Guillain-Barr´e Syndrome in Arab Countries: A Systematic Review Hani T.S. Benamer, Amin Bredan PII: DOI: Reference:
S0022-510X(14)00369-4 doi: 10.1016/j.jns.2014.05.065 JNS 13242
To appear in:
Journal of the Neurological Sciences
Received date: Accepted date:
6 May 2014 29 May 2014
Please cite this article as: Benamer Hani T.S., Bredan Amin, Guillain-Barr´e Syndrome in Arab Countries: A Systematic Review, Journal of the Neurological Sciences (2014), doi: 10.1016/j.jns.2014.05.065
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Guillain-Barré Syndrome in Arab Countries: A Systematic Review
PT
Hani TS Benamer PhD, FRCP1,2, Amin Bredan PhD3,4
Neurology Department, New Cross Hospital, Wolverhampton, United Kingdom
2
Department Neurology, Queen Elizabeth Neuroscience Centre, Birmingham, United
RI
1
3
SC
Kingdom
Department of Biomedical Molecular Biology, Ghent University, 927 Technologiepark,
VIB Inflammation Research Center, 927 Technologiepark, 9052 Ghent, Belgium
MA
4
NU
9052 Ghent, Belgium
D
Correspondence to:
TE
Hani TS Benamer
Neurology Department, New Cross Hospital
AC CE P
Wolverhampton, United Kingdom WV 10 0QO
Tele: +44 1902 695055 Fax: +44 1902 695631
E-mail: [email protected]
1
ACCEPTED MANUSCRIPT Abstract Systematic review of the frequency and clinical pattern of Guillain-Barré syndrome (GBS)
PT
in Arab countries was initiated by a keyword search of PubMed, Medline and Embase
RI
and examination of references in all relevant papers. Seven articles were included from
SC
Iraq (n=1), Kuwait (n=1), Libya (n=2), Saudi Arabia (n=3). The only incidence report from the Arab world, a 1987 study from Libya, gives an incidence of 1.7 per 100,000 person-
NU
years. Some studies reported that GBS was more common in males and in people in
MA
their twenties and thirties. Five studies showed that GBS occurred more frequently during the colder months, and antecedent infection was reported in 26–76% of cases.
D
Most patients had an ascending pattern of weakness and almost all patients had lower
TE
limb weakness and reduced or absent reflexes. Facial weakness was the most common
AC CE P
cranial nerve involvement. Only one study classified the GBS patients according to electrophysiological findings and reported that 68% of the patients had demyelination type and 15% axonal type. Protein level in cerebrospinal fluid is elevated in most of GBS patients. Mortality rate from GBS was up to 8%. This systematic review shows an immense deficit in epidemiological data on GBS in Arab countries. The limited data show that clinical pattern, sex and age distribution, reported antecedent infection, and GBS subtypes are similar to those in Western countries. However, further well-designed epidemiological studies on GBS in the Arab world are needed. Key Words: Guillain-Barré syndrome, Epidemiology, Incidence, Clinical pattern, Arab countries, neuropathy
2
ACCEPTED MANUSCRIPT The Arab world covers a large geographical area spanning Africa and Asia and has an estimated population of 360 million. Though Arabs live in different countries and
PT
possess economic, demographic and social diversity, the populations of the Arab
RI
countries share, to a large extent, similar beliefs, customs, practices and social
SC
behaviour, which collectively constitute the Arab culture. Hence, they are generally considered as a cohesive unit. There has been a major improvement in living standards,
NU
education and health services over the past four decades in most Arab countries. Since
MA
the 2011 "Arab spring" some of the Arab countries have been going through major political changes that will have long-lasting effects on the region and repercussions
D
elsewhere.
TE
Guillain-Barré syndrome (GBS) is an acute peripheral neuropathy leading to a flaccid
AC CE P
paralysis that is thought to be due to an autoimmune reaction triggered by a preceding infection, mainly respiratory or gastrointestinal. Possible links between GBS and vaccination have also been debated1. GBS can be a demyelinating or an axonal neuropathy. The axonal type of GBS is up to 10 times more common in China, Japan and South America than in Europe and North America2. Therefore, determining the frequency and the pattern of GBS in different populations could help in understanding this condition. To our knowledge, no previous systematic reviews of GBS in Arab populations have been undertaken, and this study aimed to do just that. Methods
3
ACCEPTED MANUSCRIPT Arab countries are defined as members of the Arab league: Algeria, Bahrain, Comoros, Djibouti, Egypt, Iraq, Jordan, Kuwait, Lebanon, Libya, Mauritania, Morocco, Oman,
PT
Qatar, Saudi Arabia, Somalia, Sudan, Syria, Tunisia, United Arab Emirates, and Yemen.
