNt on human T-cells at high doses and its immunregulatory properties at equivalent doses. Our experiments confirmed the remarkably non-toxic nature of OvIFNt on human cells at high concentrations as well as immunomodulating properties consistent with other type 1 IFNs including an antilymphoproliferative effect and inhibition of IFNg-induced HLA class II expression. These results suggest that OvIFNt could be developed into a potentially less toxic therapeutic option for immune-mediated disorders including MS.

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Levamisole/Cocaine Induced Systemic Vasculitis and Immune Complex Glomerulonephritis

Case Reports in Nephrology ◽

10.1155/2015/372413 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 9

Author(s):

Lohit Garg ◽

Sagar Gupta ◽

Abhishek Swami ◽

Ping Zhang

Keyword(s):

Immune Complex ◽

Systemic Vasculitis ◽

Renal Involvement ◽

Morphological Properties ◽

High Dose ◽

Cocaine Use ◽

Immune Mediated ◽

History Of ◽

Psychotropic Effects ◽

Complex Deposition

Levamisole is an antihelminthic and immunomodulator medication that was banned by the USFDA in 1998. It has been increasingly used to adulterate cocaine due to its psychotropic effects and morphological properties. Adverse reactions including cutaneous vasculitis, thrombocytopenia, and agranulocytosis have been well described. Despite systemic vasculitis in this setting, renal involvement is uncommon. We report here a case of ANCA positive systemic vasculitis with biopsy proven immune complex mediated glomerulonephritis likely secondary to levamisole/cocaine. A 40-year-old Caucasian male with no past medical history presented with 3-week history of fatigue, skin rash, joint pains, painful oral lesions, oliguria, hematuria, worsening dyspnea on exertion, and progressive lower extremity edema. He had a history of regular tobacco and cocaine use. Lab testing revealed severe anemia, marked azotemia, deranged electrolytes, and 4.7 gm proteinuria. Rheumatologic testing revealed hypocomplementemia, borderline ANA, myeloperoxidase antibody, and positive atypical p-ANCA. Infectious and other autoimmune workup was negative. Kidney biopsy was consistent with immune mediated glomerulonephritis and showed mesangial proliferation and immune complex deposition consisting of IgG, IgM, and complement. High dose corticosteroids and discontinuing cocaine use resulted in marked improvement in rash, mucocutaneous lesions, and arthritis. There was no renal recovery and he remained hemodialysis dependent.

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Anecdotal Response to Amphotericin in a Patient with Probable Susac Syndrome: Implications in the Pathogenesis

Acta Médica Portuguesa ◽

10.20344/amp.10541 ◽

2019 ◽

Vol 32 (11) ◽

pp. 727

Author(s):

Raquel Gil-Gouveia ◽

Natália Marto ◽

Pedro Vilela ◽

Ana Catarino

Keyword(s):

Hearing Loss ◽

Fungal Infection ◽

Infectious Agent ◽

Brain Biopsy ◽

High Dose ◽

Radiological Findings ◽

Susac Syndrome ◽

Infection Treatment ◽

Immune Mediated

Susac syndrome is a rare, probably immune-mediated endotheliopathy presenting with encephalopathy, sensorineural hearing loss and retinal arterial occlusions. A 33-year-old female with Susac syndrome was worsening despite high-dose steroids so a brain biopsy was performed which suggested a possible fungal infection. Treatment with amphotericin B resulted in prompt reversal of symptoms and radiological findings, and no further symptoms occurred during 8 years of follow-up. A diagnosis of fungal infection was not confirmed. The etiology of Susac syndrome is unknown and this anecdotal observation suggests that an infectious agent susceptible to amphotericin might have caused or triggered Susac syndrome in this patient.

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Adrenal Crisis in Long Term Use of Exogenous Steroid With Inappropriate Tapering off Process

Journal of the Endocrine Society ◽

10.1210/jendso/bvab048.218 ◽

2021 ◽

Vol 5 (Supplement_1) ◽

pp. A108-A109

Author(s):

Nani Oktavia ◽

Chici Pratiwi ◽

Jerry Nasaruddin ◽

Muhammad Ikhsan Mokoagow ◽

Marina Epriliawati ◽

...

Keyword(s):

Abdominal Pain ◽

Blood Glucose ◽

Adrenal Insufficiency ◽

Clinical Manifestations ◽

Adrenal Crisis ◽

High Dose ◽

Symptomatic Therapy ◽

Bowel Sound ◽

Primary Adrenal Insufficiency

Abstract Background: Adrenal crisis is an emergency condition in endocrinology that commonly found in primary adrenal insufficiency but also occur in chronic adrenal insufficiency triggered by various conditions such as sepsis, infection, trauma, burns, surgery, and myocardial infarction. In chronic adrenal insufficiency, adrenal crisis can be induced by excessive reductions or inadequate discontinuation of steroid treatment. Case Illustration: A 40-year old-man admitted with chief complaint abdominal pain since seven days before admission. He felt pain in the umbilical area and slowly radiated to all the part of abdomen. Other than that, he also felt nausea, had vomitus, fever, and constipation for five days. He was immobilized for four months, with muscles weakness and atrophy. He was diagnosed with Focal Segmented Glomerulosclerosis and had a high dose of methylprednisolone (48 mg) for 6 months, 40 mg for the next 2 months and methylprednisolone 12 mg for the last 2 months. The last two month, he began to have severe general weakness and hypotension. On physical examination we found hypotension, fever (38.1o C), pale conjunctiva, moon face, buffalo hump, slightly distended and tenderness of abdomen, normal bowel sound, and purple striae all over the abdomen and extremities. On laboratory examination, Hb was 8.2 (n 11.7 – 15.5 g/dl), leukocytes 10,400 (5.00 – 10.00 x 103/μL), Na 123 (n 135 – 147 mmol/L), random blood glucose 74 (n 70 – 140 mg/dL). On abdominal X ray, there was prominent faecal material and no signs of ileus. No sign of infection found in urinalysis. He had sodium correction, packed red cell transfusion, symptomatic therapy including laxative, methylprednisolone 12 mg, but no improvement of signs and symptoms beside be able to defecate. The abdominal ultrasound gave a normal result. The morning cortisol level was then examined, with the result 14.4 (n 3.7–19.4). The patient was then diagnosed with adrenal crisis based on the clinical manifestations and had hydrocortisone therapy 100 mg a day for 2 consecutive days. After hydrocortisone administration, the symptoms improved, no fever and abdominal pain, he had normotension, increased sodium level 132 (n 135 – 147 mmol/L) and blood glucose level 118 (n 70 – 140 mg/dL). On the third day the patient discharged with oral hydrocortisone 15 mg in the morning and 10 mg in the afternoon. Conclusion: Adrenal crisis was generally found in primary adrenal insufficiency but could also occur in secondary adrenal insufficiency due to inappropriate tapering off process of long term glucocorticoid use.

