Acute Motor Sensory Axonal Neuropathy Research Articles

The acute motor-sensory axonal neuropathy variant of Guillain-Barré syndrome after thoracic spine surgery

Guillain-Barré syndrome (GBS) is the eponym used to describe acute inflammatory polyradiculoneuropathies, which manifest with weakness and diminished reflexes. Although the classic form of GBS is considered to be an ascending demyelinating polyneuropathy, several variants have been described in the literature, including the Miller-Fisher syndrome, acute panautonomic neuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy (AMSAN). Few cases of postoperative GBS have been documented, particularly for the AMSAN variant. The authors describe the case of a patient who developed AMSAN after thoracic spine surgery and highlight the importance of investigating new-onset weakness in the postoperative period.

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.

Clinical features and prognosis with Guillain-Barré syndrome

Background:Guillain-Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterized by rapidly progressive, symmetric weakness and areflexia.Materials and Methods:We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 36 children diagnosed with GBS.Results:Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (n = 25), acute motor axonal neuropathy (AMAN) (n = 10) and acute motor-sensory axonal neuropathy (AMSAN) (n = 1). Twenty (55.5%) patients were males and 16 (44.5%) patients were females. The mean age of the 36 patients was 68.1 ± 45.01 months (range, 6–180 months). Five (13.8%) patients were younger than 2 years. The most common initial symptoms were limb weakness, which was documented in 34 (94.4%) patients. In our study, 18 patients (51.4%) showed albuminocytological dissociation (raised protein concentration without pleocytosis) on cerebrospinal fluid (CSF) examination. Three patients (8.3%) required mechanical ventilation therapy during hospitalization. Unfortunately, three (8.3%) patients died; one patient had AIDP and two patients had axonal involvement (one case was AMAN and another case was AMSAN). When we compared the cases of residual sequel/dead and cases of complete recovery for neural involvement type including AIDP, AMAN and AMSAN, we did not find a statistically significant difference between the groups (P > 0.05).Conclusion:Our findings showed that cases of GBS was not uncommon in children younger than 2 years of age, and CSF protein level might be found high in the first week of the disease in about one half of the patients, with a higher rate of morbidity and mortality in patients with axonal involvement than in those with AIDP.

Guillian–Barré syndrome in high tetraplegia following acute respiratory illness

A case report of a Guillain-Barré syndrome (GBS) variant presenting in a patient with a high cervical spinal cord injury (SCI). To illustrate a clinical presentation of GBS in an individual with chronic SCI. Vancouver General Hospital, Vancouver, BC, Canada. A 31-year-old man with chronic C2 AIS B (American Spinal Injury Association Impairment Scale) SCI and diaphragmatic pacing presented with respiratory failure with sepsis. His sepsis resolved with antibiotic therapy, but he continued to have autonomic instability and was unable to be weaned off his ventilator. Concurrently he developed flaccidity and facial diplegia. Investigations including nerve conduction studies and cerebrospinal fluid analysis prompted a diagnosis of acute motor-sensory axonal neuropathy, a variant of Guillian-Barré syndrome. Owing to ongoing autonomic instability, he was treated with intravenous immunoglobulin. His autonomic dysfunction resolved and he regained some facial muscle function, but 6 months post injury he remained dysphagic and required 24-h ventilator support. Careful neurological reassessment prompted the diagnosis of acute polyradiculoneuropathy following respiratory sepsis as the root cause of diaphragmatic pacer failure and autonomic instability.

Tumor necrosis factor–α polymorphisms and expression in Guillain–Barré syndrome

Tumor necrosis factor–α (TNF-α) polymorphisms with increased expression is associated with many autoimmune and inflammatory diseases. Possible role of TNF-α polymorphism in the pathogenesis of Guillain–Barré syndrome (GBS) largely remains unknown. We investigated polymorphisms in the promoter region of TNF-α gene and its expression in GBS patients and healthy controls. TNF-α (−308 G>A, −857 C>T, and −863 C>A) polymorphisms in 140 GBS patients and 206 healthy controls were studied using polymerase chain reaction–restriction fragment length polymorphism (PCR-RFLP) and allele specific-PCR. TNF-α level in serum by ELISA was determined in 60 patients and an equal number of controls. Prevalence of TNF-α −308 G > A polymorphic A allele was associated with increased risk of GBS ( p < 0.001; OR = 2.58, 95% CI = 1.61–4.14). Heterozygous genotype (G/A) had an association with acute motor axonal neuropathy ( p < 0.001; OR = 4.23, 95% CI = 2.00–8.95) and variant genotype A/A with both axonal subtypes, acute motor axonal neuropathy ( p = 0.015, OR = 7.00, 95% CI = 1.46–33.57) and acute motor sensory axonal neuropathy ( p = 0.017; OR = 7.73, 95% CI = 1.44–41.37). Variant genotype T/T of TNF-α −857 C>T polymorphism was also significantly associated with acute motor axonal neuropathy ( p = 0.034; OR = 3.93, 95% CI = 1.11–13.91). Patients with A and T alleles had higher TNF-α level in serum. TNF-α −308 G > A and −857 C>T (only T/T) polymorphisms with increased TNF-α level may predict susceptibility to axonal subtypes of GBS.

