Diagnosis of Guillain‐Barré syndrome and validation of the Brighton criteria in Malaysia

We aimed to evaluate the key diagnostic features of Guillain‐Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated. A total of 128 patients (95 GBS, 33 MFS) were included. In the GBS cohort, 92 (97%) patients presented with symmetrical limb weakness. Reflexes were depressed or absent in 90 (95%) patients. Almost all patients (94, 99%) followed a monophasic disease course, with 5 (5%) patients experiencing treatment‐related fluctuations. Cerebrospinal fluid (CSF) albuminocytological dissociation was seen in 62/84 (73%) patients. Nerve conduction study (NCS) revealed neuropathy in 90/94 (96%) patients. In GBS patients with complete dataset (84), 56 (67%) patients reached level 1 of the Brighton criteria, 21 (25%) reached level 2, 3 (4%) reached level 3, and 4 (5%) reached level 4. In MFS, the clinical triad was present in 25 (76%) patients. All patients had a monophasic course. CSF albuminocytological dissociation was present in 10/25 (40%) patients. NCS was normal or showed sensory neuropathy in 25/33 (76%) patients. In MFS patients with complete dataset (25), 5 (20%) patients reached level 1 of the Brighton criteria, 14 (56%) reached level 2, 2 (8%) reached level 3, and 4 (16%) reached level 4. Inclusion of antiganglioside antibodies improved the sensitivity of the Brighton criteria in both cohorts. In the Malaysian cohort, the Brighton criteria showed a moderate to high sensitivity in reaching the highest diagnostic certainty of GBS, but the sensitivity was lower in MFS.     

Delayed facial weakness in Guillain‐Barré and miller fisher syndromes

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‐Barré 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     

Clinical and nerve conduction features in Guillain−Barré syndrome associated with Zika virus infection in Cúcuta,Colombia

Background and purpose

Zika virus (ZIKV) infection has been associated with an increased incidence of Guillain?Barré syndrome (GBS) but the relative frequency of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and axonal GBS subtypes is controversial.

Methods

Twenty GBS patients diagnosed according to the Brighton criteria during the ZIKV outbreak in Cúcuta, Colombia, were evaluated clinically and electrophysiologically. The electrodiagnosis of GBS subtypes was made according to a recently described criteria set that demonstrated a high diagnostic accuracy on the basis of a single test. The electrophysiological features of 34 Italian AIDP patients were used as control.

Results

All patients had symptoms compatible with ZIKV infection before the onset of GBS and ZIKV infection was laboratory confirmed through a plaque reduction neutralization test (PRNT₉₀) in 100% of patients. The median time from onset of ZIKV infection symptoms to GBS was 5 days (interquartile range 1–6 days). Cranial nerve palsy was present in 85% of patients (facial palsy in 75%, bulbar nerve involvement in 60%), autonomic dysfunction in 85%, and 50% of patients required invasive mechanical ventilation. AIDP was diagnosed in 70% of patients. 40% of nerves of AIDP patients showed a prevalent distal demyelinating involvement but this pattern was not different from the Italian AIDP patients without ZIKV infection.

Conclusions

Guillain?Barré syndrome associated with ZIKV infection in Cúcuta is characterized by a high frequency of cranial nerve involvement, autonomic dysfunction and requirement of mechanical ventilation indicating an aggressive and severe course. AIDP is the most frequent electrophysiological subtype. Demyelination is prevalent distally but this pattern is not specific for ZIKV infection.

     

Clinical and electrophysiological characteristics of Guillain‐Barré syndrome in Colombia

Despite the wide literature describing the features of Guillain‐Barré syndrome (GBS) in different populations worldwide, Colombian data are very scarce. We aim to characterize patients with GBS in a general hospital setting in Colombia. We conducted a retrospective chart review of GBS cases managed at the Hospital Universitario Fundación Santa Fe de Bogotá, from 2011 to 2016. Twenty‐three patients were included. The most commonly reported symptoms were paresthesias (65%), pain (61%), proximal (22%) and distal (74%) limb weakness, and facial palsy (30%). 9% of patients had Fisher syndrome and 21% had other variants: Bickerstaff, pharyngeal‐cervical‐brachial pattern, and facial diplegia. There was a predominance of the demyelinating form (70%), with only 22% of patients presenting with the axonal variants. Our results are concordant with previous studies in Colombia.     

