Bilateral leg pain and dysarthria: The heterogenic clinical presentation of Guillian-Barré-Syndrome

Introduction

Guillain-Barré syndrome (GBS) is a rare disease of an acute or subacute immune-mediated polyradiconeuritis with multifocal demyelination and/or axonal damage (Hughes RA et al., Lancet 2005; 366: 1653–66). Among seven subtypes of GBS, the Acute Inflammatory Demyelinating Polyneuropathy (AIDP) is the most common type in children and in Europe (Korinthenberg R et al., J Peripher Nerv Syst 2020; 25: 344–349). Up to date, the pathomechanism of GBS is not fully understood yet. One hypothesis is that by molecular mimicry of surface molecules of pathogenic germs, autoantibodies are formed against myelin and axon components, causing a multifocal inflammatory response after a previous history of a Campylobacter jejuni infection (Heikema, A. P. et al., Clin Microbiol Infect 2015; 21: 852.e1–9). Classical symptoms of GBS are bilateral and flaccid weakness beginning in the lower extremities and decreased or absent muscle reflexes of the weak limbs and and the rapid course with respiratory failure (Van der Meché FG et al., Eur Neurol. 2001; 45:133–9). The diagnosis of GBS is not always straightforward because the extent of the disease and the rate of progression are highly variable (van den Berg B et al., Nat Rev Neurol. 2014: 8:469–82). The quality criteria of the Brighton Collaboration GBS Working Group are helpful and clinically proven. They provide five levels of diagnostic certainty for GBS, addressing the different presentation of the syndrome on the one hand, and the different levels of the diagnostic possibilities on the other hand (Sejvar, J et al., Vaccine 2011; 29: 599–612). [Table 1] summarizes the criteria for GBS. For the highest level of diagnostic certainty, a suitable clinical history and presentation, a detection of an increase in cerebrospinal fluid protein with a slightly increased cell count (=‘cyto-albuminous dissociation’), electrodiagnostic signs of neuropathy and the exclusion of alternative causes are required ([Table 2]) (ibid). We would like to report about a patient with a presentation of symptoms initially shadowed as an unspecific pain syndrome after a slump of a cognitively impaired child to remind physicians that GBS also in children is not just a motor disorder but that it frequently has a strong sensory and cranial nerve involvement.

Table 1 Diagnostic criteria for Guillain-Barré-Syndrome (GBS) according to Sejvar et al. (2011) and Korinthenberg et al. (2020).

Level 1 of diagnostic certainty
Bilateral and flaccid weakness of the limbs AND decreased or absent tendon reflexes of the weak limbs AND monophasic illness pattern and interval between onset and nadir 12 hours to 28 days and subsequent clinical plateau AND electrophysiological findings consistent with GBS AND cytoalbuminologic dissociation (elevation of CSF protein level above laboratory normal value and CSF total white cell count <50 cells/μL) AND absence of an identified alternative diagnosis for weakness
Level 2 of diagnostic certainty
All of the abovementioned clinical criteria present AND CSF total white cell count <50 cells/µl (with or without cerebrospinal fluid protein elevation above laboratory normal value) OR if CSF not collected or results not available electrophysiological studies consistent with GBS AND absence of an identified alternative diagnosis for weakness
Level 3 of diagnostic certainty
All of the abovementioned clinical criteria present AND absence of an identified alternative diagnosis for weakness
Level 4 of diagnostic certainty
Cases reported as GBS, but data not sufficient for level 3 AND absence of an identified alternative diagnosis for weakness
Level 5 of diagnostic certainty
GBS excluded due to an alternative diagnosis

Table 2 Exclusionary criteria for a diagnosis of Guillain–Barré-syndrome modified according to Sejvar et al. (2011).

Intracranial Meningeosis carcinomatosa Brain stem encephalitis Spinal cord Infarct, myelitis, compression Anterior horn cells of the spinal cord Polio and other enteroviruses that can cause poliomyelitis (incl. West Nile Virus) Spinal nerve roots Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Cauda equina compression Peripheral nerves Metabolic disorders such as hypermagnesemia or hypophosphatemia. Axonal subacute recurrent neuropathy with elevated CSF lactate in PDHcIα-mutation Tick paralysis Heavy metal toxicity such as arsenic, gold and thallium Drug-induced neuropathy (e. g., vincristine, platinum compounds, nitrofurantoin, paclitaxel) Porphyria Critical illness neuropathy Vasculitis Diphtheria Neuromuscular junction Myasthenia gravis Organophosphate poisoning Botulism Muscle Critical illness myopathy Polymyositis Dermatomyositis Hypo-/hyperkalemia Periodic paralysis

Publication History

Article published online:
08 November 2022