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Research ArticleClinical Image
Open Access

Unilateral Lennox–Gastaut syndrome associated with Klippel–Trénaunay syndrome

Brahim Tabarki, Khaled Hundallah and Nabil Biary
Neurosciences Journal April 2021, 26 (2) 218-219; DOI: https://doi.org/10.17712/nsj.2021.2.20200181
Brahim Tabarki
From the Division of Pediatric Neurology (Tabarki, Hundallah)Department of Pediatrics, and Department of Neurology (Biary), Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia.
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  • For correspondence: [email protected]
Khaled Hundallah
From the Division of Pediatric Neurology (Tabarki, Hundallah)Department of Pediatrics, and Department of Neurology (Biary), Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia.
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Nabil Biary
From the Division of Pediatric Neurology (Tabarki, Hundallah)Department of Pediatrics, and Department of Neurology (Biary), Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia.
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A 2-year-old boy with Klippel-Trénaunay syndrome, characterized by extensive port-wine staining and left lower limb hemihypertrophy, and developmental delay, with impairments in cognition, presented with a history of seizures since the age of 18 months. The seizures were of multiple types, including focal seizure with eye deviation to the right side, tonic drop attacks with eye deviation to the right side, and focal-onset myoclonic seizures. Epilepsy was refractory to 2 antiseizure medications. The interictal EEG showed features described in figures 1A and 1B, together with left paroxysmal fast activity during slow sleep in the left hemisphere (not shown) are characteristic features of asymmetric Lennox–Gastaut syndrome.

Figure 1
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Figure 1
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Figure 1

- The interictal EEG a) reveals an abnormal asymmetric slow spike-wave activity of 1.5 to 2 Hz and wave discharges associated with electrodecrement in the left hemisphere, consistent with findings of asymmetric Lennox–Gastaut syndrome. Settings: sensitivity, 7 μV/mm; high-frequency filter, 70 Hz. b) shows many spikes in multiple left-brain areas (T3, F7, T5, C3, and P3). Settings: sensitivity, 7 μV/mm; high-frequency filter, 70 Hz.

Magnetic resonance imaging (MRI) of the brain revealed left perisylvian cortical malformation and abnormal white matter (Figure 2).

Figure 2
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Figure 2

- MRI of the brain. Axial a) and coronal b) T2-weighted images show polymicrogyria with thickened disorganized cortex, mainly perisylvian (arrowheads), associated with mild enlargements of the left hemisphere, and lateral ventricle with an abnormal high signal of the periventricular and deep white matter (arrow), likely representing dysmyelination.

Klippel-Trénaunay syndrome is a rare genetic disorder characterized by complex vascular abnormalities (venous and lymphatic) and overgrowth of tissues and bones. It is PIK3CA-related growth spectrum disorder and involves a somatic gain-of-function mutation in the PIK3CA gene, resulting in dysregulated cell proliferation and angiogenesis.1 Central nervous system abnormalities in Klippel-Trénaunay syndrome include venous malformation, arteriovenous malformations, cavernoma, and aneurysm; hemimegalencephaly, cortical dysplasia, and polymicrogyria are rarely reported.2,3 Seizures is a rare manifestation of Klippel-Trénaunay syndrome; described seizures are mainly focal and associated with cerebral dysgenesis. The epilepsy and EEG abnormalities described here are explained by the hemispheric developmental lesions. EEG in Lennox-Gastaut syndrome classically shows characteristic generalized epileptic activity that is similar in those with lesional, genetic, or unknown causes. Unilateral Lennox-Gastaut syndrome is rarely reported.4

This patient with Klippel–Trénaunay syndrome showed the unique unilateral EEG features of Lennox–Gastaut syndrome and cortical malformation.

  • Received December 13, 2020.
  • Accepted January 18, 2021.
  • Copyright: © Neurosciences

Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work.

References

  1. 1.↵
    1. Hughes M,
    2. Hao M,
    3. Luu M.
    PIK3CA vascular overgrowth syndromes: an update. Curr Opin Pediatr 2020; 32: 539-546.
    OpenUrl
  2. 2.↵
    1. Covington TN,
    2. Anderson KR,
    3. Tollefson MM,
    4. Guerin JB,
    5. Brinjikji W.
    Intracranial and extracranial vascular manifestations of patients with a clinical diagnosis of Klippel-Trenaunay syndrome. Neuroradiology 2020; 63: 409-415.
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  3. 3.↵
    1. Imataka G,
    2. Ogino M,
    3. Watabe Y,
    4. Kuwashima S,
    5. Hashimoto T,
    6. Arisaka O.
    Lennox-Gastaut syndrome associated with unilateral hemispheric porencephaly. Dokkyo J Med Sciences 2010, 37: 125-128.
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  4. 4.↵
    1. Camfield PR.
    Definition and natural history of Lennox-Gastaut syndrome. Epilepsia 2011; 52: 3-9.
    OpenUrlPubMed
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Unilateral Lennox–Gastaut syndrome associated with Klippel–Trénaunay syndrome
Brahim Tabarki, Khaled Hundallah, Nabil Biary
Neurosciences Journal Apr 2021, 26 (2) 218-219; DOI: 10.17712/nsj.2021.2.20200181

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Unilateral Lennox–Gastaut syndrome associated with Klippel–Trénaunay syndrome
Brahim Tabarki, Khaled Hundallah, Nabil Biary
Neurosciences Journal Apr 2021, 26 (2) 218-219; DOI: 10.17712/nsj.2021.2.20200181
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© 2025 Neurosciences Journal Neurosciences is copyright under the Berne Convention and the International Copyright Convention. All rights reserved. Neurosciences is an Open Access journal and articles published are distributed under the terms of the Creative Commons Attribution-NonCommercial License (CC BY-NC). Readers may copy, distribute, and display the work for non-commercial purposes with the proper citation of the original work. Electronic ISSN 1658-3183. Print ISSN 1319-6138.

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