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Table of Content - Volume 20 Issue 1 - October 2021


 

Study of etiological profile and neurological outcome of children’s presenting with status epilepticus

 

Ramchandra Nagargoje1*, Sandhya Bilaspur2, Sunil Holikar3

 

1Assistant Professor, 2Resident, Department of Pediatrics, Swami Ramanand Teerth Rural Government Medical College, Ambajogai, Beed, Maharashtra, INDIA.

3Associate Professor, Department of Pediatrics, Vilasrao Deshmukh Government Medical Medical College, Latur, Maharashtra, INDIA.

Email: nagargojeramchandra@gmail.com

 

Abstract              Background: Status epilepticus is a neurological emergency requiring immediate evaluation and management to prevent significant morbidity or mortality. 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 undertaken to provide additional information about etiological profile and neurological outcome of children’s presenting with status epilepticus. Material and Methods: Present study was prospective and observational study, conducted in children of age 1month to 12 years who presented with status epilepticus. Results: Maximum numbers of male and female patients were in the age group of 2 to 5 years (46%) followed by 5 to10 years (28%). In present study, in majority of cases age of 1st onset of seizure was 6-12 months (23%), GTCS was the commonest type of seizure (97%), focal Neurological deficit was seen in 24% cases, Prehospital resuscitation was done in 31% cases. Most common symptom was vomiting (35%) followed by fever with URI (22%) and ALOC (20%) Most common etiology associated with status epilepticus in children was seizure disorder 44% (44 cases), next being acute CNS infection 34% (34 cases), fever provoked seizures 24% (24 cases) and Quadriplegia 19% (19 cases). Out of 20 children with abnormal neuroimaging, 7 children had refractory status epilepticus (RSE). 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: Male preponderance and younger age at presentation were the highlights. The commonest type of seizure was a generalised tonic-clonic seizure. Seizure disorder, acute CNS infections, and febrile seizures were commonest etiological.

Keywords: Status epilepticus, Seizures, generalised tonic-clonic seizure, Neuroimaging

 

INTRODUCTION

Status epilepticus is a neurological emergency requiring immediate evaluation and management to prevent significant morbidity or mortality. According to International League Against Epilepsy (ILAE) defines status epilepticus as a seizure which shows no clinical sings of an arresting after a duration encompassing the great majority of seizures of that type in most patients or recurrent seizures without resumption of baseline central nervous system function interictally.1 An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.1 A seizure that lasts longer than 5 minutes, or having more than 1 seizure within a 5 minutes period, without returning to a normal level of consciousness between episodes is called status epilepticus. This is a medical emergency that may lead to permanent brain damage or death.2 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%.3 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.4 Present study was undertaken to provide additional information about etiological profile and neurological outcome of children’s presenting with status epilepticus.

              

MATERIAL AND METHODS

Present study was prospective and observational study, conducted in Department of Pediatrics, Swami Ramanand Teerth Rural Government Medical College, Ambajogai, India. Study duration was of 2 years (1st November 2018 to 31st October 2020). The study was commenced after the institution ethical committee clearance.

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.

All consecutive cases of status epilepticus-those admitted during the study period. 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 postnatal 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

A total 100 Children’s of age 1month to 12 years who presented with status epilepticus were enrolled in the study. A total 100 Children’s of age 1month to 12 years who presented with status epilepticus were studied during this study period. 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. 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%

Total

58

42

100

100%

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

SGA

05

95%

AGA

90

Preterm

SGA

02

5%

AGA

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%

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%

In present study, in majority 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 (GTCS)

97

97%

Focal seizures

00

0.0%

Focal with secondary GTCS

03

3%

Myoclonus

00

0.0%

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%

Positive family history

11

11%

Preceding aura

46

46%

Temperature

 

 

<100F

08

8%

100-104 F

53

53%

105-106 F

08

8%

Fever duration

 

 

< 2 days

40

40%

2-4 days

13

13%

4-8 days

14

14%

>8 days

01

1%

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%. 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 diarrhoea

18

18%

Abdominal pain

10

10%

Lethargy

13

13%

Crying during micturition

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 (CBG) mg/dl

 

 

<54

01

1%

54-120

40

40%

120-200

50

50%

>200

09

9%

Dyselectrolytemia

 

 

Na+ Low

18

18%

K+ High

03

3%

K+ Low

21

21%

Ca+ Low

33

33%

Ongoing Nonconvulsive status epilepticus

66

66%

intracranial pressure clinical features

36

36%

Clinical meningeal signs

31

31%

Involuntary movements

38

38%

Cerebellar signs

06

6%

Incontinence bowel/bladder +

38

38%

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

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 was 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%). Out of 20 children with abnormal neuroimaging, 7 children had refractory status epilepticus (RSE). 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.

