The Prevalence of Epilepsy Among Mongols Related to Age

Rm, Veall

doi:10.1111/j.1365-2788.1974.tb01224.x

Cited by 34 publications

(22 citation statements)

References 12 publications

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“…Early reports of the rate of seizures were significantly lower than more recent prevalence estimates. One early estimate of the rate of seizures was 12.2% for adults with DS over the age of 55 and 15.8% for adults over the age of 60 (Veall, 1974). However, early estimates were consistent with more recent estimates that abnormal readings reflective of seizure activity are high, with 71.4% of adults over 55 showing this abnormal activity (Tangye, 1979).…”

Section: Seizure Disordermentioning

confidence: 99%

Health Conditions Associated with Aging and End of Life of Adults with Down Syndrome

Esbensen

2010

International Review of Research in Mental Retardation

148

113

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Expectations for the life course of individuals with Down syndrome (DS) have changed, with life expectancy estimates increasing from 12 in 1949 to nearly 60 years of age today (Bittles & Glasson, 2004;Penrose, 1949). Along with this longer life expectancy comes a larger population of adults with DS who display premature age-related changes in their health. There is thus a need to provide specialized health care to this aging population of adults with DS who are at high risk for some conditions and at lower risk for others. This review focuses on the rates and contributing factors to medical conditions that are common in adults with DS or that show changes with age. The review of medical conditions includes the increased risk for skin and hair changes, early onset menopause, visual and hearing impairments, adult onset seizure disorder, thyroid dysfunction, diabetes, obesity, sleep apnea and musculoskeletal problems. The different pattern of conditions associated with the mortality of adults with DS is also reviewed. KeywordsDown syndrome; physical health; aging; mortality A highly significant change in the survival of people with Down syndrome (DS) has occurred during the last two generations. In the 1940s, the average life expectancy for individuals with DS was 12 years (Penrose, 1949). With medical breakthroughs and improvements in services, individuals with DS now enjoy life expectancies into their 60s (Bittles & Glasson, 2004). As a result, we are now witness to the first generation of individuals with DS who have benefited from a revolution during their lifetime of better knowledge, health care, advocacy and services (Yang, Rasmussen & Friedman, 2002). Along with this longer life expectancy comes a larger population of adults with DS who display premature age-related changes in their health. There is thus a need to provide specialized health care to this aging population of adults with DS who are at high risk for some conditions and at lower risk for others. Adults with DS are at age-related increased risk for dementia, skin and hair changes, early onset menopause, visual and hearing impairments, adult onset seizure disorder, thyroid dysfunction, diabetes, obesity, sleep apnea and musculoskeletal problems. Along with these increased risks for some conditions comes a different pattern of conditions associated with the mortality of adults with DS. This review focuses on the rates and contributing factors to medical conditions that are common in adults with DS or that show changes with age. Skin and hair changesAdults with DS experience a number of dermatological and autoimmune symptoms characteristic of accelerated aging, including premature graying of the hair, hair loss and wrinkling of the skin (Lott, 1982). Significant differences in the skin and chronological age of the individual have been found in post-mortem examinations of the skin of adults with DS NIH Public Access

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Section: Seizure Disordermentioning

confidence: 99%

Health Conditions Associated with Aging and End of Life of Adults with Down Syndrome

Esbensen

2010

International Review of Research in Mental Retardation

148

113

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“…However, these drugs are convulsant at high doses, precluding their use as cognition enhancers in humans, particularly considering that DS patients are more prone to convulsions (Menendez, 2005). Frequency of seizures has been reported to reach 6–17% in DS people (Veall, 1974) with a triphasic distribution of seizure onset depending on age (infancy, early adulthood and late onset) (Pueschel et al., 1991). …”

