OBJECTIVE: To study the clinical features of children with Guillain-Barré syndrome (GBS) and the significance of Brighton criteria in childhood GBS. METHODS: A retrospective analysis was performed on the medical data of 72 children with GBS. Brighton criteria were used for the grading of diagnostic certainty (level 1 as the highest level, and level 4 as the lowest level). A Spearman's rank correlation analysis was used to evaluate the correlation of auxiliary examinations with the level of diagnostic certainty of Brighton criteria. RESULTS: A total of 72 children with GBS were enrolled, with a mean age of onset of (98±32) months. All children (100%, 72/72) had weakness of bilateral limbs and disappearance or reduction of tendon reflex, and limb weakness reached the highest level of severity within 4 weeks. Of all the 72 children, 68 (94%) had positive results of neural electrophysiological examination and 51 (71%) had positive results of cerebrospinal fluid (CSF) examination, and the positive rate of neural electrophysiological examination was significantly higher than that of CSF examination ( CONCLUSIONS Most of the children with GBS meet Brighton criteria level 1, and the positive results of CSF examination and neural electrophysiological examination play an important role in improving the level of diagnostic certainty of Brighton criteria. Neural electrophysiological examination has a higher positive rate than CSF examination in the early stage of the disease.
Child ; Child, Preschool ; Extremities ; Guillain-Barre Syndrome/diagnosis* ; Humans ; Muscle Weakness ; Physical Examination ; Retrospective Studies

Guillain-Barré syndrome (GBS) is nowadays consider as an umbrella term that has heterogenous presentation depend on their subtypes. GBS is clinical diagnosis and its diagnosis can be supported by laboratory findings from cerebral spinal fluid study, nerve conduction study, anti-ganglioside antibodies, spinal magnetic resonance imaging and nerve ultrasound. Understanding atypical subtypes and GBS mimicking diseases are crucial for correct diagnosis. Both proper medical care for respiratory and autonomic dysfunction and immunotherapy are essential to improve outcome of GBS. Here, we summarized the current concept on diagnosis, immunopathophysiology and treatment of GBS.
Antibodies ; Diagnosis ; Guillain-Barre Syndrome ; Immunotherapy ; Magnetic Resonance Imaging ; Neural Conduction ; Ultrasonography

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis that commonly affects the peripheral nervous system. EGPA rarely presents with acute polyneuropathy resembling Guillain-Barré syndrome (GBS). A 51-year-old female patient with a history of asthma suddenly developed bilateral lower extremityparesthesia that progressed to asymmetric ascending paralysis within 10 days of onset. Nerve conduction study results were compatible with acute motor sensory axonal neuropathy, consistent with a GBS subtype. A clinical and neurophysiological diagnosis of GBS was made, and high-dose intravenous immunoglobulins were administered. However, the patient's painful motor weakness persisted. Furthermore, she had newly developed skin lesions on her back, face, and arms. Her blood test revealed marked eosinophilia (>60%). In addition, antineutrophil cytoplasmic antibodies were reported positive. A Water's view radiographic image showed bilateral maxillary sinusitis. Considering the history of asthma, we suspected EGPA-associated polyneuropathy and started steroid treatment. The patient's strength and eosinophilia improved rapidly and dramatically. EGPA can mimic GBS and should be differentiated because of different treatment strategies. Early diagnosis and prompt treatment help achieve a good outcome.
Antibodies, Antineutrophil Cytoplasmic ; Arm ; Asthma ; Axons ; Diagnosis ; Early Diagnosis ; Eosinophilia ; Eosinophils ; Female ; Granulomatosis with Polyangiitis ; Guillain-Barre Syndrome ; Hematologic Tests ; Humans ; Immunoglobulins, Intravenous ; Maxillary Sinus ; Maxillary Sinusitis ; Middle Aged ; Neural Conduction ; Paralysis ; Peripheral Nervous System ; Polyneuropathies ; Skin ; Systemic Vasculitis

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Ankle ; Cerebrospinal Fluid ; Cough ; Demyelinating Diseases* ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated ; Extremities ; Female ; Fever ; Guillain-Barre Syndrome ; Humans ; Knee ; Korea ; Leukocytosis ; Lymphocytes ; Magnetic Resonance Imaging ; Nervous System Diseases ; Neural Conduction ; Neuroimaging ; Peripheral Nervous System* ; Pons ; Reflex, Stretch ; Spinal Cord

