Development of a standardized MRI scoring tool for CNS demyelination in children.

BACKGROUND AND PURPOSE: The degree to which MR imaging is useful in the diagnosis of MS is predicated on standardized and reliable evaluation of MR imaging parameters. We aimed to devise items for an MR imaging scoring tool that would have high inter-rater agreement and would be straightforward to apply. MATERIALS AND METHODS: On the basis of a literature search and consensus of an expert panel, we identified 48 parameters that describe acute CNS demyelination, predict MS diagnosis, or characterize demyelinating disorder mimics. MR images of children with clinically confirmed MS, monophasic ADEM, and angiography-negative biopsy-positive small-vessel primary angiitis of the CNS were scored by 2 neuroradiologists independently, using the preliminary 48-parameter tool. Parameters with Cohen κ ≥ 0.6 and deemed important in predicting diagnosis were retained. Parameters not visualized on routine clinical imaging or not important in differentiating MS, ADEM, and SV-cPACNS were discarded. RESULTS: Of 65 eligible patients, 55 children were enrolled (16 with monophasic ADEM, 27 with MS, 12 with SV-cPACNS); 10 were excluded (6 had hard-copy films, 4 did not meet MR imaging quality requirements). Of the 48 parameters, 16 were retained in the final scoring tool. The remaining 28 parameters were discarded: 4 had κ < 0.6 and were not deemed useful in predicting diagnosis; 9 were not visible on routinely acquired clinical images; and 15 had inter-rater agreement ≥0.6 but were not useful in differentiating monophasic ADEM, MS, and SV-cPACNS. CONCLUSIONS: We propose a 16-parameter MR imaging scoring tool that is straightforward to apply in the clinical setting and demonstrates high inter-rater agreement.

Validation of MRI predictors of multiple sclerosis diagnosis in children with acute CNS demyelination.

BACKGROUND: In a recent Canadian prospective study of children with acute demyelinating syndromes (ADS), we demonstrated that the presence of T2 periventricular and T1-hypointense lesions predicted MS diagnosis. We aimed to validate these predictors in a Dutch cohort of children with ADS. METHODS: Participants with ADS were identified from a prospective cohort or archived dataset. MS was diagnosed based on clinical or MRI evidence of relapsing disease. Baseline MRI scans were evaluated for the presence of the two predictive parameters. Sensitivity, specificity, positive (LR+) and negative likelihood ratios (LR-), and positive (PPV) and negative predictive value (NPV) were calculated to evaluate the performance of the MRI parameters at classifying children as having MS or monophasic demyelination. FINDINGS: Of 115 children identified with ADS between December 1993 and December 2009, MRI scans from 87 children (45 prospective; 47 archived) were evaluated; scans of 28 children were excluded due to incomplete or poor quality imaging. Mean duration of observation was longer in the archived group (7.1 years, SD 3.5) than the prospective cohort (3.3 years, SD 1.4). 30 children were diagnosed with MS. Performance of the parameters was not statistically different between the prospective cohort (sensitivity 93.3% [68.1-99.8]; specificity 86.7% [69.3-96.2]; LR+ 7.0 [2.8-17.6]; LR- 0.08 [0.01-0.5]; PPV 77.8% [52.4-93.6]; NPV 96.3% [81.0-99.9]) and archived group (sensitivity 66.7% [38.4-88.2]; specificity 85.2% [66.3-95.8]; LR+ 4.5 [1.7-11.9]; LR- 0.4 [0.2-0.8]; PPV 71.4% [41.9-91.6]; NPV 82.1% [63.1-93.9]). INTERPRETATION: In an independent Dutch cohort, we confirm that the presence of ≥1 T2 periventricular and ≥1 T1-hypointense lesions reliably identifies children with MS. FUNDING: Dutch MS Research Foundation.

Quality of life in childhood epilepsy: what is the level of agreement between youth and their parents?

Children and parents evaluate the child's quality of life (QOL) from their own perspectives; therefore, responses may differ, especially in abstract domains. We examined differences between self- and proxy-reported QOL of children with epilepsy. Children with active epilepsy (N=375) and their parents (N=378) separately completed the CHEQOL-25, a condition-specific QOL measure. The intraclass correlation coefficient was used to determine interrater agreement. Concordance on the Total CHEQOL-25 was 0.45 (P<0.01). Discrepancies were greatest for the subscales of Secrecy (0.24, P<0.01) and Present Concerns (0.32, P<0.01). School placement correlated with discrepancy in the Intrapersonal/Emotional subscale (r=0.19, P<0.05), and the child's age at testing correlated with discrepancy of the Total measure (r=0.15, P<0.01). This study demonstrates that parent perspectives alone are insufficient to measure their child's QOL. The CHEQOL-25 is a practical tool, with complementary parent and child versions, which can be used to determine health-related quality of life in children with epilepsy.

Intracranial Hodgkin's lymphoma in an HIV positive patient.

Intracranial Hodgkin's lymphoma (ICHL) in the HIV positive population is a rarely reported event with only three prior cases reported in the literature. In these prior reports, the outcome of the patients was uncertain or very poor. We report here the case of an HIV seropositive patient with an isolated intracranial recurrence of Hodgkin's Lymphoma (HL) who achieved long-term control of his cranial disease and continues to have a high performance status more than 3 years following treatment.