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ORIGINAL ARTICLE  
Year : 2014  |  Volume : 7  |  Issue : 3  |  Page : 149-154
Acute Flaccid paralysis in adults: Our experience


1 Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana, India
2 Department of Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana, India

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Date of Submission21-Sep-2013
Date of Acceptance04-Nov-2013
Date of Web Publication16-Jul-2014
 

   Abstract 

Acute flaccid paralysis (AFP) is a complex clinical syndrome with a broad array of potential etiologies that vary with age. We present our experience of acute onset lower motor neuron paralysis. Materials and Methods: One hundred and thirty-three consecutive adult patients presenting with weakness of duration less than four weeks over 12 months period were enrolled. Detailed history, clinical examination, and relevant investigations according to a pre-defined diagnostic algorithm were carried out. The patients were followed through their hospital stay till discharge or death. Results: The mean age was 33.27 (range 13-89) years with male preponderance (67.7%). The most common etiology was neuroparalytic snake envenomation (51.9%), followed by Guillain Barre syndrome (33.1%), constituting 85% of all patients. Hypokalemic paralysis (7.5%) and acute intermittent porphyria (4.5%) were the other important conditions. We did not encounter any case of acute polio mylitis in adults. In-hospital mortality due to respiratory paralysis was 9%. Conclusion: Neuroparalytic snakebite and Guillain Barre syndrome were the most common causes of acute flaccid paralysis in adults in our study.

Keywords: Guillain Barre syndrome, lower motor neuron, neuroparalytic snakebite, paralysis, weakness

How to cite this article:
Kaushik R, Kharbanda PS, Bhalla A, Rajan R, Prabhakar S. Acute Flaccid paralysis in adults: Our experience. J Emerg Trauma Shock 2014;7:149-54

How to cite this URL:
Kaushik R, Kharbanda PS, Bhalla A, Rajan R, Prabhakar S. Acute Flaccid paralysis in adults: Our experience. J Emerg Trauma Shock [serial online] 2014 [cited 2019 Dec 15];7:149-54. Available from: http://www.onlinejets.org/text.asp?2014/7/3/149/136847



   Background and aim Top


Acute flaccid paralysis (AFP) is a clinical syndrome characterized by rapid onset of weakness of lower motor neuron type, including weakness of the respiratory and pharyngeal muscles, progressing to maximum severity within several days to weeks. AFP is a complex clinical syndrome with a broad array of potential etiologies that vary remarkably with age. Patients presenting to the emergency department with acute onset of quadriparesis or paraparesis pose a unique challenge to the clinician. An accurate and early diagnosis is the key to a positive outcome. Various studies are available in the pediatric population regarding AFP prevalence and differential diagnoses, mainly as an offshoot of the global polio eradication initiative. However, few such studies have been conducted among adults. We evaluated cases of acute lower motor neuron type of weakness presenting to the emergency

department and analyzed the underlying spectrum of clinical conditions.


   Materials and methods Top


We conducted a prospective, observational study to determine the underlying diagnosis and in-hospital course of patients presenting with acute flaccid paralysis to the emergency department of PGIMER, a tertiary care hospital in North-West India. One hundred and thirty-three consecutive patients of age above 12 years presenting with weakness of duration less than four weeks, between the period of 13 April 2010 and 12 April 2011, were enrolled in the study after informed consent. Patients with longer duration of illness, trauma, UMN involvement or hemiparesis on clinical examination were excluded. Detailed history and clinical examination were performed by a single examiner, and relevant investigations according to a pre-defined diagnostic algorithm were carried out [Figure 1]. The patients were followed through their hospital stay till discharge or death.
Figure 1: Algorithm for diagnosis of acute flaccid paralysis

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Statistical analysis was carried out using Statistical Package for Social Sciences (SPSS Inc., Chicago, IL, version 15.0 for Windows. Normality of data was checked by measures of skewness and Kolmogorov-Smirnov tests of normality. As our data for duration of stay was skewed, we applied Kruskal Wallis test for comparison of medians for different diagnoses. Proportions were compared using Chi-square or Fisher's exact test, whichever was applicable. To estimate the risk factors for death, Multinomial regression analysis was applied.