Acute Motor Axonal Neuropathy Associated with Pandemic H1N1 Influenza A Infection

BackgroundGuillain–Barre syndrome (GBS) is a well known entity that has many infectious agents reported as antecedent events. The spectrum of GBS includes acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and some other variants like Miller-Fisher syndrome (MFS).MethodsPatient with AMAN variant of GBS after severe bilateral pneumonia and ARDS due to the novel pandemic H1N1 influenza A virus is presented.Results28-year-old white female was admitted to our Intensive Care Unit during the influenza pandemic because of severe ARDS due to bilateral pneumonia. The course of the disease was complicated with the new onset tetraplegia due to the AMAN variant of GBS. Treatment with plasma exchange was conducted and the patient had satisfactory recovery.ConclusionWe report a case of AMAN variant of GBS associated with proven H1N1 influenza A infection. This virus has not been reported previously as the agent of antecedent infection that induced this disorder. Risk factors for other causes of ICU neuromuscular weakness are usually present in the ICU patients and should not be the reason for reluctance in active quest for GBS. Once the diagnosis of GBS is established or suspected the treatment with plasma exchange or intravenous immune globulin is indicated.

Severe pulmonary hypertension associated with the acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome

To evaluate pulmonary hypertension associated with acute motor sensory axonal neuropathy subtype of Guillain-Barré syndrome. Guillain-Barré syndrome consists of a group of autoimmune disorders that generally manifest as symmetric, progressive, ascending paralysis. There are five subtypes of Guillain-Barré syndrome, and autonomic involvement has been described in all subtypes, including cardiovascular, vasomotor, or pseudomotor dysfunction of both the sympathetic and parasympathetic systems. Case report. Tertiary care pediatric intensive care unit. Three-yr-old female patient. None. Serial measurements of pulmonary artery pressure. We report the case of a young girl with acute motor sensory axonal neuropathy who presented with severe cardiovascular collapse secondary to severe pulmonary hypertension. In this patient, multiple factors may have played a role in the development of pulmonary hypertension including autonomic dysfunction, hypoventilation, and immobility as a risk for thrombosis and pulmonary emboli. It is possible that many other individuals suffering from severe forms of Guillain-Barré syndrome, especially those with significant autonomic dysfunction, may actually have undiagnosed and therefore untreated pulmonary hypertension. Therefore, it is recommended that clinicians caring for critically ill children with Guillain-Barré syndrome have a high index of suspicion for pulmonary hypertension and consider echocardiography if there are clinical signs of this potentially fatal process.

Could Helicobacter pylori play an important role in axonal type of Guillain-Barré Syndrome pathogenesis?

In this case-control study, ELISA and Western blot with whole bacterial protein lysate were performed on serum and cerebrospinal fluid (CSF) of 15 controls and 15 patients. According to Griffin subtypes, 10 of our patients were in acute inflammatory demyelinating polyradiculoneuropathy (AIDP) group, 3 in acute motor axonal neuropathy (AMAN) group, and 2 in acute motor sensory axonal neuropathy (AMSAN) subtype. 86.6% of patients were positive for Helicobacter pylori ( H. pylori) IgG. Serum anti- H. pylori IgG of patients and controls were significantly different. CSF anti- H. pylori IgG was significantly higher in patients than controls. In patients, the titer of anti- H. pylori IgG in serum was significantly higher than CSF, which may indicate extra-neural antibody synthesis. CSF IgG titer was higher in patients having axonal pattern. Western blot was positive in CSF of 13 patients and negative in all controls. There was a correlation between the number of antibody types against H. pylori particles and the titer of anti- H. pylori IgG in CSF and serum. Also, antibody against cytotoxin associated protein (CagA) was associated with primary axonal pattern. The association between the presence of anti-CagA and primary axonal pattern, is in favor of the relation between axonal neuropathy and H. pylori infection.

