Normal Pressure

Hydrocephalus

Jerry Ryan MD
University of Wisconsin - Madison
Objectives
 1. Evaluate patients suspected of having NPH
and distinguish NPH from other causes of gait
disturbance, incontinence and dementia
 2. Identify patients who need referral for
consideration of treatment of NPH.
 3. Understand treatment of NPH and follow
patients who have received neurosurgical
interventions for NPH in the office.
 4. Educate patients with NPH and their families
about the disorder.
How big is the problem?
 Prevalence Normal Pressure Hydrocephalus
(NPH)
 Estimates vary from 0- 5% as a cause for
dementia
 Some of variation due to inconsistent
definition of NPH
 Study of 166 patients shunted for presumed
NPH calculated incidence of shunt responsive
NPH to be one patient per 2.2 million persons
per year
J Vanneste, P Augustijn, C Dirven, WF Tan and ZD Goedhart
Neurology 1992;42:54–9
So why do I need to know
about NPH?
 Potentially reversible cause of
significant morbidity
 Recent direct to consumer advertising
What should Family
Physicians know about NPH?
 Diagnostic features
 Diagnostic studies
 Limitations of prognostic studies
 Patients likely to benefit from treatment
 Complications of treatment
 Patient follow up
Etiology
 50% cases idiopathic
 Leading theory is impairment of CSF
outflow
 Intraventricular pressure studies reveal
waves of increased pressure- B-waves
 Adult hydrocephalus syndrome
 Adult symptomatic hydrocephalus
Etiology
 50% cases NPH secondary to other
illnesses
 Subarachnoid hemorrhage
 Meningitis
 Cranial trauma
 Secondary NPH has higher response
rate to shunting than idiopathic NPH
Pathophysiology
 Ventricle enlargement leads to
periventricular ischemia regardless of
etiology
 Compression and stretching of
arterioles and venules
 Arterial hypertension and cerebral
arteriosclerosis increased in NPH
CSF pathway
 CSF produced by choroid plexus at rate
approximately 20 ml/hr
 Flows from lateral ventricles through foramina
of Monro into third ventricle
 Enters fourth ventricle through aqueduct of
Sylvius
 Enters subarachnoid space
 Resorbed by arachnoid villi at top of brain
CSF pathway
Diagnostic Triad
 Gait Disturbance
 Urinary Incontinence

 Dementia
Diagnostic Triad
 Gait disturbance
 No classic gait disturbance
 Gait may be wide based, shuffling
 More severely affected patients have “magnetic gait”-
feet stuck to ground and difficult to initiate walking
 Difficulties with walking motions resolve with minimal
support of patient or lying patient down
 May resemble Parkinson’s gait
 Not associated with limb weakness
 Hyperreflexia
Diagnostic Triad
 Urinary Incontinence
 True incontinence found only in severely
affected patients
 Urinary urgency in most patients with NPH
 Due to stretching of periventricular nerve
fibers and loss of detrusor inhibition
 Bladder sphincter muscle unaffected
Diagnostic Triad
 Dementia
 Presence of dementia in NPH extremely
variable
 Some shunt responsive patients have little or
no dementia
 Dementia usually least responsive of symptoms
to intervention
 Mental status changes may resemble
depression
Differential Diagnoses-
Alzheimer’s (AD)

