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Table of Content - Volume 19 Issue 3 - September 2021


 

A Study of clinical profile of status epilepticus in pediatric age group at tertiary health care center

 

Kiran B Bhaisare(Holikar)1, Sunil S Holikar2*

 

1,2Associate Professor, Department of Paediatrics, Vilasrao Deshmukh Government Medical College, Latur, Maharashtra, INDIA.

Email: sunilholikar@gmail.com

 

Abstract              Background: Status epilepticus is a neurological emergency requiring immediate evaluation and management to prevent significant morbidity or mortality. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. The short-term mortality (within 30 days) of SE ranges from 7.6% to 22% across all age groups and is highest amongst the elderly. Present study was aimed to study clinical profile and neurological outcome of children’s presenting with status epilepticus at a tertiary health care center. Material and Methods: Present study was hospital based, prospective and observational study conducted in children of age 1month to 12 years who presented with status epilepticus. Results: In present study, male to female ratio was 1.38: 1. The mean age of patients was 3.75 ± 2.43 years, ranged from 1 month -12 years. Maximum number of cases age of 1st onset of seizure was 6-12 months (23%) followed by 2-6 months (22%) and 5-10 years (21%). GTCS was the commonest type of seizure seen in 97 cases (97%) and focal seizure with secondary GTCS observed in 3(3%) cases. Most common etiology associated with status epilepticus in children was seizure disorder (44%), followed by acute CNS infection (34%), fever provoked seizures (24%) and Quadriplegia (19%). Out of 20 children with abnormal neuroimaging, 7 children had refractory status epilepticus (RSE). Out of 100 cases, 76 cases (76%) recovered. Among those recovered, 6 cases (6%) recovered with new neurological sequel; 13 cases (13%) died and 5 cases (5%) discharged against medical advice (AMA). Conclusion: In present study, children below 5 years had a higher incidence of status epilepticus. The commonest type of seizure was a generalized tonic-clonic seizure. Seizure disorder, acute CNS infections, and febrile seizures were commonest etiological reasons. CSF analysis, Neuroimaging and EEG have the most important role in diagnosis of seizures.

Keywords: status epilepticus. generalised tonic-clonic seizure, Seizure disorder, acute CNS infections, febrile seizures

 

INTRODUCTION

Status epilepticus is a neurological emergency requiring immediate evaluation and management to prevent significant morbidity or mortality. The Neurocritical Care Society guidelines defined seizure as with 5 minutes or more of continuous clinical and/or electrographic seizure activity, or recurrent seizure activity without recovery between seizures.1,2 ILAE in 2014 suggested seizures and epilepsy are not the same. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy is a disease characterized by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition.3 The reported mortality at hospital discharge in SE is 9–21%. The short-term mortality (all age groups) rates reported from India and other developing countries range between 10.5% and 28%.4 Morbidity and mortality from status epilepticus seems to be due to CNS damage due to the underlying illness or acute insult causing the status epilepticus, due to repetitive electrical discharge of the seizure itself and systemic and metabolic factors related to repeated Generalized tonic-clonic seizures (GTCS) type of seizures.5 The short-term mortality (within 30 days) of SE ranges from 7.6% to 22% across all age groups and is highest amongst the elderly.6 Present study was aimed to study clinical profile and neurological outcome of children’s presenting with status epilepticus at a tertiary health care center.

              

MATERIAL AND METHODS

Present study was hospital based, prospective and observational study conducted in Department of Paediatrics, Vilasrao Deshmukh Government Medical College, Latur, India. Study duration was of 2 years (1st November 2018 to 31st October 2020).

Inclusion criteria: Children of age 1month to 12 years who presented with status epilepticus.

Exclusion criteria: Child with status epilepticus with head injury. Children less than 1 month and more than 12 years of age. Patients whose parents do not give consent. The study was commenced after the institution ethical committee clearance. Informed consent was obtained from the concerned parents/guardian. All consecutive cases of status epilepticus-those admitted during the study period. A total 100 Children’s of age 1month to 12 years who presented with status epilepticus were enrolled in the study.

All children who met the inclusion criteria were enrolled in the study and informed consent was obtained from their parents/guardian. A detailed history in all cases was taken with emphasis on the onset of seizure, duration of seizure, number of convulsion, type of seizure, antenatal, natal and post natal risk factors. Thorough clinical examination was done – vitals, general physical examination and systemic examination with special reference to central nervous system. All relevant investigations to arrive at the diagnosis and further management performed; children were followed up to evaluate their immediate outcome. i.e., till discharge or death.

