Delayed maturation of P2 flash visual evoked potential (VEP) latency in newborns of gestational diabetic mothers.

BACKGROUND: The prevalence of gestational diabetes mellitus (GDM) has rapidly increased, yet few prior studies have investigated parameters of early brain development in infants born to gestational diabetic mothers. The present study assessed visual evoked potentials (VEPs) in healthy infants born to gestational diabetic mothers and matched controls. METHODS: After exclusions, in this prospective study we examined VEPs in 73 neonates between 37 weeks and 41 weeks gestation at birth (n = 37 infants of gestational diabetic mothers). Stroboscopic flashes were presented through closed eyelids during passive electroencephalography (EEG) recording to derive VEP waveforms during natural sleep. RESULTS: There was a statistically significant moderate correlation between gestational age at birth and P2 latency of the flash VEP where P2 latency significantly decreased with increasing gestational age (Pearson's R(73) = -0.32, p < .01). There was also a significant moderate correlation between postnatal age (hours of life) and P2 latency of the flash VEP where P2 latency significantly decreased with increasing postnatal age (Pearson's R(73) = -0.23, p < .05). When controlling for gestational age at birth, postnatal age, and sex, there was a significant effect of group (GDM-exposed vs. control) on P2 latency of the flash VEP (p < .05). Infants of gestational diabetic mothers had a significantly longer P2 latency (M: 215.29 ± SD: 2.58 ms) than controls (M: 206.41 ± SD: 2.62 ms). CONCLUSION: Our findings suggest P2 flash VEP latency is a potential measure of cortical maturation and marker of immature development in infants of gestational diabetic mothers.

Asymmetric optic disc edema in a young patient with POEMS: A rare presentation of a rare disease.

PURPOSE: To describe a case of asymmetric optic disc edema presenting as the initial ocular feature of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) syndrome. OBSERVATIONS: A 29-year-old female patient presented with 3 weeks history of blurred vision, proptosis, and peripheral neuropathy as well as hypothyroidism. Fundoscopy revealed optic disc edema associated with visual loss in the left eye. Following a computed tomography (CT) scan and a positron emission tomography/CT (PET/CT) scan which respectively revealed hepatomegaly and multiple osteosclerotic lesions, as well as laboratory findings of monoclonal gammopathy and elevated vascular endothelial growth factor (VEGF) levels, she was diagnosed with POEMS syndrome. After treatment with an autologous stem cell transplant, the optic disc edema and blurred vision resolved. CONCLUSIONS AND IMPORTANCE: The most reported ocular manifestation of POEMS syndrome, a rare and complex multisystem disorder, is bilateral optic disc edema that typically occurs in older males. Therefore, this report presents an uncommon case of asymmetric optic disc edema in a younger, female patient.

Reduced Levels of Proteasome Products in a Mouse Striatal Cell Model of Huntington's Disease.

Huntington's disease is the result of a long polyglutamine tract in the gene encoding huntingtin protein, which in turn causes a large number of cellular changes and ultimately results in neurodegeneration of striatal neurons. Although many theories have been proposed, the precise mechanism by which the polyglutamine expansion causes cellular changes is not certain. Some evidence supports the hypothesis that the long polyglutamine tract inhibits the proteasome, a multiprotein complex involved in protein degradation. However, other studies report normal proteasome function in cells expressing long polyglutamine tracts. The controversy may be due to the methods used to examine proteasome activity in each of the previous studies. In the present study, we measured proteasome function by examining levels of endogenous peptides that are products of proteasome cleavage. Peptide levels were compared among mouse striatal cell lines expressing either 7 glutamines (STHdhQ7/Q7) or 111 glutamines in the huntingtin protein, either heterozygous (STHdhQ7/Q111) or homozygous (STHdhQ111/Q111). Both of the cell lines expressing huntingtin with 111 glutamines showed a large reduction in nearly all of the peptides detected in the cells, relative to levels of these peptides in cells homozygous for 7 glutamines. Treatment of STHdhQ7/Q7 cells with proteasome inhibitors epoxomicin or bortezomib also caused a large reduction in most of these peptides, suggesting that they are products of proteasome-mediated cleavage of cellular proteins. Taken together, these results support the hypothesis that proteasome function is impaired by the expression of huntingtin protein containing long polyglutamine tracts.