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Introduction We present a painful small-fiber neuropathy variant of Guillain-Barre syndrome characterized by antecedent infectious symptoms, hyporeflexia, and albuminocytologic dissociation. Methods Two patients received intravenous immunoglobulin, one corticosteroids. Results The patients subsequently improved. Immunoglobulin G (IgG) antibodies in their acute phase sera strongly bound to murine small nerve fibers, and the binding disappeared during the convalescent phase. Serum transfer to a murine nociceptive model induced transient alteration in thermal pain responses. Discussion Our case series suggest that an acute transient immune response can be directed against small nerve fibers, and that patients so affected can exhibit features of Guillain-Barre syndrome. Muscle Nerve 57: 320-324, 2018.

Introduction: Paraparetic Guillain-Barre syndrome (GBS) is a rare subtype of GBS characterized by leg weakness and areflexia in the absence of neurological involvement of the arms, cranial nerves, or respiratory muscles. Onset is characterized by lower back, buttock, or leg pain, followed by development of symmetric flaccid limb weakness in the absence of sensory disturbance. Methods: We describe an elderly woman who developed postinfectious symmetric flaccid leg weakness in the absence of sensory disturbance. Serial nerve conduction studies were carried out over 5 months. Results: Antecedent infection, a monophasic disease course, and the presence of cerebrospinal fluid albuminocytological dissociation suggested a diagnosis of paraparetic GBS. Serial nerve conduction studies demonstrated nondemyelinating reversible conduction failure, which was restricted to the legs. Axonal neuropathy was supported by the presence of anti-GM1 IgG antibodies. Conclusions: These findings suggest that patients with paraparetic GBS have axonal neuropathy, which is restricted to the lower limbs. Muscle Nerve 55: 281–285, 2017

Introduction: Acute bulbar, neck, and limb weakness carries several potential differential diagnoses. Although a diagnosis can often be established clinically, investigations such as electrodiagnostic and antibody testing can provide support for the clinical diagnosis and may aid in understanding the pathogenesis. A 65-year-old woman presented with acute bulbar, neck, and rapidly progressive bilateral upper limb weakness. Methods: Clinical evaluation, electrophysiological, and serological studies were undertaken. Results: Neurophysiology demonstrated proximal conduction block. A clinical diagnosis of pharyngeal-cervical-brachial weakness, a localized variant of Guillain-Barre syndrome, was made. The patient received treatment with intravenous immunoglobulin and made a remarkable recovery over the next month. She was found to have serum monospecific anti-GT1a antibodies. Conclusions: We report a case of pharyngeal-cervical-brachial weakness with monospecific anti-GT1a antibodies and discuss the differential diagnosis of acute bulbar, neck, and limb weakness. Muscle Nerve, 2015

Bifacial weakness with paresthesias (BFP) is a subtype of Guillain-Barre syndrome defined by rapidly progressive bilateral facial weakness in the absence of other cranial neuropathies, ataxia, or limb weakness. Many patients also complain of distal limb paresthesias and display diminished or absent deep tendon reflexes. BFP is a localized form of Guillain-Barre syndrome and is thought to be caused exclusively by demyelinating- rather than axonal-type neuropathy. Patients with BFP do not display anti-ganglioside IgG antibodies. Since it is rare, many physicians are unfamiliar with BFP, as bilateral facial weakness is more commonly associated with sarcoidosis, Lyme disease, or meningeal pathology. Many patients diagnosed with bilateral Bell palsy may instead have BFP. In this review, we highlight the clinical features of BFP and outline diagnostic criteria.

Introduction: Conduction block (CB) has been included in the Rajabally criteria for axonal Guillain–Barre syndrome (GBS). Because the nerve roots may be affected early in GBS, detection of proximal CB by the triple stimulation technique (TST) can be useful. Methods: We describe TST findings in 2 patients who presented with the pharyngeal–cervical–brachial (PCB) variant of axonal GBS. Results: In the first patient, although conventional nerve conduction studies (NCS) did not fit electrodiagnostic criteria for axonal GBS, the TST detected proximal CB in the median and ulnar nerves. In the second patient, NCS fulfilled criteria for axonal GBS, and the TST detected proximal CB in the median nerve. After plasmapheresis, NCS and TST findings were normalized, suggesting reversible conduction failure rather than demyelinating CB. Conclusion: The TST may be useful for diagnosis of PCB when NCS remain inconclusive. The technique provides additional clues for classifying PCB into the acute nodo-paranodopathies. Muscle Nerve, 2015

