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BMC Pediatrics Jun 2024Guillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral...
BACKGROUND
Guillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral nerves. Anti-sulfatide antibody-mediated complement is associated with acute sensorimotor peripheral neuropathy in GBS, which is characterized by pain and paresthesias.
CASE PRESENTATION
The child was a 7-year-old girl with headache and abdominal pain, followed by limb numbness and pain. Cranial imaging showed ventricular dilatation, peripheral nerve function conduction examination showed polyradiculopathy, and cerebrospinal fluid tests showed normal cell counts but elevated protein levels, all of which led to the diagnosis of GBS. After treatment with intravenous immunoglobulin (400 mg/kg × 5 days), the symptoms did not improve, and muscle strength progressively worsened, accompanied by paroxysmal complexion flushing, heart rate fluctuation, hyperhidrosis, and a progressive increase in cerebrospinal fluid protein (up to 3780.1 mg/L). On the basis of these findings combined with serum anti-sulfatide IgM positivity, anti-sulfatide antibody-related GBS was considered, and treatment with low-dose prednisolone (1 mg/kg/d) led to symptom improvement.
CONCLUSIONS
Anti-sulfatide antibody-associated GBS is associated with small fiber peripheral neuropathy. The main manifestations are pain, paresthesias and autonomic dysfunction. In addition to the dysfunction of spinal nerve root absorption caused by increased cerebrospinal fluid protein, autonomic dysfunction may be involved in pain. When the therapeutic effect of immunoglobulin is not satisfactory, a low dose and short course of corticosteroids can be considered, and the prognosis is good.
Topics: Humans; Female; Child; Guillain-Barre Syndrome; Abdominal Pain; Headache; Sulfoglycosphingolipids; Autoantibodies; Prednisolone
PubMed: 38926645
DOI: 10.1186/s12887-023-04287-5 -
The American Journal of Case Reports Jun 2024BACKGROUND Antibodies against tumor necrosis factor alpha (anti-TNF-alpha) are currently widely used in the treatment of inflammatory bowel diseases (IBD), despite a...
BACKGROUND Antibodies against tumor necrosis factor alpha (anti-TNF-alpha) are currently widely used in the treatment of inflammatory bowel diseases (IBD), despite a number of reported adverse effects. Diverse neurologic syndromes, including the Guillain-Barre syndrome (GBS), an immune-mediated disease characterized by evolving ascending limb weakness, sensory loss, and areflexia, have been described in association with anti-TNF-alpha therapy. CASE REPORT A 45-year-old White woman was in follow-up with fistulizing ileocolonic Crohn disease using combination therapy (infliximab plus azathioprine) as CD maintenance therapy. After 3 years of this immunosuppressive therapy, she presented with symmetrical and ascending paresis in the lower limbs, and later in the upper limbs, in addition to reduced reflexes in the knees, 1 day after an infliximab infusion. The patient was hospitalized and treatment for CD was suspended. Neurophysiology studies demonstrated a pattern compatible with acute inflammatory demyelinating polyradiculopathy, with predominantly motor involvement, consistent with Guillain-Barre syndrome (GBS). Clinical, laboratory, and imaging exams were unremarkable. She was treated with intravenous immunoglobulins, with a progressive and complete resolution of neurological symptoms. After 1-year follow-up, she presented with active Crohn disease, and we opted for treating her with vedolizumab, with which she achieved clinical and endoscopic remission. CONCLUSIONS Patients receiving biological therapy with anti-TNF-alpha agents should be monitored for central or peripheral neurological signs and symptoms. The development of GBS can be secondary to anti-TNF-alpha treatment. The positive temporal relationship with TNF-alpha therapy and onset of neurological symptoms reinforces this possibility.
Topics: Humans; Guillain-Barre Syndrome; Female; Crohn Disease; Middle Aged; Infliximab; Tumor Necrosis Factor-alpha
PubMed: 38824385
DOI: 10.12659/AJCR.943709 -
Cureus Apr 2024We present a case of cytomegalovirus (CMV) polyradiculopathy which occurred concomitantly with CMV encephalitis and CMV retinitis in a patient with HIV/AIDS. Our...
