1. Case Report Our patient is a 16 y/o Latin American girl referred from an outside hospital for symptoms of left flank pain radiating to the left lower leg for several days. She also has tingling and numbness in bilateral lower extremities extending from knees to the soles with intermittent spasms for 2 months causing difficulty maintaining balance. She has been having urinary hesitancy for the past few days, but no hematuria or dysuria. Her symptoms started about 3 years ago when she experienced diminishing vision first in the right eye and a week later in the left eye. She did not have any spinal cord related symptoms. She was seen by a neuro-opthalmologist in another facility who did a lumbar puncture and treated her with a course of IV methylprednisolone. Due to financial constraints she did not receive further medical care thereafter. Her symptoms continued to progress and she had completely lost her vision bilaterally at the time of presentation to our facility. No family history of multiple sclerosis, or other autoimmune diseases. She denies recent travel, tick exposure, skin lesions or fever. Physical Findings: Vital Signs: normal Pupils: dilated bilaterally and non-reactive to direct and consensual light stimuli. Fundus: very pale, normal sized optic disc bilaterally with normal maculae and vessels. Extraocular movements: intact. Neurological: Normal mental status and Cranial Nerves exam except for CN II. Strength and Tone: 5/5 both UE and LE. Sensation: temperature decreased in lower extremity bilaterally. DTR’s: UE: normal. LE: Knee: trace; Ankle: 1+ Babinski sign: positive bilaterally. Gait: normal; Cerebellar signs: negative Other systems: normal. Conclusion & Take Home Points References 1.Tillema JM, McKeon A. The spectrum of neuromyelitis optica (NMO) in childhood. J Child Neurol. 2012;27:1437-1447. 2.Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinsehnker BG. Revised diagnostic criteria for Neuromyelitis optica. Neurology. 2006;66:1485-9. 3.Wingerchuk DM, Lennon VA, Luchcinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6:805-15. 4.Maras H, Kara B, Anik Y. Seropositive Neuromyelitis Optica: A Pediatric Case Report and 6-Year Follow up. Pediatric Neurology.2013 Sep;49(3):198-202. 5. Lotze T.E., Northrop J.L., et al: Spectrum of pediatric Neuromyelitis optica. Pediatrics 2008; 122(5):e1039-e1047. 6. Jiwon Oh and Michael Levy, “Neuromyelitis Optica: An Antibody- Mediated Disorder of the Central Nervous System,” Neurology Research International, vol. 2012, Article ID 460825, 13 pages, 2012. doi:10.1155/2012/460825 7.Selner J, Boggild M, Clanet M. EFNS Guideline on Diagnosis and Management of Neuromyelitis Optica. Eur J Neurology. 2010 Aug; 17(8):1019-32. Texas Pediatric Society Electronic Poster Contest WBC : 8.1x10 3 cells/µL; H/H: 13.1gm/dL/39%; Platelets : 278,000/dL. CSF : Clear, Glu: 65mg/dL; Protein : 38 mg/dL; Lactic acid : 2.5 mg/dl (↑); CSF Multiple sclerosis Profile- IgG : 2.8mg/dL; Albumin : 21mg/dL; IgG/Albumin ratio : 0.13; Oligoclonal bands : +ve. CSF Myelin Basic Protein: 2.5 ng/ml (↑); CSF angiotensin converting enzyme : 0.9 U/L Serum NMO/AQP 4 : IgG : 59.5 U/ml (↑) MRI Brain: Normal MRI spine: see Fig 1. and 2. below Diagnostic Results Discussion NMO is a relatively rare demyelinating disease of the CNS that typically begins in adulthood and published data on patients of NMO beginning in childhood and adolescents is limited. Tillema et al., reported that before the age of 18 there is clear female preponderance (8: 1 to 9:1) and poorer prognosis [1]. It can occur a single attack extending over a month or two, or a more common relapsing form in which there are multiple attacks. Bilateral simultaneous Optic neuritis is a hallmark of NMO [6]. Unlike Multiple sclerosis, other autoimmune disorders, including SLE, type I diabetes, juvenile idiopathic arthritis and Graves' disease, occur in 42–66 % of children with NMO [5]. NMO has to be differentiated from other CNS disorders like : Multiple sclerosis, Acute demyelinating encephalomyelitis (ADEM), Transverse myelitis, Sarcoidosis, SLE, Behcet’s disease, Spinal Cord tumors. To establish laboratory diagnosis, detection of NMO-IgG autoantibody in the serum is present in greater than 70% of those with NMO. This marker has a sensitivity of 76% and specificity of 94% which helps it distinguish from other demyelinating disorders [2,3]. Normal brain MRI is a common finding at the onset of NMO but follow up scans are to be performed to look for asymptomatic lesions [4] It is important to recognize that there are currently no FDA approved medications for NMO. Use of pulse dose steroids during acute attacks is generally recommended and is effective in upto 80% to abort symptoms [3]. Plasmapharesis is used for those with severe myelitis who fail