   Results Top


The mean age of the study population was 33.27 (range 13-89) years. Males constituted 67.7% of the population, and there was no statistically significant difference in the gender distribution across age groups (P = 0.910). 51.1% were less than 30 years of age, 42.9% were between the ages of 30 and 55, and 6.0% were above 55 years of age. 66.9% of the entire population was inhabitants of rural regions, and 33.1% were urban dwellers.

The spectrum of diagnoses that presented as acute flaccid paralysis and their frequencies during the study period is shown in [Table 1].
Table 1: Etiologies of acute flaccid paralysis

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The most common etiology of acute flaccid paralysis in this entire population was neuroparalytic snake envenomation, which was responsible for 51.9% of the cases, followed by the Guillain Barre syndrome (33.1%). These two etiologies accounted for 85% of all patients. Hypokalemic paralysis (7.5%) and acute intermittent porphyria (4.5%) were also encountered in considerable numbers. Rest of the population consisted of single representations of viral meningoencephalitis, early acute transverse myelitis, Miller Fisher syndrome, and myasthenic crisis.

Maximum number of cases of acute flaccid paralysis was encountered during the monsoon period (N = 63). A marked variation in the epidemiology of acute flaccid paralysis was noted over the four seasons. During the monsoon season, majority of cases were related to neurotoxic snake envenomation (81.0%). In contrast, GBS was encountered more commonly during the post-monsoon and winter periods making up 50.0% and 80.0% of the cases, respectively, compared to snakebite, which progressively declined to 20.0% and 6.7%. In the summer months, GBS and snake envenomation were encountered in almost similar frequencies 40.0% and 37.1%, respectively [Figure 2]. This seasonal variability in the etiology of acute flaccid paralysis was statistically significant (P = 0.000).
Figure 2: Seasonal distribution of acute flaccid paralysis

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Among individuals younger than 30 years, snake envenomation was the most common etiology encountered (60.3%) followed by GBS (29.4%). In the 31-55 years age group, snake envenomation accounted for 45.6% of the cases followed by GBS (35.1%). To the contrary, GBS was the more common etiology encountered in individuals above the age of 55 years, accounting for 50.0% of the cases. However, there was no statistically significant difference in the distribution of etiologies according to age in this study. There was no statistically significant difference between rural and urban patients in the etiology of acute flaccid paralysis. However, among patients with snake envenomation, a statistically significant numbers (75%) were from rural areas.

Ascending motor paralysis was the mode of presentation in 31.6%. 33.8% of the patients presented initially with weakness in the cranial nerve distribution. Simultaneous involvement of the upper limbs and lower limbs was noted in 27.1% and a descending type of weakness in 6.8%. One patient (0.8%) presented with respiratory distress as an initial manifestation. There was no statistically significant difference in the distribution of mode of presentation (ascending, descending, bulbar or respiratory) as well.

Among patients presenting with ascending paralysis, 81.0% were diagnosed to have LGBS, as were 66.7% of those with descending paralysis. Contrary to this, 69.4% of patients with simultaneous onset of weakness in the upper and lower limbs and 97.8% of patients with bulbar/extraocular weakness were accounted for by snake envenomation [Figure 3]. This difference in distribution was highly statistically significant (P = 0.000). The single patient who presented with respiratory distress at onset was diagnosed to have myasthenic crisis.
Figure 3: Pattern of weakness in different etiologies leading to acute flaccid paralysis

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Weakness in the cranial nerve distribution was encountered on clinical examination in 33.8%. Bilateral ptosis was the most common abnormality, seen in 50.4% during the course of hospital stay, and unilateral ptosis was noticed in one patient (0.8%). This was followed by external ophthalmoplegia (24.1%), bilateral ninth and tenth cranial nerve palsies (19.5%), bilateral facial nerve palsy (17.3%), and unilateral ninth and tenth palsy (6.0%). Bilateral cranial nerve 11 (3.0%) and 12 (1.5%) involvement and unilateral facial palsy (0.8%) were also seen. Cranial nerve involvement was most frequently encountered in patients with snake envenomation. Ptosis was the most common manifestation, and all cases of external ophthalmoplegia (other than only ptosis) were accounted for by snake envenomation (P = 0.000). Bilateral ptosis was seen in 92.8% of the snakebite cases compared to 2.3% of the GBS cases (P = 0.000). Bilateral facial nerve palsy was observed in 47.7% cases of GBS and unilateral facial palsy in 2.3%. Bilateral facial palsy was also observed in two cases of acute intermittent porphyria. In contrast, no facial palsy was seen in snake envenomation or any of the other diagnoses (P = 0.000). There was no statistically significant difference in the distribution of bulbar paralysis according to etiology (P = 0.982).