PURPOSE: Influenza-associated neurologic complications in children are diverse. But there has been little long-term and large-scale research about neurologic complications of seasonal influenza. This study aimed to identify the incidence, characteristics, and risk factors for neurologic complications in children hospitalized with influenza. METHODS: Retrospective analysis was conducted on the clinical data of 940 children hospitalized with confirmed influenza infection from Oct, 2010 to May, 2016 in Kwangju Christian Hospital. RESULTS: A total of 940 children with influenza were hospitalized, of whom 96 (10.2%) had neurologic complications:81 children presented febrile seizures (8.6%) and some included 12 other seizures (1.3%),1 encephalitis (0.1%), 1 Guillain-Barré syndrome (0.1%), 1 aseptic meningitis (0.1%). They had good prognosis except the encephalitis child. The incidence of neurologic complications was significantly higher in influenza A than in influenza B (11.9% vs. 7.0%, P=0.036). The incidence of influenza A was highest in February, while that of influenza B was highest in March and April. The monthly distribution of neurological complications reflected the influenza incidence. The risk factors for influenza-associated neurologic complications were underlying neurologic disease and young age. No significant clinical differences were observed between influenza A and B in febrile seizure. CONCLUSION: Febrile seizures are the most common neurologic complication with good prognosis. Although encephalitis/encephalopathy is rare, it can be severe with sequelae, so prompt diagnosis and treatment should be initiated. And influenza vaccine should be encouraged to children with underlying neurologic disease.
Child* ; Diagnosis ; Encephalitis ; Guillain-Barre Syndrome ; Gwangju ; Humans ; Incidence ; Influenza A virus ; Influenza B virus ; Influenza Vaccines ; Influenza, Human ; Meningitis, Aseptic ; Prognosis ; Retrospective Studies ; Risk Factors ; Seasons* ; Seizures ; Seizures, Febrile

BACKGROUND AND PURPOSE: Middle East respiratory syndrome (MERS) has a high mortality rate and pandemic potential. However, the neurological manifestations of MERS have rarely been reported since it first emerged in 2012. METHODS: We evaluated four patients with laboratory-confirmed MERS coronavirus (CoV) infections who showed neurological complications during MERS treatment. These 4 patients were from a cohort of 23 patients who were treated at a single designated hospital during the 2015 outbreak in the Republic of Korea. The clinical presentations, laboratory findings, and prognoses are described. RESULTS: Four of the 23 admitted MERS patients reported neurological symptoms during or after MERS-CoV treatment. The potential diagnoses in these four cases included Bickerstaff's encephalitis overlapping with Guillain-Barré syndrome, intensive-care-unit-acquired weakness, or other toxic or infectious neuropathies. Neurological complications did not appear concomitantly with respiratory symptoms, instead being delayed by 2–3 weeks. CONCLUSIONS: Neuromuscular complications are not rare during MERS treatment, and they may have previously been underdiagnosed. Understanding the neurological manifestations is important in an infectious disease such as MERS, because these symptoms are rarely evaluated thoroughly during treatment, and they may interfere with the prognosis or require treatment modification.
Cohort Studies ; Communicable Diseases ; Coronavirus ; Coronavirus Infections* ; Diagnosis ; Encephalitis ; Guillain-Barre Syndrome ; Humans ; Middle East Respiratory Syndrome Coronavirus ; Middle East* ; Mortality ; Neurologic Manifestations ; Pandemics ; Peripheral Nervous System Diseases ; Prognosis ; Republic of Korea

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.
Asthma ; Brain ; Caudate Nucleus ; Cerebral Infarction ; Cerebrospinal Fluid ; Churg-Strauss Syndrome ; Cyclophosphamide ; Diagnosis ; Diffusion Magnetic Resonance Imaging ; Eosinophilia ; Eosinophils* ; Extremities ; Fever ; Gadolinium ; Gait ; Granulomatosis with Polyangiitis* ; Guillain-Barre Syndrome ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Muscle Weakness ; Neural Conduction ; Peripheral Nervous System Diseases ; Polyneuropathies* ; Sensation ; Vasculitis ; Vasculitis, Central Nervous System*

