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Hemispheric cerebral agenesis — Clinical Guidelines

Overview

Hemispheric cerebral agenesis refers to the congenital absence or severe underdevelopment of one cerebral hemisphere, often associated with significant neurological and developmental challenges, including epilepsy and spatial neglect syndromes. 1 2 6 7

Diagnosis

Clinical Presentation: Intractable epilepsy, developmental delays, and signs of spatial neglect (e.g., neglect of one side of space).

Imaging Studies: MRI or CT scans essential for confirming absence or severe hypoplasia of the hemisphere.

Neuropsychological Testing: Assess spatial neglect, cognitive functions, and motor skills to evaluate extent of impairment. 1 6 7 11 17

Management

Surgical Interventions: Hemispherotomy may be considered for intractable epilepsy, though postoperative hydrocephalus risk factors include younger age and higher intraventricular hemorrhage volume should be carefully monitored. 1

Rehabilitation Therapy: Occupational therapy focusing on compensatory strategies for spatial neglect, including prism adaptation for visual neglect improvement. 2 3 4 12

Pharmacological Management: Antiepileptic drugs tailored to control seizures; specific dosing not detailed in abstracts. 1

Special Populations

Pediatrics: Hemispherotomy is a treatment option for intractable epilepsy in children, with careful consideration of postoperative complications like hydrocephalus. 1

Comorbidities: Depression is notably higher in patients with left hemispheric brain injury compared to traumatic brain injury, warranting mood assessment and potential antidepressant therapy. 20

Key Recommendations

Consider Hemispherotomy for Intractable Epilepsy in Pediatric Patients with thorough risk assessment for postoperative hydrocephalus, particularly in younger patients and those with higher intraventricular hemorrhage volumes. (Evidence: Moderate) 1

Implement Comprehensive Rehabilitation Programs including occupational therapy and prism adaptation to address spatial neglect symptoms effectively. (Evidence: Moderate) 2 3 4 12

Screen for and Manage Mood Disorders, particularly depression, in patients with left hemispheric agenesis, given higher prevalence compared to traumatic brain injury patients. (Evidence: Moderate) 20

References

1 Yoshitomi M, Iijima K, Kosugi K, Takayama Y, Kimura Y, Kaneko Y et al.. Intraventricular hemorrhage volume and younger age at surgery may be risk factors for postoperative hydrocephalus after hemispherotomy in children. Journal of neurosurgery. Pediatrics 2024. link 2 Nijboer TC, Nys GM, van der Smagt MJ, van der Stigchel S, Dijkerman HC. Repetitive long-term prism adaptation permanently improves the detection of contralesional visual stimuli in a patient with chronic neglect. Cortex; a journal devoted to the study of the nervous system and behavior 2011. link 3 Ptak R, Schnider A, Müri R. Bilateral impairment of concurrent saccade programming in hemispatial neglect. Neuropsychologia 2010. link 4 Riddoch MJ, Bodley Scott S, Humphreys GW. No direction home: Extinction is affected by implicit motion. Cortex; a journal devoted to the study of the nervous system and behavior 2010. link 5 Wisneski KJ, Anderson N, Schalk G, Smyth M, Moran D, Leuthardt EC. Unique cortical physiology associated with ipsilateral hand movements and neuroprosthetic implications. Stroke 2008. link 6 Funayama M, Muramatsu T, Kato M. Differential hand-neglect after a callosal lesion. Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology 2008. link 7 Committeri G, Pitzalis S, Galati G, Patria F, Pelle G, Sabatini U et al.. Neural bases of personal and extrapersonal neglect in humans. Brain : a journal of neurology 2007. link 8 Loetscher T, Brugger P. A disengagement deficit in representational space. Neuropsychologia 2007. link 9 Di Stefano M, Marano E, Viti M. Stimulus-dominance effects and lateral asymmetries for language in normal subjects and in patients with a single functional hemisphere. Brain and cognition 2004. link 10 Riestra AR, Womack KB, Crucian GP, Heilman KM. Is the middle between both halves?: midpoint location and segment size estimation in neglect. Neurology 2002. link 11 Adair JC, Na DL, Schwartz RL, Heilman KM. Analysis of primary and secondary influences on spatial neglect. Brain and cognition 1998. link 12 Grujic Z, Mapstone M, Gitelman DR, Johnson N, Weintraub S, Hays A et al.. Dopamine agonists reorient visual exploration away from the neglected hemispace. Neurology 1998. link 13 Aglioti S, Smania N, Barbieri C, Corbetta M. Influence of stimulus salience and attentional demands on visual search patterns in hemispatial neglect. Brain and cognition 1997. link 14 Shaw A, Jackson SR, Harvey M, Newport R, Krämer T, Dow L. Grip force scaling after hemispatial neglect. Neuroreport 1997. link 15 Anderson B. Pieces of the true crossover effect in neglect. Neurology 1997. link 16 Ro T, Rafal RD. Perception of geometric illusions in hemispatial neglect. Neuropsychologia 1996. link00018-8) 17 Tipper SP, Behrmann M. Object-centered not scene-based visual neglect. Journal of experimental psychology. Human perception and performance 1996. link 18 Harvey M, Milner AD, Roberts RC. Differential effects of line length on bisection judgements in hemispatial neglect. Cortex; a journal devoted to the study of the nervous system and behavior 1995. link80022-6) 19 Guariglia C, Padovani A, Pantano P, Pizzamiglio L. Unilateral neglect restricted to visual imagery. Nature 1993. link 20 Robinson RG, Szetela B. Mood change following left hemispheric brain injury. Annals of neurology 1981. link

Hemispheric cerebral agenesis