JICNA®| Journal of the International

Child Neurology Association

A peer reviewed open access e-journal in Child Neurology

Epileptic spasms at Muhimbili National Hospital Tanzania, a

retrospective study
Joseph Mwalongo1 and Edward Kija MPhil2
1 Department of Paediatrics and Child Health, School of Medicine, Muhimbili University of Health and Allied Sciences: Dar es
salaam, Tanzania
2 Paediatric Neurology Unit, Department of Paediatrics and Child Health, Muhimbili National Hospital, Dar es salaam, Tanzania

Corresponding author: Edward Kija; [email protected]

https://doi.org/10.17724/jicna.2021.209
Received: 18 February, 2021
Accepted: 30 December, 2021

Abstract
Background: Epileptic spasms (ES) is an epileptic encephalopathy occurring during infancy and early childhood. Early recognition
and management are important to prevent severe neurological impairment. This study aimed at describing the clinical presentation,
management, and outcome of patients with Epileptic Spasms attending Muhimbili National Hospital (MNH) in Dar Es Salaam,
Tanzania Methods: A retrospective cross-sectional study of all patients diagnosed with epileptic spasms was conducted at MNH
from July 2016 to January 2021. Results: A total of 73 patients diagnosed with epileptic spasms were retrieved with a male to
Open Access. Published by the JICNA

female ratio of 3:2. In this study, 37 (50.7%) patients had a documented history of perinatal insult. The median age of onset of
spasms was five months (IQR 1-12 months). Thirty (88%) out of 34 patients whose electroencephalography (EEG) findings were
retrieved had abnormal EEG findings showing generalized epileptiform discharges in 11(32.4%), generalized slowing in 5(14.7%),
hypsarrhythmia in 7(20.6%), focal epileptiform discharges in 3 (8.8%), and suppression burst pattern in 4(11.8%). Fifty- six (77%)
received prednisolone, with a median time of spasms cessation of 1 month with a range of 10 days to 3 months in about 80% of them.
Conclusion: The median age of onset of epileptic spasms at MNH is five months, with the most common cause being a perinatal
insult in more than 50% of the patients. High dose prednisolone showed a good response in patients with epileptic spasms at MNH.
Keywords: Epileptic spasms, hypsarrhythmia, prednisolone.
© J Mwalongo & E Kija; licensee JICNA

Introduction This study was conducted to describe the clinical presentation,

etiology, management, and outcome of patients with Epilep-
Epileptic spasms (ES), formerly called infantile spasms, is a tic Spasms attending Muhimbili National Hospital in Dar Es
sudden flexion, extension, or mixed extension–flexion of pre- Salaam, Tanzania.
dominantly proximal and truncal muscles lasting about 2-3 sec-
onds, that is usually more sustained than a myoclonic event but
not as sustained as a tonic seizure. Other manifestations which Methods
may occur during an episode of epileptic spasms include Gri- A retrospective cross-sectional study of all patients diagnosed
macing, head nodding, and subtle eye movements. Epileptic with epileptic spasms was conducted at MNH. Paper based case
spasms frequently occur in clusters predominantly upon awak- notes and an electronic database of all patients with neurolog-
ening. Epileptic spasms commonly occur during infancy. How- ical conditions under the age of five years for both inpatients
ever, these events have been reported to occur in children who and outpatients from July 2016 to January 2021 were retrieved
are older than one year [1]. It is one of the commonest epilep- and thoroughly reviewed. Patients who met the diagnostic cri-
tic encephalopathies causing developmental regression and in- teria for ES as stipulated by the International League Against
tellectual disability. The incidence of epileptic spasms has been Epilepsies (ILAE) 2017 edition were recruited into the study [1].
estimated to range from 2–5/10,000 newborns globally. In most A structured questionnaire specifically designed for this study
cases, ES is due to perinatal hypoxia, with some cases being a re- was used to collect data from the paper-based case notes and
sult of different post-natal factors. The most common etiologies electronic database of the hospital. The data collected included
of ES reported are hypoxic-ischemic encephalopathy, chromo- social demographic characteristics, clinical presentations, elec-
somal abnormalities, brain malformations, stroke, tuberous scle- troencephalography (EEG) findings, Magnetic Resonance Imag-
rosis complex, and periventricular leukomalacia or hemorrhage ing (MRI) features, medications used, and the outcome. The
[2, 3, 4]. Poor antenatal, natal, and post-natal care stands out as outcome assessed in this study was the duration of cessation of
the main underlying problem in developing countries. the spasms.
 J Mwalongo & E Kija – JICNA 2021, 22(209)

