Guillain-Barré syndrome occurring in two women after ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus

We report two women who presented with a Guillain-Barré syndrome just after a ketoacidosic comatose state disclosing an insulin-dependent diabetes mellitus. One had characteristic clinical signs and the other had major motor involvement. At neurophysiologic investigations, one had typical demyelinating neuropathy whereas the second had mainly axonal degeneration. At ultrastructural examination of a peripheral nerve biopsy, features of macrophage-associated demyelination were present in both nerve specimens, thus confirming the diagnosis of acute inflammatory demyelinating polyneuropathy, i.e., Guillain-Barré syndrome. Prominent axonal involvement was also present in the motor nerves of the second patient. Insulin therapy had to be permanently continued and these two cases are quite different from the transient diabetes sometimes observed in certain cases of Guillain-Barré syndrome. Both the latter and insulin-dependent diabetes mellitus probably have auto-immune mechanisms. It is likely that in our two patients both auto-immune diseases were triggered by a common event. Such cases of Guillain-Barré syndrome have to be distinguished from other acute diabetic neuropathies.     

Three patients with ophthalmoplegia associated with Campylobacter jejuni

Cranial polyneuropathy is idiopathic in most patients. Idiopathic cranial polyneuropathy is an acute postinfectious syndrome, along with Guillain-Barré syndrome and Miller Fisher syndrome, in which the common preceding pathogen is Campylobacter jejuni. Serum anti-GQ1b antibodies are elevated in Miller Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia. Three patients with idiopathic cranial polyneuropathy with predominant ocular involvement are presented. C. jejuni isolated from stool specimens belonged to Penner serotypes O:4, O:23, and O:33. Serum anti-GQ1b antibodies were elevated in all patients but demonstrated rapid reduction concomitant with clinical recovery. All patients recovered completely. Because both preceding C. jejuni infection and elevated anti-GQ1b antibodies decreasing with time were seen in all patients, the pathogenesis of idiopathic cranial polyneuropathy with ophthalmoplegia may be similar to that of Miller Fisher syndrome.     

Hereditary neuropathy with liability to pressure palsy presenting with an acute inflammatory demyelinating polyneuropathy

INTRODUCTION: Hereditary neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant peripheral neuropathy characterized by compressive focal neuropathies and an underlying sensorimotor demyelinative polyneuropathy. It is usually caused by a 1.5 Mb deletion of the PMP22 gene (17p11.2). CASE REPORT: We describe the case of a 31 year-old woman who presented with acute demyelinative peripheral polyneuropathy affecting the four limbs and elevated cerebrospinal fluid protein content a few days after a viral illness. Acute inflammatory demyelinating polyneuropathy (AIDP, Guillain-Barré syndrome) was suspected. However, electrophysiologic examination suggested HNPP and subsequent genetic testing was confirmatory. CONCLUSION: This case demonstrates that HNPP can present in an acute manner, mimicking AIDP.     

Elevated serum levels of endothelial leukocyte adhesion molecules in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy

We determined the serum concentrations of the soluble form of endothelial leukocyte adhesion molecule-1 (sELAM-1) in 25 patients with Guillain-Barré syndrome, 14 patients with chronic inflammatory demyelinating polyneuropathy, 15 patients with amyotrophic lateral sclerosis, 15 patients with other polyneuropathies, 10 patients with acute stroke, and 12 healthy control subjects. The patients with Guillain-Barré syndrome in the acute stage had a higher sELAM-1 serum level compared with the control subjects (p < 0.001). In 7 of the 9 patients the elevated level returned to normal by 14 days after the onset of neurological symptoms. In addition, a significantly elevated serum level of sELAM-1 was found in patients with chronic inflammatory demyelinating polyneuropathy (p < 0.01). These findings suggest a role for ELAM-1 in the inflammatory process of these disorders.     

Relapsing polyneuropathy in the post-partum period

A patient had five relapses of polyneuropathy: four developed during post-partum. The rapid onset of symptoms with subsequent complete recovery are in favor of a recurrent Guillain-Barré syndrome rather than a chronic relapsing inflammatory polyneuropathy.     