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Immunological status in employees of the mining and processing enterprise and chemical risk factors

Hygiene and Sanitation ◽

10.47470/0016-9900-2021-100-5-471-475 ◽

2021 ◽

Vol 100 (5) ◽

pp. 471-475

Author(s):

Inga N. Alikina ◽

Oleg V. Dolgikh

Keyword(s):

Immune System ◽

Working Conditions ◽

Comparison Group ◽

Mineral Resources ◽

Social Consequences ◽

Production Environment ◽

Immune Mediated ◽

Direct Exposure ◽

Early Health ◽

Cellular And Humoral Immunity

Introduction. Currently, occupational health problems acquire particular relevance due to the inconsistency of working conditions with hygienic standards. Unfavorable conditions lead to the development of occupational and production conditioned diseases, increase the risk of developing non-specific diseases and exacerbate their medico-social consequences. In this regard, it becomes essential to develop diagnostic criteria for early health disorders of workers under the influence of working conditions [1]. Materials and methods. One hundred ninety-five workers of potash production (men) were examined. The observation group consisted of 120 workers exposed to harmful chemical occupational factors (air pollution of the working area with silvinite dust, chemicals on the example of hexane). Seventy-five workers were not exposed to harmful factors of the production environment (comparison group). CD-phenotypes of cellular immunity were determined by flow cytometry. The chemical and analytical study of the content of contaminants in urine was carried out by the method of analysis of the equilibrium vapor phase (hexane). The content of IgG specific to the studied substance was determined by the allergosorbent method. Results. Changes in the immune system were significant concerning the comparison group and characterized by excessive expression of many CD-immunophenotypes (CD11a, CD3+CD95+) and the TNFR receptor. At the same time, an increased concentration of hexane was registered in the biological media of workers in the main occupational groups compared to the comparison group. Reliable differences in the content of specific antibodies to hexane (IgG) between the studied groups of workers (p<0.05) were established. Conclusion. The results of this work allowed substantiating the list of priority indicators of the state of the immune system in mining workers exposed to direct exposure to contaminants at the stage of chemical treatment of mineral resources. Abnormalities of cellular and humoral immunity have been identified, which can impair the functioning of the body’s immune system and the formation of immune-mediated pathological conditions (inflammatory and proliferative processes in the lungs, asthenia).

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ACUTE HEMICEREBELLITIS IN ADULTS: LITERATURE REVIEW AND PERSONAL OBSERVATION

The Scientific Notes of the I P Pavlov St Petersburg State Medical University ◽

10.24884/1607-4181-2019-26-1-68-73 ◽

2019 ◽

Vol 26 (1) ◽

pp. 68-73

Author(s):

Alina V. Kupaeva ◽

Evgenia A. Skornyakova ◽

Leonid G. Zaslavskii

Keyword(s):

Crohn’S Disease ◽

Crohn's Disease ◽

Clinical Status ◽

Epileptic Seizures ◽

High Dose ◽

Basic Therapy ◽

Acute Cerebellitis ◽

Immune Mediated ◽

Bowel Diseases ◽

History Of

Acute cerebellitis is a common inflammatory syndrome typically occurring in children and only on very rare occasions in adults. Thus, there was no description of acute cerebellitis, associated with Crohn’s disease, found in Russian sources of literature. We reported a case of acute cerebellitis in 45-year-old woman with a long history of Crohn’s disease treated with Mesalazine as a basic therapy and a resection of ileotransversoanastomosis together with small intestine two months before the onset of neurological symptoms. She first presented with severe headache and left-sided cerebellar dysfunction. The MRI showed signs of pronounced edema of the left hemisphere of the cerebellum, pial Gd-enhancement along the cerebellar folia, difficulty of CSF- flow. The application of lumbar puncture was impeded by the possibility of complications, such as brain herniation. Treatment with high-dose intravenous Methylprednisolone resulted in clinical and radiological improvement. Over the following year, her clinical status remained unchanged, except for one case of epileptic seizures as a result of switching of basic therapy of Crohn’s disease on Adalimumab. In this case, hemicerebellitis most likely has an immune-mediated nature. The temporal relationship with increased activity of Crohn’s disease and a significant positive response to corticosteroid therapy favored this hypothesis. It was known that inflammatory bowel diseases (IBD) had many extra-intestinal manifestations including neurological ones. With regard to that, the alterations in gut microbiota could be a possible common base for systemic inflammatory and autoimmune diseases, such as Crohn’s disease or autoimmune cerebellitis, described in this study.

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