Brain MRI Findings in Infantile Spasm: Outcome Correlations in a Patient Cohort

Khatami, Alireza; Sell, Erick; Aggag, Mohamed Farouk; Miller, Elka

doi:10.4236/ojmi.2016.63008

Cited by 4 publications

(4 citation statements)

References 22 publications

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“…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%).…”

Section: Discussionmentioning

confidence: 85%

Epileptic Spasms at Muhimbili National Hospital Tanzania, A Retrospective Study.

Mwalongo¹,

Kija²

2020

Preprint

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Background; Epileptic spasms (ES) is an epileptic encephalopathy occurring during infancy and early childhood. Early recognition and management is 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 October 2018. Results; A total of 40 patients diagnosed with epileptic spasms were retrieved with male to female ratio of 3:2. In this study, 17 (42.5%) patients had a documented history of perinatal insult. The median age of onset of spasms was 5 months (IQR 1–12 months). In 14 (80%) out of 17 patients whom electroencephalography ( 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%). Twenty-nine (73%) received prednisolone, with a median time of spasms subsiding of 1 month with a range of 10 days to 3 months in about 90% of them. Conclusion; The median age of onset of epileptic spasms at MNH is 5 months with the most common cause being perinatal insult in more than 40% of the patients. High dose prednisolone showed a good response in patients with epileptic spasms at MNH.

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Section: Discussionmentioning

confidence: 85%

Epileptic Spasms at Muhimbili National Hospital Tanzania, A Retrospective Study.

Mwalongo¹,

Kija²

2020

Preprint

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“…In a retrospective review by Khatami et al, MRI of the brain helped to establish the etiology in 73%. 72 He has demonstrated that one-third of cases considered idiopathic on clinical assessment are classified as structural-metabolic following MRI and that the MRI finding cannot predict the treatment response. 72 There could be clues in the MRI pointing to an inborn error of metabolism.…”

Section: Brain Imagingmentioning

confidence: 99%

“…72 He has demonstrated that one-third of cases considered idiopathic on clinical assessment are classified as structural-metabolic following MRI and that the MRI finding cannot predict the treatment response. 72 There could be clues in the MRI pointing to an inborn error of metabolism. 73 As the immature white matter could hide subtle dysplasias serial MRI would be needed in intractable cases that show focal EEG changes, to consider whether surgical resection would lead to seizure freedom.…”

Section: Brain Imagingmentioning

confidence: 99%

Management of Infantile Spasms: An Updated Review

Iype

Koshy

2020

International Journal of Epilepsy

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Infantile spasms remain the most challenging of the epileptic encephalopathies of childhood. Infantile spasms are classified as an epileptic encephalopathy, as the adverse cognitive and behavioral burden of the condition is out of proportion to the burden one would expect from the underlying etiology or the accompanying magnetic resonance imaging. The ictal and interictal electroencephalographic (EEG) activity is presumed to contribute to the progressive cerebral dysfunction. In many of these children, the underlying etiology also contributes to the severe mental subnormality and autistic behavior. Though it is the syndromic approach that guides the pediatric epileptologist, it is best to keep in mind that one syndrome may evolve into another in infancy and early childhood. A baby with Ohtahara syndrome may, after 2 to 7 months, begin to have spasms. Lennox-Gastaut syndrome with its typical seizure types and EEG may evolve in a child with infantile spasms.The unique modalities used in the treatment of infantile spasms make early recognition important. It is, however, also of paramount importance to make an etiological diagnosis as the underlying etiology may be eminently treatable. The treating physician cannot abandon them as wholly “intractable” epilepsy. The excellent response to treatment in the few who just cannot be defined or accurately predicted drives the physician to exercise his brain. Use of the two well-accepted modalities of treatment; vigabatrin and adrenocorticotrophic hormone singly or in combination, oral steroids in high dose, ketogenic diet, the conventional antiepileptic medications, and strategies to target the basic cause have been tried out by various clinicians. Here, we have made an attempt to collate evidence and describe the progress in the management of infantile spasms.

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“…MRI abnormalities are reported in approximately 70-80% of patients with West syndrome 31,32) . The duration of spasms tends to be shorter in patients with normal MRI findings than in those with abnormal MRI findings.…”

Section: Diagnosis and Treatment Of West Syndromementioning

confidence: 99%

Age-Based Characteristics of West Syndrome in Patients with Mitochondrial Disease

Choi¹,

Baek²,

Na³

et al. 2018

Child Neurol

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West syndrome is a severe form of age-specific epilepsy that typically affects infants younger than 2 years of age with mitochondrial disease. We aimed to examine age-specific characteristics of the syndrome in these patients. Methods: We retrospectively analyzed 54 patients with West syndrome diagnosed with mitochondrial disease between March 2006 and March 2016. We compared treatment strategies and diagnostic and clinical variables between patients with early-onset (<6 months of age) and late-onset (≥6 months of age) seizures. Results: Seizure was the first symptom in 30 (90.9%) and 13 (65%) patients of the early-onset and late-onset groups, respectively (P=0.046). Delayed development was observed in 3 (9.1%) and 7 (35%) patients of the early-onset and late-onset groups, respectively (P=0.023). Lactate levels were normal in 17 patients (55%) of the early-onset group and 5 (25%) of the late-onset group (P=0.036), while initial brain magnetic resonance imaging (MRI) findings were normal in 23 (67.6%) and 8 (40%) patients of the early-onset and late-onset groups, respectively. Final MRI findings were abnormal in 32 patients (94.1%) of the early-onset group and 18 (90%) of the late-onset group (P=0.036). Although ketogenic diets reduced seizure frequency in both groups, the difference was not significant. Conclusion: There is no significant difference in epilepsy-related variables when patients are divided based on a cutoff age of 6 months. However, differences in the first symptom at onset and MRI findings were observed. Although lactate levels were not of significant diagnostic value in the early-onset group, they may be in the lateonset group.

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Brain MRI Findings in Infantile Spasm: Outcome Correlations in a Patient Cohort

Cited by 4 publications

References 22 publications

Epileptic Spasms at Muhimbili National Hospital Tanzania, A Retrospective Study.

Epileptic Spasms at Muhimbili National Hospital Tanzania, A Retrospective Study.

Management of Infantile Spasms: An Updated Review

Age-Based Characteristics of West Syndrome in Patients with Mitochondrial Disease

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