What are the typical symptoms and presentation of intracranial hypertension?

Typical Presentation of Intracranial Hypertension

The typical presentation of intracranial hypertension includes headache that is progressively more severe and frequent, often worse in the morning or when lying flat, accompanied by visual disturbances such as transient visual obscurations, papilledema, pulsatile tinnitus, and diplopia.

Clinical Symptoms and Signs

Primary Symptoms

• Headache (most common symptom):

  • Progressively more severe and frequent 1
  • Typically diffuse and worse in the morning or when lying flat 2
  • Exacerbated by Valsalva maneuvers (coughing, straining) 2
  • Variable phenotype that may mimic other primary headache disorders 1
  • Affects quality of life in 49.6% of patients more than other symptoms 3

• Visual Disturbances:

  • Transient visual obscurations (unilateral or bilateral darkening of vision lasting seconds) 1, 2
  • Blurred vision 2
  • Double vision (diplopia, typically horizontal from sixth nerve palsy) 1, 2
  • Visual field defects 1

• Papilledema:

  • Present in approximately 60% of cases 2
  • Key diagnostic finding on fundoscopic examination 1

Associated Symptoms

• Pulsatile tinnitus (whooshing sound in ears) 1, 2
• Nausea and vomiting 2, 4
• Dizziness 1
• Neck pain 1
• Cognitive disturbances 1, 2
• Radicular pain 1
• Photophobia 4

Severe Cases

• Altered mental status that may progress to decreased consciousness 2, 4
• Pupillary abnormalities (unequal, dilated, poorly reactive) 2
• Cushing's triad (hypertension, bradycardia, irregular respiratory pattern) - a late finding 2
• Abnormal posturing (decorticate or decerebrate) 2
• Focal neurological deficits including hemiparesis or cranial nerve palsies 2, 5
• Seizures (may be subtle or subclinical in 25% of cases) 2
• Complete ophthalmoplegia (rare but severe manifestation) 5

Diagnostic Findings

Imaging Findings

• MRI findings may include:
  • Empty sella 2
  • Flattening of the posterior aspect of the globes (56% sensitivity, 100% specificity) 2
  • Distention of the perioptic subarachnoid space 2
  • Transverse sinus stenosis 2
  • Intraocular protrusion of the optic nerve (40% sensitivity, 100% specificity) 2
  • Horizontal tortuosity of the optic nerve 2
  • Enlarged optic nerve sheath 2
  • Smaller pituitary gland size 2

Lumbar Puncture Findings

• Elevated opening pressure (>250 mm CSF in adults, >280 mm CSF in children) 2
• Normal CSF composition (required for diagnosis of idiopathic intracranial hypertension) 1, 2

Special Populations

Pediatric Presentation

• Increasing head circumference 2
• Bulging fontanelle 2
• Loss of developmental milestones 2
• Irritability 2
• Poor feeding 2
• Poor school performance 2
• Fatigue 2
• Personality changes 2

Demographic Patterns

• Most common in overweight women of reproductive age 2
• Increasing incidence (reported as 2.4 per 100,000 within the last decade) 1

Clinical Pitfalls and Caveats

• Misdiagnosis is common - IIH is thought to be both highly underdiagnosed and misdiagnosed 1
• Normal pressure readings don't exclude the diagnosis - CSF opening pressure can occasionally be within normal range in spontaneous intracranial hypotension 4
• Medication-induced cases - certain medications can contribute to development including tetracycline-class antibiotics, vitamin A and retinoids, steroids, growth hormone, thyroxine, and lithium 2
• Post-treatment rebound headache can occur after treatment of intracranial hypotension and may indicate secondary intracranial hypertension 1
• Visual symptoms require urgent assessment as permanent vision loss is a serious complication 1
• Headache phenotype variability can lead to misdiagnosis as other primary headache disorders 1

Understanding this constellation of symptoms is crucial for early diagnosis and management to prevent serious complications such as permanent vision loss and neurological deterioration.