RI
Publications on GBS in Arabs were identified by searching the keywords "Guillain-Barre"
SC
with “Specific Country Names” in PubMed, Medline (1946–date) and Embase (1974– date). References in all relevant papers were searched for additional publications.
NU
Using a clear and widely accepted case definition for GBS, only studies containing data
MA
on the frequency and clinical pattern of GBS published before March 30th 2014 and written in English were included. Studies on selected populations such as children and
D
those available only in abstract form were excluded.
TE
Results
AC CE P
The search identified 410 titles. After removal of duplicates, screening of the remaining 385 titles/abstracts led to exclusion of 372 of them. The remaining 13 publications were appraised in full. Seven articles fulfilled the inclusion criteria and were included in the study. These papers are from Iraq3 (n=1), Kuwait6 (n=1), Libya4, 5 (n=2), Saudi Arabia7-9 (n=3) (Table 1). Epidemiology There is only one incidence study, from Benghazi, Libya. It reported an incidence of 1.74 per 100,000 person-years and an age-adjusted incidence of 1.734. Seven studies provided information about age and gender of GBS patients (Table 1). GBS was more common in people in their twenties and thirties. Four studies showed no significant difference in the disease frequency between males and females4,
5, 8, 9
,
4
ACCEPTED MANUSCRIPT whereas the other three showed a higher frequency in males (average 75%)3, 6, 7 (Table 1).
PT
Seasonal Variation
RI
One study reported that there was no seasonal variation in incidence of GBS 5, another
SC
showed clustering of GBS cases during the early summer8, and five studies showed that more cases occurred during the colder months3, 4, 6, 7, 9.
NU
Antecedent Infection
MA
Antecedent infection (less than 4 weeks before onset of GBS) was reported in 26–76% of cases (Table 2). Five papers described the types of infection5-9. Respiratory infection was
D
the most common antecedent infection, as it was reported in 28–58% of cases5-9,
AC CE P
Clinical Features
TE
followed by gastrointestinal infection in 6–17%6, 7, 9.
Most patients had an ascending pattern of weakness and almost all patients had lower limb weakness and absent or reduced reflexes (Table 2). Facial weakness was the most common cranial nerve involvement, and 6–75% of the patients needed ventilation (Table 2). Autonomic disturbance was reported in 4–17% of the patients (Table 2). Laboratory Features Only
one
study
(from
Kuwait)
classified
the
GBS
patients
according
to
electrophysiological findings. It reported that 68% of the patients had demyelination type, 15% axonal type and 5% mixed type, whereas 10% had either normal nerve conduction or H reflex abnormality alone6.
5
ACCEPTED MANUSCRIPT Six papers reported the results of cerebrospinal fluid (CSF) analysis. Three of them showed elevated protein levels in about two-thirds of the patients6-8, whereas one study 5
and another in all the patients 4.
PT
reported high protein levels in 81% of the patients
RI
Three studies reported that none of their patients had an increased number of
SC
mononuclear cells in the CSF4, 5, 9, one study showed that 9% of the patients had 11 to 50 lymphocytes7, while the Kuwaiti study reported that 70% of the patients had no cells
NU
in the CSF and 22% had < 10 cells6.
