P94 Case series of three patients with segmental nerve enlargement in immune-mediated and hereditary polyneuropathies

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

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76 FECAL MICROBIOTA TRANSPLANTATION (FMT) FOR THE TREATMENT OF IMMUNE-MEDIATED DIARRHEA AND COLITIS: A CASE SERIES

Gastroenterology ◽

10.1016/s0016-5085(21)00791-5 ◽

2021 ◽

Vol 160 (6) ◽

pp. S-17

Author(s):

Arielle Elkrief ◽

Jonathan U. Peled ◽

Matthew D. Hellmann ◽

Jamie E. Chaft ◽

Juliana Eng ◽

...

Keyword(s):

Fecal Microbiota Transplantation ◽

Case Series ◽

Fecal Microbiota ◽

Immune Mediated

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Covid-19 in Immune-Mediated Inflammatory Diseases — Case Series from New York

New England Journal of Medicine ◽

10.1056/nejmc2009567 ◽

2020 ◽

Vol 383 (1) ◽

pp. 85-88 ◽

Cited By ~ 148

Author(s):

Rebecca Haberman ◽

Jordan Axelrad ◽

Alan Chen ◽

Rochelle Castillo ◽

Di Yan ◽

...

Keyword(s):

New York ◽

Inflammatory Diseases ◽

Case Series ◽

Immune Mediated

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Use of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors in Statin‐Associated Immune‐Mediated Necrotizing Myopathy: A Case Series

Arthritis & Rheumatology ◽

10.1002/art.40919 ◽

2019 ◽

Vol 71 (10) ◽

pp. 1723-1726 ◽

Cited By ~ 8

Author(s):

Eleni Tiniakou ◽

Erika Rivera ◽

Andrew L. Mammen ◽

Lisa Christopher‐Stine

Keyword(s):

Case Series ◽

Proprotein Convertase ◽

Necrotizing Myopathy ◽

Immune Mediated

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Peripheral Nerve Imaging Aids in the Diagnosis of Immune-Mediated Neuropathies—A Case Series

Diagnostics ◽

10.3390/diagnostics10080535 ◽

2020 ◽

Vol 10 (8) ◽

pp. 535

Author(s):

Marc Dörner ◽

Frank Schreiber ◽

Heike Stephanik ◽

Claus Tempelmann ◽

Natalie Winter ◽

...

Keyword(s):

Peripheral Nerve ◽

Treatment Options ◽

Case Series ◽

Multifocal Motor Neuropathy ◽

Motor Neuropathy ◽

Magnetic Resonance Neurography ◽

Immune Mediated ◽

Nerve Imaging ◽

Diagnostic Work ◽

Peripheral Nerve Imaging

Background: Diagnosis of immune-mediated neuropathies and their differentiation from amyotrophic lateral sclerosis (ALS) can be challenging, especially at early disease stages. Accurate diagnosis is, however, important due to the different prognosis and available treatment options. We present one patient with a left-sided dorsal flexor paresis and initial suspicion of ALS and another with multifocal sensory deficits. In both, peripheral nerve imaging was the key for diagnosis. Methods: We performed high-resolution nerve ultrasound (HRUS) and 7T or 3T magnetic resonance neurography (MRN). Results: In both patients, HRUS revealed mild to severe, segmental or inhomogeneous, nerve enlargement at multiple sites, as well as an area increase of isolated fascicles. MRN depicted T2 hyperintense nerves with additional contrast-enhancement. Discussion: Peripheral nerve imaging was compatible with the respective diagnosis of an immune-mediated neuropathy, i.e., multifocal motor neuropathy (MMN) in patient 1 and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) in patient 2. Peripheral nerve imaging, especially HRUS, should play an important role in the diagnostic work-up for immune-mediated neuropathies and their differentiation from ALS.

