Flaccid Tetraparesis Research Articles

P-PN006. Guillain barré syndrome treatment related fluctuation (GBS-TRF) following meningococcus vaccination: A case report

Introduction . GBS is an autoimmune neurological disorder, characterized by acute limb weakness and areflexia. The pathogenesis of GBS is triggered mostly by infection process. However, the consequence of vaccination can cause GBS has been widely discussed. Some studies address the potential relationship of GBS to influenza vaccination, while other vaccination is rarely described. In this study, we reported a case of GBS-TRF after administration of meningococcus vaccination. Case presentation: A 64-year-old man presented with glove-stocking hypoesthesia followed by flaccid tetraparesis, which occurred two weeks after meningococcus vaccination. There were no history of any infectious diseases preceding his symptoms. He and his family were administered these vaccinations, as the prerequisite for going to Umrah. After coming back of his Umrah, he was diagnosed with GBS based on history, clinical features and test results. Neurological examination revealed absent of knee and ankle reflexes. Nerve conduction studies indicated the decreased of sensory nerve action potential, amplitudes and conduction velocity, also prolongation of distal latency. He had six times therapeutic plasma exchange (TPE) and the symptoms slightly resolved at discharge. One month after plasma exchange, he was readmitted due to dyspnea, worsening of his weakness and severe hyponatremia. He was transferred to intensive care unit with mechanical ventilation. He was diagnosed as GBS-TRF. The treatment was intravenous immunoglobulin (IVIG) with full dose of 400 mg/kg/day. Unfortunately, the patient developed some complications during intensive care. He did not survive, suffering from cardiac arrythmia and septic shock, after IVIG treatment. Conclusion . Meningococcus vaccination may cause GBS as an unexpected abnormal autoimmune response. It is important to note the possibility of neurological complications after vaccine administration.

Teaching Video NeuroImage: Reflex Facilitation in Lambert-Eaton Myasthenic Syndrome.

A 41-year-old woman presented with progressive fluctuating lower limb weakness, urinary incontinence, and dry eyes and mouth. Physical examination revealed proximal flaccid tetraparesis (Medical Research Council 4). The patellar reflex was absent, but with postexercise facilitation (video 1). Eyelid and eye movements were normal. Electroneuromyography revealed a presynaptic neuromuscular junction disorder (figure). Antibodies to anti-voltage-gated calcium channels were positive. Periodic cancer screening, including whole-body FDG-PET, was negative during 2 years of follow-up. These findings were consistent with nonparaneoplastic Lambert-Eaton myasthenic syndrome.1 The patient had a good response to IV immunoglobulin. The presence of ascending muscle weakness, autonomic dysfunction, and postexercise facilitation of reflexes should raise suspicion for the diagnosis.

Fatal imported case of Plasmodium mixed infection: cerebral malaria, thrombocytopenia and multi organ dysfunctions (MODs)

Background: Plasmodium falciparum accounts for nearly all severe malaria cases among European travellers, even though, in areas of endemicity, Plasmodium vivax seems to cause severe malaria to a degree which is comparable with P. falciparum. Furthermore, unlike countries at high risk, the incidence of mixed infections among imported cases is very low. We report the case of a man from non-endemic country with a mixed infection diagnosed after a two-year stay in Guinea. Case Presentation: A 43-year-old male Italian patient developed hyperpyrexia and chills the day after his return to Italy. Within 7 days from fever onset, he was found unresponsive with vomiting and seizures. On admission to Emergency Room, the patient was hypotonic with flaccid tetraparesis, with a GCS score of 7. On the basis of personal and working history and clinical features, after exclusion of viral encephalitis, acute pyogenic meningitis, tubercular meningitis and pontine infarct, cerebral malaria infection was suspected. Rapid diagnostic test and peripheral blood smear were positive for plasmodium mixed infection (P. falciparum, P. vivax, Plasmodium ovale). Over the first 30 hours from admission, thrombocyte count dropped, and the patient developed disseminated intravascular coagulation. Renal failure required renal replacement therapy. Death occurred after 48 hours of ICU admission. Conclusion: The fatal course of the severe malaria with related cerebral impairment, lung and kidney failure, disseminated intravascular coagulation, severe acidosis, circulatory collapse with refractory septic shock and subsequent multi organ failure was likely due to delayed medical presentation by the patient and the mixed plasmodium infection. Diagnosis could only be established nine days after onset of symptoms.

