Immune-mediated ophthalmoparesis with anti-GD1a antibodies

2021 ◽  
Vol 14 (11) ◽  
pp. e244273
Author(s):  
Norma McKean ◽  
Charmaine Chircop
Keyword(s):  
Neurological Examination ◽  
Intravenous Immunoglobulins ◽  
Immune Mediated ◽  
Post Infusion ◽  
History Of ◽  
Disease Modifying ◽  
Ct Brain ◽  
Disease Modifying Treatment ◽  
Mr Brain

A young woman presented to neurology with a 1 month history of progressive diplopia on lateral gaze and a 1 week history of headaches. On examination she was found to have complex ophthalmoparesis with binocular horizontal diplopia, failure of abduction bilaterally and limited upgaze with convergence-retraction nystagmus. The rest of the neurological examination was normal. She was admitted for investigations: blood, CT brain, MR brain and lumbar puncture results were normal. Anti-GD1a antibodies were strongly positive; anti-GM1, anti-GM2 and anti-GD1b were also positive. On follow-up 3 weeks later, the complex ophthalmoplegia persisted. It was decided to treat with intravenous immunoglobulins (IVIgs) with good response but recurrence at 2 weeks post infusion. She was treated with 4 weekly IVIg courses and remains responsive and controlled over 1 year since presentation but becomes symptomatic in the week running up to each dose; thus, disease modifying treatment is currently being considered.

THUR 103 Chorea associated with atrial myxoma

2018 ◽  
Vol 89 (10) ◽  
pp. A11.1-A11
Author(s):  
Healy Sarah ◽  
Ellis Richard James Booth ◽  
Steiger Malcolm
Keyword(s):  
Primary Tumour ◽  
Behavioural Change ◽  
Intravenous Immunoglobulins ◽  
Blood Film ◽  
Atrial Myxoma ◽  
Csf Analysis ◽  
Immune Mediated ◽  
Oral Corticosteroids ◽  
Mobile Mass ◽  
History Of

BackgroundChorea is a hyperkinetic movement disorder characterised by involuntary, unpredictable muscle contractions and is associated with a broad range of primary and secondary aetiologies.CaseA 73-year-old gentleman presented with a 12 month history of progressive behavioural change and involuntary movements. His family had become concerned about periods of erratic behaviour and confusion. He appeared agitated and restless. On examination he had choreiform movements predominantly affecting his upper limbs. An abnormal diastolic heart sound was detected on auscultation.MRI brain and CSF analysis were normal; ASO titre, paraneoplastic, anti-NMDAr antibodies and Huntington’s genetics were negative. A blood film was normal. Transoesophageal echocardiogram demonstrated a variegated mobile mass within the left atrium.An immune-mediated mechanism was suspected. Intravenous immunoglobulins and oral corticosteroids were trialled with initial major benefit but he relapsed several months later and did not respond to a further trial. The atrial mass was resected and histological analysis confirmed an atrial myxoma. The chorea and cognitive symptoms significantly improved after surgery and he remains well.DiscussionAtrial myxoma is the most common primary tumour of the heart and is rare with an incidence of 0.5/1,000,000 population/year. There has only been one other case in the paediatric literature.

scholarly journals Not Just Another Cause of Dyspnea: Common Complaint Leads to a Rare Diagnosis

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Hemant Goyal ◽  
Shitij Arora ◽  
Frantz Duffoo ◽  
Utpal Bhat ◽  
Rekha Bhandari ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
Ivig Therapy ◽  
Intravenous Immunoglobulins ◽  
Rapid Improvement ◽  
Immune Mediated ◽  
History Of ◽  
Rare Diagnosis

A 62-year-old man with past medical history of type 2 diabetes mellitus (DM-2) and hypertension presented with progressive shortness of breath since three months. He was diagnosed with diabetic polyradiculopathy with diaphragmatic involvement and was started on intravenous immunoglobulin (IVIg) therapy. Rapid improvement was seen as evidenced by increased vital capacity and other pulmonary function parameters. Considering the patient’s positive response to intravenous immunoglobulins (IVIg), this case strengthens the fact that diaphragmatic involvement in Type 2 Diabetes Mellitus can be a part of focal or polyneuropathy and that the pathogenesis is immune mediated.

scholarly journals Guillain-Barre Syndrome following COVID-19 Infection: First Case Report from Kuwait and Review of the Literature

2021 ◽  
pp. 1-5
Author(s):  
Walaa A. Kamel ◽  
Ismail Ibrahim Ismail ◽  
Jasem Yousef Al-Hashel
Keyword(s):  
Intravenous Immunoglobulins ◽  
Lower Limbs ◽  
Guillain Barre Syndrome ◽  
Global Pandemic ◽  
First Case ◽  
Immune Mediated ◽  
Guillain Barré Syndrome ◽  
Electrophysiological Studies ◽  
History Of ◽  
Guillain Barré