MA
Treatment
In the Kuwaiti study, 95% of the patients received intravenous immunoglobulin (IVIG), of
D
whom 10% required two courses of IVIG and 13% needed plasma exchange (PE)6. In
TE
another study, 38% of the patients were treated with IVIG 5, and at two other centres
AC CE P
34%7 and 56%8 were treated with PE. In one of the Saudi Arabian studies, all the patients received IVIG treatment, two patients (17%) also received PE before IVIG, and one patient (8%) had two courses of IVIG9. Outcome
In one study, 92% of the patients recovered within 3 months8, while another study reported that 39% of patients remained disabled (wheel-chair-bound or using calipers and/or foot splints) after the last follow-up (mean duration of 11 months)7. A third study showed that 7% of the patients were disabled (wheel-chair-bound or very limited walking capacity) one year after disease onset4. Death due to GBS was reported in five studies. The rate varied from 0% in one study8 to 8% in two studies4, 9. The other two studies reported mortality rates of 4%7 and 6%5. 6
ACCEPTED MANUSCRIPT Discussion The estimated worldwide GBS incidence is 1.1–1.8 per 100,000 person-years10. Sejvar
PT
and colleagues, applying a regression model based on 16 population-based studies,
RI
reported a similar range (0.62–2.2 per 100,000 person-years) across all age groups in
SC
North America and Europe11. Our review shows that data on GBS incidence in the Arab countries are scanty. The only incidence report from the Arab world, from Benghazi,
NU
Libya, gives an incidence of 1.7 per 100,000 person-years, which is within the ranges
MA
reported elsewhere, but that study dates back to 1987. Therefore, no conclusion can be drawn on the frequency of GBS in the region.
D
Five studies showed that GBS is more frequent in males that in females3, 5-7, 9. The same
TE
pattern has been reported from other parts of the world11-13, but the reason for the
AC CE P
higher frequency of GBS in males is not clear11. Three studies showed that the mean age of GBS patients is in the twenties3-5 while two other studies showed a mean age in the forties6, 9. As some of the studies included only adults, methodological differences probably contributed to the differences in the mean ages of the GBS patients. Most studies on GBS from other parts of the world demonstrate a higher frequency of the disease in patients aged 20–30 years than in those above the age of 50 years12. The age distribution in Arab countries seems to point to a peak in the twenties. On average, 50% of GBS patients had antecedent infection4-9, most commonly in the respiratory tract. The link between antecedent infection and GBS is well recognised 10, so Arab countries are not different. But retrospective studies tend to under-report preceding infection relative to prospective studies10, and most studies from Arab 7
ACCEPTED MANUSCRIPT countries are retrospective. Notably, one study reported an antecedent infection rate of 76%9, but that study was a single-author paper describing 12 GBS patients treated by
PT
the author9. Therefore, it is not surprising that the reported preceding infection rate was
RI
high.
SC
The clinical patterns of GBS in Arab countries, such as the patterns of motor weakness, sensory involvement, absent reflexes and cranial nerve involvement are not different
NU
from the classic features of GBS. However, one series showed that most patients (75%)
MA
needed ventilation 9. The reason for that is not clear, but it is possible that only severe cases where referred to the reporting centre. It is well recognised that there are
D
regional differences in the frequencies of GBS subtypes such as acute inflammatory
TE
demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy, and acute
AC CE P
motor-sensory axonal neuropathy2. However, only one study (from Kuwait) reported GBS subtypes, in which AIPD was the most common variant6, consistent with reports from Europe and North America2. All Arab centres treated the GBS patients with the standard methods, such as IVIG and/or PE, and the outcome resembles that in other regions in the world.
In conclusion, this systematic review shows an immense deficit in epidemiological data on GBS in Arab countries. The limited data show that clinical pattern, sex and age distribution, reported antecedent infection, and GBS subtypes are similar to those in Western countries. However, further well-designed epidemiological studies on GBS in the Arab world are needed. Such research would enhance our understanding of this important neurological disorder in the Arab world. 8
ACCEPTED MANUSCRIPT
Country
Published year
Radhakrishnan et al4
Libya
1987
Abduljabbar8
Saudi Arabia
1991
Bahou et al7
Saudi Arabia
1996
El Zunni et al5
Libya
1997
Alzaidi & Nouri3
Iraq
2002
Nagarajan & Al-Shubaili6
Kuwait
2006
Dahbour9
Saudi Arabia
2009
Study type
Diagnostic criteria
RI
Study
PT
Table 1. The Guillain-Barré syndrome studies from various Arab countries.
