Papilledema in obstructive hydrocephalus caused by giant cell astrocytoma of tuberous sclerosis.

A 5-year-old girl with progressive hemiparesis and headache was found by brain imaging to have a large tumor centered at the foramen of Monro, blocking cerebrospinal outflow and producing massive lateral ventriculomegaly. Total excision of the mass led to a pathologic diagnosis of giant cell astrocytoma. Dermatologic abnormalities had been detected shortly after birth but were unexplained. Abdominal imaging disclosed renal cysts, and ophthalmologic examination disclosed papilledema and retinal plaques. On this basis, a diagnosis of tuberous sclerosis (TS) was finally made. Two months after surgery, papilledema had resolved, and visual function appeared to be normal. Although the patient apparently escaped visual loss, other reports affirm that giant cell astrocytoma, a common tumor in TS, may go undetected for long enough to produce irreversible optic neuropathy from chronic papilledema. Because patients with TS may not report visual loss, they should undergo periodic ophthalmologic screening.     

Clinical and polysomnographic data of positional sleep apnea and its predictors

Introduction

In Asian population, facial structure may contribute to the primary pathophysiology of obstructive sleep apnea (OSA). We hypothesized that sleep position may have more effect on OSA in Asians compared to the Western population. If this hypothesis is accurate, positional therapy will have a major impact on treatment of OSA among Asians.

Patients/methods

We reviewed 263 polysomnographic studies from our laboratory from January 1, 2010 to June 30, 2010. Criteria for positional and non-positional OSA were (1) supine respiratory disturbance index (RDI)/non-supine RDI ??2 and total RDI ??5 and (2) supine RDI/non-supine RDI <2 and total RDI ??5, respectively. We aimed to determine the difference in baseline characteristics, polysomnographic findings, and predictors for positional OSA.

Results

We found 144 patients diagnosed with OSA (RDI ??5), and 96 patients met the criteria for positional OSA (67%), in which in almost half of these patients (47%), RDI was normalized (RDI?<?5) in non-supine position. Snoring frequency were significantly lower among positional OSA and OSA was less severe indicated by lower RDI and arousal index, higher mean and nadir oxygen saturation, and higher %NREM3. We also found that low snoring frequency (less than 20% of total sleep time) was a significant predictor for positional OSA (odd ratio of 3.27; p?=?0.011), contrarily to low mean oxygen saturation (<95%) which was found to be a negative predictor (odd ratio of 0.31; p?=?0.009). Among OSA patients, low RDI (<15) was a significant predictor for normalization of RDI in non-supine position (odd ratio of 8.77; p?=?<?0.001), contrarily to low mean oxygen saturation (<95%) which was also found to be a negative predictor (odd ratio of 0.13; p?=?0.001).

Conclusion

Positional OSA is very prevalent and noted in almost 70% of our patients. Low snoring frequency was noted to be a positive predictor for positional OSA, contrarily to low mean oxygen saturation which was found to be a negative predictor. These findings are encouraging that positional therapy can be very beneficial as the treatment modality for OSA among Asians.     

Lymphoplasmacyte-rich meningioma mimicking idiopathic hypertrophic pachymeningitis.

A 24-year-old man presented with long-term headache and progressive visual loss. Neuro-ophthalmic manifestations included finger counting acuity in both eyes, weakly reactive pupils, pale optic discs, and increased deep tendon reflexes. Brain MRI showed meningeal thickening that involved the optic nerves and chiasm and enveloped and displaced the brainstem as far caudally as the foramen magnum. The diffuse extensive nature of the lesion suggested an inflammatory process such as idiopathic hypertrophic pachymeningitis (IHP), but anterior temporal brain biopsy disclosed a relatively high proportion of meningothelial cells with islands of polyclonal inflammatory reaction consistent with a diagnosis of lymphoplasmacyte-rich meningioma (LRM), a rare variant. Among the 19 reported cases of LRM, none has shown as extensive a mass as seen in our patient. Distinguishing between LRM and IHP is important because these entities are treated differently.     

