Steroid-Responsive Encephalopathy, Dropped Head Syndrome, and Hypertension in a Toddler: Is There a Clue? - 19/03/16

Doi : 10.1016/j.pediatrneurol.2016.01.005

Arushi Gahlot Saini, MD, DM (Pediatric Neurology) ^a, Jitendra Kumar Sahu, MD, DM (Pediatric Neurology) ^a, Saptharishi Lalgudi Ganeshan, MD, DM (Pediatric Critical Care) ^a, Kushaljit Singh Sodhi, MD (Radiodiagnosis) ^b, Katragadda Laxmi Narayan Rao, MS, FACS, FAMS, FICS ^c, Pratibha Singhi, MD (Pediatrics), FIAP, FNAMS ^a,^⁎
^a Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
^b Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
^c Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

^∗Communications should be addressed to: Dr. Singhi; Head, Department of Pediatrics; Chief Pediatric Neurology and Neurodevelopment Unit; Advanced Pediatrics Centre; Postgraduate Institute of Medical Education and Research; Madhya Marg, Sector 12; Chandigarh 160012; India.Department of PediatricsChief Pediatric Neurology and Neurodevelopment UnitAdvanced Pediatrics CentrePostgraduate Institute of Medical Education and ResearchMadhya Marg, Sector 12Chandigarh160012India

Abstract

Background

Paraneoplastic manifestations may provide an early clue to underlying malignancies in children.

Methods

We describe a 3-year-old girl with an unusual neurological paraneoplastic syndrome of steroid-responsive encephalopathy and dropped head syndrome associated with paraspinal neuroblastoma.

Results

Cranial neuroimaging, metabolic screen, and thyroid functions were normal. Abdominal imaging identified a paraspinal neuroblastoma. Encephalopathy and neck extensor weakness responded to pulse corticosteroids followed by tumor resection.

Conclusions

This is a unique child with paraneoplastic steroid-responsive encephalopathy and dropped head syndrome in association with neuroblastoma.

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Keywords : neuroblastoma, encephalopathy, dropped head syndrome, paraneoplastic, cardiomyopathy

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Vol 57

P. 95-97 - avril 2016 Retour au numéro

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Steroid-Responsive Encephalopathy, Dropped Head Syndrome, and Hypertension in a Toddler: Is There a Clue? - 19/03/16

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