Pediatric NeurologyCME

August 1, 2012

Case presentation

Carol M. Sanders, MD

• K.S.

• African American female

• 7/2005 for 4-year PE

• NKDA. Resolving Bronchial asthma.

• Otherwise well

• BMI>97th %ile

• 8 years of age• Fasting lipids – Cholesterol 222• Triglycerides 164

• LDL 146• HDL 43• Glu 92

Referred to CHM lipid clinic

• 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

• 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

• 3/9/11 Double vision since ER, emesis with headache

• Decrease headache when supine, decrease po, increase sleep

• Congestion resolved

• No fever, no trauma

• 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

• Possible increased ICP

Emergency CT scan of the head

Normal

• Possible pseudotumor cerebri

• 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”

• 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.

• Diamox 250 mg BID started with improvement of symptoms.

• Patient discharged on 3-15-11

• Dx: Pseudotumor cerebri

• Neurology and ophthalmology follow up

• 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(5th 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.

• 4/27/12 Menarche 6/11.

• Visit for baseline labs to start out patient weight management program.

• Ophthalmology follow up scheduled.

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

• Most common symptom – Headache

• Most common signs -PapilledemaVisual field loss6th nerve palsy

•

• 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

• Evaluation – R/O other causes of increased ICP

mass hydrocephalusobstruction of venous outflow

• PE, absence of focal neurologic signs, medication hx, fundoscopic, MRI, L/P, visual field testing

• Prognosis – not benign. Disabling headachesRisk 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

• 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

• References:• Kliegman, R. M., Behrman, R. E.,Jenson, H. B., Stanton,

B. F., Nelson Textbook of Pediatrics, 18th 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, 2012