One patient (0.8%) presented with history of respiratory distress before development of limb weakness. However, respiratory involvement was noted in 75.2% of our patient population during hospital stay. Among the patients with respiratory involvement, snake envenomation was the commonest cause (66%) followed by GBS (25%), acute intermittent porphyria(5%), hypokalemic paralysis (3%), and myasthenia gravis (1%). 95.7% of snakebite cases, 83.3% of porphyria patients, and 56.8% of GBS cases had respiratory involvement.

Autonomic involvement of the cardiovascular system was noted in 11.3% of the population, of whom 60.0% had LGBS, 20.0% had acute intermittent porphyria, and 6.6% each had snake envenomation and myasthenia gravis as the contributory cause. 8.3% (11/133) had lower motor neuron type of bladder involvement during the course of stay. Overall, 12.8% (17/133) cases had sensory involvement. GBS accounted for most cases with sensory involvement (13/17), 76.4% of all cases. One case each of porphyria and acute transverse myelitis had sensory involvement.

29.3% (39/133) of all patients had one or other complication during hospital stay [Table 2]. Irrespective of the etiological diagnosis, all patients seemed to be equally susceptible to complications (P = 0.458). Hospital-acquired pneumonia was the most common complication (15%), seen exclusively in intubated patients. This was followed by cardiac arrhythmia, predominantly in patients of LGBS and acute intermittent porphyria. Acute kidney injury was seen in a significant number of patients of hypokalemic paralysis (P = 0.000). Gastrointestinal bleeding was encountered more commonly in snakebite patients (P = 0.000). There was no statistically significant difference in the distribution of other complications.
Table 2: Complications encountered during hospital stay in patients with acute flaccid paralysis

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Exposure keratitis, upper respiratory tract infection, steroid-induced hyperglycemia, aspiration pneumonia, catheter-related blood stream infection, and tracheo-brachiocephalic artery fistula were seen in one patient each.

The median duration of hospital stay for all etiologies of acute flaccid paralysis was 6.00 days (25 th percentile-4 days, 75 th percentile-12 days). Mechanical ventilatory support was required in 66.2% cases. The median duration of ventilatory support was 4.00 days (25 th percentile-3 days, 75 th percentile-8.25 days). In the entire population, 63.2% completely recovered at discharge, 18% improved but had residual deficits at discharge, 9% died, 7% left against medical advice, and 4 cases (3%) had no improvement in muscle power at the time of discharge. A statistically significant difference was noted in outcomes among the various etiologies (P = 0.000). While 88.4% of the snake envenomations had complete recovery at discharge, only 25.0% of the LGBS group had a similar outcome [Figure 4].
Figure 4: Outcomes according to etiologies in patients with acute flaccid paralysis

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A total of 12 (9%) patients died in hospital. Six patients had neuroparalytic snakebite, three had GBS, and one each with porphyria, distal RTA, and meningoencephalitis died. Respiratory involvement was present in 83.3% of the patients who died. Autonomic involvement was present in 16.7%. There was no statistically significant difference among the various etiologies in terms of death as an outcome (P = 0.292).

Cardiac arrhythmia (P = 0.000), sepsis (P = 0.001), and Hypoxic Ischemic Encephalopathy (P = 0.004) were significantly related to death. Health care-associated pneumonia was present in 25.0%, sepsis in 33.3%, cardiac arrhythmias in 41.7%, prior cardiac arrest in 16.7%, acute kidney injury, aspiration pneumonia, and tracheo-brachiocephalic artery fistula in 8.3% each before demise. No other complication, clinical or demographic feature was significantly related to mortality.