Features of Guillain — Barre syndrome associated with COVID-19 infection

In December 2019, an epidemic of coronavirus infection SARS-CoV-2 (Severe acute respiratory syndrome-related coronavirus 2) emerged in Wuhan (China). In February 2020, the World Health Organization assigned the official name to the infection caused by the new coronavirus SARS-CoV-2 — Coronavirus disease 2019 or COVID-19, which was accompanied by various neurological complications, including Guillain — Barre syndrome. The purpose — to study the features of Guillain — Barre syndrome (GBS) with a new COVID-19 infection. Results. Recently, various neurological complications of COVID-19 have been described: severe viral hemorrhagic encephalitis, toxic encephalopathy, acute demyelinating lesions, acute cerebral disorders blood circulation, etc. It is proposed to consider Guillain — Barre syndrome (GBS) a neurological complication of CO VID-19, since the main route of coronavirus infection is respiratory, and most patients with GBS describe a respiratory infection before the development of neurological symptoms. Typically, GBS develops 11-13 days after the onset of COVID-19. Recent studies have shown that GBS is one of the most common lesions of the peripheral nervous system in COVID-19. The clinical picture, as a rule, is represented by increasing flaccid tetraparesis, less often there is respiratory failure. In a pandemic, it is advisable to test patients with GBS for COVID-19 if there are no respiratory disorders, since laboratory signs of SARS-CoV-2 infection were detected in a number of patients with acute neuropathy. Given the higher demand for respiratory support in GBS patients infected with CoV-2, it is assumed that COVID-19 may trigger the progression of neurological symptoms. Diagnosing GBS in SARS-CoV-2 patients is particularly challenging because symptoms such as shortness of breath and fatigue can be misinterpreted as a consequence of SARS-CoV-2 lung damage, delaying the timely diagnosis of GBS. Conclusion. Successful GBS management depends on clinical vigilance and its early diagnosis in patients with COVID-19.

COVID-19 associated with sensorimotor polyradiculoneuropathy and skin lesions: A case report

A novel betacoronavirus,SARS-CoV-2, causes Coronavirus disease 2019 typically presented with fever, myalgia and cough, but central and peripheral nervous system manifestations such as stroke, encephalitis and Guillain-Barre-Syndrome are being increasingly reported. Acute immune-mediated polyradiculoneuropathy (Guillain-Barre-Syndrome) mostly occurs after viral or bacterial infections, presenting with ascending flaccid tetraparesis, dysautonomia and respiratory failure. We reported a patient with COVID-19 (confirmed with Lung HRCT scan and positive SARS-CoV-2 PCR) who developed acute progressive flaccid tetraparesis and maculopapular pigmented plaques on the limbs, 2 weeks after respiratory symptoms. He was treated with IVIg as the Electrophysiologic study showed sensorimotor polyradiculoneuropathy with demyelinating features and skin biopsy showed interface dermatitis and vasculopathic reaction. The causal association between Guillen-Barre-Syndrome and COVID-19 is uncertain yet, but neurologists should be aware of early diagnosis and treatment of acute polyradiculoneuropathy which may cause fatal dysautonomia and respiratory failure in the context of COVID19 pandemic.

Is Guillain-Barr\xe8 syndrome triggered by SARS-CoV-2? Case report and literature review

BackgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the infectious agent responsible for coronavirus disease 2019 (COVID-19). Respiratory and gastrointestinal manifestations of SARS-CoV-2 are well described, less defined is the clinical neurological spectrum of COVID-19. We reported a case of COVID-19 patient with acute monophasic Guillain-Barré syndrome (GBS), and a literature review on the SARS-CoV-2 and GBS etiological correlation.Case DescriptionA 68 years-old man presented to the emergency department with symptoms of acute progressive symmetric ascending flaccid tetraparesis. Oropharyngeal swab for SARS-CoV-2 tested positive. Neurological examination showed bifacial nerve palsy and distal muscular weakness of lower limbs. The cerebrospinal fluid assessment showed an albuminocytologic dissociation. Electrophysiological studies showed delayed distal latencies and absent F waves in early course. A diagnosis of Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) subtype of GBS was then made.ConclusionsNeurological manifestations of COVID-19 are still under study. The case we described of GBS in COVID-19 patient adds to those already reported in the literature, in support of SARS-CoV-2 triggers GBS. COVID-19 associated neurological clinic should probably be seen not as a corollary of classic respiratory and gastrointestinal symptoms, but as SARS-CoV-2-related standalone clinical entities. To date, it is essential for all Specialists, clinicians and surgeons, to direct attention towards the study of this virus, to better clarify the spectrum of its neurological manifestations.