A novel paradigm in the treatment of oligometastatic non-small cell lung cancer

Background

Stage IV non-small cell lung cancer (NSCLC) is thought to uniformly carry a poor prognosis with a median survival of less than 1 year and 5-year survival of less than 5%. In patients with a low volume (i.e. single site) of distant disease, the prognosis is slightly more favorable than that of more advanced (i.e. multiple sites of metastases) disease. For those with limited metastases, we developed a paradigm of adding concurrent chemotherapy and radiotherapy to the primary tumor once the tumor demonstrated chemotherapy sensitivity.

Methods

Charts of patients from 1999-2006 with non-small cell lung cancer were reviewed to find those with a single extra-thoracic site of disease treated with combined modality therapy. We found nine patients of 640 who met these criteria. Initial treatment consisted of induction chemotherapy, except for brain metastases which were managed first (n=1). If patients experienced a response to chemotherapy without new metastases, the extra-thoracic site was treated for total control with curative dose chemoradiotherapy to the primary site. Survival, time to progression, and sites of progression were assessed.

Results

Median survival was 28 months (95% CI 18-50 mo) with median time to progression of 15 months (95% CI 8-24 mo). All except one patient progressed in the CNS, either with brain metastases (n=7) or leptomeningeal disease (n=1).

Conclusions

Such an approach offers the potential for enhanced quality and quantity of survival by incorporating aggressive RT for select patients without disease progression after induction chemotherapy. Patients tended to fail in the CNS, suggesting the importance of continued surveillance of the neuraxis or possibly prophylactic cranial irradiation. Future plans will correlate outcomes with molecular markers.     

Age related differences in the neural substrates of motor sequence learning after interleaved and repetitive practice

Practice of tasks in an interleaved order generally induces superior retention compared to practicing in a repetitive order. Younger and older adults practiced serial reaction time tasks that were arranged in a repeated or an interleaved order on 2 successive days. Retention was tested on Day 5. For both groups, reaction times in the interleaved condition were slower than the repetitive condition during practice, but the reverse was true during retention on Day 5. After interleaved practice, changes in M1 excitability measured by paired-pulse TMS were greater than after repetitive practice, and this effect was more pronounced in older adults. Moreover, the increased M1 excitability correlated with the benefit of interleaved practice. BOLD signal was also increased for interleaved compared to repetitive practice in both groups. However, the pattern of correlations between increased BOLD during practice and subsequent benefit of the interleaved condition differed by group. In younger adults, dorsolateral-prefrontal activity during practice was related to this benefit, while in older adults, activation in sensorimotor regions and rostral prefrontal cortex during practice correlated with the benefit of interleaving on retention. Older adults may engage compensatory mechanisms during interleaved practice such as increasing sensorimotor recruitment which in turn benefits learning.     

Patient-Reported Outcome Measures in the Food and Drug Administration Pilot Compendium: Meeting Today’s Standards for Patient Engagement in Development?

Background

In 2016, the Food and Drug Administration (FDA) released a Pilot Clinical Outcome Assessment Compendium (COA Compendium) intended to foster patient-focused drug development (PFDD). However, it is unclear whether patient perspectives were solicited during development or validation of the included patient-reported outcome (PRO) measures.

Determination of discriminatory power of genetic markers used for genotyping Giardia duodenalis

Small subunit ribosomal DNA (SSU-rDNA), glutamate dehydrogenase (gdh), beta-giardin, triosephosphate isomerase (tpi), and elongation factor 1-alpha (ef1-alpha) genes are useful genetic markers for genotypic analysis of the intestinal protozoan, Giardia duodenalis (syn. G. lamblia, G. intestinalis), the cause of enteric disease in humans. To quantitatively compare the discriminatory power of these loci, 43 fecal samples were collected from central, northern and eastern Thailand and G. duodenalis specimens were analyzed using PCR-based genotyping and subcloning methods. Approximately equal prevalence of assemblage A (21) and B (22) were present among these populations. Analysis of Simpson's index and Wallace coefficient values from assemblage B isolates together with the data obtained from GenBank showed that the combination of two loci provides a higher discrimination power for subgenotyping G. duodenalis than using any single locus.     

