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Cairan Serebrospinal

Normal anatomy and physiology:


In adults, the following features are encountered in posttraumatic hydrocephalus[9] :

Normal intracranial pressure (ICP) is approximately 8 mm Hg.

The average intracranial volume is about 1700 mL.

The average CSF volume is about 104 mL.

By volume, the intracranial contents include the following[11] :

Brain parenchyma - About 80%

CSF - About 10%

Blood - About 10%

CSF is primarily produced in the lateral ventricles by the choroids plexus at a rate of 500
mL/d. The CSF flows down toward the third ventricle through the foramen of Monro and into
the fourth ventricle through the cerebral aqueducts. The CSF then exits the ventricular system
through the foramen of Magendie (medially) and the foramen of Luschka (laterally) and
flows into the perimedullary and perispinal subarachnoid spaces. The CSF continues around
the brainstem to the basal and ambient cisterns. It then flows to the lateral and superior
surfaces of the cerebral hemispheres, where it is largely absorbed through the arachnoid villi.
The total volume of CSF is replaced several times daily.
http://emedicine.medscape.com/article/326411-clinical

Symptoms
The (gradually progressive) classic triad of symptoms is:[3]

Gait disturbance - this is due to distortion of the corona radiata by the dilated
ventricles. This area contains the sacral motor fibres than innervate the legs.
Movements are slow, broad-based and shuffling. The clinical impression is thus one
of Parkinson's disease, except that rigidity and tremor are less marked and there is no
response to carbidopa/levodopa. Freezing episodes can also occur. True ataxia and
weakness are absent and the gait disturbance is referred to as gait apraxia.

Sphincter disturbance - this is also due to involvement of the sacral nerve supply.
Urinary incontinence is predominant although bowel incontinence can also occur.

Dementia - this is due to distortion of the periventricular limbic system. The


prominent features are memory loss, inattention, inertia and bradyphrenia (slowness

of thought). The dementia progresses less rapidly than that seen with Alzheimer's
disease.

INPH is characterized clinically by the triad of gait disturbance, dementia and urinary
incontinence.[1,2] Symptoms typically develop insidiously, and generally occur
between the sixth and eighth decades of life.[11,12,13,14] Gait disturbances are typically
the first signs of INPH, and have been variously described as apraxic, bradykinetic,
glue-footed, magnetic, parkinsonian and shuffling.[8,15] Patients often present with a
history of falls. The aberrant ambulation observed in INPH is characterized by a slow,
wide-based gait, short shuffling steps, and difficulty in turning and tandem walking.
Notably, there is no significant motor weakness.

The cognitive deficits are typically of the subcortical type, characterized by


inattention, psychomotor retardation and difficulty with executive function.[1,16]
Apraxia, agnosia and aphasia are rare in INPH.

Urinary incontinence is the third primary symptom of INPH. In early stages of INPH,
urinary frequency and urgency are present. With disease progression, urinary and even
fecal incontinence can be observed. Urodynamic testing demonstrates bladder
hyperactivity.[17]

There is significant variation in the clinical presentation, severity and progression of


these symptoms, and the entire triad need not be present in order to consider a
diagnosis of INPH. Typically, however, gait and balance impairment appear either
before or concurrently with urinary incontinence or the onset of dementia. The full
diagnosis of INPH requires evidence from the patient's clinical history, physical
examination and neuroimaging.

An essential part of the evaluation of patients with suspected INPH is neuroimaging


with either CT or MRI to assess ventricular size (Figure 1). Although no findings on
imaging studies of the brain are sufficient on their own to diagnose INPH, ventricular
enlargement is necessary to establish the diagnosis of INPH for patients with
appropriate symptoms. A frontal horn ratio (Evans' index), defined as the maximal
frontal horn ventricular width divided by the transverse inner diameter of the skull,
signifies ventriculomegaly if it is 0.3 or greater.[8,18] Other radiographic findings
associated with INPH include the following: periventricular hyperintensities, which
are often associated with subcortical microvascular ischemia (so-called small-vessel
disease), but do not exclude the possibility of INPH; increased CSF flow velocity in
the aqueduct; thinning and elevation of the corpus callosum on sagittal images; and no
visible evidence of obstruction to CSF flow.[8,14,19,20,21,22]

Neuroimaging of two patients with idiopathic normal pressure hydrocephalus. (A)


Head CT scan demonstrating ventriculomegaly without significant cortical atrophy.
(B) Brain MRI demonstrating ventriculomegaly and evidence of subcortical ischemic
changes. Both patients' idiopathic normal pressure hydrocephalus symptoms
improved following shunt placement.