Introduction: Dr. C. Miller Fisher described the appearance of unilateral facial palsy after resolution of ataxia in a patient with the eponymic Miller Fisher syndrome (MFS). However, there have been very few reports of delayed appearance of facial weakness in Guillain-Barre syndrome (GBS) and MFS when the other neurological signs reached nadir or started improving. Methods: In this study we reviewed the clinical and laboratory findings of consecutive patients with GBS (n = 195) and MFS (n = 68). Results: Delayed facial weakness occurred in 12 (6%) GBS and 4 (6%) MFS patients and was unilateral in 5 (42%) GBS and 2 (50%) MFS patients. In those patients with delayed facial weakness, neither limb weakness nor ataxia progressed, and facial weakness disappeared without immunotherapy. Conclusions: Because facial weakness can lead to further morbidity, it would be prudent for clinicians to warn patients of this possibility, although additional immunotherapy is usually not required. Muscle Nerve 51: 811–814, 2015

Acute ataxic neuropathies with disialosyl antibodies include Fisher syndrome, ataxic Guillain-Barre syndrome (GBS), and acute sensory ataxic neuropathy. Fisher syndrome and ataxic GBS are more strongly associated with IgG anti-GQ1b and anti-GT1a than with anti-GD1b antibodies, whereas the association is reversed in the case of acute sensory ataxic neuropathy. Chronic ataxic neuropathy with disialosyl antibodies is associated with IgM paraprotein to GD1b and GQ1b, which occasionally reacts with GT1a. The clinical, electrophysiological, and pathological features, along with experimental findings, suggest that acute and chronic ataxic neuropathies with disialosyl antibodies form a continuous clinical and pathophysiological spectrum characterized by a complement-mediated disruption at the nodal region and are better classified in the new category of nodo-paranodopathies.

Introduction: Differing patterns of neurophysiological abnormalities have been reported in patients with Fisher syndrome. Fisher syndrome is rare, and few series have incorporated prospective serial studies to define the natural history of nerve conduction studies in Guillain–Barre syndrome. Methods: In an ongoing prospective study of Guillain–Barre syndrome patients, patients who presented with Fisher syndrome and its spectrum of illness were assessed through serial neurological examinations, nerve conduction studies, and serological testing of IgG against gangliosides and ganglioside complexes. Results: Of the 36 Guillain–Barre syndrome patients identified within 2 years, 17 had features of Fisher syndrome. Serial nerve conduction studies detected significant abnormalities in sensory nerve action potential amplitude in 94% of patients associated with 2 patterns of recovery—non-demyelinating reversible distal conduction failure and axonal regeneration. Similar changes were seen in motor nerves of 5 patients. Conclusions: Patients with the Fisher syndrome spectrum of illness have significant sensory involvement, which may only be evident with serial neurophysiological studies. Muscle Nerve 49:558–563, 2014

The possibility that some patients diagnosed with an acute sensory neuropathy could actually have Guillain-Barre syndrome (GBS) has been repeatedly advanced in the literature, but the number of cases reported is small. The reports have shown different clinical presentations and electrophysiological findings and are variously named, thus generating terminological and nosological confusion. We operatively defined sensory GBS as an acute, monophasic, widespread neuropathy characterized clinically by exclusive sensory symptoms and signs that reach their nadir in a maximum of 6 weeks without related systemic disorders and other diseases or conditions. We reviewed the literature through searches of PubMed from 1980 to March 2011 and our own files. On the basis of the size of fibers involved and the possible site of primary damage, we propose tentatively classifying sensory GBS and related disorders into three subtypes: acute sensory demyelinating polyneuropathy; acute sensory large-fiber axonopathy-ganglionopathy; and acute sensory small-fiber neuropathy-ganglionopathy.

In two patients with the pharyngeal-cervical-brachial variant (PCB) of Guillain-Barré syndrome (GBS), low amplitude distal compound muscle action potentials and partial motor conduction blocks normalized without development of excessive temporal dispersion within 4 weeks. Sensory nerve action potentials significantly improved in amplitude or, when absent, rapidly became recordable at follow-up. Besides axonal degeneration, PCB is characterized by reversible conduction failure in both motor and sensory fibers and is in the continuous spectrum of axonal GBS subtypes.

Four criteria must be satisfied to conclude that a given microorganism causes Guillain-Barré (GBS) or Fisher (FS) syndrome associated with anti-ganglioside antibodies: (1) an epidemiological association between the infecting microbe and GBS or FS; (2) isolation in the acute progressive phase of illness of that microorganism from GBS or FS patients with associated anti-ganglioside IgG antibodies; (3) identification of a microbial ganglioside mimic; and (4) a GBS or FS with associated anti-ganglioside antibodies model produced by sensitization with the microbe itself or its component, as well as with ganglioside. Campylobacter jejuni is a definitive causative microorganism of acute motor axonal neuropathy and may cause FS and related conditions. Haemophilus influenzae and Mycoplasma pneumoniae are possible causative microorganisms of acute motor axonal neuropathy or FS. Acute and chronic inflammatory demyelinating polyneuropathies may be produced by mechanisms other than ganglioside mimicry.

