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1. Long term outcome in non-multiple sclerosis paediatric acquired demyelinating syndromes

Author

Wassmer, Evangeline, Billaud, Charly, Absoud, Michael, Abdel-Mannan, Omar, Benetou, Christina, Cummins, Carole, Forrest, Katharine, De Goede, Christian, Eltantawi, Noha, Hickson, Helga, Hussain, Nahin, Jardine, Phil, livingston, John H., Mordekar, Santosh, Ramdas, Sithara, Taylor, Micheal, Vijayakumar, K., West, Siobhan, Whitehouse, William P., Kneen, Rachel, Hemingway, Cheryl, Lim, Ming, Hacohen, Yael, and Wright, Sukhvir

Published
2024
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2. Classic ketogenic diet versus further antiseizure medicine in infants with drug-resistant epilepsy (KIWE): a UK, multicentre, open-label, randomised clinical trial

Author

Schoeler, Natasha E, Marston, Louise, Lyons, Laura, Halsall, Sally, Jain, Ruchika, Titre-Johnson, Siobhan, Balogun, Maryam, Heales, Simon J R, Eaton, Simon, Orford, Michael, Neal, Elizabeth, Reilly, Colin, Eltze, Christin, Stephen, Elma, Mallick, Andrew A, O’Callaghan, Finbar, Agrawal, Shakti, Parker, Alasdair, Kirkpatrick, Martin, Brunklaus, Andreas, McLellan, Ailsa, McCullagh, Helen, Samanta, Rajib, Kneen, Rachel, Tan, Hui Jeen, Devlin, Anita, Prasad, Manish, Rattihalli, Rohini, Basu, Helen, Desurkar, Archana, Williams, Ruth, Fallon, Penny, Nazareth, Irwin, Freemantle, Nick, and Cross, J Helen

Published
2023
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3. Neurological and neuropsychiatric complications of COVID-19 in 153 patients: a UK-wide surveillance study

Author

Allen, Claire, Archibald, Neil, Arkell, James, Arthur-Farraj, Peter, Baker, Mark, Ball, Harriet, Bradley-Barker, Verity, Brown, Zoe, Bruno, Stefania, Carey, Lois, Carswell, Christopher, Chakrabarti, Annie, Choulerton, James, Daher, Mazen, Davies, Ruth, Di Marco Barros, Rafael, Dima, Sofia, Dunley, Rachel, Dutta, Dipankar, Ellis, Richard, Everitt, Alex, Fady, Joseph, Fearon, Patricia, Fisniku, Leonora, Gbinigie, Ivie, Gemski, Alan, Gillies, Emma, Gkrania-Klotsas, Effrossyni, Grigg, Julie, Hamdalla, Hisham, Hubbett, Jack, Hunter, Neil, Huys, Anne-Catherine, Ihmoda, Ihmoda, Ispoglou, Sissi, Jha, Ashwani, Joussi, Ramzi, Kalladka, Dheeraj, Khalifeh, Hind, Kooij, Sander, Kumar, Guru, Kyaw, Sandar, Li, Lucia, Littleton, Edward, Macleod, Malcolm, Macleod, Mary Joan, Madigan, Barbara, Mahadasa, Vikram, Manoharan, Manonmani, Marigold, Richard, Marks, Isaac, Matthews, Paul, Mccormick, Michael, Mcinnes, Caroline, Metastasio, Antonio, Milburn-McNulty, Philip, Mitchell, Clinton, Mitchell, Duncan, Morgans, Clare, Morris, Huw, Morrow, Jasper, Mubarak Mohamed, Ahmed, Mulvenna, Paula, Murphy, Louis, Namushi, Robert, Newman, Edward, Phillips, Wendy, Pinto, Ashwin, Price, David Ashley, Proschel, Harald, Quinn, Terry, Ramsey, Deborah, Roffe, Christine, Ross Russell, Amy, Samarasekera, Neshika, Sawcer, Stephen, Sayed, Walee, Sekaran, Lakshmanan, Serra-Mestres, Jordi, Snowdon, Victoria, Strike, Gayle, Sun, James, Tang, Christina, Vrana, Mark, Wade, Ryckie, Wharton, Chris, Wiblin, Lou, Boubriak, Iryna, Herman, Katie, Plant, Gordon, Varatharaj, Aravinthan, Thomas, Naomi, Ellul, Mark A, Davies, Nicholas W S, Pollak, Thomas A, Tenorio, Elizabeth L, Sultan, Mustafa, Easton, Ava, Breen, Gerome, Zandi, Michael, Coles, Jonathan P, Manji, Hadi, Al-Shahi Salman, Rustam, Menon, David K, Nicholson, Timothy R, Benjamin, Laura A, Carson, Alan, Smith, Craig, Turner, Martin R, Solomon, Tom, Kneen, Rachel, Pett, Sarah L, Galea, Ian, Thomas, Rhys H, and Michael, Benedict D

