Idiopathic intracranial hypertension: Difference between revisions
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+ | == Background == |
||
aka. pseudotumor cerebri |
aka. pseudotumor cerebri |
||
− | == |
+ | ===Definition=== |
− | * |
+ | *Increase in intracranial pressure without an identifiable cause |
− | * |
+ | *Defined as elevated opening pressure > 25cm H2O on lumbar puncture without identifiable intracranial structural pathology |
− | == |
+ | ===Pathophysiology=== |
− | * |
+ | *Most likely from elevated venous pressure causing decreased CSF resorption |
+ | |||
+ | ===Risk Factors=== |
||
+ | |||
+ | *Obese female of child-bearing age |
||
+ | *[[Hypervitaminosis A]] |
||
+ | *[[Tetracycline]] antibiotics |
||
+ | *[[Isotretinoin]] |
||
+ | *Oral contraceptive pills |
||
+ | *[[Phenytoin]] |
||
+ | *[[Pregnancy]] |
||
+ | *[[Glucocorticoid]] use or withdrawal |
||
+ | |||
+ | ==Clinical Manifestations== |
||
+ | |||
+ | *History |
||
+ | **Diffuse, steady or throbbing headache that is worse in the morning |
||
+ | **Visual obscurations: bilateral dimming of vision lasting seconds |
||
+ | **Blurred vision, scotomas, and diplopia |
||
+ | **Pulsatile tinnitus, dizziness, and neck pain |
||
+ | *Signs & Symptoms |
||
+ | **Papilledema |
||
+ | **Strabismus from CN VI palsy |
||
== Differential Diagnosis == |
== Differential Diagnosis == |
||
+ | *Primary or idiopathic: includes obesity, recent weight gain, [[polycystic ovarian syndrome]], and thin children |
||
− | * Severe iron deficiency anemia |
||
+ | *Secondary |
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− | * Sickle cell anemia |
||
+ | **Cerebral venous abnormalities |
||
− | * Leukemia |
||
+ | ***Cerebral venous sinus thrombosis |
||
− | * Addison disease |
||
+ | ***Bilateral jugular vein thrombosis or surgical ligation |
||
− | * Pregnancy, PCOS |
||
+ | ***[[Otitis media]] or mastoid infection |
||
− | * Hypothyroidism, thyrotoxicosis |
||
+ | ***Right heart failure |
||
− | * Hypocalcemia |
||
+ | ***[[Superior vena cava syndrome]] |
||
− | * Renal failure |
||
+ | ***[[Arteriovenous fistula]] |
||
− | * COPD |
||
+ | ***Decreased CSF absorption from prior infection or [[subarachnoid hemorrhage]] |
||
− | * Right heart failure with lumpnary hypertension |
||
+ | **Medications and other exposures |
||
− | * OSA |
||
+ | ***Antibiotics: [[tetracycline]], [[minocycline]], [[doxycycline]], [[nalidixic acid]], [[Sulfa drug|sulfa drugs]] |
||
− | * SLE, sarcoid |
||
+ | ***Vitamin A and retinoids: [[hypervitaminosis A]], [[isotretinoin]], [[all-trans retinoic acid]], excessive liver ingestion |
||
− | * CSVT/IJV thrombus |
||
+ | ***Hormones: [[human growth hormone]], [[thyroxine]] (in children), [[leuprorelin acetate]], [[levonorgestrel]], [[Anabolic steroid|anabolic steroids]] |
||
− | * Meningitis |
||
+ | ***Withdrawal from chronic [[Corticosteroid|corticosteroids]] |
||
− | * Otitits media, lyme, HIV, varicella, sinusitis, viral disease, febrile illness, Strep B pharyngitis |
||
+ | ***[[Lithium]] |
||
− | * Medication |
||
+ | ***[[Chlordecone]] |
||
+ | **Medical conditions |
||
+ | ***Endocrine disorders: [[Addison disease]], [[hypoparathyroidism]], [[hypothyroidism]] |
||
+ | ***Hypercapnia: [[sleep apnea]], [[Pickwickian syndrome]], [[COPD]] |
||
+ | ***Infections: [[otitis media]], [[Lyme disease]], [[HIV]], [[sinusitis]], [[VZV]], [[pharyngitis]] from group B streptococci, other febrile illnesses |
||
+ | ***[[Anemia]] |
||
+ | ***[[Renal failure]] |
||
+ | ***[[Systemic lupus erythematosus]] |
||
+ | ***[[Sarcoidosis]] |
||
+ | ***[[Turner syndrome]] |
||
+ | ***[[Down syndrome]] |
||
− | == |
+ | ==Investigations== |
+ | *Imaging |
||
− | * Obese female of child-bearing age |
||
+ | **MRI brain may show small ventricles, partially empty sella turcica, widening of the optic nerve sleeves, optic nerve buckling, or flattening of the optic globes, or may be normal |
||
− | * Hypervitaminosis A |
||
+ | *Other |
||
− | * Tetracycline antibiotics |
||
+ | **[[Lumbar puncture]] with opening pressure for diagnosis |
