Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri (PTC), is a disorder of diffuse increase in intracranial pressure leading to headache, diplopia, tinnitus, papilledema, and visual loss in the absence of a space-occupying lesion in the brain or other secondary causes of raised intracranial pressure. Benign intracranial hypertension is a misnomer for PTC because this disease is not benign and impairment of visual function has been reported in 25% of cases.[1,2] Blindness is a serious and permanent long-term complication of IIH.
The prevalence of PTC in the United States is 1 case per 100,000 general population, 1.6-3.5 cases per 100,000 in women, and 2.9-20 cases per 100,000 in young women with obesity. IIH can be seen in infants through old age. Men and women are both affected, but it is often seen in women of childbearing age with obesity. More than 90% of affected patients are women.
Presumed risk factors for IIH include recent weight gain of 3.96 lb (1.8 kg) over 2 months or 20 lb (9 kg) over 1 year; particular medications (eg, retinoid, tetracycline, growth hormone, thyroid hormone replacement, nitrofurantoin, lithium); and systemic diseases, such as Addison disease, hypothyroidism, hypoparathyroidism, anemia, sleep apnea, systemic lupus erythematosus, Behçet syndrome, polycystic ovary syndrome, hypercoagulable states, uremia, chronic respiratory insufficiency, hypertension, multiple sclerosis, chronic kidney disease, sarcoidosis, menstrual irregularities, and diseases of cerebral venous drainage.[4,5]
Clinical presentation usually occurs because of raised intracranial pressure and optic disc edema. Various presenting complaints include headache, diplopia, pulsatile tinnitus, photopsias, and transient or complete visual loss. Headache is the chief complaint in 92%-94% of patients. It is nonspecific in nature, with variation in type, frequency, and duration; it can be diffuse or lateralized to one half and is mostly seen in the morning, with worsening after straining, sneezing, and coughing.[6,7] Retrobulbar pain or backache may be associated. Some patients have a good response to analgesics, and some children can present without headache.
Diplopia occurs secondary to sixth nerve palsy; olfactory, occulomotor, trochlear, trigeminal, facial, or auditory nerve involvement may occur. Pulsatile tinnitus is typically in synchronization with pulse and is seen in 64%-87% of patients. Photopsias (ie, sparkles or flashes of light) are seen in some patients. Transient visual loss is most commonly seen with Valsalva maneuver, posture changes, or on exposure to bright lights; the visual loss can occur multiple times a day. Complete visual loss can be seen in fulminant cases; usually visual field deficits precede visual loss.
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Cite this: Sumaira Nabi, Muhammad Fateen Rashid, Shahzad Ahmed. Neurology Case Challenge: A 19-Year-Old With Tinnitus, Vision Problems, and Headaches - Medscape - May 31, 2022.