Published
2020
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6. Herpesvirus Infections and Childhood Arterial Ischemic Stroke

Author

Elkind, Mitchell SV, Hills, Nancy K, Glaser, Carol A, Lo, Warren D, Amlie-Lefond, Catherine, Dlamini, Nomazulu, Kneen, Rachel, Hod, Eldad A, Wintermark, Max, deVeber, Gabrielle A, and Fullerton, Heather J

Subjects
Medical Microbiology, Biomedical and Clinical Sciences, Prevention, Brain Disorders, Stroke, Sexually Transmitted Infections, Infectious Diseases, Aetiology, 2.2 Factors relating to the physical environment, Infection, Adolescent, Brain Ischemia, Case-Control Studies, Child, Child, Preschool, Female, Herpesviridae Infections, Humans, Infant, Infant, Newborn, Internationality, Male, Prospective Studies, child, herpesviridae infections, infection, pediatrics, stroke, VIPS Investigators*, Cardiorespiratory Medicine and Haematology, Clinical Sciences, Public Health and Health Services, Cardiovascular System & Hematology, Cardiovascular medicine and haematology, Clinical sciences, Sports science and exercise
Abstract

BackgroundEpidemiological studies demonstrate that childhood infections, including varicella zoster virus, are associated with an increased risk of arterial ischemic stroke (AIS). Other herpesviruses have been linked to childhood AIS in case reports. We sought to determine whether herpesvirus infections, which are potentially treatable, increase the risk of childhood AIS.Methods and resultsWe enrolled 326 centrally confirmed cases of AIS and 115 stroke-free controls with trauma (29 days to 18 years of age) with acute blood samples (≤3 weeks after stroke/trauma); cases had convalescent samples (7-28 days later) when feasible. Samples were tested by commercial enzyme-linked immunosorbent assay kits for immunoglobulin M/immunoglobulin G antibodies to herpes simplex virus 1 and 2, cytomegalovirus, Epstein-Barr virus, and varicella zoster virus. An algorithm developed a priori classified serological evidence of past and acute herpesvirus infection as dichotomous variables. The median (quartiles) age was 7.7 (3.1-14.3) years for cases and 10.7 (6.9-13.2) years for controls (P=0.03). Serological evidence of past infection did not differ between cases and controls. However, serological evidence of acute herpesvirus infection doubled the odds of childhood AIS, even after adjusting for age, race, and socioeconomic status (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P=0.007). Among 187 cases with acute and convalescent blood samples, 85 (45%) showed evidence of acute herpesvirus infection; herpes simplex virus 1 was found most often. Most infections were asymptomatic.ConclusionsHerpesviruses may act as a trigger for childhood AIS, even if the infection is subclinical. Antivirals like acyclovir might have a role in the prevention of recurrent stroke if further studies confirm a causal relationship.

Published
2016

7. Risk of Recurrent Arterial Ischemic Stroke in Childhood

Author

Fullerton, Heather J, Wintermark, Max, Hills, Nancy K, Dowling, Michael M, Tan, Marilyn, Rafay, Mubeen F, Elkind, Mitchell SV, Barkovich, A James, deVeber, Gabrielle A, Plumb, Patricia A, Benedict, Susan L, Bernard, Timothy J, Fox, Christine K, Friedman, Neil R, Lo, Warren D, Ichord, Rebecca N, Mackay, Mark T, Kirton, Adam, Hernandez-Chavez, Marta I, Humphreys, Peter, Jordan, Lori C, Sultan, Sally, Rivkin, Michael J, Titomanlio, Luigi, Kovacevic, Gordana S, Yager, Jerome Y, Amlie-Lefond, Catherine, Dlamini, Nomazulu, Condie, John, Yeh, Ann, Kneen, Rachel, Bjornson, Bruce, Pergami, Paola, Zou, Li Ping, Elbers, Jorina M, Abdalla, Abdalla, Chan, Anthony K, Farooq, Osman, Lim, Mingming J, Carpenter, Jessica L, Pavlakis, Steven, Wong, Virginia C, and Forsyth, Robert

