Objective
Respiratory insufficiency is a frequent and serious complication of the Guillain‐Barré syndrome (GBS). We aimed to develop a simple but accurate model to predict the chance of respiratory ...insufficiency in the acute stage of the disease based on clinical characteristics available at hospital admission.
Methods
Mechanical ventilation (MV) in the first week of admission was used as an indicator of acute stage respiratory insufficiency. Prospectively collected data from a derivation cohort of 397 GBS patients were used to identify predictors of MV. A multivariate logistic regression model was validated in a separate cohort of 191 GBS patients. Model performance criteria comprised discrimination (area under receiver operating curve AUC) and calibration (graphically). A scoring system for clinical practice was constructed from the regression coefficients of the model in the combined cohorts.
Results
In the derivation cohort, 22% needed MV in the first week of admission. Days between onset of weakness and admission, Medical Research Council sum score, and presence of facial and/or bulbar weakness were the main predictors of MV. The prognostic model had a good discriminative ability (AUC, 0.84). In the validation cohort, 14% needed MV in the first week of admission, and both calibration and discriminative ability of the model were good (AUC, 0.82). The scoring system ranged from 0 to 7, with corresponding chances of respiratory insufficiency from 1 to 91%.
Interpretation
This model accurately predicts development of respiratory insufficiency within 1 week in patients with GBS, using clinical characteristics available at admission. After further validation, the model may assist in clinical decision making, for example, on patient transfer to an intensive care unit. ANN NEUROL 2010;67:781–787
Mycoplasma pneumoniae (Mp) triggers Guillain-Barré syndrome (GBS) and elicits anti-galactocerebroside (GalC) antibodies. Specifically anti-GalC IgG is associated with Mp-GBS, possibly because of its ...better ability to cross the blood-nerve barrier (BNB). We here investigated CSF for the presence of anti-GalC in GBS. Intrathecal anti-GalC was found in 46% of Mp-GBS patients (n=6/13), in contrast to 16% of GBS controls (n=4/25) and 0% of non-GBS controls (n=0/7). The antibodies most likely originated from increased BNB permeability and/or intrathecal synthesis. Intrathecal anti-GalC IgG was specifically associated with Mp-GBS, further supporting that anti-GalC IgG contributes to the pathogenesis of GBS.
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•Although GBS is a peripheral neuropathy anti-GalC antibodies are found in CSF.•Intrathecal anti-GalC IgG is specifically associated with M. pneumoniae-GBS.•Anti-GalC IgG in CSF may be derived from blood and related to increased blood-nerve barrier permeability.•In one patient with additional CNS involvement, anti-GalC IgG most likely derived from intrathecal synthesis.
Background:
Current diagnostic methods for nerve compression headaches consist of diagnostic nerve blocks. A less-invasive method that can possibly aid in the diagnosis is ultrasound, by measuring ...the cross-sectional area (CSA) of the affected nerve. However, this technique has not been validated, and articles evaluating CSA measurements in the asymptomatic population are missing in the current literature. Therefore, the aim of this study was to determine the feasibility of ultrasound measurements of peripheral extracranial nerves in the head and neck area in asymptomatic individuals.
Methods:
The sensory nerves of the head and neck in healthy individuals were imaged by ultrasound. The CSA was measured at anatomical determined measurement sites for each nerve. To determine the feasibility of ultrasound measurements, the interrater reliability and the intrarater reliability were determined.
Results:
In total, 60 healthy volunteers were included. We were able to image the nerves at nine of 11 measurement sites. The mean CSA of the frontal nerves ranged between 0.80 ± 0.42 mm
2
and 1.20 ± 0.43 mm
2
, the mean CSA of the occipital nerves ranged between 2.90 ± 2.73 mm
2
and 3.40 ± 1.91 mm
2
, and the mean CSA of the temporal nerves ranged between 0.92 ± 0.26 mm
2
and 1.40 ± 1.11 mm
2
. The intrarater and interrater reliability of the CSA measurements was good (ICC: 0.75–0.78).
