Normal Pressure

Hydrocephalus (NPH)
Definition

• First described in 1965 by Hakim and Adams

• Normal CSF pressure
• Ventriculomegaly
• Clinical triad:
• Slowly progressive gait disorder
• Impairment of mental function
• Sphincteric incontinence
Epidemiology

• 1 per 25.000
• Accounts for approximately 0.5-5% (up to 6%) of dementias
• One of the few treatable causes of dementia
• Most common in patient > 60 y/o
• Male > Female
Etiology

• Idiopathic: Elderly, unknown cause,

50% of NPH
• With a preceding cause: Young
• Subarachnoid hemorrhage (SAH)
• Trauma
• Meningitis (TB, syphilitic, etc.)
• Surgery, irradiation
• Storage disease (mucopolysaccharidosis)
 Pathophysiology of Hydrocephalus

Communicating hydrocephalus

Obstructive hydrocephalus
Physiology of CSF Flow
 Pathophysiology of NPH

Enlarged lateral Incontinence

ventricles

Gait disturbance

Dementia
Pathophysiology of NPH

• On the basis of both dynamic and ischemic factors

• Ventricular enlargement
• Vascular stretching → Ischemia
• Decreased compliance of ventricular wall
• High pulse pressure
• Barotrauma or shearing stress
Dynamics of NPH

• Transmantle pressure gradient

• Difference in pressure between ventricle and subarachnoid
space
• Gradient ↑ temporarily → Ventricle↑
• B wave (plateau)
• Transient elevations of mean and pulse pressure
• Water-hammer effect → Ventricle↑
• More than 50% of time
Dynamics of NPH

• Aqueductal CSF flow void

• Increased CSF flow velocity
• Favorable response to CSF diversion
• Aqueductal CSF stroke volume
• CSF pulsating back and forth through the aqueduct during
systole and diastole
• Favorable response to shunting
• Hyperdynamic CSF flow
Dynamics of NPH

• Saline infusion test

• CSF resorption in NPH is abnormal
• Arachnoid granulation? Arachnoidal villi?
• Venous compromise
• Increased transvenular resistance in superior saggital sinus cause
NPH
• What cause venous compromise? Microangiopathy? Deep white
matter ischemia?
Ischemia of NPH

• Acetazolamide challenge test

• Cerebral blood flow (CBF)↑ in normal person
• Failed to cause CBF↑ in NPH p’t
• Indicate the arterioles are already maximally dilated because of
ischemia
• CSF diversion → CBF improve and response to acetazolamide
Ischemia of NPH

• Compensatory CSF flow

• Periventricular white matter
• Increased interstitial fluid
• Loss of parenchymal compliance
Pathophysiology of NPH

• Dynamic
• Hyperdynamic CSF flow
• Impaired CSF resorption
• Ischemic
• Reduced CBF
• Periventricular white matter lesion
Diagnosis

• Clinical symptoms and signs

• Gait disturbance
• Dementia
• Urinary incontinence
• The moment when highly suspect NPH !!
• Image
• MRI (T2WI) with CSF flow study
• CT with lumbar puncture
Image Findings – CT Scan

• Ventriculomegaly
• Sulcal atrophy
• Ventriculosulcal disproportion
• Can DDx with other dementia
syndromes
Image Findings – CT Scan

• Rounded frontal and temporal

horns
• Periventricular lucency
• Transependymal CSF flow
• Corpus callosum thinning
Image Findings - MRI

• The same as CT
• Temporal horn out of
proprotion to hippocampal
atrophy
• Corpus callosum bowed
upward
Image Findings - MRI

• Periventricular lesions in
T2WI
• Transependymal CSF flow
• Deep white matter damage
Image Findings - MRI

• Aqueductal flow void sign

• Jet sign
• A jet of turbulent CSF flow on
the distal aqueduct
• Predictive of shunt
responsiveness
Image Studies – MRI

• CSF flow study

• Aqueductal stroke volume
• Increased velocity (hyperdynamic flow)
• VV/ICV ratio
• VV/ICV ratio > 30% (in 13 of 14 pts)
(VV: ventricular volume; ICV: intracranial CSF
space volume)
• MRS
• Intraventricular lactate peaks (ischemia)
Differential Diagnosis

• Dementia syndromes
• Alzheimer’s disease
• Hydrocephalus ex vacuo
• Intraventricular lactate

• Parkinsonism
• Parkinson’s disease
• Periventricular leukomalacia
Treatment

• Surgical shunting
• VP shunt
• Lumbar puncture
• Miller Fisher test: Gait assessment before and after 30mL CSF
drainage
(high rate of false negative)
• Continuous CSF drainage of 200 mL per day for 3-5 days
Complications of shunt

• Infection: S. aureus, S. epidermidis

• Subdural hematoma
• Shunt obstruction
• Low pressure state
• Epilepsy
• Pneumocephalus
• Ascites
Prognosis

• Response rate for shunt

• 50-70% with known preceding cause
• 30% with idiopathic group

• Non-selective patient
• 1/3 improve, 1/3 arrest, 1/3 deteriorate
Prognosis

• Positive response to shunting:

• Absence of central atrophy or ischemia
• Gait apraxia as dominant symptoms
• Prominent CSF flow void (stroke volume > 42 mL)
• Known history of cause (nonidiopathic type)