Subjects
Paediatrics, Biomedical and Clinical Sciences, Brain Disorders, Pediatric, Stroke, Prevention, Neurosciences, Atherosclerosis, Adolescent, Brain Ischemia, Cerebral Arterial Diseases, Child, Child, Preschool, Cohort Studies, Female, Follow-Up Studies, Humans, Infant, Internationality, Male, Prospective Studies, Recurrence, Risk Factors, child, pediatrics, risk factor, stroke, vaccination, VIPS Investigators, Cardiorespiratory Medicine and Haematology, Clinical Sciences, Neurology & Neurosurgery, Clinical sciences, Allied health and rehabilitation science
Abstract

Background and purposePublished cohorts of children with arterial ischemic stroke (AIS) in the 1990s to early 2000s reported 5-year cumulative recurrence rates approaching 20%. Since then, utilization of antithrombotic agents for secondary stroke prevention in children has increased. We sought to determine rates and predictors of recurrent stroke in the current era.MethodsThe Vascular Effects of Infection in Pediatric Stroke (VIPS) study enrolled 355 children with AIS at 37 international centers from 2009 to 2014 and followed them prospectively for recurrent stroke. Index and recurrent strokes underwent central review and confirmation, as well as central classification of causes of stroke, including arteriopathies. Other predictors were measured via parental interview or chart review.ResultsOf the 355 children, 354 survived their acute index stroke, and 308 (87%) were treated with an antithrombotic medication. During a median follow-up of 2.0 years (interquartile range, 1.0-3.0), 40 children had a recurrent AIS, and none had a hemorrhagic stroke. The cumulative stroke recurrence rate was 6.8% (95% confidence interval, 4.6%-10%) at 1 month and 12% (8.5%-15%) at 1 year. The sole predictor of recurrence was the presence of an arteriopathy, which increased the risk of recurrence 5-fold when compared with an idiopathic AIS (hazard ratio, 5.0; 95% confidence interval, 1.8-14). The 1-year recurrence rate was 32% (95% confidence interval, 18%-51%) for moyamoya, 25% (12%-48%) for transient cerebral arteriopathy, and 19% (8.5%-40%) for arterial dissection.ConclusionsChildren with AIS, particularly those with arteriopathy, remain at high risk for recurrent AIS despite increased utilization of antithrombotic agents. Therapies directed at the arteriopathies themselves are needed.

Published
2016

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

Author

Abdel-mannan, Omar, Champsas, Dimitrios, Tur, Carmen, Lee, Vanessa, Manivannan, Sharmila, Usman, Haroon, Skippen, Alison, Desai, Ishita, Chitre, Manali, Forsyth, Rob, Kneen, Rachel, Ram, Dipak, Ramdas, Sithara, Rossor, Thomas, West, Siobhan, Wright, Sukhvir, Palace, Jacqueline, Wassmer, Evangeline, Hemingway, Cheryl, and J. Lim, Ming

Subjects
NEUROMYELITIS optica, BRAIN damage, DISEASE complications, DISEASE progression, MEDICAL care, POSTVACCINAL encephalitis
Published
2024
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11. Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course

Author

Abdel-mannan, Omar, primary, Champsas, Dimitrios, additional, Tur, Carmen, additional, Lee, Vanessa, additional, Manivannan, Sharmila, additional, Usman, Haroon, additional, Skippen, Alison, additional, Desai, Ishita, additional, Chitre, Manali, additional, Forsyth, Rob, additional, Kneen, Rachel, additional, Ram, Dipak, additional, Ramdas, Sithara, additional, Rossor, Thomas, additional, West, Siobhan, additional, Wright, Sukhvir, additional, Palace, Jacqueline, additional, Wassmer, Evangeline, additional, Hemingway, Cheryl, additional, Lim, Ming J, additional, Mankad, Kshitij, additional, Ciccarelli, Olga, additional, and Hacohen, Yael, additional

Published
2023
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12. Intravenous immunoglobulin treatment in childhood encephalitis (IgNiTE): a randomised controlled trial

Author

Hill, Matilda, primary, Iro, Mildred, additional, Sadarangani, Manish, additional, Absoud, Michael, additional, Cantrell, Liberty, additional, Chong, Kling, additional, Clark, Christopher, additional, Easton, Ava, additional, Gray, Victoria, additional, Kneen, Rachel, additional, Lim, Ming, additional, Liu, Xinxue, additional, Pike, Michael, additional, Solomon, Tom, additional, Vincent, Angela, additional, Willis, Louise, additional, Yu, Ly-Mee, additional, and Pollard, Andrew J, additional

Published
2023
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14. Do Early Relapses Predict the Risk of Long‐Term Relapsing Disease in an Adult and Paediatric Cohort with MOGAD?

