1. Fluid-refractory orthostatic hypotension in preteen female Christine Hrach, MD Pediatric Hospitalist

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3. Case Presentation AF is a 12 y.o. female presenting with 5 days of malaise, emesis, weakness, intermittent diaphoresis She endorsed rigors, neck pain, and a burning, pruritic rash to neck and arms 11 pound weight loss in the previous 3 weeks Tmax of 100-101 Initially evaluated in the ER, found to be monospot positive, received 20 ml/kg NS Bolus and admitted for dehydration

4. Admission exam Initial exam: – Vital signs: Wt 44kg (50%), T 37.2, P 76, R 24, BP 131/74 – Neck stiffness – Report of diffuse weakness, but 5/5 strength on exam – Symmetric mild hyperreflexia, 3+ brachial and patellar – Excoriations above L clavicle, and macular erythematous rash in distribution of t-shirt collar

5. Hospital course Admitted for four days Received iv fluids and Zofran Neck stiffness resolved with NSAIDs Lab findings: wbc 9.2, hgb 16.5, plt 411, 78% N/11% L – Ua: SG 1.030, 2+ ketones – Normal CMP, mg, and phos (BUN = 22) – Normal TSH and free T4 AF’s emesis and oral intake gradually improved PT consult done – exercise regimen established for home while she was recovering from mono Discharged to home

6. Return to ER AF presented again to the ER 4 days after discharge with decreased po intake and persistent NBNB emesis Now a 15 pound weight loss New symptoms of dizziness, blurry/ tunnel vision, “seeing black” when sitting, feeling faint upon standing, and unable to ambulate without assistance Difficulty initiating micturition, urinating only 2-3 times a day

7. ER course Lab analysis – Prior EBV titers reviewed: IgM negative IgG reactive – CMV non-reactive – Na – 131, BUN – 20 – Wbc 6.8, hgb 14.9, plts 404, 58% N/ 27%L EKG = normal sinus rhythm Received 20 ml/kg NS bolus, admitted to the floor

8. Additional history Visual changes with sitting or standing, black spots Pruritus and rash to upper chest Uncomfortable sensations of burning or itching to bilateral upper extremities involving thumbs and radial aspects of hands and forearm Constipation – last BM 11 days prior to admission Hiccups – up to one hour at times, 2-3 times a day

9. Vital signs On admissionAfter 2 nd BolusFollowing AM Lying BP111/6894/70105/68 Lying Pulse746658 Sitting BP83/5784/6092/55 Sitting Pulse96 88 Standing BP69/5356/3869/42 Standing Pulse119106110

10. Admission exam Significant orthostatic hypotension Ill-appearing, thin, pale CV – RRR without murmur Neuro exam: – 4/5 strength in upper and lower extremities – 3-4+ DTR’s throughout – Required assistance with ambulation

11. Differential Diagnosis Summary – 12 year old female with dysaesthesias, weakness, hyperreflexia, severe orthostatic hypotension, fifteen pound weight loss, and urinary retention Infectious Malignancy Neurologic Eating disorder

12. Hospital course Neurology consultation – concern for a cervical spine lesion MRI/MRA ordered of brain and MRI with and without contrast of cervical, thoracic, and lumbar spine Echo obtained: normal segmental anatomy and normal cardiac structure

14. MRI Results Bright T2 signal within the R optic nerve Diffuse swelling of the cord with hyperintense T2 signal extending from C1 to T3-4 level Cord swelling is most significant at the cervical and upper thoracic level Most likely represents neuromyelitis optica Less likely considerations include transverse myelitis, viral/postviral myelitis

15. Hospital course LP done: cell count 84, elevated protein 72.9, glucose 47 Transferred to PICU for emergent plasmapheresis Laboratory data: positive serum and CSF NMO antibodies Completed 7 cycles of plasmapheresis and a 5-day steroid burst followed by a one-month steroid wean Ophthalmology consulted – normal eye exam Rituximab infusion given for immunosuppression, plans to continue infusions as an outpatient

16. NMO Neuromyelitis optica A relapsing demyelinating disorder, distinct from multiple sclerosis Predilection for the optic nerves and spinal cord NMO-IgG is an antibody that seems to be present in about 70% of those with NMO (not found in MS) Usual presenting symptoms include visual impairment, weakness and gait disturbance

17. NMO Treatment guidelines are based on consensus opinion and retrospective studies Treatment usually consists of a combination of intravenous corticosteroids and plasma exchange Rituximab is a second-line agent 3 aspects to treatment – Relapse treatment – Relapse prevention – Symptom management and rehabilitation

18. Conclusion Orthostasis is commonly attributed to dehydration in children Orthostasis out of proportion to history of fluid loss or the apparent degree of dehydration on exam should prompt consideration for other causes Spinal cord pathology should be in the differential diagnosis as it may present with autonomic dysfunction

19. Thank you Co-authors on this case: – Kim Hamlin, MD – Liza Harrison, MD

20. Questions??