PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS
Pseudo-Lennox syndrome (PLS), or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized...
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| Format: | Article |
| Language: | Russian |
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ABV-press
2016-04-01
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| Series: | Русский журнал детской неврологии |
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| Online Access: | https://rjdn.abvpress.ru/jour/article/view/134 |
| _version_ | 1826558021322407936 |
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| author | K. Yu. Mukhin |
| author_facet | K. Yu. Mukhin |
| author_sort | K. Yu. Mukhin |
| collection | DOAJ |
| description | Pseudo-Lennox syndrome (PLS), or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG) by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy), atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years) are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis. |
| first_indexed | 2024-04-10T01:32:27Z |
| format | Article |
| id | doaj.art-47a0987a69944747a9d9a61bff995032 |
| institution | Directory Open Access Journal |
| issn | 2073-8803 2412-9178 |
| language | Russian |
| last_indexed | 2025-03-14T08:37:52Z |
| publishDate | 2016-04-01 |
| publisher | ABV-press |
| record_format | Article |
| series | Русский журнал детской неврологии |
| spelling | doaj.art-47a0987a69944747a9d9a61bff9950322025-03-02T12:58:00ZrusABV-pressРусский журнал детской неврологии2073-88032412-91782016-04-0111171310.17650/2073-8803-2016-11-1-7-13110PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICSK. Yu. Mukhin0Svt. Luka’s Institute of Child Neurology and EpilepsyPseudo-Lennox syndrome (PLS), or atypical benign partial epilepsy of childhood, is a disease from a group of age-related epileptic encephalopathies with a phenomenon of continuous spike-wave activity during slow sleep, which manifests itself as frequent polymorphic focal motor and pseudogeneralized seizures, cognitive impairments, as well as regional and diffuse epileptiform activity on electroencephalogram (EEG) by the morphology identical to that of benign epileptiform patterns of childhood. The disease was first described by J. Aicardi and J. J. Chevrie in 1982, based on a study of 7 cases. Its diagnostic complexity is the polymorphism of both epileptic seizures and EEG data, as well as low awareness of the syndrome among physicians and its absence in the international classification of epilepsies. The typical triad of seizures, which occurs in nearly 100 % of patients, encompasses focal motor paroxysms (identical to those as observed in Rolandic epilepsy), atypical absences, and atonic seizures. Seizures in PLS in its active period (generally up to 7–8 years) are highly resistant to antiepileptic drugs. Only a few agents have been proven to be effective in PLS; these include valproates, succinimides, benzodiazepines, topiramate, and sulthiame. The frequency of seizures are noted to increase in patients with PLS treated with drugs, such as vigabatrin, gabapentin, lamotrigine, phenobarbital, or phenytoin. The author considers in detail the history of studies of the disease, clinical manifestations, diagnostic criteria, therapeutic approaches, and prognosis.https://rjdn.abvpress.ru/jour/article/view/134epilepsyepileptic encephalopathypseudo-lennox syndromeelectroencephalographybenign epileptiform discharges of childhoodclinical presentationdiagnosistreatmentprognosis |
| spellingShingle | K. Yu. Mukhin PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS Русский журнал детской неврологии epilepsy epileptic encephalopathy pseudo-lennox syndrome electroencephalography benign epileptiform discharges of childhood clinical presentation diagnosis treatment prognosis |
| title | PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS |
| title_full | PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS |
| title_fullStr | PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS |
| title_full_unstemmed | PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS |
| title_short | PSEUDO-LENNOX SYNDROME: CLINICAL AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS |
| title_sort | pseudo lennox syndrome clinical and electroencephalographic characteristics |
| topic | epilepsy epileptic encephalopathy pseudo-lennox syndrome electroencephalography benign epileptiform discharges of childhood clinical presentation diagnosis treatment prognosis |
| url | https://rjdn.abvpress.ru/jour/article/view/134 |
| work_keys_str_mv | AT kyumukhin pseudolennoxsyndromeclinicalandelectroencephalographiccharacteristics |