Disorders of CSF

Reynaldo B. Sta. Mina, Jr., M.D.
DISORDERS OF THE
VENTRICULAR SYSTEM
MED 721 (CLINICAL NEUROLOGY)
CONTENTS
▪ Chiari Malformation
▪ Learning Objectives
 Definition and Epidemiology
▪ Introduction  Etiology and Pathophysiology
 The Cerebrospinal Fluid and Its Circulation  Differential Diagnosis
 YouTube video links  Clinical Features
 Investigations and Diagnosis
▪ Aqueductal Stenosis with Hydrocephalus
 Treatment
 Prognosis
 Etiology and Pathophysiology
 YouTube video links
 Differential Diagnosis
 Clinical Features ▪ Syringomyelia/Syringohydromyelia
 Investigations and Diagnosis  Definition and Epidemiology
 Treatment  Etiology and Pathophysiology
 YouTube video links  Differential Diagnosis
 Clinical Features
▪ Communicating Hydrocephalus
 Treatment
 Prognosis
 Investigations and Diagnosis ▪ Idiopathic Intracranial Hypertension/Pseudotumor Cerebri
 Treatment  Definition and Epidemiology
 Prognosis  Etiology and Pathophysiology
 YouTube video links  Clinical Features
▪ Normal Pressure Hydrocephalus
 Treatment
 Prognosis
 Treatment
 Prognosis
▪ Intracranial Hypotension Syndrome
 Investigations
 Imaging
 Other Investigations
 Treatment
 Prognosis
▪ Clinical Cases
▪ Main Reference
▪ Related Readings
LEARNING OBJECTIVES
▪ To review the anatomy and physiology of the

ventricular system of the brain and spinal cord, as well
as the dynamics of CSF circulation
▪ To identify and get familiarized with the more common
disorders that involve the CSF circulation and the
ventricular system
▪ To know the epidemiology, etiology, pathophysiology,
clinical features, differential diagnosis, investigations,
treatment, and prognosis for such disorders
▪ To understand the clinical correlations related to such
disorders
INTRODUCTION
THE CEREBROSPINAL FLUID AND ITS CIRCULATION
▪ Cerebrospinal fluid (CSF) is produced by the choroid plexus
within the ventricles (mainly residing in the roof of the third
ventricle and the floors of the lateral ventricles) at a rate of
approximately 0.35 ml/min or 500 ml/24 hr.
▪ Total CSF volume is estimated to be approximately 150 ml. It is
larger in men than women, and larger in older individuals than
younger individuals due to an increase in ventricular size with
age.
▪ CSF circulates downward from the lateral and third ventricle,
through the cerebral aqueduct into the fourth ventricle where it
exits into the subarachnoid space around the brain, and
communicates with the subarachnoid space around the spinal
cord.
▪ Spinal CSF volume is estimated to be about 50 ml.
▪ CSF is absorbed back into the superior sagittal sinus through the
arachnoid villi, via a valve-like one-way mechanism that prevents
sinus blood from entering into the subarachnoid space.
CSF is produced at the floor of the lateral ventricles and the roof of the third
ventricle by the choroid plexus. It then flows downward through the aqueduct of Sylvius
into the fourth ventricle and out into the subarachnoid space through the foramen of
Magendie and the paired foramina of Luschka. CSF flows downward into the spinal
subarachnoid space and upward around the cerebral convexities, where it is eventually
resorbed back into the superior sagittal sinus by the arachnoid villi.
▪ Resorption of CSF at the arachnoid villi requires a positive
pressure in the subarachnoid space of at least 60 mm of
water.
▪ At CSF pressures of less than 60 mmH2O, resorption
probably does not occur at the arachnoid villi.
▪ Normal CSF pressure in the recumbent position is 65–195
mmH2O.
▪ In the recumbent position, CSF pressure is equalized
between the ventricles and the brain and spinal
subarachnoid spaces.
▪ In the erect position, a pressure gradient forms with lower
pressures at the vertex (sometimes negative) and higher
pressures in the lumbar subarachnoid space.
YouTube Video Links
▪ Cerebrospinal Fluid
 https://www.youtube.com/results?search_query=cere
brospinal+fluid
▪ Cerebrospinal Fluid Flow/Circulation