Several other brain imaging techniques have been investigated in patients with INPH,
including single-photon emission CT, PET, nuclear cisternography, and CSF flow
velocity. The diagnostic value of these tests has not been established, and, at present,
these examinations are not part of the routine work-up of patients with suspected
INPH.[7,8]

Normal-pressure hydrocephalus
Etiology
Approximately one half of all cases of normal-pressure hydrocephalus are idiopathic without
apparent cause. Subarachnoid hemorrhage, head trauma, infectious or carcinomatous
meningitis, and elevated CSF protein levels (including elevation due to intraspinal tumors)
are causes of secondary normal-pressure hydrocephalus (Black 1990). Normal-pressure
hydrocephalus may also result from aqueductal stenosis, arachnoid cysts of the third and
fourth ventricles, basilar artery ectasia, and intracranial and spinal tumors. A familial form of
normal-pressure hydrocephalus with autosomal dominant inheritance has been described, and
some patients also had coexisting essential tremor (Zhang et al 2010; McGirr and Cusimano
2012).

Clinical manifestations
By Peter Hedera MD and Robert P Friedland MD
Gait impairment associated with urinary incontinence and cognitive decline is suggestive of
normal-pressure hydrocephalus. Gait and stance difficulties are usually an early sign, and
they may appear before, shortly after, or simultaneously with intellectual changes. Urinary
incontinence is typically a late symptom. Gait disturbance may be a unique presentation of
normal-pressure hydrocephalus, and incontinence is the most commonly missing symptom
from the triad. Urinary urgency may sometimes precede the development of incontinence.
Fecal incontinence is rare and appears in more advanced stages. The clinical manifestations
of normal-pressure hydrocephalus are insidious and, if untreated, this condition is inevitably
progressive with gradual progression of dementia and advancement to an akinetic state.
Patients with normal-pressure hydrocephalus walk slowly, are unsteady, and have a wide
base. Steps are short with reduced step height, a pattern of gait referred to as "magnetic gait"
(Fisher 1982). Postural instability is also common, with increased risk of falls. Turning is
awkward and en block with multiple steps. Patients often complain about leg weakness,
tiredness, and occasional numbness or burning sensations. These symptoms fluctuate from
day to day. In advanced stages, standing, and later sitting, may become impossible.
Neurologic examination may reveal a mild degree of spasticity with increased muscle stretch
reflexes in the lower extremities, and Babinski signs also may be present. Upper extremities
are initially spared, but changes in handwriting, poor coordination on fine motor tasks, and
postural tremor have been reported in later stages.
The gait difficulties in normal-pressure hydrocephalus must be distinguished from those
caused by weakness. Lower extremity strength in normal-pressure hydrocephalus is