Guillain‐Barré syndrome: Novel treatment by complement inhibition

AbstractGuillain‐Barré syndrome (GBS) is an immune‐mediated polyneuropathy that can cause acute flaccid tetraparesis and respiratory failure. Intravenous immunoglobulin and plasmapheresis were established as a standard of care, but 20% to 30% of patients require mechanical ventilation during the acute phase and up to 20% of patients cannot walk independently even 1 year after the onset. As the pathophysiology of GBS has been elucidated, complement activation draws attention as a key treatment point. In acute motor axonal neuropathy, anti‐ganglioside antibody deposition and resulting complement activation lead to nerve conduction failure and eventually axonal degeneration. In acute inflammatory demyelinating polyneuropathy, although the process has not been clearly revealed, autopsied specimens showed that terminal complement complex rimmed the surface of the Schwann cells. Eculizumab is an antibody to the complement protein C5 and can potently inhibit complement activation. In a murine model with anti‐ganglioside antibody–mediated neuropathy, eculizumab can avert respiratory failure and motor neuropathy. A randomized controlled phase II trial showed that eculizumab might be effective also for GBS patients. Inhibition of complement activation can be a new therapeutic option in the near future.

Guillain-Barré Syndrome associated with Chikungunya virus infection:

Objective. The epidemic of infection by the Chikungunya virus currently represents one of the largest epidemiological occurrences in south and Central America, being considered a serious public health problem, since, in addition to the typical clinical symptomatology, there are reports of Associated neurological diseases, such as Guillain-Barré Syndrome. Guillain-Barré Syndrome is an acute inflammatory demyelinating polyneuropathy that leads to various functional impairments, such as flaccid paralysis, areflexia and muscular weakness. Method. A case of Guillain-Barré Syndrome was reported after infection by the chikungunya virus. Results. The case presented severe functional complications, with a picture of flaccid tetraparesis, with motor and sensory impairment, mainly in the left hemibody. Conclusion. From this perspective, from this report, and from reports of previous cases, it is considered that chikungunya virus infection may cause Guillain-Barré Syndrome, among other severe neurological complications, in an acute and progressive way, especially in areas considered endemic.

SOP myasthenic crisis

IntroductionThe overall prevalence of myasthenic crisis is quite low at 30/1 million inhabitants because myasthenia gravis is a rare disease per se. But it should be noted that 15–20% of patients with myasthenia gravis experience at least one crisis in their lives. Most often, the crisis occurs within the first 2 years of the disease or is even the first manifestation of a yet undiagnosed myasthenia gravis in up to 20%.Median duration of MC is about 2 weeks (median 12–14 days of ventilation) under sufficient treatment, but prolonged courses are not uncommon and often due to comorbidities and complications, so that about 20% are still mechanically ventilated after 1 month.The lifetime risk of recurrence of a crisis is approx. 30%. Data on mortality differ between about 2–5% to even more than 16%. Lethal outcomes are almost never caused by the crisis itself, but because comorbidities or complications eventually become limiting.DefinitionMyasthenic crisis (MC) is the life-threatening maximal manifestation of myasthenia gravis (MG) necessitating mechanical ventilation, supportive feeding and (neuro-)intensive care. Weakness may develop within minutes to days and encompass flaccid tetraparesis with immobility, severe dyspnea, respiratory insufficiency and aspiration. Globus events may be life threatening due to rapidly exhausting coughing and swallowing.First steps: immediate measures● Check and secure vital functionsComments● not applicableConclusionThe main symptom of (imminent) myasthenic crisis is the rapidly progressive weakness of the respiratory and bulbar muscles, which lead to a decompensation with aspiration and respiratory insufficiency. Clinical examination and clinical history should lead early to the diagnosis of MG with (impending) crisis. The detection of red flags and the dynamic deterioration of symptoms entail admission to the intensive care unit. Due to bulbar symptoms with aspiration and/or respiratory insufficency, early intubation to secure the airway is essential. Therapy includes symptomatic treatment with pyridostigmine or neostigmine and acute causal treatment by immunoadsorption/plasmapheresis or alternatively with immunoglobulins. If used early, intubation may still be prevented and clinical improvement can be achieved within a few days. At the same time, immunosuppression with corticosteroids and azathioprine should be initiated or optimized. For escalation rituximab is an option. The early diagnosis and consequent treatment of infections and other complications such as delirium influence the further course.