Electrodiagnosis of reversible conduction failure in Guillain–Barré syndrome

Introduction: In this study we propose electrodiagnostic criteria for early reversible conduction failure (ERCF) in axonal Guillain–Barré syndrome (GBS) and apply them to a cohort of GBS patients. Methods: Serial nerve conduction studies (NCS) were retrospectively analyzed in 82 GBS patients from 3 centers. The criteria for the presence of ERCF in a nerve were: (i) a 50% increase in amplitude of distal compound muscle action potentials or sensory nerve action potentials; or (ii) resolution of proximal motor conduction block with an accompanying decrease in distal latencies or compound muscle action potential duration or increase in conduction velocities. Results: Of 82 patients from 3 centers, 37 (45%) had ERCF, 21 (26%) had a contrasting evolution pattern, and 8 (10%) had both. Sixteen patients did not show an amplitude increase of at least 50%. Conclusion: Our proposed criteria identified a group of patients with a characteristic evolution of NCS abnormality that is consistent with ERCF. Muscle Nerve 56 : 919–924, 2017     

Anti‐ganglioside antibodies in Guillain–Barré syndrome and chronic inflammatory demyelinating polyneuropathy in Chinese patients

Introduction: In this study we investigated the relationships between anti‐ganglioside antibodies and Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: Samples from 48 Chinese patients diagnosed with GBS and 18 patients diagnosed with CIDP were retrospectively reviewed. Results: In the GBS patients, 62.5% were classified as having acute inflammatory demyelinating polyneuropathy (AIDP), 27.1% were found to have acute motor axonal neuropathy (AMAN), and 10.4% were unclassified. Serum IgG anti‐ganglioside antibodies were detected in 46.2% of the AMAN patients and in 6.7% of the AIDP patients (P < 0.05); 5.6% of the 18 CIDP patients were IgG antibody positive, and 27.8% were IgM antibody positive. Facial palsy and sensory impairment were significantly associated with IgM antibodies. Conclusions: These results suggest that IgG anti‐GM1 antibodies are associated with AMAN, but not with AIDP, and that IgM antibodies against GM1, GM2, and GM3 are associated with facial nerve palsy. Muscle Nerve 55 : 470–475, 2017     

Electrophysiological subtypes and prognosis of Guillain–Barré syndrome in Northeastern China

Introduction: The dystrophic features in hindlimb skeletal muscles of female mdx mice are unclear. Methods: We analyzed force‐generating capacity and force decline after lengthening contraction‐induced damage (fragility). Results: Young (6‐month‐old) female mdx mice displayed reduced force‐generating capacity (?18%) and higher fragility (23% force decline) compared with female age‐matched wild‐type mice. These 2 dystrophic features were less accentuated in young female than in young male mdx mice (?32% and 42% force drop). With advancing age, force‐generating capacity decreased and fragility increased in old (20 month) female mdx mice (?21% and 57% force decline), but they were unchanged in old male mdx mice. Moreover, estradiol treatment had no effect in old female mdx mice. Conclusions: Female gender–related factors mitigate dystrophic features in young but not old mdx mice. Further studies are warranted to identify the beneficial gender‐related factor in dystrophic muscle. Muscle Nerve, 2013     

Clinical features of patients with Guillain‐Barré syndrome at seven hospitals on the East Coast of Australia

To document the clinical features of Guillain‐Barré syndrome (GBS) in Australia, we performed a retrospective analysis of all patients admitted to several hospitals along the East Coast of Australia from 2000 to 2012. Using hospital records, we reviewed all patients with a diagnosis of GBS admitted to seven hospitals. From these, we report information of subjects who fulfilled standard diagnostic criteria. We excluded patients where inadequate information was available or who were under the age of 18. We report the features of 335 patients, in 228 of whom neurophysiological data were available. There were 168 cases of acute inflammatory demyelinating polyneuropathy (AIDP), 17 of acute motor axonal neuropathy (AMAN), 4 of acute motor and sensory axonal neuropathy (AMSAN), and 35 of Miller‐Fisher syndrome (MFS). The median age at onset was 52.5 years (18–89 years) with a male : female ratio of 1.61 : 1. Upper respiratory tract infections were the most frequently identified trigger (151 subjects, 44.5%). Most patients were severely affected, with 42.7% of subjects bedbound, and an additional 24% requiring ventilatory support. GBS affects adults of all ages and usually follows a severe clinical course. In contrast to other autoimmune diseases, males are more frequently affected. A wide variety of triggering factors leads to a relatively stereotypical clinical syndrome. The most common variant of GBS in Australia is AIDP. This study shows that the clinical features of GBS in Australia are similar to that previously reported and confirms the male predominance, increased incidence with age, and frequent evidence of peripheral nerve demyelination as features of GBS.     

