galactocerebroside and Guillain-Barre-Syndrome

Recent work identified anti-GM2 and anti-GalNAc-GD1a IgG ganglioside antibodies as biomarkers in dogs clinically diagnosed with acute canine polyradiculoneuritis, in turn considered a canine equivalent of Guillain-Barré syndrome. This study aims to investigate the serum prevalence of similar antibodies in cats clinically diagnosed with immune-mediated polyneuropathies. The sera from 41 cats clinically diagnosed with immune-mediated polyneuropathies (IPN), 9 cats with other neurological or neuromuscular disorders (ONM) and 46 neurologically normal cats (CTRL) were examined for the presence of IgG antibodies against glycolipids GM1, GM2, GD1a, GD1b, GalNAc-GD1a, GA1, SGPG, LM1, galactocerebroside and sulphatide. A total of 29/41 IPN-cats had either anti-GM2 or anti-GalNAc-GD1a IgG antibodies, with 24/29 cats having both. Direct comparison of anti-GM2 (sensitivity: 70.7%; specificity: 78.2%) and anti-GalNAc-GD1a (sensitivity: 70.7%; specificity: 70.9%) antibodies narrowly showed anti-GM2 IgG antibodies to be the better marker for identifying IPN-cats when compared to the combined ONM and CTRL groups (P = .049). Anti-GA1 and/or anti-sulphatide IgG antibodies were ubiquitously present across all sample groups, whereas antibodies against GM1, GD1a, GD1b, SGPG, LM1 and galactocerebroside were overall only rarely observed. Anti-GM2 and anti-GalNAc-GD1a IgG antibodies may serve as serum biomarkers for immune-mediated polyneuropathies in cats, as previously observed in dogs and humans.

Topics: Animals; Autoantibodies; Biomarkers; Cats; Dogs; G(M1) Ganglioside; G(M2) Ganglioside; Galactosylceramides; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Polyneuropathies

A 79-year-old woman with a history of Guillain-Barré syndrome (GBS) developed somnolence and tetraparesis after pneumonia. Based on clinical and laboratory findings, she was diagnosed with complications of acute inflammatory demyelinating polyneuropathy (AIDP) and acute disseminated encephalomyelitis (ADEM). Anti-galactocerebroside (Gal-C) IgG antibodies were detected in her serum. Cases of recurrent GBS in patients who are positive for this antibody are extremely rare. The anti-Gal-C IgG antibodies likely played an important role in the pathogenesis of the AIDP and ADEM.

Topics: Adult; Aged; Antibodies; Encephalomyelitis, Acute Disseminated; Female; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulins; Immunologic Factors; Male; Treatment Outcome

Mycoplasma pneumoniae (Mp) triggers Guillain-Barré syndrome (GBS) and elicits anti-galactocerebroside (GalC) antibodies. Specifically anti-GalC IgG is associated with Mp-GBS, possibly because of its better ability to cross the blood-nerve barrier (BNB). We here investigated CSF for the presence of anti-GalC in GBS. Intrathecal anti-GalC was found in 46% of Mp-GBS patients (n=6/13), in contrast to 16% of GBS controls (n=4/25) and 0% of non-GBS controls (n=0/7). The antibodies most likely originated from increased BNB permeability and/or intrathecal synthesis. Intrathecal anti-GalC IgG was specifically associated with Mp-GBS, further supporting that anti-GalC IgG contributes to the pathogenesis of GBS.

Topics: Adolescent; Adult; Autoantibodies; Autoantigens; Female; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Mycoplasma pneumoniae; Pneumonia, Mycoplasma; Young Adult

We report the case of a 12-year-old girl who developed Guillain-Barré syndrome (GBS) and optic neuritis (ON) following Mycoplasma pneumoniae infection. Her symptoms, including bilateral vision impairment and tingling in her hands and right foot, were resolved after methylprednisolone pulse therapy. Serum anti-galactocerebroside (Gal-C) IgM antibodies were detected in our patient. This is the first report of a child with GBS and ON associated with M. pneumoniae infection.

