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Objective: To comprehensively investigate the relationship between antibodies to single glycolipids and their complexes and Guillain-Barre syndrome subtypes and clinical features. Methods: In acute sera from 199 patients with Guillain-Barre syndrome, immunoglobulin G (IgG) antibodies to glycolipids and ganglioside complexes were tested using ELISA against individual antigens from single glycolipids including gangliosides (LM1, GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, GT1a, GT1b, GQ1b) and a neutral glycolipid, asialo-GM1 (GA1), and antigens from the combination of 2 different glycolipids. Based on serial nerve conduction studies, the electrodiagnoses were as follows: 69 demyelinating subtype, 85 axonal subtypes, and 45 unclassified. Results: Significant associations were detected between acute motor axonal neuropathy subtype and IgG antibodies to GM1, GalNAc-GD1a, GA1, or LM1/GA1 complex. Reversible conduction failure was significantly associated with IgG antibodies to GM1, GalNAc-GD1a, GD1b, or complex of LM1/GA1. No significant association was demonstrated between acute inflammatory demyelinating polyneuropathy and any of the glycolipids or ganglioside complexes. Anti-ganglioside complex antibodies alone were detected in 7 patients (5 axonal subtype). Conclusions: The current study demonstrates that antibodies to single glycolipids and ganglioside complexes are associated with acute motor axonal neuropathy or acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy. Classification of evidence: This study provides Class II evidence that antibodies to glycolipids are increased in patients with acute motor axonal neuropathy and acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy.

A 52-year-old man presented with acute onset of weakness and paresthesias involving all 4 extremities in 2008 preceded by an episode of viral catarrh 3 weeks prior. Examination revealed symmetrical upper and lower extremity weakness (Medical Research Council [MRC] 3/5) and absent deep tendon reflexes. Sensory and cranial nerve examination was normal. CSF showed albumino-cytologic dissociation with leukocyte count of 3 and protein of 110 mg/dL. Acute demyelinating polyneuropathy (AIDP) was diagnosed based on electrodiagnostic studies (table), and IV immunoglobulin (IVIg) was commenced. Recovery was slow, taking 1 year. In 2014, he was admitted with similar symptoms. Examination revealed normal power of the upper extremities and grade 4/5 power of the lower extremities. There was loss of vibration and proprioception over his toes. Deep tendon reflexes were absent. There was facial diplegia. Considering the clinical presentation and electrodiagnostic studies, he was diagnosed with recurrent AIDP and treated with IVIg. He continued to deteriorate and by the third week, his power was MRC grade 3/5 in his upper and lower extremities. There was no bulbar involvement.

Objective: To determine the prevalence and specificity of antibodies against single gangliosides and ganglioside complexes in serum from 88 patients with multifocal motor neuropathy (MMN) and to study the association with clinical features. Methods: ELISA was used to detect immunoglobulin (Ig)M, IgG, and IgA antibodies against GM1, GM2, GD1a, GD1b, GM1b, GT1a, GT1b, GQ1b, GalNAc-GD1a, and the glycolipid SGPG; absorption studies were performed to study cross-reactivity. Presence of antibodies against ganglioside complexes consisting of any of combinations of GM1, GM2, GD1a, GD1b, GT1b, and GQ1b was also tested. Results: Anti-GM1 IgM, IgG, and IgA antibodies were detected in serum from 43%, 1%, and 5% of patients with MMN. Anti-GM2 IgM antibodies were detected in 6% and anti-GD1b IgM antibodies in 9% of patients. Patients with MMN with anti-GM1 IgM antibodies had more severe weakness (p < 0.01), more disability (p < 0.01), and more axon loss (p = 0.05) than patients without anti-GM1 IgM antibodies. Anti-GM1 IgM antibody titers correlated with Medical Research Council scores (correlation coefficient = 0.43; p < 0.0001). Anti-GD1b IgM antibody activity was associated with reduced vibration sense (p < 0.01). Absorption studies showed that anti-GD1b and anti-GM2 IgM antibodies cross-reacted with GM1. Antibodies against ganglioside complexes were not detected. Complexes containing GD1a, GD1b, GT1b, or GQ1b with GM1 lowered antibody activity against GM1. Conclusion: Anti-ganglioside IgM antibodies in MMN display limited specificity and are associated with severity and clinical characteristics. Results of this study suggest that anti-GM1 IgM antibodies may play a role in MMN pathogenesis. Neurology (R) 2010;75:1961-1967