Ultrasonography of the peripheral nervous system in the early stage of Guillain‐Barré syndrome

Ultrasonography can be used to visualize peripheral nerve abnormalities in immune‐mediated neuropathies. The objective of this study was to prove the role of ultrasonography (US) in acute phase of Guillain‐Barré syndrome (GBS). Systematic ultrasonic measurements of several peripheral nerves including the vagal nerve as well as the sixth cervical nerve root were performed in 18 patients with GBS at days 1–3 after symptom onset and compared to 21 healthy controls. Nerve conduction studies (NCS) of corresponding nerves were undertaken. Consequently, significant differences between the groups were found in compound muscle action potential amplitudes, F‐wave latency, and persistency. Ultrasonic cross‐sectional areas (CSAs) showed significant enlargement in all nerves except of the ulnar nerve (upper arm) and the sural nerve compared to healthy controls, most prominent in proximal and middle median nerve (p < 0.01). The vagal nerve also showed enlargement compared to controls (p < 0.05), which was most pronounced in patients with autonomic dysfunction compared to patients without (p < 0.05). C6 root diameter showed a significant correlation to the amount of cerebrospinal fluid (CSF)‐protein (Pearson correlation, p < 0.05). US shows nerve enlargement in several peripheral nerves including vagal nerve and C6 root in acute phase of GBS and could be an additional diagnostic tool for example, in GBS of atypical onset and autonomic dysfunction.     

Epidemiology of Guillain‐Barré syndrome in Finland 2004–2014

At total mean incidence of 0.84–1.1/100,000 the occurrence of Guillain‐Barré syndrome (GBS) is reported to be low in Finland compared to other Caucasian populations. However, a recent study from Southwestern Finland reported an incidence of 1.82/100,000 which is comparable to other Caucasian populations. We analyzed discharge data covering the years 2004 through 2014 on all neurological admissions in all Finnish university and central hospitals with a primary diagnosis of GBS. A total of 989 admissions due to GBS (917 individuals) were identified. The standardized (European population) annual incidence rate was 1.70/100,000 person‐years (95% confidence interval 1.60–1.81). GBS incidence had an increasing trend with age. The likelihood of GBS was higher among girls and adolescent women than boys and men of same age (male:female incidence rate ratio [IRR] 0.56), while in the older age groups (>19 years) the occurrence of GBS was higher among males than females (male:female IRR 1.59). The incidence of GBS remained stable during the study period. There was no seasonal variation in GBS admission frequencies (p = 0.28). No significant effect of the 2009–2010 H1N1 influenza or vaccination against it for GBS occurrence was observed. We suggest that GBS is as common, and has similar age‐distribution in Finland as in other European countries. Sex‐associated susceptibility for GBS appears to be different in children‐adolescents and adults.     

Guillain‐Barré syndrome associated with CASPR2 antibodies: two paediatric cases

The pathogenesis of Guillain‐Barré syndrome (GBS) is considered to be, at least in part, mediated by autoantibodies directed against neuronal antigens. Antibodies to contactin‐associated protein‐like 2 (CASPR2), part of the voltage‐gated potassium channel complex (VGKC‐complex), are associated with neurological disease predominantly affecting the peripheral nervous system but are not known to be associated with GBS. We report two cases of ganglioside antibody‐negative paediatric GBS associated with CASPR2 antibodies. Both patients made a complete clinical recovery. The tissue distribution and function of CASPR2 make it a biologically plausible autoimmune target in GBS and its clinical relevance in GBS should be determined in further studies.