Topics: Autoantibodies; Child; Female; Galactosylceramides; Guillain-Barre Syndrome; Humans; Methylprednisolone; Mycoplasma pneumoniae; Optic Neuritis

BACKGROUND A rare variant of Guillain-Barré syndrome (GBS) consists of facial diplegia and paresthesia, but an even more rare association is with facial hemiplegia, similar to Bell's palsy. This case report is of this rare variant of GBS that was associated with IgG antibodies to galactocerebroside and phosphatidic acid. CASE REPORT A 54-year-old man presented with lower left facial palsy and paresthesia of his extremities, following an upper respiratory tract infection. Physical examination confirmed lower left facial palsy and paresthesia of his extremities with hyporeflexia of his lower limbs and sensory loss of all four extremities. The differential diagnosis was between a variant of GBS and Bell's palsy. Following initial treatment with glucocorticoids followed by intravenous immunoglobulin (IVIG), his sensory abnormalities resolved. Serum IgG antibodies to galactocerebroside and phosphatidic acid were positive in this patient, but not other antibodies to glycolipids or phospholipids were found. Five months following discharge from hospital, his left facial palsy had improved. CONCLUSIONS A case of a rare variant of GBS is presented with facial diplegia and paresthesia and with unilateral facial palsy. This rare variant of GBS may which may mimic Bell's palsy. In this case, IgG antibodies to galactocerebroside and phosphatidic acid were detected.

Topics: Antibodies; Facial Paralysis; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Paresthesia; Phosphatidic Acids; Respiratory Tract Infections

Guillain-Barré syndrome (GBS) is an acute postinfectious immune-mediated polyneuropathy. Although preceding respiratory tract infections with Mycoplasma pneumoniae have been reported in some cases, the role of M. pneumoniae in the pathogenesis of GBS remains unclear. We here cultured, for the first time, M. pneumoniae from a GBS patient with antibodies against galactocerebroside (GalC), which cross-reacted with the isolate. This case prompted us to unravel the role of M. pneumoniae in GBS in a case-control study.. We included 189 adults and 24 children with GBS and compared them to control cohorts for analysis of serum antibodies against M. pneumoniae (n = 479) and GalC (n = 198).. Anti-M. pneumoniae immunoglobulin (Ig) M antibodies were detected in GBS patients and healthy controls in 3% and 0% of adults (p = 0.16) and 21% and 7% of children (p = 0.03), respectively. Anti-GalC antibodies (IgM and/or IgG) were found in 4% of adults and 25% of children with GBS (p = 0.001). Anti-GalC-positive patients showed more-frequent preceding respiratory symptoms, cranial nerve involvement, and a better outcome. Anti-GalC antibodies correlated with anti-M. pneumoniae antibodies (p < 0.001) and cross-reacted with different M. pneumoniae strains. Anti-GalC IgM antibodies were not only found in GBS patients with M. pneumoniae infection, but also in patients without neurological disease (8% vs 9%; p = 0.87), whereas anti-GalC IgG was exclusively found in patients with GBS (9% vs 0%; p = 0.006).. M. pneumoniae infection is associated with GBS, more frequently in children than adults, and elicits anti-GalC antibodies, of which specifically anti-GalC IgG may contribute to the pathogenesis of GBS. Ann Neurol 2016;80:566-580.

Topics: Adolescent; Adult; Aged; Antibodies, Bacterial; Autoantibodies; Case-Control Studies; Child; Cross Reactions; Female; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Male; Middle Aged; Mycoplasma Infections; Mycoplasma pneumoniae; Young Adult

Whether patients who have GBS with antibodies to galactocerebroside (Gal-C) and gangliosides (Gal-C-GS-GBS) more often have demyelinating or axonal neuropathy remains controversial. We assessed the electrophysiological data from 16 patients with Gal-C-GS-GBS based on the two established criteria to clarify this issue. In this largest cohort of Gal-C-GS-GBS, eight patients had demyelinating neuropathy and none exhibited axonal neuropathy on either criterion. These data indicated that antibodies to Gal-C, a myelin antigen, might predominantly be associated with demyelinating neuropathy, even in the presence of concomitant antibodies to gangliosides.

