Transcript Pediatric Neurology CME August 1, 2012 Case presentation

Pediatric Neurology
CME
August 1, 2012
Case presentation
Carol M. Sanders, MD
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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
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Triglycerides 164
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LDL 146
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HDL 43
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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
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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 Papilledema
Visual field loss
6th nerve palsy
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• 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
hydrocephalus
obstruction of venous outflow
• PE, absence of focal neurologic signs,
medication hx, fundoscopic, MRI, L/P,
visual field testing
• 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
• Treatment – alleviate symptoms
preserve vision
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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