 Both AD and NPH cause memory impairment

 AD- “cortical” abnormalities

 Aphasia, Apraxia, Agnosia
 Impaired recognition and encoding deficits

 NPH- “subcortical” abnormalities

 Memory impairment but intact recognition
 Slow information processing
 Difficulty with complex tasks
 Cognitive Impairments AD
versus NPH
AD NPH
Memory
Learning
Psychomotor slowing
Orientation
Impaired Attention/concentration
Fine motor speed
Fine motor accuracy
Executive function
Writing
Auditory memory
Motor and psychomotor skills
Attention/concentration
Borderline Visuospatial skills
Executive function
Impaired Language
Behavior/personality
Reading
changes
Differential Diagnoses-
Alzheimer’s (AD)
 AD and NPH can usually be distinguished
with formal neuropsychological testing
 Primary care office testing may not be
adequate to distinguish
 Mental impairment early in course of AD but
usually late in course of NPH and often
minimal impairment
 AD often associated with hippocampal
atrophy on imaging studies
Differential Diagnoses-
Parkinson’s Disease
 Both NPH and Parkinson’s Disease
(PD) can have similar gait disturbances
 Hypokinesia
 Freezing
 Imbalance
 Extrapyramidal symptoms
 Trial of levadopa can help distinguish
between PD and NPH
Differential Diagnoses- Other
 Depression
 Subcortical arteriosclerotic encephalopathy
 Multi-infarct encephalopathy
 Chronic alcoholism
 B12, Folate deficiency
 Electrolyte abnormalities
 Cervical or lumbar stenosis
 Peripheral neuropathy
Diagnostic studies
 Ventricle enlargement on CT or MRI
 Severity graded by ratio of maximal frontal horn
width divided by transverse inner diameter of skull
 0.32 minimal for NPH but 0.40 more typical
 Lack of hippocampus or cortical atrophy
 Periventricular and cortical white matter
lesions may be found in patients with NPH
 Large number white matter lesions may be
marker for poor response to shunting
Normal Ventricles
Enlarged
Ventricles
Enlarged
Ventricles
Enlarged Ventricles
Enlarged
Ventricles
So now that I know it’s NPH
what next?
 Response to shunting varies
significantly between patients
 Study 166 shunted NPH patients
 Overall response 36%, only 21%
significant improvement
 Only 15% of patients with idiopathic NPH
showed marked improvement
J Vanneste, P Augustijn, C Dirven, WF Tan and ZD Goedhart
Neurology 1992;42:54–9
Any Problems With Shunting?
 Complications of shunting
 Low immediate post-surgical risks
 Severe to moderate shunt related morbidity
of 28%
 Infection
 Shunt malfunction
 Intracranial bleed
 Death or severe morbidity 7%
J Vanneste, P Augustijn, C Dirven, WF Tan and ZD Goedhart
Neurology 1992;42:54-9
Overdrainage
Are Benefits of Shunting Long
Lasting?
 Most studies show fairly significant
decline in benefits over time
 Initial improvement 60-75% of patients
 Sustained improvement only 24-42%
 Results confounded due to high
mortality from co-morbid conditions
 57% patients dead within 5 years in study
by Raftopoulos et.al.
How can I tell who will
benefit?
 Good response to shunting
 Clinical presentation
 Gait disturbance preceded mental impairment
 Short duration of mild mental impairment
 Known cause of NPH- e.g. infection, bleed
How can I tell who will
benefit?
 Good response to shunting
 Special studies
 Lack of white matter lesions on MRI
 Marked resolution of symptoms with CSF drainage
 One time removal 30-50 cc CSF
 Multi-day drainage of 100-150 cc CSF
 B-waves greater than 50% of time with continuous
intracranial pressure (ICP) monitoring
 Resistance to CSF outflow greater than 18 mmHg
How can I tell who will
benefit?
 Poor response to shunting
 Severe dementia
 Dementia presenting symptom
 MRI abnormalities
 Cerebral atrophy
 Multiple white matter lesions
How can I tell who will
benefit?
 Indeterminate significance
 Patient age
 Duration of symptoms
 Lack of response to removal CSF
How accurate are predictors of
response to shunting?

Normal pressure hydrocephalus: an update, Stein, SC, Neurosurgery

Quarterly (2001)11(1):26–35
How accurate are predictors of
response to shunting?

Normal pressure hydrocephalus: an update, Stein, SC, Neurosurgery Quarterly

(2001)11(1):26–35
NPH Guidelines
 Worldwide group of experts assembled to
develop guidelines for diagnosis and
treatment of NPH
 Meetings supported by shunt manufacturer
 Limited number of RCT’s noted by group
 Report published in Neurosurgery: Vol. 57
(3), Sept 2005 Supplement
Diagnosis- Probable Idiopathic
NPH
 History
 Insidious onset
 Age over 40
 Symptom duration 3-6 months
 No antecedent event known to cause secondary
NPH
 Progressive over time
 No other medical, psychiatric or neurological
condition that could cause symptoms
Diagnosis- Probable Idiopathic
NPH
 Brain imaging
 Ventricular enlargement not attributable to
cerebral atrophy or congenital disorder
 No macroscopic obstruction present
 At least one of the following
 Enlargement of lateral horns not attributable to
hippocampus atrophy
 Callosal angle greater or equal to 40 degrees
 Evidence of altered brain water content on imaging not
attributable ischemia or demylination
 An aqueductal or fourth ventricular flow void on MRI
Callosal angle
 Angle of roof of lateral ventricles in A-P projection
MRI flow void
 Loss of MRI signal due to flow of CSF

