Description of Guillain-Barre syndrome on the basis of clinical features using Hughes scoring system among children in Karachi, Pakistan
Prem Chand M.D. , Farida Jan, Sidra Kaleem, Mohammad Tahir Yousafzai, Shahnaz Ibrahim
Department of Paediatrics and Child Health, Aga Khan University Hospital, Karachi, Pakistan
|Date of Web Publication||28-Apr-2017|
Department of Paediatrics and Child Health, Aga Khan University, Hospital, Karachi
Source of Support: None, Conflict of Interest: None
Background: Guillain-Barre syndrome (GBS) is an acquired inflammatory polyneuropathy characterized by rapidly progressive symmetrical flaccid limb weakness and areflexia. Here, we aimed to describe GBS on the basis of clinical features using Hughes scoring system (HSS) in children.
Methods: We conducted a descriptive study, retrieving medical records of children between 2–16 years old admitted with GBS during January 2011–December 2013 at Aga Khan University Hospital, Karachi. Information on demographics, predisposing factors of GBS, clinical features at presentation, investigations, managements, short- and long-term outcomes were recorded on data extraction sheet. Ethical approval was obtained before data collection.
Results: Totally 31 children with GBS (21 males) were admitted during the study period. The mean age was 6.7 years. Thirteen cases were seen in summer (January–October) followed by 11 in spring (March–May) and 7 in winter (November–February). Preceding illnesses including upper respiratory tract infections in 15 and diarrhea was seen in 4 patients. None of the patients had history of prior immunization. The nerve conduction study/electromyography showed acute inflammatory demyelinating polyradiculoneuropathy in 18 (58%), acute motor axonal neuropathy in 8 (25.8%), acute motor and sensory axonal neuropathy in 3 (9.7%) and Miller Fisher syndrome in 2 (6.5%) patients. Twenty-one patients had received intravenous immunoglobulin, four had plasmapharesis, four had both while two patients received none of these. Ventilator support was required by seven patients. Tracheostomy was performed on two patients. The HSS was calculated at 3-month follow-up. Nineteen children (61.2%) had an HSS score of 0–1, eight had a score of 2–5 (25.8%), and four patients were lost to follow-up.
Conclusion: HSS is a good tool to identify and follow children with GBS. More than two-thirds of the patients had recovered complete mobility at 3-month follow-up.
Keywords: Guillain-Barre syndrome; Hughes scoring system; Medical Research Council scale; intravenous immunoglobulins; plas-mapharesis; outcome
|How to cite this article:|
Chand P, Jan F, Kaleem S, Yousafzai MT, Ibrahim S. Description of Guillain-Barre syndrome on the basis of clinical features using Hughes scoring system among children in Karachi, Pakistan. Asia Pac J Clin Trials Nerv Syst Dis 2017;2:45-9
|How to cite this URL:|
Chand P, Jan F, Kaleem S, Yousafzai MT, Ibrahim S. Description of Guillain-Barre syndrome on the basis of clinical features using Hughes scoring system among children in Karachi, Pakistan. Asia Pac J Clin Trials Nerv Syst Dis [serial online] 2017 [cited 2018 Jan 22];2:45-9. Available from: http://www.actnjournal.com/text.asp?2017/2/2/45/205193
| Introduction|| |
Guillain-Barre syndrome (GBS), is an acquired inflammatory polyneuropathy characterized by flaccid limb weakness and albuminocytologic dissociation in cerebrospinal fluid (CSF), it remains a potentially disabling condition (Shafqat et al., 2006; Lee et al., 2008). It is characterized by rapidly progressive, essentially symmetric weakness and areflexia (Hiraga et al., 2003; Lee et al., 2008). It is believed to be an immune response to an antecedent event (The Italian Guillain-Barré Study Group, 1996; Ryan, 2005). Approximately two-thirds of patients report a respiratory or gastrointestinal illness in the weeks preceding onset of symptoms (van Koningsveld et al., 2007). Many viruses and bacteria have been implicated; the strongest association is with the bacterium Campylobacter jejuni (Rosen, 2012).
With the widespread eradication of poliomyelitis, GBS is the most common cause of acute and sub-acute flaccid paralysis in infants and children (Rosen, 2012). GBS occurs all year round and can occur at any age but it is rare in children under the age of 2 years (The Italian Guillain-Barré Study Group, 1996; Rosen, 2012). Adults are affected more commonly than children. The incidence in children is lower, with estimates between 0.4 to 1.3 cases per 100,000 person-year.
