|Year : 2020 | Volume
| Issue : 4 | Page : 429-432
Clinical audit on the management of Guillain-Barre syndrome cases admitted to Assiut University Children Hospital
El S.K A. El Kareem, Eman F Gad, Sherif H Nader
Department of Pediatrics, Assiut University Hospital, Assiut, Egypt
|Date of Submission||12-Feb-2020|
|Date of Decision||07-Mar-2020|
|Date of Acceptance||19-Mar-2020|
|Date of Web Publication||20-Nov-2020|
Sherif H Nader
Department of Pediatrics, Assiut University Hospital, 68 El Gomhoria Street, Assiut 71111
Source of Support: None, Conflict of Interest: None
Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. It is characterized by progressive, symmetrical weakness, and areflexia in a previously healthy child. GBS should be diagnosed quickly in patients presenting with rapidly progressive paralysis. Meticulous monitoring and supportive care are needed in all GBS patients. Efforts focus on the follow-up of clinical course and outcome to improve the care and treatment of individual patients.
Materials and methods
The study included pediatric patients with GBS referred to Assiut University Children Hospital in 9 months from November 2016 to July 2017. Information was taken from resident doctors and mothers.
Intravenous immunoglobulin (IVIG) was administered to 17 (80.95%) cases. Four (19.05%) cases who presented in the improving phase of motor disability were managed supportively and recovered completely. Plasmapheresis was done to three (14.3%) cases, who did not respond well to IVIG. Complete recovery was observed in 17 (80.95%) cases and four (19.05%) cases experienced only incomplete recovery.
Nerve conduction velocity is very useful to diagnose GBS. IVIG and supportive care are considered key elements in the management of childhood GBS. IVIG administration early in the course of the disease results in better recovery. Plasmapheresis may be an effective option in cases with poor response to IVIG therapy.
Keywords: Guillain-Barre syndrome, intravenous immunoglobulin and plasmapheresis, progressive paralysis
|How to cite this article:|
A. El Kareem ES, Gad EF, Nader SH. Clinical audit on the management of Guillain-Barre syndrome cases admitted to Assiut University Children Hospital. J Curr Med Res Pract 2020;5:429-32
|How to cite this URL:|
A. El Kareem ES, Gad EF, Nader SH. Clinical audit on the management of Guillain-Barre syndrome cases admitted to Assiut University Children Hospital. J Curr Med Res Pract [serial online] 2020 [cited 2020 Dec 1];5:429-32. Available from: http://www.jcmrp.eg.net/text.asp?2020/5/4/429/301051
| Introduction|| |
In 1916, Georges Guillain and Jean–Alexandre Barré published the very first comprehensive detailed classic paper on Guillain-Barré syndrome (GBS). The initial diagnostic criteria were published in 1981 and refined in 1990,. GBS is an immune-mediated disorder of the peripheral nervous system, which is triggered by either infectious or noninfectious factors. It is a rapid-onset muscle weakness caused by an autoimmune response damaging the peripheral nervous system. GBS is mostly preceded by infection or other immune stimulation inducing autoimmune response. Molecular mimicry between microbial and nerve antigens is obviously a major triggering factor behind the development of the disorder.
Genetic and environmental factors that affect an individual's susceptibility to develop the disease are unknown. Abnormal autoimmune response does not develop in most individuals (>99%) exposed to an immune stimulus associated with GBS such as campylobacter jejuni. Acute progressive limb weakness, often with sensory and cranial nerves affection, usually develops 1–2 weeks after immune stimulation and progresses to peak clinical disability in 2–4 weeks. Nerve conduction velocity (NCV) is very useful to diagnose GBS. Many studies consider the treatment of choice to be intravenous immunoglobulin (IVIG) or plasmapheresis with supportive care. IVIG should be given for 5 days at a dose of 400 mg/kg/day.
The aim was to implement the use of the guidelines for management of GBS according to the American Academy of Neurology 2012 in Assiut University Children Hospital for 3 months and then audit the degree of adherence of the physicians to the recommended guidelines for 9 months.
| Materials and Methods|| |
The study included all pediatric cases with GBS referred to Assiut University Children Hospital in 9 months. Information was taken from resident doctors and mothers.
Site of the study
Assiut University Children Hospital.
Duration of the study
A period of 9 months from November 2016 to July 2017.
All cases presented with classic presentation of symmetrical ascending acute lower limb weakness, hypotonia, and hyporeflexia under the age of 18 years supported by NCV showing delayed F-wave and cerebrospinal fluid (CSF) examination in selected cases showing the characteristic cytoalbuminologic dissociation.
