Abstract
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with predilection for early childhood. Delayed onset of ADEM is rare, and herein we present a previously healthy 5-year-old boy, with an unusual clinical course of ADEM with high intracranial pressure (ICP) and acute visual loss that was at first diagnosed as idiopathic intracranial hypertension without papilledema (IIHWOP). The boy underwent acute neurosurgical intervention with ventriculoperitoneal (VP) shunt using Miethke valve and sensor reservoir system and received high-dose steroid treatment with symptom relieve within days. This is the first case report using this system in such a young child, and we find it feasible and valuable also in younger children when VP shunt with ICP measurement is indicated.
Highlights
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with predilection for early childhood
Diagnosis is made by a combination of clinical and magnetic resonance imaging (MRI) findings of the brain and medulla, and provided other etiologies of inflammatory and infectious causes have been excluded [1, 2]
Severe presentations of ADEM resulting in admission to intensive care units have been described [3]; alleviation of very high intracranial pressure (ICP) with neurosurgical interventions such as hemicraniectomy or VP shunt are limited to case reports [4, 5]
Summary
Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with predilection for early childhood. A lumbar puncture (LP) demonstrated an elevated opening pressure of 39 cm H2O and slightly elevated white cell count (2 poly, 8 mono) in the cerebrospinal fluid (CSF). He was diagnosed with idiopathic intracranial hypertension without. One month after initiation of acetazolamide treatment, his medical condition deteriorated rapidly He became encephalopathic within 24 h and experienced worsening of headache, nausea, and impaired vision. An acute CT scan showed signs of high ICP At this stage, he was transferred to Uppsala University Hospital, a tertiary referral center for children and adolescents in need of neurosurgical and pediatric neurology care and treatment. Neuroinflammation (CSF) CXCL13 NMO-ab (aquaporin4-ab) MOG-ab Anti-neuronal ab CSF Glucose Albumin Lactate White blood cells IgG index Oligoclonal bands Infectious Toxoplasmosis Meningoencephalitis block* TBE and Lyme Parvo Rubella Other CBC Creatinine ASAT, ALAT TFT ACE
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