Background: The action mechanism of IV immunoglobulin (IVIg) for Guillain-Barre syndrome has yet to be clarified. Objective: To evaluate clinical, histologic, and immunologic effects in a disease model of acute motor axonal neuropathy (AMAN) treated by IVIg. Methods: Rabbits were sensitized with gangliosides including GM1 and divided randomly into two groups at disease onset. One group received IV homologous γ-globulin (400 mg/kg/day) for 5 days (n = 15), and the other received saline (n = 15). Disease severity was scored (0 to 13 points) daily. Sixty days after onset, anti-GM1 antibodies were tested by ELISA, and the number of degenerative axons was counted in spinal anterior roots. Results: Between both groups at onset, there was no difference in any characteristics including clinical score. The IVIg group had faster recovery than the saline group ( p = 0.03). The percentage of rabbits that improved by a score of ≤4 was higher in the IVIg (53%) than in the saline (13%) group 60 days after onset ( p = 0.03). Anti-GM1 IgG titers 60 days after onset did not differ between the groups. The anterior roots of rabbits surviving 60 days after onset showed lower frequency of axonal degeneration in the IVIg-treated (n = 11; mean 4.5%) than in the saline-treated (n = 8; mean 11.1%) rabbits ( p = 0.01). Conclusions: The therapeutic efficacy of IVIg in an AMAN model was confirmed. IVIg may not affect the production or catabolism of anti-GM1 IgG, but it may prevent axonal degeneration of motor nerves.

Background:Patients with Guillain-Barré syndrome (GBS) afterMycoplasma pneumoniaeinfection often have antibodies to galactocerebroside (GalC). Electrodiagnosis may show acute inflammatory demyelinating polyneuropathy (AIDP).Methods:The authors report a patient with acute motor axonal neuropathy (AMAN) afterMycoplasmainfection and review seven cases ofMycoplasma-associated GBS. They investigated anti-GalC serology under various conditions associated withMycoplasmainfection.Results:The patient had immunoglobulin (Ig)G and IgM antibodies against GM1 and GalC, which cross-reacted. During the acute phase, IgM selectively immunostained axons. The cholera toxin B-subunit and rabbit anti-GM1 IgG stained a band in the lipid extract fromM pneumoniae, indicative of the presence of a GM1 epitope. SixMycoplasma-associated GBS patients with anti-GalC antibodies had non-AIDP electrodiagnoses, whereas one withMycoplasma-associated AIDP had no anti-GalC antibodies. Anti-GalC antibodies were positive in two of five patients who had neurologic diseases other than GBS afterMycoplasmainfection and in one of 12 who had acute respiratory disease caused byM pneumoniaenot followed by a neurologic disease.Conclusions:Anti-GalC antibodies inMycoplasma-associated GBS may be an epiphenomenon. In certain cases, anti-GM1 antibodies induced by molecular mimicry withM pneumoniaemay cause acute motor axonal neuropathy.

Objective: To examine the association between Miller Fisher syndrome (MFS) and antecedent Haemophilus influenzae infection. Background: Little is known about agents in prior respiratory tract infection of MFS, whereas antecedent upper respiratory symptoms are frequent. H. influenzae is a major pathogen that can cause human respiratory tract infection. Methods: The authors used ELISA to detect serum antibody against the bacterium in 70 consecutive patients with MFS and 110 with Guillain–Barre syndrome (GBS). Results: Serum anti– H. influenzae IgG and IgM antibody activities were significantly higher in the MFS group than in age- and sex-matched patients with other neurologic diseases (n = 62) and normal control subjects (n = 82). The GBS group showed no significant increase in any class of antibody activities compared with control groups. Serologic evidence of recent infection was found in five (7%) of the patients with MFS and two (2%) of 110 patients with GBS, all of whom had a history of antecedent respiratory tract infection. They frequently showed ophthalmoplegia, but other neurologic features were not remarkable. Serum anti-GQ1b IgG antibody that had cross-reactivity with GT1a ganglioside was detected in six of these seven patients. Thin-layer chromatography with immunostaining showed that serum IgG from H. influenzae –seropositive patients with high anti-GQ1b and anti-GT1a IgG antibody titers bound to the lipopolysaccharide fraction extracted from the type b H. influenzae serostrain. These bands were also stained by anti-GT1a monoclonal antibody (GMR11), indicating that the lipopolysaccharide bears the GT1a epitope. Conclusions: These findings point to H. influenzae being an agent associated with MFS. Epitopic overlap between H. influenzae and human nerve tissue may be involved in the development of MFS much as GBS is associated with Campylobacter jejuni enteritis.

