Alzheimer's disease (AD) is known to be associated with an increased risk of epilepsy, which is not exclusively related to the late stage of the disease - when a major cognitive impairment is observed, previously known as the dementia stage - but also to its prodromal stage (mild cognitive impairment). Moreover, published case reports and cohorts have shown that epilepsy may occur even earlier, at the preclinical stage of AD: Epileptic seizures may therefore be the sole objective manifestation of the disease. Such a situation is called the epileptic variant of AD (evAD). EvAD is one of the etiologies of late-onset epilepsy, which means that it carries a risk of later progression to dementia and that it can only be diagnosed by assessing amyloid and tau biomarkers. However, evAD is a window of therapeutic opportunity that is probably optimal for preventing, through antiseizure medication treatment, the accelerated cognitive decline associated with AD-related brain hyperexcitability (manifested by seizures or interictal epileptiform activities).
Introduction: Nodding syndrome (NS), a poorly understood severe neurological disorder develops in children. In Uganda, some NS cases have grown into child-bearing adults. Babies born to mothers with NS may be prone to impaired neurodevelopmental outcomes. Cognitive deficits in mothers with NS may further inhibit care offered to their children hence compromising neurocognitive development, physical growth, and behaviour.
Objectives: The study aimed to determine the neurodevelopmental, behavioural, nutritional, and physical growth outcomes of children whose mothers have nodding syndrome.
Methods: A comparative cross-sectional study was conducted between May 2021 and April 2022 in Northern Uganda. Children aged 0-5 years of mothers with NS were compared to those of mothers without NS, matched by age, gender and neighbouring residence. Neurodevelopment, behaviour, nutrition, and physical growth were assessed using standardized measures and t-tests employed for group comparisons of outcomes.
Results: Overall,106 children participated. Fifty-three (53) were offspring of mothers with NS and 53 of mothers without NS; having a mean age of 26.9 (2.22) and 27.5(2.12) months respectively. Children whose mothers have NS had significantly lower neurodevelopmental scores than those of NS-unaffected mothers in fine motor (37.5(12.1) vs 44.2(14.3), p = 0.011), receptive language (37.8(10.8) vs 43.9(12.9), p = 0.010), overall cognitive development (74.36(17.8) vs 83.34(19.6), p = 0.015), and attention (0.64(0.20) vs 0.76(0.15), p = 0.001). There were no differences in the behaviour scores. Children of mothers with NS also had significantly lower weight-for-age z scores (WAZ) (p = 0.003) and length/height-for-age z scores (LAZ/HAZ) (p = 0.001); with 19(35.9 %) of them stunted.
Conclusion: Children whose mothers have NS have poorer neurodevelopmental, nutritional, and physical growth outcomes. Interventions to improve outcomes in these children are warranted.
Imaging and resection strategies for pediatric gangliogliomas (GG) and dysembryoplastic neuroepitheliomas (DNET) presenting with epilepsy were retrospectively analyzed in a consecutive institutional series of surgically treated patients.
Twenty-two children (median 8 years, 3–18 years) presented with seizures for 30 months median (14–55.2 months) due to a histologically verified GG/DNET.
There were 20 GG and 2 DNT, 68 % located temporal, 32 % extra-temporal. Seizure history was significantly longer in temporal cases (38 versus 14 months median, p < 0.01). MRI contrast enhancement was present in 50 % and methionine (MET) positron emission tomography (PET) uptake in 70 % (standard uptake values (SUVs) 2.92 mean, from 1.6 to 6.4). 27 % had glucose PET hypometabolism. Primarily, in temporal GG, ECoG (electrocorticography) -guided lesionectomies were performed in 87 % and antero-mesial temporal lobe resections (AMTLR) in 13 %, whereas in extra-temporal GG/DNETs, lesionectomies were performed in 100 %. ILAE Class 1 seizure outcome was primarily achieved in 73 % of the temporal cases, and was increased to 93 % by performing six repeat surgeries using AMTLR. Extratemporal patients experienced ILAE Class 1 seizure outcomes in 86 % without additional surgeries, although harboring significantly more residual tumor (p < 0.005, mean follow-up 28 months).
In children, MET PET imaging for suspected GG is proposed preoperatively showing a high diagnostic sensitivity and an option to delineate the lesions for navigated resection, whereas MRI contrast behavior was of no differential diagnostic use. As a surgical strategy we propose primarily lesionectomies for extratemporal but AMTLR for temporal GG respecting eloquent brain areas.