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Hippocampal malrotation with normal corpus callosum: a new entity?
1Department of Radiology, National Institute of Psychiatry and Neurology, Hungary. H9129bar@ella.hu
Abstract:
Among 527 MRI examinations of patients with a suspicion of epilepsy in 5 years, we found 32 cases of hippocampal malrotation (HIMAL). The characteristic features are: incomplete inversion of the hippocampus with and abnormally round shape; unilateral involvement of the whole hippocampus; normal signal intensity and size; blurred internal structure; an abnormal angle of collateral sulcus; abnormal position and size of the fornix; normal size of the temporal lobe; enlargement and particular configuration of the temporal horn, typical of corpus callosum agenesis; and a normal corpus callosum. In 7 cases (22%) HIMAL occurred together with developmental disorders. It was predominantly seen in men. The clinical features were varied. Based on some MRI features, the presence of developmental disorders, the male predominance, the frequently positive family history, and a review of the literature, we think HIMAL may be the consequence of a mild hemisphere developmental disorder. It is probably not the basic cause of epilepsy in such varied clinical setting, but may be a sign of a developmental disorder and can help in selecting patients for more meticulous investigation. It also may give some new understanding of brain development.
Insights
Hippocampal malrotation (HIMAL) is a rare finding in epilepsy patients, often associated with developmental disorders. This condition may indicate broader brain development issues rather than being the primary cause of seizures.
Area of Science:
- Neuroimaging
- Developmental Neuroscience
- Epilepsy Research
Background:
- Epilepsy is a neurological disorder characterized by recurrent seizures.
- Magnetic Resonance Imaging (MRI) is crucial for identifying structural brain abnormalities.
- Hippocampal malrotation (HIMAL) is a rare congenital brain malformation.
Purpose of the Study:
- To identify and characterize hippocampal malrotation (HIMAL) in patients with suspected epilepsy.
- To explore the association between HIMAL and other developmental disorders.
- To understand the potential role of HIMAL in the context of epilepsy and brain development.
Main Methods:
- Retrospective analysis of 527 MRI examinations in patients with suspected epilepsy over 5 years.
- Identification of 32 cases exhibiting characteristic features of HIMAL.
- Review of clinical features, associated developmental disorders, and family history.
Main Results:
- Hippocampal malrotation (HIMAL) was identified in 32 (6%) of 527 epilepsy patients.
- Characteristic MRI findings include incomplete inversion, round shape, abnormal collateral sulcus, and specific temporal horn enlargement.
- HIMAL was associated with developmental disorders in 22% of cases, predominantly in males, and often had a positive family history.
Conclusions:
- Hippocampal malrotation (HIMAL) may represent a mild hemisphere developmental disorder.
- HIMAL is likely a marker of underlying developmental abnormalities rather than the primary cause of epilepsy.
- Identifying HIMAL can aid in selecting patients for further investigation and contribute to understanding brain development.