Study Objectives: Guillain-Barre syndrome (GBS) is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that often follows an infection. The outcome and prognosis of GBS depend on many factors such as the etiology, clinical features, neurophysiology and immunological parameters. The aim of this study was to assess the factors (clinical, investigatory tools, and therapies) that may affect the outcome of patients with GBS. Patients and methods: this was an analytical observational study that was conducted at Ain Shams university hospitals and Kobri Elkoba Military Hospital including twenty patients with the diagnosis of Guillain Barre Syndrome in the duration from 2016 to 2018. This study included twenty patients with the diagnosis of GBS within two weeks from onset of neurologic symptoms, whom their diagnosis based on the established clinical criteria and verified by investigations. Patients were selected from both genders and aged from 18 to 65 years old. Nerve conduction studies and electromyography were performed within two weeks from admission. Various lines of treatment such as plasma exchange (PE), intravenous immunoglobulins (IVIG) or both were used during the period of admission in hospital. Outcome was assessed by the Hughes functional score (F-score), that was applied to the patients on admission, at end of 4 weeks from onset of neuropathy and at the end of 8 weeks. The final outcome at the end of 8 weeks was classified as follow: Group I: good prognosis (0 - 2) on the Hughes functional score (15 patients) and Group II: poor prognosis (3 - 6) on the Hughes functional score (5 patients). Results: the age of the study population ranged from 18 to 65 years with mean of 36.10 ± 16.08 years. Fifteen (75%) patients were males and 5 (25%) patients were females. There was no statistically significant difference found between poor and good prognosis regarding gender. The most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Most patients (75%) had a good outcome at end of study period. It was found that the different line of treatment administered (plasma exchange or IVIG or both) was not associated with poor or good outcome. The patients who needed mechanical ventilation had significantly poor prognosis. Conclusion: the most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Ascending pattern of weakness was more common than descending pattern in this study population and was not related to prognosis. High Hughes score at admission was associated with poor outcome at 8 weeks.
Guillain Barré syndrome (GBS) is an acute postinfectious immune mediated peripheral neuropathy characterized by rapidly progressive weakness and sensory loss, usually followed by slow clinical recovery [
This is an analytical observational study that was conducted at Ain Shams university hospitals and Kobri Elkoba Military Hospital including twenty patients with the diagnosis of GBS in the duration from May 2016 to December 2018 after the patients or their relatives signed an informed consent. Inclusion criteria included patients from both genders aged from 16 to 70 years old who were diagnosed as GBS within two weeks from onset of neurologic symptoms, depending on the history, clinical examination and investigatory tools.
Exclusion criteria included patients with other causes of polyneuropathy such asdiabetic, uremic, hypothyroidism, drug-related neuropathy, para neoplastic neuropathy or hereditary neuropathy.
All patients were subjected to the following; a full medical, neurological history and the history of any antecedent events occurring during the few weeks before the onset of neurologic symptoms. Full Neurological examination at the time of presentation and laboratory investigations; Random blood sugar, Complete blood picture, Serum urea, Serum creatinine, Serum electrolytes (sodium, potassium, calcium), Liver function tests, Erythrocyte sedimentation rate, and C-reactive protein (quantitative). Nerve conduction studies and electromyography were performed within two weeks from admission. Motor and sensory nerve conduction studies (NCS) and F wave response were done for the median and ulnar nerves in both upper limbs, posterior tibial and common peroneal in both lower limbs and sensory conduction for sural nerve. Electromyography (EMG) study was done for motor units to the abductor pollicus brevis, deltoid and triceps muscles in upper limbs, and tibialis anterior and gastrocnemius in the lower limbs. Various lines of treatment such as PE, IVIG or both were received during the period of admission in hospital. Outcome was assessed by the Hughes functional score (F-score), that applied to the patients on admission, at end of 4 weeks from onset of neuropathy and at the end of 8 weeks for follow up and to assess outcome [
All data collected were tabled and statistically analyzed by Microsoft Office 2003 (excel) and Statistical Package for Social Science (SPSS) version 16. Parametric data were expressed as mean ± SD, and non-parametric data were expressed as number and percentage of the total. Comparing the mean ± SD of 2 groups was done using the paired student’s t-test. P value < 0.05 is considered significant.