Section: Introductionmentioning

confidence: 99%

Specific targeting of the GABA-A receptor α5 subtype by a selective inverse agonist restores cognitive deficits in Down syndrome mice

et al. 2011

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An imbalance between inhibitory and excitatory neurotransmission has been proposed to contribute to altered brain function in individuals with Down syndrome (DS). Gamma-aminobutyric acid (GABA) is the major inhibitory neurotransmitter in the central nervous system and accordingly treatment with GABA-A antagonists can efficiently restore cognitive functions of Ts65Dn mice, a genetic model for DS. However, GABA-A antagonists are also convulsant which preclude their use for therapeutic intervention in DS individuals. Here, we have evaluated safer strategies to release GABAergic inhibition using a GABA-A-benzodiazepine receptor inverse agonist selective for the α5-subtype (α5IA). We demonstrate that α5IA restores learning and memory functions of Ts65Dn mice in the novel-object recognition and in the Morris water maze tasks. Furthermore, we show that following behavioural stimulation, α5IA enhances learning-evoked immediate early gene products in specific brain regions involved in cognition. Importantly, acute and chronic treatments with α5IA do not induce any convulsant or anxiogenic effects that are associated with GABA-A antagonists or non-selective inverse agonists of the GABA-A-benzodiazepine receptors. Finally, chronic treatment with α5IA did not induce histological alterations in the brain, liver and kidney of mice. Our results suggest that non-convulsant α5-selective GABA-A inverse agonists could improve learning and memory deficits in DS individuals.

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“…PTZ is also known for its ability at higher dose to induce seizure, by impairing GABA-mediated inhibition [12], [13]. Frequency of epilepsy in DS has been reported ranging from 6 to 17% [14]–[16] with phenotype features varying with the age of the patient and a triphasic distribution of seizure onset (infancy, early adulthood and late onset) having been suggested [17]. A prevalence reaching 46% in patients over 50 years was even reported [14], [16].…”

Section: Introductionmentioning

confidence: 99%

Characterization of PTZ-Induced Seizure Susceptibility in a Down Syndrome Mouse Model That Overexpresses CSTB

et al. 2011

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Down syndrome (DS) is a complex genetic syndrome characterized by intellectual disability, dysmorphism and variable additional physiological traits. Current research progress has begun to decipher the neural mechanisms underlying cognitive impairment, leading to new therapeutic perspectives. Pentylenetetrazol (PTZ) has recently been found to have positive effects on learning and memory capacities of a DS mouse model and is foreseen to treat DS patients. But PTZ is also known to be a convulsant drug at higher dose and DS persons are more prone to epileptic seizures than the general population. This raises concerns over what long-term effects of treatment might be in the DS population. The cause of increased propensity for epilepsy in the DS population and which Hsa21 gene(s) are implicated remain unknown. Among Hsa21 candidate genes in epilepsy, CSTB, coding for the cystein protease inhibitor cystatin B, is involved in progressive myoclonus epilepsy and ataxia in both mice and human. Thus we aim to evaluate the effect of an increase in Cstb gene dosage on spontaneous epileptic activity and susceptibility to PTZ-induced seizure. To this end we generated a new mouse model trisomic for Cstb by homologous recombination. We verified that increasing copy number of Cstb from Trisomy (Ts) to Tetrasomy (Tt) was driving overexpression of the gene in the brain, we checked transgenic animals for presence of locomotor activity and electroencephalogram (EEG) abnormalities characteristic of myoclonic epilepsy and we tested if those animals were prone to PTZ-induced seizure. Overall, the results of the analysis shows that an increase in Cstb does not induce any spontaneous epileptic activity and neither increase or decrease the propensity of Ts and Tt mice to myoclonic seizures suggesting that Ctsb dosage should not interfere with PTZ-treatment.

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The Prevalence of Epilepsy Among Mongols Related to Age

Cited by 34 publications

References 12 publications

Health Conditions Associated with Aging and End of Life of Adults with Down Syndrome

Health Conditions Associated with Aging and End of Life of Adults with Down Syndrome

Specific targeting of the GABA-A receptor α5 subtype by a selective inverse agonist restores cognitive deficits in Down syndrome mice

Characterization of PTZ-Induced Seizure Susceptibility in a Down Syndrome Mouse Model That Overexpresses CSTB

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