Multiple sclerosis in pediatric patients in Slovenia.

AIM: The purpose of this study is to review the Slovenian experience with the diagnostics, treatment and outcome in pediatric multiple sclerosis (MS) patients. METHODS: Children and adolescent diagnosed with MS and followed by Department of Child, Adolescent and Developmental Neurology, University Childrens' Hospital Ljubljana, between 1 January 2000 and 31 December 2012 were included. Data from patients' documentation were analyzed retrospectively to record demographic data, clinical presentation, paraclinical findings, disability progression, relapse rate and treatment strategies. RESULTS: The study includes 38 patients up to 18 years with MS diagnosis, with female: male ratio 2.8:1 and the incidence of 0.81 per 100.000 children of 0-18 years. The mean age at the time of diagnosis was 15 years 4 months. Most frequent presenting symptoms were sensory, motor, brain-stem, visual and ataxia and 65% of patients had a relapse in the first year. The value of paraclinical findings was asessed. 74% of patients with definite MS and 36% of those with clinically isolated syndrome received disease modifyng therapy and 68% of them was not affected at the follow-up. INTERPRETATION: The characteristics of pediatric MS patients in Slovenia disclose higher annual relapse rates than in adults but also favorable impact of disease modifying treatment on a clinical course. Our data suggest a good treatment tolerance but also the influence of the formulation on a decision to start or switch the treatment.

Prognostic value of motor evoked potentials elicited by multipulse magnetic stimulation in a surgically induced transitory lesion of the supplementary motor area: a case report.

Surgery involving the supplementary motor area (SMA) places the patient at risk of transient motor deficit. To predict outcome in patients with early postoperative hypokinesis would be relevant to both the patient and the surgical team. A 15 year old girl with a large left thalamic tumour removed through a left transcallosal approach is described. Despite intraoperatively preserved muscle motor evoked potentials (mMEPs) from all limbs, elicited by multipulse electrical stimulation, she awoke with a right hemiplegia and mutism. On the first postoperative day, neurophysiological evaluation using a multipulse magnetic stimulation technique, with a train of four magnetic stimuli, confirmed the presence of mMEPs from the hemiplegic right limbs. Slight spontaneous motor activity of the right limbs and initial speech were seen later on the same day with dramatic improvement over subsequent days. It is concluded that multiple rather than single magnetic stimulation techniques may be needed to elicit mMEPs for an early postoperative differential diagnosis of SMA damage versus injury to the primary motor cortex or the corticospinal tract.

Embolization of a spinal arteriovenous malformation: correlation between motor evoked potentials and angiographic findings: technical case report.

OBJECTIVE AND IMPORTANCE: Endovascular procedures for the treatment of spinal arteriovenous malformations place the spinal cord at risk of ischemia. This report illustrates the usefulness of motor evoked potentials (MEPs) in detecting functional changes within the spinal cord motor pathways during embolization of a spinal arteriovenous malformation under general anesthesia. CLINICAL PRESENTATION: A 28-year-old man presented with a history of progressive lower extremity numbness and weakness followed by bladder dysfunction. Magnetic resonance imaging and angiography disclosed a T11-T12 spinal arteriovenous malformation. INTERVENTION: During the endovascular procedure, before injection of particles, the disappearance of MEPs from the tibialis anterior muscle led to prompt angiographic reevaluation, which disclosed the arrest of spinal blood flow secondary to radiculomedullary artery occlusion by the catheter. Embolization and catheter withdrawal were followed by temporary recovery of spinal blood flow and MEPs. A second arrest of spinal cord blood flow, caused by severe vasospasm of the feeding radiculomedullary artery, was documented by a control angiogram, and its functional relevance was revealed by a second disappearance of MEPs. The therapeutic effect of papaverine infusion and induced moderate hypertension was confirmed angiographically by complete reopacification of the anterior spinal artery and confirmed neurophysiologically by the complete recovery of MEPs. At the end of the procedure, no additional neurological deficits were noted. CONCLUSION: During spinal cord embolization, MEPs may play a critical role in early detection of spinal cord dysfunction by aiding in the prevention of damage to the spinal cord as well as by predicting the clinical outcome.