Visual pathways involvement in clinically isolated syndrome in children

AIM: To investigate extent and nature of visual pathways involvement in children with clinically isolated syndrome(CIS).METHODS: Forty-seven patients(age 11-17y) with CIS, which later proved to be multiple sclerosis(MS)onset, and 30 controls underwent visual evoked potentials(VEP) investigation within 12 d from the appearance of the first signs of disease. Latency and amplitude of P100 peak were compared with normative data and between groups.RESULTS: In 58% patients, including those without signs of retrobulbar neuritis, significant slowing of conduction along the central visual pathways(P100latency lengthening) is seen. P100 amplitudes drop(signs of axonal damage) are registered less frequently(29% cases).CONCLUSION: The results indicate that visual pathways are often affected in the MS onset; mostly demyelination signs are seen. Despite MRI significance for MS diagnostic, VEPs proved to be still effective in early diagnosis of MS in children.

Magnetic Resonance Imaging in the Acquired Demyelinating Disorders： A Pediatric Cohort Study

Objective： Describing the characteristics of the MRI （magnetic resonance imaging） of patients with ADD （acquired demyelinating disorder） followed in a specialized pediatric clinic. Methods： Descriptive and retrospective study of the MRIs of a pediatric ADD cohort. The included images were acquired in machines with 1.5 or 3T magnets. Low-quality images were excluded. The radiological characteristics of the lesions were described using the 2010 Revised McDonald Diagnostic Criteria regarding localization, contrast enhancement and optic nerve alterations. Results： Twenty-three patients were included （55% female）. The mean age of the first clinical event was 7.7 years. Most common diagnosis was Clinically Isolated Syndrome （35%）, followed by Multiple Sclerosis （30%）, Neuromyelitis Optica （17%） and Acute Disseminated Encephalomyelitis （17%）. Mean time elapsed until diagnosis was 1.8 years. Follow-up MRIs of ADEM patients showed complete or partial resolution of lesions; MS lesions were mostly localized in the brain and four patients had contrast enhancement of lesions in their last available MRI. All NMO （neuromyelitis optica） patients had extensive spinal lesions, and two had optic neuritis. All patients with CIS （clinically isolated syndrome） had focal spinal lesions and evolved with radiologic improvement. Conclusions： ADEM and CIS patients＇ MRIs showed lesion reduction, while MS and NMO patients developed new lesions during follow-up.

Neurofilament light chain in demyelinating conditions of the central nervous system: a promising biomarker

Neurofilaments are the major structural proteins of the neuronal cytoskeleton and are classified according to molecular weight into heavy,intermediate,and light chains.They are released into the interstitial fluid and cerebrospinal fluid(CSF)as a consequence of axonal damage.In particular,the light chain(NfL)represents the most abundant and soluble subunit and has been demonstrated to be increased in the CSF of patients with inflammatory,degenerative,vascular,or traumatic injuries in correlation with clinical and radiological activity.Similar results have been obtained measuring serum NfL with high-sensitivity single-molecule array,which enables reliable and repeatable measurement of the low NfL concentrations in serum.In particular,CSF and serum NfL values are strongly correlated in patients with multiple sclerosis(MS)and have been demonstrated to be increased in patients with MS and clinically isolated syndromes(CIS)in accordance with clinical and radiological activity.NfL levels increase in patients with a recent relapse and seem to predict cognitive impairment,long-term outcome,and conversion of CIS to MS.The few available data on patients with other demyelinating diseases suggest that NfL levels are also increased in neuromyelitis optica spectrum disorders and related conditions in correlation with attack severity,suggesting that axonal damage may occur in these disorders.We herein report and discuss published data on the role of NfL as a possible predictor of disease activity,clinical outcome and treatment response in patients with demyelinating conditions of the central nervous system.