SC
Three years of intensive search of neuromuscular disorders (1983-1985)
PT ED
MA
NU
Retrospective analysis of records of patients with GBS admitted to a single hospital (1982-1990) Analysis of all clinical records of all GBS patients admitted to a single hospital (1984-1994) Analysis of all clinical records of all GBS patients admitted to a single hospital (1994-1995) Cross-sectional study of patients with GBS admitted to 5 neurology centres Analysis of all clinical records of all GBS patients admitted to neurology tertiary centre (1997-2003) All patients with GBS admitted to a single hospital (1999-2004)
Number of cases Male Female
Mean age (range) in years
NINCDS
13
14
27.9 (7-60)
NINCDS
11
14
(15-65)*
Asbury
37
10
(0.5-81)*
Asbury
9
7
29 (18-68)
Asbury
38
12
21.1 (1-60)
Asbury
30
11
41 (16-72)
Not stated
7
5
45 (7-75)
AC
CE
NINCDS = National Institute of Neurological and Communicative Disorders and Stroke, GBS = Guillain-Barré syndrome. * Mean not given.
9
ACCEPTED MANUSCRIPT
lower limbs
NR
NR 100 94 100 NR
NR
32 53 56 NR
NR 85
72 85
100 100
80
96
100
48 75 NR
66
91
98
98
56
76
NR
100
100
50
100
Facial weakness
Ophthalmoplegia
Other
Ventilator support
30
NR
NR
15
40
12 64
20
56 64
4
6 40
26 6 NR
51
10
15
17
42
75
Autonomic disturbance Urinary Labile bladder BP disturbance NR 8 17 NR NR 8
4 15 NR NR 2 12
NR
NR
AC
CE
PT ED
BP = blood pressure, NR = not reported.
98
Cranial nerve involvement
SC
26
upper limbs
NU
Radhakrishnan et al4 Abduljabbar8 Bahou et al7 El Zunni et al5 Alzaidi & Nouri3 Nagarajan & AlShubaili6 Dahbour9
Ascending pattern
Absent of reduced tendon Reflexes
MA
Study
Sensory involvement
RI
Motor weakness Antecedent infection
PT
Table 2. The clinical features of Guillain-Barré syndrome amongst Arabs (data presented as percentages).
10
ACCEPTED MANUSCRIPT References
AC CE P
TE
D
MA
NU
SC
RI
PT
1. Haber P, Sejvar J, Mikaeloff Y, DeStefano F. Vaccines and Guillain-Barré syndrome. Drug Saf 2009;32:309-323. 2. Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet 2005;366:16531666. 3. Alzaidi MA, Nouri KA. Guillain-Barré syndrome Neurosciences (Riyadh) 2002;7:176-178. 4. Radhakrishnan K, el-Mangoush MA, Gerryo SE. Descriptive epidemiology of selected neuromuscular disorders in Benghazi, Libya. Acta NeurolScand 1987;75:95-100. 5. Elzunni S, Prakash P, Saiti K, Busnaina I. Guillain Barré Syndrome (GBS): an appraisal. Central African Journal of Medicine 1997;43:99-103. 6. Nagarajan V, Al-Shubaili A. Clinical and neurophysiological pattern of GuillainBarre syndrome in Kuwait. Med PrincPract 2006;15:120-125. 7. Bahou YG, Biary N, al DS. Guillain-Barre syndrome: a series observed at Riyadh Armed Forces Hospital January 1984--January 1994. J Neurol 1996;243:147-152. 8. AbdulJabbar MS. Pattern of Guillain-Barré syndrome in saudi Arabia. Journal of Tropical and Geographical Neurology 1991;1:35-38. 9. Dahbour SS. Clinical experience with Gullain Barre syndrome over a 6-year period in one hospital in the Middle East. Jordan Med J 2009;43:280-285. 10. McGrogan A, Madle GC, Seaman HE, de Vries CS. The epidemiology of Guillain-Barré syndrome worldwide. A systematic literature review. Neuroepidemiology 2009;32:150-163. 11. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011;36:123-133. 12. Franklin GM. Peripherial Neuropathy. In: Nelson LM, Tanner CM, Van Den Eeden SK, McGuire VM, eds. Neuroepildemiology: From Principles to Practice: Oxford University Press, 2004: 279-302. 13. Bharucha NE, ward CD. Peripheral neuropathy. In: Feigin VL, Bennett DA, eds. Handbook of Clinical Neuroepidemiology. New York: Nova Science Publishers, 2007: 233-300.
