Gait is not Affected by Hemispherotomy-Case Report from Two Children.

In children with therapy refractory epilepsy, the functional disconnection of one hemisphere (hemispherotomy) may be considered as a treatment option. The visual field defects and hand function effects associated with the procedure have been extensively studied. However, the effect of the hemispherotomy on gait pattern has thus far only been analyzed qualitatively, and there is limited quantitative data. At the Children's Hospital, we regularly perform standardized quantitative gait analysis studies and care for children with complex epilepsies. During the standard routine of care for two children with structural therapy refractory epilepsy, gait analysis was performed prior to and after hemispherotomy. Both patients had prenatal ischemic brain lesions, had developed severe epilepsy during the first 3 years of life, and were treated with the hemispherotomy at about 7 years of age. Interestingly, one patient did not show any changes in gait pattern, while for the other patient, differences could be observed by means of three-dimensional gait analysis. However, greater deviations to controls postoperatively may also be related to day-to-day variability.

First Clinical Experience with Anti-myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis.

BACKGROUND: Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been consistently found in a range of demyelinating disorders. In this context, MOG-IgG-associated optic neuritis (ON) has been suggested as a new subset of optic neuropathy. However, clinical manifestations and distinctive characteristics have only rarely been described. PATIENTS AND METHODS: A retrospective case series of three patients with MOG-IgG-associated ON. Clinical morphological features using imaging techniques are presented. RESULTS: Three patients (8-year-old boy, 28-year-old female, 48-year-old male) were included. An 8-year-old boy suffered from a bilateral ON with severe visual loss. The best-corrected visual acuity (BCVA) was 0.05 in the right eye and finger counting in the left eye. The patient had a previous episode of acute disseminated encephalomyelitis (ADEM) with a right abducens nerve palsy. Visual acuity recovered after repeated cycles of intravenous methylprednisolone pulse therapy and 10 cycles of plasma exchange. During the last follow-up, BCVA was 0.9 in the right eye and 0.8 in the left eye. A 28-year-old female presented with a bilateral ON. Her BCVA was 0.5 in the right eye and 0.8 in the left eye. She fully recovered with pulse methylprednisolone therapy (1000 mg/d) with tapering after the second cycle and had a BCVA of 1.0 during the last follow-up visit. A 48-year-old male suffered from a relapsing bilateral ON. At first presentation, BCVA was 0.1 in the right eye and finger counting in the left eye. BCVA fully recovered after each pulse therapy with intravenous methylprednisolone (two cycles). Since the first relapse, the patient has been receiving long-term immunosuppression with rituximab. Despite rituximab and low-dose oral prednisone, the patient had another relapse with a left ON. After a third cycle with intravenous methylprednisolone, he partially recovered. BCVA at last follow-up was 1.0 in the right and 0.8 in the left eye. CONCLUSIONS: MOG-IgG antibodies have been identified in different acquired demyelinating syndromes. The patients reported had an ADEM followed by bilateral ON, an isolated bilateral ON, and a relapsing bilateral ON. Individual treatment strategies led to substantial visual recovery in all patients. We recommend inclusion of MOG-IgG antibodies in the diagnostic workup at least after the first recurrence of ON since they can serve as a diagnostic and potential prognostic tool and might lead to specific therapeutic recommendations.

Functional MRI-guided probabilistic tractography of cortico-cortical and cortico-subcortical language networks in children.

In this study, we analyzed the structural connectivity of cortico-cortical and cortico-subcortical language networks in healthy children, using probabilistic tractography based on high angular resolution diffusion imaging. In addition to anatomically defining seed and target regions for tractography, we used fMRI to target inferior frontal and superior temporal cortical language areas on an individual basis. Further, connectivity between these cortical and subcortical (thalamus, caudate nucleus) language regions was assessed. Overall, data from 15 children (8f) aged 8-17 years (mean age 12.1 ±3 years) could be included. A slight but non-significant trend towards leftward lateralization was found in the arcuate fasciculus/superior longitudinal fasciculus (AF/SLF) using anatomically defined masks (p>.05, Wilcoxon rank test), while the functionally-guided tractography showed a significant lateralization to the left (p<.01). Connectivity of the thalamus with language regions was strong but not lateralized. Connectivity of the caudate nucleus with inferior-frontal language regions was also symmetrical, while connectivity with superior-temporal language regions was strongly lateralized to the left (p<.01). To conclude, we could show that tracking the arcuate fasciculus/superior longitudinal fasciculus is possible using both anatomically and functionally-defined seed and target regions. With the latter approach, we could confirm the presence of structurally-lateralized cortico-cortical language networks already in children, and finally, we could demonstrate a strongly asymmetrical connectivity of the caudate nucleus with superior temporal language regions. Further research is necessary in order to assess the usability of such an approach to assess language dominance in children unable to participate in an active fMRI study.

Counter-movement jump characteristics in children with Charcot-Marie-Tooth type 1a disease.

BACKGROUND: Poor performance in sports, especially activities that require explosive movements, is a common reason for initial presentation of children with Charcot-Marie-Tooth type 1a (CMT1a) to the paediatric neuromuscular specialist. RESEARCH QUESTION: The aim of this descriptive, retrospective study was to analyse counter-movement jump characteristics in children with CMT1a in comparison to those in typically developing children (TDC). METHODS: This retrospective study included seven patients with CMT1a and 44 TDC from our data pool. All the participants performed counter-movement jumps, and jump height, peak force, time to peak force, average and peak rate of force development and net vertical impulse were then calculated. For statistical comparison by means of an independent Student's t-test, children with CMT1a were compared to seven sex- and age-matched TDC. Correlation coefficients were calculated to determine the relationship between the force-time variables and jump height. RESULTS: Peak force, net vertical impulse and jump height values in the CMT1a group were significantly lower than those in the TDC group. There were no between-group differences in the time to peak force or average and peak rate of force development. In terms of task symmetry, the correlation between the time-force curve of the left and right leg in the CMT1a group was reduced as compared with that in the TDC group. In both groups, among the parameters measured, there was a significant correlation between jump height and net vertical impulse. SIGNIFICANCE: This study showed that reduced jump performance in children with CMT1a, as demonstrated by decreased counter-movement jump height, was due to a reduced net impulse during this explosive movement task. This finding is critical for children with CMT1a and has to be considered in clinical management and activities of daily living (e.g. sports lessons in school).