|Year : 2016 | Volume
| Issue : 2 | Page : 196-201
Clinical profile of acute flaccid paralysis
Naveed Mohsin1, Ravouf Asimi2
1 Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
2 Department of Neurology, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
|Date of Web Publication||29-Sep-2016|
Department of General Medicine, Sher-I-Kashmir Institute of Medical Sciences, Married Doctors Hostel G 15, Soura, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Background and Aims: As a part of the Global Polio Eradication program by the World Health Organization (WHO, 1988), surveillance of acute flaccid paralysis (AFP) was an important public health activity in many countries. With nearing eradication of poliomyelitis, other causes of AFP are gaining importance in both children and adults. Our study was designed to know the clinical characteristics and differential diagnosis of causes of AFP, including distribution by age, gender, and time. This was a prospective observational study.
Methods: AFP cases were diagnosed on history and physical examination. The underlying etiology was ascertained by appropriate laboratory investigations such as arterial blood gas analysis, urinary pH, electrolytes, thyroid profile, electrophysiological studies, cerebrospinal fluid (CSF) analysis, and imaging.
Results: Between July 2010 and September 2012, 106 cases of AFP were enrolled in the study. The mean age of males was 40.2 years (standard deviation [SD] 17.1) and the mean age of females was 33.4 years (SD 14.1). Of 106 patients, 61 (57.5%) were male and 45 (42.5%) were female. Out of 106 patients, 58 (54.7%) were suffering from Guillain-Barre' syndrome (GBS), 15 (14.2%) from hypokalemic paralysis, 8 (7.5%) from myasthenia gravis, 8 (7.5%) from thiamine deficiency, 5 (4.7%) from transverse myelitis, and 2 (1.9%) from cord compression. Other diagnoses include acute motor axonal neuropathy 3 (2.8%), acute disseminated encephalomyelitis 2 (1.9%), meningoencephalitis 2 (1.9%), diabetic polyneuropathy 2 (1.9%), and chemotherapy-induced neuropathy 1 (0.9%). Of 106 patients, 42 (39.6%) were admitted during the spring season.
Conclusion: GBS was the most common cause of AFP in all age groups. Most AFP cases occurred during the spring season. No case of poliomyelitis was found.
Keywords: Acute flaccid paralysis, Guillain-Barre′ syndrome, hypokalemic paralysis, standard deviation, World Health Organization
|How to cite this article:|
Mohsin N, Asimi R. Clinical profile of acute flaccid paralysis. Indian J Health Sci Biomed Res 2016;9:196-201
| Introduction|| |
The World Health Organization (WHO) defines acute flaccid paralysis (AFP) syndrome as "characterized by rapid onset of weakness of an individual's extremities, often including weakness of the muscles of respiration and swallowing, progressing to maximum severity within 1-10 days. The term 'flaccid' indicates the absence of spasticity or other signs of disordered central nervous system motor tracts such as hyperreflexia, clonus, or extensor plantar responses." 
With the Global Polio Eradication Initiative by the WHO in 1988, surveillance for AFP is an important public health activity in many countries. With the WHO's impressive progress, poliomyelitis is nearing its eradication in the world, and other causes of AFP has gained importance. 
AFP is a heterogeneous group and medical conditions that fall under AFP were discussed by Marx et al. as lesions of the anterior horn cell including poliomyelitis, spinal cord (as in transverse myelitis), peripheral nerve (as in Guillain-Barré syndrome [GBS]), and toxic neuropathies from various infections including diphtheria, neuromuscular junction (as in botulism), and muscle disorders, such as metabolic myopathies including hypokalemia and myositis. 
The differential diagnosis of AFP varies from region to region. In Malaysia, GBS, central nervous system infection, and transverse myelitis were the most common causes of AFP.  Apart from GBS and transverse myelitis that were the most common, the conditions classified under AFP in Australia included acute disseminated encephalomyelitis (ADEM), tick-bite paralysis, and infant botulism.  In Southwest Nigeria, the classification of AFP included traumatic sciatic nerve palsy, acute polyneuritis, neuropathy, and anterior poliomyelitis. 