 https://www.youtube.com/results?search_query=cere
brospinal+fluid+flow
▪ Ventricular System
 https://www.youtube.com/results?search_query=vent
ricular+system+of+the+brain
AQUEDUCTAL STENOSIS WITH
HYDROCEPHALUS
DEFINITION AND EPIDEMIOLOGY
▪ Aqueductal stenosis is the most common cause of noncommunicating
hydrocephalus.
▪ In hydrocephalus due to aqueductal stenosis, ventricles are variously
enlarged depending on the severity of the stenosis and the compliance
(stiffness) of the ventricles.
▪ The hydrocephalus of aqueductal stenosis is classified as
‘noncommunicating’ because there is either incomplete or absent
communication between the lumbar subarachnoid space and the lateral
ventricles of the brain.
▪ Aqueductal stenosis with hydrocephalus may become apparent either in
infancy (shortly after birth) or in adult-hood.
▪ The incidence of congenital hydrocephalus at birth is estimated to be
0.2–0.8 children per 1000 live births.
▪ Accurate figures of incidence of adult onset aqueductal stenosis are not
available.
ETIOLOGY AND PATHOPHYSIOLOGY
▪ Since the obstruction in aqueductal stenosis is above the
fourth ventricle, the lateral and third ventricles are enlarged
whereas the fourth ventricle is small or normal-sized.
▪ In many cases, the stenosis at the aqueduct is incomplete
causing a decrease in CSF flow through the aqueduct rather
than complete blockage.
▪ Complete blockage at the aqueduct is generally not
compatible with survival in the absence of a CSF diversion
procedure.
▪ Aqueductal stenosis (narrowing of the cerebral aqueduct)
may be due a variety of causes:
 Congenital narrowing.
 Midbrain tumors.
 Perinatal intraventricular hemorrhage.
 Meningitis.
 Ventriculitis.
Congenital aqueductal stenosis with severe hydrocephalus not compatible with survival.
DIFFERENTIAL DIAGNOSIS
▪ The etiology of aqueductal stenosis should be
established when possible.
 Meningitis, ventriculitis, peri-aqueductal tumors, and
intraventricular hemorrhages as causes should be
distinguished from congenital aqueductal stenosis.
 Other causes of hydrocephalus should be considered
including communicating hydrocephalus and normal
pressure hydrocephalus (NPH).
 Distinguish aqueductal stenosis from macrocephaly (a large
head and brain without hydrocephalus).
 Distinguish aqueductal stenosis from hydrocephalus ex-
vacuo (enlargement of the ventricles due to cortical
atrophy).
CLINICAL FEATURES
▪ Adult-onset patients with aqueductal stenosis generally
have headache.
▪ Other frequent symptoms include:
 Gait disorder.
 Memory impairment.
 Urinary incontinence.
▪ The key symptoms of patients with adult-onset aqueductal
stenosis closely resemble those of patients with NPH.
▪ Less common symptoms include:
 Blurred vision.
 Tremor.
 Parinaud’s syndrome (paralysis of upgaze with large pupils).
INVESTIGATIONS AND DIAGNOSIS
▪ Magnetic resonance imaging (MRI) and computed
tomography (CT) show enlarged lateral and third
ventricles with a normal or small fourth ventricle.
▪ Phase contrast MRI is a method for determining if
there is flow in the aqueduct. This test can be useful
in confirming the diagnosis of aqueductal stenosis
and the need for shunting or ventriculostomy of the
third ventricle.
▪ In cases of aqueductal stenosis, radionuclide
cisternography will show no tracer entering the
ventricles from the lumbar subarachnoid space.
Hydrocephalus due to aqueductal stenosis. CT scan shows enlarged lateral and third
ventricle. The fourth ventricle is normal in size. The cerebral aqueduct is difficult to
visualize on CT.
CT scan of aqueductal stenosis. Note the massively enlarged lateral and third ventricles
Aqueductal stenosis with enlarged lateral and third ventricles. MRI shows narrowed
cerebral aqueduct and small fourth ventricle.
TREATMENT
▪ Most patients are treated with ventricular drainage
by use of shunt with valves.
▪ Current treatment usually involves placement of an
adjustable valve (programmable) to allow the
amount of drainage to be adjusted:
 Some shunts may under-drain. Under-drainage may be
indicated by intracranial pressure (ICP) greater than 200
mmH2O when lying flat.
 Some shunts may over-drain. Over-drainage is indicated by
negative ICP of 100 mmH2O or more when standing or
sitting up.
 Some patients are being treated by ventriculostomy (third
ventriculostomy) which obviates the need for a shunt.
PROGNOSIS
▪ The main problem with treatment of aqueductal stenosis by ventriculo-
peritoneal shunting is the high complication rate, including:
 Shunt infection.
 Over-drainage.
 Subdural hematoma.
 Subdural hygroma.
▪ At 5 years, the complication rate after shunting can be as high as 50%.
▪ Some adult-onset aqueductal stenosis patients with hydrocephalus are
asymptomatic and can be followed longitudinally.
▪ Symptomatic aqueductal stenosis patients who are not shunted are
likely to have continuation of their headache, as well as progressive
difficulties with gait, cognition, and sphincter control.
YouTube Video Links
▪ Cerebral Aqueduct (of Sylvius)

 https://www.youtube.com/results?search_query=
cerebral+aqueduct+of+sylvius
▪ Aqueductal Stenosis with Hydrocephalus