preserved. Motor abilities can be tested with patients sitting or lying down by asking them to
imitate walking, riding a bicycle, or other motions. Typically, they can perform these tasks
without obvious deficit, and these abilities are preserved until relatively late stages. This
pattern of gait disturbance indicates gait "apraxia" rather than muscle weakness.
Normal-pressure hydrocephalus may have additional manifestations beyond a classic triad.
Motor impairment may also affect the upper limbs, and slowing of rapid alternating
movements is seen commonly in patients with normal-pressure hydrocephalus; a successful
shunting of hydrocephalus also improves the dexterity in the upper extremities (Nowak et al
2006).
Cognitive decline varies widely in its severity. Dementia is characterized by prominent
attention deficit, memory impairment, and executive (frontal lobe) dysfunction. Most patients
are apathetic; bradyphrenia and depression must be considered. Aphasia, other than anomia,
and apraxia are usually not prominent. However, cognitive symptoms in normal-pressure
hydrocephalus are not limited to features of so-called subcortical dementia (eg, abulia,
bradyphrenia, distractibility, memory deficit). Focal cortical deficits (aphasia, apraxia,
agnosia) may occur as well, blurring the distinction between dementia in normal-pressure
hydrocephalus and the primary degenerative dementias. Behavioral symptomatology can be
marked in some patients. Akinetic mutism and alteration of consciousness are found in the
later stages.
Urinary incontinence occurs in about 4590%
of NPH patients. There is mostly a neurogenic
disturbance of micturation, especially as the way to the toilet is more difficult due to the
presence of the gait disturbance 4. In the further course a functional disorder of the frontal
lobe prevents recognition of the urinary urge. The bladder disorders in NPH are the result of a
hyperactive detrusor muscle due to reduced or absent central inhibition. At first the patient
suffers from an increased micturition frequency. In the further course, urge incontinence
usually follows and can progress to an absolute urinary incontinence. Faecal incontinence
usually only occurs in the advanced stages 1. The presence of 2 of the 3 criteria is necessary
to make the diagnosis, the gait disturbance is considered as obligatory. Diseases such as
Parkinsons disease, Alzheimers disease, vascular dementia or Lewy body disease are some
of the differential diagnoses.
Pathogenesis and pathophysiology
By Peter Hedera MD and Robert P Friedland MD
Hydrocephalus in normal-pressure hydrocephalus is a consequence of the disequilibrium
between production and absorption of CSF. It is thought that, in the majority of cases,
ventricular enlargement results from an obstruction of the CSF flow around the brain
convexities and insufficient absorption through the arachnoid granulations and arachnoid villi
of the superior sagittal sinus. Subarachnoid hemorrhage, meningitis, head injuries, and
elevated levels of CSF protein all cause scarring and fibrosis in the subarachnoid space.
Deficiency of the arachnoid granulations and obstruction of the pacchionian villi due to
meningioma of the superior sagittal sinus are other examples of this mechanism. This subtype
of normal-pressure hydrocephalus is often referred to as communicating hydrocephalus.
Communication between the ventricles and subarachnoid space is unobstructed; however, the
communication between the subarachnoid space and arachnoid granulations is not intact.
Other lesions (intracranial tumors, cerebellar hematomas, nontumorous stenosis of the
aqueduct of Sylvius, arachnoid cysts) can result in normal-pressure hydrocephalus due to

obstruction of CSF circulation within the ventricular system. This subtype of normal-pressure
hydrocephalus is sometimes called noncommunicating.
A relatively large proportion of normal-pressure hydrocephalus patients do not have an
identifiable cause of hydrocephalus. The preponderance of evidence in these cases points to
reduced absorption of CSF. Patients with normal-pressure hydrocephalus (both idiopathic and
secondary) have increased resistance to outflow (ROUT) of CSF (ROUT, mmHg/mL per
minute), suggestive of an impediment of CSF absorption (Kosteljanetz 1986). This has been
corroborated by pathological studies showing fibrotic changes of the leptomeninges and
pacchionian villi (Bech et al 1997).
Normal opening pressure of CSF and abnormally high outflow resistance indicate that normal
pressure is an endpoint of previously elevated intracranial pressure (Kosteljanetz 1986).
Indeed, intracranial pressure is not always normal, and long-term monitoring has shown
transient elevation of intracranial pressure. Thus, elevation of intracranial pressure causes
enlargement of ventricles, and a new balance is reached with normal (ie, nonelevated)
pressure but higher force, affecting a bigger ventricular surface according to Pascal's law of
pressure in fluids (Hakim and Adams 1965). Reduced compliance of the brain, further
enhanced by degenerative changes of the periventricular white matter, may also contribute to
abnormal distensibility of the ventricles. Increased CSF pulse amplitude is another possible
explanation for the development of hydrocephalus despite normal intracranial pressure (Foltz
and Aine 1980). It is interesting in this regard that hypertension, with a potential to contribute
to higher pulse amplitude, is positively associated with normal-pressure hydrocephalus
(Graff-Radford and Godersky 1987).
The development of the clinical symptoms of normal-pressure hydrocephalus is
multifactorial. Motor fibers innervating the legs and sphincters project through the vicinity of
the frontal horns of the lateral ventricles. The frontal horns of the lateral ventricles are often
disproportionally expanded. Stretching of these fibers is thought to be responsible for gait and
urinary symptoms. Volumetric changes after CSF removal are most pronounced in the
periventricular region of the lateral ventricles and in the frontal and temporal lobes (Singer et
al 2012). Corticospinal tract fibers show signs of axonal degeneration, which can be clinically
assessed by diffusion tensor imaging (Hattori et al 2011; Jurcoane at al 2014).
Early urinary involvement is characteristic of the loss of voluntary supraspinal control with
bladder hyperactivity and detrusor instability, which is manifested as urinary urgency. Late
frank incontinence also has a frontal component with indifference and lack of concern. Many
symptoms of cognitive decline can also be accounted for by compression of the frontal white
matter (deficits in attention, initiation, and other executive functions).
Compromised microcirculation due to increased intraparenchymal pressure is another
putative factor in the pathogenesis of dementia and the apractic features of gait. Studies of
regional cerebral blood flow in normal-pressure hydrocephalus have shown regional
reduction of blood flow in the periventricular white matter with gradual improvement toward
the cortical regions. Induction of increased intracranial pressure further reduced cerebral
blood flow in the subcortical regions (Momjian et al 2004).
Impaired vascular autoregulation with reduced cerebrovascular reactivity, suggesting a
limited capacity to compensate for transitional increases of intracranial pressure, is also a
feature of normal-pressure hydrocephalus (Chang et al 2009). Clinical changes do not tightly
correlate with regional cerebral blood flow changes; the degree of clinical improvement has
been disproportionally higher than changes of regional cerebral blood flow. This suggests that