Various data analyses of AFP cases over the last two decades have consistently reported GBS as the most common cause of nonpolio AFP all over the world, , with a frequency ranging from 21.09% in Pakistan  to 72.2% in Central America. 
With this aim, a prospective observational study was conducted to know the clinical characteristics and differential diagnosis of individual cases of AFP, including distribution by age, gender, and time.
| Methods|| |
Our study was a prospective, observational descriptive study. Cases admitted to the Neurology Department from July 2010 to September 2012 with provisional diagnosis of AFP were enrolled in the study. All cases with AFP, irrespective of age, gender, and time were selected. All the cases of traumatic, spastic, chronic flaccid paralysis or sudden onset of weakness as in cerebrovascular accident were excluded from the study.
The clinical evaluation of AFP is often limited by meager resources and health infrastructure. Cases were selected on clinical grounds. In addition to routine baseline investigations, lactate dehydrogenase, creatine kinase, arterial blood gas analysis, urinary pH, electrolytes, thyroid profile, electrophysiologic studies (nerve conduction studies, electromyography, etc.), CSF examination, imaging of the spine and brain (radiography, computed tomography [CT], or magnetic resonance imaging [MRI]), stool examination were carried out in relevant cases.
| Results|| |
Results are shown in [Table 1] [Table 2] [Table 3] [Table 4] [Table 5]. A total of 106 cases of AFP were enrolled from July 2010 to September 2012. Of 106 patients, the majority 58 (54.7%) were suffering from GBS.
|Table 1: Frequency distribution of cases of acute flaccid paralysis (n=106)|
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|Table 3: Age and gender distribution of the acute flaccid paralysis patients|
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Age and gender distribution of AFP cases are shown in [Table 3]. Of 106 cases of AFP, most cases, i.e., 42/106 (39.6%) were admitted during the spring season [Figure 1].
Of the 58 patients with GBS, 37 (63.8%) were male and 21 (36.2%) were female. The mean age was 35.38 years (standard deviation [SD] 16.72) with a range of 13 years to 70 years [Table 5]. Most patients (40 [69%]) were <40 years of age. Out of 58 cases of GBS, most cases (25 [43.1%]) occurred during the spring season.
The clinical characteristics of GBS are shown in [Table 4]. Ascending lower limb weakness, i.e., 58 (100%) was the presenting symptom followed by upper limb weakness, i.e., 36 (62.1%). Cranial nerve (CN) VII was most often involved in 6 (10.3%) patients.
Albuminocytological dissociation in CSF was present in 42/58 (72.4%) patients during the 2 nd week, and reduced motor conduction velocity, slowed F-wave response, and prolonged distal latencies were evident in all on electrophysiology studies. Clinically, six patients were objectively found to have respiratory weakness and received intravenous (IV) immunoglobulins, of which 2/58 (3.4%) needed ventilatory support. All cases survived and there was no mortality.
Fifteen patients with hypokalemic paralysis (HP) were enrolled. The etiological causes of HP were renal tubular acidosis Type I (n = 8), HP secondary to diuretics (n = 5), and hypokalemic periodic paralysis (n = 2). Lower limb weakness, both proximal and distal, was the predominant symptom in all, and upper limb weakness was present in 14/15 (93.3%) and two had familial history.
Myasthenia gravis (MG) constituted 8/106 (7.5%) cases of AFP. Females were 5/8 (62.5%) and 3/8 (37.5%) were male. The mean age was 37.8 years (SD 11.88), with a range of 23-50 years.
The clinical characteristics of MG are depicted in [Table 5]. Electrophysiological studies showed decremental nerve conduction in all, and CT scan of the chest showed thymus gland enlargement in 3/8 (37.5%). Tensilon test was positive in all patients. Of 8 patients, 2 (25%) had respiratory distress with single breath count <20 and respiratory expansion <5 cm. Ventilatory support was needed in 1/8 (12.5%) and one patient had recurrence of symptoms.
Of the eight patients with thiamine deficiency, 6 (75%) were female and 2 (25%) were male. Of 8 patients, 6 (75%) were of <40 years age.