Author

Chen, Bo, primary, Gomez‐Figueroa, Enrique, additional, Redenbaugh, Vyanka, additional, Francis, Anna, additional, Satukijchai, Chanjira, additional, Wu, Yan, additional, Messina, Silvia, additional, Sa, Mario, additional, Woodhall, Mark, additional, Robertson, Neil P., additional, Lim, Ming, additional, Wassmer, Evangeline, additional, Kneen, Rachel, additional, Huda, Saif, additional, Blain, Camilla, additional, Halfpenny, Christopher, additional, Hemingway, Cheryl, additional, O'Sullivan, Eoin, additional, Hobart, Jeremy, additional, Fisniku, Leonora K., additional, Martin, Roswell J., additional, Dobson, Ruth, additional, Cooper, Sarah A., additional, Williams, Victoria, additional, Waters, Patrick, additional, Chen, John J., additional, Pittock, Sean J., additional, Ramdas, Sithara, additional, Leite, Maria Isabel, additional, Flanagan, Eoin P., additional, Geraldes, Ruth, additional, and Palace, Jacqueline, additional

Published
2023
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34. Dynamic MRI Lesion Evolution in Pediatric Myelin-Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD) (S27.006)

Author

Abdel-Mannan, Omar, primary, Champsas, Dimitrios, additional, Manivannan, Sharmila, additional, Mankad, Kshitij, additional, Kneen, Rachel, additional, Chitre, Manali, additional, Wright, Sukhvir, additional, Wassmer, Evangeline, additional, Hemingway, Cheryl, additional, Rossor, Thomas, additional, Lim, Ming, additional, Ciccarelli, Olga, additional, and Hacohen, Yael, additional

Published
2023
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35. Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course

Author

Abdel-Mannan, Omar, Champsas, Dimitrios, Tur, Carmen, Lee, Vanessa, Manivannan, Sharmila, Usman, Haroon, Skippen, Alison, Desai, Ishita, Chitre, Manali, Forsyth, Rob, Kneen, Rachel, Ram, Dipak, Ramdas, Sithara, Rossor, Thomas, West, Siobhan, Wright, Sukhvir, Palace, Jacqueline, Wassmer, Evangeline, Hemingway, Cheryl, Lim, Ming J, Mankad, Kshitij, Ciccarelli, Olga, Hacohen, Yael, Abdel-Mannan, Omar, Champsas, Dimitrios, Tur, Carmen, Lee, Vanessa, Manivannan, Sharmila, Usman, Haroon, Skippen, Alison, Desai, Ishita, Chitre, Manali, Forsyth, Rob, Kneen, Rachel, Ram, Dipak, Ramdas, Sithara, Rossor, Thomas, West, Siobhan, Wright, Sukhvir, Palace, Jacqueline, Wassmer, Evangeline, Hemingway, Cheryl, Lim, Ming J, Mankad, Kshitij, Ciccarelli, Olga, and Hacohen, Yael

Abstract

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.

Published
2023

36. Neuroimaging in encephalitis: analysis of imaging findings and interobserver agreement

Author

Ambrose, Helen E., Clewley, Jonathan P., Walsh, Amanda L., Morgan, Dilys, Cunningham, Richard, Zuckerman, Mark, Mutton, Kenneth J., Ward, Katherine N., Lunn, Michael P.T., Irani, Sarosh R., Vincent, Angela, Ford, Craig, Rothwell, Emily, Tong, William, Lin, Jean-Pierre, Ahmed, Javeed, Cubitt, David, Hemingway, Cheryl, Muir, David, Lyall, Hermione, Thompson, Ed, Keir, Geoff, Worthington, Viki, Griffiths, Paul, Bennett, Susan, Kneen, Rachel, Klapper, Paul, Granerod, J., Davies, N.W.S., Mukonoweshuro, W., Mehta, A., Das, K., Lim, M., Solomon, T., Biswas, S., Rosella, L., Brown, D.W.G., and Crowcroft, N.S.