We used cycles of plasma exchange and intravenous cyclophosphamide to treat a patient who had chronic inflammatory demyelinating polyneuropathy with anti-myelin-associated glycoprotein/sulfoglucuronylparagloboside IgM antibody. After treatment, serum anti-sulfoglucuronylparagloboside IgM antibody titers were reduced significantly, and clear symptomatic improvement followed. The percentage of CD57-positive lymphocytes and natural killer cell activity had also returned to normal. Our findings indicate that serial measurements of these parameters are useful for monitoring treatment effect in this disease.

To compare the effects of intravenous immunoglobulin (IVIg) therapy and plasmapheresis for the IgG anti-GM1-positive subtype of Guillain-Barre syndrome (GBS), clinical and electrophysiological recoveries were analyzed in 24 patients treated with IVIg (n = 10) or plasmapheresis (n = 14). At entry, there were no significant differences between the two patient groups in age, sex, clinical severity (Hughes grade), sum scores of distally evoked amplitudes of compound muscle action potentials (CMAPs), and frequency of Campylobacter jejuni infection. The patients treated with IVIg had significantly lower Hughes grade scores 1, 3, and 6 months after onset (P = 0.03), and a higher probability to regain independent locomotion at 6 months [P(logrank) = 0.044]. In the IVIg group, markedly rapid recovery (improvement by two or more Hughes grade scores within 4 weeks) was more frequent (6 of 10 vs. 3 of 14, P = 0. 03), and delayed recovery (unable to walk independently at 6 months) was less frequent (0 of 10 vs. 4 of 14, P = 0.06). CMAP sum score at 6 months tended to be greater for the IVIg group (P = 0.07). For the IgG anti-GM1-positive subgroup of GBS patients, IVIg therapy may be a more efficacious treatment than plasmapheresis.

If anti-GM1 antibody plays a role in the axonal damage in Guillain-Barré syndrome, the common entrapment sites may be preferentially involved with evidence of axonal dysfunction. To assess this hypothesis, we studied nerve conduction across the cubital tunnel in 44 patients. Abnormal amplitude reduction of compound muscle action potentials (CMAPs) was found in 45% of 20 immunoglobulin G (IgG) anti-GM1-positive and in 29% of 24 anti-GM1-negative patients. The time course and sequel were distinct between the two groups. In the former group, the amplitude reduction was prominent in weeks 1 to 2 and was followed by a decrease in distal CMAPs (axonal degeneration) or an increase in proximal CMAPs (resolution of conduction block). In contrast, anti-GM1-negative patients showed slower resolution with temporal dispersion. In anti-GM1-positive cases, amplitude reduction at the common entrapment site is frequent and may reflect wallerian degeneration or physiological conduction block at the nodes of Ranvier, both suggesting axonal involvement.

A 49-year-old woman developed acute left facial, hypoglossal, and phrenic nerve palsies, as well as dysphagia and weakness in the neck and arms. Electrophysiologic studies showed an acute motor axonal neuropathy. Serum anti-GM1 IgG antibody was positive. Intavenous immunoglobulin treatment resulted in good clinical recovery. The present report indicates that the cranial and phrenic nerves may be affected unilaterally in Guillain–Barre syndrome, and that there is clinical variability in the axonal subtype of this syndrome. © 2002 John Wiley & Sons, Inc. Muscle Nerve 25: 297–299, 2002 DOI 10.1002/mus.10041

We earlier reported cases of 2 patients with severe acute Guillain-Barre syndrome (GBS) associated with high-IgG anti-GD1a antibody titer. We now have investigated the autoantibody against GD1a or GM1 in 37 GBS patients using the enzyme-linked immunosorbent assay and have found a statistically significant association between IgG anti-GD1a antibody and the severity of the disease (need of a respirator for more than 1 month and a poor functional prognosis 3 months after neurologic onset). An autopsy which showed severe GBS associated with IgG anti-GD1a antibody produced the following findings: (1) severe axonal degeneration and segmental demyelination of peripheral nerves; (2) lymphocytic infiltration; and (3) marked central chromatolysis of the lower motoneurons.

We report cases of 2 patients with pure motor Guillain--Barre syndrome of explosive onset who required mechanical ventilation for more than 2 months. Their electrophysiologic findings and poor clinical recoveries suggested severe axonal degeneration involving the motor nerves. Enzymelinked immunosorbent assay and thin-layer chromatogram-immunostaining showed the sera of both patients had high IgG antibody titer against GD1a ganglioside. Their titers decreased with the clinical course of the illness. GD1a as well as GM1, appears to be the target pathogenic antigen in motor axon disorders. Elevated IgG anti-GD1a antibody titer may prove useful for predicting severe GBS. © 1992 John Wiley & Sons, Inc.