Characterized by the clinical features of hydrocephalus (i.e., levodopa-unresponsive gait apraxia with or without cognitive impairment or urinary symptoms), but without significantly raised cerebrospinal fluid pressure.
Cannot be reliably diagnosed using neuroimaging alone.
Differential diagnosis includes other causes of parkinsonism and common dementia disorders.
Responds to ventriculoperitoneal shunting if treated early enough.
Selection of patients for surgery is challenging as many are considered a high surgical risk.
Normal pressure hydrocephalus (NPH), also known as idiopathic normal pressure hydrocephalus (INPH), is a condition characterized by the clinical features of hydrocephalus (i.e., levodopa-unresponsive gait apraxia with or without urinary incontinence or cognitive impairment), but without significantly elevated cerebrospinal fluid (CSF) pressure as measured by lumbar puncture. Despite this, the condition responds to a reduction in CSF pressure and/or a CSF diversion procedure.
NPH is a confusing and misleading term. The word "hydrocephalus" is usually used to describe elevated CSF pressure in the brain. In NPH, there is no obvious buildup of CSF, and the pressure in the ventricular system of the brain is not considered significantly abnormal. Intracranial CSF pressure is slightly higher than normal, as CSF outflow resistance from the skull is relatively high. Some physicians doubt the existence of NPH.
History and exam
Key diagnostic factors
- levodopa-unresponsive gait apraxia
Other diagnostic factors
- cognitive impairment
- urinary frequency, urgency, or incontinence
- fecal incontinence
- age >65 years
- vascular disease
- diabetes mellitus
1st investigations to order
- CT head (without contrast) or MRI head
- levodopa challenge
Investigations to consider
- lumbar puncture
- lumbar puncture with large-volume cerebrospinal fluid (CSF) tap
- prolonged external lumbar drainage
- cerebrospinal fluid (CSF) infusion procedure
suitable for surgery
not suitable for surgery
Richard Adam Grünewald, MA, DPhil, FRCP
Honorary Clinical Senior Lecturer
Sheffield Teaching Hospitals NHS Foundation Trust
RAG has chaired a NICE guideline development group on neurological conditions, including recognition of movement disorders associated with normal pressure hydrocephalus. RAG received standard remuneration for chairing a NICE guideline development group. RAG is the author of a paper cited in this topic.
Dr Richard Grünewald would like to gratefully acknowledge Dr Jeremy Rowe, a previous contributor to this topic.
Robin Wilson, MD, PhD
Adult Hydrocephalus Center
Sandra and Malcolm Berman Brain & Spine Institute
RW declares that she has no competing interests.
- Parkinson disease
- Parkinson-plus syndromes
- Cerebrovascular parkinsonism
- Practice guideline: idiopathic normal pressure hydrocephalus: response to shunting and predictors of response
- Guidelines for the diagnosis and management of idiopathic normal-pressure hydrocephalus
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