aqueductal+stenosis+hydrocephalus+
COMMUNICATING HYDROCEPHALUS
▪ In communicating hydrocephalus the ventricles are enlarged but
the ventricles freely communicate with the lumbar subarachnoid
space.
▪ The usual cause of communicating hydrocephalus is blockage of
absorption of the CSF (either in the subarachnoid space around
the brain or at the arachnoid villi or pacchionian granulations
where resorption occurs).
▪ ICP is usually increased except in cases of NPH.
▪ Communicating hydrocephalus in adults is uncommon but not
rare.
▪ Prevalence rates in the USA are estimated to be between 1 and 2
per 1000 persons.
▪ No gender differences in incidence or prevalence of
communicating hydrocephalus have been established.
▪ In most cases of communicating hydrocephalus the flow of CSF is
blocked in the subarachnoid space over the convexities of the brain, or
resorption of CSF is blocked at the arachnoid villi along the sagittal
sinus.
▪ In communicating hydrocephalus, the ventricular system communicates
freely with the lumbar subarachnoid space.
▪ Due to problems in resorption of CSF at the arachnoid villi, intracranial
CSF volume and ICP increases. This increase in ICP causes the ventricles
to enlarge. With increased ICP, CSF resorption increases and equilibrium
between production and resorption is re-established.
▪ Usual causes of communicating hydrocephalus include:
 Infection (meningitis, ventriculitis).
 Inflammation (neurosarcoidosis, lupus cerebritis).
 Hemorrhage (intraventricular hemorrhage, aneurysmal subarachnoid
hemorrhage).
▪ Communicating hydrocephalus needs to be distinguished
from other causes of ventricular enlargement, such as:
 Hydrocephalus due to aqueductal stenosis.
 Ventricular enlargement due to cortical atrophy (hydrocephalus
ex vacuo).
CLINICAL FEATURES
▪ Headache is common with communicating hydrocephalus.
If ventricular enlargement is marked and ICP increased,
lethargy, obtundation, or even coma can ensue.
▪ Frontal lobe dysfunction is common. Mental slowness,
cognitive impairment, and abulia are clinical features of
communicating hydrocephalus.
▪ Gait disorders are common with a shuffling slow gait that
may be confused with Parkinson’s disease.
▪ Progressive ventricular enlargement with frontal lobe
dysfunction can lead to urinary incontinence and sphincter
disturbances.
▪ MRI and CT will confirm ventricular enlargement
that is disproportionate to any cortical atrophy.
▪ In communicating hydrocephalus, all ventricles
including the fourth ventricle are enlarged.
▪ Lumbar puncture usually shows elevated ICP except
in cases of NPH.
▪ An examination of the CSF is essential to
establishing etiology of the communicating
hydrocephalus:
 Cultures for bacteria, such as M. tuberculosis, and fungi
should be obtained to exclude acute or subacute infection.
 Pleocytosis may point to either an inflammatory or
infectious cause for the communicating hydrocephalus.
 Elevated CSF protein with minimal pleocytosis may suggest
neurosarcoidosis or other noninfectious inflammatory
processes that can produce communicating hydrocephalus.
TREATMENT
▪ Treatment for communicating hydrocephalus includes treatment
of any underlying conditions.
▪ Most cases are treated with a ventriculo-peritoneal shunt.
▪ Recently third ventriculostomy has emerged as an alternative to
ventriculo–peritoneal shunting.
PROGNOSIS
▪ Most patients improve dramatically with ventriculo-peritoneal
shunting and treatment of any underlying etiological illnesses.
▪ Shunt failures and shunt infections may be common
complications.
▪ Other problems may include the shunt over-drainage syndrome
with headache, subdural hematoma, or subdural hygroma.
▪ Shunt revisions are commonly required.
YouTube Video Links
▪ Hydrocephalus
hydrocephalus
▪ Communicating Hydrocephalus
communicating+hydrocephalus
NORMAL PRESSURE HYDROCEPHALUS
▪ NPH should be considered a special instance of communicating
hydrocephalus with normal ICP.
▪ NPH is often recognized because of the clinical triad of gait
disorder, urinary incontinence, and cognitive impairment.
▪ The exact prevalence of NPH is unknown.
▪ In the USA, the total number of cases is estimated to be about