The prognosis of patients after 8 weeks of from onset of symptoms was classified as good or favorable prognosis (Hughes score < 3) in 15 patients (75.0%) of the study population and poor or unfavorable prognosis (Hughes score ≥ 3) in 5 patients (25.0%).
The age of the study population ranged from 18 to 65 years with mean of 36.10 ± 16.08 years. 15 patients (75%) were males and 5 patients (25%) were females (
As regards the clinical characteristics of the study population, 16 patients (80.0%) had preceding respiratory tract infection, 3 patients (15.0%) had preceding gastrointestinal tract infection and 1 patient (5.0%) had preceding both respiratory and gastrointestinal tracts infection (
Total No. = 20 | ||
---|---|---|
Gender | Female | 5 (25.0%) |
Male | 15 (75.0%) | |
Age | Mean ± SD | 36.10 ± 16.08 |
Range | 18 - 65 | |
16 - 39 | 12 (60.0%) | |
40 - 59 | 4 (20.0%) | |
60 - 70 | 4 (20.0%) |
No. (%) | ||
---|---|---|
Infection preceding illness | RTI | 16 (80.0%) |
GE | 3 (15.0%) | |
GE and RTI | 1 (5.0%) | |
Pattern of weakness | Ascending | 16 (80.0%) |
Descending | 4 (20.0%) | |
First symptom | Sensory | 5 (25.0%) |
Motor | 3 (15.0%) | |
Motor and sensory | 12 (60.0%) |
RTI; respiratory tract infection, GE; gastroenteritis.
Symptoms | Total No. = 20 | |
---|---|---|
Sensory | No | 3 (15.0%) |
Yes | 17 (85.0%) | |
Motor | No | 0 (0.0%) |
Yes | 20 (100.0%) | |
Autonomic | No | 16 (80.0%) |
Yes | 4 (20.0%) |
The duration between the preceding infection and onset of symptoms ranged from 6 to 15 days with mean 9.35 ± 2.35 days. The duration between the onset of first symptoms and starting the treatment ranged from 2 to 12 days with mean of 5.15 ± 2.89 days. The total duration of admission to hospital ranged from 1 to 14 weeks with mean 4.30 ± 2.52 weeks. This duration was less than 4 weeks in 15 patients (75.0%) and more than 4 weeks in 5patients (25.0%). Only 4 patients (20.0%) needed mechanical ventilation and among those patients the duration lapsed between the first symptoms and mechanical ventilation ranged from 2 to 11 days with median (IQR) 3.00 (2.5 - 7). This duration was less than 3 days in 3 patients (75.0%) from ventilated patients and 3 or more days in one patient (25.0%) from ventilated patients (
As regards the data of NCS and EMG, 3 patients (15.0%) had evidence of axonal motor neuropathy, 5 patients (25.0%) had evidence of demyelinating motor and sensory neuropathy while 12 (60.0%) had evidence of mixed demyelinating and axonal motor neuropathy. All of the patients had abnormal F-wave (
Total No. = 20 | ||
---|---|---|
Time between infection and illness (days) | Mean ± SD | 9.35 ± 2.35 |
Range | 6 - 15 | |
<7 day | 5 (25.0%) | |
≥7 day | 15 (75.0%) | |
Time starting medication from onset of first symptoms (days) | Mean ± SD | 5.15 ± 2.89 |
Range | 2 - 12 | |
Duration stay in hospital (weeks) | Mean ± SD | 4.30 ± 2.52 |
Range | 1 - 14 | |
<4 weeks | 15 (75.0%) | |
≥4 weeks | 5 (25.0%) | |
Mechanical ventilation | No | 16 (80.0%) |
Yes | 4 (20.0%) | |
Time between first symptom and mechanical ventilation (days) | Median (IQR) | 3.00 (2.5 - 7) |
Range | 2 - 11 | |
<3 | 3 (75.0%) | |
≥3 | 1 (25.0%) |
No. (%) | ||
---|---|---|
NCV and EMG | Demyelinating motor and sensory | 5 (25.0%) |
