What are the symptoms of hydrocephalus?

Symptoms of Hydrocephalus

The symptoms of hydrocephalus include headache, nausea/vomiting, altered mental status, visual disturbances, and gait abnormalities, with specific presentations varying by age group and type of hydrocephalus. 1

Common Symptoms Across All Age Groups

• Headache is a common symptom, often worse when upright or with activity, and may be relieved by lying down 1
• Nausea and vomiting are particularly common in acute presentations 1
• Altered mental status, ranging from mild confusion to lethargy and obtundation 1
• Visual disturbances, including diplopia, blurred vision, and visual field defects 1
• Gait abnormalities, ranging from subtle changes to frank instability 1
• Cognitive changes and developmental delays, affecting quality of life 2

Age-Specific Presentations

Infants

• Increasing head circumference (macrocephaly) is the most common presentation 1, 3
• Bulging fontanel, often tense and non-pulsatile 1
• Splaying of cranial sutures, with progressive widening, especially of the sagittal suture 1
• Sunset eyes, with downward deviation of eyes and visible sclera above iris 1
• Irritability and lethargy, often fluctuating 1

Children (>2 years)

• Signs and symptoms of intracranial hypertension predominate 4
• Headache, nausea, and vomiting are common presenting symptoms 4
• Visual changes including diplopia and blurred vision 1
• School performance issues and cognitive changes 5

Young and Middle-Aged Adults

• Subtle symptoms with discrepancy between symptom severity and clinical findings 6
• Gait disturbances (70% of patients) that may be minor and easily missed 6
• Cognitive impairment (70% of patients) affecting job performance 6
• Urinary urgency (48% of patients) without frank incontinence 6
• Headaches (56% of patients) 6
• Impaired job performance (84% of patients) 6

Elderly

• Classic triad of normal pressure hydrocephalus: gait disturbance, urinary incontinence, and cognitive impairment 7
• Shuffling gait with difficulty initiating movement 7
• Progressive dementia 7
• Urinary incontinence that may begin as urgency 7

Symptoms by Type of Hydrocephalus

Acute Obstructive Hydrocephalus

• Rapid onset of symptoms, within hours to days 1
• Severe headache, often described as the worst headache of life 1
• Altered consciousness, ranging from confusion to coma 1
• Papilledema due to increased intracranial pressure 1
• Cranial nerve palsies, particularly affecting eye movements (Parinaud's syndrome) 1

Communicating Hydrocephalus

• May present with more gradual symptom progression 7
• Post-infectious cases common in lower-middle-income countries 7
• Post-hemorrhagic cases often follow subarachnoid or intraventricular hemorrhage 7

Congenital Hydrocephalus

• Present at or near birth 3
• Progressive macrocephaly in infants 3
• May be associated with other developmental abnormalities 3

Warning Signs Requiring Urgent Evaluation

• Sudden severe headache may indicate acute hydrocephalus or hemorrhage 1
• Rapid deterioration in mental status, from lethargy to obtundation 1
• New onset seizures, particularly in previously stable patients 1
• Bradycardia with hypertension, indicating critical elevation of intracranial pressure 1
• Respiratory irregularities, including apnea in infants 1

Clinical Pitfalls

• Symptoms may be subtle, especially in young and middle-aged adults, with discrepancy between prominent symptoms and subtle clinical signs 1, 6
• Patients with young and middle-aged adult hydrocephalus (SHYMA) are often followed for an average of 6 years before diagnosis 6
• Normal pressure hydrocephalus in the elderly may be misdiagnosed as other neurodegenerative conditions 7
• Postural headache may be confused with spontaneous intracranial hypotension 2
• Visual symptoms may be attributed to primary ophthalmologic conditions rather than increased intracranial pressure 1

Diagnostic Considerations

• Brain imaging (MRI or CT) is essential to document ventriculomegaly 7
• Contrast-enhanced MRI helps distinguish between communicating and non-communicating types 7
• Transependymal edema on imaging suggests acute hydrocephalus 7
• Lumbar puncture with opening pressure measurement may be helpful in communicating hydrocephalus 7
• Early diagnosis and treatment are critical to reduce morbidity and mortality from hydrocephalus 2