Objective: To investigate the prognostic value of anti-GM1 antibody. Background: Whether anti-GM1 antibody is a marker of poor prognosis due to axonal degeneration in Guillain-Barre syndrome (GBS) is a matter of controversy. Methods: The clinical recovery of 41 consecutive GBS patients was analyzed. Results: The Hughes functional grading scores were similar at the peak, and 1, 3, and 6 months after onset for the groups of patients with (n = 19) and without (n = 22) immunoglobulin (Ig) G anti-GM1 antibodies. However, the anti-GM1-positive group included significantly higher proportions of patients with poor recovery (inability to walk independently at 6 months, 5 of 19 versus 0 of 22; p = 0.01) and those with a markedly rapid recovery (improvement by two or more Hughes grades within a month, 9 of 19 versus 4 of 22; p = 0.05). The positivity of IgG anti-GM1 antibody correlated well with the electrodiagnosis of the acute motor axonal neuropathy pattern but was not always associated with poor prognosis. Anti-GM1-positive patients showed two different patterns of clinical recovery-their conditions improved slower or faster than those of the anti-GM1-negative patients, most of whom had acute inflammatory demyelinating polyneuropathy. Conclusions: Anti-GM1 antibody is not always a marker of poor prognosis and, besides axonal degeneration, early reversible effects other than demyelination could be part of the pathophysiology of Guillain-Barre syndrome with IgG anti-GM1 antibody.

Sera from patients with Fisher's syndrome in the acute phase contain immunoglobulin (Ig)G anti-GQ1b ganglioside antibody. Removal of the autoantibody should lead to earlier recovery with less residual neurologic involvement. A tryptophan- or phenylalanin-immobilized polyvinyl alcohol gel column (IM-TR 350 or IM-PH 350) semiselectively adsorbs such autoantibodies as rheumatoid factor, anti-DNA antibody, or anti-acetylcholine receptor antibody. A batchwise adsorption test showed that an IM-TR gel adsorbed a larger amount of the IgG anti-GQ1b antibody than did an IM-PH column. Several patients with Fisher's syndrome therefore were given immunoadsorbent therapy using the IM-TR column without adverse reactions. An ex vivo plasma perfusion study done with the IM-TR column confirmed that it effectively adsorbs the IgG anti-GQ1b antibody. Results of adsorption tests done with various amino acid-immobilized gels suggest that both the hydrophobic force of the side chain and the anionic charge of the carboxylic acid in tryptophan are important in the adsorption of the autoantibody by the IM-TR gel. Immunoadsorption using the IM-TR column, which does not need replacement fluids, offers an alternative type of plasmapheresis for Fisher's syndrome.NEUROLOGY 1996;46: 1644-1651

Two patients with acute facial diplegia and hyperreflexia are described. Both patients had serologic evidence of preceding Campylobacter jejuni infection and antiganglioside IgG antibodies as well as other laboratory and electrophysiologic findings suggesting Guillain-Barre syndrome (GBS). IV immunoglobulin produced recovery. Hyperreflexia does not necessarily exclude the diagnosis of a GBS variant. Antiganglioside antibodies can help with diagnosis in difficult cases.