Axonal motor neuropathy | 3 (15.0%) | |
Both | 12 (60.0%) | |
F-wave | Abnormal | 20 (100.0%) |
As regards Hughes scale of the studied population, on presentation one patient (5.0%) was Grade 1, 2 patients (10.0%) was Grade 2, 6 patients (30.0%) was Grade 3, 8 patients (40.0%) was Grade 4 and 3 patients (15.0%) was grade 5. After 4 weeks from onset of symptoms 3 patients (15.0%) was Grade 1, 8 patients (40.0%) was Grade 2, 5 patients (25.0%) was Grade 3, 3 patients (15.0%) was Grade 4 and one patient (5.0%) was Grade 6. After 8 weeks from onset of symptoms 5 patients (25.0%) was grade 0, 8 patients (40.0%) was Grade 1, 2 patients (10.0%) was grade 2, 3 patients (15.0%) was Grade 3, one patient (5.0%) was grade 4 and one patient (5.0%) was grade 6 (
As regards the treatments received during period of admission, 10 patients (50.0%) received plasma exchange sessions, 4 patients (20.0%) received IVIG only and 6 patients (30.0%) received plasma exchange session then followed by IVIG due to unsatisfactory response after sessions. The sessions of PE ranged from 3 to 9 sessions with mean of 5.44 ± 1.55 sessions. 11 patients (68.8%) received less than 5 plasma exchange sessions while 5 patients (31.3%) received 5 or more sessions (
Grade | No. (%) | |
---|---|---|
Hughes scale at presentation | Grade 1 | 1 (5.0%) |
Grade 2 | 2 (10.0%) | |
Grade 3 | 6 (30.0%) | |
Grade 4 | 8 (40.0%) | |
Grade 5 | 3 (15.0%) | |
Hughes scale at 4 weeks | Grade 1 | 3 (15.0%) |
Grade 2 | 8 (40.0%) | |
Grade 3 | 5 (25.0%) | |
Grade 4 | 3 (15.0%) | |
Grade 6 | 1 (5.0%) | |
Hughes scale at 8 weeks | Grade 0 | 5 (25.0%) |
Grade 1 | 8 (40.0%) | |
Grade 2 | 2 (10.0%) | |
Grade 3 | 3 (15.0%) | |
Grade 4 | 1 (5.0%) | |
Grade 6 | 1 (5.0%) |
Total No. = 20 | ||
---|---|---|
Mode or treatment | PE | 10 (50.0%) |
IVIG | 4 (20.0%) | |
PE and IVIG | 6 (30.0%) | |
Sessions of PE | Mean ± SD | 5.44 ± 1.55 |
Range | 3 - 9 | |
<5 sessions | 11 (68.8%) | |
≥5 sessions | 5 (31.3%) |
The prognosis of patients after 8 weeks of from onset of symptoms was classified as group I; good or favorable prognosis (Hughes score < 3) in 15 patients (75.0%) of the study population and group II; poor or unfavorable prognosis (Hughes score ≥ 3) in 5 patients (25.0%) (
There was no statistically significant difference found between both groups regarding gender and age of the study population. But it was noticed that patients with age ranging from 16 to 39 years showed significantly good or favorable prognosis compared to those with age ranging from 40 - 59 or ≥60 years (
There was no significant difference between both groups regarding infection preceding illness, the pattern of weakness either ascending or descending and the nature of first symptoms (
Prognosis | Total No. = 20 |
---|---|
Group I (good) (Hughes score < 3) | 15 (75.0%) |
Group II (poor) (Hughes score ≥ 3) | 5 (25.0%) |
Total | 20 (100%) |
Group I | Group II | Test Value | P-Value | Sig. | ||
---|---|---|---|---|---|---|
No. = 15 | No. = 5 | |||||
Gender | Female | 5 (33.3%) | 0 (0.0%) | 2.222 | 0.136 | NS |
Male | 10 (66.7%) | 5 (100.0%) | ||||
Age | Mean ± SD | 33.93 ± 15.92 | 42.60 ± 16.47 | 1.046 | 0.309 | NS |
Range | 18 - 65 - 3 | 19 - 65 | ||||