11
S0022-510X(14)00369-4 doi: 10.1016/j.jns.2014.05.065 JNS 13242
To appear in:
Journal of the Neurological Sciences
Received date: Accepted date:
6 May 2014 29 May 2014
Please cite this article as: Benamer Hani T.S., Bredan Amin, Guillain-Barr´e Syndrome in Arab Countries: A Systematic Review, Journal of the Neurological Sciences (2014), doi: 10.1016/j.jns.2014.05.065
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Guillain-Barré Syndrome in Arab Countries: A Systematic Review
PT
Hani TS Benamer PhD, FRCP1,2, Amin Bredan PhD3,4
Neurology Department, New Cross Hospital, Wolverhampton, United Kingdom
2
Department Neurology, Queen Elizabeth Neuroscience Centre, Birmingham, United
RI
1
3
SC
Kingdom
Department of Biomedical Molecular Biology, Ghent University, 927 Technologiepark,
VIB Inflammation Research Center, 927 Technologiepark, 9052 Ghent, Belgium
MA
4
NU
9052 Ghent, Belgium
D
Correspondence to:
TE
Hani TS Benamer
Neurology Department, New Cross Hospital
AC CE P
Wolverhampton, United Kingdom WV 10 0QO
Tele: +44 1902 695055 Fax: +44 1902 695631
E-mail: [email protected]
1
ACCEPTED MANUSCRIPT Abstract Systematic review of the frequency and clinical pattern of Guillain-Barré syndrome (GBS)
PT
in Arab countries was initiated by a keyword search of PubMed, Medline and Embase
RI
and examination of references in all relevant papers. Seven articles were included from
SC
Iraq (n=1), Kuwait (n=1), Libya (n=2), Saudi Arabia (n=3). The only incidence report from the Arab world, a 1987 study from Libya, gives an incidence of 1.7 per 100,000 person-
NU
years. Some studies reported that GBS was more common in males and in people in
MA
their twenties and thirties. Five studies showed that GBS occurred more frequently during the colder months, and antecedent infection was reported in 26–76% of cases.
D
Most patients had an ascending pattern of weakness and almost all patients had lower
TE
limb weakness and reduced or absent reflexes. Facial weakness was the most common
AC CE P
cranial nerve involvement. Only one study classified the GBS patients according to electrophysiological findings and reported that 68% of the patients had demyelination type and 15% axonal type. Protein level in cerebrospinal fluid is elevated in most of GBS patients. Mortality rate from GBS was up to 8%. This systematic review shows an immense deficit in epidemiological data on GBS in Arab countries. The limited data show that clinical pattern, sex and age distribution, reported antecedent infection, and GBS subtypes are similar to those in Western countries. However, further well-designed epidemiological studies on GBS in the Arab world are needed. Key Words: Guillain-Barré syndrome, Epidemiology, Incidence, Clinical pattern, Arab countries, neuropathy
2
ACCEPTED MANUSCRIPT The Arab world covers a large geographical area spanning Africa and Asia and has an estimated population of 360 million. Though Arabs live in different countries and
PT
possess economic, demographic and social diversity, the populations of the Arab
RI
countries share, to a large extent, similar beliefs, customs, practices and social
SC
behaviour, which collectively constitute the Arab culture. Hence, they are generally considered as a cohesive unit. There has been a major improvement in living standards,
NU
education and health services over the past four decades in most Arab countries. Since
MA
the 2011 "Arab spring" some of the Arab countries have been going through major political changes that will have long-lasting effects on the region and repercussions
D
elsewhere.
TE
Guillain-Barré syndrome (GBS) is an acute peripheral neuropathy leading to a flaccid
AC CE P
paralysis that is thought to be due to an autoimmune reaction triggered by a preceding infection, mainly respiratory or gastrointestinal. Possible links between GBS and vaccination have also been debated1. GBS can be a demyelinating or an axonal neuropathy. The axonal type of GBS is up to 10 times more common in China, Japan and South America than in Europe and North America2. Therefore, determining the frequency and the pattern of GBS in different populations could help in understanding this condition. To our knowledge, no previous systematic reviews of GBS in Arab populations have been undertaken, and this study aimed to do just that. Methods
3
ACCEPTED MANUSCRIPT Arab countries are defined as members of the Arab league: Algeria, Bahrain, Comoros, Djibouti, Egypt, Iraq, Jordan, Kuwait, Lebanon, Libya, Mauritania, Morocco, Oman,
PT
Qatar, Saudi Arabia, Somalia, Sudan, Syria, Tunisia, United Arab Emirates, and Yemen.
