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Distinctive electro-clinical, neuroimaging and histopathological features of temporal encephaloceles associated to epilepsy

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Abstract

Objective

Encephaloceles (ENCs) may cause clinical complications, including drug-resistant epilepsy that can be cured with epilepsy surgery.

Methods

We describe clinical, diagnostic, and neuropathological findings of 12 patients with temporal ENC and epilepsy evaluated for surgery and compare them with a control group of 26 temporal lobe epilepsy (TLE) patients.

Results

Six patients had unilateral and 6 bilateral temporal ENCs. Compared to TLEs, ENCs showed i) later epilepsy onset, ii) higher prevalence of psychiatric comorbidities, iii) no history of febrile convulsions, and iv) ictal semiology differences. Seven patients had MRI signs of gliosis, and 9 of intracranial hypertension. Interictal EEG analysis in ENCs demonstrated significant differences with controls: prominent activity in the beta/gamma frequency bands in frontal regions, interictal short sequences of low-voltage fast activity, and less frequent and more localized interictal epileptiform discharges. Ictal EEG patterns analyzed in 9 ENCs showed delayed and slower contralateral spread compared to TLEs. All ENCs that underwent surgery (7 lobectomies and 1 lesionectomy) are in Engel class I. Neuropathological examination revealed 4 patterns: herniated brain fragments, focal layer I distortion, white matter septa extending into the cortex, and altered gyral profile.

Conclusions and significance

The described peculiarities might help clinicians to suspect the presence of largely underdiagnosed ENCs.

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Data availability

Raw data are available in national repository.

Abbreviations

BMI:

Body mass index

ENC:

Encephalocele

FCD:

Focal cortical dysplasia

GFAP:

Glial-fibrillary acid protein

HE:

Hematoxylin and eosin

IED:

Interictal epileptic discharge

IHC:

Immunohistochemistry

IIH:

Idiopathic intracranial hypertension

LFB:

Luxol fast blue

LVFA:

Low voltage fast activity

RP:

Relative power

SNR:

Signal-to-noise ratio

TLE:

Temporal lobe epilepsy

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Author notes

  1. Roberta Di Giacomo and Alessandra Burini contributed equally to this work.

Authors and Affiliations

  1. Epilepsy Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Roberta Di Giacomo, Alessandra Burini, Elisa Visani, Rita Garbelli, Dalia De Santis, Giuseppe Didato, Francesco Deleo, Chiara Pastori, Andrea Stabile & Marco de Curtis

  2. Department of Medicine (DAME), Neurology Unit, University of Udine, Udine, Italy

    Alessandra Burini

  3. Neuroradiology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Fabio Martino Doniselli & Valeria Cuccarini

  4. Neuropathology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Gianluca Marucci

  5. Clinical Neurophysiology Unit and Epilepsy Center, IRCCS Ospedale Policlinico San Martino, Genoa, Italy

    Flavio Villani

  6. Neurosurgery Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy

    Michele Rizzi

  7. Department of Enviromental System Science, Swiss Federal Institute (ETH) Zürich, Zurich, Switzerland

    Luca Girardi

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  1. Roberta Di Giacomo
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Contributions

All authors

1) made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data; or the creation of new software used in the work;

2) drafted the work or revised it critically for important intellectual content;

3) approved the version to be published; and.

4) agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Corresponding author

Correspondence to Roberta Di Giacomo.

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Di Giacomo, R., Burini, A., Visani, E. et al. Distinctive electro-clinical, neuroimaging and histopathological features of temporal encephaloceles associated to epilepsy. Neurol Sci 44, 4451–4463 (2023). https://doi.org/10.1007/s10072-023-06939-x

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