Objectives: We asked whether autoantibodies against neurofascin (NF)186 or NF155, both localized at the nodes of Ranvier, are present in serum of patients with inflammatory neuropathy, and whether NF-specific monoclonal antibodies are pathogenic in vivo. Methods: We cloned human NF155 and NF186, and developed an ELISA and cell-based assay to screen for antibodies to human NF in a total of 434 donors including 294 patients with Guillain-Barre syndrome variants acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy, and chronic inflammatory demyelinating polyneuropathy (CIDP). We characterized reactive samples by isotyping, tissue section staining, and epitope mapping. We also injected NF-specific monoclonal antibodies IV into rats with experimental autoimmune neuritis. Results: We detected autoantibodies to NF by ELISA in 4% of patients with AIDP and CIDP, but not in controls. Most positive samples contained immunoglobulin G (IgG)1, IgG3, or IgG4 antibodies directed to only one isoform of NF. Two patients with CIDP showed particularly high (1:10,000 dilution) NF155-specific reactivity in both assays and stained paranodes. Two other patients with CIDP who benefited from plasma exchange exhibited antibodies to NF155 by ELISA, and upon affinity purification, antibodies to both isoforms were observed by both assays. Anti-NF monoclonal antibodies enhanced and prolonged induced neuritis in rats. Conclusions: Autoantibodies to NF are detected in a very small proportion of patients with AIDP and patients with CIDP, but may nevertheless be pathogenic in these cases.

The authors describe two patients with acute sensory ataxic neuropathy. Both had a profound loss of proprioception and generalized areflexia. High titers of monospecific anti-GD1b IgG antibody were detected in their sera during the acute phase. Sensory ataxia resolved within 2 weeks after the onset. Taken together with the induction of experimental sensory ataxic neuropathy sensitized with GD1b ganglioside, GD1b may be a target molecule for autoantibody in some patients with acute sensory ataxic neuropathy.

The authors reviewed the medical records of seven patients with anti-GQ1b immunoglobulin G (IgG) who had no or minimal external ophthalmoplegia but showed ataxia. The clinical features of the patients were consistent with the ataxic form of Guillain-Barré syndrome (GBS). Anti-GQ1b IgG antibodies from the patients, as well as from those with Miller Fisher syndrome (MFS), were absorbed by GT1a. The finding that ataxic GBS and MFS have in common an autoantibody with the same fine specificity suggests that they form a continuous spectrum.

Ganglioside epitopes on Campylobacter jejuni are hypothesized as the key to the development and characterization of Guillain-Barré syndrome (GBS), but a comprehensive theory has yet to be established. A C jejuni gene, cst-II, involved in the biosynthesis of ganglioside-like lipo-oligosaccharide, shows a polymorphism (Asn/Thr51) that affects ganglioside epitopes.To examine the hypothesis that this polymorphism determines autoantibody reactivity, and thereby neurologic presentations in GBS.C jejuni isolates were collected from 105 GBS (including its variants) and 65 uncomplicated enteritis patients. The authors examined the frequency of cst-II and polymorphism (Asn/Thr51) in connection with the bacterial ganglioside epitopes, autoantibody reactivities against GM1, GD1a, and GQ1b, and patients' neurologic findings.Neuropathic strains more frequently had cst-II, in particular cst-II (Thr51), than did enteritic ones (85% vs 52%; p0.001). Strains with cst-II (Asn51) regularly expressed the GQ1b epitope (83%), whereas those with cst-II (Thr51) had the GM1 (92%) and GD1a (91%) epitopes. The presence of these bacterial epitopes in neuropathy patients corresponded to autoantibody reactivity. Patients infected with C jejuni (Asn51) more often were positive for anti-GQ1b IgG (56% vs 8%; p0.001) and had ophthalmoparesis (64% vs 13%; p0.001) and ataxia (42% vs 11%; p = 0.001). Patients who had C jejuni (Thr51) more frequently were positive for anti-GM1 (88% vs 35%; p0.001) and anti-GD1a IgG (52% vs 24%; p = 0.006) and had limb weakness (98% vs 71%; p0.001).The genetic polymorphism of C jejuni determines autoantibody reactivity as well as the clinical presentation of Guillain-Barré syndrome (GBS), possibly through modification of the host-mimicking molecule. The GBS paradigm is the first to explain the detailed pathogenesis of a postinfectious, autoimmune-mediated, molecular mimicry-triggering disorder.

