Background Waldenstrom’s Macroglobulinaemia (WM) is an IgM secreting lymphoplasmocytic lymphoma characterized by involvement mainly in the bone marrow, and occasionally in the lymph nodes and spleen. Central nervous system involvement of WM, known as Bing-Neel syndrome (BNS), is very uncommon and as such the clinical characteristics and treatment outcomes remain to be clarified. Methods We evaluated the incidence, clinical characteristics, and treatment outcome of BNS among patients with WM who were diagnosed at our Institution. Results We identified 13 patients with BNS from our database of 1,523 patients diagnosed with WM from 1999 -2013. The median age at diagnosis of BNS was 60 (range 51-75 years). The median time to development of BNS after WM diagnosis was 6.3 (range 0.3-11.9 years). Patients presented with a variety of neurological signs and symptoms including seizures, hearing loss, cognitive impairment, gait instability and lower extremity weakness. The diagnosis of BNS was confirmed by MRI in 11, and by examination of the CSF in 12 patients. CSF cytopathologic analysis demonstrated definitive evidence of malignant lymphoplasmacytic cells in 6 patients, and was suspicious for malignant cells in 2 patients. CSF flow cytometry was positive in 8, and a clonal immunoglobulin heavy chain gene rearrangement was identified in 6 patients. MYD88 L265P was identified in CSF sample of two patients in whom this examination was undertaken, and who demonstrated malignant disease by flow cytometry and cytological examination. Seven patients were treated with high-dose methotrexate (HD-MTX), 2 with intrathecal liposomal cytarabine, 1 with rituximab and intrathecal methotrexate, and 1 with rituximab and bendamustine. One patient was recommended treatment with HD-MTX and was lost to follow-up, 1 has been initiated on HD-MTX. Of 11 patients, 5 had a response in CSF and/or MRI, 4 had stable disease and 1 progressed following therapy. Of the responders, 2 patients had received HD-MTX, 2 intrathecal liposomal cytarabine and 1 rituximab-bendamustine. Four patients with stable disease had received HD-MTX. One patient who received intrathecal MTX developed treatment related “chemical meningitis”, and was subsequently treated with HD-MTX to which she had a CSF response. The median overall survival was not reached. Eleven patients are alive, 1 died and 1 was lost to follow-up. Conclusions BNS is an uncommon late complication of WM. MYD88 L265P may help in the diagnosis of BNS, and may represent a novel approach for targeted therapy of WM. HD-MTX is active in the treatment of BNS, though prospective studies are required to standardize treatment and improve outcomes. Disclosures: No relevant conflicts of interest to declare.

Neonates who have been deprived of Enteral feeding for a long time such as extremely low birth weight infants, are at high risk of

Preterm and low birth weight (LBW) infants are vulnerable to invasive candidiasis, usually caused by Candida albicans and Candida parapsilosis. Neonatal candidiasis, acquired after 6 days of life, is the most common form of invasive candidiasis in neonates. Once the Candida species penetrate any organ system, it can lead to complications such as meningitis, endocarditis, pyelonephritis, septic arthritis and pneumonia. In recent years, the incidence of invasive candidiasis has decreased in neonatal intensive care units (NICU) due to improvements in neonatal care; however, in the affected neonates, mortality remains a major concern. This is primarily attributable to the limitations of currently used diagnostic tests and delay in treatment. In addition, there is a lack of data on pharmacokinetics and pharmacodynamics of various anti-fungal therapeutic regimens in neonates. This review provides an overview of invasive candidiasis in neonates, including modes of transmission, its risk factors, and management, with special focus on the recent updates in diagnosis and treatment.

We report a case of a neonate who was diagnosed to have intracardiac mass as a fetus and presented with white linear papules that was diagnosed to be Linear Verrucous Epidermal Nevus. Apart from the intracardiac mass, most likely rhabdomyoma, MRI Brain also showed presence of tubers and the Next Generation Sequence Analysis confirmed the diagnosis of Neonatal Tuberous Sclerosis. The neonate remained asymptomatic and was discharged home and remains under close follow up without any symptoms. It is thus suggested that Linear Verrucous Epidermal Nevus, a cutaneous manifestation not described with Tuberous Sclerosis could be considered under the ever-expanding cutaneous signs of Tuberous Sclerosis and should alert the physician toward its possibility.

Background:The Lacey Assessment of Preterm Infants (LAPI) is used in clinical practice to identify premature babies at risk of neuromotor impairments, especially cerebral palsy. There is a shortage of studies on the Lacey assessment despite its wide clinical use. This study attempted to find the diagnostic accuracy of the Lacey assessment of preterm infants to predict neuromotor outcomes of premature babies at 12 months corrected age and to compare their predictive ability with brain ultrasound. Methods: This prospective cohort study included 89 preterm infants (45 females & 44 males) born below 35 weeks gestation. An initial assessment was done using the Lacey Assessment of Preterm Infants (LAPI) after babies reached 33 weeks postmenstrual age. Follow up assessment on neuromotor outcomes was done at 12 months (±1 week) corrected age using two standardized outcome measures, i.e., Infant Neurological International Battery and Alberta Infant Motor Scale. Brain ultrasound data were collected retrospectively. Data were statistically analyzed, the diagnostic accuracy of the Lacey Assessment of Preterm Infants (LAPI) alone and in combination with brain ultrasound was calculated. Results: Fisher's exact test showed p<.01, indicating that there is an association between the Lacey Assessment of Preterm Infants (LAPI) and the neuromotor outcomes at one year corrected age. A combination of Lacey Assessment (LAPI) and brain ultrasound results showed higher sensitivity in predicting abnormal neuromotor outcomes than Lacey Assessment alone (80% vs. 66.7%, respectively). Lacey Assessment also showed high specificity (96.3%) and negative predictive value (97.5%). Conclusion:Results of this study suggest that the Lacey Assessment of Preterm Infants (LAPI) can be used as a supplementary assessment tool for premature babies to identify those at risk of abnormal neuromotor outcomes. These findings have applications to identify premature babies eligible for early intervention services.