What are the signs and symptoms of pseudo tumor cerebri (idiopathic intracranial hypertension)?

Signs and Symptoms of Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)

Headache is the dominant symptom, occurring in nearly 90% of patients with pseudotumor cerebri, typically presenting as a progressively worsening holocephalic or unilateral throbbing pain that is worse in the morning after supine positioning and improves with upright posture throughout the day. 1, 2

Primary Clinical Features

Headache Characteristics

• The headache phenotype is highly variable and may mimic other primary headache disorders, making diagnosis challenging 1
• Headaches are progressively more severe and frequent over days to weeks 2
• Pain is typically worse upon awakening from sleep, suggesting increased intracranial pressure 3
• Symptoms worsen with Valsalva maneuver 3

Visual Symptoms

• Transient visual obscurations (unilateral or bilateral darkening of vision lasting typically seconds) are common 1, 2
• Visual blurring occurs frequently 1
• Papilledema is a key diagnostic finding and is present in the majority of patients 3, 2
• Progressive visual field constriction and decreased visual acuity can develop if untreated 4, 5

Auditory Symptoms

• Pulsatile tinnitus (whooshing sound in the ears synchronized with pulse) is characteristic 1, 2
• This symptom can occasionally be the presenting manifestation 6

Cranial Nerve Involvement

• Horizontal diplopia is typical, most commonly due to sixth nerve palsy 1, 2
• Cranial nerve deficits occur in a minority of cases, with sixth nerve palsies being most frequent 4
• Rarely, diffuse ophthalmoparesis can occur in severe cases 4

Secondary Symptoms

The following symptoms are less specific but commonly reported 1:

• Dizziness 1
• Neck pain 1
• Back pain 1
• Cognitive disturbances 1
• Radicular pain 1
• Nausea and vomiting related to elevated intracranial pressure 3

Important Clinical Patterns

Patient Demographics

• Pseudotumor cerebri predominantly affects overweight females of childbearing age 3, 2
• The condition can also occur in obese males and prepubertal thin children 2
• Incidence is increasing parallel to the obesity epidemic 2

Neurological Examination

• The neurological examination is typically normal except for papilledema and possible sixth nerve palsy 3
• There are no focal or lateralized neurological deficits such as hemiparesis or sensory changes in the extremities 3
• Any abnormal neurological findings beyond sixth nerve palsy should prompt consideration of alternative diagnoses 3

Critical Pitfalls to Recognize

Atypical Presentations

• None of the symptoms are pathognomonic for IIH, requiring careful diagnostic evaluation 1
• Isolated headache without papilledema can occur in up to 25% of patients, presenting a significant diagnostic challenge 2
• Rare cases may present with diffuse ophthalmoparesis and polyradiculopathy, mimicking inflammatory processes in the subarachnoid space 4

Asymptomatic Patients

• Some patients may be asymptomatic at presentation and remain asymptomatic during recurrence, requiring heightened vigilance during monitoring 7

Visual Loss Risk

• Visual loss is the main morbidity of IIH and is present in most patients 5
• This can usually be reversed if recognized early and treated promptly 5
• Despite adequate treatment, children can rarely experience permanent loss of visual field and acuity 8