Topics: Action Potentials; Adult; Aged; Aged, 80 and over; Autoantibodies; Child; Cytokines; Enzyme-Linked Immunosorbent Assay; Female; Galactosylceramides; Gangliosides; Guillain-Barre Syndrome; Humans; Longitudinal Studies; Male; Middle Aged; Mycoplasma pneumoniae; Neural Conduction

A 67-year-old man noticed paresthesia in both legs in July 2011. Three days later, he was found on a street where he was unable to stand up. On admission, the deep sensation in both legs was severely disturbed, but their muscle strength remained normal. Cranial nerves and autonomic functions were normal. The deep tendon reflexes were diminished in both legs. Magnetic resonance imaging of the spine was normal. Motor nerve conduction studies revealed normal conduction velocity, amplitude, and F-wave latency. However, sensory nerve conduction studies revealed severe reduction of amplitude in the upper and lower extremities. Cerebrospinal fluid analysis showed normal cell counts but elevated protein levels. Screening for glycolipid antibodies showed a selective increase of galactocerebroside (Gal-C) IgG antibody. We diagnosed him with pure-sensory-type Guillain-Barré syndrome (GBS). We administered intravenous immunoglobulin (IVIG) for 5 days. After IVIG therapy, his gait disturbance improved slightly but the disturbance of deep sensation remained severe and he was transferred to a rehabilitation ward 53 days after admission. To the best of our knowledge, this is the first report of a case of pure-sensory-type GBS with Gal-C antibody alone. This case suggests a close relationship between Gal-C antibody and sensory nerve disturbance.

Topics: Aged; Autoantibodies; Biomarkers; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Treatment Outcome

Guillain-Barré syndrome (GBS) has often been associated with antibodies to glycolipids, such as galactocerebroside (Gal-C), a component of myelin. Whether patients who have GBS with anti-Gal-C antibody (Gal-C-GBS) more often have demyelinating neuropathy or axonal neuropathy remains controversial. Their clinical features have also been unestablished.. We enrolled 47 patients with Gal-C-GBS. Their clinical and electrophysiological data were retrospectively reviewed and compared to 119 patients with GBS without anti-Gal-C antibody (non-Gal-C-GBS).. Demyelinating polyneuropathy occurred 4 times more frequently than axonal polyneuropathy in patients with Gal-C-GBS, but without statistical significance compared to patients with non-Gal-C-GBS (2.2:1). Patients with Gal-C-GBS had more frequent sensory deficits, autonomic involvements, and antecedent Mycoplasma pneumoniae (MP) infection than patients with non-Gal-C-GBS.. This is the largest study clarifying the clinical and electrophysiological findings that more frequent sensory deficits, autonomic involvements, and antecedent MP infection are associated with Gal-C-GBS.

Topics: Adult; Aged; Autoantibodies; Chi-Square Distribution; Enzyme-Linked Immunosorbent Assay; Female; Galactosylceramides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Neural Conduction; Peripheral Nerves; Pneumonia, Mycoplasma; Retrospective Studies

Topics: Antibodies; Betainfluenzavirus; Coinfection; Galactosylceramides; Guillain-Barre Syndrome; Humans; Influenza, Human; Male; Middle Aged; Mycoplasma; Mycoplasma Infections

We report an 11-year-old boy with apparently the motor axonal form of Guillain-Barre syndrome (GBS) who presented with severe paralysis and respiratory insufficiency by the 3rd day from onsets of symptoms. His serum anti-Mycoplasma pneumoniae and anti-Galactocerebroside (Gal-C) IgM antibody were significantly elevated. Magnetic resonance imaging, following contrast injection, showed enhancement of the cauda equina. The patient responded quickly and dramatically to immunoadsorption therapy using a tryptophan-immobilized column, with recovery of respiratory failure and muscle strength, dominantly in the left extremities. Immunoadsorption therapy should be considered for patients with anti Gal-C antibody-associated GBS.

Topics: Antibodies, Bacterial; Cauda Equina; Child; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin M; Immunosorbent Techniques; Magnetic Resonance Imaging; Male; Mycoplasma pneumoniae; Plasmapheresis; Pneumonia, Mycoplasma

The authors previously reported the presence of antibody against galactocerebroside (Gal-C) in sera from patients with Guillain-Barré syndrome subsequent to Mycoplasma pneumoniae infection. Anti-Gal-C antibody activities in these sera were inhibited specifically by the M. pneumoniae reagent. A rabbit anti-Gal-C antibody recognized several glycolipids in M. pneumoniae. These data show that a Gal-C-like structure is present in M. pneumoniae, indicative of molecular mimicry between a major myelin glycolipid, Gal-C, and M. pneumoniae.

Topics: Animals; Autoantibodies; Galactosylceramides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Molecular Mimicry; Pneumonia, Mycoplasma; Rabbits