||
− | * Isotretinoin |
||
+ | **[[Visual field testing]] is crucial |
||
− | * Oral contraceptive pills |
||
− | * Phenytoin |
||
− | * Pregnancy |
||
− | * Glucocorticoid use or withdrawal |
||
+ | ==Diagnosis== |
||
− | == Clinical Presentation == |
||
+ | === Modified Dandy criteria[[CiteRef::friedman2013re]] === |
||
− | * History |
||
− | ** Diffuse, steady or throbbing headache that is worse in the morning |
||
− | ** Visual obscurations: bilateral dimming of vision lasting seconds |
||
− | ** Blurred vision, scotomas, and diplopia |
||
− | ** Pulsatile tinnitus, dizziness, and neck pain |
||
− | * Signs & Symptoms |
||
− | ** Papilledema |
||
− | ** Strabismus from CN VI palsy |
||
+ | *'''Papilledema''', with all of the following: |
||
− | == Investigations == |
||
+ | **Normal neurologic examination except for cranial nerve abnormalities |
||
+ | **Neuroimaging showing |
||
+ | ***Typical patients (female and obese): normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion, and no abnormal meningeal enhancement on MRI (with and without gadolinium) |
||
+ | ***Others: MRI (with and without gadolinium) plus MRV |
||
+ | ***If MRI unavailable, then CT with contrast |
||
+ | **Normal CSF |
||
+ | **Elevated [[lumbar puncture]] opening pressure ≥250 mm in adults or ≥280 mm in children |
||
+ | *'''No papilledema''': |
||
+ | **All of the above criteria are met, plus there is unilateral or bilateral abducens nerve palsy |
||
+ | *'''No papilledema, and no abducens nervy palsy''': |
||
+ | **Cannot be diagnosed, but can be suggested if all of the above criteria are met, plus at least 3 of the following are seen on neuroimaging: |
||
+ | ***Empty sella |
||
+ | ***Flattening of the posterior aspect of the globe |
||
+ | ***Distention of the perioptic subarachnoid space with or without a tortuous optic nerve |
||
+ | ***Transverse venous sinus stenosis |
||
+ | ==Management== |
||
− | * Labs |
||
− | * Imaging |
||
− | ** MRI brain may show small ventricles, partially empty sella turcica, widening of the optic nerve sleeves, optic nerve buckling, or flattening of the optic globes, or may be normal |
||
− | * Other |
||
− | ** [Lumbar puncture] with opening pressure for diagnosis |
||
− | ** [Visual field testing] is crucial |
||
− | == |
+ | === Acute === |
+ | *Discontinue offending medication, if any |
||
− | * modified dandy's criteria |
||
+ | *LP for decompression, including repeated |
||
− | * s/s of increased ICP |
||
+ | *May need surgical intervention if sight is threatened or treatment with medication fails |
||
− | * no neuro signs except CN VI palsy |
||
− | * normal CSF |
||
− | * no hydrocephalus/mass/etc on MRI |
||
− | * no other cause of incranranial hypertension |
||
− | * opening pressure >25cm |
||
− | * clear clinical response to LP drainage |
||
− | == |
+ | === Chronic === |
+ | *Medication |
||
− | * Acute |
||
+ | **[[Acetazolamide]] 250-500 mg p.o. twice daily, and titrated up to a maximum of 4 g total daily dose |
||
− | ** Discontinue offending medication, if any |
||
+ | **[[Topiramate]] |
||
− | * Chronic |
||
+ | **[[Corticosteroids]] |
||
− | ** Acetazolamide |
||
+ | *Repeat LPs, often every 1-3 months despite medications |
||
− | ** Topiramate |
||
− | * |
+ | *Remove causative factors, including counselling on weight loss |
+ | *Refer to Ophthalmology and Neurology |
||
− | * Medication |
||
− | ** Acetazolamide |
||
− | ** Topiramate |
||
− | ** Corticosteroids |
||
− | * Repeat LPs, often every 1-3 months despite medications |
||
− | * Remove causative factors, including counselling on weight loss |
||
− | * Refer to Ophthalmology and Neurology |
||
− | * May need surgical intervention if sight is threatened or treatment with medication fails |
||
[[Category:Neurology]] |
[[Category:Neurology]] |
Latest revision as of 12:27, 2 May 2023
Background
aka. pseudotumor cerebri
Definition
- Increase in intracranial pressure without an identifiable cause
- Defined as elevated opening pressure > 25cm H2O on lumbar puncture without identifiable intracranial structural pathology