RI
Publications on GBS in Arabs were identified by searching the keywords "Guillain-Barre"
SC
with “Specific Country Names” in PubMed, Medline (1946–date) and Embase (1974– date). References in all relevant papers were searched for additional publications.
NU
Using a clear and widely accepted case definition for GBS, only studies containing data
MA
on the frequency and clinical pattern of GBS published before March 30th 2014 and written in English were included. Studies on selected populations such as children and
D
those available only in abstract form were excluded.
TE
Results
AC CE P
The search identified 410 titles. After removal of duplicates, screening of the remaining 385 titles/abstracts led to exclusion of 372 of them. The remaining 13 publications were appraised in full. Seven articles fulfilled the inclusion criteria and were included in the study. These papers are from Iraq3 (n=1), Kuwait6 (n=1), Libya4, 5 (n=2), Saudi Arabia7-9 (n=3) (Table 1). Epidemiology There is only one incidence study, from Benghazi, Libya. It reported an incidence of 1.74 per 100,000 person-years and an age-adjusted incidence of 1.734. Seven studies provided information about age and gender of GBS patients (Table 1). GBS was more common in people in their twenties and thirties. Four studies showed no significant difference in the disease frequency between males and females4,
5, 8, 9
,
4
ACCEPTED MANUSCRIPT whereas the other three showed a higher frequency in males (average 75%)3, 6, 7 (Table 1).
PT
Seasonal Variation
RI
One study reported that there was no seasonal variation in incidence of GBS 5, another
SC
showed clustering of GBS cases during the early summer8, and five studies showed that more cases occurred during the colder months3, 4, 6, 7, 9.
NU
Antecedent Infection
MA
Antecedent infection (less than 4 weeks before onset of GBS) was reported in 26–76% of cases (Table 2). Five papers described the types of infection5-9. Respiratory infection was
D
the most common antecedent infection, as it was reported in 28–58% of cases5-9,
AC CE P
Clinical Features
TE
followed by gastrointestinal infection in 6–17%6, 7, 9.
Most patients had an ascending pattern of weakness and almost all patients had lower limb weakness and absent or reduced reflexes (Table 2). Facial weakness was the most common cranial nerve involvement, and 6–75% of the patients needed ventilation (Table 2). Autonomic disturbance was reported in 4–17% of the patients (Table 2). Laboratory Features Only
one
study
(from
Kuwait)
classified
the
GBS
patients
according
to
electrophysiological findings. It reported that 68% of the patients had demyelination type, 15% axonal type and 5% mixed type, whereas 10% had either normal nerve conduction or H reflex abnormality alone6.
5
ACCEPTED MANUSCRIPT Six papers reported the results of cerebrospinal fluid (CSF) analysis. Three of them showed elevated protein levels in about two-thirds of the patients6-8, whereas one study 5
and another in all the patients 4.
PT
reported high protein levels in 81% of the patients
RI
Three studies reported that none of their patients had an increased number of
SC
mononuclear cells in the CSF4, 5, 9, one study showed that 9% of the patients had 11 to 50 lymphocytes7, while the Kuwaiti study reported that 70% of the patients had no cells
NU
in the CSF and 22% had < 10 cells6.
