Clinical Spectrum of Hereditary Spastic Paraplegia in Children : A study of 74 cases

Objectives: The aim of the study was to explore the spectrum of hereditary spastic paraplegia (HSP) in children in Oman. Methods: This retrospective study was carried out between January 1994 and August 2011 on children with delayed development, gait disorders and motor handicaps, with signs of symmetrical pyramidal tract involvement. A detailed perinatal and family history, including the age of onset of symptoms, was recorded. The children were labelled as having either the pure or complicated form of HSP based on the established diagnostic criteria. In families with more than one affected child, parents and all other siblings were also examined. Results: Within the study, 74 children from 31 families were diagnosed with HSP. Parental consanguinity was seen in 91% of cases, with 44 children (59.4%) experiencing onset of the disease under one year of age. Complicated HSP was the most common type, seen in 81.1%. Speech involvement, mental retardation, and epilepsy were the most common associated abnormalities. Nonspecific white matter changes and corpus callosum abnormalities were noted in 24.3% of cases on magnetic resonance imaging. Conclusion: The study described clinical features of 74 children with HSP. Autosomal recessive complicated HSP was seen in 81.1% of cases.

Search for TeV γ\gamma -rays from H1426+428 during 2004-07 with the TACTIC telescope

The BL Lac object H1426+428 ($z\equiv 0.129$) is an established source of TeV $\gamma$-rays and detections of these photons from this object also have important implications for estimating the Extragalactic Background Light (EBL) in addition to the understanding of the particle acceleration and $\gamma$-ray production mechanisms in the AGN jets. We have observed this source for about 244h in 2004, 2006 and 2007 with the TACTIC $\gamma$-ray telescope located at Mt. Abu, India. Detailed analysis of these data do not indicate the presence of any statistically significant TeV $\gamma$-ray signal from the source direction. Accordingly, we have placed an upper limit of $\leq1.18\times10^{-12}$ $photons$ $cm^{-2}$ $s^{-1}$ on the integrated $\gamma$-ray flux at 3$\sigma$ significance level.Comment: 11 pages, 5 figures accepted for publication in Journal of Physics G: Nuclear and Particle Physic

Very High Energy gamma-ray observations of Mrk 501 using TACTIC imaging gamma-ray telescope during 2005-06

In this paper we report on the Markarian 501 results obtained during our TeV $\gamma$-ray observations from March 11 to May 12, 2005 and February 28 to May 7, 2006 for 112.5 hours with the TACTIC $\gamma$-ray telescope. During 2005 observations for 45.7 hours, the source was found to be in a low state and we have placed an upper limit of 4.62 $\times$ 10$^{-12}$ photons cm$^{-2}$ s$^{-1}$ at 3$\sigma$ level on the integrated TeV $\gamma$-ray flux above 1 TeV from the source direction. However, during the 2006 observations for 66.8h, detailed data analysis revealed the presence of a TeV $\gamma$-ray signal from the source with a statistical significance of 7.5$\sigma$ above $E_{\gamma}\geq$ 1 TeV. The time averaged differential energy spectrum of the source in the energy range 1-11 TeV is found to match well with the power law function of the form ($d\Phi/dE=f_0 E^{-\Gamma}$) with $f_0=(1.66\pm0.52)\times 10^{-11}cm^{-2}s^{-1}TeV^{-1}$ and $\Gamma=2.80\pm0.27$.Comment: 16 pages and 8 Figures Accepted for publication in the Journal of Physics