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Case report

Indian Pediatr 2015;52: 151-152

Benign Infantile Seizures


Devendra Mishra, Niraj Kumar Nikunj, Monica Juneja and *Bibek Talukdar

From Departments of Pediatrics, Lok Nayak Hospital and *Chacha Nehru Bal Chikitsalaya, Maulana Azad Medical College, Delhi, India.

Correspondence to: Dr Devendra Mishra, Department of Pediatrics, Maulana Azad Medical College and
associated Lok Nayak Hospital, Delhi 110 002, India.
Email: [email protected]

Received: July 15, 2014;
Initial review: August 21, 2014;
Accepted: November 26, 2014.

 

 

Background: Benign infantile seizures are a common form of idiopathic seizures in infants, but infrequently reported. Case characteristics: Four cases identified over a 9-month period. Observation: All had a cluster of focal seizures, normal development and no abnormality on hematological and biochemical work-up. Outcome: No recurrence of seizures over a follow-up of 5 to 9 months. Message: Identification of this syndrome has important therapeutic and prognostic implications.

Keywords: Epilepsy syndrome, Idiopathic, Infant.


Benign infantile seizures (BIS) are characterized by onset of seizures in the first 2 years of life, with no known cause and excellent outcome [1]. Infants with similar features but with a positive family history for infantile epilepsy are labelled as Benign familial infantile seizures (BFIS), which has an autosomal dominant inheritance [3]. Caraballo, et al. [4] reported BIS as the third most common form of epilepsy in the first two years of life. However, not much information is available on this syndrome among Indian infants, although previously it has been pointed out that benign epilepsies are under-represented in Indian studies on epilepsy [5]. We herein report four children with BIS identified prospectively.

Case Reports

These four children were identified during a 9-month period (April-December, 2012) wherein 75 infants with first seizure were prospectively studied. The various clinical characteristics of these four children are detailed in Table I. All had a cluster of focal seizures (secondary generalization in two), had normal development, normal neuroimaging and inter-ictal electroencephalography (EEG), and did not have recurrence of seizures. Ictal EEG was not possible.

TABLE I Salient Features of Seizures in the Study Subjects
Case 1 Case 2 Case 3# Case 4
Age (mo/Sex 9/F 1.5/M 3/M 3/M
No. of seizures 3 in 48 hr 4 in 24 hr 3 in 48 hr 2 in 24 hr
Further seizures in hospital No Yes, 9 No Yes, 3
CSF Not done Normal Not done Not done
DQ* (motor, mental) 104, 96 Normal$ 79, 93 79, 88
Follow-up 7 mo 5.5 mo 9 mo 5 mo
Outcome 2 seizure more, no further Discharged in 3 d on AED, No seizures No   seizures
seizures off-AED^ tapered in 3 mo, No seizures off-AED off-AED
*Developmental quotient using DASII, 3mo after discharge; #Father and paternal uncle had history of seizures in infancy; $by non-formal assessment; ^Antiepileptic drugs discontinued by parents on their own.

Discussion

In a well-infant presenting with the first afebrile seizure, the initial concern is the possibility of a metabolic cause or an idiopathic epilepsy syndrome. Other than West syndrome, BIS and BFIS are the commonest epileptic syndromes reported in this age [4]. Onset is mostly within the first year of life in both syndromes and seizures occur in clusters, have focal features including behavioral arrest, cyanosis, head/eye version, tonic stiffening of the limbs, and bilateral clonus [3]. The cluster can last 1 to 3 days. Interictal EEG is normal in the majority; ictal abnormalities have been described in a few patients. Initial genetic studies reported specific chromosomal-linkages, though later studies have suggested genetic heterogeneity. More recent data supports the hypothesis that this disease may be a channelopathy [2,6]. Another subgroup with associated paroxysmal choreoathetosis has also been described [6]. As per ILAE classification [7], both BFIS and BIS are considered similar, except for the family history.

Generally, no further seizures are observed in cases treated pharmacologically. In untreated cases, there can be isolated or brief clusters within one year of age. Treatment with antiepileptic medication is not mandatory [8]. We started antiepileptic drugs in first two patients, but with increasing confidence about this diagnosis, we discharged the next two infants without drugs, and they did not have any seizure recurrence over the next 5 to 9 months.

The most characteristic feature of the syndrome is the occurrence of a cluster of few brief seizures, lasting for 1-3 days, with the child being well inter-ictally [9]. Recognition of this syndrome helps in avoiding long term anti-epileptic therapy.

Contributors: DM: diagnosed the cases, prepared the manuscript, and will be the guarantor; NKN: managed the patients, searched the literature, and helped in manuscript preparation. MJ, BT: Provided important intellectual inputs during patient management and manuscript preparation; BT: reported EEG findings. All authors approved the final manuscript.

Funding: None; Competing interests: None stated.

References

1. Watanabe K, Okumura A. Benign partial epilepsies in infancy. Brain Dev. 2000; 22:296-300.

2. Specchio N, Vigevano F. The spectrum of benign infantile seizures. Epilepsy Res. 2006;70 Suppl 1:S156-67.

3. Vigevano F, Fusco L, Di Capua, Ricci S, Sebastianelli R, Lucchini P. Benign infantile familial convulsions. Eur J Pediatr. 1992;151:608-12.

4. Caraballo RH, Cersósimo RO, Espeche A, Fejerman N. Benign familial and non-familial infantile seizures: A study of 64 patients. Epileptic Disord. 2003;5:45-9.

5. Udani V. Pediatric epilepsy – An Indian perspective. Indian J Pediatr. 2005;72:309-13.

6. Vigevano F. Benign familial infantile seizures. Brain Dev. 2005; 27:172-7.

7. Engel J, Jr. Report of the ILAE Classification Core Group. Epilepsia. 2006; 47:1558-68.

8. Caraballo RH, Cersósimo RO, Amartino H, Szepetowski P, Fejerman N. Benign familial infantile seizures: Further delineation of the syndrome. J Child Neurol. 2002;17: 696-9.

 

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