Table 1. Etiology, EEG, MRI findings, and medications received

Results
by patients with epileptic spasms.
Paper based case notes and an electronic database for 2740 Etiology of ES (N=37) N (%)
patients were reviewed, and 73 met the criteria for ES. Most of
Birth asphyxia 22 (59.5%)
the patients were males, constituting about 60% of the studied
patients. More than 80% (60 patients) included in the study were Intrauterine infections 4(10.8%)
below three years of age, with the rest being 4 to 5 years old. Prematurity 4(10.8%)
In this study, most patients with epileptic spasms had a his- Preeclampsia 1(2.7%)
tory of a neurological insult that may have occurred during the Hyperbilirubinemia 2(5.4%)
prenatal, natal, or post-natal period. Among 37 patients whose Brain malformations 4 (10.8%)
data on etiology were retrieved, 22 (59.5%) had birth asphyxia,
EEG Findings (N=34)
2(5.4%) had neonatal hyperbilirubinemia, and 1(2.7%) had a his-
tory of preeclampsia. Moreover, intrauterine infections, congen- Generalized epileptiform discharges 11 (32.4%)
ital brain formation, and prematurity occurred in 4(10.8%) each. Generalized slowing 5 (14.7%)
The genetic component of epileptic spasms could not be eluci- Hypsarrhythmia 7 (20.6%)
dated in this study as it is not available in our setting. The median Abnormal focal epileptiform discharges 3 (8.8%)
age of onset of patients with Epileptic spasms was five months Suppression burst 4 (11.8%)
(IQR 1-12 months). The nature of spasms demonstrated in a
Normal finding 4 (11.8%)
large number of patients with epileptic spasms was flexor type
68 (93%), with the remainder having both flexor and extensor MRI Findings (N=15)
type (mixed type). Most patients in this study had a variable doc- Abnormal 12 (80.0%)
umented number of frequencies of spasms per day, with most of Normal
Open Access. Published by the JICNA