Published
2016
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37. Evolution of brain MRI lesions in paediatric myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and its relevance to disease course

Author

Abdel-mannan, Omar, Champsas, Dimitrios, Tur, Carmen, Lee, Vanessa, Manivannan, Sharmila, Usman, Haroon, Skippen, Alison, Desai, Ishita, Chitre, Manali, Forsyth, Rob, Kneen, Rachel, Ram, Dipak, Ramdas, Sithara, Rossor, Thomas, West, Siobhan, Wright, Sukhvir, Palace, Jacqueline, Wassmer, Evangeline, Hemingway, Cheryl, Lim, Ming J, Mankad, Kshitij, Ciccarelli, Olga, and Hacohen, Yael

Abstract

BackgroundLesion 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).ObjectiveWe aimed to evaluate brain MRI changes over time in paediatric MOGAD.MethodsRetrospective 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.Results200 children were included (MOGAD 97; MS 103). At first MRI post attack, new symptomatic and asymptomatic lesions were seen more often in MS versus MOGAD (52/103 vs 28/97; p=0.002 and 37/103 vs 11/97; p<0.001); 83% of patients with MOGAD showed at least one lesion’s resolution at first follow‐up scan, and 23% had normal MRI. Only 1 patient with MS had single lesion resolution; none had normal MRI. Disappearing lesions in MOGAD were seen in 40% after the second attack, 21% after third attack and none after the fourth attack.New lesions at first follow-up scan were associated with increased likelihood of relapse (p=0.02) and persistent MOG-Ab serostatus (p=0.0016) compared with 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).ConclusionsThese 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.

Published
2024
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38. Baló’s concentric sclerosis presenting asymptomatically in a child: clinico-radiologicalpathological correlation.

Author

Southin, Justine-Clair, Avula, Shivaram, Plessis, Daniel du, Kumar, Ram, and Kneen, Rachel

Abstract

We report a unique case of Baló’s concentric sclerosis presenting in a child who remained asymptomatic during a biphasic radiological evolution with expansion and resolution of the lesion without treatment. The advanced neuroimaging in this case demonstrated integrity of motor tracts and normal neuronal markers, concordant with the normal outcome. Immunohistochemistry showed features typical of a demyelinating disorder, including the predominant role for T lymphocytes, with a perivascular distribution but a notable B lymphocyte component. A literature review of published cases with combined histopathological and radiological information was also undertaken. [ABSTRACT FROM AUTHOR]

Published
2023
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40. Clinical features, course, and outcomes of a UK cohort of pediatric moyamoya

Author

Tho-Calvi, Sara C., Thompson, Dominic, Saunders, Dawn, Agrawal, Shakti, Basu, Anna, Chitre, Manali, Chow, Gabriel, Gibbon, Frances, Hart, Anthony, Tallur, Krishnaraya Kamath, Kirkham, Fenella, Kneen, Rachel, McCullagh, Helen, Mewasingh, Leena, Vassallo, Grace, Vijayakumar, Kayal, Wraige, Elizabeth, Yeo, Tong Hong, and Ganesan, Vijeya

Published
2018
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41. Clinical and molecular characterization of KCNT1-related severe early-onset epilepsy

Author

McTague, Amy, Nair, Umesh, Malhotra, Sony, Meyer, Esther, Trump, Natalie, Gazina, Elena V., Papandreou, Apostolos, Ngoh, Adeline, Ackermann, Sally, Ambegaonkar, Gautam, Appleton, Richard, Desurkar, Archana, Eltze, Christin, Kneen, Rachel, Kumar, Ajith V., Lascelles, Karine, Montgomery, Tara, Ramesh, Venkateswaran, Samanta, Rajib, Scott, Richard H., Tan, Jeen, Whitehouse, William, Poduri, Annapurna, Scheffer, Ingrid E., Chong, W.K. “Kling”, Cross, J. Helen, Topf, Maya, Petrou, Steven, and Kurian, Manju A.

Published
2018
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44. Clinical predictors of encephalitis in UK adults–A multi-centre prospective observational cohort study.

