Ulcerative colitis associated mononeuritis multiplex in 75-year-old patient: Rare case report.

Inflammatory bowel disease (IBD) refers to a collection of chronic, idiopathic inflammatory/autoimmune disorders of the gastrointestinal tract characterized by relapsing and remitting episodes. In this case report, we will report a patient who has encountered ulcerative colitis related to mononeuritis multiplex as a rare clinical scenario. A 75-year-old male patient, with a prior medical history including long-standing hypertension, recurring episodes of peripheral joint arthritis, leg skin lesions reminiscent of erythema nodosum, and persistent chronic diarrhea over the past 2 years, was recently hospitalized at the rheumatology department of Imam Reza Hospital in Tabriz. Throughout the patient's hospital stay, a series of diagnostic assessments were conducted, encompassing procedures such as colonoscopy, electromyography and nerve conduction studies, echocardiography, renal ultrasonography, and standard hematological analyses. The patient underwent the following treatment regimen, which resulted in a significant improvement in his condition.

Severe Mononeuritis Multiplex in Eosinophilic Granulomatous Polyangiitis: A Case Report.

This report describes a 48-year-old man who presented with a month history of weakness and paraesthesia associated with severe pain of all four limbs. Initially diagnosed and treated as Guillain Barre syndrome due to the severity of his extremity weakness, it was later discovered to be eosinophilic granulomatous polyangiitis (EGPA). Mononeuritis multiplex should not be underestimated or overlooked in the setting of diagnosing EGPA and requires prompt treatment with biologics to limit the permanent consequences on patient's quality of life with regard to developing limb weakness and pain.  Although peripheral neuropathy, namely, mononeuritis multiplex, is not the most common feature of EGPA, it is important to consider it in order not to delay treatment with biologic agents that as seen in our patient can both halt the progress of the disease as well as give the patient a better quality of life.

Eosinophilic Granulomatosis With Polyangiitis Gradually Worsening With Consecutive Pregnancies: A Case Report.

This case report elucidates the diagnosis and management of eosinophilic granulomatosis with polyangiitis (EGPA), a form of systemic vasculitis, in a 32-year-old female presenting with progressive dermal, respiratory, and gastrointestinal symptoms following multiple pregnancies. The patient's history of allergic reactions and pregnancies suggested a gradual progression of EGPA, a condition rarely associated with pregnancy. Initial symptoms were misinterpreted as allergic reactions and acute gastroenteritis, delaying the correct diagnosis. Laboratory findings included eosinophilia and elevated immunoglobulin E, while further investigations ruled out other differential diagnoses, such as chronic eosinophilic leukemia. A clinical diagnosis of EGPA was made based on symptom progression, eosinophilia, and mononeuritis multiplex, absent typical granulomatous changes in the skin biopsy. Treatment with high-dose prednisolone and rituximab halted disease progression and improved symptoms, highlighting the critical importance of prompt diagnosis and treatment in preventing irreversible complications. This case emphasizes the need for general physicians to consider pregnancy as a potential trigger for autoimmune diseases like EGPA, especially in patients presenting with multi-symptom allergic reactions and high inflammatory markers.

Eosinophilic Granulomatosis With Polyangiitis Presenting With Purpura, Mononeuritis Multiplex, and Cerebral Infarction.

We present a case of a 52-year-old woman who had transient speech impediment and progressive numbness, weakness, and a purpuric rash affecting her limbs, with severe joint pains. Because she had a chest infection two weeks prior, her clinical presentation gave rise to a suspicion of post-infective vasculitis or post-infective polyneuritis. Further investigation proved this to be eosinophilic granulomatosis with polyangiitis (EGPA) presenting with purpura, mononeuritis multiplex, and cerebral infarction. Treatment with glucocorticoids and cyclophosphamide led to rapid remission. This case highlights the potential difficulty in diagnosing EGPA because of its multiple clinical manifestations and emphasizes the importance of a thorough review of the past medical history.

Mononeuritis multiplex following immune checkpoint inhibitors in malignant pleural mesothelioma.