A case of neuropathy mimicking Guillain-Barré syndrome after arsenic intoxication

INTRODUCTION: Chronic arsenic toxicity is a global health problem affecting millions of people. Acute arsenic poisoning is less frequent and it is most often lethal. Therefore, its consequences are not well known, more precisely its neurological consequences. OBSERVATION: We report a case of Guillain-Barré-like syndrome and encephalopathy after acute arsenical poisoning in a 50 year-old man. After 4 month follow-up, the improvement was slow and limited with persistent motor and proprioceptive deficits. DISCUSSION: The most frequent neurological complication induced by acute arsenical poisoning is a distal, symmetrical, sensory, axonal polyneuropathy. Yet the clinical course and the electrophysiological findings may also suggest a Guillain-Barré like syndrome. Moreover, the chelating is not very effective on the neurological complications. CONCLUSION: Any discrepancies in the clinical course of a Guillain-Barré syndrome shall lead to reconsider the diagnosis. The association of gastro-intestinal disorders, skin lesions, and encephalopathy and mood disorders leads to discuss intoxication with heavy metal and more precisely with arsenic. Moreover, the chelating is not very effective on the neurological complications.     

A case of Guillain-Barré syndrome treated with intravenous high doses of immunoglobulins

A case of severe Guillain-Barré syndrome unresponsive to plasma exchange was treated with high-dose intravenous immunoglobulins. The same therapy has been already successfully used in cases of chronic polyneuropathy. Immunoglobulin administration was scheduled as follows: a pulse dose (400 mg/kg/day) for three consecutive days, followed by maintenance dose (400 mg/kg every 15 days) until achievement of sustained clinical improvement. We observed a time-related response between immunoglobulin administration and clinical improvement. To our knowledge this is the first case of Guillain-Barré syndrome treated with high-dose intravenous immunoglobulins. Further studies are obviously necessary to confirm the effectiveness of this treatment in Guillain-Barré syndrome.     

Treatment of acute post-infective polyneuropathy by means of plasma exchange

15 patients with acute post-infective polyneuropathy of the Guillain-Barré type have been treated by means of plasma exchange. The clinical course and outcome of these patients is compared to that in a retrospectively matched control series who were treated with supportive therapy only. All patients had severe rapidly evolving muscle weakness and approximately half the patients in each group required ventilatory assistance. Both the duration of muscle weakness and the hospitalisation time was significantly shorter in the patients treated by means of plasma exchange. These results suggest that plasma exchange is of significant benefit in the treatment of patients with the Guillain-Barré syndrome.     

Epidemiology of childhood Guillain-Barré syndrome as a cause of acute flaccid paralysis in Honduras: 1989-1999

The objective of this study was to investigate the incidence of acute flaccid paralysis in the pediatric population of Honduras over an 11-year period, determine what percentage of acute flaccid paralysis was Guillain-Barré syndrome, and identify the epidemiologic features of Guillain-Barré syndrome. There were 546 childhood cases of acute flaccid paralysis seen between January 1989 and December 1999 at the Hospital Escuela Materno-Infantil in Tegucigalpa, Honduras. Of these cases with acute flaccid paralysis, 394 (72.2%) were diagnosed with Guillain-Barré syndrome. Our incidence of Guillain-Barré syndrome in the Honduran pediatric population (1.37/100,000 per year) is higher than that shown in other studies. There was a significantly higher incidence of Guillain-Barré syndrome in younger children (ages 1-4 years), a significant preponderance of cases from rural areas, and a mild predominance in boys but a typical clinical presentation. The Honduran pediatric Guillain-Barré syndrome population had an increased mortality rate. Guillain-Barré syndrome has become the leading cause of childhood paralysis in Honduras. A better understanding of the population at highest risk and opportunities for earlier intervention with more effective therapeutic modalities may permit reducing the mortality among Honduran children who develop Guillain-Barré syndrome.     

Immune attack on the schwann cell surface in acute inflammatory demyelinating polyneuropathy

The localization, mode of action, and roles of complement in the Guillain-Barré syndrome have been controversial. We used high-resolution immunocytochemistry to localize complement activation products in early stages of the acute inflammatory demyelinating polyneuropathy (AIDP) pattern of Guillain-Barré syndrome. Three AIDP subjects who were autopsied had had symptoms for 3 to 9 days at the time of death. Immunocytochemistry was performed on etched, epoxy resin-embedded sections, and the next thin section was compared by electron microscopy (thick/thin sections). Many fibers had a rim of the complement activation marker C3d and the terminal complement complex neoantigen C5b-9 along the outer surface of the Schwann cells. Ultrastructural analysis of these C3d-positive fibers showed mild vesicular changes of the outermost myelin lamellae. Vesicular degeneration was seen before the invasion of macrophages into the myelin, and was the predominant change in the subject with symptoms for 3 days. C3d staining was not found on myelin membranes. The results suggest that at least some forms of AIDP are complement mediated. We speculate that complement is activated by antibody bound to epitopes on the outer surface of the Schwann cell and that the resulting complement activation initiates the vesiculation of myelin.     