175,000 or 1 per 2000 persons.
▪ The prevalence in the elderly is much higher, possibly as high as 1
in 200 persons.
▪ Among persons with dementia, the prevalence is at least 1 in 100
or higher.
▪ No gender differences in prevalence or incidence have been
established.
▪ In NPH, ventricular enlargement occurs in the absence of
an increase in ICP .
▪ The degree of ventricular enlargement is disproportionate
to any cortical atrophy.
▪ The ventricles enlarge due either a block in flow of the CSF
in the subarachnoid spaces, or a failure to resorb CSF at the
arachnoid villi.
▪ Some cases of NPH appear to be entirely idiopathic with no
obvious precipitating cause.
▪ Other cases can be related to prior head trauma, brain
surgery, subarachnoid hemorrhage, or meningitis.
▪ Conditions that can either sclerose the subarachnoid CSF
pathways or damage the arachnoid villi seem especially
prone to cause NPH.
MRI (T2-weighted image) shows ventricular enlargement in normal pressure hydrocephalus
(NPH). Note the ischemic infarct in the right thalamic area. Ischemic changes are often
noted in elderly patients with NPH.
▪ NPH must be distinguished from hydrocephalus originating in
childhood such as aqueductal stenosis or communicating
hydrocephalus.
▪ If hydrocephalus begins before age 6 years when skull sutures are
closing, head circumference will generally be increased (above 59
cm in men and above 57.5 cm in women).
▪ NPH should be distinguished from other neurologic conditions
that produce a shuffling gait such as Parkinson’s disease, Lewy
body dementia, frontotemporal dementia, stroke, and
Alzheimer’s disease.
▪ Other causes of gait disorder should be considered including
cervical spondylosis with myelopathy, lumbar stenosis, cauda
equina syndrome, polyneuropathy, and spinocerebellar
atrophies.
CLINICAL FEATURES
▪ Patients with NPH often do not have classic features
of hydrocephalus associated with increased ICP such
as headache, nausea, or vomiting.
▪ The diagnosis is usually made when the triad of
urinary incontinence, gait disorder, and cognitive
impairment is observed in the setting of enlarged
ventricles.
▪ Because of the many other possible causes for the
symptoms of NPH in the elderly that must be ruled
out, the existence of NPH as an independent
condition has been viewed with skepticism by some.
▪ Either MR or CT scanning will show enlargement of the ventricles
consistent with NPH .
▪ Some enlargement of the subarachnoid space over the
convexities of the brain does not definitely rule out NPH but may
cast doubt on the diagnosis.
▪ Some patients have coexisting cerebrovascular disease and some
patients will remain candidates for shunting for NPH, despite the
presence of deep lacunar infarcts on CT or MR.
▪ Patients with severe cortical atrophy on CT or MRI are unlikely to
be responsive to ventriculo-peritoneal shunting.
▪ A positive response to CSF drainage after 72 hours is a positive
predictor of shunt responsiveness in patients with possible NPH.
▪ Continuous CSF pressure monitoring has not proven to be
predictive of shunt responsiveness.
CT scan showing ventricular enlargement in normal pressure hydrocephalus. Both lateral
ventricles are enlarged. Note the absence of cortical atrophy.
TREATMENT
▪ Patients who are felt to be appropriate candidates for ventriculo-peritoneal
shunting can be treated with shunts with programmable valves.
▪ A programmable valve minimizes the risk of siphoning or over-drainage of the
ventricles and may be useful in preventing either subdural hygromas or subdural
hematomas.
PROGNOSIS
▪ Patients with NPH have a higher risk of dying than the general population.
▪ One study found that patients with NPH were 3.3 times more likely to die than a
comparably aged population9.
▪ After shunting, gait is improved in 64% at 3 months and 26% are improved at 3
years.
▪ Fewer than 10% show sustained improvement on cognitive tests 5 years after
shunting.
▪ Nearly one-half of patients who are shunted will require shunt revision within 3
years for shunt malfunction or infection.
YouTube Video Link
▪ Normal Pressure Hydrocephalus