Data entered in Microsoft-Excel sheet and its analysis performed by the statistical software SPSS. Chi square test and Fischer test are used for nominal data. ‘P’ value less than 0.05 was considered statistically significant.

 

RESULTS

In present study, out of total 100 patients participants 58% patients were males and 42% patients were females. This distribution shows the predominance of males with male to female ratio of 1.38: 1 and difference was not statistically significant, Maximum numbers of male and female patients were in the age group of 2 to 5 years (46%) followed by 5 to10 years (28%) then less than 2 years (20%) and 10-12 years (6%). The mean age of patients was 3.75 ± 2.43 years, ranged from 1 month -12 years. There was no statistically significant difference in male and female according to age distribution (p>0.05).


 

Table 1: Distribution of the Study subjects according to Age and gender

Age group (in years)

Males

Females

Total

Percentage

<2

13

07

20

20%

2 to 5

26

20

46

46%

5 to10

15

13

28

28%

10 to12

04

02

06

6%

In present study, maximum numbers of children were born after 37 weeks gestation (term pregnancy) while 5% children born before 37 weeks (pre-term), more commonly known as premature. Most of the children 55% were born as 1st child followed by 2nd order of birth were 35%. Institutional birth was most common in 99% cases. NICU candidate were 60% and most common factor for addition in NICU was respiratory distress syndrome (RDS) 16% followed by neonatal sepsis (NNS) 13% and early onset sepsis (EOS) 11%. Duration of breastfeeding <6 months was in 44% cases and 6-12months in 45%.

Table 2: Distribution of study subjects according to Birth characteristics

Birth characteristics

No. of Cases

Percentage

 

Term

Small for Gestational Age

05

95%

Average Gestational Age

90

Preterm

Small for Gestational Age

02

5%

Average Gestational Age

03

Order of birth

1

55

55%

2

35

35%

3

08

8%

>3

02

2%

Place of birth

Home

01

1%

Institution

99

99%

NICU Candidate

Neonatal Hyperbilirubinemia

13

13%

Respiratory Distress Syndrome

16

16%

Early Onset Sepsis

11

11%

Neonatal Sepsis

09

9%

Low Birth Weight /Very Low Birth Weight

06

6%

Meconium Aspiration Syndrome

05

5%

Duration of breastfeeding

<6 months

44

44%

6-12 months

45

45%

1-2 years

10

10%

>2 years

01

1%

History of iron deficiency anaemia was observed in 5% cases, Stroke in 11%, space occupying lesion in 6%, CNS Malformation 6%, Immunization appropriate in 96%, milestones appropriate for age in 73% and delay in 27%. History of failure to thrive observed in 24% and vitamin/micronutrient deficiency was observed in 20%.

 

Table 3: Distribution of study subjects according to Past history

Past history

No. of Cases

Percentage

H/O Iron deficiency anaemia

5

5%

H/O Stroke

11

11%

space occupying lesion

6

6%

CNS Malformation

6

6%

Appropriate

96

96%

Not appropriate Immunization as per national immunization programme

04

4%

Delayed milestones

27

27%

H/O failure to thrive

24

24%

Vitamin/Micronutrient deficiency

20

20%

Maximum number of cases age of 1st onset of seizure was 6-12 months (23%) followed by 2-6 months (22%) and 5-10 years (21%). GTCS was the commonest type of seizure seen in 97 cases (97%) and focal seizure with secondary GTCS observed in 3(3%) cases. In most of the cases (24%) 2-4 times recurrence of seizure was observed since 1st diagnosis followed by 10-20 times in 15%, 5-10 times in 14, <2 times in 9 and >20 times in only one child. Focal Neurological deficit was seen in 24% cases, Positive family history in 11%, preceding aura 46%. The maximum number of children presented with fever of 100-104 F (53%) and 8% child with 105-106 F. Duration of fever was <2 days in 40% cases, between 4-8 days in 14% , 2-4 days in 13% and in one child duration of fever was >8days

 

Table 4: Spectrum of clinical presentation

Clinical presentation

No. of Cases

Percentage

Age of 1st Onset

 

 