We present a case of cytomegalovirus (CMV) polyradiculopathy which occurred concomitantly with CMV encephalitis and CMV retinitis in a patient with HIV/AIDS. Our patient is a 43-year-old male who was admitted with progressive changes in mentation. Cerebrospinal fluid (CSF) analysis showed elevated white blood cell (WBC), low glucose, and elevated protein. The polymerase chain reaction (PCR) panel of CSF was positive for CMV, and other microbiology results were negative. Extensive bilateral CMV retinitis was also noted. The patient was started on ganciclovir and foscarnet, and two weeks after, highly active antiretroviral therapy (HAART) was initiated using Truvada and dolutegravir. The hospital course was complicated by urinary retention and bilateral lower extremity weakness with hypotonia, severe hyperalgesia, and allodynia. An electromyography (EMG) study demonstrated bilateral lumbosacral root dysfunction at L2-S1 with active neurologic changes indicating significant axon loss. Neurology was consulted, and the patient was diagnosed with CMV-induced polyradiculopathy. After three months of treatment, no improvement was noted on lower limbs as he continued with intravenous (IV) ganciclovir. The therapeutic response to induction therapy was discordant as improvement of encephalitis was noted, but not on polyradiculopathy after 180 days of treatment. This highlights the lack of data and treatment guidelines for established CMV polyradiculopathy and not only the necessity for prolonged treatment of CMV polyradiculopathy but also the difficulty in recovery of function once it has developed.
PubMed: 38752099
DOI: 10.7759/cureus.58230 -
Cureus Feb 2024Guillain-Barré syndrome (GBS) in post-transplant patients is a rare clinical presentation. Although in the literature this neurological condition has been mainly...
Guillain-Barré syndrome (GBS) in post-transplant patients is a rare clinical presentation. Although in the literature this neurological condition has been mainly associated with viral infections secondary to immunosuppression, GBS should not only be suspected in patients with an acute condition. It is essential to always rule out a viral or bacterial cause, looking for the most common sources, i.e., urinary, respiratory, and gastrointestinal. The diagnosis of GBS is clinically based, and its management is based on the use of intravenous immunoglobulin (IVIG) or plasma exchange. Its timely diagnosis allows treatment to be started early, thus improving the prognosis of these patients and reducing the time of hospitalization and complications associated with it. This report shows how an interdisciplinary approach is vital in such cases, as both the precipitant and the disease must be managed to decrease the morbidity and mortality associated with this condition. It is crucial to evaluate the benefits and risks of withdrawing immunosuppressive treatment in post-transplant patients, and being able to recognize when restarting them is indicated.
PubMed: 38505462
DOI: 10.7759/cureus.54380 -
Cureus Jan 2024The Epstein-Barr virus (EBV) is a DNA virus that has been infecting humans since ancient times, capable of causing a wide range of pathologies and affecting...
The Epstein-Barr virus (EBV) is a DNA virus that has been infecting humans since ancient times, capable of causing a wide range of pathologies and affecting approximately 90% of the population. A 61-year-old male with no significant medical history presented with a 5-day history of imbalance and difficulty walking. Neurological examination revealed specific findings, including absent reflexes, bilateral asynergy, and gait abnormalities. Contrasting with Guillain-Barré Syndrome, lumbar puncture suggested a central nervous system infection. Serological testing confirmed Epstein-Barr virus (EBV) positivity, and intravenous immunoglobulin led to significant improvement. Electromyogram results suggested inflammatory/ipnfectious polyradiculopathy. Repeat EBV serology, showing strongly positive IgG and negative IgM, confirmed the diagnosis of Polyradiculoneuropathy secondary to EBV. This case underscores the rare neurological complications of EBV and the importance of considering viral infections in such presentations.
PubMed: 38435902
DOI: 10.7759/cureus.53302 -
PM & R : the Journal of Injury,... Feb 2024
PubMed: 38362606
DOI: 10.1002/pmrj.13124 -
Annals of Clinical and Translational... Apr 2024Recent studies have reported the involvement of peripheral nervous system (PNS) in association with MOG-IgG, including isolated neuropathies. In this retrospective study...
Recent studies have reported the involvement of peripheral nervous system (PNS) in association with MOG-IgG, including isolated neuropathies. In this retrospective study we characterized the PNS involvement in MOG antibody associated disease (MOGAD). Six out of 215 MOGAD patients had PNS involvement (all polyradiculopathy) that occurred concurrently with a CNS demyelinating episode. We also demonstrated MOG expression in healthy human controls' proximal nerve root. Nine patients with true-positive MOG-IgG1 had PNS involvement temporally unrelated to a CNS demyelinating event. All these patients had an alternate etiology of PNS involvement. Isolated peripheral neuropathy is not a feature of MOGAD, but inflammatory nerve root involvement can occur concurrently with CNS demyelinating events.
Topics: Humans; Peripheral Nerves; Peripheral Nervous System Diseases; Retrospective Studies
PubMed: 38234084
DOI: 10.1002/acn3.52001 -
Clinical Neurophysiology : Official... Feb 2024The mechanisms underlying neuropathic tremor remain incompletely understood and a distinction has not been drawn between proximal and distal neuropathies. Lower limb...