normal+pressure+hydrocephalus
CHIARI MALFORMATION
▪ Chiari Type I malformation is downward herniation of the
hindbrain through the foramen magnum.
▪ Herniation always involves the cerebellar tonsils.
▪ In more severe cases the medulla and fourth ventricle may
descend below the foramen magnum.
▪ Chiari Type I has an estimated prevalence of 1 in 1000
persons.
▪ Chiari Type II malformation is a more severe malformation
with the cerebellar tonsils herniating below the foramen
magnum. Myelomeningocele is always present. About 80%
will have hydrocephalus and 40–80% will have
syringomyelia of the spinal cord. Elongation and kinking of
the medulla may also occur.
MRI (T1-weighted image) shows downward herniation of the cerebellar tonsils below the
foramen magnum in a patient with Chiari malformation Type I.
Pathologic specimen showing impression of the foramen magnum on the cerebellum in a
patient with Chiari malformation Type II. Note the abnormality of lower brainstem and
upper spinal cord.
▪ The cause of Chiari Type I malformation is a volume discrepancy
between the posterior fossa and the neural components of the
posterior fossa (cerebellum, fourth ventricle, and brainstem).
▪ Oversized structures in the posterior fossa cause these structures
to descend caudally below the foramen magnum.
▪ The exact cause of the Chiari Type I malformation is unknown.
▪ No gene abnormality has been identified although familial
clustering of cases has been reported.
▪ Chiari Type I malformation must be differentiated from a variety
of conditions:
 Neoplasms in the vicinity of the foramen magnum.
 Intrinsic neoplasms of the cerebellum and lower brainstem.
 Low-lying cerebellar tonsils due to intracranial hypotension syndrome.
 Multiple sclerosis.
 Syringomyelia without Chiari malformation.
CLINICAL FEATURES
▪ Patients with Chiari Type I malformation commonly have neck
pain and headache.
▪ The headaches may be severe and paroxysmal or steady and dull.
▪ The Valsalva maneuver may exacerbate the headache.
▪ Compression of the posterior fossa structures can cause lower
brainstem symptoms:
 Nystagmus.
 Cerebellar ataxia.
 Dysphagia and aspiration.
 Facial numbness and pain.
 Sleep apnea.
 Hoarseness.
 Tongue atrophy.
▪ Chiari Type I malformation can be demonstrated on MRI.
▪ Gadolinium-enhanced images are needed to exclude a
posterior fossa tumor.
▪ MRI is adequate to exclude hydrocephalus as a cause of the
low-lying tonsils.
▪ In questionable cases, phase contrast MRI can determine
whether the flow of CSF is impaired across the cranio-
cervical junction.
▪ Brainstem auditory evoked responses and somatosensory
evoked responses may demonstrate slowing consistent
with brainstem or spinal cord compression related to Chiari
Type I malformation.
TREATMENT
▪ No medical treatment is effective for Chiari Type I malformation.
▪ Surgical treatment of Chiari I involves decompression of the
foramen magnum and restoration of normal CSF flow.
▪ Some surgeons open the dura in the posterior fossa and add a
graft (duraplasty); others do not manipulate the dura.
▪ Surgical management of Chiari II is complex and involves closure
of the myelomeningocele. CSF shunting and decompression of
the posterior fossa and upper cervical spinal cord may be needed.
PROGNOSIS
▪ Approximately 65–90% of patients with Chiari Type I
malformation show clinical improvement after posterior fossa
decompression.
▪ Prognosis in Chiari Type II malformation depends upon the
severity and complexity of the malformation.
YouTube Video Link
▪ Chiari Malformation
chiari+malformation
SYRINGOMYELIA
(SYRINGOHYDROMYELIA)
▪ Syringomyelia (syringohydromyelia) is the presence of a fluid-filled
cavity within the spinal cord10.
▪ It is a rare disorder, and exact incidence and prevalence are unknown.
▪ The cause of syringomyelia is unknown.
▪ Low-lying cerebellar tonsils likely play a role in many cases.
▪ Normally, during the cardiac cycle CSF flows freely across the foramen
magnum (downward during cardiac systole and upward during cardiac
diastole). When the cerebellar tonsils are low (as in Chiari I and II
malformations), the free flow of CSF is obstructed and backs up into the
cranial cavity during diastole. CSF may become trapped in the cervical
subarachnoid space, eventually tracking into the substance of the spinal
cord causing a syrinx to form.
▪ Syringomyelia may occur due to a variety of disease processes
including spinal cord trauma with myelomalacia, Chiari I
malformations, Chiari II malformations, spinal cord tumors,
communicating hydrocephalus, spinal cord inflammation, and
meningitis.
▪ An estimated 20–85% of children with Chiari I and 48–88% of
children with Chiari II have an associated syringohydromyelia.
▪ Syringomyelia of cervical spinal cord needs to be distinguished
from other diseases that affect the cervical spinal cord:
 Sarcoidosis.
 Multiple sclerosis.
 Amyotrophic lateral sclerosis.
 Spinal cord compression by meningioma or neurofibroma.
 Spinal cord compression by metastatic tumors.
 Intrinsic tumors of the spinal cord such as ependymoma or astrocytoma.
 Cervical spondylosis with spinal cord compression, and neurosyphilis.
▪ MRI is the test of choice in differentiating syringomyelia from
other disorders.
CLINICAL FEATURES
▪ The classic early presentation of syringomyelia is bilateral upper
extremity weakness associated with ‘cape-like’ sensory loss over
the shoulders and upper extremities.
▪ There may be a syndrome of ‘sensory dissociation’ with loss of
pain and temperature as the syrinx is located centrally in the
spinal cord and in the immediate pathway of the crossing fibers
which will become the spinothalamic tracts, but sparing of
position and vibration sensation as the syrinx does not yet
involve the posterior columns of the spinal cord.
▪ If the syrinx continues to enlarge, increasing myelopathic
features can be seen including spasticity, bladder dysfunction,
weakness, other sensory loss, and gait disturbance.
▪ If the syrinx extends into the brainstem there can be a Horner’s
syndrome, nystagmus, and lower cranial nerve abnormalities.
▪ Some children with syrinxes will present with scoliosis prior to
the development of any motor or sensory symptoms.
▪ MRI can detect most syrinxes.
▪ Axial and sagittal images should be reviewed.
▪ The syrinx may be confined to the cervical
spinal cord or also extend into the thoracic
spinal cord.
▪ Somatosensory evoked responses may be
delayed due to involvement of the cervical or
thoracic spinal cord.
▪ When the syrinx extends into the brainstem,
brainstem auditory evoked responses may be
