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Abstract
Objectives: Hippocampal abnormalities may coexist with malformations of cortical development (MCD). This cross-sectional MRI study aimed at categorizing hippocampal abnormalities in a large group of MCD and comparing MCD patients with (group W) and without (group W/O) hippocampal abnormalities.
Methods: Hippocampal anatomy, rotation, size, internal structure, and MRI signal alterations were assessed visually by 3 independent raters in patients with MCD and epilepsy. Four types of hippocampal abnormalities were examined in 220 patients (116 women, mean age 31 ± 16.6, range 2-76 years): partially infolded/hypoplastic hippocampus (HH), hippocampal sclerosis (HS), malrotated hippocampus (MH), and enlarged hippocampus (EH). The commonest MCD in the cohort were focal cortical dysplasia (27%), polymicrogyria (PMG) (21%), developmental tumors (15%), and periventricular nodular heterotopia (PNH) (14%).
Results: Hippocampal abnormalities were seen in 69/220 (31%) patients: HH in 34/69 (49%); HS in 18/69 (26%); MH in 15/69 (22%); and EH in 2/69 (3%). PNH (21/30 [70%]) and PMG (22/47 [47%]) were most commonly associated with hippocampal abnormalities. Compared to the W/O group, patients in the W group had a higher rate of learning disability (W 41/69 [59%] vs W/O 56/151 [37%]; p = 0.003) and delayed developmental milestones (W 36/69 [52%] vs W/O 53/151 [35%]; p = 0.025); groups did not differ otherwise with regard to clinical presentation. HH was associated with symptomatic generalized epilepsies (11/34 [32%]) and high rate of learning disability (27/34 [79%]), neurologic deficits (25/34 [73%]), and delayed developmental milestones (23/34 [68%]).
Conclusions: About a third of patients with malformations of cortical development had hippocampal abnormalities. Patients with hypoplastic hippocampus had the most severe clinical phenotype.
Glossary
- DT=
- developmental tumor;
- EH=
- enlarged hippocampus;
- FCD=
- focal cortical dysplasia;
- FLAIR=
- fluid-attenuated inversion recovery;
- FS=
- febrile seizure;
- HH=
- hypoplastic hippocampus;
- HMGE=
- hemimegalencephaly;
- HS=
- hippocampal sclerosis;
- LISS=
- lissencephaly-agyria-pachygyria;
- MCD=
- malformations of cortical development;
- MH=
- malrotated hippocampus;
- PMG=
- polymicrogyria;
- PNH=
- periventricular nodular heterotopia;
- SBH=
- subcortical band heterotopia;
- TLE=
- temporal lobe epilepsy;
- TS=
- tuberous sclerosis.
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