Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course

Background: Lesion resolution is often observed in children with myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and asymptomatic lesions are less commonly reported in MOGAD than in multiple sclerosis (MS). Objective: We aimed to evaluate brain MRI changes over time in paediatric MOGAD. Methods: Retrospective study in eight UK paediatric neuroscience centres. Acute brain MRI and available follow-up MRIs were reviewed. Predictors for lesion dynamic were evaluated using multivariable regression and Kaplan-Meier survival analyses were used to predict risk of relapse, disability and MOG-Ab status. Results:200 children were included (MOGAD 97; MS 103). At first MRI post-attack, new symptomatic and asymptomatic lesions were seen more often in MS vs MOGAD (52/103 vs 28/97; p=0.002 and 37/103 vs 11/97; p<0.001); 83% of MOGAD patients showed at least one lesion’s resolution at 1st follow‐up scan, and 23% had normal MRI. Only 1 MS patient had single lesion resolution; none had normal MRI. Disappearing lesions in MOGAD were seen in 40% after the 2nd attack, 21% after 3rd attack and none after the 4th attack. New lesions at 1st follow-up scan were associated with increased likelihood of relapse (p=0.02) and persistent MOG-Ab serostatus (p=0.0016) compared to those with no new lesions. Plasma exchange was associated with increased likelihood of lesion resolution (p=0.01). Longer time from symptom onset to steroids was associated with increased likelihood of new lesions; 50% increase at 20 days (p=0.01). Conclusions and Relevance: These striking differences in lesion dynamics between MOGAD and MS suggest greater potential to repair. Early treatment with steroids and plasma exchange is associated with reduced likelihood of new lesions

Use of the hemifrontal flap in reconstruction of the forehead

The hemifrontal flap is an efficient way of reconstructing large unilateral defects that involve the hemiforehead. Six patients had their foreheads reconstructed after the excision of neglected tumours in the frontal region. The flap was designed to provide adequate repair of large defects including the periosteum. The width of the flap above the eyebrow is almost equal to the vertical height of the forehead in the midline. Efficient reconstruction of the primary defect including surface and depth was achieved in all patients. There were no complications with the viability of the flap. The hairline and the line of the eyebrows were preserved in their original sites, with no alteration in the aesthetic landmarks of the forehead in 3 cases, where there was no invasion of tumour

Double scalping flap: a versatile technique in scalp reconstruction

Scalp reconstruction is a challenging area in plastic surgery. The susceptibility of potentially exposed brain tissue is almost always a concern along with the provision of adequate soft tissue coverage in cases of full-thickness defects. The applied reconstructive strategy also affects efficiency in malignant disease treatment providing both local control of the disease and vigorous monitoring for recurrence or metastasis. The general condition of the patients presenting with malignant lesions of the scalp is often impaired because of old age or concomitant disease. Therefore, demanding, long-lasting, or multistage procedures may often be undesirable. The double scalping flap comprises a 1-stage procedure, which can be used in most full-thickness defects of the vertex of the scalp. Some of the primary advantages of the double scalping flap procedure are its versatility and arc of rotation, its minimal donor site morbidity, being relatively simple, and being a short technique. Disadvantages that may be considered are the loss of hair-bearing skin at the occipital region and the poor color and texture match between scalp skin and the split-thickness skin graft that is used on the donor site. We present 2 patients with exemplary case of neglected malignancy of the scalp and their successful treatment using the double scalping flap. Comparison and contrast of alternative reconstructive procedures are also included to further investigate scalp reconstruction

NORSE/FIRES: how can we advance our understanding of this devastating condition?

Introduction: New onset refractory status epilepticus (NORSE) is a rare and devastating condition characterised by the sudden onset of refractory status epilepticus (RSE) without an identifiable acute or active structural, toxic, or metabolic cause in an individual without a pre-existing diagnosis of epilepsy. Febrile infection-related epilepsy syndrome (FIRES) is considered a subcategory of NORSE and presents following a febrile illness prior to seizure onset. NORSE/FIRES is associated with high morbidity and mortality in children and adults. Methods and results: In this review we first briefly summarise the reported clinical, paraclinical, treatment and outcome data in the literature. We then report on existing knowledge of the underlying pathophysiology in relation to in vitro and in vivo pre-clinical seizure and epilepsy models of potential relevance to NORSE/FIRES. Discussion: We highlight how pre-clinical models can enhance our understanding of FIRES/NORSE and propose future directions for research