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IS GUILLAIN-BARRE SYNDROME DIFFERENT IN PAKISTAN?

Byline: Waseem Iqbal, Tahir Mukhtar Sayed, Wasim Wali, Nadeem Ahmed, Aamir Waheed Butt and Zaheer Ahmad Gill

ABSTRACT

Objective: To assess clinical presentations and subtypes of Guillain-Barre Syndrome (GBS) in Pakistan.

Study Design: Retrospective study.

Place and Duration of Study: CMH Lahore, Abbotabad, Quetta and Armed Forces Institute of Rehabilitation Medicine form Jan 2007 to Feb 2015.

Material and Methods: The relevant history, demographic features, clinical presentations and subtypes of GBS in 211 patients fulfilling the clinical and electrodiagnostic criteria were investigated.

Results: The average age of the patients was 37.36 years (62.7% M, 37.3% F). Clinically 66.8%, 17.8% and 15.4% presented as ascending paralysis, simultaneous quadriparesis and paraparesis respectively. About 38.1% of the patients presented with cranial nerves involvement, 87.4% presented with areflexia while 59.5% reported pain. The GBS subtypes identified were acute inflammatory demyelinating polyneuropathy (21.9%), acute motor axonal neuropathy (38.9%), acute motor sensory axonal neuropathy (35.6%) and fisher syndrome (3.6%). Conclusion: The GBS presents in young, as an ascending paralysis, main subtypes of GBS in Pakistan are axonal and this highlights the importance of local management guidelines and preventive medicine.

Keywords: Acute inflammatory demyelinating polyneuropathy, Acute motor axonal neuropathy, Acute motor sensory axonal neuropathy, Fisher syndrome, Guillain-Barre syndrome.

INTRODUCTION

Guillain-Barre Syndrome (GBS) is an autoimmune inflammatory polyneuropathy presenting classically as a rapid, progressive ascending paralysis with global areflexia1,2. It involves the peripheral nervous system including motor, sensory and autonomic components. The typical history involves neurological symptoms a few weeks following an acute illness in most of the patients. The diagnosis of GBS is based upon history, relevant clinical examination, albumin-cytological dissociation in cerebrospinal fluid and characteristic electrophysiological studies3. The disease has clinically indistinguishable axonal, demyelinating or mixed types4. In a typical GBS history, there is a preceding history of infection5-7, followed by weakness, which commonly involves the lower limbs initially. On the other hand, there is sometimes involvement of the upper limbs or facial muscles in the early course of the disease.

Clinical examination typically reveals absent or impaired deep tendon reflexes with motor and sensory system involvement. The primary pathology may be demyelinating with secondary axonal or, demyelinating features may follow significant axonal loss on electrophysiological studies8.

Acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN) and Fisher syndrome (FS) are three different variants of GBS described in the literature in addition to the classic acute inflammatory demyelinating polyneuropathy (AIDP). Initially, GBS was considered to be a demyelinating type of polyneuropathy9-12 but later, with the introduction of electrophy-siological studies in clinical practice, various subtypes were identified. The geographical distribution of various electrophysiological subtypes of GBS has been described in the literature13-15, AIDP5,6,16 being the most common as followed by AMAN17,18 AMSAN19 and Fisher Syndrome (FS)20. Fewer studies have been conducted on subtypes of GBS in Pakistan21-24 and there is no study in literature as regards to the clinical presentation of GBS in Pakistan.

As long-term, follow-up studies had shown different recovery patterns and pathophysiology in subtypes of GBS25,26 it is important to identify the various subtypes, keeping in mind the diagnostic, therapeutic and financial implications.

With this background, this study was conducted in the Rehabilitation Medicine and Neurology Departments of the Combined Military Hospital (CMH) Lahore, the Combined Military Hospital Abbottabad, the Combined Military Hospital Quetta and the Armed Forces Institute of Rehabilitation Medicine, Rawalpindi between January 2007 to February 2015, in order to identify various clinical presentations and subtypes of GBS in Pakistan.

Table-I: Clinical diagnostic criteria for Guillain-Barre Syndrome27.