- Cases of lower limb weakness due to causes other than GBS [Table 1].
- Cases of respiratory failure needing ICU other than GBS.
The diagnosis of GBS is mainly based on the clinical evaluation and exclusion of possible alternative diagnoses. We conducted a complete neurological examination on all cases. NCV was done in all cases.
CSF examination was done in selected hemodynamically stable cases by lumbar puncture. We observed all cases for respiratory insufficiency and we provided mechanical ventilation in the presence of any of these factors:
- Clinical use of accessory muscles.
- Respiratory muscle fatigue.
- Severe bulbar weakness with risk of aspiration.
Clinical course, results of electrodiagnostic studies, treatment given, and outcome were assessed.
Patients signed an informed consent.
Assiut Faculty of Medicine approved the study.
Data were analyzed and processed using the SPSS software (Chicago, Ill., USA), version 16 in the form of frequency mean ± SD, range, and percentages.
| Results|| |
A total of 21 children with GBS were admitted to our pediatric hospital during the period of the study, of which 11 (52.4%) were men and 10 (47.6%) were women [Figure 1]. Age of the patients ranged from 1.5 to 13 years with a mean of 6.6 ± 3.2 and majority of them were 7–10 years age group (42.8%) [Table 2]. History of preceding infection was found in 13 (61.9%) cases. The main symptoms included weakness of upper and lower limbs, symptoms of cranial nerve affection, and symptoms of autonomic dysfunction, gait disturbance, dyspnea, fever, and muscular and radicular pain as shown in [Table 3].
The main signs included hyporeflexia, hypotonia, signs of cranial nerve affection, signs of autonomic dysfunction, symmetry of weakness, ataxia, and sensation as shown in [Table 4].
CSF analysis was performed in six (28.6%) cases and cytoalbuminologic dissociation was seen in only three (14.3%) cases. Nerve conduction studies were done and showed delayed F-wave in 19 (90.48%) cases and two (9.52%) cases were normal [Table 5].
IVIG was administered to 17 (80.95%) cases. Plasmapheresis was done to three (14.3%) cases, who did not respond well to IVIG. Four (19.05%) cases who presented in the improving phase of motor disability were given supportive care without IVIG or plasmapheresis and recovered completely [Table 6].
Respiratory insufficiency was found in six (28.6%) cases, requiring assisted ventilation [Table 7].
|Table 7: Comparison of demographic and clinical characteristics of cases with two other studies|
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Complete recovery was observed in 17 (80.95%) cases and four (19.05%) cases experienced only incomplete recovery [Figure 2]. In our hospital, the American Academy of Neurology guidelines for management of GBS are being followed to a great extent and with meticulous care and attention resulting in a favorable outcome.
| Discussion|| |
GBS is the most common cause of acute flaccid paralysis in children and infants after the eradication of polio. GBS has shown significant presence of preceding infection before its onset. In our study, history of preceding infection was found in 13 (61.9%) cases, with respiratory illness in most cases and diarrhea and nonspecific febrile illness in the remaining cases similar to Dhadke et al. and Sadek et al.. In our study, all cases presented with limb weakness at the onset of illness similar to Sadek et al. and in correspondence to Dhadke et al., who observed 13 of 40 cases in their study with sensory symptoms in the form of paresthesia, tingling, numbness, or pain. In our study, seven (33.3%) cases had cranial nerve involvement mostly in the form of chocking and absent gag reflex (bulbar affection) in correspondence to Dhadke et al., who found 62.5% of their cases showing cranial nerve involvement mostly the facial nerve. In our study, six (28.6%) cases developed respiratory insufficiency and required assisted ventilation similar to Kumar et al., who in their study found five (25%) cases suffering from respiratory insufficiency and requiring assisted ventilation. In our study, CSF analysis was done in six cases. Typical CSF finding of cytoalbuminologic dissociation was found in only three (14.3%) cases similar to Kumar et al., who in their study performed CSF analysis on five cases. Typical CSF finding was found in only two cases [Table 7].
| Conclusion|| |
IVIG and supportive care are considered the key elements in the management of GBS in children. IVIG administration early in the course of the disease results in better recovery. Plasmapheresis may be an effective option in cases with poor response to IVIG. More studies are needed to be conducted on childhood GBS, on a wider base of cases, to gain more information about the disease and its treatment options and to achieve better results.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6], [Table 7]