to improve after corticosteroid treatment [Level C recommendation][4]. It is crucial to differentiate NMO from Multiple sclerosis as recent studies show that IFN-β which are used in MS may exacerbate relapses in NMO patients [3]. For those with relapsing attacks various immunomodulators eg. Azathioprine, Rituximab, Mycophenolate Mofetil have been used. They are suggested in seropositive patients after first attack and in polyphasic seronegative patients to prevent future attacks [7]. Within 5 years of disease onset, > 50% with relapsing NMO are blind in one or both eyes [2]. About 31% go on to develop monoplegia or paraplegia. The 5 year survival of patients with paraplegia is approximately 90%. NMO vs. MS NMOMS Areas of involvement Restricted to optic nerves & spinal cord Any white matter track Head MRIUsually non-specific or normalMultiple periventricular white matter lesions Spinal Cord MRILongitudinal extensive central necrotic lesions Multiple small peripheral lesions CSF Pleocytosis during attacks (> 50 white cells) Rarely > 25 cells Oligoclonal bands usually absent, but present in about 20-30% pts. Usually present Permanent disability Usually attack-relatedUsually in late progressive phase Female patients80-90%60-70% Anti-NMO ab/Aquaporin 4 PresentAbsent We describe a case of a 16 year old girl who was not treated appropriately during the first presentation of optic neuritis which led to complete vision loss. Timely diagnosis, management and follow up could have helped preserve her vision. We conclude that NMO IgG be checked in patients with optic neuritis, longitudinally extensive transverse myelitis, recurrent optic neuritis. This serum marker was elevated in our patient as well. Also consider doing brain and spinal MRI for every patient with optic neuritis to look for asymptomatic lesions. The distinction between NMO and MS is very important for treatment and prognosis. Although there are currently no well established therapeutic options many centers around USA are holding trials to find evidence based guidelines. Figure 1: MRI of Cervical spine: Ill- defined enhancing intramedullary lesion from C2-C3 of 4.4x15mm with mild cord enlargement with an associated syrinx beginning at C5-C6 and extending distally without definite enlargement of cord. Figure 2: MRI of Thoraco- lumbarspine: Ill-defined enhancing lesions from T3-T7 with evidence of a syrinx or intramural cysts from T7 to T10- T11. Lumbar spine is normal (not shown here). Abstract Abstract Neuromyelitis Optica is an autoimmune disorder with characteristic symptoms of transverse myelitis and optic neuritis. A 16 year old girl presented to our hospital with sensory-motor deficits and visual impairment. MRI of the spinal cord revealed white matter lesions extending over several contiguous vertebral segments. Serum marker for Devic’s disease (Aquaporin-4 Antibody) was positive. Her symptoms started about 3 years ago and progressed rapidly to complete blindness despite the initial treatment with pulse dose of IV methylprednisolone. This disease progression is very different from other CNS demyelinating diseases such as multiple sclerosis. We present this unique case to increase awareness of Devic’s disease and hope that early diagnosis and more aggressive immunomodulation may prevent the tragic outcome of complete blindness. Results Hospital Stay: After MRI brain/spine and lumbar puncture was performed and she was treated with a full course of IV methylprednisolone. This led to improvement in paresthesia and urinary hesitancy. However, this treatment did not improve her vision. She was discharged home on oral steroid taper for 20 days. She remained in stable condition with no new symptoms at 3 months follow-up. However, she had another relapsing course of sensory deficit requiring hospital admission for steroids during 15 month follow-up. Due to parental refusal to do a trial of Rituximab, she was not prescribed further immunomodulation therapy. Introduction Neuromyelitis Optica (Devic’s disease) is a rare, yet potentially devastating demyelinating autoimmune disorder, involving both spinal cord and optic nerves. The relapsing course of optic nerve demyelination may progress to severe visual impairment. We present a patient who, despite an initial treatment of IV methylprednisolone, suffered complete blindness due to lack of follow-up care. This case indicated that early diagnosis and continuing treatment maybe needed in patients with Devic’s disease. All are required: Optic Neuritis Acute Myelitis At least 2 of 3 supportive criteria: 1)Contiguous spinal cord MRI lesion extending over 3 vertebral segments. 2)Brain MRI not meeting diagnostic criteria for multiple sclerosis 3)Seropositive status for Aquaporin-4/NMO IgG Diagnostic Criteria for NMO: Revised in 2013