Objective: To assess the production mechanism of anti-GQ1b autoantibody in Fisher syndrome (FS). Methods: The authors conducted a prospective case-control serologic study of five antecedent infections ( Campylobacter jejuni , cytomegalovirus, Epstein–Barr virus, Mycoplasma pneumoniae , and Haemophilus influenzae ) in 73 patients with FS and 73 sex- and age-matched hospital controls (HCs). Serologic evidence in FS patients of C. jejuni (21%) and H. influenzae (8%) infections was present significantly more often than in the HCs. None of the five pathogens examined was found in the 49 (67%) patients with FS. Anti-GQ1b IgG antibody was detected in most FS patients infected with C. jejuni or H. influenzae . Mass spectrometry analysis identified a C. jejuni strain (CF93-6) carrying a GT1a-like lipo-oligosaccharide (LOS) that had been isolated from an FS patient. Immunization of complex ganglioside-lacking knockout mice with the GT1a-like LOS generated IgG class monoclonal antibodies (mAbs) that reacted with GQ1b and GT1a. Thin-layer chromatography with immunostaining showed that anti-GQ1b mAb bound to the C. jejuni LOS (50% of the 20 FS-related strains) more commonly than in the Guillain–Barre syndrome (GBS)–related (7% of 70) or enteritis-related (20% of 65) strains. Anti-GM1 and anti-GD1a mAbs also reacted with the LOS from some FS-related strains (both 20%), but binding frequencies were higher in the GBS-related strains (74 and 57%). The GQ1b epitope was detected in 4 (40%) of the 10 FS-related H. influenzae strains but was absent in strains from patients with GBS ( n = 4) and uncomplicated respiratory infections ( n = 10). Conclusions: C. jejuni and H. influenzae are related to Fisher syndrome (FS) development, and production of anti-GQ1b autoantibody is mediated by the GQ1b-mimicking lipo-oligosaccharides on those bacteria. The causative agents remain unclear in the majority of patients with FS.

Background: In Guillain–Barre syndrome (GBS), anti-ganglioside antibodies are strongly associated with the acute motor axonal neuropathy (AMAN) form, but there are also cases of the demyelinating form of GBS (acute inflammatory demyelinating polyneuropathy [AIDP]) with anti-ganglioside antibodies. Objective: To elucidate the patterns and sequential changes in electrodiagnostic abnormalities of anti-ganglioside-positive GBS. Methods: Detailed serial electrodiagnostic findings were reviewed for 51 patients with GBS. Anti-ganglioside antibodies were measured by ELISA. Results: Antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a were present in 25 patients. Of these, 12 (48%) showed the AMAN pattern, 5 (20%) the AIDP pattern, and 3 (12%) isolated F-wave absence in the first examination. All five patients with the AIDP pattern showed prolonged distal latencies, but three eventually showed the AMAN pattern or rapid normalization. The remaining two still had similarly prolonged distal latencies in weeks 4 to 6, but the serial changes were distinct from those in the anti-ganglioside-negative AIDP patients who showed progressive increases in distal latencies over 2 months after onset. Conclusions: Besides the simple axonal degeneration pattern, patients with anti-ganglioside-positive Guillain–Barre syndrome can show transient conduction slowing/block in the distal or proximal nerve segments, mimicking demyelination, but anti-ganglioside antibodies do not appear to be associated with acute inflammatory demyelinating polyneuropathy.

Campylobacter jejuni enteritis is the most common antecedent infection in Guillain-Barré syndrome (GBS). C. jejuni-related GBS is usually acute motor axonal neuropathy (AMAN), but previous reports described many cases of the demyelinating subtype of GBS (acute inflammatory demyelinating polyneuropathy [AIDP]) after C. jejuni infection.To investigate whether C. jejuni infection elicits AIDP.In 159 consecutive patients with GBS, antibodies against C. jejuni were measured using ELISA. Antecedent C. jejuni infection was determined by the strict criteria of positive C. jejuni serology and a history of a diarrheal illness within the previous 3 weeks. Electrodiagnostic studies were performed weekly for the first 4 weeks, and sequential findings were analyzed.There was evidence of recent C. jejuni infection in 22 (14%) patients. By electrodiagnostic criteria, these patients were classified with AMAN (n = 16; 73%) or AIDP (n = 5; 23%) or as unclassified (n = 1) in the first studies. The five C. jejuni-positive patients with the AIDP pattern showed prolonged motor distal latencies in two or more nerves and had their rapid normalization within 2 weeks, eventually all showing the AMAN pattern. In contrast, patients with cytomegalovirus- or Epstein-Barr virus-related AIDP (n = 13) showed progressive increases in distal latencies in the 8 weeks after onset.Patients with C. jejuni-related Guillain-Barré syndrome can show transient slowing of nerve conduction, mimicking demyelination, but C. jejuni infection does not appear to elicit acute inflammatory demyelinating polyneuropathy.