Introduction: Mononeuritis multiplex is frequently related to vasculitic neuropathy and has been reported only sporadically as an adverse event of immune checkpoint inhibitors. Methods: Case series of three patients with mononeuritis multiplex-all with mesothelioma-identified in the databases of two French clinical networks (French Reference Center for Paraneoplastic Neurological Syndromes, Lyon; OncoNeuroTox, Paris; January 2015-October 2022) set up to collect and investigate n-irAEs on a nationwide level. Results: Three patients (male; median age 86 years; range 72-88 years) had pleural mesothelioma and received 10, 4, and 6 cycles, respectively, of first-line nivolumab plus ipilimumab combined therapy. In patient 1, the neurological symptoms involved the median nerves, and in the other two patients, there was a more diffuse distribution; the symptoms were severe (common terminology criteria for adverse events, CTCAE grade 3) in all patients. Nerve conduction studies indicated mononeuritis multiplex in all patients. Peripheral nerve biopsy demonstrated necrotizing vasculitis in patients 1 and 3 and marked IgA deposition without inflammatory lesions in patient 2. Immune checkpoint inhibitors were permanently withdrawn, and corticosteroids were administered to all patients, leading to complete symptom regression (CTCAE grade 0, patient 2) or partial improvement (CTCAE grade 2, patients 1 and 3). During steroid tapering, patient 1 experienced symptom recurrence and spreading to other nerve territories (CTCAE grade 3); he improved 3 months after rituximab and cyclophosphamide administration. Discussion: We report the occurrence of mononeuritis multiplex, a very rare adverse event of immune checkpoint inhibitors, in the three patients with mesothelioma. Clinicians must be aware of this severe, yet treatable adverse event.

IgG4 disease with multiorgan involvement: a case report.

BACKGROUND: IgG4-related disease (IgG4RD) is a rare fibroinflammatory disease with multiorgan involvement. It presents insidiously over several years and can be a diagnostic enigma. Delays in diagnosis occur due to failure to consider IgG4 as a differential diagnosis, atypical presentations, and an insidious clinical course. CASE PRESENTATION: We report the case of a 70-year-old Sri Lankan man with pulmonary, renal, and neurological involvement of IgG4-related disease. Clinical manifestations evolved over a 4-year period and included exertional shortness of breath and dysesthesia of extremities. The diagnosis was established with clinical, radiological, and pathological criteria laid down by The American College of Rheumatology/European League Against Rheumatism in 2019. Following diagnosis, the patient was started on oral steroids, with rapid improvement of his respiratory and neurological symptoms. He is currently under follow-up and will be monitored with clinical and radiological parameters, complement levels, and lung function tests. CONCLUSION: This case outlines the presentation of a patient with IgG4-related disease with concurrent involvement of three uncommon sites. It highlights methods of diagnostic deduction by considering the clinical course of illness, imaging, and histopathology. It also describes evolving associations of IgG4-related disease with tuberculosis and lymphomas, which bear important diagnostic and therapeutic considerations.

B-Cell Lymphoma Presenting With Seventh Cranial Nerve Palsy and Mononeuritis Multiplex: A Case Report and Comprehensive Literature Review.

Diagnosing B-cell lymphoma-associated mononeuritis multiplex is challenging due to its rarity and the potential co-existence of other causes of mononeuritis multiplex. Here, we report a case of a 74-year-old male who initially presented with left cranial neuropathies followed by right-sided extremity weakness with hyporeflexia, right facial involvement, and subsequently asymmetric weakness and multifocal muscle wasting. Minor improvements were observed with multiple rounds of steroid treatment. The diffuse large B-cell lymphoma diagnosis was eventually established six months later upon a repeat mediastinal lymph node biopsy and cerebrospinal fluid cytology. A nerve biopsy demonstrated severe axonal neuropathy with loss of axons in all fascicles without evidence of vasculitis. A muscle biopsy showed atrophy in both type 1 and type 2 fibers. A presentation of mononeuritis multiplex warrants concern for B-cell lymphoma, mainly when other mechanisms of peripheral neuropathy are less likely.

Mononeuritis Multiplex Due to Hansen's Disease: A Look Through Ultrasound and Electrodiagnosis.

