Hipertensión endocraneana idiopática Afección que se produce cuando la presión dentro del cráneo aumenta sin motivo aparente.

1. SERVICIO DE NEURO OFTALMOLOGIA
DICIEMBRE 2023
M.R MAGALY MARIBEL YUJRA PARI

2. IDIOPATHIC INTRACRANIAL HYPERTENSION
 (benign intracranial hypertension;
venous intracranial hypertension;
pseudotumor cerebri)

3. Epidemiology
Idiopathic intracranial hypertension usually affects women of childbearing age.
The incidence is 1/100,000 in women of normal weight but 20/100,000 in obese women.
Intracranial pressure (ICP) is elevated (> 250 mm H2O)
It is rare in the pediatric population, being more common in the 12-17 year age group than in the 2-12 year age
group
In children, this disorder sometimes develops after stopping corticosteroids or after using growth hormone.
Idiopathic intracranial hypertension can also develop after takingtetracyclinesor large amounts of vitamin A.

4.  The cause is unknown, but may involve
obstruction of cerebral venous return
flow, which may be the result of
increased CSF pressure, which may
aggravate the venous obstruction and
lead to a vicious cycle

5. Symptoms and signs of idiopathic intracranial
hypertension
Headache(84to92percent)
●Transientvisualobscurations(68to72percent)
●Intracranialnoises(pulsatiletinnitus; 52to60percent)
●Photopsia(48to54percent)
●Backpain(53percent)
●Retrobulbarpain(44percent)
●Diplopia(18to38percent),typicallyfromnonlocalizingsixthnervepalsy
●Sustainedvisualloss(26to32percent)
●Neckpain(41percent)

6. Bilateral papilla
edema iscommon;Somepatientshaveunilateralpapilledemaornopapilledema.
Insomeasymptomaticpatients,papilledemaisdiscoveredduringroutineophthalmoscopicexa
mination.
Neurologicalexaminationmaydetect partialpalsyoftheVIcranialnerve,butotherwiseitisunrem
arkable.

7. Diagnosis of idiopathic intracranial
hypertension
 If clinical findings
suggest idiopathic
intracranial
hypertension,
clinicians should
evaluate visual fields
and fundus, even in
patients without visual lumbar puncture

8. Thediagnosis
ofidiopathicintracranialhypertensionissuspectedclinicallyandestablishedbyimagingstudies(preferablyMRIwithvenography)thathavenormalresults(e
xceptfornarrowingofthetransversevenoussinus).
Ifnotcontraindicated, a lumbarpuncturewithcerebrospinalfluid (CSF)analysisisperformed.
Elevatedopeningpressureand normal CSFcompositionsuggestidiopathicintracranialhypertension.
RM convenografíapor
resonancia magnética
Punción lumbar

9. The diagnosis is commonly based on the modified Dandy criteria, which has been
updated for the Idiopathic Intracranial Hypertension Treatment Trial as follows
presence of signs and symptoms of increased intracranial pressure
absence of localizing findings on neurologic exam except those known to occur from increased
intracranial pressure
absence of deformity, displacement, or obstruction of the ventricular system and otherwise
normalneurodiagnosticstudies, except for evidence of increased CSF pressure (>20.0 cm H2O)*;
abnormalneuroimagingexcept for emptysellaturcica, optic nerve sheath with filled out CSF spaces, and
smooth-walled non-flow-related venous sinusstenosisor collapse should lead to another diagnosis
awake and alert patient
no other cause of increased intracranial pressure present

10. *The opening CSF pressure should be either >25.0 cm H2O or 20.0-
25.0 cm H2O with at least one of the following additional findings:
pulse-synchronous tinnitus
abducens nerve palsy
echography negative for drusen or other disc anomalies
mimicking disc edema (pseudopapilledema)
lateral sinus stenosis or collapse
partially empty sella and optic nerve sheaths with filled
out CSF spaces

11. •prominent subarachnoid
space around the optic
nerves (yellow)
•mild vertical tortuosity of
the optic nerves (red
arrows)
•flattening of the posterior
sclera (blue dotted lines)

12. •partially empty sella turcica (red
circle)

13. •stenosis of the lateral segments of the
transverse sinuses (green arrows)

14. There is high T2 signal expanding
dural sheath of the optic nerves
bilaterally (i.e. dilated CSF spaces
around the optic nerves
bilaterally). Partially empty sella.
No area of abnormal signal
intensity elicited in supra- or
infratentorial regions.

15. Disorders associated with papilledema that resemble idiopathic
intracranial hypertension. idiopathic intracranial hypertension

16. Treatment of idiopathic intracranial
hypertension
Pressure
reduction
Vision
Preservation
Relieve
symptoms

17. GENERAL MEASURES
Acetazolamideortopiramate Weight loss if necessary
Medicines used to prevent
migraines,
especiallytopiramate
Sometimes surgery

18. The
carbonicanhydraseinhibitoracetazolamide(250
mg orally 4 times a day)
Acetazolamidereduces
the production of
cerebrospinal fluid.
Topiramate(which also inhibits
carbonicanhydrase) up to 200 mg 2 times a
day is indicated.
Topiramatecan help
patients lose weight,
reduce intracranial
pressure, and relieve
headache.

19. Serial lumbar punctures are controversial, but are
sometimes used, especially if, while awaiting definitive
treatment, vision is threatened.
Definitive treatment includes creation of a fenestration
in the optic nerve, bypass, or placement of an
endovascular stent in the venous sinus.
Any potential cause (disorders, drugs, or drugs) is
corrected or eliminated whenever possible.

20.  Frequent ophthalmologic evaluation
(including quantitative study of visual fields)
is required to monitor response to
treatment; Visual acuity testing is not
sensitive enough to warn of impending
visual loss
 If vision deteriorates despite treatment, one
of the following may be indicated:
Optic nerve sheath fenestration
Shunt (lumboperitonealorventriculoperitoneal)
Intravascular prosthesis via venous .

21. Digre KB, Bruce BB, McDermott MP, et al. Quality of life in idiopathic intracranial hypertension at
diagnosis: IIH Treatment Trial results. Neurology 2015; 84:2449.
Wall M, George D. Idiopathic intracranial hypertension. A prospective study of 50 patients. Brain
1991; 114 ( Pt 1A):155.
Wall M, Kupersmith MJ, Kieburtz KD, et al. The idiopathic intracranial hypertension treatment
trial: clinical profile at baseline. JAMA Neurol 2014; 71:693.
Giuseffi V, Wall M, Siegel PZ, Rojas PB. Symptoms and disease associations in idiopathic
intracranial hypertension (pseudotumor cerebri): a case-control study. Neurology 1991; 41:239.
Silberstein, S. D. (n.d.). Idiopathic Intracranial Hypertension. Manual Professional Edition. Retrieved December
15, 2021.
Referencias