Clinical presentation and prognosis of childhood Guillain-Barré syndrome

Aim:   Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy commonly characterised by rapidly progressive, symmetric weakness and areflexia. This study is to assess the clinical characteristics of paediatric GBS, as well as its long-term functional prognosis.
Methods:   We retrospectively assessed the clinical manifestations, results of electrodiagnostic tests, functional status and prognosis of 56 children diagnosed with GBS. Based on clinical and electrophysiological findings, the patients were classified as having acute inflammatory demyelinating polyradiculoneuropathy ([AIDP] n  = 34), acute motor axonal neuropathy ([AMAN] n  = 14), acute motor and sensory axonal neuropathy ( n  = 1) and Miller Fisher syndrome ([MFS] n  = 7).
Results:   Upper respiratory infection was the most frequent preceding event, and limb weakness was the most frequent symptom at GBS onset. There was no significant difference in the mean time from the onset of illness to nadir between any of these groups. Both the AIDP and AMAN groups showed significantly poorer functional status, measured by the Hughes scale, than the MFS group. Two years after nadir, however, the three groups did not differ significantly. Functional status at nadir, as estimated by the Hughes scale, is a more important factor than electrophysiological types in predicting long-term outcome.
Conclusion:   The most common symptom at onset in paediatric GBS was limb weakness. Functional status at nadir in AMAN was not significantly different from that of AIDP, and both types achieved good functional outcome for ambulation after 2 years. Functional status at nadir was more important than the electrophysiological type in predicting long-term outcomes.     

Guillain-Barré syndrome

? Based on strong research evidence, in countries where poliomyelitis has been eliminated, GBS is the most common cause of acquired paralysis in children. (9) ? Based on strong research evidence, GBS describes a spectrum of disorders caused by an autoimmune reaction against peripheral nerve components, including the myelin sheath and the axon. (10)(11) ? Based on strong research evidence, GBS usually is preceded by a bacterial or viral infection, less likely by vaccination in the 1 to 4 weeks before onset. The strongest relationship is with infection by C jejuni. (12)(13) ? Based on strong research evidence, GBS in children most often presents with symmetrical ascending paralysis, diminished or absent reflexes, and often severe pain. Pain may lead to a delay in diagnosis. (2) (3)(9)(14) ? Based on strong research evidence, the progressive phase peaks in 7 to 14 days and can lead to various levels of weakness, from abnormal gait to total paralysis, cranial nerve weakness, pain, respiratory compromise, and autonomic instability. (2) ? Based on some research evidence and consensus, children require hospitalization and often intensive care until their condition stabilizes because of significant risk of respiratory compromise and autonomic instability. (15) ? Based on strong research evidence in adults and some research evidence in children, IVIG and plasma exchange hasten recovery from GBS in patients with impaired ability to ambulate. (4)(6) ? Based primarily on consensus, IVIG is the treatment of choice in children with GBS. (6)(9)(15) ? Based on strong research evidence, the prognosis for full functional recovery in childhood GBS is excellent. (2)(8)(9).     

Guillain-Barré syndrome: clinical presentation and prognosis in children under six years-old

IntroductionGuillain-Barré syndrome (GBS) is an acute polyneuropathy that is difficult to diagnose in young children.ObjectivesTo review the form of presentation of GBS in children under six years-old at the time of onset and the symptoms they had until the diagnosis, the findings in the complementary tests, and the progression and prognosis.Patients and methodsAll patients less than 6 years-old who fulfilled the Asbury et al criteria for the diagnosis of GBS were reviewed.ResultsEight patients with a mean age of 3.4 years were included. Of those 75% recorded a previous infection. The symptoms prior to the diagnosis were very heterogeneous which entailed a wide differential diagnosis with many complementary examinations. The mean time to diagnosis was 8.5 days. All of them (100%) had motor involvement in the lower limbs, 75% in the upper limbs and 12% in the respiratory muscles. Sensory and cranial nerve involvement was observed in 62.5% and 25%, respectively. Admission to intensive care was required for 25% of the patients. Albumino-cytological dissociation was observed in the CSF in 83.3% and all the electrophysiological tests were positive with different patterns. The prognosis was excellent in all patients.ConclusionsGBS in children under 6 years-old is difficult to diagnosis due to the signs of onset often being unspecific. This entails a wide differential diagnosis, with the subsequent diagnostic delay. There is a good prognosis in all the electrophysiological sub-types in this age group.     

Prolonged Guillain-Barré syndrome

Outcome in Guillain-Barré syndrome (GBS) in childhood is usually favorable although prolonged and severe forms may develop. We report the case of a 12-year-old boy with rapidly ascending, slowly-progressive GBS with severe cranial nerve and autonomic dysfunction. Diagnosis was based on clinical presentation, electromyography, cerebrospinal fluid analysis and plasmatic antiganglioside GM1 antibodies. Early treatment with an initial loading dose of 2 g/kg of intravenous gammaglobulins over 5 days was given with a significant but slow clinical improvement. A repeat infusion of gammaglobulins was given two months later. Response was satisfactory with motor, sensory and autonomic dysfunction recovery. A third course of immunoglobulins 28 days later produced little benefit. At the present time, 5 months after the onset of the disease, flaccid paralysis of the lower limbs and the distal portion of the upper limbs persists but autonomic instability has disappeared. The patient has been decannulated and is following an ambulatory rehabilitation program. Although repeated intravenous immunoglobulins may be useful in the treatment of prolonged GBS, the key issue is excellent intensive care unit management. Further research examining potentially more effective treatment such as beta-interferon and immunosuppressive agents is required.     

