Axonal Guillain Research Articles

Guillain–Barre syndrome in a 7-month-old boy successfully applied plasma exchange

Despite being the most common cause of acute flaccid paralysis in children Guillain–Barré syndrome has a low incidence under 18 years old, and is even rarer under the age of 2. Established treatment regimens include intravenous immunoglobulin and plasma exchange in older children and adults. However very limited data are available for the efficacy and safety of plasma exchange in infants younger than 12 month-old. This article presents the experience of plasma exchange in the case of 7-month-old boy diagnosed with Guillain–Barré syndrome.A 7-month-old boy was referred to the pediatric intensive care unit with a 10-day history of progressive weakness, feeding difficulty and constipation. He was diagnosed with axonal Guillain–Barré syndrome on the basis of clinical and electromyographical findings. The patient recovered fully with intravenous immunoglobulin and plasma exchange.Plasma exchange may be a safe option in the treatment in infants with Guillain–Barré syndrome as young as 7-month-age.

Unilateral hypoglossal nerve palsy with asymmetric facial and limb paresis in axonal Guillain–Barré syndrome

AbstractWe report a 45‐year‐old man with motor axonal Guillain–Barré syndrome who developed left facial nerve palsy, right hypoglossal nerve palsy, and predominantly left‐sided upper limb paresis and left lower limb paresis. Blood examination identified immunoglobulin G antibodies against gangliosides GD1a, GD1b and GQ1b, and GD1b/GD1a and GD1b/GT1b complexes. He was treated with intravenous immunoglobulin (400 mg/kg/day for 5 days) twice, and tongue deviation and facial palsy resolved in 3 months. Unilateral or asymmetric involvement of the cranial and limb nerves represent a variant form of axonal Guillain–Barré syndrome.

Miller Fisher syndrome is a nodo-paranodopathy, but not a myelinopathy

ABSTRACT Miller Fisher syndrome, characterized by ophthalmoplegia, ataxia and areflexia, is a variant of Guillain–Barré syndrome. There have been controversies over the electrophysiological studies of Miller Fisher syndrome, as both demyelinating and axonal changes have been reported. In recent years, reversible conduction failure has been reported in patients with Miller Fisher syndrome with the use of serial nerve conduction studies. The similarity between Miller Fisher syndrome and axonal Guillain–Barré syndrome has led to the suggestion of a common autoimmune mechanism at the nodes and paranodes.

3-O-sulfo-β-d-galactose moiety of endogenous sulfoglycolipids is a potential ligand for immunoglobulin-like receptor LMIR5

Axonal Guillain–Barré syndrome (GBS) is an autoimmune neuropathy characterized by limb weakness and/or paralysis due to the presence of autoantibodies against brain glycolipids. The immune receptors that recognize these autoimmune targets have not been described. In this study, 12 C-type lectin and 10 immunoglobulin-like receptors were screened for their potential ligands from the brain glycolipids, which are the binding targets for GBS autoantibodies. These glycolipids were GM1, GM2, GD1a, GD1b, GQ1b, crude gangliosides, and 3-O-sulfo-β-d-galactosylceramide C24:1 (designated as C24:1). A direct interaction between ligand and receptor was examined using an ELISA-based binding assay. C-type lectin (CLEC5a, SIGNR3) and immunoglobulin-like receptors (TREM2, TREM3, LMIR2, LMIR5, LMIR7, LMIR8) interacted with C24:1. In addition, TREM3 did bind to GQ1b. LMIR5 interacted with GD1a, GQ1b, and crude gangliosides. Binding with highest affinity was observed for the LMIR5–C24:1 interaction, which was selected for further verification. C24:1 was found to induce MCP-1 production, but not proinflammatory cytokines, in basophils. C24:1-induced MCP-1 production was significantly reduced in DAP12−/− basophils. Importantly, LMIR5 ligation by C24:1 resulted in NFAT activation through DAP12 in LMIR5-expressing reporter cells. Structural analysis showed that LMIR5 recognized the 3-O-sulfo-β-d-galactose moiety of C24:1. The findings indicated that C24:1 is a potential ligand for DAP12-coupled LMIR5.

