Background
In White populations more than 60% of clinically isolated syndrome (CIS)
convert to multiple sclerosis (MS) on a long-term follow-up; several
predictors for conversion have been identified.
Objective
This study aimed to determine the conversion rate and the predictors of
conversion from CIS to MS (McDonald 2010) among Indians. The other objective
was to evaluate the diagnostic accuracy of the new McDonald 2017 criteria in
prediction of a second clinical attack.
Methods
Clinical and demographic data of CIS cohorts were collected. Baseline
investigations included cerebrospinal magnetic resonance imaging (MRI) with
contrast and cerebrospinal fluid (CSF) testing for oligoclonal band (OCB).
Follow-up clinical and MRI examinations were performed annually for at least
24 months.
Results
Of the 82 subjects (age range 15–58 years), 36 (43.9%) converted to MS; 31/82
(37.8%) converted in 24 months. The predictors for conversion were earlier
age of onset, CSF-OCB, cerebral MRI T2 lesion count, and periventricular and
juxtacortical location of lesions. Twenty-two (26.83%) CIS fulfilled the
McDonald MS 2017 criteria at baseline.
Conclusion
In this first prospective study of CIS in India, the risk factors for
conversion are similar but the conversion rate to MS is lower than that in
the western nations.
We conclude that these mutations are rare causes of PD in the Eastern Indian population and, therefore, of little help for genetic counseling and diagnostic purposes.
Associated abnormalities of other organ systems result in considerable long-term morbidity in patients of prune-belly syndrome (PBS). The gastrointestinal anomalies commonly observed with PBS are persistence of the embryonic wide mesentery and absence of fixation to the posterior abdominal wall, whereas anorectal malformations (ARM) are rarely associated with PBS. We describe one such case of PBS associated with high ARM and urethral stenosis in a one-day-old male newborn. The neonate underwent preliminary sigmoid colostomy for ARM, but he subsequently developed necrotizing gastritis and gastric perforation in the postoperative period and had to undergo partial gastrectomy and vesicostomy. The neonate developed fatal septicemia after second surgery. A review of all the reported cases of this association is presented and the dilemmas in the management of these cases are discussed.
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