A rare form of Guillain-Barré syndrome: pharyngeal-cervical-brachial variant

Guillain-Barré syndrome is clinically characterized by acute onset of generalized, symmetrical, and ascending muscle weakness and areflexia from peripheral nerve involvement. In Guillain-Barré syndrome variants, however, some patients have unusual distribution of muscle involvement. Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is characterized by oropharyngeal, neck, and upper limb muscle involvement. Although Guillain-Barré syndrome is one of several post-infectious diseases that cause limb muscle weakness, the incidence of pharyngeal-cervical-brachial variant is relatively low. Here we report the case of a 16-month-old boy who developed a rare form of Guillain-Barré syndrome, the pharyngeal-cervical-brachial variant of the disease. We concluded that taking all the other etiologic reasons into consideration, pharyngeal-cervical-brachial variant of Guillain-Barré syndrome should be remembered in patients with symptoms of bulbar and upper extremity weakness not only for early diagnosis but also to plan the treatment early and follow up the potential complications.     

Risk of Guillain-Barré syndrome after measles-mumps-rubella vaccination

OBJECTIVE: To assess the postulated causal association between measles-mumps-rubella (MMR) vaccination and Guillain-Barré syndrome (GBS). STUDY DESIGN: Active retrospective study based on linkage of the nationwide hospital discharge register with individual vaccination records. All patients hospitalized for treatment of GBS in Finland between November 1982 and December 1986 were included in the study. RESULTS: During the study period, 189 patients were hospitalized for treatment of GBS, and approximately 630,000 vaccine recipients received 900,000 doses of MMR vaccine; 24 of the 189 patients represented the prevailing target population for MMR vaccination, of whom 20 were vaccinated. MMR vaccination did not cause any increase over the background incidence of GBS, and no clustering of cases of GBS occurred at any time point after administration of MMR vaccine. The interval between vaccination and onset of symptoms of GBS exceeded the designated risk period of 6 weeks in all cases, varying from 80 days to years. MMR vaccination after recovery from GBS did not cause relapses of the illness. Respiratory or gastrointestinal tract infection predated the onset of GBS by 3 to 30 days in 20 (83%) of the 24 patients. CONCLUSIONS: No causal association seems to prevail between MMR vaccination and GBS.     

Guillain-Barré syndrome in childhood

The Guillain-Barré syndrome (GBS) is characterized by the acute onset of rapidly progressive, symmetric muscle weakness with absent or decreased muscle stretch reflexes. GBS is the most common cause of acute flaccid paralysis in childhood, with an incidence in Australia of 0.8 per 100,000 children per year. Recent advances in this field have included identification of a number of clinical and neurophysiologic subtypes of GBS, enabling improved characterization of etiology and improved prognostication in this disorder.     

Outcome in childhood Guillain-Barré syndrome

Objective  

To prospectively assess the outcome of children diagnosed with Guillain-Barré syndrome (GBS), followed up for a median duration of 25 months.     

Spinal MRI of vincristine neuropathy mimicking Guillain-Barré syndrome

A 4.3-year-old girl with acute leukaemia, who was being treated with chemotherapy (including vincristine), developed paraplegia. Spinal MRI showed diffusely enhancing nerve roots on contrast-enhanced images. Spinal fluid analysis showed a normal protein level. Vincristine neuropathy mimicking Guillain-Barré syndrome is thought to be the cause of the MRI abnormalities.     

Guillain-Barré syndrome after exanthem subitum

A female infant developed Guillain-Barré syndrome 20 days after having exanthem subitum confirmed serologically as human herpesvirus 6 infection. DNA of human herpesvirus 6 was detected in peripheral blood mononuclear cells collected on admission.     

Possible association of Guillain-Barré syndrome and hepatitis A vaccination

We report a case of Guillain-Barré syndrome in a previously healthy child who received a hepatitis A vaccination (HAVRIX) 5 days before onset of symptoms. No other precipitating factor could be identified. The relevant literature is reviewed and discussed.     

Guillain-Barré syndrome associated with Legionnella infection

This is the first report of Guillain-Barré syndrome (GBS) related to Legionnella pneumophilia infection. A 13-year-old boy presented with acute dysphagia and dyspnea. He lived in a rural area and had a history of drinking potable deep-hole water. The patient was intubated because of increased respiratory distress. A positive direct fluoresein antigen test confirmed L. pneumophilia infection in BAL. One week after the first admission, acute weakness was noticed including the lower extremities and was more prominent in the distal than the proximal portions. GBS was considered as the initial diagnosis. Tests for all causes known to trigger GBS were negative. Specific serology for L. pneumophilia IgG was positive. He was treated with intravenous immunoglobulins and discharged with minor weakness and difficulty in walking in the second month. On the basis of this case, L. pneumophilia should be included in the etiologic spectrum of GBS.     

Varicosities in an adolescent girl on laparoscopy: an unusual presentation of May–Thurner syndrome

May–Thurner syndrome is a rare clinical entity involving venous obstruction of the left lower extremity. May–Thurner syndrome most commonly presents with deep vein thrombosis. We describe an unusual presentation of a girl with a dragging abdominal pain mimicking the symptoms of a varicocele. Diagnosis can be made with MR venogram. There are many different treatment options but if compression of the vein is minimal conservative management is possible.