More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma Uğur Demirsoy 1 , Burcu Alparslan 2 , Mehmet Celal Şen 3 , Yonca Anık 2 , Gür Akansel 2 , Gözde Görür 4 , Bora Gürel 5 , Görkem Aksu 6 , Funda Çorapcıoğlu 1 Departments of 1 Pediatric Oncology, 2 Radiology, 3 Pediatric Surgery, 4 Nuclear Medicine, 5 Pathology and 6 Radiation Oncology, Kocaeli University Faculty of Medicine, Kocaeli, Turkey. E-mail: udemirsoy@yahoo.com Received: 2nd January 2018, Accepted: 27th January 2018 SUMMARY: Demirsoy U, Alparslan B, Şen MC, Anık Y, Akansel G, Görür G, Gürel B, Aksu G, Çorapçıoğlu F. More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma. Turk J Pediatr 2019; 61: 139-141. Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported paraneoplastic components in pediatric HL. In this report, we present an 11-year-old girl who had all these three paraneoplastic components synchronously before HL was identified. Key words: achalasia, Hodgkin lymphoma, Holmes-Adie pupil, limbic encephalitis, paraneoplastic syndrome. Hodgkin lymphoma (HL), with prominent advances on the way toward identification of both pathogenesis and biology of the disease, is still not totally understood. Paraneoplastic syndromes (PNS) in HL can show up with various symptoms and different organ findings and some of them may regress after treatment of HL. 1 Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported as paraneoplastic components of pediatric HL. 2,3 Hodgkin lymphoma with accompanying limbic encephalitis is named ‘Ophelia syndrome’. Herein, we report a child who had achalasia first, developed several neurological symptoms afterwards and finally was diagnosed with HL. Case Report An eleven-year-old girl was referred in our pediatric outpatient clinic with complaints of loss of balance with laughter, dizziness, excessive sleeping, behavioral changes and bilateral low eye lids. She had a history of difficulty in swallowing both fluids and solid food that occurred 12 months before which ended up with diagnosis of achalasia (Fig. 1). Heller myotomy procedure was performed in another hospital 4 months after diagnosis but her symptoms persisted despite surgery. A few weeks after surgery, she started to sleep for more than 12 hours at night, only woke up for meals and slept excessively at school even during school breaks at daytime. She stopped communicating with other children and playing games. She was evaluated by a pediatric psychiatrist but no specific diagnosis was made. She also had ocular accommodative symptoms and blurry vision while looking at close up objects for the last few months and was diagnosed with Holmes-Adie pupil after ophthalmologic examination (bilateral dilated pupils unresponsive to light stimulation and proven increased sensitivity response to diluted pilocarpine). She was prescribed corrective eye-glasses. On physical examination, she was conscious and cooperative with normal motor functions. Auscultation revealed normal lung and heart sounds and there was no hepatosplenomegaly. All cranial nerves were functional and no pyramidal, cerebellar pathology was recognized. There was bilateral ptosis. She had multiple non-tender, enlarged lymph nodes (maximum diameter: 3 cm) on right anterior, infracervical and submandibular region. Magnetic resonance imaging (MRI) of the neck revealed a significant conglomeration of lymphadenopathies on the The Turkish Journal of Pediatrics 2019; 61: 139-141 Case Report DOI: 10.24953/turkjped.2019.01.024