Pathophysiology
- Most likely from elevated venous pressure causing decreased CSF resorption
Risk Factors
- Obese female of child-bearing age
- Hypervitaminosis A
- Tetracycline antibiotics
- Isotretinoin
- Oral contraceptive pills
- Phenytoin
- Pregnancy
- Glucocorticoid use or withdrawal
Clinical Manifestations
- History
- Diffuse, steady or throbbing headache that is worse in the morning
- Visual obscurations: bilateral dimming of vision lasting seconds
- Blurred vision, scotomas, and diplopia
- Pulsatile tinnitus, dizziness, and neck pain
- Signs & Symptoms
- Papilledema
- Strabismus from CN VI palsy
Differential Diagnosis
- Primary or idiopathic: includes obesity, recent weight gain, polycystic ovarian syndrome, and thin children
- Secondary
- Cerebral venous abnormalities
- Cerebral venous sinus thrombosis
- Bilateral jugular vein thrombosis or surgical ligation
- Otitis media or mastoid infection
- Right heart failure
- Superior vena cava syndrome
- Arteriovenous fistula
- Decreased CSF absorption from prior infection or subarachnoid hemorrhage
- Medications and other exposures
- Antibiotics: tetracycline, minocycline, doxycycline, nalidixic acid, sulfa drugs
- Vitamin A and retinoids: hypervitaminosis A, isotretinoin, all-trans retinoic acid, excessive liver ingestion
- Hormones: human growth hormone, thyroxine (in children), leuprorelin acetate, levonorgestrel, anabolic steroids
- Withdrawal from chronic corticosteroids
- Lithium
- Chlordecone
- Medical conditions
- Endocrine disorders: Addison disease, hypoparathyroidism, hypothyroidism
- Hypercapnia: sleep apnea, Pickwickian syndrome, COPD
- Infections: otitis media, Lyme disease, HIV, sinusitis, VZV, pharyngitis from group B streptococci, other febrile illnesses
- Anemia
- Renal failure
- Systemic lupus erythematosus
- Sarcoidosis
- Turner syndrome
- Down syndrome
- Cerebral venous abnormalities
Investigations
- Imaging
- MRI brain may show small ventricles, partially empty sella turcica, widening of the optic nerve sleeves, optic nerve buckling, or flattening of the optic globes, or may be normal
- Other
- Lumbar puncture with opening pressure for diagnosis
- Visual field testing is crucial
Diagnosis
Modified Dandy criteria1
- Papilledema, with all of the following:
- Normal neurologic examination except for cranial nerve abnormalities
- Neuroimaging showing
- Typical patients (female and obese): normal brain parenchyma without evidence of hydrocephalus, mass, or structural lesion, and no abnormal meningeal enhancement on MRI (with and without gadolinium)
- Others: MRI (with and without gadolinium) plus MRV
- If MRI unavailable, then CT with contrast
- Normal CSF
- Elevated lumbar puncture opening pressure ≥250 mm in adults or ≥280 mm in children
- No papilledema:
- All of the above criteria are met, plus there is unilateral or bilateral abducens nerve palsy
- No papilledema, and no abducens nervy palsy:
- Cannot be diagnosed, but can be suggested if all of the above criteria are met, plus at least 3 of the following are seen on neuroimaging:
- Empty sella
- Flattening of the posterior aspect of the globe
- Distention of the perioptic subarachnoid space with or without a tortuous optic nerve
- Transverse venous sinus stenosis
- Cannot be diagnosed, but can be suggested if all of the above criteria are met, plus at least 3 of the following are seen on neuroimaging:
Management
Acute
- Discontinue offending medication, if any
- LP for decompression, including repeated
- May need surgical intervention if sight is threatened or treatment with medication fails
Chronic
- Medication
- Acetazolamide 250-500 mg p.o. twice daily, and titrated up to a maximum of 4 g total daily dose
- Topiramate
- Corticosteroids
- Repeat LPs, often every 1-3 months despite medications
- Remove causative factors, including counselling on weight loss
- Refer to Ophthalmology and Neurology
References
- ^ D. I. Friedman, G. T. Liu, K. B. Digre. Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children. Neurology. 2013;81(13):1159-1165. doi:10.1212/wnl.0b013e3182a55f17.