Guillain-Barré syndrome (GBS) is characterized by ascending symmetric paralysis, paresthesia, and autonomic dysfunction. Autonomic dysfunctions develop in two-thirds of the patients, and urinary retention and paralytic ileus usually develop in severe and advanced cases. There has been no pediatric case with paralytic ileus as a presenting symptom of GBS. Reported herein is a case of GBS presenting vomiting as an initial symptom. A 28-month-old girl was brought to the emergency room due to a 2-day history of vomiting. She vomited multiple times 1 day before the visit, and had only single voiding on admission day. Her abdomen was distended, with decreased bowel sound. Intravenous fluid was given under the diagnosis with acute gastroenteritis with dehydration. The abdominal computed tomography revealed severe paralytic ileus and urinary bladder distention. After having two seizures, she developed respiratory failure necessitating ventilator care. On day 8 after admission, motor weakness with areflexia was noticed. The cerebrospinal fluid analysis showed elevated proteins (80 mg/dL) with no white blood cells (0/mm³). The nerve conduction study showed axonal-type peripheral polyneuropathy. GBS was diagnosed. During the follow-up, the patient was found to have motor weakness in the lower extremities. Paralytic ileus with protracted vomiting can be an initial presentation of GBS in children. Autonomic dysfunction with GBS can be considered in a differential diagnosis in vomiting patients with unclear etiologies.
Abdomen ; Cerebrospinal Fluid ; Child* ; Child, Preschool ; Dehydration ; Diagnosis ; Diagnosis, Differential ; Emergency Service, Hospital ; Female ; Follow-Up Studies ; Gastroenteritis ; Guillain-Barre Syndrome* ; Humans ; Ileus ; Intestinal Pseudo-Obstruction* ; Leukocytes ; Lower Extremity ; Neural Conduction ; Paralysis ; Paresthesia ; Polyneuropathies ; Respiratory Insufficiency ; Seizures ; Urinary Bladder ; Urinary Retention ; Ventilators, Mechanical ; Vomiting

Autochthonous hepatitis E virus (HEV) is an emerging pathogen in developed countries, and several cases of acute HEV infection have been reported in South Korea. However, there have been no reports on HEV-associated Guillain-Barré syndrome (GBS) in Korea. We recently experienced the case of a 58-year-old Korean male with acute HEV infection after ingesting raw deer meat. Persistent cholestasis was resolved by the administration of prednisolone. At 2.5 months after the clinical presentation of HEV infection, the patient developed weakness of the lower limbs, and was diagnosed with GBS associated with acute hepatitis E. To our knowledge, this is the second report on supportive steroid therapy for persistent cholestasis due to hepatitis E, and the first report of GBS in a Korean patient with acute HEV infection.
Acute Disease ; Alanine Transaminase/blood ; Antibodies, Viral/blood ; Aspartate Aminotransferases/blood ; Bilirubin/analysis ; Cholestasis/*drug therapy ; Guillain-Barre Syndrome/complications/*diagnosis ; Hepatitis E/*diagnosis/etiology ; Hepatitis E virus/immunology ; Humans ; Immunoglobulin M/blood ; Liver/pathology ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Republic of Korea ; Steroids/*therapeutic use

Guillain-Barré syndrome and acute transverse myelitis manifest as demyelinating diseases of the peripheral and central nervous system. Concurrency of these two disorders is rarely documented in literature. A 4-year-old girl presenting with cough, fever, and an impaired walking ability was admitted to hospital. She had no previous complaints in her medical history. A physical examination revealed lack of muscle strength of the lower extremities and deep tendon reflexes. MRI could not be carried out due to technical problems; therefore, both Guillain-Barré syndrome and acute transverse myelitis were considered for the diagnosis. Intravenous immunoglobulin treatment was started as first line therapy. Because this treatment did not relieve the patient's symptoms, spinal MRI was carried out on the fourth day of admission and demyelinating areas were identified. Based on the new findings, the patient was diagnosed with acute transverse myelitis, and high dose intravenous methylprednisolone therapy was started. Electromyography findings were consistent with acute polyneuropathy affecting both motor and sensory fibers. Therefore, the patient was diagnosed with concurrency of Guillain-Barré syndrome and acute transverse myelitis. Interestingly, while concurrency of these 2 disorders is rare, this association has been demonstrated in various recent publications. Progress in diagnostic tests (magnetic resonance imaging and electrophysiological examination studies) has enabled clinicians to establish the right diagnosis. The possibility of concurrent Guillain-Barré syndrome and acute transverse myelitis should be considered if recovery takes longer than anticipated.
Central Nervous System ; Child, Preschool ; Cough ; Demyelinating Diseases ; Diagnosis ; Diagnostic Tests, Routine ; Electromyography ; Female ; Fever ; Guillain-Barre Syndrome* ; Humans ; Immunoglobulins ; Lower Extremity ; Magnetic Resonance Imaging ; Methylprednisolone ; Muscle Strength ; Myelitis, Transverse* ; Physical Examination ; Polyneuropathies ; Reflex, Stretch ; Walking