   Discussion Top


Historically, the predominant cause of AFP has been infection with poliovirus. The availability of an effective poliovirus vaccine led to a dramatic decline in poliovirus infections worldwide. Since the elimination of poliovirus from large parts of the world, Guillain-Barré syndrome (GBS) has become the most important clinical cause of AFP. [1] Various studies around the world have found prevalence of GBS among acute flaccid paralysis patients of 42- 47%. [2],[3] Higher prevalence has been reported from Honduras (72%). [4] Studies around the world have identified envenomation, prophyria, hypokalemia, early acute transverse myelitis, rhabdomyolysis, botulism, and myasthenia gravis as other causes of acute flaccid paralysis. Neuroparalytic snakebite has been previously reported as a significant cause of acute flaccid paralysis in young rural men from Northern India. [5]

Our study shows a male preponderance among patients with acute flaccid paralysis due to higher proportion of males in both snake envenomation and LGBS groups. Males are at a higher risk for snake envenomation due to occupational and recreational outdoor activities that predispose them to encounters with venomous snakes. Male preponderance in GBS cases has been reported to be 1.36-2: 1 in various studies and our study reflects similar results. [6],[7] The reasons for such a predilection are not clear. A preponderance of younger individuals was observed in both GBS (90% ≤ 55 yrs) and snake envenomation patients (97% ≤ 55 yrs). Two-third of our patients were from rural areas. 47% of acute flaccid paralysis patients were seen during the monsoon season due to increased incidence of snake envenomation as a consequence of flooding of the habitat of snakes and their prey. GBS was seen in higher frequency during post-monsoon and winter, and there are other studies which show similarly increased incidence in winter season. An aberrant immune response to infectious agents like the influenza virus is thought to account for such seasonal variations. [8]

Weakness that begins distally and progresses proximally with no or minimal sensory involvement could be a pointer towards GBS. On the other hand, patients who present with weakness in the cranial nerve distribution at onset are more likely to have a diagnosis of neuroparalytic envenomation. In the present series, all patients of complete ophthalmoplegia had a diagnosis of neuroparalytic envenomation. Bilateral third nerve palsy is a highly specific and sensitive indicator of neuroparalytic snakebite [Table 3]. Similarly, bilateral facial palsy has a high specificity and positive predictive value for the diagnosis of GBS, although its sensitivity and negative predictive value for the same diagnosis is not as good.
Table 3: Pattern of involvement in acute flaccid paralysis

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Simultaneous upper and lower limb weakness was seen mainly in snake envenomation, which involves the neuromuscular junction. Similar pattern of weakness was also observed in hypokalemic paralysis, which causes myopathy. Nine patients had descending type of weakness, seven out of which were GBS and the rest two were diagnosed as Acute Intermittent Porphyria. Hence, in patients presenting with ascending paralysis, a diagnosis of GBS should be considered and in those with ptosis and external ophthalmoplegia at presentation, snakebite should be the first possibility, more so in monsoon season. Similarly, in patients presenting with bilateral LMN facial palsy along with flaccid paralysis, diagnosis of GBS is more likely. LGBS and snake envenomation accounted for 96% of patients with bulbar paralysis. Bulbar weakness was seen in 29.5% of GBS patients in this study, which comes in range of other reported studies. [9],[10] Bulbar weakness was seen in 17.4% of neuroparalytic snakebite patients, which is lower than that reported in previous retrospective studies. [5]

Respiratory involvement was seen in 75% of our patients and 88% of them required mechanical ventilation. This is significantly higher than the respiratory involvement seen in similar case series published from other parts of world. [6],[7] This difference could be due to an overrepresentation of neuroparalytic snakebite patients who had severe respiratory involvement. Patients of acute intermittent porphyria and axonal variants of GBS who have more severe illness may also have contributed to the higher mechanical ventilatory requirement. Similar to previous studies, we also found predominance of axonal involvement in our GBS patients. [11] The higher percentage of respiratory involvement in the present study may also be a reflection of referral bias with more severe cases requiring mechanical ventilation being referred to our hospital.

