• Idiopathic intracranial hypertension (IIH) • Pseudotumour cerebri (PTC)• Benign intracranial hypertension (BIH)
• Increased intracranial pressure (ICP) without
clinical, laboratory or radiological evidence of intracranial pathology
• Original criterias were formulated in 1937 by
• Increased ICP with normal cerebrospinal fluid
(CSF) findings and no sign of a brain tumour onventriculography
International Headache Society’s classification of IIH
1. Alert patient with neurological examination that either is normal or
demonstrates any of the following abnormalities:(a) Papilloedema(b) Enlarged blind spot(c) Visual field defect(d) Sixth nerve palsy
(>200 mm H2O in the non-obese,>250 mm H2O in the obese)
3. Normal CSF chemistry (low CSF protein is acceptable) and cellularity.
4. Intracranial disease (including venous sinus thrombosis) ruled out.
5. No metabolic, toxic or hormonal cause of intracranial hypertension.
A presenting headache is attributed to idiopathic intracranial hypertension when the headache develops in close temporal relation to the increased intracranial pressure and improves after withdrawal of CSF. The headache should be progressive with at least one of the following:(a) Daily occurrence(b) Diffuse and/or constant non-pulsating pain(c) Aggravated by coughing or straining
childbearing age, incidence up to 21 / 100 / year
• Approximately 70% of the females are obese, (BMI
• Association with obesity has not been proven in
• In prepubertal children no female predominance
• Parenchymal oedema
• Slit-like / normal sized ventricles• Possible increased water content and difussion in subcortical white matter
• Increased cerebral blood volume (CBV)
• Increased CBV in anaesthetized patients / no differences in regional CBV IIH/controls
• Excessive CSF production
• Animal studies, no support human studies - normal CSF flow in the cerebral aqueduct
• Compromised CSF resorption
• Venous outflow obstruction
• Association with venous sinus hypertension • Unrecognized sinus thrombosis, extraluminal compression, AVM with venous
• Venous sinus hypertension > insufficiently high driving pressure gradient from the
subarachnoidal space to the venous system > increase in resistance of CSF absorption > raised ICP
Neuroimaging
• Empty sel a• Flattening of the posterior sclera• Dilation or tortuosity of the optic nerve sheet• Gadolinium enhancement of the optic disc
• Focusing on lowering the CSF pressure• Preventing visual defect development
• Reduces the CSF production• Initial dose of 250 mg twice a day gradually increasing to 1000–1250 mg • Side-effects dosedependent acroparaesthesias, nausea, anorexia,
• (40–120 mg/day) combined with potassium• Mechanism is unclear • Less potent than acetazolamide
• Lumboperitoneal (LP) shunt• Ventriculoperitoneal (VP) shunt • CSF shunting effective• Dysfunctions and infections
• Local reduction of the pressure around the
• Intracranial hypertension persists• Postoperative complication rate is high,
• Up to 32% experience deterioration in
visual function after an initial successfulfenestration
Spontaneous Intracranial Hypotension (SIH)
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Volleyboll smashad mot ansiktet ca 4 v innan
Söker akut LP 7 cm H2O, 16 mono, 0 poly, 20 ery.
Försämras, 2 v senare ny LP 16 cm H2O, 10 mon, 1 poly, 0 ery
Senare personlighetsförändring, förvirrad, glömsk, synhallucinationer, huvudvärk
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Spontaneous Intracranial Hypotension (SIH)
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• Structutal weaknesss of the spinal meninges?
• Dural holes• Fragile meningeal diverticula• Abscence of the dura• Mainly cervico-thoracic location
Spontaneous Intracranial Hypotension (SIH)
ICHD-II diagnostic criteria for spontaneous intracranial hypotension headache
• Diffuse and/or dull headache that worsens within 15 min after sitting
or standing and with 1 or more of the following:
– Neck stiffness– Tinnitus– Hypoacusis– Photophobia– Nausea
– Evidence of low CSF pressure on MRI (eg, pachymeningeal enhancement)– Evidence of CSF leakage on conventional myelography, CT myelography, or
– CSF opening pressure < 60 mm water in sitting position
• No history of dural puncture or other cause of CSF fistula• Headache resolves within 72 h after epidural blood patching
• CT• MRI• Radionuclide myelography• CT myelography• MRI myelography• Fluorescininjection
Probably due to increased permeability of dilated meningeal blood vessels and a decrease of CSF flow in the lumbar subarachnoid space. Diagnostic criteria for spontaneous spinal CSF leak and intracranial hypotension
Criterion A, demonstration of a spinal CSF leak (ie, presence of extrathecalCSF), or, if criterion A not met:
Criterion B, cranial MR imaging changes of intracranial hypotension (ie,presence of subdural fluid collections, enhancement of thepachymeninges, or sagging of the brain), and the presence of at least oneof the following:1) low opening pressure (60 mm H2O);2) spinal meningeal diverticulum;3) improvement of symptoms after epidural blood patching;or, if criteria A and B not met:
Criterion C, the presence of all of the following or at least 2 of thefollowing if typical orthostatic headaches are present:1) low opening pressure (<60 mm H2O),2) spinal meningeal diverticulum, and3) improvement of symptoms after epidural blood patching.
Patients with onset of symptoms after dural puncture or other penetrating spinal trauma are excluded. Gadolinium-Enhanced MR Cisternography to Evaluate Dural Leaks in Intracranial Hypotension Syndrome Am J Neuroradiol 29:116 –21 Jan 2008
• Lumbar puncture with injection of 0.5 mL gadopentetate
dimeglumine into the lumbar subarachnoid space
• MR images of the cervical, thoracic, and lumbar regions in axial,
coronal, and sagittal planes with fat-saturated T1-weighted images
• CSF leakage in 17 (89%) of 19 patients
• In 14 of the patients, the site of the dural tear was demonstrated • In 3, the contrast leakage was diffuse
• No complications during the first 24 hours or the 6- to 12-month
• Some resolves spontanously• Bed rest, somewhat less successful in SIH• Hydration • Adding caffeine, scientific proof of its efficacy is lacking
• Epidural blood patch •
Typically, 10 to 30 mL. The patient should remain lying flat
A larger volume of blood, up to 100 mL, could be used
The EBP can be repeated in 5 days if the SIH headache recurs or ifheadache relief is incomplete
In some patients, additional trials of EBP may be needed
46 – 96 % good outcome when repeated up to three times
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