 

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%

CT brain positive

48

48%

MRI brain positive

43

43%

EEG- Abnormal epileptiform activity

73

73%

Structural abnormality

 

 

Cystic encephalomalacia

06

30%

Porencephaly

04

20%

Space occupying lesion-DNET

04

20%

Polymicrogyria cortex

04

20%

Arachnoid cyst in temporal region

02

10%

Mean duration of hospital stay was 7 days and 16 . 10 children (10%) were discharged within 5 days after admission. 59 children (59%) were discharged within 15 days and 29 (29%) children were discharged within 25 days after admission. Prolonged hospital stay of >25 days observed in 2 cases.

 

Table 8: Number of days of hospital stay

Hospital stay (days)

No. of patients

Percentage

<5

10

10%

5-10

31

31%

10-15

28

28%

15-20

17

17%

20-25

12

12%

>25

02

2%

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%

Total

100

100%

 


DISCUSSION

Status epilepticus is a common paediatric neurological emergency with an estimated incidence of 18–23 per 100,000 children per year and a mortality of 2%–7%.5 In developing countries, optimal management of status epilepticus is associated with major barriers such as poor health care infrastructure, delay in transportation, lack of ambulance services, intermittent availability of drugs and economic affordability.6In present study male predominance was noted, which was not statistically significant Similar findings were noted by Thandavarayan et al.,7 study out of 92 children, male children were 46 (50%) and female children were 46 (50%).. Likewise, in Vafaee-Shahi et al.,8 study the prevalence of S.E in male and female children was 57.1% and 42.9%, respectively. Male to female ratio in our study was found to be 1.38:1. Predominant involvement of younger age group has been reported previously in many studies.3,5,9 The reason for this predominance is 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. 54% of our cases presented as SE without prior history of seizures. Purusothaman and Kumar10 reported 40.7% children presented as SE without a prior history of seizures. 53.3% by Gulati et al.11., in Kumar et al.3 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. Therefore, it was observed that association of prior seizure episodes with occurrence of SE was not significant (p>0.005). GTCS was the commonest type of seizure seen in 97 cases (97%) and focal seizure with secondary GTCS observed in 3(3%) cases which is comparable with study done by Purusothaman and Kumar10 where a generalized tonic-clonic seizure was the most common type 77.7% and Focal seizures observed in 2(3.7%). The most common seizure type observed in the Gulati et al.11 study was GTCS (63.3%), which is similar to the present study. The aetiological spectrum of SE in developing countries is distinctly different when compared to developed countries. In present study, the most common etiology associated with status epilepticus in children was seizure disorder (44 %), next being acute CNS infection (34 %), fever provoked seizures (24 %) and Quadriplegia(19 %). In developing countries CNS infections accounted for 28–67% of aetiological spectrum6,12 and this was much more so in the paediatric age group12,13. 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.,7 study the most common etiology associated with SE in 92 children was remote causes 27.2%, next being acute CNS infection (19.6%), febrile seizure (18.5%) and cryptogenic/idiopathic SD (16.3%). Murthy et al.,14 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.15 Neuroimaging of children with status epilepticus showed that out of 20 children with abnormal neuroimaging, 7 children had refractory status epilepticus (RSE). In Purusothaman and Kumar10 study, structural abnormality was seen in 10 (18.5%) cases of them 7 children (12.2%) presented with refractory status epilepticus. Out of 100 cases, 76 cases (76%) recovered. Among those recovered, 6 cases (6%) recovered with new neurological sequele; 13 cases (13%) died and 5 cases (5%) discharged against medical advice (AMA). In Thandavarayan et al.,7 study out of 92 cases, 74 cases (75%) recovered. Among those recovered, 5 cases (5.5%) recovered with new neurological sequele; 13 cases (14%) died and 5 cases (5.5%) discharged against medical advice (AMA). 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%.16,17 Morbidity and mortality from status epilepticus seems to be related to three factors: CNS damage due to the underlying illness or acute insult causing the status epilepticus, CNS damage caused by repetative electrical discharge of the seizure itself and Systemic and metabolic factors related to repeated Generalized tonic-clonic seizures (GTCS) type of seizures.17The major determinants of fatal outcome identified by Sagduyu et al.19 were increasing age, longer duration of SE before initiating therapy, CNS infection as a cause for SE. These factors correlate well with our study. However, in current study 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. 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. The commonest type of seizure was a generalised tonic-clonic seizure. Seizure disorder, acute CNS infections, and febrile seizures were commonest etiological. A good clinical evaluation and if required CSF analysis, Neuroimaging and EEG have the important role in diagnosis. We suggest a long term follow-up study in patients with seizures with regards to their neuro-behavioral outcomes.

 

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