Periventricular Nodular Heterotopia: Pathogenesis, Epileptogenesis and Implications in Higher Cerebral Functions

Passarelli, Valmir; Moreira, Camila Hobi

doi:10.14800/mce.20

Cited by 2 publications

(3 citation statements)

References 28 publications

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“…This process is required for correct neuron placement ( Kriegstein and Noctor, 2004 ; Singh and Solecki, 2015 ), and this in turn is necessary for subsequent formation of functional neuronal circuitry. Neuronal delamination defects are collectively known as periventricular heterotopias and lead to a spectrum of deficits including epilepsy, dyslexia and intellectual disability ( Lian and Sheen, 2015 ; Passarelli and Moreira, 2014 ).…”

Section: Introductionmentioning

confidence: 99%

Inter-dependent apical microtubule and actin dynamics orchestrate centrosome retention and neuronal delamination

Kasioulis

Das

Storey

2017

eLife

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Detachment of newborn neurons from the neuroepithelium is required for correct neuronal architecture and functional circuitry. This process, also known as delamination, involves adherens-junction disassembly and acto-myosin-mediated abscission, during which the centrosome is retained while apical/ciliary membranes are shed. Cell-biological mechanisms mediating delamination are, however, poorly understood. Using live-tissue and super-resolution imaging, we uncover a centrosome-nucleated wheel-like microtubule configuration, aligned with the apical actin cable and adherens-junctions within chick and mouse neuroepithelial cells. These microtubules maintain adherens-junctions while actin maintains microtubules, adherens-junctions and apical end-foot dimensions. During neuronal delamination, acto-myosin constriction generates a tunnel-like actin-microtubule configuration through which the centrosome translocates. This movement requires inter-dependent actin and microtubule activity, and we identify drebrin as a potential coordinator of these cytoskeletal dynamics. Furthermore, centrosome compromise revealed that this organelle is required for delamination. These findings identify new cytoskeletal configurations and regulatory relationships that orchestrate neuronal delamination and may inform mechanisms underlying pathological epithelial cell detachment.

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Section: Introductionmentioning

confidence: 99%

Inter-dependent apical microtubule and actin dynamics orchestrate centrosome retention and neuronal delamination

Kasioulis

Das

Storey

2017

eLife

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“…[3] e impairment of neuronal migration occurs during the early period of fetal brain development, between the 6 th and 24 th weeks of gestation. [24] e classic presentation of the FLNA gene mutation includes bilateral periventricular heterotopic gray matter nodules, which are mainly concentrated around the anterior horns and bodies of the lateral ventricles. In addition, dysplasia of the corpus callosum or hippocampus, cerebellar hypoplasia, polymicrogyria, and microcephaly may also be observed.…”

Section: Discussionmentioning

confidence: 99%

“…[ 3 ] The impairment of neuronal migration occurs during the early period of fetal brain development, between the 6 th and 24 th weeks of gestation. [ 24 ]…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of epilepsy in a patient with bilateral periventricular nodular heterotopia: A case report

Kim,

Ivanov,

Halepa

et al. 2023

Surgical Neurology International

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Background: Periventricular nodular heterotopia (PNH) is a rare pathological condition characterized by the presence of nodules of gray matter located along the lateral ventricles of the brain. The condition typically presents with seizures and other neurological symptoms, and various methods of surgical treatment and postoperative outcomes have been described in the literature. Case Description: We present a case study of a 17-year-old patient who has been experiencing seizures since the age of 13. The patient reported episodes of loss of consciousness and periodic freezing with preservation of posture. Two years later, the patient experienced his first generalized tonic-clonic seizure during nocturnal sleep and was subsequently admitted to a neurological department. A magnetic resonance imaging scan of the brain with an epilepsy protocol (3 Tesla) confirmed the presence of an extended bilateral subependymal nodular heterotopy at the level of the temporal and occipital horns of the lateral ventricles, which was larger on the left side, and a focal subcortical heterotopy of the right cerebellar hemisphere. The patient underwent a posterior quadrant disconnection surgery, which aimed to isolate the extensive epileptogenic zone in the left temporal, parietal, and occipital lobes using standard techniques. As of today, 6 months have passed since the surgery and there have been no registered epileptic seizures during this period following the surgical treatment. Conclusion: Although PNHs can be extensive and located bilaterally, surgical intervention may still be an effective way to achieve seizure control in selected cases.

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Periventricular Nodular Heterotopia: Pathogenesis, Epileptogenesis and Implications in Higher Cerebral Functions

Cited by 2 publications

References 28 publications

Inter-dependent apical microtubule and actin dynamics orchestrate centrosome retention and neuronal delamination

Inter-dependent apical microtubule and actin dynamics orchestrate centrosome retention and neuronal delamination

Surgical treatment of epilepsy in a patient with bilateral periventricular nodular heterotopia: A case report

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