Hansen's disease is caused by Mycobacterium leprae. This bacillus can invade the peripheral nerves asymmetrically, including the ulnar, median, and radial nerves, causing mononeuritis multiplex. We present the case of a 41-year-old man with a history of Hansen's disease with sensory and motor symptoms. Electrodiagnostic studies and ultrasound showed asymmetric lesions of the median, ulnar, and radial nerves. Because this is the main complication of this pathology, electrodiagnosis is clearly valuable for its diagnosis, demonstrating axonal and myelin involvement, as well as signs of denervation and reinnervation. Ultrasound is valuable in the detection, diagnosis, and assessment of the extent of mononeuritis multiplex due to Hansen's disease. It aids in identifying significant inflammatory deterioration, as indicated by increased blood flow in the nerves and enlargement of the nerves. This technique allows for the exploration of nerves such as the ulnar nerve and branches of the brachial plexus. In a complementary way, ultrasound provides information on the severity of the disease. Early diagnosis of this entity is essential because it can generate aesthetic and functional permanent affectation.

Aseptic meningitis followed by mononeuritis multiplex in a patient with primary Sjögren's syndrome.

A 38-year-old woman was admitted to hospital because of fever and headache. Increased cerebrospinal cell count and protein without evidence of infection led to a diagnosis of aseptic meningitis. Although she improved with acyclovir and glyceol, she experienced left forearm pain and sensory disturbance with drop fingers. Poor derivation of compound muscle action potentials in the left radial nerve was observed, leading to a diagnosis of mononeuritis multiplex with sensorimotor neuropathy. Because the patient had primary Sjögren's syndrome with anti-Ro/SS-A antibody and salivary gland hypofunction, treatment with methylprednisolone, intravenous immunoglobulin, and intravenous cyclophosphamide was followed by oral glucocorticoid therapy. After these intensive therapies, her drop fingers gradually improved, although sensory disturbance remained. In conclusion, we report a case of aseptic meningitis and subsequent mononeuritis multiplex that was successfully treated with intensive immunotherapy in a patient with primary Sjögren's syndrome.

New-onset severe eosinophilic granulomatosis with polyangiitis following the third dose of mRNA COVID-19 vaccine: A case report.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a complex multifactorial disease that results in multisystemic inflammation of the small- and medium-sized arteries. The exact pathogenesis of this syndrome is poorly understood, but it is postulated to result from a combination of eosinophilic dysfunction, genetic predisposition, and the development of autoantibodies after exposure to an unknown stimulus. We describe a case of new-onset EGPA following the third dose of the Pfizer-BioNTech mRNA vaccine in an infection-naive middle-aged man with a background history of allergic respiratory symptoms. The patient developed acute onset of mononeuritis multiplex, pauci-immune glomerulonephritis, and leucocytoclastic vasculitis 10 days after receiving the booster dose. His laboratory markers including eosinophil count, antineutrophil cytoplasmic antibodies, and renal function tests improved markedly after the initiation of pulse steroid therapy and rituximab infusion. However, his peripheral muscle weakness and neuropathic pain did not respond to the initial therapy but improved later with intravenous cyclophosphamide and intravenous immunoglobulin. To the best of our knowledge, this is the fourth case report of post-coronavirus disease 2019 vaccination precipitation of EGPA. All reported cases including our report were in patients with previous allergic manifestations who received mRNA-based coronavirus disease 2019 vaccines, and all the patients developed mononeuritis multiplex at presentation. Despite the few reported cases of post-vaccination autoimmune phenomena, the temporal association between vaccination administration and disease onset does not indicate causality, given the mass vaccination programmes employed. However, the novel use of the mRNA platform in vaccine delivery necessitates vigilant monitoring by the scientific committee.

Mononeuropathy multiplex as an uncommon presentation of intravascular lymphoma: A case report.

Key Clinical Message: Although intravascular lymphoma rarely presents with peripheral neuropathy, learning about this presentation can lead to timely diagnosis and improved prognosis in patients with intravascular lymphoma. Abstract: A 64-year-old man presented with asymmetric paresthesia and subsequent weakness of his feet and a 10 kg weight loss over 40 days. Electrodiagnostic studies revealed distal axonal sensory-motor polyneuropathy with ongoing axonal loss. A peroneal nerve biopsy showed intravascular proliferation of CD-20 positive lymphocytes, which suggested intravascular large B-cell lymphoma.

Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) Masquerading As Mononeuritis Multiplex: A Case Report.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders that causes severe small-vessel inflammation with systemic manifestations. There are three subtypes of AAV, namely granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA). The most commonly affected organs are the upper and lower respiratory tract and the kidneys with occasional and varied neurological manifestations. Here we report a case of a 61-year-old female who presented with a one-month history of numbness, paresthesia, and asymmetric distal weakness of both lower limbs without any bladder or bowel involvement. Similar complaints appeared in her upper limbs three days prior to admission. She also suffered from myalgia, arthralgia, reduced appetite, and lost 8-10 kg weight over the past six months. Her nerve conduction study (NCV) revealed asymmetrical, predominantly motor, mixed, axonal and demyelinating polyneuropathy affecting both lower limbs, which was suggestive of mononeuritis multiplex. After a detailed workup, she tested strongly positive for cytoplasmic ANCA (c-ANCA). Although there was no clinical involvement of the respiratory tract, a contrast-enhanced computed tomography scan of the thorax and abdomen showed multifocal subpleural and lung parenchymal soft tissue lesions and mediastinal and bilateral hilar lymphadenopathy suggestive of a granulomatous lesion. She was diagnosed with the GPA variant of ANCA-associated vasculitis. Remission induction was achieved with high-dose methylprednisolone and cyclophosphamide along with alternate-day cotrimoxazole. Remission was maintained with tapering doses of steroid and mycophenolate mofetil with a slow but sustained recovery. On follow-up after one year, she walked without support with mild residual burning paresthesia in both feet. This case highlights the fact that neurological symptoms can be the presenting manifestation of AAV, and clinicians should have a high level of suspicion for AAV in patients presenting with mononeuritis multiplex, especially after ruling out common causes. By considering such etiologies, it may be possible to diagnose the condition at an earlier stage and initiate treatment to prevent potential pulmonary or renal damage.

Clinical and Neurophysiological Evidence of Mononeuritis Multiplex During the COVID-19 Era.

Infection with the novel coronavirus that causes coronavirus disease 2019 (COVID-19) results in a variety of clinical symptoms, including various neurological abnormalities. Peripheral nervous system symptoms, such as peripheral neuropathies, were often recorded in the medical literature, primarily as Guillain-Barré syndrome. Mononeuropathy multiplex is a multifocal axonal neuropathy commonly associated with vasculitis or connective tissue disease. Recent evidence about its associations with severe COVID-19 infection and intensive care unit hospitalization is being considered. A 58-year-old man with clinical and electrophysiological confirmation of mononeuropathy multiplex was reported during the peri-COVID-19 illness. He was treated with steroids and achieved a satisfactory response. Therefore, clinical and neurophysiological evaluation is recommended for any patient presenting with neurological manifestations following COVID-19 infection.

[Diagnosis of cryoglobulinemic vasculitis based on B symptoms and peripheral neuropathy: a case report].

A man in his 50s presented with a 2-month history of paresthesia and hypoesthesia of the extremities and B symptoms including low-grade fever, weight loss, and night sweats. He also reported a 3-year history of skin discoloration in cold weather. Laboratory test results showed a high white blood cell count and elevated serum C-reactive protein and rheumatoid factor (RF) levels. Complement levels were low, and tests for cryoglobulin showed positive results. Computed tomography revealed generalized lymphadenopathy, and 18F-fluorodeoxyglucose-positron emission tomography showed increased uptake; therefore, we performed cervical lymph node and muscle biopsies. The patient was diagnosed with nodular marginal zone lymphoma and cryoglobulinemic vasculitis (CV) and received chemotherapy and steroid treatment with improvement in symptoms. CV is a rare immune complex small-vessel vasculitis. It is important to measure RF and complement levels and consider infections, collagen diseases, and hematological disorders in the differential diagnosis in patients with suspected vasculitis or CV.

A severe case of neuroleukemiosis caused by B cell chronic lymphocytic leukemia, presenting as mononeuritis multiplex.

AIMS: To report an exceptional case of nerve infiltration by an otherwise benign chronic B cell leukemia, inducing severe mononeuritis multiplex. METHODS: The patient underwent extensive evaluation, including nerve conduction study and myography, brain and plexus MRI, and nerve biopsy. RESULTS: The clinical and electrophysiological diagnosis was a mononeuritis multiplex with severe motor and sensory involvement; only the nerve biopsy allowed definite diagnosis and introduction of chemotherapy, leading to resolution of sensory deficit and progressive motor improvement. DISCUSSION: Neuroleukemiosis caused by chronic lymphoid leukemia is an exceptional diagnosis. The presence of other possible causes like cryoglobulinemia could induce avoidance of nerve biopsy thus undertreating patient, since steroid treatment is not expected to be efficient on lymphocytic proliferation. Our case stretches the importance of nerve biopsy and raises neuromuscular specialist's awareness of this rare entity.