English

Abstract Introduction This article reports a case of the difficulty to differentiate between critical illness polyneuropathy and axonal Guillain–Barre syndrome when triggered by subarachnoid haemorrhage. Case report An 81-year-old man was admitted comatose (Glasgow coma scale score 4/15) after a subarachnoid haemorrhage. His neurological condition gradually improved with as best motor response (M4) withdrawal from pain at the four limbs. The patient developed early complications such as septicaemia and acute renal injury. After 3 weeks, a marked decrease of motor response (M1) was noted in the lower, and, to lesser extent, upper limbs. Deep tendon reflexes were abolished. The cerebrospinal fluid examination showed elevated protein level. After electrophysiological examination, the diagnosis of acute motor axonal neuropathy, a variant of Guillain–Barre syndrome, was discussed versus critical illness polyneuropathy. Specific therapy for Guillain–Barre syndrome could not be administered. No significant motor recovery was observed after 7 months. Conclusion The distinction between critical illness polyneuropathy and Guillain– Barre syndrome remains difficult in critically ill patients. It is not known if subarachnoid haemorrhage could be considered as a possible triggering factor for Guillain–Barre syndrome.

Reversible conduction failure is distinct from neurophysiological patterns of recovery in mild demyelinating Guillain–Barré syndrome

Axonal Guillain–Barré syndrome (GBS) can present with rapid resolution of nerve conduction failure also referred to as early reversible conduction failure. It is unclear if this reversible conduction failure represents successful immunotherapy or part of the natural evolution of mild axonal disease, or whether it is in fact recovery from mild demyelination. Here we describe two patients with axonal and demyelinating GBS with favorable outcomes without immunotherapy. One was a 32-year-old pregnant woman with axonal GBS. She presented with acute limb weakness, areflexia and mild sensory symptoms. Serial neurophysiology revealed reversible conduction block across elbow segment of the ulnar nerve without remyelinating features in the recovery stage. The second patient was a 38year-old pregnant woman with demyelinating GBS presenting with acute limb weakness, reduced lower limb reflexes and sensory disturbance in a glove and stocking distribution. Although nadir was reached on Day 8, nerve conduction studies revealed progressive prolongation of median distal motor latency and conduction slowing across the elbow segment of the ulnar nerve over 2weeks. Conduction failure in axonal GBS can spontaneously recover, and is distinct from the recovery pattern seen in mild demyelinating GBS. Serial nerve conduction studies can help distinguish between the two pathophysiologies.

132. Axonal Guillain–Barré syndrome in pregnant woman following fetal demise

132. Axonal Guillain–Barré syndrome in pregnant woman following fetal demise

Antiganglioside antibodies are associated with axonal Guillain–Barré syndrome: A Japanese–Italian collaborative study

BackgroundWhether or not antiganglioside antibodies are related to axonal or demyelinating Guillain–Barré syndrome (GBS) is still a matter of controversy, as detailed in previous studies conducted in Western and Asian...

Acute motor-sensory axonal Guillain–Barré syndrome with unilateral facial nerve paralysis after rotavirus gastroenteritis in a 2-year-old boy

Guillain-Barré syndrome (GBS) is well known as presenting with acute immune-mediated polyneuropathies, with strong associations with antecedent infections. Several variant forms of GBS have been described, including acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy, and sensory GBS. We present a rare case of 2-year-old boy with acute motor and sensory polyneuropathy and left-sided facial nerve paralysis after rotavirus infection. He received immunoglobulin i.v. with subsequent satisfactory recovery.

