1. Pediatric Neurology CME August 1, 2012 Case presentation Carol M. Sanders, MD

2. K.S. African American female 7/2005 for 4-year PE NKDA. Resolving Bronchial asthma. Otherwise well BMI>97 th %ile

4. 8 years of age Fasting lipids – Cholesterol 222 Triglycerides 164 LDL 146 HDL 43 Glu 92 Referred to CHM lipid clinic

5. 3/3/11 10 y.o. Pubertile. Nasal congestion. Puffy eyelids. Headache. BP 118/74, temp 98.3, Wt. 228# EOM’s nl, PERRL Dx: Sinusitis Rx: Amoxicillin 875 mg. bid

6. 3/5/11 CHM ER Emesis for 2 days Intermittent frontal headache, neck and back pain Awake, alert, clear rhinorrhea Temp 36.4 BP 132/80 Sinus films normal. Continue present therapy

7. 3/9/11 Double vision since ER, emesis with headache Decrease headache when supine, decrease po, increase sleep Congestion resolved No fever, no trauma

8. PE – patient covers one eye due to diplopia; cervical pain with flexion Full EOM’s,no photophobia, no proptosis ?blurred optic discs, +SVP’s Facial symmetry, nl grip, nl gait, no ataxia Nl joints, no rash Rest of PE nl

9. Possible increased ICP Emergency CT scan of the head Normal Possible pseudotumor cerebri

10. CHM ER Temp 36.9, P 70, RR 18, BP 115/61 Spo2 99%, Wt.222# IV tordal, zantac, zofran MRI – edema of optic nerve, cannot exclude optic neuritis, question some demyelination L/P – “elevated opening pressure”

11. Neurology, Infectious Disease, Rheumatology consults CSF studies normal including viral studies; negative blood for bartonella and mycoplasma; ppd negative – infectious cause unlikely Negative or nl ANCA, ANA, CRP, ENA, C3, C4, PT, PTT, DVVT, beta 2 glycoprotein Ab. No afferent pupillary defect. Optic neuritis or thrombosis unlikely.

12. Diamox 250 mg BID started with improvement of symptoms. Patient discharged on 3-15-11 Dx: Pseudotumor cerebri Neurology and ophthalmology follow up

13. 4/22/11 Ophthalmology – Diamox increased to tid for persistent papilledema; vision 20/20; increased blind spot 9/2/11 Dr. Constantinou – hx of intermittent hedaches since 9/10, daily since 2/11, increase intensity PTA. Grades C’s and D’s previous school year with headaches(5 th grade). On honor roll by end of school year with headache treatment. Plan repeat MRI in future. 1/12 Ophthalmology – Diamox D/C’d. Optic discs normal. No headaches one month later.

14. 4/27/12 Menarche 6/11. Visit for baseline labs to start out patient weight management program. Ophthalmology follow up scheduled.

15. Idiopathic Intracranial Hypertension = Pseudotumor Cerebri Disorder defined by clinical criteria that include symptoms and signs isolated to those produced by increased intracranial pressure (eg, headache, papilledema, vision loss), elevated intracranial pressure with normal cerebrospinal fluid composition, and no other cause of intracranial hypertension evident on neuroimaging or other evaluations. UpToDate, 2012

16. Most common symptom – Headache Most common signs - Papilledema Visual field loss 6 th nerve palsy

17. Pathogenesis – unknown. Most common in obese women of childbearing age. Link to obesity and gender difference not as apparent in prepubertile children. Medications – growth hormone therapy, steroid withdrawal, TCN, Excessive vitamin A and other retinoids Systemic illness - Obesity

18. Evaluation – R/O other causes of increased ICP mass hydrocephalus obstruction of venous outflow PE, absence of focal neurologic signs, medication hx, fundoscopic, MRI, L/P, visual field testing

19. Prognosis – not benign. Disabling headaches Risk of permanent vision loss Gradual, fluctuating or fulminant course Variable response to treatment No reliable predicative factors for risk of vision loss Recurrence can occur particularly with weight gain

20. Treatment – alleviate symptoms preserve vision –Carbonic anhydrase inhibitor –Loop diuretic –Corticosteroids –Analgesics –Serial L/P –Surgery – shunting, optic nerve fenestration –Weight loss –Eliminate offending medication –Close follow up of visual acuity and visual fields

21. References: Kliegman, R. M., Behrman, R. E.,Jenson, H. B., Stanton, B. F., Nelson Textbook of Pediatrics, 18 th edition, Philadelphia, Saunders, 2007 Robertson, Jr., W. C., “Pediatric Idiopathic Intracranial Hypertension,”Medscape Reference, July 5, 2012 Wall, M., “Idiopathic Intracranial Hypertension (Pseudotumor Cerebri),” Curr Neurol Neuroscience Rep. 2008 Mar;8(2):87-93. Lee, A. G., Wall, M., “Idiopathic Intracranial Hypertension (Pseudotumor Cerebri), www.uptodate.com, 2012www.uptodate.com