Outcomes in acute flaccid paralysis patients were skewed due to preponderance of snake envenomation, a potentially reversible condition. Majority of neuroparalytic snakebite patients improved completely (72.6%). Other studies also showed similar results. [5],[12] Prognosis of neuroparalytic snakebite patients was excellent if care was provided before development of complications. On the other hand, majority of patients with GBS or porphyria were improving but had residual weakness at the time of discharge. This is because, in this study, patients were followed only till time of discharge. Patients of GBS and porphyria take longer to recover fully (weeks to months) as compared to those of neuroparalytic snakebite, who improve within days. Cardiac arrhythmia, sepsis, and hypoxic ischemic encephalopathy were identified to be significantly associated with a poorer outcome.

Our study has a few limitations. Being a single-center study in North Western India, our observations may not be representative of the entire country. As this study was conducted in a tertiary care referral center, our patients included the more severely afflicted along the spectrum of flaccid paralysis as evidenced by higher need for mechanical ventilatory support. The less severe cases may not have reached us and skewed our results. In spite of these limitations, our study shows that excellent outcomes are possible in these patients when provided optimal supportive care. Larger sample size and longer duration of follow-up is necessary to identify other conditions causing acute flaccid paralysis and their long-term outcomes.


   Conclusion Top


This study shows AFP to be predominantly an illness of young men, with significant seasonal variations in the inciting causes. The study also identifies features such as pattern of progression of weakness, external ophthalmoplegia, and facial palsy that are strong pointers to a particular clinical diagnosis. These could be useful to physicians who work in resource-limited settings.

 
   References Top

1.Ward NA, Milstien JB, Hull HF, Hull BP, Kim-Farley RJ. The WHO-EPI initiative for the global eradication of poliomyelitis. Biologicals 1993;21:327-33.  Back to cited text no. 1
    
2.Koul R, Chako A, Javed H, Al-Hinai K, Zachariah M, Bulusu S, et al. A profile of childhood neuropathies at a university hospital in Oman. Saudi Med J 2002;23:450-6.  Back to cited text no. 2
    
3.Morris AM, Elliott EJ, D'Souza RM, Antony J, Kennett M, Longbottom H. Acute flaccid paralysis in Australian children. J Paediatr Child Health 2003;39:22-6.  Back to cited text no. 3
    
4.Molinero MR, Varon D, Holden KR, Sladky JT, Molina IB, Cleaves F. Epidemiology of childhood Guillain-Barré syndrome as a cause of acute flaccid paralysis in Honduras: 1989-1999. J Child Neurol 2003;18:741-7.  Back to cited text no. 4
    
5.Sharma N, Chauhan S, Faruqi S, Bhat P, Varma S. Snake envenomation in a north Indian hospital. Emerg Med J 2005;22:118-20.  Back to cited text no. 5
    
6.Soysal A, Aysal F, Caliskan B, Dogan Ak P, Mutluay B, Sakalli N, et al Clinico-electrophysiological findings and prognosis of Guillain-Barré syndrome--10 years' experience. Acta Neurol Scand 2011;123:181-6.  Back to cited text no. 6
    
7.Arami MA, Yazdchi M, Khandaghi R. Epidemiology and characteristics of Guillain-Barré syndrome in the northwest of Iran. Ann Saudi Med 2006;26:22-7.  Back to cited text no. 7
    
8.Sivadon-Tardy V, Orlikowski D, Porcher R, Sharshar T, Durand MC, Enouf V, et al . Guillain-Barré syndrome and influenza virus infection. Clin Infect Dis 2009;48:48-56.  Back to cited text no. 8
    
9.Halawa EF, Ahmed D, Nada MA. Guillain-Barré syndrome as a prominent cause of childhood acute flaccid paralysis in post polio eradication era in Egypt. Eur J Paediatr Neurol 2011;15:241-6.   Back to cited text no. 9
    
10.Durand MC, Porcher R, Orlikowski D, Aboab J, Devaux C, Clair B, et al. Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: A prospective study. Lancet Neurol 2006;5:1021-8.  Back to cited text no. 10
    
11.Govoni V, Granieri E. Epidemiology of the Guillain-Barré syndrome. Curr Opin Neurol 2001;14:605-13.  Back to cited text no. 11
    
12.Tan HH. Epidemiology of snakebites from a general hospital in Singapore: A 5-year retrospective review (2004-2008). Ann Acad Med Singapore 2010;39:640-7.  Back to cited text no. 12
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Correspondence Address:
Ashish Bhalla
Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2700.136847

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