them, about 20 (30%), having 2 to 5 clusters per day and 3 (4%) Medications received before the diagnosis (N= 73)
having more than 10 clusters per day. Thirty (88%) out of 34
Sodium Valproate 61 (83.6%)
patients whose EEG reports were retrieved had abnormal EEG
findings showing hypsarrhythmia 7(20.6%), generalized epilep- Phenobarbitone 18 (24.7%)
tiform discharges 11(32.4%), generalized slowing 5(14.7%), fo-
cal epileptiform discharges 3 (8.8%) and suppression burst pat-
tern 4(11.8%). Twelve (80%) out of 15 patients whose magnetic
resonance imaging (MRI) results were found had documented
Discussion
abnormal MRI findings, with most of them showing features sug- The study aimed at describing the clinical presentation, eti-
gestive of hypoxic-ischemic encephalopathy (HIE), old subdural ology, management, and outcome of patients with Epileptic
hematoma, megalencephaly, and bilateral hippocampal atrophy, Spasms seen at Muhimbili National Hospital. The median age
severe brain atrophy, multicystic encephalomalacia, congenital at onset was five months, and most patients showed a good re-
hydrocephalus, and dandy walker malformations. MRI costs sponse to high-dose prednisolone.
stand out to be the limiting factor as many parents cannot afford
The study showed most of the affected children had their onset
them.
of epileptic spasms before the first year of life, with a peak inci-
The majority of the patients, 65 (89%) in this study, went dence in almost 60% being between 2 to 6 months and affecting
through a course of different medications singly or in com- slightly more males than females. Similar findings were reported
bination before the symptoms subsided. Before the diagno- in a multicenter randomized, double-blind clinical trial to assess
sis of epileptic spasms was made, many patients were receiv- the efficacy of Vigabatrin, and ACTH conducted in the United
ing the following medications: sodium valproate 61(84%) and States by Pellock et al., where the peak onset was 3 to 7 months
phenobarbitone 18 (25%). Other medications given were car- which occurred in 50-70% of the patients [5]. The pathogene-
bamazepine, clonazepam and Baclofen. After the diagnosis of sis of epileptic spasms is postulated to result from disruption of
epileptic spasms was made, 56 (77%) patients were documented neuronal network function as a result of a defect either at the
to receive prednisolone, and 4 (6%) received Vigabatrin. cellular, receptor, or molecular level, leading to abnormal inter-
Thirty-three (80%) out of 41 documented patients treated for action between cortical and subcortical structures [6, 7, 8]. Sig-
epileptic spasms with prednisolone, their symptoms subsided nificant and rapid brain development occurs during the first year
within the first month of treatment with an interquartile range of life involving myelination, synaptogenesis, and pruning; sub-
from 10 days to 3 months. sequently, if any of these processes is severely affected, epileptic
spasms and/or other seizure types are likely to arise during this
period.
Patients in this study demonstrated two clinical types of
spasms, with the majority of patients, 68 (93%), showing flexor
type, and 7% had mixed flexor-extensor type involving the mus-
cles of the neck, trunk, and extremities. This is different from

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 J Mwalongo & E Kija – JICNA 2021, 22(209)

the study done by Kellaway et al. in Texas, U.S.A using time- patients received sodium valproate 61(84%) and phenobarbitone
synchronized video EEG recording in 24 infants who showed 18 (25%). Other medications given were carbamazepine, clon-
most infants tend to have more than one type of spasms as 42% azepam, and baclofen. After the diagnosis of epileptic spasms
had a mixed type, 34% flexor, and 23% extensor type [9]. The was made, 56 (77%) patients received prednisolone. Thirty-three
differences in results are attributed to the variations in study de- (80%) out of 41 patients who received prednisolone, spasms sub-
signs where the retrospective method carried out in this study sided within the first month of treatment with an interquartile
could have missed some important detailed semiology of the range of 10 days to 3 months, similar to a randomized clinical
spasms as parents/caretakers are usually frightened by the event, trial by Lux et al. in which 70% responded with a median du-
and some forget the details of the semiology of the events which ration of 14 days after being given high dose prednisolone for
were easily picked up in the prospective study with video record- 14 days. This study emphasizes that high-dose prednisolone
ing done in Texas, U.S.A. is effective in the cessation of spasms even in resource-limited
Perinatal insults accounted for 50% of the etiologies of ES settings where the diagnosis is often made late [12]. The pred-
in this study compared to 10%, which were found in a multi- nisolone dose given was 4mg/kg/day during the first two weeks
center study done in Canada, France, and the United States of in divided dosages, then tapered down for the next four weeks.
America. Inadequate perinatal care in our setting, as compared
to developed countries, leads to an increased number of chil-
dren who experience perinatal insults leading to a higher propor-
Conclusion
tional contribution to ES. Most patients with ES in this study had The median age of onset of epileptic spasms at MNH is five
Cerebral Palsy. The risk factors for cerebral palsy found in this months. The most common cause of epileptic spasms in children
study were birth asphyxia, intrauterine infections, neonatal hy- at MNH is perinatal insults occurring in more than 50%. Despite
perbilirubinemia, preeclampsia, and prolonged labor, which are late presentation, most patients showed a good response to high-
all preventable with improved antenatal care. Other etiologies dose prednisolone.
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found in that multicenter study included genetic (8%), Tuberous

Sclerosis (7%), stroke (8%), cerebral malformations including
porencephaly (8%), and periventricular leukomalacia (5%) [2]. Abbreviations
In this study, 30 (88%) out of 34 patients whose EEG reports
were retrieved had abnormal EEG findings. The typical EEG EEG Electroencephalogram
finding in ES of hypsarrhythmia was only found in 7(20.6%) ES Epileptic spasm
compared to 75%, which has been reported in other studies [10]. HIE Hypoxic ischemic encephalopathy
This could be attributed to late diagnosis in most of our patients,
IQR Inter quartile range
as well as EEG being done late during the course of illness af-
ter receiving several medications, which could have altered the MRI Magnetic resonance imaging
EEG patterns. Poor awareness of the condition among health MUHAS Muhimbili University of Health and Allied Sciences
care workers and family financial constraints are key in limiting MNH Muhimbili National Hospital
early access to EEG. Other EEG findings reported in this study SPSS Statistical package for the social sciences
were generalized epileptiform discharges 11 (32.4%), general-
ILAE International League Against Epilepsy
ized slowing 5(14.7%), focal epileptiform discharges 3 (8.8%),
or suppression burst 4(11.8%).
In this study, 12 (80%) out of 15 patients whose MRI re- Ethical issues
sults were found had documented abnormal features, with most
of them showing features suggestive of hypoxic-ischemic en- Permission for data collection was obtained from the admin-
cephalopathy (HIE) due to perinatal insults. The abnormal MRI istration of Muhimbili National Hospital (MNH) and MUHAS
findings included old subdural hematoma, megalencephaly, bi- with letter Ref.No.DA.282/298/01.C, and the data were kept as
lateral hippocampal atrophy, severe brain atrophy, multicystic confidential information.
encephalomalacia, congenital hydrocephalus, and dandy walker
malformations. In a retrospective study done by Khatami et al. Acknowledgements
in Canada on Brain MRI findings in patients with ES where a
The authors would like to thank the MNH administration for
total of 26 patients were included, 19 (73%) had abnormal MRI
permission to access their data system and for the cooperation
findings, which were features of HIE sequalae, tuberous sclero-
received from the pediatrics and child health department.
sis (including one with megalencephaly), Lissencephaly, infarcts
secondary to meningitis and frontal heterotopia. This shows
structural causes of ES are quite similar between Africa and the
Competing interests
west [11]. The authors declare that they have no competing interests.
The majority of the patients, 65 (89%) in this study, went
through a course of different medications before the spasms sub-
sided. Before the diagnosis of epileptic spasms was made, many

3
 J Mwalongo & E Kija – JICNA 2021, 22(209)

Author contributions [10] Hrachovy RA, Frost JD. Infantile Epileptic En-
cephalopathy with Hypsarrhythmia (Infantile Spasms/West
JM and EK assisted with study design, identifying patients for Syndrome). Journal of Clinical Neurophysiology.
study inclusion, data collection, and results analysis. JM drafted 2003;20(6):408-25. PubMed.
the manuscript. EK contributed to the manuscript content and
[11] Khatami A, Sell E, Aggag M, Miller E. Brain MRI
revised the final manuscript.
Findings in Infantile Spasm: Outcome Correlations in
This is an Open Access article distributed under the a Patient Cohort. Open Journal of Medical Imaging.
terms of the Creative Commons Attribution License 2016;06(03):80-92.
(http://creativecommons.org/licenses/by/4.0), which per-
[12] Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy
mits unrestricted use, distribution, and reproduction in any
CR, Newton RW, et al. The United Kingdom Infan-
medium, provided the original work is properly credited.
tile Spasms Study comparing vigabatrin with prednisolone
The Creative Commons Public Domain Dedication waiver
or tetracosactide at 14 days: a multicentre, randomised
(http://creativecommons.org/publicdomain/zero/1.0/) applies to
controlled trial. The Lancet. 2004;364(9447):1773-8.
the data made available in this article, unless otherwise stated.
PubMed.
Cite this article as: J Mwalongo & E Kija. (2021). Epileptic
spasms at Muhimbili National Hospital Tanzania, a retrospective
study . Journal of the International Child Neurology Association,
22(209). https://doi.org/10.17724/jicna.2021.209

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