Elsevier

World Neurosurgery

Volume 117, September 2018, Pages 439-442
World Neurosurgery

Case Report
Simultaneous Treatment of Epilepsy and Secondary Dystonia After Anterior Temporal Lobectomy and Amygdalohippocampectomy

https://doi.org/10.1016/j.wneu.2018.04.003Get rights and content

Highlights

  • The relationship between temporal lobe epilepsy (TLE) and dystonia is known, but its etiology is not well understood.

  • Here, we describe 2 patients with TLE and dystonia who underwent anterior temporal lobectomy for seizure control.

  • Anterior temporal lobectomy and selective amygdala-hippocampectomy was curative of both patients’ epilepsy and dystonia.

  • Our cases demonstrate a medial temporal-basal ganglia network dysfunction in dystonia that resection possibly modulated.

Background

The relationship between temporal lobe epilepsy and focal limb dystonia is a well-recognized phenomenon, yet its pathogenesis and anatomic foundation are not well understood. Here, we describe 2 patients with refractory focal epilepsy and contralateral focal limb dystonia whose seizures and dystonic symptoms simultaneously resolved after anterior temporal lobectomy and amygdalohippocampectomy.

Case Description

We identified 2 patients within the Mount Sinai Health system with improvement in dystonia after medial temporal lobectomy. Retrospective chart reviews for the clinical history were performed. Patient 1 suffered a traumatic injury of the right temporal lobe, developing left hemidystonia and epilepsy. He received a right amygdala-hippocampectomy, which resolved both. Patient 2 has a history of right temporal glioma resection complicated by an infarct, resulting in left hemidystonia and epilepsy. He received a right medial temporal resection, which nearly resolved both.

Conclusion

Our cases demonstrate a medial temporal-basal ganglia network dysfunction in dystonia-epilepsy that was modulated and cured by resective surgery. We hypothesize that the mechanisms behind these observed phenomena were due to a pathologic connectivity of the basal ganglia and amygdala-hippocampus. To our knowledge, these are the first reported cases of dystonia and concomitant epilepsy resolving with temporal lobectomy and provide valuable prognostic information for similarly affected patients.

Introduction

The relationship between temporal lobe epilepsy and focal limb dystonia is a well-recognized phenomenon, yet its pathogenesis and anatomic foundation are not well understood.1, 2 We describe 2 patients with refractory focal epilepsy and contralateral focal limb dystonia whose seizures and dystonic symptoms simultaneously resolved after anterior temporal lobectomy and amygdalohippocampectomy (ATLAH). Our experience illustrates how alteration of temporal lobe circuitry can ameliorate multiple pathologies related to these embryologically linked regions of the basal ganglia and anterior temporal lobe.

Section snippets

Patient 1

A 29-year-old right-handed male with a 10-year history of focal epilepsy and posttraumatic left hemidystonia was referred to our comprehensive epilepsy center (Supplementary Video 1). In 2003 he suffered a traumatic right temporal contusion injury from a motor vehicle accident. Several months later, he developed dystonic posturing of the left arm, hand, and foot. When walking, the patient's left foot would invert while his toes would flex downwards. In his left upper extremity, full strength

Discussion

Our cases demonstrate simultaneous resolution of aberrant epileptogenic and dystonic circuits and build on the association between the embryogenesis and function of the mesial temporal lobe and basal ganglia. The basal ganglia includes structures that originate from the telencephalon, diencephalon, and midbrain. The amygdala is a basal ganglia structure, which originates from the telencephalon and is located in the mesial temporal lobe. The amygdala and hippocampus are adjacent to one another,

Conclusion

We have presented 2 patients with secondary dystonia and epilepsy, who experienced simultaneous relief of their seizures and dystonic movements after epilepsy surgery. We hypothesize that the mechanisms behind these observed phenomena are due to the shared functional connectivity of the basal ganglia and amygdala-hippocampus. Our patients both experienced basal ganglia injury before and in the setting of epilepsy, which led to disruption of normal neuronal circuitry connecting the mesial

References (11)

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Cited by (1)

  • Perturbed Ca<sup>2+</sup>-dependent signaling of DYT2 hippocalcin mutant as mechanism of autosomal recessive dystonia

    2019, Neurobiology of Disease
    Citation Excerpt :

    Thus, we have hypothesized that the DYT2 mutations perturb HPCA signaling and sAHP gating leading to increased neuronal excitability. We tested this hypothesis using hippocampal neurons, which potentially belong to disease-causing network (Kuba et al., 2010; Rasouli et al., 2018) and are the most studied cellular model of HPCA-dependent gating of sAHP (Andrade et al., 2012; Tzingounis et al., 2007). In this work, we initially tested effects of the dystonia-causing mutations on the biophysical properties of HPCA and found that N75K mutant (rather than T71N) had lower Ca2+ buffer capacity and decreased Ca2+-sensitivity compared to wild type (WT) HPCA.

Supplementary digital content available online.

Conflict of interest statement: The authors declare that there are no conflicts of interest and no specific funding was received for this work.

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