Objective: To determine whether the anti-GM1 antibody IgG subclass (IgG1 to 4) is associated with clinical profiles and patterns of recovery in Guillain–Barre syndrome (GBS). Methods: The IgG subclassification of anti-GM1 antibody was examined and compared with clinical data on 42 GBS patients positive for the antibody. Results: Frequent anti-GM1 antibody subclasses were IgG1 (76%) and IgG3 (31%). IgG1 antibody was associated with preceding gastroenteritis and Campylobacter jejuni serology, whereas IgG3 antibody was associated with preceding respiratory infection. Although the severity at nadir was similar for IgG1- and IgG3-positive patients, the percentage of patients who could not walk independently was greater for the IgG1-positive group 1 month (42 vs 0%; p = 0.02), 3 months (28 vs 0%), and 6 months (25 vs 0%) after onset. Rapid recovery within 1 month occurred frequently in the patients with the IgG3 antibody but rarely in those with the IgG1 antibody (67 vs 11%; p = 0.003). Conclusions: The IgG1 subclass of anti-GM1 antibody is a major subtype indicative of slow recovery, whereas isolated elevation of IgG3 subclass antibody titer suggests rapid recovery. Variation in subclass patterns may depend on which pathogen precipitates GBS.

We describe a patient with acute unilateral oculomotor nerve palsy that occurred after an upper respiratory infection. His serum showed a high titer of anti-GQ1b IgG antibody during the acute phase of the illness, suggesting a possible postinfectious immune-mediated mechanism. A previously healthy, 47-year-old man developed diplopia (day 1) 2 weeks after experiencing a fever and cough. On admission (day 12), his blood pressure was 110/70 mm Hg, his pulse rate was 78 beats per minute, and his body temperature was 36.5 °C. Neurologic examination revealed blepharoptosis and limitation of adduction and vertical gaze on the left side (figure). Convergence was impossible. Although both pupils were symmetric (4 mm) and promptly reactive to light, pupillometry revealed a delayed reaction to light in the left pupil (data not shown). These findings were compatible with left oculomotor nerve palsy. The other cranial nerves were intact. His gait was normal, and no muscle weakness, ataxia, or sensory …

Taste loss is a rare sign in Guillain–Barre syndrome (GBS). Two of 100 patients reported subjective alteration of taste in one study,1 whereas none of 169 experienced taste loss in another.2 We have investigated the frequency of patients with GBS who present with taste loss as the initial symptom. We conducted retrospective examinations of 457 patients with GBS who had been referred to our neuroimmunologic laboratory between 1997 and 2001 for serum antiganglioside antibody tests. Five patients had accompanying subjective alteration of taste during the illness. As the initial symptom, only three patients had taste loss. An illustrative case is described here. A 45-year-old man had a respiratory infectious illness, which was resolved within 2 days. Three days after resolution (day 1), he could not identify the flavor of a nutrition drink. On day 4, he experienced numbness in the perioral region and his four limbs. He had deficiencies for salty, bitter, sour, and …

The authors reviewed the clinical features and outcome of Miller Fisher syndrome (MFS) for 50 consecutive patients with MFS including 28 patients who received no immunotherapy. Besides the characteristic clinical triad (ophthalmoplegia, ataxia, and areflexia), pupillary abnormalities, blepharoptosis, and facial palsy are frequent in MFS, whereas sensory loss is unusual despite the presence of profound ataxia. Patients with MFS usually had good recovery and no residual deficits.

Plasma exchange (PE) in Guillain-Barré syndrome (GBS) probably removes pathogenic antibodies. Results of a recent clinical study by the French Cooperative Group suggest that at least two sessions of PE are required. As an alternative procedure, we examined the effect of the number of PEs on the reduction of immunoglobulins in 11 patients. A significant immunoglobulin decrease was obtained in the first two sessions but not in subsequent ones. Based on the French trial results and our findings, we conclude that at least two PEs are needed for treating GBS.