Normal aqueduct Abnormal aqueduct

MRI flow void

Normal fourth ventricle Abnormal fourth ventricle

Diagnosis- Probable Idiopathic
NPH
 Clinical
 Gait/Balance- at least two of following present
 Decreased step height
 Decreased step length
 Decreased cadence/speed
 Decreased trunk sway
 Widened stance
 Toes turned outward while walking
 En bloc turning- turns take three or more steps
 Impaired balance- two or more corrective steps for eight
steps on tandem gait testing
Diagnosis- Probable Idiopathic
NPH
 Cognition- two of following present
 Psychomotor slowing
 Decreased fine motor speed
 Decreased fine motor accuracy
 Difficulty dividing or maintaining attention
 Impaired recall especially for recent events
 Impairment of executive functions- multi-step
procedures, working memory, formulation of
abstractions, insight
 Behavioral or personality changes
Diagnosis- Probable Idiopathic
NPH
 Urinary Symptoms- one of following
 Episodic urinary incontinence not attributable to other
causes
 Persistent urinary incontinence
 Fecal and urinary incontinence
 OR
 One of following
 Urinary urgency
 Urinary frequency- 6 or more voids in 12 hour period
 Nocturia- more than two voids in night
Diagnosis- Probable Idiopathic
NPH
 Physiological
 Opening pressure 5-18 mmHg
Possible INPH
 History- Symptoms are
 Subacute or indeterminate onset
 Onset any time after childhood
 <3 months or indeterminate duration
 May follow trauma, hemorrhage or meningitis
 Symptoms not entirely explained by co-existing
neurological conditions
 Non-progressive or not clearly progressive
Possible INPH
 Brain imaging- Ventricular enlargement
associated with following
 Cerebral atrophy of sufficient severity to
explain ventricular enlargement
 Structural lesion that may increase
ventricular size
Possible INPH
 Clinical
 Incontinence and/or cognitive impairment
in absence of gait or balance dysfunction
 Gait disturbance or dementia alone

 Physiological
 Opening pressure unavailable or outside of
range for probable NPH
Unlikely INPH
 No ventriculomegaly
 Signs of increased intracranial pressure
such as papilledema
 No component of clinical triad
 Symptoms explained by other causes
(eg, spinal stenosis)
UCLA workup for NPH and
selecting shunt candidates
 Ventricular enlargement by CT or MRI (Evans
Index >0.3)
 Complete history and neurological exam,
neuropsychiatric testing and gait analysis
 Patients with significant dementia component
referred for more extensive evaluation to rule
out Alzheimer’s Disease of other forms of
dementia
UCLA workup for NPH and
selecting shunt candidates
 Patients felt at risk for NPH undergo
intracranial pressure monitoring
 Inserted with local anesthesia
 Fine wire placed just under calvarium
 Elevated pressure- shunt
 B-waves- further evaluation
UCLA workup for NPH and
selecting shunt candidates
 Cerebrospinal Fluid Outflow Resistance
 Lumbar puncture performed
 Artificial spinal fluid infused
 Rise in ICP recorded by previously inserted
ICP monitor
 Resistance to absorbtion of infused fluid
calculated
 High resistance- shunt
 Normal resistance- further testing
UCLA workup for NPH and
selecting shunt candidates
 Trial CSF drainage
 3 day trial
 Small volumes removed- 30-50 cc
 Improved symptoms- shunt
 No improvement- no further studies,
shunt no longer considered
UCLA workup for NPH and
selecting shunt candidates
 Studies not performed
 Cisternogram
 High volume CSF drainage
 PET scan
 SPECT scan
 Tests felt not warranted due to expense or
increased patient risk
 MRI flow void not routinely done as felt to be non-
specific
 Further testing felt to add minimal additional
prognostic information
Yet another workup

Treating Normal Pressure Hydrocephalus, Luciano, M,

AAFP CME Bulletin, 2004, Vol. 3 (4)
Yet another workup

Treating Normal Pressure Hydrocephalus, Luciano, M,

AAFP CME Bulletin, 2004, Vol. 3 (4)
What kind of shunt is used?
 Externally programmable valve allows
transcutaneous adjustment CSF outflow
resistance
What kind of shunt is used?
Shunt placement
Shunt Valve Adjustments
Now that my patient has had a
shunt what happens next?
 Monitoring of mental function
 Patients should have neuropsychiatric
testing prior to shunt
 Periodic testing post shunt to document
improvement
Now that my patient has had a
shunt what happens next?
 Monitor for complications of shunt
 Infection
 Shunt malfunction
 Excessive CSF drainage
 Subdural hematoma
Summary
 Best patients for shunt have gait
disturbance with mild mental impairment
 Improvement with CSF drainage predict
good response to shunt but lack of
improvement of limited prognostic value
 Patients with significant dementia and
limited gait disturbance unlikely to
benefit from shunt.
Cochrane Database
 Conclusion: There is no evidence to
indicate whether placement of a shunt is
effective in the management of NPH.
 Conclusion based upon lack of randomized
controlled trials
 Suggested references

 Diagnosis and management of normal pressure

hydrocephalus, Vanneste, JA, JNeurol (2000) 247:5–14
 Neurosurgery: Vol. 57(3) Supplement, September 2005
 Normal pressure hydrocephalus: an update, Stein, SC,
Neurosurgery Quarterly (2001)11(1):26–35
 University of California- Los Angeles-
http://www.neurosurgery.ucla.edu/Diagnoses/Adult/AdultDis_1.html
 University of Virginia-
http://www.healthsystem.virginia.edu/internet/neurogram/neurogram3_3_
nph.cfm