DIFFERENCES AND SIMILARITIES BETWEEN IDIOPATHIC INTRACRANIAL HYPERTENSION, OCULAR MYASTHENIA GRAVIS, OPTIC NEURITIS, AND HORNER’S IN CHILDREN VS. ADULTS Grant T. Liu, MD University of Pennsylvania School of Medicine Philadelphia, PA LEARNING OBJECTIVE IDIOPATHIC INTRACRANIAL HYPERTENSION (IIH) Understand the differences and similarities between children Similarities between children and adults with IIH. and adults with common neuro-ophthalmic problems • Diagnosis is made similarly (papilledema, normal imaging, CME QUESTIONS: TRUE/FALSE elevated opening pressure on lumbar puncture) • Morbidity (vision loss) 1. Like adults, children with IIH tend to be obese and female. • Treatment algorithms (acetazolamide, weight loss, optic 2. White matter lesions on MRI at presentation are predictive nerve sheath fenestration/shunting when vision loss is of conversion to MS in children with optic neuritis. severe or progressive despite maximal medical therapy) (Rangwala and Liu, 2007). 3. Carotid dissection is a primary consideration in children • Postpubertal patients tend to be female and with Horner’s syndrome. overweight (Balcer et al. 1999). Differences between children and adults with IIH. KEYWORDS 1. Idiopathic Intracranial Hypertension (Pseudotumor • In prepubescent children, more thin children, boys, and Cerebri) asymptomatic presentations. Fewer with headaches as well. (Balcer et al. 1999; Rangwala and Liu, 2007) 2. Pediatric Optic Neuritis • More sixth nerve palsies 3. Ocular Myasthenia Gravis • Young age makes following with computerized visual field testing difficult 4. Horner’s Syndrome • More cases associated with medication use (tetracycline derivatives, synthetic growth hormone, for instance) (Ko and Liu, 2010) INTRODUCTION • MRI-v recommended in addition to MRI in all cases for Pediatric neuro-ophthalmology is more than just neuro- diagnosis (Rangwala and Liu, 2007) ophthalmology of little people. Compared with neuro- • Definition of elevated opening pressure varies (280 mm ophthalmology in adults, in the pediatric subspecialty the H20 vs. 250 mm H20) (Avery et al. 2010) diseases are different, with greater emphases on congenital • Better visual prognosis (Soiberman et al. 2011) malformations and genetic disorders and less on vascular problems. The approach also varies, particularly with OCULAR MYASTHENIA GRAVIS (OMG) regard to examination techniques and interaction with the parents. In many instances, it is still unclear from a Similarities between children and adults with OMG. physiologic standpoint why such striking differences exist between disease profiles in adults and children. • Juvenile and adult myasthenia gravis are both autoimmune disorders The purpose of this talk is to highlight the similarities and • Presentation with ptosis, strabismus, and/or differences between adults and children with four common ophthalmoplegia (Kim et al. 2003) neuro-ophthalmic problems. • Diagnosis with acetylcholine receptor antibody testing • Use of ice test or rest test • Treatment options include pyridostigmine, prednisone, immunosuppression, and thymectomy. 2012 Annual Meeting Syllabus | 95 Differences between children and adults with OMG. one or more white matter lesions on MRI were more likely to develop MS (3/7 vs. 0/11, p=0.04, Fisher’s • Other forms to consider in infancy: neonatal exact test). We concluded that children with brain myasthenia gravis MRI abnormalities at the time of the diagnosis of optic • Use of the edrophonium test, repetitive stimulation, or neuritis have an increased risk of MS. single fiber EMG may not be possible in some children because of lack of cooperation. Differences between children and adults with optic • Therefore when the acetylcholine receptor antibody neuritis. testing is normal, the diagnosis of ocular myasthenia gravis in a child may lack confirmatory testing. • Visual acuities at presentation may be worse • Amblyopia due to ptosis (deprivational) and ocular (Bonhomme et al. 2009) misalignment (strabismic) make aggressive treatment more • More bilateral optic neuritis and optic neuritis with of a priority (Ortiz and Borchert 2008; Pineles et al. 2010) optic disc edema • Thymectomy in younger children can be performed • More optic neuritis in association with intercurrent illness transthorascopically rather than transcervically or • Presentation as ADEM, a demyelinating or inflammatory trans-sternally. event, and includes white or gray matter lesions on • Thymoma rare MRI, which is i) polysymptomatic and ii) includes • In our series (Pineles et al. 2010), the development encephalopathy (i.e. behavioral or mental status of generalized symptoms (23%) was lower than early change) (Krupp et al., 2007). Acute disseminated case series of pediatric OMG (36-43%) (Mullaney et