g(m1)-ganglioside and Cranial-Nerve-Diseases

LM1 is the predominant glycolipid in human peripheral nerve myelin and antibodies to LM1 and LM1-containing ganglioside complexes are detected in some patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The clinical features of patients with such antibodies have not yet been investigated.. Serum antibodies to LM1, a mixture of GM1 and LM1 (GM1/LM1), and that of GD1b and LM1 (GD1b/LM1) were examined in 75 consecutive patients with CIDP. The clinical features of the CIDP patients with such antibodies in the present series and those in the previous reports were investigated and compared with those of antibody-negative patients.. Of the 75 patients with CIDP, two had antibodies to LM1, three had anti-GM1/LM1 complex antibody, one had anti-GD1b/LM1 complex antibody and two had antibodies to both the GM1/LM1 and GD1b/LM1 complexes. Patients with the LM1-associated antibodies did not have cranial nerve deficits (p<0.05) and exhibited ataxia more frequently than the antibody-negative patients (p<0.01).. In humans, LM1 is contained more in the dorsal root than in the cranial nerves. The clinical features of CIDP patients with antibodies to LM1 and LM1-containing complexes may be associated with the distribution of the LM1 antigen. LM1-associated antibodies are possible markers for a subclass of CIDP.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Ataxia; Autoantibodies; Child; Cranial Nerve Diseases; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Glycolipids; Humans; Immunoglobulin G; Immunotherapy; Male; Middle Aged; Neural Conduction; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Young Adult

A 46-year-old male patient reported difficulties in speech and swallowing following gastroenteritis. Marked open nasality (open rhinophonia) and swallowing difficulties with occasional passing of food into the nasopharynx was observed during speaking with the head held in an upright position. The patient was able to articulate clearly with the head reclined or in a lying position. Endoscopy identified complete bilateral soft palate paresis consistent with bilateral glossopharyngeal nerve palsy. Additional symptoms of cranial nerve palsy appeared in the course of the disease. Intravenous corticosteroids were ineffective. A marked improvement of symptoms was achieved after i.v. immunoglobulin therapy that was initiated following identification of serum IgM anti-GM 1 ganglioside antibodies under suspicion of cranial polyneuritis. Nasality was largely resolved under additional speech exercise therapy.

Topics: Articulation Disorders; Autoantibodies; Cranial Nerve Diseases; Deglutition Disorders; Diagnosis, Differential; G(M1) Ganglioside; Glossopharyngeal Nerve Diseases; Humans; Immunization, Passive; Immunoglobulin M; Male; Middle Aged; Neuritis; Palate, Soft; Voice Disorders

Ganglioside complexes (GSCs) are known as target antigens in Guillain-Barré syndrome (GBS). To elucidate the clinical importance of the anti-GSC antibodies in GBS, we investigated serum antibodies to GSCs containing two of the gangliosides, GM1, GD1a, GD1b and GT1b, and analyzed clinical features of anti-GSC-positive GBS patients. Thirty-nine (17%) of 234 GBS patients had IgG anti-GSC antibodies. Anti-GSC-positive GBS had antecedent gastrointestinal infection and lower cranial nerve deficits more frequently than control GBS. The presence of antibody specificity to GD1a/GD1b and/or GD1b/GT1b was significantly associated with severe disability and a requirement for mechanical ventilation.

Topics: Adult; Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Cranial Nerve Diseases; Disability Evaluation; Electrophysiology; Female; G(M1) Ganglioside; Gangliosides; Gastrointestinal Diseases; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunosorbent Techniques; Male; Middle Aged; Respiration, Artificial; Severity of Illness Index

We report a case of partially treated brucellosis that developed quadriparesis, sixth and seventh cranial nerve palsy, and apnea. Electrodiagnostic studies were in favor of acute axonal poly-radiculoneuropathy. Crossreactive immunological responses due to molecular mimicry between Brucella lipooligosaccharide and GM1 ganglioside may justify the development of acute axonal polyradiculoneuropathy after brucellosis.

Topics: Acute Disease; Adult; Apnea; Brucellosis; Cranial Nerve Diseases; Electrodiagnosis; G(M1) Ganglioside; Humans; Male; Polyradiculoneuropathy

This study reports the efficacy of i.v. immunoglobulin in a patient with cranial polyneuropathy resulting from Campylobacter jejuni infection who had high titers of serum IgG antibodies against gangliosides GD1a and GT1b in the acute phase. Treatment with i.v. immunoglobulin (400 mg/kg/day x 5 days) led to rapid partial resolution of his neurologic manifestations, but complete recovery was not obtained until 6 months later. The present case suggests that i.v. immunoglobulin therapy prevents further progression of the disease but that it may not shorten the clinical course of cranial polyneuropathy in some cases associated with Campylobacter jejuni infection.

Topics: Acute Disease; Adolescent; Antibodies, Bacterial; Campylobacter Infections; Campylobacter jejuni; Cranial Nerve Diseases; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Immunoglobulins, Intravenous; Male; Treatment Outcome

The serum antibodies to N-acetylgalactosaminyl GD1a (GalNAc-GD1a) and other gangliosides as well as to Campylobacter jejuni were determined in 147 patients with Guillain-Barré syndrome (GBS). We found a distinctive clinical pattern in patients with anti-GalNAc-GD1a antibodies compared with those without the antibodies, that is, lack of cranial nerve involvement (87% versus 38%), distal-dominant weakness (80% versus 25%), and no sensory disturbance (73% versus 22%). The frequency of distal-dominant weakness was significantly higher in patients with both C. jejuni infection and anti-GalNAc-GD1a positivity (100%) than in C. jejuni-negative/anti-GalNAc-GD1a-positive (25%), C. jejuni-positive/anti-GalNAc-GD1a-negative (32%) and C. jejuni-negative/anti-GalNAc-GD1a-negative patients (20%). Lack of cranial nerve involvement and sensory disturbance were found in most C. jejuni-positive/anti-GalNAc-GD1a-positive and C. jejuni-negative/anti-GalNAc-GD1a-positive patients, but not in C. jejuni-positive/anti-GalNAc-GD1a-negative and C. jejuni-negative/anti-GalNAc-GD1a-negative patients. Although the anti-GM1-positive/anti-GalNAc-GD1a-negative patients mostly (75%) lacked cranial nerve involvement, distal-dominant weakness (38%) and lack of sensory disturbance (13%) were infrequent. These results may indicate that (1) the combination of C. jejuni infection and anti-GalNAc-GD1a antibodies, but not anti-GalNAc-GD1a, anti-GM1, or C. jejuni infection alone, is associated with a predominantly distal weakness, (2) the presence of anti-GalNAc-GD1a, rather than C. jejuni infection or anti-GM1 antibody, is associated with a lack of sensory disturbance, (3) both anti-GalNAc-GD1a and anti-GM1 antibodies are independently associated with a lack of cranial nerve impairment.

Topics: Acetylgalactosamine; Adult; Age Distribution; Autoantibodies; Cranial Nerve Diseases; Cranial Nerves; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Humans; Male; Polyradiculoneuropathy; Sex Distribution