Miller Fisher syndrome (MFS) is characterized by an acute onset of external ophthalmoplegia, cerebellar ataxia, and areflexia. Because MFS is often preceded by an episode of acute inflammatory symptoms and, in most cases, the neurologic symptoms recover after a certain period of time, the disease is regarded as a variant of Guillain-Barre syndrome (GBS). [1] Although the involvement of peripheral nerves is evident in GBS, controversy still exists as to the localization of lesions in MFS. [2] Here we report two cases of MFS in which, using contrast-enhanced three-dimensional (3-D) MRI, the abducens and oculomotor nerves were enhanced by Gd-DTPA. The patient was a 26-year-old man with a history of transient diarrhea and sore throat 1 week before he complained of diplopia and unstable gait. On admission (the fifth day of illness), his external ocular movements were limited to all directions with the right abducens nerve being most severely affected. His gait was ataxic and his deep tendon reflexes …

Cytomegalovirus (CMV) is known to be associated with various central and peripheral neurologic disorders in patients with AIDS or after bone marrow or solid organ transplantation.1 We report a patient with acute pharyngeal and brachial palsy after CMV colitis following bone marrow transplantation. This is the first report of regional neuropathy in a patient with CMV colitis. A 37-year-old woman developed acute onset of progressive dysarthria, dysphagia, and distal weakness of bilateral upper extremities. She had chronic myeloid leukemia and received bone marrow transplantation. Two months later, she developed intractable watery diarrhea and pancytopenia. Stool cultures for common intestinal pathogens, including Campylobacter jejuni , were negative. Colonic biopsy showed swelling of mucosal epithelium with nuclear inclusion bodies with immunohistochemical evidence of CMV. Serum PCR for CMV was also positive. She was treated with anti-CMV immunoglobulins (Ig) (100 mg/kg/day three times per week) and ganciclovir (5 mg/kg/day twice a week). The diarrhea stopped and the serum CMV-PCR …

Guillain-Barre syndrome (GBS) has been classified on a pathologic basis into demyelinating and axonal forms. Axonal GBS has been classified further into two groups: acute motor axonal neuropathy (AMAN) and acute motor and sensory axonal neuropathy (AMSAN).1 The principal clinical method for distinguishing AMAN, AMSAN, and acute inflammatory demyelinating polyneuropathy (AIDP) is electrodiagnostic, and clear criteria have been formulated.2,3⇓ The pathology of AMAN and AMSAN are very similar, and both conditions may follow Campylobacter jejuni enteritis.1 Anti-GM1, anti-GM1b, and anti-GD1a immunoglobulin (Ig)G antibodies can be used as immunologic markers to differentiate AMAN from AIDP, and patients with AMAN and AMSAN shared these autoantibodies.4 These data suggest that AMAN and AMSAN share a common …

In acute motor axonal neuropathy (AMAN), deep tendon reflexes are occasionally preserved or exaggerated,1-5⇓⇓⇓⇓ although the mechanism is unknown. We report the first known patient with AMAN presenting hyperreflexia in whom a CNS lesion was detected by MRI. A previously healthy 22-year-old woman felt numbness and weakness in her right upper limb 5 days after resolution of febrile condition with cough (day 1). She did not have preceding diarrhea. The weakness spread to other limbs, and she could not walk without assistance on day 3. She was admitted on day 7. Her consciousness and eye movement were intact. Neither facial nor bulbar palsy was noted. Muscle strength in her four limbs was symmetrically decreased to Medical Research Council grade 3. The deep tendon reflexes were remarkably hyperactive in all limbs and muscle tonus was spastic in lower limbs. The plantar response was extensor on the left side. Mild palmar and plantar hypalgesia were noted, and …

We used the enzyme-linked immunosorbent assay to investigate autoantibodies against the gangliosides G M1 , G D1a , G D1b , G T1b , G D2 , and G Q1b , in sera from 16 patients with Fisher9s syndrome. We found high anti-G Qlb antibody titers, mainly those of the IgG class, in the sera of 13 of these patients. The titers decreased with the clinical course of the illness. Moreover, anti-G Qlb antibody-positive patients had more severe ataxia of cerebellar type than the antibody-negative patients.