al. encephalomyelitis (ADEM) is an autoimmune 2003; McCreery et al. 2002), and that of adult OMG demyelinating disease that typically follows an illness (31-49%) (Bever et al. 1983; Sommer et al. 1997). or vaccination. As opposed to MS, ADEM is typically These rates corroborate the notion that development a monophasic illness that does not require long-term of generalized symptoms may be less common in treatment. ADEM is more common in children than pediatric OMG than in the adult population. adults (Dale et al. 2000). Although patients with ADEM can present with fulminate neurologic signs and OPTIC NEURITIS (ON) symptoms, most patients have an excellent recovery. • More recurrent optic neuritis in children. In our study Similarities between children and adults with optic neuritis. of pediatric optic neuritis (Bonhomme et al. 2009), nine patients (31%) had relapses of optic neuritis during the • Clinically, optic neuritis in the pediatric age group is study period and 5 had more than one relapse. diagnosed by the same criteria used in adults, including • Neuromyelitis optica (NMO), although seen, is less sudden or subacute visual loss, central or cecocentral common in children. visual field defect, impairment of color vision, afferent • Older age is a risk factor for development of MS. pupillary defect, and ocular pain on eye movements. (Waldman et al., in press). • Often the initial manifestation of multiple sclerosis (MS). • Postpubertal patients tend to be female and have a According to the ONTT, after acute unilateral optic neuritis, presentation (unilateral without disc swelling) and adults have a 50% chance of developing MS within 15 years course similar to adults. (Optic Neuritis Study Group 2008). Children with optic neuritis are also at risk for development of MS (29%, meta- HORNER’s SYNDROME analysis) (Waldman et al., in press). • White matter lesions on MRI are predictive of Similarities between children and adults with Horner’s conversion to MS. As established by the Optic Neuritis syndrome. Treatment Trial, an abnormal baseline brain MRI with white matter lesions is a strong predictor of MS after • Presentation with ptosis, miosis, and anhidrosis isolated optic neuritis in adults. Fifteen years after • Neuro-anatomy is the same. Consideration of lesions a bout of optic neuritis, 72% of adults with one or affecting the first, second, and third-order neurons more brain MRI lesions at presentation developed MS, in contrast with a 25% conversion rate in those Differences between children and adults with Horner’s with no lesions (Optic Neuritis Study Group 2008). In syndrome. children, an abnormal MRI at presentation is likely also predictive. In our study (Bonhomme et al. 2009) 18 • Congenital cases, with birth trauma in the differential patients were followed for more than 24 months, and diagnosis (Weinstein et al. 1980) 3 of the 18 (17%) developed MS. All three patients had • More presentations with iris heterochromia an abnormal brain MRI scan at their initial presentation • Carotid dissection, lungancer, c and microvascular of optic neuritis. None of the patients with a normal causes more common in adults. brain MRI scan at presentation developed MS over • Need to avoid apraclonidine testing because of risk of an average follow-up of 88.5 months. Patients with drowsiness and unresponsiveness in young children 96 | North American Neuro-Ophthalmology Society • Confirmation with cocaine drop testing preferred Ocular Myasthenia Gravis • Neuroblastoma is a consideration, so workup should 1. Bever C, Aquino A, Penn A, Lovelace R, Rowland L. Prognosis of include urine vanillylmandelic acid (VMA) and ocular myasthenia. Ann Neurol. 1983;14:516-519. homovanillic acid (HVA) testing. 2. Kim J, Hwang J, Hwang Y, Kim K, Chae J. Childhood ocular • Despite opinion to the contrary (Smith et al. 2010), we myasthenia gravis. Ophthalmology. 2003;110:1458-1462. believe that all patients with an obvious or confirmed 3. McCreery K, Hussein M, Lee A, Paysse E, Chandran R, Coats D. Horner’s syndrome also should undergo MR imaging Major review: the clinical spectrum of pediatric myasthenia of the head, neck, and upper chest to rule out a gravis: blepharoptosis, ophthalmoplegia, and strabismus. A report of 14 cases. Binocul V Strabismus Q. 2002;17:181-186. responsible mass lesion. In our study (Mahoney et 4. Mullaney P, Vajsar J, Smith R, Buncic J. The natural history and al. 2006), of 18 children who had complete imaging ophthalmic involvement in childhood myasthenia gravis at The and urine studies, and the diagnosis was unknown, Hospital for Sick Children. Ophthalmology. 2000;107:504-510. responsible mass lesions were found in six (33%). 5. Ortiz S, Borchert M. Long-term outcomes of pediatric ocular • Even children with a history