16 - 39 | 11 (73.3%) | 1 (20.0%) | 7.111 | 0.029 | S | |
40 - 59 | 1 (6.7%) | 3 (60.0%) | ||||
≥60 | 3 (20.0%) | 1 (20.0%) |
Group I | Group II | Test Value | P-Value | Sig. | ||
---|---|---|---|---|---|---|
No. (%) | No. (%) | |||||
Infection preceding illness | RTI | 13 (86.7%) | 3 (60.0%) | 3.444 | 0.179 | NS |
GE | 1 (6.7%) | 2 (40.0%) | ||||
GE and RTI | 1 (6.7%) | 0 (0.0%) | ||||
Pattern of weakness | Ascending | 12 (80.0%) | 4 (80.0%) | 1.852 | 0.172 | NS |
Descending | 3 (20.0%) | 1 (20.0%) | ||||
First symptom | Sensory | 5 (33.3%) | 0 (0.0%) | 2.222 | 0.329 | NS |
Motor | 2 (13.3%) | 1 (20.0%) | ||||
Motor and sensory | 8 (53.3%) | 4 (80.0%) |
There was no significant difference between both groups regarding Sensory symptoms and autonomic symptoms. All of the patients of good and poor prognosis had motor symptoms (
The comparison between both groups showed that the patients who needed mechanical ventilation had significantly poor prognosis; among 4 patients who needed mechanical ventilation, 3 patients had poor prognosis and among 5 patients with poor prognosis, 3 (60.0%) of them were mechanically ventilated, while among 15 patients with good prognosis, 14 (93.3%) of them did not need mechanical ventilation. For those who needed mechanical ventilation, 3 patients were mechanically ventilated within the first two days from the onset of first symptoms and these patients had significantly poor prognosis, while one patient only was mechanically ventilated after 2 days from onset of symptoms and this patient had good prognosis. Also patients who started treatment earlier tended to have significant good prognosis; the duration between the onset of first symptoms and starting the treatment ranged from 2 to 5 days with mean 2.80 ± 1.30 days among patients with good prognosis while it ranged from 2 to 12 days with mean of 5.93 ± 2.87 days among patients with poor prognosis. Also patients who stayed at hospital during treatment for less than 4 weeks had significant good prognosis; among 15 patients with good prognosis, 13 (86.7%) of them stayed at hospital for less than 4 weeks while among 5 patients with poor prognosis, 3 (60.0%) of them stayed at hospital 4 weeks or more. There was no significant difference between patients with good and poor prognosis as regarding duration between preceding infection and illness (
There was no significant difference between patients with good and poor prognosis as regarding NCS and EMG findings (
The comparison between the two groups as regards Hughes scale done at presentation showed that group II patients had significantly higher scores as all of them had either Grade 4 or Grade 5, while group I patients had variable scores ranging from grade 1 to grade 4 and none had Grade 5 score. As regards Hughes scale done at 4 weeks from onset of symptoms, group I patients had score ranging from 1 to 3 while those of group II had scores ranging from 3 to 6 (
Regarding modes of treatment and number of sessions of plasma exchange there was no statistically significant differences between both groups (
Symptoms | Group I | Group II | Test Value | P-Value | Sig. | |
---|---|---|---|---|---|---|
No. =15 | No. = 5 | |||||
Sensory | No | 2 (13.3%) | 1 (10.0%) | 0.131 | 0.718 | NS |