P90-F Painful pupil-involving oculomotor palsy as a manifestation of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Background Opthalmoplegia is a rare complication of CIDP and usually occurs in the setting of a longstanding disease. Moreover, very few cases describe an autonomic involvement. Material and methods Case report. Results and conclusions A 25-year-old man with no relevant past medical history was admitted to the hospital due to limb weakness and numbness which evolved over two months. He denied recent infectious illness. Neurological examination revealed a flaccid, symmetric and distal-predominant tetraparesis, areflexia and vibration sense impairment of the lower limbs. Nerve conduction studies showed demyelinating changes fulfilling 2010 EFNS/PNS criteria for the definite diagnosis of CIDP. CSF had increased protein content and no paraprotein or ganglioside antibodies were found. As unaided ambulation was initially preserved, immunotherapy was not immediately started. However, over the following days, he developed dyplopia with right ocular pain. A partial third right palsy with a fixed mydriasis was found and IV immunoglobulin was then initiated. A few days after starting immunotherapy dyplopia resolved and tetraparesis also improved. We report an unusual case of CIDP with an early unilateral, painful, third nerve palsy with pupil involvement.

P84-T Neuromyositis associated with cytomegalovirus infection in an immunocompetent patient with antineutrophil cytoplasmic antibodies-associated vasculitis

The role of CMV in ANCA-positive vasculitis is controversial. A 40-year-old man, with an unremarkable medical history, presented with progressive muscle weakness of all four limbs, accompanied by diffuse myalgia, arthralgia, and a 10 kg weight loss 2 months before admission to the Neurology Department of our hospital. Clinical examination revealed a flaccid tetraparesis with absent reflexes on the lower limbs and marked muscle atrophy. Electrophysiological investigations were consistent with a demyelinating motor polyradiculoneuropathy. Laboratory data showed AST = 1137 IU/L (normal < 37 IU/L), ALT = 582 IU/L (normal < 41 IU/L), C-reactive protein (CRP) = 19 mg/L (normal < 8 mg/L), CPK = 13779 IU/L (normal < 397). and LDH = 911 IU/L (normal < 192). CMV ELISA (serum) was strongly positive and CMV DNA by polymerase chain reaction (PCR) in blood and CSF also was positive. Immunologic screening revealed positive anti-myeloperoxidase antibodies anti-MPO = 56,9 IU/mL (normal < 9 IU/mL; ELISA). The inflammatory process involving the vessel walls and muscle fibers was histologically found in biopsy specimen of the muscle, with positive staining for CMV. The patient was treated initially with corticosteroids and intravenous immunoglobulins, but these therapies had very poor effect. Immunosuppressive therapy was instituted, resulting in a progressive partial recovery of all four limb weakness without relapses. We hypothesize that the CMV infection triggered an ANCA-positive vasculitis and its particular severe neurological involvement. An awareness of the association between CMV infection and vasculitis may have important therapeutic consequences.

Metastatic Infection by Methicillin-Sensitive Staphylococcus aureus and its Potential Association to an Unusual Humoral Immunodeficiency

Multifocal metastatic infection by Staphylococcus aureus is severe and uncommon. We describe the case of a previously asymptomatic 63-year-old man diagnosed with complicated staphylococcal endocarditis of the mitral valve along with cervical and lumbar spondylodiscitis, and multiple pre/para-vertebral abscesses; establishing a persistent flaccid tetraparesis, neurogenic bladder and anal sphincter dysfunction. The patient overcame infection after an 8-week Cloxacillin/ Rifampicin treatment associated to multiple surgical procedures. Thereafter, immunological assessment was performed, driving to the diagnosis of selective IgM deficiency. We propose a physiopathological relationship between this rare dysgammaglobulinemia and such fulminant staphylococcal invasive infection; that would, perhaps, justify humoral immunity assessment in patients undergoing staphylococcemia.

