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  • Updated 03.06.2023
  • Released 04.22.1994
  • Expires For CME 03.06.2026

Normal pressure hydrocephalus



Normal pressure hydrocephalus is characterized by enlarged ventricles and tightened parasagittal cortical sulci with a characteristic clinical presentation of gait disorder, cognitive decline, and urinary incontinence. Shunt surgery is the only established effective treatment. In this updated article, the authors 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.

Historical note and terminology

The triad of progressive dementia, gait disturbances, and urinary incontinence was originally described as a distinct syndrome by Adams and colleagues (02; 38). Several other terms have been used to describe this condition, reflecting uncertainties about the pathogenesis of normal pressure hydrocephalus: "communicating," "low-pressure," "nonobstructive," "internal," "tension," "hydrostatic," "intermittent-tension," and "variotensive" hydrocephalus. The eponym "Hakim-Adams syndrome" is also occasionally used.

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