Lower limb weakness (distal > proximal) was present in all cases followed by upper limb weakness. Of 8 patients, 2 (25%) had features of Wernicke's disease with extraocular movement paresis, nystagmus, and confusion with mini mental state examination <22. Of 8 patients, 3 (37.5%) had features of wet beriberi with swelling of the feet and tachycardia. CN VI was bilaterally involved in 2/8 (25%), and MRI of the brain was abnormal in 2/8 (25%).
Out of the five patients with transverse myelitis, 3 (60%) were female and 2 (40%) were male. All patients were <40 years of age. Two (40%) had transverse myelitis secondary to multiple sclerosis with objective evidence of pyramidal tract involvement; T2-weighted (T2W) MRI images of the brain showed multiple periventricular hyperintensities, abnormal brain stem evoked response audiometry, and oligoclonal bands in CSF. T2W images of the spinal cord showed hyperintensities with swollen cord over variable segments in 3 (60%).
Two patients with diagnosis of cord compression were admitted. Both patients were male, one with age of 32 years and other with age of 60 years. One had cord compression secondary to cervical tuberculosis and other secondary to multiple cervical disc prolapse.
Acute motor axonal neuropathy (AMAN) a variant of GBS constituted 3/106 (2.8%) cases. Two were male and one was female. Albuminocytological dissociation of CSF was found in all, and nerve conduction studies were of motor axonal neuropathic type in all and 2/3 had respiratory distress with single breath count <20 and respiratory expansion 5 cm and were in need of ventilatory support. All survived.
ADEM was present in 2/106 (1.9%) patients. One patient was male with 16 years of age, and other was female with 15 years of age. Both upper and lower limb weakness were present in all, bulbar weakness in 1/2, nonspecific respiratory infection preceded in 1/2. Out of 2 patients, 1 had signs of meningismus and was confused and 1 had bilateral CN VII weakness, with high protein in CSF, in both. T2W MRI images of the brain showed widespread hyperintensities in periventricular and subcortical areas.
| Discussion|| |
With poliomyelitis nearing its elimination in the world, the other causes of AFP in children and adults have become significant. Unlike other studies which are important from the epidemiological point of view because the WHO is running polio eradication campaign, our study was mainly conducted to know the clinical characteristics and differential diagnosis of individual cases of AFP, including distribution by age, gender, and time.
Various data analyses of AFP cases over the last two decades have consistently reported GBS as the most common cause of nonpolio AFP all over the world with a frequency ranging from 20% in Oman  to 72.2% in Central America.  Mostly, the frequency lies between 32.3%  and 47.29%  although the results of our study confirm to the rest of the national and international literature in having GBS as the most frequent cause of nonpolio AFP, the frequency stands on higher side at 54.7%, may be because of cumulative frequency of GBS across all age ranges, unlike to most other studies carried out in children <15 years.
In our study, the frequency of HP was 14.2%, consistent with other studies. , Most AFP studies conducted in children <15 years of age do not mention MG to occur with a significant frequency as in a study by Morris et al. with the frequency of 2/143 (1.4%).  In our study, MG was present with frequency of 7.5%, likely due to more aged profile of our patients. The frequency of transverse myelitis in our study, 4.7%, was consistent with other studies. , AMAN, subtype of GBS, was present with a frequency of 2.8% of AFP cases and 4.9% of GBS cases consistent with 3.2% of GBS cases in a study by Koul et al.  but lower than a study in China.  The frequency of ADEM in our study of 1.9% lies between 3.5% and 26.6% in other studies. , Viral meningoencephalitis varies in frequency from 6.75% to 12.5% in other studies, , which in contrast to our study stands at 1.9%.
The males outnumbered (57.5%) females (42.5%), which was in accordance with a study by Shah et al. 
Most cases of AFP (39.6%) occurred during the spring season (March to May) which was in contrast to summer season (June to August) in a study by Lam et al. 
There was a preponderance of males (63.8%) over females (36.2%) in GBS, consistent with most of earlier studies. ,,, However, in a study by Prevots and Sutter  the relative risk for GBS according to sex varied with age. This could be due to small sample size of our study and regional variation in the occurrence of GBS.
Acute gastroenteritis preceded in 20.7% and mild upper respiratory infection preceded in 13.8% cases, in our study, in contrast to airway infections, dominating over gastrointestinal infections, in other studies. ,
All of the patients had progressive muscle weakness in a roughly symmetrical distribution, with areflexia of lower limbs in 100% of the cases and hyporeflexia of upper limbs in 12.1%, and this was consistent with data from a study by Olivé et al.  CN involvement was present in 37.9% of cases with CN VII involved in most (10.3%) as against 50% in a study by Morris et al.  to 21% in a study by Olivé et al.  with CN VII, most often affected. Respiratory depression was present in 10.3% in comparison to 16% in a study by Olivé et al. 
Clinically, six patients were objectively found to have respiratory depression, out of which 2 (3.4%) needed ventilatory support against 16%, in need of artificial ventilation in a study by Korinthenberg and Mönting. 
HP was present in 14.2% patients lower than when compared to a study by Shah et al.  This discrepancy may be due to under-reporting of cases, transient nature of paralysis and variable sample size of the study.
Female predominance, i.e., 5/8 (62.5%) of MG in our study was consistent with other studies. , Of 8 (25%) patients, two had respiratory depression, and ventilatory support was needed in one patient, against 12/50 (24%) requiring ventilatory support in a study by Jacob et al. 
Out of the eight patients with thiamine deficiency, 6 were female and 2 were male. The mean age was 35.63 years (SD 9.9) with a range of 24 years to 50 years. In comparison, out of 17 patients with thiamine deficiency in a study by Koike et al.,  12 were male and 5 were female, with mean age of 57.4 years (SD 9.4 years) and range of 42-69 years. This signifies female predominance and young age of presentation in our patients in contrast to latter. Recurrent vomiting preceded in 5/8 patients, suffering from acid peptic disease (APD) and were females. As against, no patient in a study by Koike et al.  had vomiting, who were operated for gastric problems, APD/neoplasm.
Lower limb weakness (distal > proximal) was present in all cases followed by upper limb weakness, 7/8. Burning sensation of limbs was present in 6/8. In comparison to a study by Koike et al.,  the initial symptom was weakness of the limbs in 11/17 patients and a burning sensation in the feet in 6/17 patients.
In our study, 2/8 had features of Wernicke's disease with extraocular movement paresis, nystagmus and confusion and 3/8 had features of wet beriberi. In comparison to a study by Koike et al.,  four patients manifested Wernicke-Korsakoff syndrome, and 10 manifested signs of heart failure.
Female predominance, 3/5 (60%), of transverse myelitis was evident in our study as seen in a study by Morris et al.  but against male predominance in other studies. , With respect to the clinical presentation, our findings agree with a study by al Deeb et al.  In our study, 2/5 (40%) had transverse myelitis secondary to multiple sclerosis compared with 21% multiple sclerosis in a study by Jeffery et al.  All patients received methylprednisolone, and 4/5 had good outcome, consistent with a study by Dowling et al. 
AMAN constituted 3 (2.8%) cases of AFP cases and 3/61 (4.9%) of GBS comparable with 3.2% of GBS in a study by Koul  but lower than 14.7% in a study by Dourado et al.  and 47% in a study by McKhann et al.  Two of our patients had respiratory distress compared to 22% in a study by Dourado et al.  which needed ventilatory support with IV immunoglobulins. All patients improved.
Two cases (1.9%) of AFP had viral meningoencephalitis compared with 6.75% in a study by Anis-ur-Rehman et al.  to 12.5% in a study by Saraswathy et al.  and 16.2% in a study by Hussain et al.  About 1.9% AFP cases had diabetic polyneuropathy and 0.9% had chemotherapy-induced neuropathy as against 3% cases of diabetic neuropathy and 8% cases of chemotherapy-induced neuropathy in a study by Lee et al. 
| Conclusion|| |
GBS was the most common cause of AFP in all age groups. Most AFP cases occurred during the spring season. No case of poliomyelitis was found.
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Conflicts of interest
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[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]