OBJECTIVE
The mechanisms underlying neuropathic tremor remain incompletely understood and a distinction has not been drawn between proximal and distal neuropathies. Lower limb tremor contributes to imbalance in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but this is unexplored in other neuropathies. We characterized upper and lower limb tremor in chronic immune sensory polyradiculopathy (CISP) and distal acquired demyelinating neuropathy with anti-MAG antibodies (DADS-MAG), contrasted to CIDP.
METHODS
This was a cross-sectional study of 38 patients (CIDP [n = 25], CISP [n = 7], DADS-MAG [n = 6]). Clinical assessment, tremor study recordings, nerve conduction studies, and somatosensory evoked potentials were performed. Balance was measured by force platform.
RESULTS
Upper limb tremor was prevalent (CIDP 66%, CISP 70%, DADS-MAG 100%). Peak frequencies followed a gradient along the upper limb, unchanged by weight-loading. Lower limb tremor was also present (CIDP 32%, CISP 29%, DADS-MAG 66%) and associated with imbalance. Nerve conduction parameters correlated with upper limb tremor in DADS-MAG and CISP, and imbalance in CISP.
CONCLUSIONS
Upper limb tremor is mediated by peripheral and central mechanisms regardless of distal or proximal pathology. Lower limb tremor correlates with peripheral nerve function and contributes to imbalance.
SIGNIFICANCE
This study contributes to the understanding of neuropathic tremor. Addressing lower limb tremor may be of therapeutic importance for neuropathy-associated imbalance.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Tremor; Cross-Sectional Studies; Peripheral Nerves; Neuritis; Neural Conduction; 4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid
PubMed: 38194761
DOI: 10.1016/j.clinph.2023.12.005 -
Medical Journal, Armed Forces India Dec 2023Systemic lupus erythematosus (SLE) can affect multiple systems in which central nervous system (CNS) involvement is common, but peripheral nervous system involvement is...
Systemic lupus erythematosus (SLE) can affect multiple systems in which central nervous system (CNS) involvement is common, but peripheral nervous system involvement is also increasingly being recognized. Guillian-Barre syndrome (GBS) as the first manifestation of SLE has been reported, but rare and not well understood. A 39-year female presented with GBS-like illness but on evaluation found to have features of SLE. Cerebrospinal fluid (CSF) showed characteristic albuminocytological dissociation and nerve conduction study (NCS) was suggestive of demyelinating polyradiculoneuropathy. On evaluation, she was found to have polyarthralgia, autoimmune hemolytic anemia, class I Lupus nephritis, mild splenomegaly, and pleural effusion. Serum antinuclear antibody was 4+ positive (coarse speckled) and extractable nuclear antigen profile revealed anti-dsDNA and anti-Sm antibody positivity, with low complement level. She fulfilled the diagnostic criterion of SLE and was managed with both plasmapheresis and pulse steroids followed by cyclophosphamide monthly pulse and oral hydroxychloroquine maintenance and showed significant improvement. The literature review showed only 26 cases reported till now. GBS without any obvious trigger should be extensively evaluated, as the underlying etiology will affect the treatment protocol as well as the prognosis. Our report highlights the significance of early recognition of SLE as a trigger of GBS, which changes conventional GBS treatment.
PubMed: 38144622
DOI: 10.1016/j.mjafi.2023.02.007 -
Tumori Dec 2023Melanoma is an aggressive malignancy, historically characterized with a poor prognosis and few treatment options. The advent of target therapy with BRAF and MEK...
BACKGROUND
Melanoma is an aggressive malignancy, historically characterized with a poor prognosis and few treatment options. The advent of target therapy with BRAF and MEK inhibitors, as well as immunotherapy, changed this scenario and improved the prognosis of patients with BRAF V600E mutation. These therapies are generally well tolerated. Neurological toxicities, especially polyradiculopathy, are very rare with BRAF inhibitors and MEK inhibitors although some cases have been described in recent years, regardless of the type of target therapies combination used.
CASE REPORT
We report the case of a patient with BRAF V600E-mutated metastatic melanoma treated with dabrafenib and trametinib who has developed a demyelinating polyradiculoneuropathy.
CONCLUSION
This case, once more, should draw our attention to the possibility of rare, but potentially serious side effects, even in the case of generally well-tolerated treatments. Especially in the presence of side effects, it is important a close relationship between clinicians and patients for the management of adverse events and the choice of the best treatment strategy.
Topics: Humans; Proto-Oncogene Proteins B-raf; Melanoma; Pyridones; Mitogen-Activated Protein Kinase Kinases; Polyradiculoneuropathy; Antineoplastic Combined Chemotherapy Protocols; Mutation
PubMed: 38050794
DOI: 10.1177/03008916231202102