Depressive Episode With Catatonic Features in a Case of Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS)

Three months previously, a 17-year-old girl had complained of right-hand side hemiparesis, and her brain magnetic resonance imaging (MRI) showed a signal change in the left temporoparietooccipital area. The 3243A>G mutation was found in mitochondrial DNA. She was diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) and was prescribed dichloroacetic acid to treat lactic acidosis. Her health improved. Two months later, she developed drowsiness and generalized weakness. A New lesion was not found on brain MRI, and electrodiagnostic findings were compatible with acute motor sensory axonal neuropathy. Her negative symptoms, such as depressed mood, loss of interest in activities, psychomotor retardation, and hypersomnia, were aggravated. She was prescribed antidepressants and psychostimulants by a psychiatrist after diagnosis of severe depression episode with catatonic features. One month later, her catatonic condition had improved with medication. Our experience shows that psychiatric diagnostic evaluation of abruptly regressed neurologic and clinical features is important, even in a patient with devastating underlying disease.

Evolving pattern of Guillain–Barre syndrome in a community hospital in Israel

To investigate the frequency of axonal Guillain-Barre syndrome (GBS) in our ward over 6 years (1999-2005). Clinical and electrophysiological findings of 40 patients admitted to neurology with abnormalities compatible with acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN) and acute inflammatory demyelinating polyneuropathy (AIDP) were reviewed. Electrophysiological findings showed that 25 (63%) patients had AIDP, nine (22%) AMAN and six (15%) AMSAN. There were significant differences in disease severity. Most axonal patients (87%) were hospitalized with moderate or severe symptoms (3-4 Hughes grade score) and progressed to severe grade (4-6) in comparison with AIDP patients (64% admitted with mild forms) (1-2 Hughes grade score) and progressed to severe in 44% of cases. Cranial nerve involvement was more frequent in AIDP (56%) in comparison with the axonal type (13%). Raised cerebrospinal fluid protein at the time of hospitalization was observed in 76% of demyelinating and 33% of axonal patients. Axonal GBS occurred more frequently in Israel compared with other Western countries.

Neurophysiological criteria in the diagnosis of different clinical types of Guillain–Barre syndrome

Background:The diagnostic yield of various neurophysiological criteria may vary in different subforms of Guillain–Barre syndrome (GBS), whose prevalence varies in different geographical areas.Aims:To evaluate the sensitivity of various neurophysiological criteria...

Acute motor sensory axonal neuropathy associated with Henoch-Schönlein purpura

A 41-year-old man was admitted with progressive tetraparesis with hypoesthesia. He also presented with purpura in both legs. After admission, joint pain, gastrointestinal tract bleeding, and renal dysfunction developed. A nerve conduction study revealed reduced amplitude of the motor and sensory nerve action potential, with normal conduction velocity. A skin biopsy showed leukocytoclastic vasculitis, indicating Henoch-Schönlein purpura (HSP). After administration of corticosteroids, the symptom completely disappeared. The present case is the first report in Japan of HSP associated clinically and electrophysiologically with confirmed acute motor sensory axonal neuropathy. Common pathogenesis might have a role for development for two distinct disorders.

Guillain-Barré syndrome and hemophagocytic lymphohistiocytosis in a patient with severe chronic active Epstein–Barr virus infection syndrome

Epstein–Barr virus (EBV) infection causes a wide range of neurologic and hematologic manifestations. We report a 72-year-old Japanese male patient with severe chronic active EBV infection syndrome (SCAEBV) who presented with Guillain-Barré syndrome (GBS) and developed hemophagocytic lymphohistiocytosis (HLH) several months after the onset of GBS. He showed acute onset of distal muscle weakness, ophthalmoplegia and bulbar palsy. Results of nerve conduction study revealed acute motor-sensory axonal neuropathy (AMSAN). His serum was positive for anti-LM1 IgG and anti-GM1b IgM. Titers of antibodies to EBV-related antigens indicated chronic reactivated EBV infection. Treatment with IVIg resolved the acute ophthalmoplegia, but there was no notable improvement in the AMSAN and bulbar palsy despite repeated. Finally, he developed refractory HLH resulting in a fatal outcome. In the present patient, it seems that SCAEBV was associated with the development of GBS and fatal HLH via parainfectious autoimmunity and direct infectious immune mechanisms, respectively.