< 2 month

11

11%

2-6 months

22

22%

6-12 months

23

23%

1-5 years

19

19%

5-10 years

21

21%

>10 years

04

4%

Seizure presentation

 

 

general tonic clonic seizures

97

97%

Focal with secondary general tonic clonic seizures

03

3%

Recurrence since 1st diagnosis

 

 

<2 times

09

9%

2-4 times

24

24%

5-10 times

14

14%

10-20 times

15

15%

>20 times

01

1%

Focal Neurological deficit

24

24%

No

76

76%

Positive family history

11

11%

No

89

89%

Preceding aura

46

46%

No

54

54%

Temperature

 

 

<1000F

08

8%

100-1040F

53

53%

105-1060F

08

8%

Fever duration

 

 

< 2 days

40

40%

2-4 days

13

13%

4-8 days

14

14%

>8 days

01

1%

In present study prehospital resuscitation was done in 31% cases. Most common symptom was vomiting (35%) followed by fever with URI (22%) and ALOC (20%) while the commonest sign was pallor (70%) followed by abnormal skull shape in 19% and syndromic appearance (10%). Majority of patients had- CBG in the ranged from 120 to 200 mg/dl (50%) followed by from 54 to 120 mg/dl. Sodium, potassium and calcium were low in 18%, 21% and 33% of cases respectively. Ongoing NCSE was present in 66% patients and intracranial pressure in 36%, clinical meningeal signs in 31%, involuntary movements was seen in 38%, Cerebellar signs in 6%. Sensory system activity involved in all i.e. 100% cases of status epilepticus which may have an important role in both generation and inhibition of seizures.Urinary incontinence (Incontinence bowel/bladder) occurs in 38% child with status epilepticus

 Table 5: Clinical examination profile of study population

Clinical Examination

No. of Cases

Percentage

Prehospital resuscitation

31

31%

Predominant post ictal presentation

 

 

Vomiting

35

35%

Fever

8

8%

Acute watery diarrhea

18

18%

Abdominal pain

10

10%

Lethargy

13

13%

Crying during micturation

05

5%

Fever + Upper respiratory infection

22

22%

Fever + Urinary tract infection

01

1%

Fever + Acute gastro-enteritis

03

3%

Altered Level of Consciousness

20

20%

Oliguria

01

1%

Clinical signs

 

 

Pallor

70

70%

Icterus

03

3%

Clubbing

01

15%

Lymphadenopathy

00

0.0%

Oedema

01

1%

Abnormal skull shape

19

19%

Syndromic appearance

10

10%

Cutaneous markers

01

1%

Others

06

6%

capillary blood glucose (mg/dl)

 

 

<54

01

1%

54-120

40

40%

120-200

50

50%

>200

09

9%

Nonconvulsive status epilepticus

66

66%

intracranial pressure clinical features

36

36%

Clinical meningeal signs

31

31%

Involuntary movements

38

38%

Cerebellar signs

06

6%

Sensory system

100

100%

Incontinence bowel/bladder +

38

38%

Most common etiology associated with status epilepticus in children was seizure disorder (44%), followed by acute CNS infection (34%), fever provoked seizures (24%) and Quadriplegia (19%).

Table 6: Etiology and diagnosis profile

Diagnosis

No. of Cases

Percentage

Seizure disorder

44

44%

Fever provoked seizures

24

24%

Unprovoked seizures

09

9%

Toxic encephalopathy

03

3%

Autoimmune encephalitis

04

4%

Acute CNS infection

34

34%

Stroke

08

8%

Quadriplegia

19

19%

Other - Systemic illness

03

3%

SEPSIS Screen positive in 93% cases, TORCH Screen positive in 8%, Viral Serology positive in 13% and Blood culture positive in 11%. CSF analysis was done for 81 children, cytology positive in 25%, Biochemistry positive in 30%, Culture/Sensitivity positive in 25% and CBNAAT positive for TB in 1 child. USG imaging study was done for 32 cases and USG abnormal in 26% cases, CT brain was done for 84 cases and 48 cases had abnormal findings. MRI done for 52 cases, 43% showed abnormal findings. Majority of children had abnormal epileptiform activity (73%).