additional factors (such as demyelinization and decreased clearance of various


macromolecules) may play roles in the pathogenesis of dementia in normal-pressure
hydrocephalus (Silveberg et al 2002; Tullberg et al 2002).
Results of quantitative studies of the brain metabolism are more consistent. Jagust and
colleagues found global reduction of glucose metabolism using PET; patients with normalpressure hydrocephalus had a different pattern compared to patients with Alzheimer disease
(Jagust et al 1985). The decrease of glucose uptake in normal-pressure hydrocephalus is
lower than expected for the degree of dementia and suggests widespread cortical and
subcortical dysfunction. The relationship between symptoms of normal-pressure
hydrocephalus and alterations of blood flow and cerebral metabolism is not firmly
established. Clinical motor symptoms may be caused by blood flow abnormalities in the
supplementary motor cortex, causing reversible suppression of frontal periventricular
corticobasal ganglia-thalamocortical pathways. This area showed improved blood flow after a
prolonged lumbar drainage, suggesting that motor function recovery in normal-pressure
hydrocephalus patients after CSF removal is related to enhanced activity in medial parts of
frontal motor areas that are important for motor planning (Lenfeldt et al 2008).
Impaired corticospinal excitability, assessed by transcranial magnetic stimulation, can result
in disinhibition of the motor cortex and, thus, plays a key role in mediating the effects of
frontal lobe dysfunction on motor performance in patients with normal-pressure
hydrocephalus (Chistyakov et al 2012). These abnormalities likely reflect a disturbed
connectivity of the frontal motor networks rather than a direct lesion of the primary motor
cortex or corticospinal tract, and shunting can normalize transcranial magnetic stimulation
responses
Ischemia of deep white matter may not be a mandatory precondition for the development of
normal-pressure hydrocephalus because not every patient with normal-pressure
hydrocephalus has abnormal blood flow studies; however, secondary microcirculation
changes may be caused by stretching of periventricular white matter due to ventriculomegaly.
MR imaging studies with flow quantification measuring the total blood inflow, sagittal and
straight sinus outflow, aqueduct stroke volume, and arteriovenous delay did not detect any
abnormalities in the periventricular regions, and the main changes showed alterations in
superficial venous compliance and a reduction in the blood flow returning via the sagittal
sinus (Bateman 2008). The overall load of the white matter changes also negatively
correlated with the gait improvement after shunting, further supporting the notion that white
matter lesions contribute to the irreversibility of symptoms in normal-pressure hydrocephalus
but not to the pathophysiological mechanisms that lead to them (Bugalho and Alves 2007).
Abnormalities of jugular venous system draining the blood from the brain can contribute to
abnormal absorption of CSF. Incompetent jugular valves may allow the transmission of the
high venous pressures to the subarachnoid space. This hypothesis is supported by the findings
of retrograde jugular flow during Valsalva maneuver in 95% patients with normal pressure
hydrocephalus, and this finding is rare in apparently normal controls (Kuriyama et al 2008).
This test can be also used for the diagnosis of normal pressure hydrocephalus, even though its
specificity and sensitivity have not been established in a larger cohort of patients.
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