Yes | 13 (86.7%) | 4 (80.0%) | ||||
Motor | No | 0 (0.0%) | 0 (0.0%) | - | - | - |
Yes | 15 (100.0%) | 5 (100.0%) | ||||
Autonomic | No | 13 (86.7%) | 3 (60.0%) | 1.667 | 0.197 | NS |
Yes | 2 (13.3%) | 2 (40.0%) |
Group I | Group II | Test Value | P-Value | Sig. | ||
---|---|---|---|---|---|---|
No. = 15 | No. = 5 | |||||
Time between infection and illness (days) | Mean ± SD | 9.60 ± 1.67 | 9.27 ± 2.58 | 0.268 | 0.791 | NS |
Range | 6 - 15 | 8 - 12 | ||||
<7 day | 5 (33.3%) | 0 (0.0%) | 2.222 | 0.136 | NS | |
≥7 day | 10 (66.7%) | (100.0%) | ||||
Time starting medication from onset of first symptoms (days) | Mean ± SD | 2.80 ± 1.30 | 5.93 ± 2.872 | −2.333 | 0.031 | S |
Range | 2 - 5 | 2 - 12 | ||||
Duration stay in hospital (weeks) | Mean ± SD | 3.73 ± 0.70 | 6.00 ± 4.85 | 1.854 | 0.080 | NS |
Range | 3 - 5 | 1 - 14 | ||||
<4 weeks | 13 (86.7%) | 2 (40.0%) | 4.356 | 0.037 | S | |
≥4 weeks | 2 (13.3%) | 3 (60.0%) | ||||
Mechanical ventilation | No | 14 (93.3%) | 2 (40.0%) | 6.667 | 0.010 | S |
Yes | 1 (6.7%) | 3 (60.0%) | ||||
Time between first symptom and mechanical ventilation (days) | Median (IQR) | 11 (11 - 11) | 3 (2 - 3) | -1.414≠ | 0.157 | NS |
Range | 11 - 11 | 2 - 3 | ||||
<3 | 0 (0.0%) | 3 (100.0%) | 4.000 | 0.046 | S | |
≥3 | 1 (100.0%) | 0 (0.0%) |
Group I | Group II | Test Value* | P-Value | Sig. | ||
---|---|---|---|---|---|---|
No. (%) | No. (%) | |||||
NCV and EMG | Demyelinating motor and sensory | 5 (33.3%) | 0 (0.00%) | 2.222 | 0.329 | NS |
Axonal motor polyneuropathy | 2 (13.3%) | 1 (20.0%) | ||||
Both | 8 (53.3%) | 4 (80.0%) | ||||
F-wave | Abnormal | 15 (100.0%) | 5 (100.0%) | – | – | – |
Group I | Group II | Test Value* | P-Value | Sig. | |||
---|---|---|---|---|---|---|---|
No. (%) | No. (%) | ||||||
Hughes scale at presentation | Grade 1 | 1 (6.7%) | 0 (0.0%) | 12.000 | 0.017 | S | |
Grade 2 | 2 (13.3%) | 0 (0.0%) | |||||
Grade 3 | 6 (40.0%) | 0 (0.0%) | |||||
Grade 4 | 6 (40.0%) | 2 (40.0%) | |||||
Grade 5 | 0 (0.0%) | 3 (60.0%) |
Hughes scale at 4 weeks | Grade 1 | 3 (20.0%) | 0 (0.0%) | 15.733 | 0.003 | HS |
---|---|---|---|---|---|---|
Grade 2 | 8 (53.3%) | 0 (0.0%) | ||||
Grade 3 | 4 (26.7%) | 1 (20.0%) | ||||
Grade 4 | 0 (0.0%) | 3 (60.0%) | ||||
Grade 6 | 0 (0.0%) | 1 (20.0%) | ||||
Hughes scale at 8 weeks | Grade 0 | 5 (33.3%) | 0 (0.0%) | 20.000 | 0.001 | HS |
Grade 1 | 8 (53.3%) | 0 (0.0%) | ||||
Grade 2 | 2 (13.3%) | 0 (0.0%) | ||||
Grade 3 | 0 (0.0%) | 3 (60.0%) | ||||
Grade 4 | 0 (0.0%) | 1 (20.0%) | ||||
Grade 6 | 0 (0.0%) | 1 (20.0%) |
Group II | Group I | Test Value | P-Value | Sig. | ||
---|---|---|---|---|---|---|
No. = 15 | No. = 5 | |||||
Mode or treatment | PE | 9 (60.0%) | 1 (20.0%) | 3.200 | 0.202 | NS |
IVIG | 3 (20.0%) | 1 (20.0%) | ||||
PE and IVIG | 3 (20.0%) | 3 (60.0%) | ||||
Number of session of PE | Mean ± SD | 5.67 ± 1.61 | 4.75 ± 1.26 | -1.028 | 0.322 | NS |
Range | 3 - 9 | 3 - 6 | ||||
<5 | 8 (66.7%) | 3 (75.0%) | 0.097 | 0.755 | NS | |
≥5 | 4 (33.3%) | 1 (25.0%) |
GBS is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that follows an antecedent infection [
The aim of this study was to assess the factors (clinical, investigatory tools, and therapies) that may affect the outcome of patients with GBS.