GBS is divided into four sub types those with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN) and Miller Fisher syndrome (MFS) (Rosen, 2012). In a study from Korea which showed that, according to clinical and electrophysiological findings occurrence of AIDP is 60.7%, AMAN is 25.0%, and MFS is 12.5% and AMSAN is 1.79% (Lee et al., 2008).
Prognosis is usually good, but residual motor and sensory deficits may occur (van Koningsveld et al., 2007; Lee et al., 2008). Nonetheless, about 20% of patients die from the complications of GBS or remain severely disabled (Hahn, 1998; van Doorn, 2005). Outcome from GBS is presumably determined by the extent of nerve damage in the acute phase and the capacity to recover in the convalescent phase (van Koningsveld et al., 2007). The functional grading scale for GBS, Hughes scoring system (HSS), is ranked as grade 0, healthy; grade 1, minor symptoms or signs, able to run; grade 2, able to walk without assistance but unable to run; grade 3, able to walk with assistance; grade 4, bed- or chair-bound; grade 5, requiring assisted ventilation for at least part of the day; and grade 6, dead (Fletcher et al., 2000). The objectives of this study are to observe short- and long-term outcomes of GBS according to HSS.
| Methods|| |
Study design, setting, and data archival
This was a retrospective review of hospital records for the patients with GBS admitted during January 2011 to December 2013 into the pediatrics department of the Aga Khan University (AKUH), Karachi, Pakistan. The AKUH is a 643 bedded Joint Commission International accredited private tertiary care hospital located in the metropolitan city of Karachi. There are 113 beds allocated exclusively for the pediatric patients. The hospital has about 63,000 admissions per year. The catchment population of AKUH is very wide and it caters not only to Karachi but it is also a referral center for the patients from other adjacent cities and parts of the country. The hospital provides zakat and welfare for the needy patients, allowing the lower income families to visit the hospital also.
All the patients visiting AKUH (both inpatients and outpatients) for the first time are allocated a unique medical record (MR) number. All the subsequent visits of the patients and their medical/surgical and laboratory/radiological data are archived using the unique MR number. The people at the registration desk make sure that the visit is registered by using the existing unique MR number and no duplication is made. In case the patient did not bring the MR number card or forgot the MR number, the concerned registration clerks retrieve the MR number using the date of birth, mobile phone numbers and surname registered at the time of initial registration. Therefore, the probability of having duplicate records is quite rare at the AKUH. All the records are archived using the unique MR number and international classification of disease 9 (ICD-9) coding system.
Data retrieval and recording
We used the ICD-9 code of 357.0 to retrieve the records of children aged 2–16 years admitted with GBS during January 1, 2011 to December 30, 2013. The list of eligible patients; first name and last name, date of birth, date of admission, confirmed diagnosis, and MR number was populated from the electronic medical record systems. Subsequently, this list was used to retrieve the medical record file of each individual manually. We used a structured proforma to extract data from each file. The chart reviews were conducted for the following information; 1) demographic variables included age, sex, and residence; 2) duration of admission and patient outcome; 3) time of onset (season); 4) diagnosis, differential diagnosis and clinical manifestations; 5) complications before and during admission; 6) investigations done including nerve conduction studies/electromyography; 7) history of vaccination and any illness prior to the onset of this event; 8) Hughes functional grading scale scores at admission, discharge and at 3-month follow-up; 9) the Medical Research Council (MRC) grading for power at admission, discharge and at 3-month follow-up; 10) ventilator support and duration of ventilator support. Hughes functional classification scores at three different time points were calculated by using clinical information available in the medical records. The Hughes functional classification score range from 0 to 6. A score of 0 means healthy individual and 6 means death. Detail description of each score is provided in [Table 1].
Data was collected using paper based questionnaires. After filling in the forms, the data collector (trainee resident) and supervisor (Principal Investigator, PI) reviewed the form for missing information and any illogical responses. Any error or missing information was corrected real time by consulting the medical record file or electronic medical record system. Data was kept confidential within locked cabinets and electronic data was password protected with only access to the PI.
The data was entered into the SPSS 20.0 software (IBM Corp., Armonk, NY, USA). Frequency with percentages were calculated for all the categorical variables such as signs and symptoms, complications and Hughes classification score at admission, discharge and 3-month follow-up across different types of GBS.