Multi-Stage Pyocyanic Spondylodiscitis: A Case Report

Spondylodiscitis is an intraspinal infection that affects the intervertebral disk and adjacent vertebral bodies. Neurological signs represent urgency and a criterion of gravity. Magnetic resonance imaging (MRI) is the baseline assessment to make the diagnosis. Pyocyanic bacillus represents less than 5% of bacterial causes. We report the case of a 79-year-old woman who progressively presented paresthesia of the four limbs with flaccid tetraparesis. MRI showed multi-stage cervico-dorsal involvement. Based on that, computed tomography-guided disco vertebral biopsy was performed and permitted to isolate a pyocyanic agent. The treatment consisted of a double antibiotherapy with resuscitation measures. The evolution was favorable, and the entry gate remained mysterious.

84. Atypical presentation of Guillain-Barré syndrome in childhood: A clinical case

A 31-months old child was admitted with a 4-day history of fever and weakness. The initial neurological examination revealed a meningeal syndrome with neck stiffness, without consciousness impairment. Head CT was normal. Analysis of CSF showed only increased level of proteins. Based on clinical manifestations, an empirical antibiotics and antiviral therapy was started. During the following 48 h, a progressive flaccid tetraparesis occurred. An ENG/EMG study was performed to exclude a peripheral involvement that showed decreased CMAP amplitude, partial motor conduction blocks/pseudo-blocks signs in the forearm segment of median and ulnar nerves and absence of F-waves. The MRI of spinal cord showed enhancement of the cauda equina. These data led to a diagnosis of polyradiculonevritis and a treatment with IVIG was performed with an initial stabilization of the neurological symptoms. At 24 days, patient’s symptoms did not further recover and ENG/EMG was repeated showing: diffuse absence of CMAPs, reduction of SAPs, active denervation in all muscles. The final diagnosis was AMSAN, and another cycle of IVIG was administered. As signs of meningeal irritation may be consistent with meningitis or other diseases, it is important to recognize atypical features of pediatric GBS spectrum syndromes to achieve early diagnosis and treatment.

Botulism: Diagnosis and Therapy

Background Botulism is a rare, life-threatening, time-critical neuroparalytic disease that is frequently a subject of differential diagnostic considerations. But there is much uncertainty regarding diagnosis and therapy. Rapid diagnosis, early antitoxin dose, consistent food hygiene and the sensitization of the population can help to reduce incidence, morbidity and mortality. Methods This overview is based on an epidemiological data inquiry (RKI, ECDC, CDC, WHO) and a selective literature research (pubmed till March 2017). Additionally, the German botulism guideline (2012) and own diagnostical experiences were taken into account. Results The incidence of botulinum toxin intoxication induced by ubiquitous spore-forming Clostridium botulinum (main representative) is < 0.01/100 000 EU citizens. Foodborne botulism is a pure intoxication syndrome (most common form) due to improperly prepared or incorrectly stored food. Wound and infant botulism are kinds of "toxico-infections". A "bulbar" neuroparalysis is a main symptom progressing to a flaccid tetraparesis up to respiratory paralysis. Infant botulism is presented non-specific and is treated only symptomatically; but a special human-derived antitoxin is available at international pharmacies. In case of suspected foodborne or wound botulism antitoxin must be administered as soon as possible, which may also be effective 24 hours after symptoms onset. There is no evidence for adjuvant treatment except of intensive care unit (ICU) therapy. Conclusion Despite typical symptomatology botulism is often diagnosed too late. Early antitoxin administration and ICU therapy are crucial for survival. A consultant laboratory should be contacted for advice.

Case report: subacute tetraplegia in an immunocompromised patient

BackgroundClinical reasoning in Neurology is based on general associations which help to deduce the site of the lesion. However, even “golden principles” may occasionally be deceptive. Here, we describe the case of subacute flaccid tetraparesis due to motor cortical lesions. To our knowledge, this is the first report to include an impressive illustration of nearly symmetric motor cortical involvement of encephalitis on brain MRI.Case presentationA 51 year old immunocompromized man developed a high-grade pure motor flaccid tetraparesis over few days. Based on clinical presentation, critical illness polyneuromyopathy was suspected. However, brain MRI revealed symmetrical hyperintensities strictly limited to the subcortical precentral gyrus. An encephalitis, possibly due to CMV infection, turned out to be the most likely cause.ConclusionWhile recognition of basic clinical patterns is indispensable in neurological reasoning, awareness of central conditions mimicking peripheral nervous disease may be crucial to detect unsuspected, potentially treatable conditions.

