Pediatric Neuroscience Pathways Fall 2012 - Cleveland Clinic

indications for hemispherectomy:
common disorders with hemispheric epilepsy
common disorders for which hemispherectomy is indicated are
congenital malformations such as hemimegalencephaly, hemispheric
or extensive multilobar cortical dysplasias, perinatal or
acquired ischemic strokes, Sturge-Weber syndrome and Rasmussen
syndrome. congenital malformations may occur as isolated
disorders or in association with certain sporadic or inheritable
genetic conditions. Figures 1 to 3 show brain MRI findings in
children who are candidates for hemispherectomy.
A thorough clinical evaluation — assessing age of seizure onset,
course of the neurological symptoms, family history, presence of
neurocutaneous markers, dysmorphic features or congenital
anomalies involving multiple organ systems, and abnormal
Pediatric ePilePsy
Hemispheric Epilepsy and Hemispherectomy in Children
Ajay Gupta, MD, and William E. Bingaman, MD
Figure 1
certain epilepsies are caused by congenital or acquired disorders that extensively involve all or most of one
cerebral hemisphere. these disorders usually present with catastrophic epilepsy during infancy and require urgent
diagnostic evaluation and treatment. After careful consideration of several complicating factors, hemispherectomy
may be the most effective treatment for these children.
Figure 2
neurological findings — helps in making a specific diagnosis,
determining disease progression and planning treatment. video
eeg monitoring is the initial key tool in establishing the diagnosis
of epilepsy, localizing the seizure-onset zone, and planning surgical
strategy. Brain MRI, supplemented by FDG-PET, helps in determining
the underlying epilepsy substrate and extent of hemispheric
involvement as well as the anatomical and functional integrity of
the unaffected hemisphere. other tests required may include an
eye examination, neuropsychological assessment and metabolic/
genetic consultation.
timing and Planning of hemispherectomy
hemispherectomy in children with catastrophic epilepsy is
challenging and requires careful analysis of several complex
age-related issues.
Spectrum of brain MRI findings seen in children with hemispheric epilepsy syndromes. All these patients underwent
hemispherectomy for intractable seizures.
Figure 1: T2-weighted sagittal image of hemimegalencephaly showing diffuse right hemispheric enlargement and dysplasia. Midline shift with
bulging of anterior falx to the left and compression of the right lateral ventricle suggest a mass effect due to increased volume of the brain
parenchyma. Dysplastic changes are diffuse with a thick and disorganized cortex, poor gray-white matter differentiation, and abnormal signal in
the white matter. Note the basal ganglia are also dysplastic with abnormal increased signal.
Figure 2: Axial FLAIR brain MRI image of a child with Rasmussen syndrome with left hemispheric atrophy most prominent in the insular region,
mildly dilated atrium of the left ventricle, and increased signal in the peri-insular subcortical and cortical ribbon.
Figure 3: Axial FLAIR brain MRI image of a 5-year-old with a remote ischemic stroke in the distribution of the right middle cerebral artery with
severe right hemispheric encephalomalacia and atrophy.
12 Pediatric NeuroscieNce Pathways | 2012 – 2013
Figure 3