Exemption from ethical approval was obtained from the ethical review committee (ERC) of the Aga Khan University (2828-Ped- ERC-13). Informed consent was waived due to the retrospective nature of the study. Retrospective health facility level data collection was restricted to obtaining de-identified information. No personal identification information was collected or stored and all data was aggregated for reporting. All personal identifiers were removed from the electronic data.
| Results|| |
There were 33 children with GBS admitted during January 1, 2011 to December 30, 2013. AIDP was the most common type occurring in 18 (58%) patients followed by AMAN (8 (26%)), then AMSAN (3 (10%)) and MFS (2 (7%)). Overall slightly higher than two-thirds of the patients (21 (68%)) were male. Average age of the patients was 6.8 ± 3.7 years. The age of patients with MFS was highest (mean 11 ± 4.24 years old), and the age of AMSAN patients was lowest (mean 4.10 ± 3.38 years old). Slightly less than half of the patients (45%) were between 5–<10 years old and 23% were between 10–16 years old. 42% of the patients suffered during summer season (June–October), followed by spring (36%) and winter (23%). Limb weakness and areflexia were present among all patients at the time of presentation. Slightly less than three-quarters of the patients (71%) presented with paraesthesia, followed by ataxia (65%) and muscle pain (55%). Almost half of the patients (48%) had a history of upper respiratory tract infection (URTI) prior to the onset of these symptoms [Table 2]. There was no history of prior immunization.
|Table 2: Sociodemographic characteristics and clinical presentation of the Guillain-Barre syndrome patients|
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Complications among GBS patients were shown in [Table 3]. Overall, 32% of the patients developed dysphagia/dysarthria, followed by cranial nerve palsies (19%), respiratory difficulty (16%), opthalmoplegia (7%) and autonomic instability and urinary problems (6.5% each). The average hospital stay among patients was 9 days. Patients with MFS has the longest hospital stay (18 days), followed by AIDP (9 days) and AMAN (7 days) and AMSAN (5 days).
|Table 3: Distribution of complications among patients with Guillain-Barre syndrome|
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Distribution of HFGS scores and MRC sum scores at admission, discharge and 3-month follow-up are shown in [Table 4]. Overall the score was highest for MFS and lowest for the AMSAN. At discharge, the mean HFGS score decreased to 2.90 with highest score of 3.0 among patients with MFS, AMSAN, AMAN and lowest score of 2.83 among patients with AIDP. We also calculated the HFGS score at 3-month follow-up. Except four patients who were lost to follow-up, the mean HFGS score was significantly improved with the highest score of 1.50 among patients with MFS and lowest score of 0.56 among patients with AIDP. Similarly, we calculated the MRC sum of score at admission, discharge and 3-month follow-up. The total score increased from 2.35 at admission to 3.26 at discharge and 4.50 at 3-month follow-up.
|Table 4: HFGS and MRC sum scores in our patients at admission, discharge and follow-up|
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| Discussion|| |
The incidence of GBS and its relation to seasonal variation can be seen in our study with predominance in the summer months closely followed by spring with AIDP being the most common subtype in all seasons. While Lee et al. (2008) reports a higher incidence in summer and fall. Upper respiratory tract infection preceded GBS in almost half of our patientsand with diarrhea in less than 13%. A total of 61% patients had an antecedent illness which is comparable to the Indian study by Dhadke et al. (2013), where the figure was 55%. Male/female ratio of preponderance was apparent with 2.1:1, which is consistent with studies by Dhadke et al. (2013) and Lee et al. (2008).
Limb weakness was prevalent in all the patients at presentation which is a consistent finding in most subtypes. Cranial nerve involvement is seen more in children than in adults. In our study, cranial nerve involvement was up to 74%, with dysphagia/dysarthria present in 32%, facial nerve involvement in 19%, slurred speech in 16% and ophthalmoplegia in 6% of the children. Paraesthesia was the third most common presenting complain to ccurring in 71% of patients. Muscle pain was prevalent in 55% of the patients, most common in AIDP subtype. Autonomic instability was seen in just two patients in our group.
Hughes functional scoring employed for our patients and there was considerable improvement. i.e., 89% of patients with AIDP and 50% with AMAN were at score 0–2 at 3-month follow-up. There was no difference between the two subtypes in age, ventilation requirement at the acute stage and functional status on recovery. These findings are therefore consistent with the Korean study (Lee et al., 2008).