Monospecific high-affinity and complement activating anti-GM1 antibodies are determinants in experimental axonal neuropathy

It has been difficult to replicate consistently the experimental model of axonal Guillain–Barré syndrome (GBS). We immunized rabbits with two lipo-oligosaccharides (LOS1 and LOS2) derived from the same C. jejuni strain and purified in a slightly different way. LOS1 did not contain proteins whereas several proteins were present in LOS2. In spite of a robust anti-GM1 antibody response in all animals the neuropathy developed only in rabbits immunized with LOS1. To explain this discrepancy we investigated fine specificity, affinity and ability to activate the complement of anti-GM1 antibodies. Only rabbits immunized with LOS1 showed monospecific high-affinity antibodies which activated more effectively the complement.Although it is not well understood how monospecific high-affinity antibodies are induced these are crucial for the induction of experimental axonal neuropathy. Only a strict adherence to the protocols demonstrated to be successful may guarantee the reproducibility and increase the confidence in the animal model as a reliable tool for the study of the human axonal GBS.

Glial fibrillary acidic protein: A marker of axonal Guillain–Barrè syndrome and outcome

Glial fibrillary acid protein (GFAP) is increased in serum and cerebrospinal fluid of patients with dementia, traumatic brain injury, stroke, and multiple sclerosis. To determine whether GFAP is increased in Guillain-Barré syndrome (GBS) we evaluated serum GFAP in 30 controls, 20 patients with acute inflammatory demyelinating neuropathy (AIDP), and 17 with primary axonal GBS. Serum GFAP levels were increased in axonal GBS (median, 0.74) compared with controls (median, 0.41; P < 0.0001) and AIDP (median, 0.58; P = 0.0015). GFAP levels correlated with Hughes grades (serum r = 0.74; P < 0.0001) 6 months after neuropathy onset. Applying the cutoff value in serum of 0.63 to the diagnosis of axonal GBS, we obtained a sensitivity of 76.5% and a specificity of 86%. Thus, serum GFAP levels may be used in GBS as a diagnostic marker of the axonal variant and to predict outcome.

IAP036 Acute motor axonal neuropathy (AMAN) or unclassified Guillain Barre syndrome?

IAP036 Acute motor axonal neuropathy (AMAN) or unclassified Guillain Barre syndrome?

Urinary retention and sympathetic sphincter obstruction in axonal Guillain–Barré syndrome

A 62-year-old woman with axonal Guillain-Barré syndrome developed weakness and urinary retention simultaneously. The retention failed to recover for 10 months even after she regained the ability to walk. The patient exhibited no postural hypotension. Videourodynamics showed that the retention was caused not by the bladder paralysis but rather by internal (sympathetic) sphincter obstruction, which is extremely uncommon in peripheral neuropathies. We started the patient on an alpha-adrenergic antagonist, urapidil, at 30 mg/day, and this led to successful relaxation of the urethra and reduced the postvoid residual from 200 ml to less than 30 ml. The underlying mechanisms of urinary retention in our patient appeared to involve hyperactive lumbosacral sympathetic nerves. Urinary retention and sympathetic sphincter obstruction can thus be features of axonal Guillain-Barré syndrome.

Overlap of pathology in paralytic rabies and axonal Guillain–Barré syndrome

We describe clinical and pathological features of a case of paralytic rabies with acute axonal neuropathy that closely resembled axonal Guillain-Barre syndrome. This case emphasizes that there is overlap of both clinical and pathological features in paralytic rabies and axonal Guillain-Barre syndrome. These findings raise the possibility that infectious and autoimmune etiologies can lead to similar morphological changes in the nerves.

Recovery patterns and long term prognosis for axonal Guillain–Barré syndrome

Background: Little is known about the long term prognosis for patients the severe acute motor axonal neuropathy (AMAN) form of Guillain–Barré syndrome (GBS), unlike those with acute inflammatory demyelinating neuropathy...

Patterns and serial changes in electrodiagnostic abnormalities of axonal Guillain–Barré syndrome

In Guillain-Barré 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. To elucidate the patterns and sequential changes in electrodiagnostic abnormalities of anti-ganglioside-positive GBS. Detailed serial electrodiagnostic findings were reviewed for 51 patients with GBS. Anti-ganglioside antibodies were measured by ELISA. 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. 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.