Painless progressive mononeuritis multiplex secondary to AML associated neuroleukemiosis.

Neuroleukemiosis describes peripheral nerve involvement secondary to leukemic infiltration, a rare complication of leukemia with various clinical presentations, leading to diagnostic challenges for hematologists and neurologists. We present two cases of painless progressive mononeuritis multiplex secondary to neuroleukemiosis. A literature review of previously reported cases of neuroleukemiosis was undertaken. Neuroleukemiosis may present as a progressive mononeuritis multiplex. The diagnosis of neuroleukemiosis requires a high index of suspicion and be aided by repeated CSF analysis.

Long-term Safety and Efficacy of Benralizumab for Eosinophilic Granulomatosis with Polyangiitis Complicated with Severe Neuropathy.

The efficacy of benralizumab, as well as mepolizumab, to granulomatosis with polyangiitis (EGPA) involved with mononeuritis multiplex remains unclear. We experienced a case of EGPA presenting neuropathy with severe asthma. Muscle weakness due to neuropathy involved with gait disturbance was partly ameliorated by intravenous immunoglobulin therapy. Mepolizumab (100 mg/day) did not promote further improvement of neuropathy. However, the administration of benralizumab instead of mepolizumab improved neuropathy quickly and enabled walking alone. The efficacy of benralizumab for EGPA and its complication has been maintained for over four years. Benralizumab may be a possible treatment for EGPA presenting neuropathy with severe asthma.

COVID-19 vaccine-induced vasculitis in a patient with sarcoidosis: A case report.

A 55-year-old lady with a nine-year history of controlled sarcoidosis developed vasculitis after Sinopharm COVID-19 vaccine (BBIBP- CorV). She was ultimately diagnosed with mononeuritis multiplex based on EMG-NCV findings and administered methylprednisolone and cyclophosphamide pulse therapy for 5 days, and then continue with prednisolone and a monthly pulse of cyclophosphamide.

Hypoglossal Nerve Palsy Following COVID-19 Vaccination in a Young Adult Complicated by Various Medicines.

Mononeuritis multiplex is a rare form of cerebral nerve palsy caused by various factors. Coronavirus disease 2019 (COVID-19) vaccination could be an etiology of mononeuritis multiplex, which can affect various nerves. Post-COVID-19 and vaccination-related neurological impairments involve cranial nerves such as the facial, trigeminal, and vagal nerves. We report our experience with a 34-year-old man who developed hypoglossal nerve palsy following COVID-19 vaccination, complicated by progressive mononeuritis multiplex. Hypoglossal nerve palsy may occur following COVID-19 vaccination. The symptoms vary and may progress without treatment. Physicians should consider the possibility of mononeuritis multiplex after COVID-19 vaccination and provide prompt treatment for acute symptom progression.

Perspectives on the Efficacy of Benralizumab for Treatment of Eosinophilic Granulomatosis With Polyangiitis.

Two types of interleukin (IL)-5 antibody biologics, anti-IL-5 antibodies (mepolizumab) and anti-IL-5α receptor antibodies (benralizumab), are indicated for severe asthma. While high-dose mepolizumab is also indicated for EGPA, benralizumab is indicated only for severe asthma. Benralizumab is characterized by antibody-dependent cell-mediated cytotoxicity activity, giving them specific and rapid anti-IL-5α receptor binding abilities and the ability to target a high number of eosinophils in tissues as well as peripheral blood. Recently, reports on the efficacy of benralizumab as a treatment for EGPA have been published, along with reports on some cases that are difficult to treat with existing oral corticosteroids and mepolizumab. Therefore, we focus on the perspective of the efficacy and safety of benralizumab as a treatment for EGPA patients with steroid dependence in this review. A total of 41 patients with EGPA were treated with benralizumab. After the introduction of benralizumab, oral corticosteroids could be reduced to 10 mg/day or less in all cases and to less than 5 mg/day in 80% or more of the cases. Discontinuation of oral corticosteroids was achieved in more than 40% of patients with EGPA. Benralizumab was effective in patients with mepolizumab-refractory EGPA and intractable cardiac and neuropathy complications. Efficient elimination of eosinophils is expected to improve the remission rate of EGPA with benralizumab treatment. Although the total number of patients was small, benralizumab was safe and tolerable in a wide range of age groups, suggesting efficacy in severe cases with EGPA.