Publications by authors named "E Wiggs"

Working memory (WM) impairment is one of the most frequent cognitive deficits in Parkinson's disease (PD). However, it is not known how neural activity is altered and compensatory responses eventually fail during progression. We aimed to elucidate neural correlates of WM and compensatory mechanisms in PD.

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Objective: To investigate the effect of hypercortisolism on the developing brain we performed clinical, cognitive, and psychological evaluation of children with Cushing disease (CD) at diagnosis and 1 year after remission.

Study Design: Prospective study of 41 children with CD. Children completed diverse sets of cognitive measures before and 1 year after remission.

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Article Synopsis
  • Xeroderma pigmentosum (XP) is a rare genetic disorder that leads to defective DNA repair, high skin cancer risk, and progressive neurological degeneration in some patients, especially in the XP-A and XP-D complementation groups.
  • A study from 1986 to 2015 analyzed nerve conduction studies (NCS) of 33 XP patients, revealing that most neurological issues were found in the XP-A and XP-D groups, where 78% of XP-A patients exhibited sensorimotor neuropathy and 50% of XP-D patients showed sensory neuropathy.
  • The findings indicated that sensorineural hearing loss could be an early clinical sign of XP, with distinct neurological characteristics between XP-A and XP-D patients, such
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We examined safety, tolerability, and efficacy of SGS-742, a γ-aminobutyric acid B (GABA-B) receptor antagonist, in patients with succinic semialdehyde dehydrogenase deficiency. This was a single-center randomized, double-blind crossover phase II clinical trial of SGS-742 versus placebo in patients with succinic semialdehyde dehydrogenase deficiency. Procedures included transcranial magnetic stimulation and the Adaptive Behavior Assessment Scale.

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Objective: Niemann-Pick disease type C1 (NPC1) is a lysosomal storage disease characterized by progressive neurodegeneration, with the age of diagnosis ranging from the prenatal period through adulthood. Although neurological symptoms usually precede genetic diagnosis, they do not necessarily prompt diagnosis in the early years. Few prospective data are available to describe neurological onset, including neurodevelopmental delays, in children with NPC1.

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