Some selected abstract:

1

Clarke M, Newton RW, Klapper PE, Sutcliffe H, Laing I, Wallace G. Childhood encephalopathy: viruses, immune response, and outcome. Dev Med Child Neurol. 2006 Apr;48(4):294-300.

Department of Paediatric Neurology, Leeds General Infirmary, UK.

This study examined children with an acute encephalopathy illness for evidence of viral infection, disordered blood-brain barrier function, intrathecal immunoglobulin synthesis, and interferon (IFN) production, and related their temporal occurrence to outcome. A prospective study of 22 children (13 males, 9 females; age range 1mo to 13y, median 2y 4mo), recorded clinical details, with serum and cerebrospinal fluid (CSF) analysis near presentation and then on convalescent specimens taken up to day 39 of the neurological illness. Outcome was assessed with standard scales between 18 months and 3 years after presentation. A history consistent with viral infection was given in 17 children but laboratory evidence of viral infection was found in only 7 (7/17). In 18 out of 21 children, an elevated CSF:serum albumin ratio indicative of impairment of the blood-CSF and blood-brain barriers was detected at some stage of the illness. In 14 of the 15 children with a raised immunoglobulin G index, and in 12 of the 14 children where the CSF was positive for oligoclonal bands, this was preceded by, or was observed at the same time as, an abnormal albumin ratio. Sixteen children (16/18) had elevated IFN-alpha levels in serum, or CSF, or in both. We conclude that these findings indicate an initial disruption of the blood-brain barrier followed by intrathecal antibody production by activated lymphocytes, clonally restricted to a few antigens. This is the first in vivo study to show this as an important pathogenetic mechanism of encephalitis in children. Poor outcome was associated with young age, a deteriorating electroencephalogram pattern from grade 1 to grade 2, and the degree of blood-brain barrier impairment, particularly when prolonged, but not with Glasgow Coma Scale score. The persistence of IFN-alpha was associated with a good prognosis.

Keogh JM, Badawi N.  The origins of cerebral palsy. Curr Opin Neurol. 2006 Apr;19(2):129-34. Review. 

Hornsby Ku-Ring Gai Hospital, University of Sydney, Sydney, New South Wales, Australia.

PURPOSE OF REVIEW: Cerebral palsy is the most common and visible motor disability of childhood. Its aetiology remains a topic of hot debate between those who see it as a reflection of medical mismanagement of an avoidable complication and those who see its origins in the development of the fetal brain affected at many points along a causal pathway to damage. This review outlines the themes of research publications over the year 2004/2005. RECENT FINDINGS: The review looks at recent findings relating to epidemiology, infection and inflammation, prematurity, multiple pregnancy, thrombophilias, genetics, placenta, neuroimaging and rescue therapies in cerebral palsy. SUMMARY: Papers this year have helped clarify risk groups and identify some areas (e.g. the management of thrombophilias and the potential of induced hypothermia) with the potential to be rapidly introduced into clinical practice. In this enigmatic and multifactorial condition, however, progress remains slow. New tools such as magnetic resonance imaging are providing valuable insights into the lesions that result in cerebral palsy but the pathways to injury remain unclear. The future of cerebral palsy research lies in understanding the complex interactions of multiple factors on the road to cerebral palsy or in looking for final common pathways such as inflammation which may be amenable to manipulation.

Diagnosis, Diagnostics, Immunodiagnosis & Immunodiagnostics:

14704.  Dutta A, Tonkin T, Gelman W. Postpartum convulsions--a diagnostic enigma. J R Soc Med. 2006 Apr;99(4):203-4.

14705.  Merkler D, Horvath E, Bruck W, Zinkernagel RM, Del la Torre JC, Pinschewer DD. "Viral deja vu" elicits organ-specific immune disease independent of reactivity to self. J Clin Invest. 2006 May;116(5):1254-63.

Pathogenesis:

14706.  Anlar B, Waye JS, Eng B, Oguz KK.  Atypical clinical course in juvenile metachromatic leukodystrophy involving novel arylsulfatase A gene mutations. Dev Med Child Neurol. 2006 May;48(5):383-7. 

Therapy:

14707.  Clarke M, Newton RW, Klapper PE, Sutcliffe H, Laing I, Wallace G. Childhood encephalopathy: viruses, immune response, and outcome. Dev Med Child Neurol. 2006 Apr;48(4):294-300. 

14708.  Keogh JM, Badawi N.  The origins of cerebral palsy. Curr Opin Neurol. 2006 Apr;19(2):129-34. Review