Sensory evoked potentials in infants with Down syndrome

Chen, Yung-Jung; Fang, Peng-Cheng

doi:10.1080/08035250500252609

Cited by 36 publications

(23 citation statements)

References 18 publications

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“…In the newborn period, DS infants exhibit delay in achievement of developmental motor and sensory milestones, as well as in olfactory, auditory, and visual sensitivity94138139140. Our data are congruent with previous studies that have demonstrated delay in the acquisition of developmental milestones during the early postnatal period in Ts65Dn mice1821141.…”

Section: Discussionsupporting

confidence: 91%

Early neurotrophic pharmacotherapy rescues developmental delay and Alzheimer’s-like memory deficits in the Ts65Dn mouse model of Down syndrome

Kazim

Blanchard

Bianchi

et al. 2017

Sci Rep

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Down syndrome (DS), caused by trisomy 21, is the most common genetic cause of intellectual disability and is associated with a greatly increased risk of early-onset Alzheimer’s disease (AD). The Ts65Dn mouse model of DS exhibits several key features of the disease including developmental delay and AD-like cognitive impairment. Accumulating evidence suggests that impairments in early brain development caused by trisomy 21 contribute significantly to memory deficits in adult life in DS. Prenatal genetic testing to diagnose DS in utero, provides the novel opportunity to initiate early pharmacological treatment to target this critical period of brain development. Here, we report that prenatal to early postnatal treatment with a ciliary neurotrophic factor (CNTF) small-molecule peptide mimetic, Peptide 021 (P021), rescued developmental delay in pups and AD-like hippocampus-dependent memory impairments in adult life in Ts65Dn mice. Furthermore, this treatment prevented pre-synaptic protein deficit, decreased glycogen synthase kinase-3beta (GSK3β) activity, and increased levels of synaptic plasticity markers including brain derived neurotrophic factor (BNDF) and phosphorylated CREB, both in young (3-week-old) and adult (~ 7-month-old) Ts65Dn mice. These findings provide novel evidence that providing neurotrophic support during early brain development can prevent developmental delay and AD-like memory impairments in a DS mouse model.

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Section: Discussionsupporting

confidence: 91%

Early neurotrophic pharmacotherapy rescues developmental delay and Alzheimer’s-like memory deficits in the Ts65Dn mouse model of Down syndrome

Kazim

Blanchard

Bianchi

et al. 2017

Sci Rep

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“…In the newborn period, infants with DS have hypotonia and delay in achievement of developmental motor(2) and sensory milestones as well as in olfactory, auditory and visual sensitivity(3,4). At the neuropathological level, neonates with DS have a smaller brain, delayed myelination of neurons and glial alterations(5).…”