abnormal.
MRI shows syrinx involving both the upper and lower cervical spinal cord. The cerebellar
tonsils are low-lying, indicating associated Chiari malformation Type I.
TREATMENT
▪ When the syrinx is associated with Chiari I malformation, the first-line
approach to treating syringomyelia is decompression of the posterior
fossa.
▪ This may correct CSF flow and lead to collapse of the syrinx.
▪ Second-line treatment involves shunting or draining the syrinx.
PROGNOSIS
▪ Posterior fossa decompression leads to clinical and radiological
improvement of the syrinx in 50–75% of cases.
▪ In cases that require shunting of the syrinx, surgery leads to
improvement in 30–75% of the patients initially, but failure rates of the
shunt at 3 years are high (up to 50%) and complications including
infection are frequent.
▪ Untreated syringomyelia is generally progressive and can lead to
bladder dysfunction, increasing weakness and spasticity, and ultimately
paraplegia.
YouTube Video Link
▪ Syringomyelia
 https://www.youtube.com/results?search_query=s
yringomyelia
IDIOPATHIC INTRACRANIAL
HYPERTENSION (PSEUDOTUMOR
CEREBRI)
▪ Idiopathic intracranial hypertension (IIH), also
known as pseudotumor cerebri or benign
intracranial hypertension, is characterized by
increased ICP in the absence of tumors,
masses, or hydrocephalus.
▪ Diagnostic criteria include:
 Headache.
 Normal brain imaging.
 ICP of 250 mmH2O or higher on lumbar puncture.
 Normal CSF findings with the possible exception of a low total
protein level.
 Normal alertness.
 No identifiable cause of increased ICP.
▪ IIH is more common in women than men (ratio of 4:1 or
8:1).
▪ Its prevalence is estimated to be 1 case per 100,000 women.
▪ Onset is usually between ages 11 and 58, with a mean age
of onset of 30 years.
▪ Obesity is a significant risk factor for IIH.
▪ Prevalence rises to 13 per 100,000 women 20–44 years of
age if they are 10% above ideal body weight and 19 per
100,000 women 20–44 years of age who are more than 20%
above ideal body weight.
▪ The precise mechanism causing increased ICP in IIH is unknown;
cerebral blood flow and CSF production are normal.
▪ Cerebral water and cerebral blood volumes are increased in IIH.
▪ Increased ICP in IIH may reflect impaired resorption of CSF at the
arachnoid villi due to increased venous pressure at the superior
sagittal sinus.
▪ Increased venous pressures have been recorded in patients with
IIH even in the absence of sinus thrombosis.
▪ The etiology of IIH is unknown.
▪ The condition shows a strong association with female gender and
obesity.
▪ Other known associations include pregnancy, hypothyroidism, as
well as the use of corticosteroids, minocycline, cyclosporine,
vitamin A, growth hormone, and lithium carbonate.
CLINICAL FEATURES
▪ Headache is a constant symptom in IIH. The headaches are typically
throbbing, generalized, and worse in the morning. The pain may be
retro-orbital in location. The Valsalva maneuver may worsen the
headache.
▪ Neck and shoulder pains are common, sometimes with a neuritic
component.
▪ Many patients complain of pulsatile tinnitus and transient visual
obscurations that include blurred vision and scotomata.
▪ Other, less common symptoms include numbness, incoordination,
decreased sense of smell, weakness, and dizziness.
▪ On examination, nearly all patients have papilledema.
▪ Transient visual obscurations is a key symptom suggesting IIH in
patients with headaches.
▪ Unilateral or bilateral VIth nerve palsy may be observed due to traction
on the VIth nerve.
▪ A facial palsy may occur uncommonly.
▪ If there is asymmetric optic neuropathy, an afferent pupillary defect may
be detected on the swinging flashlight test. The papilledema may be
unilateral or bilateral.
▪ IIH should be distinguished from other causes of elevated ICP:
 Lyme disease.
 Bacterial meningitis.
 Viral meningitis.
 Central nervous system (CNS) lupus.
 CNS sarcoidosis.
 Cerebral venous sinus thrombosis.
 Jugular vein thrombosis.
 Primary and metastatic brain tumors.
 Hydrocephalus.
▪ Other causes of optic disc swelling or involvement of the optic
disc should be considered:
 Optic disc drüsen.
 Optic neuritis.
 Central retinal vein occlusion.
 Temporal arteritis.
 Ischemic optic neuropathy.
▪ CT of the brain is usually normal.
▪ Ventricular size is either normal or decreased.
▪ Only about 11% of patients with IIH have so-called ‘slit
ventricles’, and this finding is not required for diagnosis.
▪ Some patients on CT show either enlarged optic sheaths or
an empty sella.
▪ MRI of the brain is usually normal.
▪ Subtle findings on MRI may be noted, including gadolinium
enhancement of the optic disc, empty sella, or tortuousity
of the optic nerve sheath.
▪ The CSF is normal on lumbar puncture in IIH.
▪ CSF opening pressure is usually greater than 250 mmH2O.
However, some patients with IIH and papilledema will have
opening pressures less than 250 mmH2O and some
asymptomatic obese women may have opening pressures
greater than 250 mmH2O.
CT scan shows small slit-like ventricles in a patient with idiopathic intracranial
hypertension.
TREATMENT
▪ Patients need to be followed closely with repeat
ophthalmoscopic examinations, visual acuity
testing, and visual field testing.
▪ Some patients respond to acetazolamide, a
carbonic anhydrase inhibitor that reduces the
production of CSF.
▪ Other patients may respond to furosemide used
as a diuretic.
▪ Weight reduction is useful in obese patients with
IIH.
▪ When headaches are not controlled or in the
setting of declining visual function, surgical
intervention should be considered which
includes optic nerve sheath fenestration and CSF
shunting (either ventricular or lumbar shunts).
▪ Although advocated by some, serial lumbar punctures to
reduce ICP are rarely practical, especially in obese patients.
▪ Current surgical recommendations include using lumbo-
peritoneal shunts as an initial therapy for IIH when medical
therapy with acetazolamide has failed.
▪ Ventriculo-peritoneal shunting or optic nerve sheath
fenestration are additional surgical options if lumbo-
peritoneal shunting fails or is impractical.
PROGNOSIS
▪ Some patients with IIH remit spontaneously without
prolonged medical treatment. However, in the absence of
aggressive medical or surgical therapy as many as 17–25%
of the patients with IIH will have either permanent visual
loss or permanent optic atrophy.
▪ The onset of visual loss is usually gradual, but in some
patients visual loss may occur precipitously, requiring rapid
surgical intervention.
YouTube Video Links
▪ Idiopathic Intracranial Hypertension