Required Features

- Progressive weakness in both arms and legs

- Areflexia (or hyporeflexia)

Features Supportive of Diagnosis

- Progression of symptoms over days to 4 weeks

- Relative symmetry

- Mild sensory signs or symptoms

- Cranial nerve involvement, especially bilateral facial weakness

- Recovery beginning 2 to 4 weeks after progression ceases

- Autonomic dysfunction

- Absence of fever at onset

- Typical CSF and EMG/nerve conduction studies features.

Features Casting Doubt on the Diagnosis

- Asymmetrical weakness

- Persistent bladder and bowel dysfunction

- Bladder or bowel dysfunction at onset

- >50 mononuclear leukocytes/mm3 or presence of polymorphonuclear leukocytes in CSF

- Distinct sensory level

Features That Rule Out the Diagnosis

- Hexacarbon abuse

- Abnormal porphyria metabolism, recent diphtheria infection

- Lead intoxication

- Other similar conditions: poliomyelitis, botulism, hysterical paralysis, toxic neuropathy

MATERIAL AND METHODS

We included all the patients presenting with acute progressive weakness, areflexia, and albuminocytological dissociation in the cerebrospinal fluid fulfilling the clinical criteria of Asbury et al27 and electrodiagnostic criteria of Hadden et al28 for GBS. The clinical and electrophysiological assessments were done by neurophysicians and physiatrists. Based upon available prevalence rate of GBS in international studies and confidence level 95%, 211 patients were inducted in the study.

The clinical features documented included the history of preceding infection, clinical examination including presentation and area of weakness, examination of deep tendon reflexes (DTR), sensory impairments, the autonomic nervous system, bladder involvement and pain. The motor weakness was documented as ascending, paraplegia or simultaneous development of weakness in upper and lower limbs. DTR were recorded as absent, depressed, or exaggerated. Sensory impairment included hyperesthesia, dysesthesia or tingling sensation. Autonomic nervous system involvement was considered when the patient presented with bradycardia, tachycardia, flushes, orthostatic hypotension or altered sweating. Cranial nerve involvement was documented when the patient presented with facial weakness, double vision or difficulty in speech or swallowing. The pain was documented as neuropathic, nociceptive or visceral, keeping in view the type or character of the pain.

Bladder involvement was considered when there was incontinence or urinary retention (table-I).

Table-II: Electrophysiological classification of Guillain-Barre Syndrome28.

- At least 3 sensory nerves and 3 motor nerves with multi-site stimulation F-waves, and bilateral

###tibial H-reflexes, need to be evaluated.

- At least one of the following in each of at least 2 nerves, or at least 2 of the following in one

###nerve if all others inexcitable and distal compound muscle action potential (dCMAP) >10%

###lower limit of normal (LLN)

- AIDP:

###Motor conduction velocity <90% LLN (85% if dCMAP 110% upper limit of normal (ULN) (>120% if dCMAP <100% LLN)

###P CMAP/dCMAP ratio 20% LLN

###F-response latency >120% ULN

- AMSAN:

###None of the features of AIDP except one demyelinating feature allowed in one nerve if dCMAP

###<10% LLN

###Sensory action potential amplitudes less than LLN

- AMAN:

###None of the features of AIDP except one demyelinating feature allowed in one nerve if dCMAP

###<10% LLN

###Sensory action potential amplitudes normal

- Inexcitable:

- dCMAP absent in all nerves or present in only one nerve with dCMAP 32AdegC. The values for each variable were compared with the upper and lower limits of normative values for our laboratory and age-adjusted for child cases. All measured segments of nerves (including entrapment sites) were included for the purpose of comparing the different classification criteria. The data were analysed using SPSS v20.

Statistical analyses, where required, were performed using Fisher's exact test and chi-square tests. Demographic data, including age and gender, and other variables including subtypes of GBS are expressed in terms of percentages.

RESULTS

A total of 211 patients (62.7% M, 37.3% F) with GBS were studied with an average age of 35.96 years (range 8-67 years; SD 15.575). On history, 136 (64.45%) of the patients remembered an infective ailment prior to the development of this condition.