49. Effectiveness of subcutaneous immunoglobulins in paraneoplastic CIDP caused by signet ring cell adenocarcinoma

We describe a women 54-year-old, heavy smoker (20 cigarettes/day) who was admitted to our hospital after emergency department admission because of an acute flaccid tetra paresis. Lumbar puncture revealed albumino-cytologic dissociation with 4, 3 gr/L proteins and no cells in CSF. Electromyography concluded for a demielinating poliradicoloneuropathy. Intravenous immunoglobulins (ivIg) 2 gr/Kg in 5 days produced discrete improvement of weakness. Cerebral and cervical spine MRI showed only a left neurinoma of left acustic nerve. Chest and abdomen CT scan revealed a non-specific lung nodule. She was transferred in a rehabilitation department with a near-complete resolution of strength deficits. Patient came back after one year with a severe strength worsening and wheel-chair bounded (MRC score: 22/60). She started 2 gr/Kg and then subcutaneous Ig 1 mg/Kg/months(0.2 mg/Kg every 5 days). In 6 months patient showed a progressive improvement up to MRCscore 40/60.TC-PET scan showed multiple areas of hypermetabolism in the back bone and in anterior-superior iliac spine. The latter was biopsied showing signet-ring cells. Clinical course of patient quickly worsened with drug-resistant seizures and obstructive hydrocephalus which required neurosurgery. After few weeks she died at home. Before neoplastic radiological evidence, subcutaneous Ig were effective in treating strength deficits of this paraneoplastic CIDP.

Immunodeficiency syndrome in a Weimaraner involving the peripheral nervous system

An 11-week-old male entire Weimaraner presented with a five-day history of pyrexia and pain. Onset of clinical signs developed within five days of routine second vaccination with a modified live...

Anterior horn syndrome: A rare manifestation of primary Sjögren's syndrome

The authors report an exceptional case of an anterior horn syndrome associated with Sjögren's syndrome in a 58-year-old patient with a flaccid tetraparesis revealed by asymmetric atrophy and diffuse fasciculations associated with xerostomia and xerophthalmia. The electroneuromyography objectified a diffuse anterior horn syndrome. The brain MRI and spinal cord were normal. Laboratory tests revealed positive anti-SSA and anti-SSB antibody. The salivary glands biopsy objectified lymphocytic sialadenitis grade 3 of Chisholm. The Schirmer's test was abnormally low. Diagnosis of anterior horn syndrome as part of Sjögren's syndrome was retained. The methylprednisolone bolus allowed partial clinical improvement after 12 months of evolution. Therefore, in patients with isolated anterior horn involvement, a correct diagnosis of the underlying SS is often delayed or overlooked entirely; in these instances, standard clinicoserological assessment is recommendable.

How often and when Fisher syndrome is overlapped by Guillain-Barré syndrome or Bickerstaff brainstem encephalitis?

Fisher syndrome (FS) may overlap with Guillain-Barré syndrome (GBS), in particular the pharyngeal-cervical-brachial variant form (PCB-GBS), or Bickerstaff brainstem encephalitis (BBE). Our aim was to elucidate the frequency of this overlap and the patterns of clinical progression in patients with FS. Sixty consecutive patients with FS were studied. FS/PCB-GBS was diagnosed when the patients developed pharyngeal, cervical and/or brachial weakness. Patients with flaccid tetraparesis were diagnosed as having FS/conventional GBS. FS/BBE was defined as the development of consciousness disturbances. All 60 patients initially developed the FS clinical triad alone (pure FS). Of these, 30 (50%) patients had pure FS throughout their course, whereas the remaining 50% of patients showed an overlap: PCB-GBS in 14 (23%) patients, conventional GBS in nine (15%) patients and BBE in seven (12%) patients. The median (range) durations from FS onset to progression to FS/PCB-GBS, FS/GBS or FS/BBE were 5 (1-7), 3 (1-4) and 3 (1-5) days, respectively. Patients with overlap syndromes more frequently received immune-modulating treatment, and the outcomes were generally favourable. The frequencies of positivity for anti-GQ1b, GT1a, GD1a, GD1b, GalNAc-GD1a and GM1 antibodies were not significantly different amongst the four groups. Of the patients with pure FS, 50% later developed an overlap with PCB-GBS, conventional GBS or BBE. The overlap occurred within 7 days of FS onset; thus, physicians should pay attention to the possible development of this overlap during the first week after FS onset.