Plasmapheresis with acute inflammatory polyneuropathy

Eleven children with acute inflammatory polyneuropathy were treated with a short course of intensive plasmapheresis. The 5 males and 6 females ranged in aged from 19 months to 16 years (mean: 7.8 years). The interval from disease onset to the initiation of plasmapheresis therapy was less than 7 days in 5 patients and less than 2 weeks in the others. At the time of the first plasmapheresis, 3 patients were on respirators (Grade 5 on the Guillain-Barré syndrome scale 0-6); 7 were bedridden (Grade 4); and 1 required assisted ambulation (Grade 3). One week after the last plasmapheresis, all but 1 patient had improved by 1 or more grades on the Guillain-Barré syndrome scale. At subsequent examination 6 months later, all patients were ambulatory and 9 of 11 had no significant neurologic findings. Electrophysiologic studies performed shortly before treatment initiation revealed predominant demyelinating neuropathy in 9 and axonal changes in 2. During the 76 plasmapheresis procedures, no severe complications were encountered. Although the number of patients treated is small, the clinical response observed would indicate plasmapheresis to be a safe and effective therapy in children with acute inflammatory polyneuropathy.     

Association of acute inflammatory demyelinating polyneuropathy with acute lymphoblastic leukaemia and HLA-A11

Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome) developed in three patients receiving chemotherapy for acute lymphoblastic leukaemia or the closely related entity lymphoblastic lymphoma, a relationship that has not been previously described. The HLA-A11 antigen was expressed by all patients, two of whom were Chinese, raising the possibility of a genetically-based immunological predisposition to GBS in this patient group.     

Guillain-Barré syndrome during treatment with interferon alpha for hepatitis B.

We describe a patient who developed acute demyelinating polyneuropathy on the sixth week of interferon (IFN)alpha therapy for chronic hepatitis B (HBV) infection. A 23-year-old man with chronic HBV infection had acute onset of demyelinating polyneuropathy shortly after completing a standard 6-week course of therapy with IFNalpha 2a. Clinical findings, electrodiagnostic studies and elevated cerebrospinal fluid protein levels without cells supported the diagnosis of Guillain-Barré syndrome (GBS). Other potential causes of GBS were ruled out. It remains unknown whether IFNalpha or the HBV infection itself was the cause of GBS, but it is evident that IFNalpha could not have prevented the development of GBS in our patient. We suggest that coexistent HBV infection and IFNalpha therapy may play a role in triggering an autoimmune response to peripheral nerve myelin.     

Acute axonal polyneuropathy associated with anti-GM1 antibodies following Campylobacter enteritis

We report 2 patients with Guillain-Barré 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.     

A Guillain-Barré syndrome variant with prominent facial diplegia

To determine the clinical features of a Guillain-Barré syndrome variant with prominent facial diplegia, we retrospectively reviewed approximately 8,600 cases referred to our neuroimmunological laboratory for serological tests during the past seven years. Patients’ histories, neurological signs, and laboratory and electrophysiological data were clarified based on their clinical records. Sera obtained during the acute phase were tested for prior infectious serology and anti-ganglioside antibodies. In 22 patients, clinical signs such as acute progressive bifacial weakness, paresthesias in the distal dominant limbs, and hypo- or areflexia, were compatible with a Guillain-Barré syndrome variant, facial diplegia and paresthesias. Other cranial nerve involvements, limb weakness, and ataxia were absent or minimal. Clinical courses were monophasic, the nadir being reached within four weeks. Eighteen patients (86%) had had infectious symptoms within the four weeks preceding the onset of neurological illness. In the infection serology tests, anti-cytomegalovirus IgM antibodies were the most frequent (35%). All the patients had cerebrospinal fluid albuminocytologic dissociation. In nerve conduction studies, 14 (64%) showed demyelination in their limbs. Anti-GM2 IgM antibodies were detected in four patients who had anti-cytomegalovirus IgM antibodies. Patients with conditions similar to facial diplegia and paresthesias, but lacking either distal paresthesias or hyporeflexia, were regarded as having marginal facial diplegia and paresthesias, because they also frequently had features of Guillain-Barré syndrome, such as an antecedent infection or cerebrospinal fluid albuminocytologic dissociation. Our findings are further evidence of a facial variant of Guillain-Barré syndrome and provide important information essential for its diagnosis.     