Lee et al. (2008) showed in their study that out of 40% children with GBS who had become non-ambulant during their illness, 90% of them started walking by 1–4 months after onset. 72% of children could walk independently at 1 year after onset. Our indices were similar to theirs as in our last follow-up which was at 3 months, 25 (80%) of children could walk independently.
AMAN subtype was seen in 26% of our children which are comparable to the Korean study who had 25% (Lee et al., 2008). This figure is less than the western countries but is nearer to the indices from the eastern countries. MFS which is rarer in children had 6% cases in our study which is consistent with other studies where it is seen much less than other subtypes but is more prevalent in eastern than in western countries (Garrett and Ryan, 2002).
The functional status of patients with AIDP and AMAN at the time of presentation, at the peak of symptoms and then in recovery did not differ much. This is actually comparable to the Korean study where a similar conclusion has been drawn in their pediatric patients (Garrett and Ryan, 2012).
Our study was done at a tertiary care hospital with good intensive care support where the patients with respiratory insufficiency promptly. There were no deaths in our patients although 7 (22%) patients required ventilatory support in the acute phase of the disease due to respiratory failure and the duration of ventilation was also variable amongst them but with an overall good outcome and no complications.
Intravenous immunoglobulin were administered to almost two third of our patients and a small proportion received Plasmapharesis. Beside these, four of our patients received both the modalities. The choice of treatment was more dependent on the cost of each modality and the results were similar.
| Conclusion|| |
GBS is more common in males than females. History of preceding infection was seen in 61% patients with the mean age of 6.7 years. Most common clinical presentation was limb weakness followed by paraesthesias. Most common subtype diagnosed on NCV was AIDP, closely followed by AMAN. Approximately one-fourth of our patients required ventilation due to respiratory failure. Bigger chunk of our study group received intravenous immunoglobulins with quick recovery but a small proportion had to have plasmapharesis as well. More than two-thirds of the patients had recovered mobility by 3 months.
| References|| |
Dhadke SV, Dhadke VN, Bangar SS, Korade MB (2013) Clinical profile of Guillain Barre syndrome. J Assoc Physicians India 61:168-172.
Fletcher DD, Lawn ND, Wolter TD, Wijdicks EF (2000) Long-term outcome in patients with Guillain-Barre syndrome requiring mechanical ventilation. Neurology 54:2311-2315.
Garrett J, Ryan P
(2002) A child with Miller Fisher syndrome. J Pediatr Child Health 38:414-416.
Hahn AF (1998) Guillain-Barré syndrome. Lancet 352:635-641.
Hiraga A, Mori M, Ogawara K, Hattori T, Kuwabara S (2003) Differences inpatterns of progression in demyelinating and axonal Guillain-Barresyndromes. Neurology 61:471-474.
Lee JH, Sung IY, Rew IS (2008) Clinical presentation and prognosis of childhood Guillain-Barré syndrome. J Paediatr Child Health 44:449-454.
Rosen BA (2012) Guillain-Barré syndrome. Pediatr Rev 33:164-171.
Ryan MM (2005) Guillain-Barre syndrome in childhood. J Paediatr Child Health 41:237-241.
Shafqat S, Khealani BA, Awan F, Abedin SE (2006) Guillain-Barré syndrome in Pakistan: similarity of demyelinating and axonal variants. Eur J Neurol 13:662-665.
The Italian Guillain-Barré Study Group (1996) The prognosis and main prognostic indicators of Guillain-Barré syndrome. A multicenter prospective study of 297 patients. Brain 119:2053-2061.
van Doorn PA (2005) Treatment of Guillain-Barré syndrome and CIDP. J Peripher Nerv Syst 10:113-127.
van Koningsveld R, Steyerberg EW, Hughes RA, Swan AV, van Doorn PA, Jacobs BC (2007) A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol 6:589-594.
Conflicts of interest
Conception, design, manuscript writing: PC. Data collection and manuscript writing: FJ and SK. Data analysis and interpretation: MTY. Data analysis and manuscript writing: SI.
This paper was screened twice using CrossCheck to verify originality before publication.
This paper was double-blinded and stringently reviewed by international expert reviewers.
[Table 1], [Table 2], [Table 3], [Table 4]