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Indian Pediatr 2015;52: 151 -152 |
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Benign Infantile Seizures
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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.
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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.
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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
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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
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No seizures
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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
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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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