In this study twenty patients with the diagnosis of Guillain Barre Syndrome within two weeks from onset of neurologic symptoms were included, whom their diagnosis based on the established clinical criteria and verified by investigations. Patients were selected from both genders and aged from 18 to 65 years old. NCS and EMG were performed within two weeks from admission. In this study various lines of treatment such as PE, IVIG or both were received during the period of admission in hospital. Outcome was assessed on the Hughes functional score (F-score), that was applied to the patients on admission, at end of 4 weeks from onset of neuropathy and at the end of 8 weeks and the final outcome (either good or poor prognosis) was at the end of 8 weeks as follow; Group I: 15 patients with good prognosis (0 - 2) on the Hughes functional score and Group II: 5 patients with poor prognosis (3 - 6) on the Hughes functional score; 5 patients.
The results of this study showed no statistical significant difference between the studied groups as regard demographic data; sex and age. However, the group age ranging from 16 to 39 years reported significantly favorable prognosis. Similar findings were reported by van Koningsveld et al. (2007) and Munayco et al. (2019) [
This study has some limitations which have to be taken into consideration. First, the sample size was relatively small, resulting in low statistical power for detecting significant differences between two groups. Second, non-usage of recent immunological tests was for the different subtypes of GBS as, IgG anti GT1b, anti GD1b, anti GM1, anti GM2. Finally, the complications should be evaluated for a long time. However, the strengths of this study were the correlation between the clinical, investigation modalities; EMG & NCT and functional outcome assessment in different modalities of treatment in GBS for better prognostic evaluation.
GBS is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that often follows an antecedent infection. This study included twenty patients with the diagnosis of GBS within two weeks from onset of neurologic symptoms, whom their diagnosis based on the established clinical criteria and verified by investigations. Nerve conduction studies and electromyography were performed within two week from admission. In this study various lines of treatment such as plasma exchange, IVIG or both were received during the period of admission in hospital. In this study, an older age group was a predictor of poor outcome. An antecedent infection was seen in all cases as gastroenteritis and respiratory tract infection. Ascending pattern of weakness was more common than descending pattern in this study population and was not related to final outcome. The most common electrophysiological subtype was demyelinating type followed by axonal neuropathy type. High Hughes score at admission was associated with poor outcome at 8 weeks. In this study, various lines of treatment such as PE, IVIG or both showed a similar outcome. Mechanical ventilation was associated with poor outcome at 8 weeks. Long Duration stay in hospital was a predictor of poor outcome. Thus early diagnosis, early management in high-quality ICU to avoid complications and the use of PE or IVIG or both in GBS are of utmost important.
The authors declare no conflicts of interest regarding the publication of this paper.
Alloush, T., Fahmy, N.A., Fouad, M.M., Albaroudy, H.O., Hamdy, M. and Salem, H.H. (2019) Prediction of Outcome in Patients with Guillain Barre Syndrome—An Egyptian Study. Neuroscience & Medicine, 10, 232-246. https://doi.org/10.4236/nm.2019.103018