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Acute Demyelinating Encephalomyelitis (ADEM) in COVID-19 infection: A Case Series

10.1101/2020.07.15.20126730 ◽

2020 ◽

Cited By ~ 2

Author(s):

Micheala McCuddy ◽

Praful Kelkar ◽

Yu Zhao ◽

David Wicklund

Keyword(s):

Early Recognition ◽

Ventilatory Support ◽

Case Series ◽

Corticosteroid Treatment ◽

Neurological Complications ◽

Plasma Therapy ◽

The North ◽

Immune Mediated ◽

Elevated Protein ◽

Acute Demyelinating Encephalomyelitis

AbstractObjectiveTo report three patients infected with COVID-19 with severe respiratory syndrome requiring intubation, who developed acute demyelinating encephalomyelitis (ADEM).MethodPatient data were obtained from medical records from the North Memorial Health Hospital, Robbinsdale, MN, USAResultsThree patients (two men and one woman, aged 38 - 63) presented with fatigue, cough and fever leading to development of acute respiratory distress syndrome secondary to COVID-19 infection requiring intubation and ventilatory support. Two patients were unresponsive, one with strong eye deviation to the left and the third patient had severe diffuse weakness. MRI in all patients showed findings consistent with ADEM. CSF showed elevated protein in all patients with normal cell count and no evidence of infection, including negative COVID-19 PCR. All three of the patients received Convalescent plasma therapy for COVID-19. All patients were treated with intravenous corticosteroids and improved, although two responded minimally. Two patients treated with IVIG showed no further improvement.ConclusionNeurological complications from COVID-19 are being rapidly recognized. Our three cases highlight the occurrence of ADEM as a postinfectious/immune mediated complication of COVID-19 infection, which may be responsive to corticosteroid treatment. Early recognition of this complication and treatment is important to avoid long term complications.

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Segmental nerve enlargement in CMT4J

Muscle & Nerve ◽

10.1002/mus.26873 ◽

2020 ◽

Vol 61 (6) ◽

Cited By ~ 1

Author(s):

Ryan Castoro ◽

James Crisp ◽

James B. Caress ◽

Jun Li ◽

Michael S. Cartwright

Keyword(s):

Segmental Nerve ◽

Nerve Enlargement

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Immune-Mediated Neuropathies following Autologous Stem Cell Transplantation for Multiple Myeloma: Case Series and Review of the Literature

Acta Haematologica ◽

10.1159/000453390 ◽

2017 ◽

Vol 137 (2) ◽

pp. 86-88 ◽

Cited By ~ 2

Author(s):

Ghulam Rehman Mohyuddin ◽

Jacqueline Uy ◽

Honhar Medhavi ◽

Muhammad Salman Faisal ◽

Muzaffar H. Qazilbash

Keyword(s):

Multiple Myeloma ◽

Stem Cell ◽

Stem Cell Transplantation ◽

Cell Transplantation ◽

Rare Complication ◽

Case Series ◽

Common Finding ◽

Temporal Association ◽

Hematopoietic Stem ◽

Immune Mediated

Neuropathy is a common finding in patients with multiple myeloma. Several different factors can cause neuropathy in these patients, such as the underlying disease itself, medications used for treatment, or immune-mediated processes. Immune-mediated neuropathies (IMN) consist of a heterogeneous spectrum of peripheral nerve disorders. Although IMN is associated with several hematological disorders, it remains a very rare complication of hematopoietic stem cell transplantation (HCT). We describe our experiences of 3 patients with multiple myeloma who experienced IMN following autologous HCT (auto-HCT). These 3 patients were felt to have IMN clearly attributable to auto-HCT because of a clear temporal association with auto-HCT and absence of any other obvious causative factor. The variety in their clinical presentations, diagnostic approach, and approaches to management are explained. The pathophysiology of how HCT may predispose to IMN remains poorly understood. Our report helps highlight several potential causes of this phenomenon, such as a paraneoplastic syndrome, immune reconstitution syndrome, or drug toxicity. We emphasize that a comprehensive approach is needed to address this rare entity, and that there should be a low threshold to initiate immune-specific therapy, such as plasmapheresis, if symptoms do not resolve spontaneously.