 https://www.youtube.com/results?search_query=i
diopathic+intracranial+hypertension
▪ Pseudotumor Cerebri
pseudotumor+cerebri
INTRACRANIAL HYPOTENSION
SYNDROME
▪ Idiopathic intracranial hypotension (also known
as spontaneous intracranial hypotension) is a
syndrome of orthostatic headache, worsening
with upright posture, due to reduced intracranial
pressure.
▪ Idiopathic intracranial hypotension is not rare,
but its exact incidence is unknown.
▪ There are no known age or gender differences in
incidence.
▪ Intracranial hypotension syndrome is caused by reduced
ICP, usually 60 mmH2O or less on lumbar puncture.
▪ The usual cause of reduced ICP is a continuing CSF leak.
▪ Two theories have been proposed to explain the headache
of intracranial hypotension syndrome:
 Traction theory: a decrease in ICP and loss of buoyancy of the CSF
causes the brain to sag in the cranium, especially in the erect
position. This sagging causes traction on pain-sensitive structures
within the head and leads to headache.
 Venous engorgement theory: the drop in ICP leads to a
compensatory venous engorgement and this vascular dilation
leads to headache.
▪ Most cases of intracranial hypotension are due to a persistent
CSF leak, often after a lumbar puncture, spinal anesthesia, or
myelogram.
▪ CSF leaks may also develop after cranial surgery, head trauma, or
after ventriculo-peritoneal shunting.
▪ Spontaneous leaks without trauma or surgery may also develop.
▪ Spontaneous CSF leaks with CSF hypovolemia can result from
trivial trauma such as coughing, lifting, and minor falls.
▪ Other causes of decreased ICP may include dehydration, diabetic
coma, hyperpnea, and uremia.
▪ Overdrainage by a ventriculo-peritoneal shunt is an important
treatable cause of intracranial hypotension syndrome.
CLINICAL FEATURES
▪ The hallmark of intracranial hypotension syndrome is postural
headache, made worse on standing and relieved in a recumbent
position.
▪ The headache may or may not be throbbing, it is usually bilateral, and
may be frontal, occipital, or holocephalic in location.
▪ Other, highly variable symptoms include nausea, vomiting, diplopia,
altered hearing, dizziness, neck pain, blurred vision, and radicular pain in
the upper extremities.
▪ Laughing, coughing, or the Valsalva maneuver can exacerbate the
headache of intracranial hypotension.
▪ Intracranial hypotension must be differentiated from other causes of
headache:
 Migraine.
 Meningitis.
 Posterior fossa tumor.
 Hydrocephalus.
▪ Low-lying tonsils in intracranial hypotension syndrome must be
differentiated from Chiari malformation Type I.
INVESTIGATIONS
▪ Imaging
 A frequent, but not constant, sign of intracranial
hypotension syndrome is diffuse pachymeningeal
(dural) enhancement with gadolinium without
leptomeningeal (pia and arachnoid) enhancement on
MRI.
 In some symptomatic patients, meningeal
enhancement resolves prior to resolution of the
headache and other symptoms.
 Other findings on MRI include:
 Diffuse thickening of the meninges, engorgement of
the venous sinuses, and downward displacement of
the brain.
 Subdural fluid collections and enlargement of the
pituitary gland may also occur.
 The ventricles may be decreased in size.
Axial (left) and coronal (right) MRI (T1-weighted) gadolinium-enhanced images showing
enhancement in dura over the surface of the brain and in the interhemispheric falx in a
patient with intracranial hypotension syndrome.
 It is currently felt that volume depletion due to
intracranial hypotension causes a compensatory
venous engorgement, thickening of the meninges,
and a downward displacement of the brain.
 The gadolinium enhancement of intracranial
hypotension is thick, diffuse, and linear and involves
the pachymeninges of both the supra-tentorial and
infra-tentorial compartments of the brain. The
leptomeninges, including the meninges around the
brainstem, are spared.
 CT scanning of the brain may show obliteration of the
basilar cisterns due to sagging downward of the brain.
 If the CSF leak is in the spinal region, CT myelography
may be useful in identifying the site of leakage.
 Spinal MRI may also be useful in identifying leakage
sites in cases of intracranial hypotension syndrome.
▪ Other investigations
 Lumbar puncture shows an opening pressure of 60
mmH2O or less. Analysis of the CSF is usually normal.
Cultures are negative for infection. Glucose levels are
usually normal. Occasionally pleocytosis,
xanthochromia, or mild increases in CSF protein may
be noted.
 Radioisotope cisternography after injection of
radioisotope into the lumbar subarachnoid space may
be useful in identifying CSF leaks. Indium-111 is used
as a tracer and when a leak is present activity does rise
up above the basal cisterns to the cerebral convexities.
Due to extravasation of CSF and vascular uptake,
radioisotope may appear in the kidneys and bladder in
less than 4 hours.
TREATMENT
▪ Initial treatment consists of bed rest. Presumably a supine
position reduces CSF pressure at the site of leakage and allows
sealing of the leak to occur.
▪ A variety of medical treatments have been proposed for
intracranial hypotension syndrome including intravenous or oral
caffeine, intravenous or oral theophylline, intravenous hydration,
increased salt intake, corticosteroid therapy, and carbon dioxide
inhalation.
▪ Controlled studies of the efficacy of these remedies are not
available and most are of questionable value.
▪ When conservative remedies fail including bed rest, relief can
usually be obtained with an epidural blood patch using
autologous blood. Blood is injected into the epidural space with
immediate and longstanding pain relief obtained in 85–90% of
cases, but some patients may require more than one patch.
▪ When a meningeal tear can be demonstrated radiologically,
surgical repair may be necessary in some cases.
PROGNOSIS
▪ Prognosis is good in most patients with a
simple leak due to puncture of the meninges.
▪ When bed rest fails, an epidural blood patch
is generally effective. Larger rents in the
meninges may need surgical repair.
▪ Complete resolution of headache is the rule
when ICP is restored to normal.
YouTube Video Link
▪ Intracranial Hypotension
 https://www.youtube.com/results?search_query=i
ntracranial+hypotension
CLINICAL CASES
▪ A 65-year-old man has gradually become