We report 2 patients with Guillain-Barre syndrome (GBS) following Campylobacter jejuni enteritis. Electrophysiologic studies indicated that the predominant process was axonal degeneration of motor nerves, and clinical recovery was poor. Serum testing by thin-layer chromatography and enzyme-linked immunosorbent assay revealed that the sera from both patients contained high titers of IgG antibody against GM1 ganglioside. These cases may represent a subgroup of GBS as acute axonal polyneuropathy following C jejuni enteritis associated with anti-GM1 antibodies.

To the Editor: Immunoglobulin G (IgG) anti-GQ1b antibody is closely associated with acute paresis of the extraocular muscles that follows infection or immunization.1,2 Radziwill et al.3 reported a patient who developed diplopia but showed internal ophthalmoplegia without external ophthalmoplegia. Serum from that patient had IgG anti-GQ1b antibody. We also examined a patient who showed isolated internal ophthalmoplegia associated with IgG anti-GQ1b antibody. A 36-year-old …

Acute sensory ataxic neuropathy (ASAN) is characterized by the acute onset of sensory ataxia and areflexia.1 The persistent neurologic deficit, absence of sensory nerve action potentials, and loss of large myelinated fibers in sural nerve biopsy could be explained by an immune-mediated or vascular process at the level of the posterior root or dorsal root ganglion (DRG).1 An autopsy study in an ASAN patient with minimal autonomic dysfunction showed T-cell-mediated ganglionitis.2 However, the site of the lesion has remained obscure in ASAN without autonomic dysfunction. Here we report the visualization of the lesion in the posterior roots of the spinal cord in an ASAN patient without autonomic dysfunction. Case report. A 70-year-old woman had been in good health until she presented with an acute onset of progressive sensory disturbance of her limbs and unsteady gait after an antecedent episode of upper respiratory tract infection. The sensory disturbance appeared at first in the left upper limb and then spread …

To investigate the presence of anti-GalNAc-GD1a antibodies in patients with Guillain-Barré syndrome (GBS) and to determine the relation of anti-ganglioside antibodies with clinical features.The GBS is heterogeneous with regard to clinical manifestations, antecedent infections, and the presence and specificity of anti-ganglioside antibodies. Recently, antibodies to minor gangliosides have been identified in serum from GBS patients.The authors used ELISA to detect anti-ganglioside antibodies in 132 GBS patients and then correlated results with a database containing information on antecedent infections and clinical parameters.Anti-GalNAc-GD1a antibodies could be detected in 19 (14%) GBS patients. The presence of anti-GalNAc-GD1a antibodies was related to antecedent Campylobacter jejuni infection (p0.001). GBS patients with anti-GalNAc-GD1a antibodies had a rapidly progressive, more severe, and predominantly distal weakness. Furthermore, they had less sensory loss, paresthesia, and cranial nerve involvement. In most patients, this reactivity was independent of reactivity to GM1. Dividing patients into separate groups based on their reactivity to GalNAc-GD1a and GM1 enabled the authors to delineate more homogeneous subgroups with regard to clinical features.This study provides further evidence for the hypothesis that antecedent infections and the specificity of subsequent anti-neural antibody responses determine the clinical manifestations in GBS patients.

We report a patient with a relapsing form of the acute sensory neuropathy syndrome associated with IgM-K type monoclonal gammopathy of undetermined significance. He rapidly developed marked sensory ataxia without weakness following an upper respiratory tract infection at age 44. The symptoms reached their maximum in a few days, followed by subsequent gradual improvement over a few weeks. However, unsteady gait remained as a chronic deficit. Stepwise progression of his symptoms occurred over 15 years with 10 similar relapses. Sensory nerve conduction studies showed the absence of action potentials, and sural nerve biopsy revealed the marked loss of large myelinated fibers. The patient's serum had an extremely high titer of an IgM monoclonal antibody directed against gangliosides GD2, GD1b, GT1b, and GQ1b.

We performed HLA typing for class I antigens on five Japanese patients with acute polyneuropathy following Campylobacter infection, all of whom showed axonopathy causing pure motor dysfunction associated with IgG anti-GM1 antibodies. We demonstrated a statistically significant association between the disease and the HLA-B35 antigen.