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Establishing idiopathic intracranial hypertension. Associated or causative conditions. Aetiological diagnostic work up. Brain imaging. HISTORY .

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aetiological diagnostic work up

Establishing idiopathic intracranial hypertension

Associated or causative conditions

Aetiological diagnostic work up

Brain imaging

HISTORY

IMPORTANT NOTICE: Any prepubertal child and male adolescent has to be considered atypical and secondary intracranial hypertension has to be suspected.

Exclude intracranial mass lesion, malformation, ventriculomegaly.

Exclude sinus venous thrombosis by MRV in every atypical case, poor treatment response, relapse!

Medication, including contraceptives?Recent rapid weight gain?Menstruational problems?Current or recent infection?Any chronic illness?

Medical disorders: Addison’s disease; Hypoparathyroidism; Hypo- and hyperthyroidism; Vitamin D deficiency; Chronic obstructive pulmonary disease; Right heart failure with pulmonary hypertension; Sleep apnoea; Renal failure; severe iron deficiency anaemia; Thrombophilia (Antiphospholipid-Syndrome); PCOS, SLE.

Medications:Tetracycline and related compounds; Nitrofuratoin; Chinolone;Vitamin A and related compounds; Anabolic steroids; Corticosteroid withdrawal following prolonged administration (including inhalative steroids); Growth hormone administration in deficient patients; Nalidixic acid, Lithium, Norplant_ levonorgestral implant system

Obstruction to venous drainage:Cerebral venous sinus thrombosis; Jugular vein thrombosis

(Post)infectious: Lyme-disease; post-varicella; ???

Lumbar puncture

Clinical examination

Performed during the morning, in lateral decubitus position, calm child. Sedation if required. Avoid ketamine and inhalitve anaesthetics.

Check CSF opening pressure: >20 cm H2O is abnormal

Check for: Cell count, protein, glucose. Consider infectious work-up.

Consider repeated LP if presentation is suggestive for IIH but pressure is within normal limits, particularly in the young child.

Overweight? Fever? Signs of CNS-infection?Focal neurological signs (suggestive for IIH: sixth nerve palsy)? Reduced level of consciousness?High blood pressure?

Laboratory

Na, Ca, Mg, phosphate, blood gases, BUN, glucose, AST, RBC, WBC, CRP, T3, TSH

Ophthalmology

More comprehensive work-up is required if secondary intracranial hypertension is suspected (see right column)

Use age adjusted standardised visual field testing.Use Papilledema Grading System Scale.Visus? Neuroophthalmology (VI palsy?).

Lit.: Pediatric Idiopathic Intracranial Hypertension. Surv Ophthalmol 52:597--617, 2007.

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Therapy of idiopathic intracranial hypertension

Side effects: GI upset; paresthesias involving the lips, fingers, and toes; anorexia; electrolyte imbalance (metabolic acidosis). Kidney stones are rare, aplastic anemia exceedingly rare

First choice

Acetazolamid 15 (-100) mg/kg/day in 2 to 3 divided doses

Regular ophthalmological follow-up.

Check for: visual acuity, colour vision, visual field, papilledema

Regular ophthalmological follow-up.

Check for: visual acuity, colour vision, visual field, papilledema

No response

Not tolerated

Replace by furosemide (0.3--0.6 mg/kg per day)

No response

Acetazolamid + furosemide OR consider topiramate (1,5-3 mg/kg/d)

No response

No response/progression

No response/ progression

Consider corticosteroids

No response

More comprehensive work-up is required as secondary intracranial hypertension is suspected.

Contact neurosurgeon