Normal-pressure hydrocephalus

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By Peter Hedera MD and Robert P Friedland MD

Normal-pressure hydrocephalus is also known as or subsumes Communicating hydrocephalus, Hakim-Adams syndrome, Hydrocephalic dementia, Internal hydrocephalus, Nonobstructive hydrocephalus, Tension hydrocephalus, and Variotensive hydrocephalus. -ed.

Normal-pressure hydrocephalus is characterized by gait disorder, cognitive decline, and urinary incontinence, and shunt surgery is the only established effective treatment. In this updated clinical summary, Dr. Peter Hedera, Associated Professor of Neurology at Vanderbilt University, and Dr. Robert Friedland, Professor of Neurology at the University of Louisville, provide an overview of clinical features suggesting normal-pressure hydrocephalus as well as diagnostic approaches to establishing this diagnosis and selecting patients who have the highest likelihood of improvement after shunting. Although normal-pressure hydrocephalus is relatively rare, prompt recognition and surgical treatment of this condition can significantly reverse clinical features and improve the long-term outcome of these patients

Key Points

  • Normal-pressure hydrocephalus is characterized by gait disorder, cognitive decline, and urinary incontinence.
  • Prompt recognition and treatment may reverse clinical symptoms of normal-pressure hydrocephalus.
  • Diagnosis of normal-pressure hydrocephalus is based on a combination of clinical symptoms, neuroradiologic abnormalities, and transient improvement after CSF drainage.
  • Shunt surgery is the only established treatment of normal-pressure hydrocephalus.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited