The study aimed at describing the aetiology, clinical presentation, management and outcome of patients with Epileptic Spasms seen at Muhimbili National Hospital. The median age at onset was 5 months and most patients have shown to respond better on high dose prednisolone.
The study showed most of the affected children had their onset of epileptic spasms before the first year of life with peak incidence of onset of almost 70% being between two and six months with males being slightly more affected thanfemales although the gender difference is non-significant. The study done by Pellock JM et al showed 50 to 77% of patients had their peak age of onset at 3 to 7 months. The results are almost similar to this study as the study population were the same as the immature central nervous system is more at risk of developing spasms.(5)
Patients in this study demonstrated two clinical types of spasms with majority of patients 38 (97%) showing flexor type and 3% had mixed flexor-extensor type involving the muscles of the neck, trunk, and extremities. This is different from the study done by M.Stand et al using time synchronized video and polygraphic recording in 24 infants which showed most infants tend to have more than one type of spasms as 42% had mixed type, 34% flexor and 23% extensor type. The differences in results is attributed to the variations in the study designs where the retrospective method carried out in this study could have missed some important parts of the spasms event which were easily picked in the prospective study (synchronized and polygraphic recordings) in their study. (6)
As far as etiology of epileptic spasms is concerned, the study pointed out that conditions associated with perinatal hypoxia remained to be the main culprit in 17 (42.5%) of the documented patients. The most common established for epileptic spams was cerebral palsy. The risk factors for cerebral palsy were birth asphyxia, intrauterine infections, neonatal hyperbilibinemia, preeclampsia and prolonged labour. Other established causes of epileptic spasms in this study were tuberous sclerosis complex, periventricular leukomalacia or haemorrhage and Arnold Chiari brain malformation. A multicentre study done on developed countries (Canada, France and United states) by S.Semiology on ES showed hypoxic ischaemic encephalopathy (10%), genetic (8%), Tuberous Sclerosis (7%), other cerebral malformation (8%), stroke, including porencephaly (8%), periventricular leukomalacia (5%).The difference observed is contributed by improved peri natal care in developed countries compared to developing countries. (2)
In this study, 14 (80%) out of 17 patients whom EEG findings were retrieved had abnormal EEG findings, showing either generalized epileptiform discharges 7(41%), generalized slowing 5(30%), hypsarrthymia 1(6%), or abnormal focal epileptiform discharges 2 (12%). A journal on Clinical Neurophysiology by Hrachovy et al reported the prevalence of hypsarrthymia can be up to 75% among the children with epileptic spams. Little findings obtained in this study is due to the fact that many children included in this study did their EEG after undergoing a long course anti epileptic medications before the diagnosis of epileptic spasm was made. (7)
In this study, 5 (71%) out of 7 patients whom MRI results were found had documented abnormal MRI findings with most of them showing features suggestive of HIE, old subdural haematoma, Megaloencephaly and bilateral hypocampal atrophy, severe brain atrophy and multicysticencephalomacia. A retrospective study done by Khatami et al in Canada on Brain MRI findings in patients with ES where a total of 26 patients were included, 19 (73%) had abnormal MRI findings which were features of HIE sequala, tuberous sclerosis (including one with megaloencephaly), Lisencephaly, meningitis infarcts and frontal heterotopia. The simirality in results depicts that the causes of epileptic spasms in the west are the same as those in Africa as they are all due to structural causes. (8)
Majority of the patients 35 (85%) in this study went through a course of different medications before the symptoms subsided. Before the diagnosis of epileptic spasms was made, many patients were receiving the following medications; sodium valproate 31(78%), and phenobarbitone 9 (23%). Other medications given were carbamazepine, clonazepam and baclofen. After the diagnosis of epileptic spasm was made 29 (73%) patients were documented to receiveprednisolone, with 12 (63%) out of 19 patients had documented spasms cessation after 10 days of high dose steroid treatment. A randomized clinical trial study done by Lux et showed 21 (70%) had spasms cessation after being given high dose prednisolone in 14 days. The results of the studies were almost similar due to almost the same duration of treatment as prednisolone works by suppressing the corticotropin releasing hormone which has been postulated to cause epileptic spasms. (9)