Occurs when the pressure in the skull increases for no obvious reason.
Primary problem is chronically elevated intracranial pressure (ICP).
Most important neurologic manifestation is papilledema.
Papilledema may lead to progressive optic atrophy and blindness.
Visual function testing, in particular, visual field, funduscopy, and ocular motility examination, are the most important parts of the neurologic examination for diagnosing and monitoring patients with idiopathic intracranial hypertension.
Symptoms mimic those of a brain tumor, but no tumor is present.
Pseudotumor cerebri signs and symptoms might include:
Often severe headaches that might originate behind the eye.
A whooshing sound in the head that pulses with your heartbea
Nausea, vomiting or dizzinesS.
Brief episodes of blindness, lasting a few seconds and affecting one or both eyes
Difficulty seeing to the sidE
Seeing light flashes.
Neck, shoulder or back pain
Can occur in children and adults, but it’s most common in obese women of childbearing age.
When no underlying cause for the increased intracranial pressure can be discovered, pseudotumor cerebri referred to as idiopathic intracranial hypertension.
Can cause swelling of the optic nerve and result in vision loss.
Etiology unknown, but it may be linked to an excess amount of cerebrospinal fluid within the brain.
May be related to decreased ability of the brain to absorb CSF.
Risk factors associated with pseudotumor cerebri: obesity, medications including lithium, oral contraceptives, tetracycline, steroids or discontinuatin of steroids and excess vitamin A.
Occurs in about one person per 100,000 in the general public.
Obese women under the age of 44 are 20 times more likely to develop the disorder.
Endocrine risk factors: Female sex, Reproductive age group, Menstrual irregularity, Obesity, Recent weight gain, Adrenal insufficiency, Cushing disease, Hypoparathyroidism, Hypothyroidism, Excessive thyroxine replacement in children,
Medication risk factors : Cimetidine, corticosteroids, danazol, isotretinoin , levothyroxine, lithium, minocycline, nalidixic acid, nitrofurantoin, tamoxifen, tetracycline, trimethoprim-sulfamethoxazole, , All-trans -retinoic acid (ATRA), cyclosporine, levonorgestrel implant , pancreatin , Recombinant human growth hormone, and Vitamin A in infants.
Increased venous red blood cell aggregation and relatively elevated fibrinogen concentration have demonstrated in patients.
Men are significantly more likely than controls to have symptoms of testosterone deficiency and obstructive sleep apnea.
May be associated with head injury, renal diseae, lupus, lyme disease, monnucleosis, and hypoparathyroidism.
Up to 10 percent of the patients experience progressively worsening vision and may eventually become blind.
Intracranial dural sinus thrombosis should have been ruled out with reasonable certainty.
Endocrine risk factors confirmed in epidemiological studies:female sex, reproductive age group, menstrual irregularity, obesity, recent weight gain, adrenal insufficiency, Cushing disease, hypoparathyroidism, hypothyroidism, and excessive thyroxine replacement in children.
Medication risk factors : Cimetidine, corticosteroids, danazol, isotretinoin , levothyroxine, lithium,12 minocycline, nalidixic acid, nitrofurantoin, tamoxifen, tetracycline, trimethoprim-sulfamethoxazole, , All-trans -retinoic acid (ATRA) used in the treatment of promyelocytic leukemia, cyclosporine, levonorgestrel implant , pancreatin , Recombinant human growth hormone, Vitamin A in infants.
Miscellaneous risk factors:Chronic renal failure, Systemic lupus erythematosus.
Increased venous red blood cell aggregation and relatively elevated fibrinogen concentration were demonstrated in patients with IIH compared with matched controls.
The retinol/retinol-binding protein ratio is elevated in the CSF of patients with IIH compared with non-intracranial hypertensive neurologic controls and with normal controls.
Men with IIH were significantly more likely than controls to have symptoms of testosterone deficiency and obstructive sleep apnea.
Papilledema can be a sign of a medical emergency, and is an indication for an urgent brain CT or brain MRI to rule out a brain mass, focal or generalized cerebral edema, or obstruction of CSF flow, which may require emergency surgical intervention.
If there is no mass, and the patient was prescribed a medication that can cause pseudotumor cerebri, the triggering agent should be discontinued.
Normally, discontinuing the triggering agent prevents worsening of the fluid pressure, but does not reduce CSF pressure.
Untreated pseudotumor cerebri can result in permanent loss of vision due to pressure on the optic nerves: Diuretics or oral steroids can be prescribed to reduce the fluid.
An LP is a more definitive step for both diagnosis and treatment of the condition.
The LP should be done with the patient lying down so that an accurate opening pressure can be obtained.
A normal opening pressure ranges from 100 cm to180 cm H2O, and patients who are obese may have opening pressures up to 200 cm or even 250 cm H2O.
A patient with an opening pressure above 200 cm H2O is considered to have a high CSF pressure.
CSF should be removed during the LP to relieve the pressure, and should be allowed to drip slowly using a small gauge needle and should not be activity withdrawn.
CSF pressure should be measured before removing the needle to ensure that the closing pressure is normal.
Excessive amount of fluid should not be removed, as a relatively rapid drop in CSF pressure can cause a severe headache.
Most patients with pseudotumor cerebri recover after treatment with LP and medication.
If clinical symptoms and papilledema recur. repeated LPs are required and ventricular shunt may be placed to prevent fluid buildup.
Confusion with normal pressure hydrocephalus (NPH), which is also characterized by excessive cerebrospinal fluid causes gait abnormalities and dementia.
Normal pressure hydrocephalus is associated with enlarged ventricles, which are usually clearly visualized on brain MRI or brain CT.
People with NPH have normal CSF opening pressure on LP and do not benefit from removal of CSF.