The most common clinical presentation was ascending paralysis (66.35%), followed by tingling sensation with weakness in 65.4% of the patients, while 18.09% of the patients reported simultaneous upper and lower limb weakness and, 15.63% of the patients presented with paraparesis. About 38.1% presented with some kind of cranial nerves involvement, 87.4% of the patients presented with absent reflexes, 9.7% presented with depressed reflexes, while 2.8% had hyperreflexia. Autonomic nervous system involvement was observed in 68.4% of patients.

Table-III: Clinical presentations.

Clinical Presentations###Frequency###Percentage (%)

Ascending Weakness###140###66.35

Upper / Lower Limb Weakness###38###18.09

Paraparesis###33###15.63

Total###211###100.0

Table-IV: GBS Subtypes.

###Frequency###Percentage (%)

AIDP###46###21.8

AMAN###82###38.86

AMSAN###75###35.54

FS###8###3.79

Total###211###100.0

The pain of any type was reported by 59.5% of the patients, of these; 52% had pain related to movement, while 48% described it as dysesthesia. Bladder involvement was reported by 17.8% of patients. The clinical presentations and GBS subtypes identified are shown in table-III and IV respectively.

DISCUSSION

GBS is the commonest cause of acute flaccid paralysis (AFP) worldwide5,6,16. The disability caused by the condition is variable in severity and duration depending upon the type and severity25,29. The diagnosis of this condition is of utmost importance but in a country like Pakistan, where resources are scarce, it becomes even more important due to the likely heavy financial impact. As different treatment regimens are advocated for different subtypes of GBS, it is very important to distinguish between the subtypes in the early course of the disease. Moreover, identification of the subtype also helps in long-term rehabilitation planning due to expected variations in recovery pattern30.

In the first place, the confirmation of the diagnosis of GBS is vital, as poliomyelitis is still prevalent in Pakistan and presentation of any flaccid paralysis in a paediatric population generates panic, because of the possibility that it could be poliomyelitis. Sometimes desperate measures taken to manage the condition lead to patients being managed by unqualified practitioners. As diagnostic facilities are not very easily accessible, especially in rural areas, the quackery and spiritual healing methods often lead to irreversible nervous system damage in the absence of appropriate timely management. Secondly, in the case of an axonal subtype of GBS, the patient will need a longer stay in the hospital. For poor people, because the health system in Pakistan involves considerable "out of pocket" expenditure, the financial burden of treatment is exceptionally high.

In this study, we observed that there is a clear difference in the types of GBS in Pakistan in comparison to Western data. Our study is consistent with a few other studies carried out in Eastern populations, especially in China which revealed a higher frequency of axonal variants17,18,21,28,31. Our results show that it is likely that patients will have a longer inpatient stay, due to the high prevalence of axonal variants in the Pakistani population. There is dire need of early treatment with immunoglobulin therapy as advocated by other studies32-34. An investigation carried out by Yuqin et al in 2007 and a few other studies revealed a strong association of the axonal subtype of GBS with infectious aetiology secondary to the unboiled water35,36. Our study therefore also highlights the importance of personal hygiene, provision of clean water and patient education at the primary and rural health care level in Pakistan.

During the process of this study, the need became apparent for a central disease registry for GBS in Pakistan for the purpose of better record management and policy-making. Moreover, there is an urgent need to develop local management guidelines due to peculiar circumstances in terms of health care facilities and finances. This study provides a platform for the development of guidelines for the provision of medical treatment in the acute phase and for long-term rehabilitation planning in patients suffering from this disabling condition in Pakistan. A long-term multi-center follow-up study is also suggested for development of better prevention and management plans.

CONCLUSION

The GBS presents in young, as an ascending paralysis, main subtypes of GBS in Pakistan are axonal and this highlights the importance of local management guidelines and preventive medicine.

ACKNOWLEDGMENT

The authors of this work sincerely pay gratitude to our department's clinical teams for all the support they provided us in the completion of data and also thankful to Prof Levent Ozcakar and Prof Mary P Galea for their kind help in preparation of the manuscript.

CONFLICT OF INTEREST

This study has no conflict of interest to declare by any author.

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Publication:Pakistan Armed Forces Medical Journal
Geographic Code:9PAKI
Date:Feb 28, 2018
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