Lennox Gastaut Syndrome, Review of the Literature and a Case Report Tareq Abu Saleh*1,2 and Lawrence Stephen1
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Head & Face Medicine BioMed Central Case report Open Access Lennox gastaut syndrome, review of the literature and a case report Tareq Abu Saleh*1,2 and Lawrence Stephen1 Address: 1Department of Oral Medicine and Periodontics, University of the Western Cape, Cape Town, South Africa and 2Future Dental and Implant Center, Jordan Hospital, Amman, Jordan Email: Tareq Abu Saleh* - [email protected]; Lawrence Stephen - [email protected] * Corresponding author Published: 9 June 2008 Received: 8 June 2007 Accepted: 9 June 2008 Head & Face Medicine 2008, 4:9 doi:10.1186/1746-160X-4-9 This article is available from: http://www.head-face-med.com/content/4/1/9 © 2008 Saleh and Stephen; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract Background: Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. In South Africa, the incidence of secondary epilepsy is higher than what is found in developed countries resulting in higher incidence of the disease. LGS seizures are often treatment resistant and the long term prognosis is poor. Report: A twenty six year old female, presented with anterior open bite, macroglossia, supragingival as well as subgingival calculus. The gingiva was red, swollen and friable and there was generalized bleeding and localized suppuration. The patient had gingival recession. After periodontal therapy a remarkable improvement in oral health status was noted. Conclusion: The clinical findings in LGS included facial deformities, periodontitis and gingival swellings. Interdisciplinary treatment of these patients is fundamental and oral attention is of outstanding importance. Non-surgical periodontal therapy was effective in controlling periodontal disease in the reported case, but prevention of periodontal and dental diseases is preferable for this high-risk group of patients. Background and literature review - A specific electroencephalogram (EEG) pattern called a Lennox-Gastaut syndrome (LGS) is one of the cata- slow spike-and-wave pattern (< 2.5 Hz), which is present strophic childhood epilepsies. It is defined by a triad of when the child is awake [1-4]. symptoms: Seizures most often present between the second and sixth - Multiple types of generalized seizures, which are difficult year of life, however; they can start a little earlier or later. to control. They rarely start after the age of eight [2,3]. - Slowness of intellectual growth, often accompanied by William Lennox described the clinical features of the syn- mental retardation and behavioral problems. drome in 1930s, and then Lennox and Davis reported the symptomatic triad of the syndrome. Later, Gastaut Page 1 of 7 (page number not for citation purposes) Head & Face Medicine 2008, 4:9 http://www.head-face-med.com/content/4/1/9 expanded on the original observations of Lennox and For example, in Atlanta, USA, LGS accounts for 4% of Davis [5,6]. patients with childhood epilepsy, with a reported inci- dence of 0.26 per 1000 live births [6]. The true incidence The diagnostic criteria of the syndrome have been refined in different populations is not known, due to the disparity by researchers, but the basic characteristics of the syn- between parameters used to define the disease, as well as drome remained unchanged since Lennox and Davis [7- to the variability of the predisposing factors to the disease 11]. among different populations. Distinguishing LGS syndrome from other epilepsy syn- Etiology dromes has been challenging as it is characterized by Etiology of LGS is variable; LGS does not usually run in plethora of underlying causes, multiple types of seizures, families but genetic factors may play a role in the etiology and cognitive impairment. Seizures are classified accord- [2,7]. In 20–50% of cases the child has previously had ing to the International League Against Epilepsy (ILAE) infantile spasms with underlying brain disorder (also classification, and specific epilepsy syndromes of child- known as symptomatic West syndrome) [3,5,7,18,19]. hood are recorded when their essential diagnostic ele- ments are fulfilled. It is now agreed that a number of For about one third of the affected children a known cause individual syndromes, including LGS, form a spectrum of cannot be identified. These cases are referred to as cryp- childhood epilepsies, each with differentiating criteria togenic Lennox-Gastaut syndrome [2,3]. [12-14]. On the other side, many of the children who develop Len- Prevalence nox-Gastaut syndrome