MA
Treatment
In the Kuwaiti study, 95% of the patients received intravenous immunoglobulin (IVIG), of
D
whom 10% required two courses of IVIG and 13% needed plasma exchange (PE)6. In
TE
another study, 38% of the patients were treated with IVIG 5, and at two other centres
AC CE P
34%7 and 56%8 were treated with PE. In one of the Saudi Arabian studies, all the patients received IVIG treatment, two patients (17%) also received PE before IVIG, and one patient (8%) had two courses of IVIG9. Outcome
In one study, 92% of the patients recovered within 3 months8, while another study reported that 39% of patients remained disabled (wheel-chair-bound or using calipers and/or foot splints) after the last follow-up (mean duration of 11 months)7. A third study showed that 7% of the patients were disabled (wheel-chair-bound or very limited walking capacity) one year after disease onset4. Death due to GBS was reported in five studies. The rate varied from 0% in one study8 to 8% in two studies4, 9. The other two studies reported mortality rates of 4%7 and 6%5. 6
ACCEPTED MANUSCRIPT Discussion The estimated worldwide GBS incidence is 1.1–1.8 per 100,000 person-years10. Sejvar
PT
and colleagues, applying a regression model based on 16 population-based studies,
RI
reported a similar range (0.62–2.2 per 100,000 person-years) across all age groups in
SC
North America and Europe11. Our review shows that data on GBS incidence in the Arab countries are scanty. The only incidence report from the Arab world, from Benghazi,
NU
Libya, gives an incidence of 1.7 per 100,000 person-years, which is within the ranges
MA
reported elsewhere, but that study dates back to 1987. Therefore, no conclusion can be drawn on the frequency of GBS in the region.
D
Five studies showed that GBS is more frequent in males that in females3, 5-7, 9. The same
TE
pattern has been reported from other parts of the world11-13, but the reason for the
AC CE P
higher frequency of GBS in males is not clear11. Three studies showed that the mean age of GBS patients is in the twenties3-5 while two other studies showed a mean age in the forties6, 9. As some of the studies included only adults, methodological differences probably contributed to the differences in the mean ages of the GBS patients. Most studies on GBS from other parts of the world demonstrate a higher frequency of the disease in patients aged 20–30 years than in those above the age of 50 years12. The age distribution in Arab countries seems to point to a peak in the twenties. On average, 50% of GBS patients had antecedent infection4-9, most commonly in the respiratory tract. The link between antecedent infection and GBS is well recognised 10, so Arab countries are not different. But retrospective studies tend to under-report preceding infection relative to prospective studies10, and most studies from Arab 7
ACCEPTED MANUSCRIPT countries are retrospective. Notably, one study reported an antecedent infection rate of 76%9, but that study was a single-author paper describing 12 GBS patients treated by
PT
the author9. Therefore, it is not surprising that the reported preceding infection rate was
RI
high.
SC
The clinical patterns of GBS in Arab countries, such as the patterns of motor weakness, sensory involvement, absent reflexes and cranial nerve involvement are not different
NU
from the classic features of GBS. However, one series showed that most patients (75%)
MA
needed ventilation 9. The reason for that is not clear, but it is possible that only severe cases where referred to the reporting centre. It is well recognised that there are
D
regional differences in the frequencies of GBS subtypes such as acute inflammatory
TE
demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy, and acute
AC CE P
motor-sensory axonal neuropathy2. However, only one study (from Kuwait) reported GBS subtypes, in which AIPD was the most common variant6, consistent with reports from Europe and North America2. All Arab centres treated the GBS patients with the standard methods, such as IVIG and/or PE, and the outcome resembles that in other regions in the world.
In conclusion, this systematic review shows an immense deficit in epidemiological data on GBS in Arab countries. The limited data show that clinical pattern, sex and age distribution, reported antecedent infection, and GBS subtypes are similar to those in Western countries. However, further well-designed epidemiological studies on GBS in the Arab world are needed. Such research would enhance our understanding of this important neurological disorder in the Arab world. 8
ACCEPTED MANUSCRIPT
Country
Published year
Radhakrishnan et al4
Libya
1987
Abduljabbar8
Saudi Arabia
1991
Bahou et al7
Saudi Arabia
1996
El Zunni et al5
Libya
1997
Alzaidi & Nouri3
Iraq
2002
Nagarajan & Al-Shubaili6
Kuwait
2006
Dahbour9
Saudi Arabia
2009
Study type
Diagnostic criteria
RI
Study
PT
Table 1. The Guillain-Barré syndrome studies from various Arab countries.