A multicenter, randomized, controlled clinical trial of ligasure small jaw vessel sealing system versus conventional technique in thyroidectomy

The objective of this work was to compare efficacy and adverse effects between vessel sealing system thyroid lobectomy (VSSL) with the new LigaSure? Small Jaw Instrument and conventional thyroid lobectomy (CTL). This is a multicenter, randomized and controlled study, conducted in the Department of Otolaryngology, King Chulalongkorn Memorial Hospital, Thai Red Cross Society, Faculty of Medicine, Chulalongkorn University, Bangkok and Division of Otolaryngology, Sukhothai General Hospital, Sukhothai, Thailand. Forty subjects with solitary thyroid nodule planned for thyroid lobectomy were enrolled consecutively in both centers. Intervention was randomized by block randomization into two groups (VSSL, CTL). Primary outcomes were operative time and intraoperative blood loss. Secondary outcomes were postoperative blood loss and recurrent laryngeal nerve paralysis. There was clinically significant less operative time (VSSL/CTL = 62.4 ± 15.9/83.3 ± 16.1 min, p < 0.001) and less intraoperative blood loss (VSSL/CTL = 40.5 ± 19.1/63.3 ± 22.4 mL, p = 0.001) in VSSL group compared with CTL group. There was no significant difference of postoperative blood loss (VSSL/CTL = 58.8 ± 30.6/76.0 ± 36.9 mL, p = 0.116) between both the groups. There was one case of permanent recurrent laryngeal nerve injury found in both groups. VSSL with the new LigaSure? Small Jaw Instrument was better than conventional technique in terms of shortening operative time. It was also better in reducing intraoperative hemorrhage, but this effect did not exceed clinically significant levels. Postoperative blood loss and recurrent laryngeal nerve injury were not significantly different between both the groups.     

Optic neuropathy associated with periostitis in relapsing polychondritis.

Optic neuropathy is an uncommon manifestation of relapsing polychondritis (RPC), a rare systemic disease affecting cartilaginous and proteoglycan-rich structures. The optic neuropathy has been attributed to ischemia, intrinsic inflammation of the optic nerve, or spread of inflammation to the nerve from adjacent intraconal orbital tissues. We report a case of recurrent corticosteroid-responsive optic neuropathy in which MRI did not show ocular, optic nerve, or intraconal orbital abnormalities but did show periosteal thickening and enhancement in the apical orbit and adjacent intracranial space consistent with periostitis. The periostitis, which is a manifestation of a systemic vasculitis or an autoimmune reaction to progenitors of cartilage, probably caused the optic neuropathy by compression or inflammation. It is important to recognize this mechanism of optic neuropathy as its imaging features may be a subtle yet critical clue to an underlying systemic condition that can be life-threatening if not properly managed.     

Interleaved practice enhances skill learning and the functional connectivity of fronto‐parietal networks

Practice of tasks in an interleaved order generally induces superior learning compared with practicing in a repetitive order, a phenomenon known as the contextual‐interference (CI) effect. Increased neural activity during interleaved over repetitive practice has been associated with the beneficial effects of CI. Here, we used psychophysiological interaction (PPI) analysis to investigate whether the neural connectivity of the dorsal premotor (PM) and the dorsolateral prefrontal (DLPFC) cortices changes when motor sequences are acquired through interleaved practice. Sixteen adults practiced a serial reaction time task where a set of three 4‐element sequences were arranged in a repetitive or in an interleaved order on 2 successive days. On Day 5, participants were tested with practiced sequences to evaluate retention. A within‐subjects design was used so that participants practiced sequences in the other condition (repetitive or interleaved) 2–4 weeks later. Functional magnetic resonance images were acquired during practice and retention. On Day 2 of practice, there was greater inter‐regional functional connectivity in the interleaved compared with the repetitive condition for both PM‐seeded and DLPFC‐seeded connectivity. The increased functional connectivity between both seeded regions and sensorimotor cortical areas correlated with the benefit of interleaved practice during later retention. During retention, a significant PPI effect was found in DLPFC‐seeded connectivity, with increased DLPFC‐supplementary motor area connectivity correlated with the benefits of interleaved practice. These data suggest that interleaved practice benefits learning by enhancing coordination of sensorimotor cortical regions, and superior performance of sequences learned under CI is characterized by increased functional connectivity in frontal cortex. Hum Brain Mapp, 2013. © 2012 Wiley Periodicals, Inc.