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Gliptin-Induced Bullous Pemphigoid: Canadian Case Series of 10 Patients

Journal of Cutaneous Medicine and Surgery ◽

10.1177/1203475420972349 ◽

2020 ◽

pp. 120347542097234

Author(s):

Miriam Armanious ◽

Mohn’d AbuHilal

Keyword(s):

Bullous Pemphigoid ◽

Case Series ◽

Rapid Improvement ◽

Drug Induced ◽

Dipeptidyl Peptidase 4 ◽

Early Withdrawal ◽

Immune Mediated ◽

Dipeptidyl Peptidase 4 Inhibitors ◽

Causative Agents

Background Bullous pemphigoid (BP) is a chronic immune-mediated vesiculobullous disorder. Recently, several reports have described dipeptidyl peptidase-4 inhibitors, also known as gliptins, as causative agents for drug-induced BP. Objective To report and describe clinical and histologic characteristics of 10 cases of gliptin-induced BP. Results We identified 10 patients with gliptin-induced BP. Nine were secondary to linagliptin, and 1 case was attributed to sitagliptin. All patients showed significant improvement after withdrawal of gliptin medications and proper medical treatment. There has been no evidence of relapse after 4 months of follow-up. Conclusion This report supports the proposed association between gliptins and BP. Physicians should be aware of this potential adverse effect, as gliptin-induced BP can be reversible once identified and the responsible medication is stopped. Early withdrawal of the offending drug and proper treatment can lead to rapid improvement and reduced morbidity.

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Polyneuropathy in Young Siberian Huskies Caused by Degenerative and Inflammatory Diseases

Veterinary Pathology ◽

10.1177/0300985820934112 ◽

2020 ◽

Vol 57 (5) ◽

pp. 666-674

Author(s):

Hanne Jahns ◽

Karen M. Vernau ◽

Catherine M. Nolan ◽

Emma J. O’Neill ◽

Robert E. Shiel ◽

...

Keyword(s):

Axonal Degeneration ◽

Inflammatory Diseases ◽

Age At Onset ◽

Case Series ◽

Clinicopathological Characteristics ◽

Autonomic Nerve ◽

Laryngeal Paralysis ◽

Immune Mediated ◽

Large Fiber ◽

Inherited Neuropathies

Polyneuropathy is defined as the simultaneous dysfunction of several peripheral nerves. In dogs, a number of breeds are predisposed to a variety of immune-mediated and/or degenerative inherited forms of polyneuropathy, with laryngeal paralysis and/or megaesophagus as important clinical features of many of these conditions. This case series describes degenerative and inflammatory polyneuropathies in 7 young Siberian huskies that were categorized based on clinicopathological characteristics as follows: (1) slowly progressive laryngeal paralysis and megaesophagus caused by primary axonal degeneration with large fiber loss (n = 2); (2) slowly progressive polyneuropathy without megaesophagus or laryngeal paralysis caused by primary axonal degeneration with large fiber loss (n = 2); (3) acute inflammatory demyelinating neuropathy causing sensory, motor and autonomic nerve deficits (n = 2); and (4) ganglioradiculitis (sensory neuronopathy; n = 1). Based on the predominantly young age at onset, slow progression, relatedness of affected dogs, and clinical and pathological similarities with inherited neuropathies reported in other dog breeds, a hereditary basis for the degenerative polyneuropathies in Siberian huskies is suspected. However, 5 different mutations in 3 genes known to cause polyneuropathy in other dog breeds ( NDRG1, ARHGEF10, or RAB3GAP1) were not detected in the affected Siberian huskies suggesting that more genetic variants remain to be identified. This study highlights the varied underlying lesions of polyneuropathies in young Siberian huskies.

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