Outcome of axonal and demyelinating forms of Guillain-Barré syndrome in children

Previous reports have suggested that outcome is worse in the axonal compared with the demyelinating form of Guillain-Barré syndrome (GBS). We performed a retrospective study of 23 children with electrophysiologically confirmed cases of predominant subtypes of GBS to investigate this issue. The patients were classified based on the electrodiagnostic features: Ten (44%) had acute inflammatory demyelinating polyradiculoneuropathy, eight (35%) had acute motor axonal neuropathy, and five (21%) had acute motor-sensory axonal neuropathy. All patients received a standard intravenous immunoglobulin therapy (0.4 g /kg /day for 5 consecutive days). In the acute phase of the disease, patients with the axonal forms of GBS were more disabled than were those with the demyelinating GBS, as measured by GBS scores. Mechanical ventilation was required in five (38%) patients in the axonal group compared with one (10%) patient in the demyelinating group. There was no significant difference at 6 months in GBS scores between demyelinating and axonal forms of GBS. All 20 survivors recovered completely by 12 months. After standard intravenous immunoglobulin therapy, children with axonal forms of GBS recover more slowly than those with the demyelinating form, but outcome at 12 months appears to be equally favorable in two groups.

Fine specificities of anti-LM1 IgG antibodies in Guillain-Barré syndrome

We investigated the prevalence of anti-LM1 IgG antibody and its fine specificity in Guillain-Barré syndrome (GBS). Anti-LM1 IgG and IgM antibodies from sera of 47 patients with GBS—19 with acute inflammatory demyelinating polyneuropathy (AIDP), 27 with acute motor axonal neuropathy (AMAN), and 1 with acute motor-sensory axonal neuropathy (AMSAN)—were tested. Anti-LM1 IgG antibody was detected in only one patient with AIDP, whereas it was present in seven with AMAN and in one with AMSAN. Sera from the eight IgG anti-LM1-positive patients with AMAN/AMSAN also had IgG activity against the gangliosides GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, or GQ1b. Anti-LM1 IgG antibodies from the AMAN/AMSAN patients cross-reacted with other gangliosides, whereas IgG antibody from the AIDP patient was monospecific against LM1. Anti-LM1 IgG antibody therefore, cannot be a marker of AIDP. In addition, whether monospecific anti-LM1 IgG antibody is associated with AIDP remains to be concluded. Larger studies are needed to verify whether monospecific anti-LM1 IgG antibody could be a marker of AIDP.

Polirradiculoneuropatías autoinmunes agudas

Landry Guillain Barr Sthrol Syndrome (LGBS) is an acute autoimmune monophasic and selflimiting polyradiculoneuropathy affecting patients aged 19 to 59 years (1.7/100,000/year) more than below 18 year of age (0.8/100,000/year). Diagnostic criteria require: I. Progressive motor weakness of more than one limb, and II) absent muscle stretch reflexes. Features that strongly support the diagnosis are: 1. Cease of progression of weakness by 4 weeks, relative symmetrical involvement of the limbs, presence of mild sensory symptoms or signs, involvement of cranial nerves VII (50%) more than IX, X, III, IV and VI, recovery within 2 to 3 weeks after progression stop, presence of autonomic dysfunction, and absent of fever at onset; 2. Cerebral spinal fluid elevation of protein after first week of symptoms and less than 10 mononuclear leukocytes/mm3 except HIV seropositive patients (<50 cells/mm3), and 3. Nerve conduction slowing (<60% of normal ) or block at some point during the illness (80%), and increased distal motor latency up to 3 times above normal and F wave latency. There are four well defined clinical, pathological, neurophysiological and serological subtypes. Acute inflamatory demyelinating polyradiculoneuropathy (90%) and Miller Fisher syndrome (5%) are primarily demyelinating. Acute motor axonal neuropathy and acute motor sensory axonal neuropathy are primarily axonal (5%). Treatment includes supportive care and immunotherapy with high doses of intravenous IgG and plasmapheresis.

Acute Motor-Sensory Axonal Neuropathy Associated with Active Systemic Lupus Erythematosus and Anticardiolipin Antibodies

Acute motor-sensory axonal neuropathy (AMSAN) is an axonal variant of Guillian-Barré syndrome (GBS) that presents with acute ascending quadriparesis. This has generally been described in association with Campylobacter jejuni infections or with anti-ganglioside antibodies. Known cases have shown a slow recovery and a poor prognosis. We report a case with clinical and electrophysiological evidence of AMSAN in association with active systemic lupus erythematosus (SLE) and anticardiolipin antibodies but not the other associations, with a rapid response to combination immunosuppressant and intravenous immunoglobulin (IVIg) therapy. The association between AMSAN and SLE has not been previously described. This case illustrates that early recognition and the utilization of electrophysiologic techniques may be beneficial in the diagnosis and management of GBS associated with SLE. Fulminant or rapidly progressive cases should be managed in specialized intensive care units. Combination therapy of immunosuppressants and IVIg may be beneficial in non-vasculitic axonal radiculo-neuropathies associated with SLE, resulting in good outcomes.

IMMUNOHISTOCHEMICAL LOCALIZATION OF CYTOKINES, C5B‐9 AND ICAM‐1 IN PERIPHERAL NERVE OF GUILLAIN‐BARRE SYNDROME

The spectrum of the Guillain‐Barre Syndrome (GBS) has recently been widened by the newly identified forms of acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN). An important question has been raised regarding the possibility for the axon to be a target in immune‐mediated damage. Although myelin breakdown is the characteristic feature of classic acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal degeneration may occasionally be observed in this form, especially in cases with explosive onset and severe clinical course. Immunohistochemical findings of five frozen sural nerves biopsies of patients with GBS (AIDP variant) tested with a panel of monoclonal antibodies raised against different molecules implicated in immune‐mediated processes have principally disclosed an immunoreactivity of tumor necrosis factor‐alpha (TNF‐alpha) on both Schwann cell membranes and in myelinated and unmyelinated axons. On the other hand, interleukin 1‐beta (IL1‐beta) labeled Schwann cells, endothelial cells and macrophages; interferon‐gamma (IFN‐gamma) was observed both in endothelial cells and lymphocytes. Membrane attack complex (C5b‐9) deposits were observed on Schwann cell membranes and finally intercellular adhesion molecule‐1 (ICAM‐1) was localized both on endothelial cells and macrophages. Our findings strongly suggest that TNF‐alpha is an important factor in the cascade of events leading to immune‐mediated demyelination and axonal damage in GBS.

Guillian Barré syndrome--recent advances.

Guillian Barré Syndrome (GBS) is an acquired disease of the peripheral nerves that is characterized clinically by rapidly progressing paralysis, areflexia, and albumino-cytological dissociation. It affects both genders, involves people of all ages, is reported worldwide, and in the post-polio era, it is the most common cause of an acute generalized paralysis. The clinical features are distinct and a history and an examination generally lead to a high suspicion of the diagnosis that can then be confirmed by supportive laboratory tests and electrodiagnostic studies. This review discusses the recent advances in understanding of the different variants of GBS such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and the Fisher syndrome. The clinical, electrodiagnostic criteria, immunopathogenesis, and management of GBS and its variants are discussed.

Immunohistochemical localization of cytokines, C5b-9 and ICAM-1 in peripheral nerve of Guillain-Barré syndrome.

The spectrum of the Guillain-Barré Syndrome (GBS) has recently been widened by the newly identified forms of acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN). An important question has been raised regarding the possibility for the axon to be a target in immune-mediated damage. Although myelin breakdown is the characteristic feature of classic acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal degeneration may occasionally be observed in this form, especially in cases with explosive onset and severe clinical course. Immunohistochemical findings of five frozen sural nerves biopsies of patients with GBS (AIDP variant) tested with a panel of monoclonal antibodies raised against different molecules implicated in immune-mediated processes have principally disclosed an immunoreactivity of tumor necrosis factor-alpha (TNF-alpha) on both Schwann cell membranes and in myelinated and unmyelinated axons. On the other hand, interleukin 1-beta (IL1-beta) labeled Schwann cells, endothelial cells and macrophages; interferon-gamma (IFN-gamma) was observed both in endothelial cells and lymphocytes. Membrane attack complex (C5b-9) deposits were observed on Schwann cell membranes and finally intercellular adhesion molecule-1 (ICAM-1) was localized both on endothelial cells and macrophages. Our findings strongly suggest that TNF-alpha is an important factor in the cascade of events leading to immune-mediated demyelination and axonal damage in GBS.