had a pre-existing brain disorder The Lennox-Gastaut syndrome is uncommon but it is very or injury. Causes identified include tuberous sclerosis, serious. The mortality rate ranges from 3% to 7% [2,5]. congenital infections, hereditary metabolic diseases, brain malformation, and brain damage (due to birth asphyxia Often the prevalence of LGS is described as a percentage or other birth injuries, encephalitis, meningitis or head of childhood epilepsy. The reported prevalence of LGS injuries) [2,3]. varies between studies but it is in the range of 3–10% of childhood epilepsy, and is more common in males than These patients tend to have worse prognosis than those of females [1-3]. cryptogenic etiology [2,3]. It was noted, however, that these potential causative factors are different in different Epilepsy is relatively common, but its prevalence varies populations, and that in developing countries LGS was widely affecting approximately 3.4/1000 of the popula- mainly secondary to trauma and infections [17]. tion in Japan, 6/1000 in the United States, and 11.2/1000 in Mexico [15,16]. In developing countries, there is a high incidence of pre- term and abnormal births. During infancy and early child- In South Africa, the prevalence of epilepsy (all ages) has hood, meningitis, tuberculosis, neurocysticercosis, and been reported as 10 per 1,000 [17]. In rural South African head trauma are common. This situation is expected to children aged 2–9 years the prevalence of epilepsy was increase the prevalence of secondary (symptomatic) epi- 0.73%. Out of the affected, 57.1% didn't receive medica- lepsy and that of intellectual handicap in the child popu- tion, and 71.4% had developmental disability. These fig- lation [17]. ures are slightly higher than those derived from other sub- Saharan African countries [15]. In a study of childhood recurrent seizures in Red Cross memorial hospital in Cape Town, South Africa, 11% suf- As LGS constitutes a fraction of childhood epilepsy it is fered from menigities or encephalitis as a precipitating overall rare. However, it is clinically prominent due to the factor and 55% were intellectually handicapped [20]. frequent and difficult to treat seizures that persist into Most patients who had both grand mal and myoclonic adulthood, as well as to the need for continuous medical seizures suffered from LGS [20]. attention [1]. In another study in Cape Town, It was found that 43% of Epidemiologic community based studies in the so-called childhood epilepsy was symptomatic (secondary) [17]. developed countries revealed that the annual incidence of This figure is even higher than other figures found in stud- LGS in childhood was approximately 2 per 100,000 chil- ies conducted predominantly in tropical African countries dren, and the prevalence of LGS was 0.1–0.28 per 1000 in [17]. Europe [4]. Studies demonstrated that the figures of LGS are relatively consistent across the developed populations. Page 2 of 7 (page number not for citation purposes) Head & Face Medicine 2008, 4:9 http://www.head-face-med.com/content/4/1/9 Perinatal hypoxia, meningitis, granulomata (cysticercosis the child and to direct families to appropriate community and tuberculosis), and trauma have high prevalence resources. These children and their families need the com- among the poor of the Western Cape, and it is expected bined support of services from healthcare workers, car- that these conditions serve as precipitating factors of sec- egivers, friends, school and social workers as well as ondary epilepsy [17]. On the other hand diagnostic facil- psychologists. ities are better in Cape Town than in the rest of the studied African cities, which is expected to reduce the number of Treatment overlooked cases, resulting in higher figures. LGS seizures are often treatment resistant. Basic treatment is pharmacological and some children may need more Clinical features than one mode of treatment. Typically, daily multiple seizure types that occur in LGS are of wide range, more than that of any other epileptic Pharmacological treatment consists mainly of one or mul- syndrome. The major kinds of seizures that usually occur tiple antiepileptic drugs (AED's). No single treatment reg- in LGS are the tonic seizures, which are often nocturnal imen could be considered superior to the others, and [3], the atonic seizures (involuntary losses of muscle tone management depends on the response of the patients. that cause drop attacks) or atypical absences (child goes Medical treatment usually starts with valproates (valproic blank lasting up to a minute) [3]. Additionally, about