Objective: Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy that is often related to a previous infectious exposure. GBS emerged as a potentially serious complication of coronavirus disease 2019 (COVID-19) since its declaration as a global pandemic. We report the first case from Kuwait, to the best of our knowledge. Clinical Presentation: A 72-year-old male presented with 3 weeks history of acute progressive and ascending lower limbs weakness. He developed these symptoms 3 weeks after testing positive to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Electrophysiological studies showed acute demyelinating polyradiculoneuropathy and cerebrospinal fluid showed protein-cell dissociation. He was successfully treated with intravenous immunoglobulins (IVIGs). Conclusion: Neurologists should be aware of GBS as a potentially serious complication associated with CO­VID-19. Our patient had a favorable outcome with IVIG with no autonomic or respiratory affection.

scholarly journals Learning disabilities: analysis of 69 children

2001 ◽  
Vol 59 (2B) ◽  
pp. 338-341 ◽  
Author(s):  
Eduardo Kaehler Meister ◽  
Isac Bruck ◽  
Sérgio Antônio Antoniuk ◽  
Ana Chrystina de Souza Crippa ◽  
Sandra Regina Baggio Muzzolon ◽  
...  
Keyword(s):  
Learning Disabilities ◽  
Elementary School ◽  
Public School ◽  
Neurological Examination ◽  
Attention Deficit ◽  
Multidisciplinary Team ◽  
Second Grade ◽  
Hyperactivity Disorder ◽  
History Of

With this article we intend to demonstrate the importance of evaluation and follow up of children with learning disabilities, through a multidisciplinary team. As well as to establish the need of intervention. We evaluate 69 children, from Aline Picheth Public School, in Curitiba, attending first or second grade of elementary school, through general and evolutionary neurological examination, pediatric checklist symptoms, and social, linguistic and psychological (WISC-III, Bender Infantile and WPPSI-figures) evaluation. The incidence was higher in boys (84,1%), familiar history of learning disabilities was found in 42%, and writing abnormalities in 56,5%. The most frequent diagnosis was attention deficit and hyperactivity disorder, in 39,1%. With this program, we aimed to reduce the retention taxes and stress the importance of this evaluation, and, if necessary, multidisciplinar intervention in the cases of learning disabilities.

Progressive Myelopathy Due to a Spontaneous Intramedullary Hematoma in a Dog: Pre- and Postoperative Clinical and Magnetic Resonance Imaging Follow-up

2008 ◽  
Vol 44 (5) ◽  
pp. 266-275 ◽  
Author(s):  
Jean-Laurent Thibaud ◽  
Antoine Hidalgo ◽  
Ghita Benchekroun ◽  
Laurent Fanchon ◽  
Francois Crespeau ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Neurological Examination ◽  
Clinical Signs ◽  
Resonance Imaging ◽  
Surgical Scar ◽  
Progressive Myelopathy ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

A 4-year-old, male Jack Russell terrier was presented for a 6-month history of progressive right hemiparesis with episodic cervical hyperesthesia. The neurological examination showed a right-sided, upper motoneuron syndrome and partial Horner’s syndrome. Two magnetic resonance imaging (MRI) examinations were performed 3 months apart and revealed a persistent cervical intramedullary hematoma. A dorsal myelotomy was performed. A subacute hematoma was confirmed histologically without underlying lesions. Eighteen months later, the dog’s clinical signs were minimal. Two MRI examinations were performed 2 weeks and 5 months after surgery and revealed regressing signal abnormalities at the surgical site, consistent with a surgical scar.

scholarly journals OP0165 RISK FOR UVEITIS EVENTS AFTER WITHDRAWAL OF DISEASE MODIFYING ANTIRHEUMATIC DRUGS IN THE TREATMENT OF PATIENTS WITH EXTENDED OLIGOARTHRITIS OR RHEUMATOID FACTOR NEGATIVE POLYARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 99-100
Author(s):  
J. Klotsche ◽  
A. Klein ◽  
M. Niewerth ◽  
T. Kallinich ◽  
D. Windschall ◽  
...  
Keyword(s):  
Rheumatoid Factor ◽  
Disease Modifying Antirheumatic Drugs ◽  
Antirheumatic Drugs ◽  
Children And Adolescent ◽  
Disease Characteristics ◽  
Treatment Data ◽  
History Of ◽  
Disease Modifying ◽  
Long Term Observation