Author

Defres, Sylviane, Tharmaratnam, Kukatharmini, Michael, Benedict D., Ellul, Mark, Davies, Nicholas W. S., Easton, Ava, Griffiths, Michael J., Bhojak, Maneesh, Das, Kumar, Hardwick, Hayley, Cheyne, Chris, Kneen, Rachel, Medina-Lara, Antonieta, Salter, Anne Christine, Beeching, Nicholas J., Carrol, Enitan, Vincent, Angela, Garcia-Finana, Marta, and Solomon, Tom

Subjects
FEVER, ENCEPHALITIS, COHORT analysis, SCIENTIFIC observation, CEREBROSPINAL fluid, AUTOIMMUNE diseases
Abstract

Objectives: Encephalitis, brain inflammation and swelling, most often caused by an infection or the body's immune defences, can have devastating consequences, especially if diagnosed late. We looked for clinical predictors of different types of encephalitis to help clinicians consider earlier treatment. Methods: We conducted a multicentre prospective observational cohort study (ENCEPH-UK) of adults (> 16 years) with suspected encephalitis at 31 UK hospitals. We evaluated clinical features and investigated for infectious and autoimmune causes. Results: 341 patients were enrolled between December 2012 and December 2015 and followed up for 12 months. 233 had encephalitis, of whom 65 (28%) had HSV, 38 (16%) had confirmed or probable autoimmune encephalitis, and 87 (37%) had no cause found. The median time from admission to 1st dose of aciclovir for those with HSV was 14 hours (IQR 5–50); time to 1st dose of immunosuppressant for the autoimmune group was 125 hours (IQR 45–250). Compared to non-HSV encephalitis, patients with HSV more often had fever, lower serum sodium and lacked a rash. Those with probable or confirmed autoimmune encephalitis were more likely to be female, have abnormal movements, normal serum sodium levels and a cerebrospinal fluid white cell count < 20 cells x106/L, but they were less likely to have a febrile illness. Conclusions: Initiation of treatment for autoimmune encephalitis is delayed considerably compared with HSV encephalitis. Clinical features can help identify patients with autoimmune disease and could be used to initiate earlier presumptive therapy. [ABSTRACT FROM AUTHOR]

Published
2023
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45. Factors Associated With Relapse and Treatment of Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease in the United Kingdom

Author

Satukijchai, Chanjira, Mariano, Romina, Messina, Silvia, Sa, Mario, Woodhall, Mark R., Robertson, Neil P., Ming, Lim, Wassmer, Evangeline, Kneen, Rachel, Huda, Saif, Jacob, Anu, Blain, Camilla, Halfpenny, Christopher, Hemingway, Cheryl, O'Sullivan, Eoin, Hobart, Jeremy, Fisniku, Leonora K., Martin, Roswell, Dopson, Ruth, Cooper, Sarah A., Williams, Victoria, Waters, Patrick J., Ramdas, Sithara, Leite, Maria Isabel, Palace, Jacqueline, Satukijchai, Chanjira, Mariano, Romina, Messina, Silvia, Sa, Mario, Woodhall, Mark R., Robertson, Neil P., Ming, Lim, Wassmer, Evangeline, Kneen, Rachel, Huda, Saif, Jacob, Anu, Blain, Camilla, Halfpenny, Christopher, Hemingway, Cheryl, O'Sullivan, Eoin, Hobart, Jeremy, Fisniku, Leonora K., Martin, Roswell, Dopson, Ruth, Cooper, Sarah A., Williams, Victoria, Waters, Patrick J., Ramdas, Sithara, Leite, Maria Isabel, and Palace, Jacqueline

Abstract

Importance Longer-term outcomes and risk factors associated with myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD) are not well established. Objective To investigate longer-term risk of relapse and factors associated with this risk among patients with MOGAD. Design, Setting, and Participants This large, single-nation, prospective cohort study was conducted among 276 patients with MOGAD at 5 health care centers in the UK. Data from January 1973 to March 2020 were collected from 146 patients at Oxford and its outreach sites, 65 patients at Liverpool, 32 patients at a children’s hospital in Birmingham, 22 patients at a children’s hospital in London, and 11 patients at Cardiff, Wales. Data were analyzed from April through July 2020. Main Outcomes and Measures Risk of relapse and annualized relapse rate were evaluated according to different baseline features, including onset age, onset phenotype, and incident vs nonincident group, with the incident group defined as patients diagnosed with antibodies against myelin oligodendrocyte glycoprotein before a second attack. Time to next relapse among patients experiencing relapse was measured and compared between the maintenance therapy subgroup and each first-line treatment group. The no-treatment group was defined as the off-treatment phase among patients who were relapsing, which could occur between any attack or between the last attack and last follow-up. Results Among 276 patients with MOGAD, 183 patients were identified as being part of the incident group. There were no differences in mean (SD) onset age between total and incident groups (26.4 [17.6] years vs 28.2 [18.1] years), and female patients were predominant in both groups (166 [60.1%] female patients vs 106 [57.9%] female patients). The most common presentation overall was optic neuritis (ON) (119 patients among 275 patients with presentation data [43.3%]), while acute disseminated encephalomyelitis (ADEM), brain, or brainstem onset was predom