mentioning

confidence: 99%

Prevention of Developmental Delays in a Down Syndrome Mouse Model

Toso

Cameroni

Roberson³

et al. 2008

Obstetrics &Amp; Gynecology

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Objective To estimate whether prenatal treatment with neuroprotective peptides prevent the developmental delay and the glial deficit in the Ts65Dn mouse model for Down syndrome, and to explore the peptides effects on achievement of normal development. Methods Pregnant Ts65Dn females were randomly assigned to NAPVSIPQ+ SALLRSIPA or control and were treated by investigators blinded to treatment and genotype on gestational days 8–12. Offspring were tested from postnatal day (P) 5 to 21 for motor and sensory milestones with standardized tests by operators blinded to the pup’s treatment and genotype. The pup’s genotype was determined after completion of all tests. Activity-dependent-neurotrophic-factor, glial fibrillary acidic protein, and vasoactive intestinal peptide expression were determined using real time polymerase chain reaction. Results Trisomic (Ts) mice achieved milestones with a significant delay in 4 of 5 motor and sensory milestones. Ts mice that were prenatally exposed to NAPVSIPQ+SALLRSIPA achieved developmental milestones at the same time as the controls in 3 of 4 motor and 1 of 4 sensory milestones (p<0.01). Euploid pups prenatally treated with NAPVSIPQ+SALLRSIPA achieved developmental milestones significantly earlier than the euploid pups prenatally treated with placebo. Activity-dependent-neurotrophic-factor, expression was significantly downregulated in the Ts65Dn brains versus the controls, prenatal treatment with NAPVSIPQ+SALLRSIPA prevented the activity-dependent-neurotrophic-factor, decrease in the Ts65Dn brains, and the expression was not different from the controls. The glial marker glial fibrillary acidic protein demonstrated the known glial deficit in the Ts65Dn mice, and treatment with NAPVSIPQ+SALLRSIPA prevented its downregulation. Lastly, vasoactive intestinal peptide levels were increased in the Ts brains, while treatment with NAPVSIPQ+SALLRSIPA did not prevent its upregulation. Conclusions Prenatal treatment with NAPVSIPQ and SALLRSIPA prevented developmental delay and the glial deficit in Down syndrome. These findings highlight a possibility for the prevention of developmental sequelae in Down syndrome and suggest a potential intervention during pregnancy that may improve the outcome.

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“…22 Recently, Chen and Fang 21 reported 18.5% slower median nerve, SEP-measured, central conduction for 6-to 18-month-old infants with DS, compared with age-matched, healthy, control subjects; their peripheral NCV was 8.7% slower (53.9 m/second, compared with 59.0 m/second for control subjects; values were calculated by dividing the reported arm lengths by the reported latencies to N9). 21 Chen and Fang, 21 as well as other investigators studying children with DS, 1,22,34 suggested that the impaired nerve conduction was related to DS-specific neuropathologic and neurochemical abnormalities, such as abnormal dendritic spine morphologic features and numbers and different myelin composition. The results of our study (ie, the absence of positive effects of postnatal thyroxine treatment on conduction) do not contradict these views.…”

Section: Discussionmentioning

confidence: 92%

“…17,18 However, animal studies suggest that the normal myelination process can be harmed by prolonged hyperthyroidism early in life, which may lead ultimately to impaired nerve conduction. 19,20 Given the presumed mildly hypothyroid state of neonates with DS and their reported somewhat slowerthan-normal nerve conduction throughout life, [21][22][23][24] we hypothesized that, in addition to improving development and growth, thyroxine treatment initiated early might improve nerve conduction. To test this hypothesis and to monitor for possible unwanted effects of longstanding plasma free thyroxine concentrations in the upper part of the reference interval, we conducted SEP studies for all infants with DS participating in our randomized, clinical trial.…”

mentioning

confidence: 99%

Median Nerve Conduction Velocity and Central Conduction Time Measured With Somatosensory Evoked Potentials in Thyroxine-Treated Infants With Down Syndrome

Trotsenburg¹,

Smit

Koelman³

et al. 2006

Pediatrics

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Postnatal thyroxine treatment of infants with Down syndrome did not alter somatosensory evoked potential-measured peripheral or central nerve conduction significantly. The absence of favorable effects suggests that pathologic mechanisms other than mild postnatal hypothyroidism underlie the impaired nerve conduction. The absence of adverse effects suggests that longstanding plasma free thyroxine concentrations in the high-normal range are not harmful to nerve maturation.

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Sensory evoked potentials in infants with Down syndrome

Abstract: Our results indicate that various sensory deficits occur in patients with DS during the first year of life.

Cited by 36 publications

References 18 publications

Early neurotrophic pharmacotherapy rescues developmental delay and Alzheimer’s-like memory deficits in the Ts65Dn mouse model of Down syndrome

Early neurotrophic pharmacotherapy rescues developmental delay and Alzheimer’s-like memory deficits in the Ts65Dn mouse model of Down syndrome

Prevention of Developmental Delays in a Down Syndrome Mouse Model

Median Nerve Conduction Velocity and Central Conduction Time Measured With Somatosensory Evoked Potentials in Thyroxine-Treated Infants With Down Syndrome

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