INTRODUCTION

Infantile epileptic encephalopathy with burst-suppression is a rare form of neonatal

encephalopathy with a characteristic electroencephalographic pattern responsible for
significant psychomotor impairment. The etiologies are dominated by cortical or
metabolic dysplasia [1,2,3], particularly in Africa, where perinatal anoxo-ischemic
causes have been reported [4,5].
CASE PRESENTATION
Clinical case 1
In this case we are dealing with a 7-month-old female infant whose parents had
consulted for generalized motor seizures occurring several times a day and delayed
psychomotor acquisition. Her ante- and perinatal history was unremarkable. At 7 days
of age, she was admitted to hospital with a nonfebrile status epilepticus of generalized
myoclonus. Examination revealed axial hypotonia and delayed psychomotor
acquisition, with head holding and sitting position not acquired. Microcrania was
noted, with a head circumference of 34cm (-4 Standard Derivation). Paraclinical
examinations revealed diffuse cortico-subcortical atrophy on cerebral MRI (Fig. 1a
and 1b). The electroencephalogram showed an overpressure-burst pattern. TORCH
serological tests revealed a long-standing infection, with toxoplasmosis serology
positive for Immunoglobulin G at 1360 IU/ml and rubella serology positive for
Immunoglobulin G at 367 IU/ml.
Clinical case2
30-month-old infant, male, with no pathological ante- or perinatal history,
hospitalized at 3 days of age for non-febrile generalized motor convulsive seizures.
He presented with epileptic seizures several times a day, consisting of generalized
tonic-clonic seizures and brief tonic left hemicorporeal focal seizures. Clinical
examination revealed axial hypotonia, hypomotility of the left hemicorpore, delayed
psychomotor acquisition and absence of head posture. Macrocrania with PC at 48cm
(+3D Standard Derivation). Cerebral MRI revealed right hemimegaly (Fig. 2a and
2b), and electroencephalogram showed a burst-suppression patern (Fig 6a).
Clinical case3
A 26-month-old male infant consulted for generalized tonic spasms and delayed
psychomotor acquisition. Ante-, peri- and postnatal history was unremarkable. The
parents had noted delayed motor acquisition at 16 months and generalized tonic motor
seizures. Clinical examination revealed axial hypotonia and spastic tetraparesis. A
significant delay in psychomotor acquisition, with head holding, sitting, standing and
walking not acquired, and an absence of eye contact. Brain MRI revealed type 1
lissencephaly (Fig. 3a and 3b) and electroencephalogram abnormalities characteristic
of suppression-burst.
Clinical case 4
This was an 8-month-old patient with a history of severe oligohydramnios on obstetric
ultrasound in the third week of pregnancy, and premature delivery at 34 weeks'
amenorrhea. A congenital toxoplasmosis infection with negative Immunoglobulin M
and positive Immunoglobulin G serologies at 12290 IU/ml had prompted
hospitalization in the neonatal period. She presented with generalized tonic seizures
several times a day. Neurological examination showed axial hypotonia. There was an
absence of eye contact and poor reactivity to sound and visual stimuli. Brain MRI
revealed hyraanephaly (Figures 4a and 4b). The electroencephalogram showed a
suppression burst pattern.
Clinical case 5
8-month-old patient consulted for generalized convulsive seizures and delayed
psychomotor acquisition. She was the offspring of a twin pregnancy. Perinatal history
showed premature delivery at 33 weeks' amenorrhea. Brief generalized tonic spasms
were noted at 1 day of age. Examination revealed axial hypotonia and spastic
tetraparesis. Eye contact was inconsistent, with poor interaction. Brain MRI revealed
bilateral type 2 schizencephaly (Figures 5a and 5b). The electroencephalogram
showed a suppression-burst pattern (Figure 6b).
DISCUSSION
Epileptic encephalopathy with burst-suppression (EEBS) is characterized by the onset
of early seizures in the first three months of life, but some cases can occur in the first
weeks of life[1,6]. In our series, seizures appeared early in the neonatal period, with
infants presenting with generalized, symmetrical tonic spasms most often, but other
types of seizures may also occur, such as generalized tonic-clonic seizures, focal
motor seizures and hemiconvulsions. These seizures occur several times a day,
isolated or in slaves in the same patient [1,7]. Our study reported several times a day
generalized motor seizures of the myoclonic or tonic spasm type. Neurocognitive
acquisition was also delayed in all our patients. Because of its frequent seizures and
refractory convulsive states, EEBS is associated with high neonatal mortality (50% of
cases in the neonatal period) and severe disability in 18.18 cases [8]. The EEG pattern
is characteristic, with alternating cerebral activity and cerebral silence or
hypofunction due to a profound state of cerebral inactivation[9,10].
Imaging revealed cortical dysplasia with significant structural lesions such as
hemimegalencephaly, lissencephaly, hydraanencephaly and shizencephaly,
corroborated by data in the literature[1,2]. Our study reports two cases of congenital
infection with toxoplasmosis and rubella.
CONCLUSION
Infantile epileptic encephalopathy with burst-suppression is responsible for a severe
clinical condition with daily early-onset epileptic seizures in the neonatal period and
significant psychomotor impairment.

CONFLICT OF INTERE0ST
We have no conflicts of interest.
DATA AVAILABILITY
All data from this study are available from the corresponding author under a
reasonable request.
AUTHORS' CONTRIBUTIONS
Julie Marie Adeline W Kyelem,Raphael Marie Kabore, Yakouba Haro contributed to
the data analysis and writing of the article. Djingri Labodi D Lompo, Alfred Anselme
Dabilgou, Christian Napon and Athanase Millogo supervised the writing.
ETHICAL CONSIDERATIONS
Our study is not unethical
We have no source of funding
We confirm that the current manuscript is not submitted to other journals.
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