Published
2022

46. Clinical features, investigations, and outcomes of pediatric limbic encephalitis:A multicenter study

Author

Sabanathan, Saraswathy, Abdel-Mannan, Omar, Mankad, Kshitij, Siddiqui, Ata, Das, Krishna, Carr, Lucinda, Eltze, Christin, Eyre, Michael, Gadian, Jon, Hemingway, Cheryl, Kaliakatsos, Marios, Kneen, Rachel, Krishnakumar, Deepa, Lynch, Bryan, Parida, Amitav, Rossor, Thomas, Taylor, Micheal, Wassmer, Evangeline, Wright, Sukhvir, Lim, Ming, Hacohen, Yael, Sabanathan, Saraswathy, Abdel-Mannan, Omar, Mankad, Kshitij, Siddiqui, Ata, Das, Krishna, Carr, Lucinda, Eltze, Christin, Eyre, Michael, Gadian, Jon, Hemingway, Cheryl, Kaliakatsos, Marios, Kneen, Rachel, Krishnakumar, Deepa, Lynch, Bryan, Parida, Amitav, Rossor, Thomas, Taylor, Micheal, Wassmer, Evangeline, Wright, Sukhvir, Lim, Ming, and Hacohen, Yael

Abstract

Objectives: To describe the clinical presentation, investigations, management, and disease course in pediatric autoimmune limbic encephalitis (LE). Methods: In this retrospective observational study, from the UK Childhood Neuroinflammatory Disease network, we identified children from six tertiary centers with LE <18 years old between 2008 and 2021. Clinical and paraclinical data were retrieved from medical records. Results: Twenty-five children fulfilling LE criteria were identified, with median age of 11 years (IQR 8, 14) and median follow-up of 24 months (IQR 18, 48). All children presented with seizures; 15/25 (60%) were admitted to intensive care. Neuroimaging demonstrated asymmetric mesial temporal changes in 8/25 (32%), and extra-limbic changes with claustrum involvement in 9/25 (38%). None were positive for LGI1/CASPR2 antibodies (Abs), 2/25 were positive for serum anti-NMDAR Abs, and 2/15 positive for anti-Hu Abs; one died from relapsing neuroblastoma. Two children had serum and CSF anti-GAD antibodies. Initial immune therapy included steroids in 23/25 (92%), intravenous immunoglobulin (IVIg) in 14/25 (56%), and plasma exchange in 7/25 (28%). The commonest second-line treatment was rituximab in 15/25 (60%). Median duration of hospital admission was 21 days (IQR 11, 30). At last follow-up, 13/25 (52%) had refractory seizures and 16/25 (64%) had memory impairment. Six children (24%) had modified Rankin Scale (mRS) scores ≥3. There was no significant difference in mRS, or long-term cognitive and epilepsy outcomes in those who received rituximab versus those who did not. Interpretation: A diagnosis of autoimmune LE was associated with significant morbidity and adverse outcomes in this pediatric cohort.

Published
2022

49. Factors Associated With Relapse and Treatment of Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disease in the United Kingdom

Author

Satukijchai, Chanjira, primary, Mariano, Romina, additional, Messina, Silvia, additional, Sa, Mario, additional, Woodhall, Mark R., additional, Robertson, Neil P., additional, Ming, Lim, additional, Wassmer, Evangeline, additional, Kneen, Rachel, additional, Huda, Saif, additional, Jacob, Anu, additional, Blain, Camilla, additional, Halfpenny, Christopher, additional, Hemingway, Cheryl, additional, O'Sullivan, Eoin, additional, Hobart, Jeremy, additional, Fisniku, Leonora K., additional, Martin, Roswell, additional, Dopson, Ruth, additional, Cooper, Sarah A., additional, Williams, Victoria, additional, Waters, Patrick J., additional, Ramdas, Sithara, additional, Leite, Maria Isabel, additional, and Palace, Jacqueline, additional

Published
2022
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