Isolated CNS Hemophagocytic Lymphohistiocytosis

Topic: Isolated CNS hemophagocytic lymphohistiocytosis;

Talk By: Dr. Maha Z. Mohammed ( Speaker ), Prof. Hoda Tomoum ( Expert ), Dr. Sonali Singh ( Moderator );

When: October 21, 2023, at 9:00 AM Eastern Time ( US / Canada )

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Dr. Maha Mohammed is an associate professor of Pediatric Neurology at Ain Shams University Cairo, Egypt. She has ten years of experience in Pediatric Neurology. She has more than 20 publications in national and international indexed journals. She has been a co-investigator in a multicenter international project on Pediatric stroke, "International Prevalence and Mechanisms of SARS-CoV-2 in Childhood Arterial Ischemic Stroke During the COVID-19 Pandemic." (2022): Stroke, DOI: 10.1161/STROKEAHA.121.038250. She is a Post Doc Fellow at the National Institute of Neurological Disorders and Stroke (NINDS), National Institute of Health (NIH), United States of America (USA) (November 2019- May 2020). Her areas of interest are Pediatric stroke, neurogenetics, and neuromuscular disorders.

Professor Hoda Tomoum is a professor of Pediatric Neurology at Ain Shams University Cairo, Egypt. Prof. Tomoum completed her master's degree in child neurology at Tor Vergata University in Rome in February 2010. Prof. Tomoum has more than 50 publications in national and international indexed journals. She has reviewed several scientific articles in the Journal of Pediatric Neurology and Epilepsia (The official journal of the International League Against Epilepsy). She is a member of the International Child Neurology Association (ICNA). Her areas of interest are Pediatric Epilepsy and Neuro-genetic Disorders.

Dr. Sonali Singh is a Pediatric Neurologist from India, having completed her DM (Pediatric Neurology) from AIIMS, New Delhi. She is an executive member of the Future Leaders of the International Child Neurology Association (FLICNA). She has over 15 national and international indexed journals. She was a co-investigator in the multicenter efficacy and safety study of ataluren in patients with nonsense mutation Duchenne Muscular Dystrophy and open-label extension. Her areas of interest are Pediatric Epilepsy and Neuro-muscular Disorders. Currently, She is a fellow in Epilepsy at SickKids, Toronto.

CNS hemophagocytic lymphohistiocytosis (CNS-HLH) is a recently recognizable entity of neuroinflammation. The genes most commonly mutated in Familial hemophagocytic lymphohistiocytosis (FHL) are PRF1 (FHL2) and UNC13D (FHL3). Although ordinary at diagnosis of systemic HLH, CNS manifestations rarely precede systemic findings. Several patients experienced delayed diagnosis of isolated CNS HLH; Patients with PRF1 mutations have been reported with isolated CNS inflammation for weeks to months before therapy initiation. Hematopoietic cell transplantation (HCT) is the only definitive cure for FHL, and it is effective in achieving or maintaining disease remission in patients with CNS-FHL. The diagnosis requires a high index of suspicion considering specific neuroimaging characterization, mainly because of rapidly progressive disease and lack of response to lines of therapy of neuroinflammatory disorders.

By attending this session, you will be able to learn about the following points:

• To highlight a treatable syndrome, isolated CNS HLH.
• To identify presenting symptoms and signs of CNS HLH.
• To recognize MRI findings suggestive of CNS HLH.

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