forgetful over the last six months. He takes less
interest in his hobbies and what is happening
around him; his thinking and his actions have
slowed down. He used to have a brisk gait, but
now he has started to drag his feet. Holding
urine in has also become difficult for him.
 Question 1: What is the likely diagnosis?

 Question 2: What could have caused this man’s
condition?
 Question 3: Is this disorder reversible?
▪ A nine-month-old boy is brought to the surgery by his
mother, because she is concerned that his head has
become very large in the last few months. She also reports
that he is less lively than before and can no longer keep his
balance when sitting up. When he was seven months old,
the boy had been admitted with a high fever and
drowsiness, and had been treated with intravenous
antibiotics. Examination reveals that his cranial
circumference is 50 cm, his fontanel is taut, his gaze tends
to drop and he has trouble looking up. Furthermore, he is
unable to sit without support. No other neurological
abnormalities are observed.
 Question 1: How does one establish whether a cranial

circumference of 50 cm is pathological in a child of nine months?
 Question 2: What is the likely relationship between the child’s
current condition and the fever he previously had?
 Question 3: What is the name of the eye movement abnormality
described, and what is its cause?
▪ For the past 5 years, a 43-year-old man began to experience some left upper
quadrant abdominal wall numbness; he also noted a patch of sensory loss under
his adjacent rib. Subsequently, he noted diminished ability to make a bathtub
water temperature differentiation when he used his left foot. During the ensuing
months, the numbness gradually spread up his left side, to his right arm, and
finally his right foot. Eventually he needed to stick his head under the shower
spray to safely discern water temperature.
Just 1 year before our evaluation, he developed a paroxysm of coughing that was
immediately followed by a peculiar feeling in his neck. Concomitantly, he lost
sensation in his right 3rd to 5th fingers and his medial forearm. Soon thereafter,
when jogging, he started tripping on his right leg. A 50-pack-year smoker, he
intermittently burned his fingers with cigarettes; however, he did not perceive
the associated pain of burning flesh. He only became aware of the events when
he later discovered the skin erythema and ulceration. Prior orthopedic
evaluations suggested that he had a cervical disk lesion. He used a fair amount of
alcohol. There was a strong family history of diabetes mellitus.
Neurologic examination demonstrated hypoactive biceps and brachioradialis

stretch reflexes, triceps was modestly brisk, and knee jerks very brisk, and he
had bilateral Babinski signs. Sensory examination demonstrated a dissociated
pattern of loss affecting temperature and pain, with a “cape” type sensory loss
over the chest and back from C5 to T2. Touch, vibration, and position sensation
were normal as was the remainder of a complete neurologic examination.
 Question 1: What is the likely diagnosis?
 Question 2: What do you expect to see in the
patient’s spinal cord MRI?
 Question 3: Is the condition compatible with life?
MAIN REFERENCE
▪ Hankey’s Clinical Neurology, 2ed, Philip B.

Gorelick et al, Chapter 16, pp 667-678
▪ The contents of this slide presentation are largely lifted from the above reference textbook, with some minor alterations in wordings,
sentence construction and sequence of sentences or paragraphs to suit the needs of the students of AMA School of Medicine. Materials
in the presentation that are not found in the above reference, including pictures and clinical cases , are taken from the references listed
under Related Readings or from various internet sources.
RELATED READINGS
▪ Current Diagnosis and Treatment Neurology,

3ed, John C.M. Brust, Chapter 30, pp 506-510
▪ Clinical Neurology and Neuroanatomy, 1ed,
Aaron L. Berkowitz, Chapter 25, pp 269-274
▪ Adams and Victor’s Principles of Neurology,
11ed, Allan H. Ropper et al, Chapter 29, pp 637-
660
▪ Netter’s Neurology, 2ed, H. Royden Jones, Jr. et
al, Chapter 45, pp 379-381
▪ Textbook of Clinical Neurology, 1ed, J.B.M. Kuks
et al, Chapter 10, pp 113-122

Disorders of CSF

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Disorders of CSF

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Reynaldo B. Sta. Mina, Jr., M.D.

▪ To review the anatomy and physiology of the

▪ Cerebrospinal Fluid Flow/Circulation

▪ Cerebral Aqueduct (of Sylvius)

▪ Aqueductal Stenosis with Hydrocephalus

▪ Normal Pressure Hydrocephalus

▪ Idiopathic Intracranial Hypertension

▪ A 65-year-old man has gradually become

 Question 1: What is the likely diagnosis?

 Question 1: How does one establish whether a cranial

Neurologic examination demonstrated hypoactive biceps and brachioradialis

▪ Hankey’s Clinical Neurology, 2ed, Philip B.

▪ Current Diagnosis and Treatment Neurology,

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