Normal pressure hydrocephalus
From IDWiki
Background
- May be idiopathic (mostly the focus here) or secondary (to prior infection, ICH, TBI, or radiation)
- Idiopathic is more common in older patients and is somewhat a disease of aging
Clinical Manifestations
- "Wobbly, wet, and wacky"
- Earliest symptoms is gait instability/apraxia ("magnetic gait") progressing over months to years
- May be shuffling, magnetic, or wide-based
- Increased mediolateral variability during quiet standing
- Postural instability worsens with eye closure
- Next is dementia
- Cognitive symptoms include psychomotor slowing, forgetfulness, decreased attention/concentration, impaired executive function
- Apathy is a notable feature
- Usually last symptom is urinary incontinence, in about 50% of patients at diagnosis
- First as urgency with an inability to reach the bathroom in time, then later progressing to incontinence with apathy
- There should be no headaches, nausea/vomiting, visual changes, or papilledema
Differential Diagnosis
- Dementia with Lewy bodies
- Parkinson disease dementia
- Progressive supranuclear palsy
- Multiple system atrophy
- Corticobasal syndrome
- Alzheimer disease
- Vascular dementia
Investigations
- Should have normal CBC, lytes, creatinine, vit B12, TSH, calcium
- CT head showing hydrocephalus
- Lumbar puncture confirming normal pressure
- CSF glucose and protein are typically normal
Grading
| Grade | Gait | Cognition | Urinary Incontinence |
|---|---|---|---|
| 0 | Normal | Within normal range | Absent |
| 1 | Unsteadiness without objective abnormality | No clear dementia, but apathy present | Frequency and/or urgency without incontinence |
| 2 | Needs a cane | Needs support in social functioning, but remains independent at home | Occasional episodes at night |
| 3 | Needs a walker, or 2 canes | Requires partial assistance at home | Intermittent episodes during the daytime |
| 4 | Unable to ambulate | Fully dependent | Frequent urinary incontinence |
Management
- High-volume lumbar puncture (30 to 50 mL)
- Observe for improvement, particularly in gait
- If there is improvement in symptoms, consider implanting a ventriculoperitoneal shunt