Background:Juvenile idiopathic arthritis (JIA) associated uveitis is an extra-articular manifestation of the JIA disease that may cause vision-threatening complications and an uncontrolled uveitis may even lead to blindness. Uveitis occurs in up to 20% of patients with JIA, depending on the JIA category. The majority of patients develop uveitis within the first two years after JIA symptom onset, but uveitis can continue into adulthood.Objectives:The main objective of this study was to analyze the risk for uveitis events after discontinuing disease-modifying antirheumatic drugs (DMARD) in patients with extended oligoarthritis and rheumatoid factor (RF)-negative polyarthritis.Methods:Data of the two ongoing multicenter biologic registers: German Biologics in Pediatric Rheumatology (BiKeR) and the Juvenile arthritis Methotrexate/Biologics long-term Observation (JuMBO) were used to analyze the adverse-event (AE) and events of special interest (ESI) reports about uveitis events during treatment and after discontinuation of DMARDs. Biker started recruitment of children and adolescent patients with JIA exposed to biological (b) or conventional (cs) DMARD’s in 2001. The patients were further followed in JuMBO after reaching the age of 18 or transitioning to an adult rheumatologist. Disease characteristics, treatment data, AE’s and ESI’s were reported by the pediatric or adults rheumatologist, respectively.Results:A total of 2,041 patients with RF-negative polyarthritis (n=1,280) or extended oligoarthritis (n=761) were included into the analyses. The mean follow-up of this study was 7.6 years (SD 5.3). About half of the patients were enrolled in BiKeR with start of etanercept (1,137, 55.7%), followed by 635 (31.1%) patients with start of methotrexate (MTX) monotherapy or adalimumab (ADA, n=198, 9.7%). A history of uveitis was reported for 238 (11.7%) patients at enrolment in BiKeR. More patients with a history of uveitis treated with ADA were included in BiKeR initiating ADA (n=98 of 238, 41.2%). Patients with uveitis had a lower age at JIA onset in comparison to patients without uveitis (mean 3.6 (SD 3.0) versus 7.0 (SD 4.5) years). A total of 142 recurrent (84% of 169) uveitis events were reported in 93 patients and for 27 patients (1.3% of 2,041) was an incident uveitis reported during follow-up. More than one uveitis event was reported for 32 patients with a maximum number of 4 uveitis flares in 3 patients. Nineteen uveitis flares (11.2% of 169) were reported for patients after the age of 18. The longer the time since DMARD discontinuation the fewer uveitis events occurred. Uveitis events were significantly more often reported in the first 24 months after MTX discontinuation (<6 months: OR=3.19, 95%CI: 1.70 to 5.96; 6 to <12 months: OR=2.06, 95%CI: 1.01 to 4.66; 12 to <24 months: OR=2.20, 95%CI: 1.14 to 4.25) and in the first three months after biological DMARD discontinuation (OR=5.4, 95%CI: 1.56 to 18.33). Patients with a MTX dose of ≤ 10 mg/m2 at last MTX intake had a higher likelihood for uveitis events (OR=1.40, 95%CI: 1.02 to 1.92).Conclusion:This is the first study that analyzed the risk of uveitis after DMARD withdrawal. Uveitis relapses are common. Patients who discontinued DMARD therapy were at high risk for uveitis within the first 3 to 24 months after discontinuation. Rheumatologists and ophthalmologists should be aware about this risk which should lead to a regular uveitis screening after DMARD withdrawal.Disclosure of Interests:Jens Klotsche: None declared, Ariane Klein: None declared, Martina Niewerth: None declared, Tilmann Kallinich: None declared, Daniel Windschall: None declared, Johannes-Peter Haas: None declared, Frank Weller-Heinemann Speakers bureau: Pfizer, Abbvie, SOBI, Roche, Novartis, Toni Hospach: None declared, Frank Dressler: None declared, Kirsten Minden: None declared, Gerd Horneff: None declared

scholarly journals Feline plasm cell pododermatitis: case report

2020 ◽  
Vol 14 (4) ◽  
pp. 220-225
Author(s):  
Adriane Strack ◽  
Luana Moretto ◽  
Carla Di Concilio ◽  
Francielli Cordeiro Zimermann ◽  
Adriano Tony Ramos ◽  
...  
Keyword(s):  
Case Report ◽  
Physical Examination ◽  
Present Report ◽  
Clinical History ◽  
Histopathological Diagnosis ◽  
Veterinary Clinic ◽  
Immune Mediated ◽  
Mixed Breed ◽  
History Of

Feline Plasmacytic Pododermatitis is an uncommon dermatological disorder that affects one or multiple paw pads and leads to swelling, ulceration, hemorrhage, scaling, erythema, and striation. Its etiopathogenesis is poorly understood, however, an immune-mediated basis is strongly suggested. A two-year-old neutered, mixed breed, male cat was referred to the Veterinary Clinic of the Federal University of Santa Catarina (UFSC), Curitibanos Campus, with a brief clinical history of progressive lesions on palmar and plantar pads for one month. On physical examination, the patient had enlarged popliteal and inguinal lymph nodes, and paw pads with thin, swollen, hyperemic, erythematous, and purple color skin with multiple white striations. Histopathological diagnosis confirmed plasmacytic pododermatitis, and glucocorticoid therapy with prednisolone was prescribed. In the present report, the patient's clinical follow-up was compromised, as the tutor did not perform the prescribed treatment and did not return with the animal for further evaluations.

Letter To The Editor

PEDIATRICS ◽  
1979 ◽  
Vol 63 (2) ◽  
pp. 347-347
Author(s):  
Alan B. Forsythe ◽  
Sheldon M. Wolf
Keyword(s):  
Brain Damage ◽  
Neurological Examination ◽  
Febrile Seizure ◽  
Febrile Convulsion ◽  
Recurrence Rates ◽  
Letter To The Editor ◽  
History Of ◽  
Abnormal Results

In a follow-up article,1 we divided the children into two groups for the purpose of analysis. In the first group were children with a single, brief, generalized initial febrile seizure and no history of prenatal, perinatal, or postnatal abnormalities that might suggest the possibility of brain damage. In the second group were those children with such birth abnormalities, and/or a severe initial febrile convulsion, and/or abnormal results of neurological examination. In the first group, the 36-month recurrence rates were 5% for those randomly assigned to continuous phenobarbital therapy and 34% for those receiving intermittent or no phenobarbital therapy (P &gt; .001).

scholarly journals Tocilizumab Treatment of Patients with Systemic Sclerosis: Clinical Data

2017 ◽  
Vol 2 (2_suppl) ◽  
pp. S29-S35 ◽  
Author(s):  
Dinesh Khanna ◽  
Angelika Jahreis ◽  
Daniel E. Furst
Keyword(s):  
Systemic Sclerosis ◽  
Treatment Options ◽  
Current Treatment ◽  
Phase 2 Trial ◽  
Immune Mediated ◽  
Interleukin 6 Receptor ◽  
Organ Specific ◽  
Pharmacologic Management ◽  
Disease Modifying ◽  
Disease Modifying Treatment

Systemic sclerosis (SSc) is an immune-mediated disease characterized by progressive, and often severe, inflammation and fibrosis of the skin and internal organs, such as the lungs, heart, kidneys, and gastrointestinal tract. There is an unmet medical need to treat patients with SSc given the high SSc-related mortality rate. Furthermore, disease-modifying therapies are lacking, and current treatment options focus on the management of individual organ-specific complications associated with the disease. Immunosuppressive therapies are the mainstay of pharmacologic management of major SSc-associated complications, such as skin and lung manifestations, but their overall effectiveness is limited and toxicity issues are common. Advances in understanding of the pathological processes involved in the immunologic and fibrotic mechanisms of SSc have led to clinical research focusing on targeted immunotherapies, in an effort to develop much needed disease-modifying treatment options. The interleukin-6 receptor-alpha antagonist tocilizumab has demonstrated promising efficacy in a phase 2 trial and is being investigated in a phase 3 trial. This article provides an overview of current pharmacologic treatment options for the management of SSc-related complications and discusses tocilizumab for the treatment of SSc in clinical trials.

scholarly journals Recurrent Hepatocellular Carcinoma in Patient with Crohn’s Disease: Incidental or Expected Outcome of Azathioprine?

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Youssef Botros ◽  
Mary Mathews ◽  
Hiren Patel ◽  
Nihar Shah ◽  
Walid Baddoura ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Case Reports ◽  
Disease Patient ◽  
Aminosalicylic Acid ◽  
Immune Mediated ◽  
Increased Risk ◽  
History Of

Hepatocellular carcinoma (HCC) usually occurs in patients with underlying risk factors such as liver cirrhosis and chronic hepatitis B. Although patients with Crohn’s disease (CD) are at an increased risk to develop malignancies such as colon cancer, the incidence of HCC in this population is extremely rare. We report a case of 62-year-old male with long history of CD treated with azathioprine (AZA) and aminosalicylic acid (ASA) who was incidentally diagnosed with HCC, for which left hepatectomy was done. Four years later during routine follow-up, patient had another hepatic lesion and underwent resection of the mass. The mechanism of occurrence of HCC in patient with CD is still controversial and may include immune mediated changes and medication related complications. AZA was reported in all case reports of CD that developed HCC. Through this report we hope to explore the complex pathophysiological mechanisms contributing to the development of HCC in the Crohn’s disease patient population.

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