 

Table 7: Investigation profile of study population

Parameters

No. of patients

Percentage

SEPSIS Screen positive

93

93%

TORCH Screen positive

08

8%

Viral Serology positive

13

13%

Blood culture positive

11

11%

CSF analysis

 

 

Cytology positive

25

25%

Biochemistry positive

30

30%

Culture/Sensitivity positive

25

25%

CBNAAT positive for TB

01

1%

Imaging study

 

 

USG positive

26

26%

USG normal

06

6%

CT brain positive

48

48%

CT brain normal

36

36%

MRI brain positive

43

43%

MRI brain normal

09

9%

EEG- Abnormal epileptiform activity

73

73%

Out of 20 children with abnormal neuroimaging, 7 children had refractory status epilepticus (RSE). Among the structural abnormality, most common was cystic encephalomalacia (6%), followed by porencephaly (4%), space occupying lesion-DNET (4%), polymicrogyria cortex (4%), and arachnoid cyst in temporal region (2%).

 

Table 8: Neuroimaging of children with status epilepticus

Neuroimaging

No. of Patients

Percentage

Normal

80

80%

Structural abnormality

20

20%

Cystic encephalomalacia

06

6%

Porencephaly

04

4%

Space occupying lesion-DNET

04

4%

Polymicrogyria cortex

04

4%

Arachnoid cyst in temporal region

02

2%

                  Mean duration of hospital stay was 7 days and 16 hours and maximum duration was 27 days, which was observed in one child who had RSE with prolonged respiratory failure and died on 27th day. Out of 100 cases, 76 cases (76%) recovered. Among those recovered, 6 cases (6%) recovered with new neurological sequel; 13 cases (13%) died and 5 cases (5%) discharged against medical advice (AMA).

 

 

 

 

Table 9: Final outcome

Outcome

No. of Patients

Percentage

Recovered without sequel

76

76%

Recovered with sequel

06

6%

Death

13

13%

AMA

05

5%

Of the 100 cases enrolled in the study, 87(87%) children survived and 13(13%) cases were died. The overall mortality rate among children with SE was 13 per 100. The various factors that influenced the morbidity and mortality following SE were selected and analyzed statistically. 13.79% of the boys and 11.90% of the girls treated for SE expired. But this gender difference was statistically insignificant (p Value = 0.435).

 

Table 10: Gender Distribution and Final Outcome

Gender

Survived

Expired

Total

Male

50 (86.20%)

08 (13.79%)

58 (100%)

Female

37 (88.09%)

05 (11.90%)

42 (100%)

Total

87 (87%)

13 (13%)

100 (100%)

P value

0.435

-

The mortality associated with SE was greatest (46%) in children between 2 to 5 years of age followed by 5 to 10 years (28%), less than 2 years (20%) and in children between 10 to 12 years of age (6%). This age difference was statistically significant with p value of 0.039.

Table 11: Age Distribution and Final Outcome

Age group (in years)

Survived

Expired

Total

<2

19 (95%)

01 (5%)

20 (20%)

2 to 5

41 (68.75%)

05 (31.25%)

46 (46%)

5 to10

22 (78.57%)

06 (21.42%)

28 (28%)

10 to12

05 (83.33%)

01 (16.66%)

06 (6%)

 

