Ultrasound and electrophysiologic findings in patients with Guillain–Barré syndrome at disease onset and over a period of six months
Introduction
Diagnosis of Guillain–Barré syndrome (GBS) is based on typical history, clinical examination, and electrophysiological studies (Asbury and Cornblath, 1990, Fokke et al., 2014, Hadden et al., 1998, Hughes and Cornblath, 2005, van den Berg et al., 2014) while serologic findings usually are available later and serve for corroboration. Many variants of GBS exist, e.g. the Miller Fisher syndrome with ataxia, areflexia, and oculomotor dysfunction (Hughes and Cornblath, 2005). The most common types are the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and the acute motor axonal neuropathy (AMAN). Autonomic dysregulation (AD) is most dangerous in the early stage of the disease. Reduced heart rate reactions to Valsalva and deep breathing as well as blood pressure measurements are suitable methods to diagnose AD. However, no clear prognostic markers exist so far to predict AD.
The value of nerve ultrasonography (NUS) in immune-mediated polyneuropathies is well described (Beekman et al., 2005, Gallardo et al., 2015, Grimm et al., 2014a, Grimm et al., 2014b, Grimm et al., 2014c, Hobson-Webb and Cartwright, 2014, Kerasnoudis et al., 2013, Kerasnoudis et al., 2014a, Kerasnoudis et al., 2014b, Padua et al., 2012, Padua et al., 2014, Scheidl et al., 2012, Scheidl et al., 2014, Zaidman et al., 2009, Zaidman et al., 2013, Zaidman and Pestronk, 2014).
In post-GBS patients patchy and slight enlargements have been reported (Kerasnoudis et al., 2013, Zaidman et al., 2009, Zaidman et al., 2013), whereas in acute stage cervical spinal nerve enlargement and hypertrophy of the vagus are probably the most obvious findings (Gallardo et al., 2014, Grimm et al., 2014d). However, only little is known about the course of nerve morphology from onset until clinical recovery. Therefore, aim of this study is to describe the time course of NUS changes in correlation to nerve conduction studies (NCS) and clinical course.
Section snippets
Subjects
Between August 2013 and December 2014, all consecutive patients suffering from acute AIDP or AM(S)AN, presenting at participating hospitals were included in this study. Inclusion criterion was the diagnosis of acute onset polyneuropathy fulfilling the published diagnostic criteria for GBS (Asbury and Cornblath, 1990, Cornblath et al., 1988, Fokke et al., 2014, Hadden et al., 1998, Hughes and Cornblath, 2005, van den Berg et al., 2014). Exclusion criterion was symptom onset >three days before
Results
27 patients with GBS and 31 controls were included in the study. Baseline characteristics of the patients and the healthy controls are shown in Table 1. Some patients and controls of this study were already described in a former study conducted in the acute phase of GBS (Grimm et al., 2014d). 25 patients fulfilled the electrophysiological criteria for a demyelinating polyneuropathy (Hadden et al., 1998), two patients had signs of a severe axonal neuropathy with typical clinical onset of a GBS,
Discussion
The current study shows, that ultrasonic enlargement of the peripheral nerves, the cervical spinal nerves as well as the vagus occurs in the very early stage of GBS, most prominently in the spinal nerves and the vagus and may, therefore, serve as an early diagnostic indicator of GBS. However, while spinal nerve enlargement and vagus nerve hypertrophy regress consistently over six months, enlargement in many peripheral nerves persists even after six months – independently from clinical
Conclusion
Although enlargement of peripheral nerves may be less pronounced than in CIDP and no significant changes have been found over a follow-up period of half a year, the ultrasonic detection of cervical spinal nerves and vagus enlargement supports the diagnosis of GBS in the early phase. The regression of this enlargement is remarkable, but must be proven in patients with worse recovery to serve as candidate prognostic marker. Moreover, detection of vagus nerve hypertrophy in early GBS may be a
Conflict of interest
The authors report no competing interests.
References (37)
- et al.
Multifocal enlargement and increased vascularization of peripheral nerves detected by sonography in CIDP: a pilot study
Clin Neurophysiol
(2014) - et al.
Ultrasound of the peripheral nerves in systemic vasculitic neuropathies
J Neurol Sci
(2014) - et al.
Nerve ultrasound in CIDP: poly-parameters for polyneuropathies
Clin Neurophysiol
(2014) - et al.
Guillain–Barré syndrome
Lancet
(2005) - et al.
Controlled trial prednisolone in acute polyneuropathy
Lancet
(1978) - et al.
Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy
Clin Neurophysiol
(2014) - et al.
Increased cerebrospinal fluid protein and motor conduction studies as prognostic markers of outcome and nerve ultrasound changes in Guillain–Barré syndrome
J Neurol Sci
(2014) - et al.
Sarcoid neuropathy: correlation of nerve ultrasound, electrophysiological and clinical findings
J Neurol Sci
(2014) - et al.
Heterogeneity of root and nerve ultrasound pattern in CIDP patients
Clin Neurophysiol
(2014) - et al.
Ultrasonography of MADSAM neuropathy: focal nerve enlargements at sites of existing and resolved conduction blocks
Neuromuscul Disord
(2012)
Different patterns of nerve enlargement in polyneuropathy subtypes as detected by ultrasonography
Ultrasound Med Biol
Very early electrodiagnostic findings in Guillain–Barré syndrome
J Peripher Nerv Syst
Assessment of current diagnostic criteria for Guillain–Barré syndrome
Ann Neurol
The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis
Medicine (Baltimore)
Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy
Neurology
High-resolution ultrasonography of peripheral nerves: measurements on 14 nerve segments in 56 healthy subjects and reliability assessments
Ultraschall Med
Motor conduction studies in Guillain–Barré syndrome: description and prognostic value
Ann Neurol
Sural sparing pattern discriminates Guillain–Barré syndrome from its mimics
Muscle Nerve
Cited by (65)
The pathophysiological role of endoneurial inflammatory edema in early classical Guillain-Barré syndrome
2024, Clinical Neurology and NeurosurgeryAssessing the structural and functional changes in vagus nerve in multiple sclerosis
2022, Medical HypothesesPathophysiological and diagnostic aspects of Guillain-Barré syndrome
2022, Revue de Medecine InterneAxonal pathology in early stages of Guillain-Barré syndrome
2022, NeurologiaThe value of sensory nerve conduction studies in the diagnosis of Guillain–Barré syndrome
2021, Clinical NeurophysiologyCitation Excerpt :To this end, several biomarkers can be used including cerebrospinal fluid analysis, auto-antibody testing, and nerve conduction studies (NCS) (Wang et al., 2015). More recently, techniques such as peripheral nerve ultrasound and magnetic resonance neurography, showing enlarged nerves and enhancement of nerve roots respectively, have been utilized as non-invasive methods to support the diagnosis of GBS in the acute phases and to monitor regression of nerve injury in the chronic phases of the disease (Grimm et al., 2016; Lv et al., 2007). Since treatment is most effective when given earlier, prompt diagnosis of GBS is essential regardless of the subtype (Dimachkie and Barohn, 2013).
- 1
Contributed equally.