Axonal Guillain–Barré syndrome associated with axonal Charcot–Marie–Tooth disease

We report the first case of axonal Guillain–Barré syndrome (GBS) associated with axonal Charcot–Marie–Tooth disease (CMT). A 30-year-old Japanese man, who had suffered leg atrophy and foot deformity since childhood, developed acute weakness in his four limbs following an upper respiratory tract infection. Nerve conduction studies showed low compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes in all the nerves tested. Serial studies showed a rapid increase in CMAP amplitude, but no significant change in SNAP, which indicates that the acute event selectively involved motor axons and was superimposed on a baseline motor–sensory axonal neuropathy, probably CMT Type 2. Elevated serum IgG antibodies against GM1 and GM1b, an increase in CSF protein, and rapid clinical and electrophysiological recovery after plasma exchange support the diagnosis of a pure motor axonal form of GBS, acute motor axonal neuropathy. The association may be coincidental, but a particular susceptibility to axonal damage of CMT2 cannot be excluded.

ACUTE PREVALENTLY MOTOR AXONAL NEUROPATHY DURING DISULFIRAM TREATMENT

Disulfiram (Di) presents hepatic and central nervous system toxicity as main side effects. Di occasionally produces peripheral neuropathy consisting of a chronic axonal sensory pattern. A 34‐year‐old woman, after a five‐year history of moderate abuse, started Di therapy (400 mg per day) and stopped alcohol intake. Three months later, painful tingling and burning sensation occurred on distal lower limbs, rapidly followed by a foot drop within three days and severe weakness spreading to proximal segments and to upper limbs within two weeks. The patient presented with moderate distal multimodal hypoaesthesia, complete loss of deep tendon reflexes, mild distal muscle hypotrophy and severe motor disability (Rankin grade 4). Electrophysiological examination showed a typical prevalently motor axonal neuropathy with inexcitability of motor responses in lower limbs, but normal latencies of “F” responses in upper limbs. Standard cerebrospinal fluid (CSF) examination was normal and investigations for cytomegalovirus, herpes viruses and Campylobacter Jejuni (CJ) were negative. Anti GM1 antibodies (Ab) were negative in serum and CSF. Sural nerve biopsy revealed axonal degeneration with moderate loss of large myelinated fibres, frequent endoneurial foamy macrophages and milder degeneration of unmyelinated axons. No axonal swellings with filamentous deposits were detected. Rows of myelin ovoids were present on teased fibres, whereas rare short remyelinated internodes and no segmental demyelination were observed. Follow‐up, three months later, shows a significant increase of general strength and sensory function (Rankin grade 3), though heels and toes gait is not recovered and painful/burning paraesthesias still require gabapentin treatment. The main diagnostic problem of this case is distinction from acute axonal Guillain Barré syndrome. Although there is no consensus regarding electrophysiological diagnostic criteria for AMSAN, significant abnormalities of “F” waves are generally observed on affected nerves. On the immunological side, anti GM1 Ab increased titer and evidence of CJ infection, though not mandatory, are very frequent in AMSAN. Moreover, pathological changes distinctive of AMSAN, such as peri‐axonal or intra‐axonal macrophages, were absent in our case. On the other hand, a similar acute onset of a sensory—motor axonal neuropathy during Di treatment has been previously described (Nukada, 1981).

Fisiopatología del síndrome de Guillain Barré axonal

Se describe la fisiopatología del síndrome de Guillain Barré axonal. Se consideran especialmente cinco aspectos: 1) Agentes etiológicos, específicamente el Campylobacter jejuni. 2) Susceptibilidad genética humana. 3) Mimetismo molecular entre lipopolisacáridos y lipoproteínas. 4) Mecanismo de acción de los anticuerpos antigangliósidos y 5) Hallazgos patológicos.

Unilateral cranial and phrenic nerve involvement in axonal Guillain–Barré syndrome

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–Barré 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