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Epilepsy combined with Wolf-Hirschhorn syndrome: a literature review and description of clinical cases

https://doi.org/10.17749/2077-8333.2018.10.4.039-052

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Abstract

This article presents the anamnestic, clinical, electro-encephalographic and neuroimaging findings in 5 patients with epilepsy combined with Wolf-Hirschhorn syndrome (WHS). According to our data and the results of others, this combination has its specific characteristics. These include: a high incidence of epilepsy in patients with WHS (50-100% of cases), an early debut of seizures (mainly in the first year of life), fever-provoked seizures, and a variety of seizure types – focal paroxysms, bilateral tonic-clonic seizures, atypical febrile seizures, atypical absences and epileptic spasms. In addition, there may be frequent epileptic seizures tending toward status epilepticus, a slowing of the major EEG activity, a local EEG slowing (mainly in the posterior and bi-frontal areas), and regional / multiregional epileptiform activity. In more than 50% of cases, the diffuse peakwave activity is observed; the broad spectrum anti-epileptic drugs are highly efficient in 80% of cases. Based on this study, we propose recommendations for the management of patients with epilepsy combined with WHS.

About the Authors

M. B. Mironov
Medical Center for Pediatric Neurology and Pediatrics.
Russian Federation

Mikhail B. Mironov – MD, PhD, Head of the Laboratory of Video-EEG Monitoring.

13/32 prospekt Andropova, Moscow 101100.



N. V. Chebanenko
St. Luka’s Clinical Research Center for Children.
Russian Federation

Natalya V. Chebanenko – MD, PhD, Head, Department of General Psychoneurology.

38 Aviatorov Str., Moscow 119620.



S. O. Ayvazyan
St. Luka’s Clinical Research Center for Children; St. Luka’s Clinical Research Center for Children.
Russian Federation

Sergey O. Ayvazyan – MD, PhD.

38 Aviatorov Str., Moscow 119620.

38 Aviatorov Str., Moscow 119620.



S. A. Vladimirova
Center of modern medicine.
Russian Federation

Svetlana A. Vladimirova – MD, Neurologist.

2/2 Lenina, Lobnja 141730, Moscow regio.



K. V. Osipova
St. Luka’s Clinical Research Center for Children.
Russian Federation

Karina V. Osipova – MD, PhD, Head, Department of Psychoneurology.

38 Aviatorov Str., Moscow 119620.



S. G. Burd
Pirogov Russian National Research Medical University, Ministry of Health of Russia.
Russian Federation

Sergey G. Burd –  MD,  PhD,  Professor  at  the  Chair  of  Neurology,  Neurosurgery  and  Medical  Genetics,  Faculty  of  Pediatrics.

1 Ostrovityanova Str., Moscow 117997.



Yu. V. Rubleva
Pirogov Russian National Research Medical University, Ministry of Health of Russia; Federal Center for Cerebrovascular Pathology and Stroke .
Russian Federation

Yulia V. Rubleva – MD, PhD, Head, First Department of Neurology; Chair  of  Neurology,  Neurosurgery  and  Medical  Genetics,  Faculty  of  Pediatrics.

1 Ostrovityanova Str., Moscow 117997.

1-10 Ostrovityanova Str., Moscow 117997.



T. M. Krasilshchikova
Pirogov Russian National Research Medical University, Ministry of Health of Russia.
Russian Federation

Tatyana M. Krasilshchikova –  Assistant,  Chair  of  Neurology,  Neurosurgery  and  Medical  Genetics,  Faculty  of  Pediatrics.

1 Ostrovityanova Str., Moscow 117997.



V. G. Bychenko
Research Center for Obstetrics, Gynecology and Perinatology named after V. I. Kulakov of the Ministry of Health of Russian Federation.
Russian Federation

Vladimir G. Bychenko – MD, PhD, Head, Department of Diagnostic Radiology.

4 akademika Oparina Str., Moscow 117198.



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For citation:


Mironov M.B., Chebanenko N.V., Ayvazyan S.O., Vladimirova S.A., Osipova K.V., Burd S.G., Rubleva Y.V., Krasilshchikova T.M., Bychenko V.G. Epilepsy combined with Wolf-Hirschhorn syndrome: a literature review and description of clinical cases. Epilepsy and paroxysmal conditions. 2018;10(4):39-52. (In Russ.) https://doi.org/10.17749/2077-8333.2018.10.4.039-052

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ISSN 2077-8333 (Print)
ISSN 2311-4088 (Online)