DISCUSSION

Status epilepticus is a condition resulting from a failure of mechanisms that terminate a seizure or from strong seizure predisposition or irritative mechanisms which may being flammatory, genetic, and cellularor sub-cellular, that perpetuate the seizure cycle. SE is often recurrent in epileptic children with structural malformations, resistant epilepsies, certain genotypes, recurrent inter-current illnesses and drug complianceissues.5 Status epilepticus is a common pediatric neurological emergency with an estimated incidence of 18–23 per 100,000 children per year and a mortality of 2%–7%.18 Access to specialist care is a major limiting factor in developing countries because of poor health infrastructure, connectivity, and delays in transportation.7 Thandavarayan et al.,8 noted that out of 92 children, male children were 46 (50%) and female children were 46 (50%). In Vafaee-Shahi et al.,9 study the prevalence of S.E in male and female children was 57.1% and 42.9%, respectively. Similar findings were noted in present study. In Thandavarayan et al.,9 study the mean age of the patient was 4.9 years and 58.6 % cases were children < 5 years. In Madhu et al.,10 study 63.2% cases were from the age group less than 5 years. Median age was 4 years. In Das et al.,11 study, it was found that children less than five years of age comprised the majority of the cases (63.8%) and median age was 3 (1-7) years. Admuthe et al.,12 in his study showed mean age was 4.5yrs and weak evidence of difference of sex. In Singh et al.,13 study the mean age of patients was 3.4 yrs. which is comparable with our study. Probably, mechanisms for control of seizure activity are fragile in younger children and may get disrupted with minimal abnormalities in neurofunction. Also, younger age is more vulnerable for acute etiologies including febrile seizures. Herdorffer et al.,14 reported when SE was associated with epilepsy it tended to be the first unprovoked seizure in 30% or it tended to be the seizure leading to diagnosis of epilepsy in 35%. They also reported 18% of unprovoked SE occurred in people with established epilepsy. Purusothaman and Kumar.,15 reported 40.7% children presented as SE without a prior history of seizures. 53.3% by Gulati et al.,16 in Kumar et al.,17 study 74.2% cases presented with SE without any prior history of seizures and 18 patients (25.7%) had one or more seizure episodes in the past. The aetiological spectrum of SE in developing countries is distinctly different when compared to developed countries. In developing countries CNS infections accounted for 28–67% of aetiological spectrum18,19 and this was much more so in the pediatric age group2,19. In the studies from developed countries, the reported frequency of CNS infections as the risk factor varied from 4% to 19%14. In Thandavarayan et al.,8 study the most common etiology associated with SE in 92 children was remote causes 27.2% (25 cases), next being acute CNS infection 19.6% (18 cases), febrile seizure 18.5% (17 cases) and cryptogenic/idiopathic SD 16.3% (15 cases). Murthy et al.,20 reported CNS infection accounts for significant number of cases. The etiology of seizures is usually analysed because this has been shown to affect the morality of SE and can affect the duration of seizures.21 Oliviera et al.,22 has made an observation similar to ours with 53% of their cases being symptomatic seizures. Majority of our children had abnormal epileptiform activity (73%) which is comparable with the study done by Purusothaman and Kumar.,15 where they reported abnormal epileptiform activity in 74.2% cases. In current study, it was observed that among the structural abnormality, most common was cystic encephalomalacia seen in 30% cases followed by porencephaly 20%, space occupying lesion-DNET 20%, polymicrogyria cortex 20 and arachnoid cyst in temporal region seen in 10% cases. This is in accordance with the study done by Purusothaman and Kumar.15 In present study out of 100 cases, 76 cases (76%) recovered. Among those recovered, 6 cases (6%) recovered with new neurological sequel; 13 cases (13%) died and 5 cases (5%) discharged against medical advice (AMA). In Thandavarayan et al.,8 study out of 92 cases, 74 cases (75%) recovered. Among those recovered, 5 cases (5.5%) recovered with new neurological sequel; 13 cases (14%) died and 5 cases (5.5%) discharged against medical advice (AMA) and acute CNS infection was commonest cause. In current study, the overall mortality rate among children with SE was 13 per 100. The studies from developed countries reported mortality between 9-24%.23,24 The various factors that influenced the morbidity and mortality following SE were selected and analyzed statistically. 13.79% of the boys and 11.90% of the girls treated for SE expired andgender difference was statistically insignificant. Similar findings were noted in other studies.10,11 The major determinants of fatal outcome identified by Sagduyu et al.,25 were increasing age, longer duration of SE before initiating therapy, CNS infection as a cause for SE. These factors correlate well with our study. Moreover the mortality rate in SE can vary significantly depending on study population and the settings in which the study is carried out. Proper prehospital therapy, early referral, proper care while transporting, anticipating risk factors involved, and protocol based approach uniformly at all hospital can reduce the mortality due to status epilepticus in children. Also considering the large number of referrals, upgradation of intensive care facilities even in tertiary care hospital is much sought to improve the overall outcome of SE.

 

CONCLUSION

Seizures are not only the cause of high morbidity and mortality in children but also are the reasons of physical, mental and financial distress for their parents. Male preponderance and younger age at presentation were the highlights. In present study, children below 5 years had a higher incidence of status epilepticus. The commonest type of seizure was a generalised tonic-clonic seizure. Seizure disorder, acute CNS infections, and febrile seizures were commonest etiological reasons. CSF analysis, Neuroimaging and EEG have the most important role in diagnosis of seizures. We suggest a long term follow-up study in patients with seizures with regards to their neuro-behavioral outcomes.

 

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