Conclusions:
Ultrasound is a feasible method to evaluate CSA measurements of peripheral extracranial nerves in the head and neck area. Further research should be done to evaluate the use of ultrasound as a diagnostic tool for nerve compression headache.
Guillain-Barré syndrome is an acute polyradiculoneuropathy with a variable clinical presentation. Accurate diagnostic criteria are essential for patient care and research, including clinical trials ...and vaccine safety studies. Several diagnostic criteria for Guillain-Barré syndrome have been proposed, including the recent set by the Brighton Collaboration. In the present study we describe in detail the key diagnostic features required to meet these Brighton criteria in a study population of 494 adult patients with Guillain-Barré syndrome, previously included in therapeutic and observational studies. The patients had a median age of 53 years (interquartile range 36-66 years) and males slightly predominated (56%). All patients developed bilateral limb weakness which generally involved both upper and lower extremities. The weakness remained restricted to the legs in 6% and to the arms in 1% of the patients. Decreased reflexes in paretic arms or legs were found initially in 91% of patients and in all patients during follow-up. Ten (2%) patients however showed persistence of normal reflexes in paretic arms. Disease nadir was reached within 2 weeks in 80%, within 4 weeks in 97% and within 6 weeks in all patients. A monophasic disease course occurred in 95% of patients, of whom 10% had a treatment-related fluctuation. A clinical deterioration after 8 weeks of onset of weakness occurred in 23 (5%) patients. Cerebrospinal fluid was examined in 474 (96%) patients. A mild pleocytosis (5 to 50 cells/μl) was found in 15%, and none had more than 50 cells/μl. An increased cerebrospinal fluid protein concentration was found only in 64% of patients, highly dependent on the timing of the lumbar puncture after onset of weakness (49% at the first day to 88% after 2 weeks). Nerve electrophysiology was compatible with the presence of a neuropathy in 99% of patients, but only 59% fulfilled the current criteria for a distinct subtype of Guillain-Barré syndrome. Patients with a complete data set (335) were classified according to the Brighton criteria, ranging from a high to a low level of diagnostic certainty, as level 1 in 61%, level 2 in 33%, level 3 in none, and level 4 in 6% of patients. Patients categorized in these levels did not differ with respect to proportion of patients with preceding events, initial clinical manifestations or outcome. The observed variability in the key diagnostic features of Guillain-Barré syndrome in the current cohort study, can be used to improve the sensitivity of the diagnostic criteria.
Objective
To investigate the association between diet quality and chronic axonal polyneuropathy.
Methods
Between June 2013 and January 2017, among 1650 participants of the Rotterdam Study (median age ...69.1 years, 54.2% women), diet quality was quantified based on food frequency questionnaires as a sum score of adherence (yes/no) to 14 components of the Dutch dietary guidelines. Presence of polyneuropathy was determined based on a questionnaire, neurological examination of the legs, and nerve conduction studies. We used logistic regression to associate diet quality with the presence of chronic axonal polyneuropathy and linear regression to associate with sural sensory nerve action potential (SNAP) amplitude in participants without polyneuropathy. Results were adjusted for age, sex, time between measurements, body mass index, blood pressure, diabetes mellitus, smoking, kidney function, and education.
Results
Overall diet quality was not associated with chronic axonal polyneuropathy (odds ratio OR = 0.99, 95% confidence interval CI 0.88; 1.12, P = 0.842), nor with sural SNAP amplitude in participants without polyneuropathy (difference = 0.01, 95% CI −0.14; 0.15, P = 0.993). Although not surviving multiple testing, a nominally significant association was found between salt intake ≤6 g/day and presence of chronic axonal polyneuropathy (OR = 0.55, 95% CI 0.35; 0.86, P = 0.008).
Interpretation
We did not find an association between diet quality and chronic axonal polyneuropathy.
Guillain-Barré syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop ...respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS. Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.
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