A unique case of Miller Fisher-Guillain-Barré overlap syndrome in a liver transplant recipient

Journal of NeuroVirology - Guillain-Barré syndrome (GBS) is an ascending demyelinating polyneuropathy often associated with recent infection. Miller Fisher syndrome represents a variant with...     

Encephalomyeloneuritis simulating Guillain-Barré syndrome

A patient with encephalomyeloneuritis (EMN) had clinical and laboratory features consistent with severe acute inflammatory polyneuropathy (Guillain-Barré syndrome). CNS involvement was suggested clinically only by transient downbeat nystagmus and by contraction of tensor fascia lata on plantar stimulation. Postmortem examination revealed pathologic changes typical of EMN without systemic neoplasm. Clinical manifestations of the profound central pathology were largely obscured by severe radiculoneuropathy. Pathologic verification of clinically diagnosed inflammatory polyneuropathy is unusual, and CNS disease, therefore, may be more frequent than appreciated, especially in clinical "variants."     

A variant of multifocal motor neuropathy with acute, generalised presentation and persistent conduction blocks

OBJECTIVE: Multifocal motor neuropathy with persistent conduction blocks is classically described as a chronic neuropathy with progressive onset, and acute forms have not previously been characterised. We report four cases of severe motor impairment with acute and generalised onset and with persistent motor conduction blocks. PATIENTS AND RESULTS: An acute tetraparesis with diffuse areflexia but little or no sensory disturbance was the clinical picture. Serial electrophysiological tests showed persistent multifocal motor conduction blocks with absent F waves in most tested motor nerves. No or minor abnormalities of the sensory nerve action potentials were observed. Cerebrospinal fluid contained normal or mildly increased protein levels (<1 g/l) without cells. Campylobacter jejuni serology was negative in three patients and consistent with past infection in one patient. Anti-ganglioside antibodies were positive in three patients. A five day course of intravenous immunoglobulins produced nearly complete symptom resolution in three patients and was ineffective in one patient. CONCLUSION: Because of the persistence of multifocal motor conduction blocks for several weeks or months as the isolated electrophysiological feature, these cases could not be consistent with Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy. They suggest an original variant of multifocal motor neuropathy with an acute and generalised initial presentation and persistent motor conduction blocks affecting all four limbs.     

Autoantibodies to GM1b and GalNAc-GD1a: relationship to Campylobacter jejuni infection and acute motor axonal neuropathy in China.

IgG antibodies to the minor gangliosides GM1b and GalNAc-GD1a frequently are present in sera of Japanese patients with Guillain-Barré syndrome. The relationship between these autoantibodies and Campylobacter jejuni infection, the type of disease (acute motor axonal neuropathy [AMAN], or acute inflammatory demyelinating polyneuropathy [AIDP]) has yet to be established. Sera samples were obtained from 55 Chinese patients with clinically defined Guillain-Barré syndrome. An electrophysiology study showed nine AIDP, 28 had AMAN, and 18 unclassified. C. jejuni serology was positively correlated with anti-GM1b and anti-GalNAc-GD1a IgG antibodies (respective P values, 0.007 and 0.02). The frequencies of positive anti-GM1b and anti-GalNAc-GD1a serology were greater in AMAN (32 and 21%) than in AIDP (11 and 0%), but the differences were not significant. Infection by C. jejuni may induce IgG anti-GM1b antibody in some patients and IgG anti-GalNAc-GD1a antibody in others. A larger population of patients must be studied to show whether there is a definite correlation.     

Acute or subacute alcoholic neuropathy mimicking Guillain-Barré syndrome

Over the last 10 years we have encountered 8 patients with chronic alcoholism who presented with severe symmetrical polyneuropathy, primarily proximal in 6, which evolved over a period of 24 h to 3 weeks. In 3 cases, artificial ventilation was required. Sensory symptoms were in all instances intense, and tendon reflexes absent. CSF protein levels were normal. The course was one of gradual improvement, often incomplete with residual motor and distal sensory deficits. Three patients died within 2 months to 2 years with multiple and severe pathologies attributable to chronic alcoholism. There was no evidence for disorders other than the alcoholism and malnutrition. Electrophysiological findings were consistent with predominantly axonal lesions and nerve biopsy specimens confirmed acute and severe axonal lesions. Several of these patients had been referred to us with a possible diagnosis of Guillain-Barré syndrome due to the severity of the neuropathy and the rapidity of its onset (Landry syndrome). Acute alcoholic neuropathy is distinguishable, however, on clinical, electrophysiological and morphological grounds.