SC
Three years of intensive search of neuromuscular disorders (1983-1985)
PT ED
MA
NU
Retrospective analysis of records of patients with GBS admitted to a single hospital (1982-1990) Analysis of all clinical records of all GBS patients admitted to a single hospital (1984-1994) Analysis of all clinical records of all GBS patients admitted to a single hospital (1994-1995) Cross-sectional study of patients with GBS admitted to 5 neurology centres Analysis of all clinical records of all GBS patients admitted to neurology tertiary centre (1997-2003) All patients with GBS admitted to a single hospital (1999-2004)
Number of cases Male Female
Mean age (range) in years
NINCDS
13
14
27.9 (7-60)
NINCDS
11
14
(15-65)*
Asbury
37
10
(0.5-81)*
Asbury
9
7
29 (18-68)
Asbury
38
12
21.1 (1-60)
Asbury
30
11
41 (16-72)
Not stated
7
5
45 (7-75)
AC
CE
NINCDS = National Institute of Neurological and Communicative Disorders and Stroke, GBS = Guillain-Barré syndrome. * Mean not given.
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lower limbs
NR
NR 100 94 100 NR
NR
32 53 56 NR
NR 85
72 85
100 100
80
96
100
48 75 NR
66
91
98
98
56
76
NR
100
100
50
100
Facial weakness
Ophthalmoplegia
Other
Ventilator support
30
NR
NR
15
40
12 64
20
56 64
4
6 40
26 6 NR
51
10
15
17
42
75
Autonomic disturbance Urinary Labile bladder BP disturbance NR 8 17 NR NR 8
4 15 NR NR 2 12
NR
NR
AC
CE
PT ED
BP = blood pressure, NR = not reported.
98
Cranial nerve involvement
SC
26
upper limbs
NU
Radhakrishnan et al4 Abduljabbar8 Bahou et al7 El Zunni et al5 Alzaidi & Nouri3 Nagarajan & AlShubaili6 Dahbour9
Ascending pattern
Absent of reduced tendon Reflexes
MA
Study
Sensory involvement
RI
Motor weakness Antecedent infection
PT
Table 2. The clinical features of Guillain-Barré syndrome amongst Arabs (data presented as percentages).
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ACCEPTED MANUSCRIPT References
AC CE P
TE
D
MA
NU
SC
RI
PT
1. Haber P, Sejvar J, Mikaeloff Y, DeStefano F. Vaccines and Guillain-Barré syndrome. Drug Saf 2009;32:309-323. 2. Hughes RA, Cornblath DR. Guillain-Barré syndrome. Lancet 2005;366:16531666. 3. Alzaidi MA, Nouri KA. Guillain-Barré syndrome Neurosciences (Riyadh) 2002;7:176-178. 4. Radhakrishnan K, el-Mangoush MA, Gerryo SE. Descriptive epidemiology of selected neuromuscular disorders in Benghazi, Libya. Acta NeurolScand 1987;75:95-100. 5. Elzunni S, Prakash P, Saiti K, Busnaina I. Guillain Barré Syndrome (GBS): an appraisal. Central African Journal of Medicine 1997;43:99-103. 6. Nagarajan V, Al-Shubaili A. Clinical and neurophysiological pattern of GuillainBarre syndrome in Kuwait. Med PrincPract 2006;15:120-125. 7. Bahou YG, Biary N, al DS. Guillain-Barre syndrome: a series observed at Riyadh Armed Forces Hospital January 1984--January 1994. J Neurol 1996;243:147-152. 8. AbdulJabbar MS. Pattern of Guillain-Barré syndrome in saudi Arabia. Journal of Tropical and Geographical Neurology 1991;1:35-38. 9. Dahbour SS. Clinical experience with Gullain Barre syndrome over a 6-year period in one hospital in the Middle East. Jordan Med J 2009;43:280-285. 10. McGrogan A, Madle GC, Seaman HE, de Vries CS. The epidemiology of Guillain-Barré syndrome worldwide. A systematic literature review. Neuroepidemiology 2009;32:150-163. 11. Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011;36:123-133. 12. Franklin GM. Peripherial Neuropathy. In: Nelson LM, Tanner CM, Van Den Eeden SK, McGuire VM, eds. Neuroepildemiology: From Principles to Practice: Oxford University Press, 2004: 279-302. 13. Bharucha NE, ward CD. Peripheral neuropathy. In: Feigin VL, Bennett DA, eds. Handbook of Clinical Neuroepidemiology. New York: Nova Science Publishers, 2007: 233-300.
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