Cerebrovascular disease

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"Cerebrovascular diseases" redirects here. For the medical journal,
see Cerebrovascular Diseases (journal).

Cerebrovascular disease

Cerebral angiogram of a carotid-cavernous fistula

Specialty Neurology

Symptoms Weakness on one side of body[1]

Types Stroke, vascular dementia, TIA, subarachnoid

haemorrhage[2]

Diagnostic method Neurological exam, physical exam[3]

Treatment Blood thinners, anti-hypertensives[4]

Cerebrovascular disease includes a variety of medical conditions that affect the blood

vessels of the brain and the cerebral circulation. Arteries supplying oxygen and nutrients
to the brain are often damaged or deformed in these disorders.[2] The most common
 presentation of cerebrovascular disease is an ischemic stroke or mini-stroke and
sometimes a hemorrhagic stroke.[2] Hypertension (high blood pressure) is the most
important contributing risk factor for stroke and cerebrovascular diseases as it can
change the structure of blood vessels and result in atherosclerosis.[5] Atherosclerosis
narrows blood vessels in the brain, resulting in decreased cerebral perfusion. Other risk
factors that contribute to stroke include smoking and diabetes. [6] Narrowed cerebral
arteries can lead to ischemic stroke, but continually elevated blood pressure can also
cause tearing of vessels, leading to a hemorrhagic stroke. [4]
A stroke usually presents with an abrupt onset of a neurologic deficit – such
as hemiplegia (one-sided weakness), numbness, aphasia (language impairment),
or ataxia (loss of coordination) – attributable to a focal vascular lesion. [7] The neurologic
symptoms manifest within seconds because neurons need a continual supply of
nutrients, including glucose and oxygen, that are provided by the blood. Therefore if
blood supply to the brain is impeded, injury and energy failure is rapid. [8]
Besides hypertension, there are also many less common causes of cerebrovascular
disease, including those that are congenital or idiopathic and
include CADASIL, aneurysms, amyloid angiopathy, arteriovenous
malformations, fistulas, and arterial dissections.[9] Many of these diseases can be
asymptomatic until an acute event, such as a stroke, occurs. [9] Cerebrovascular
diseases can also present less commonly with headache or seizures. [10] Any of these
diseases can result in vascular dementia due to ischemic damage to the brain.[11][12]

Contents

 1Signs and symptoms

 2Causes
o 2.1Congenital
o 2.2Acquired
o 2.3Idiopathic
 3Pathophysiology
o 3.1Mechanism of brain cell death
o 3.2Types of stroke
 3.2.1Ischemic
 3.2.2Hemorrhagic
 4Diagnosis
 5Treatment
 6Prognosis
 7Epidemiology
 8References
 9Further reading
 10External links

Signs and symptoms[edit]

Types of brain herniation

The most common presentation of cerebrovascular diseases is an acute stroke, which

occurs when blood supply to the brain is compromised. [13] Symptoms of stroke are
usually rapid in onset, and may include weakness of one side of the face or
body, numbness on one side of the face or body, inability to produce or
understand speech, vision changes, and balance difficulties.[1] Hemorrhagic strokes can
present with a very severe, sudden headache associated with vomiting, neck stiffness,
and decreased consciousness.[13] Symptoms vary depending on the location and the size
of the area of involvement of the stroke. Edema, or swelling, of the brain may occur
which increases intracranial pressure and may result in brain herniation. A stroke may
result in coma or death if it involves key areas of the brain. [14]
Other symptoms of cerebrovascular disease include migraines, seizures, epilepsy, or
cognitive decline. However, cerebrovascular disease may go undetected for years until
an acute stroke occurs. In addition, patients with some rare congenital cerebrovascular
diseases may begin to have these symptoms in childhood. [15]

Causes[edit]
Congenital[edit]
Congenital diseases are medical conditions that are present at birth that may be
associated with or inherited through genes.[16] Examples of congenital cerebrovascular
diseases include arteriovenous malformations, germinal matrix hemorrhage,
and CADASIL (cerebral autosomal-dominant arteriopathy with subcortical infarcts and
leukoencephalopathy).[9] Arteriovenous malformations are abnormal tangles of blood
vessels. Usually, a capillary bed separates arteries from veins, which protects the veins
from the higher blood pressures that occur in arteries. In arteriovenous malformations,
arteries are directly connected to veins, which increases the risk of venous rupture and
hemorrhage. Arteriovenous malformations in the brain have a 2–4% chance of rupture
each year. However, many arteriovenous malformations go unnoticed and are
asymptomatic throughout a person's lifetime.[17]
MRI demonstrating white matter changes in the brain of patients with CADASIL

A germinal matrix hemorrhage is bleeding into the brain of premature infants caused by
the rupture of fragile blood vessels within the germinal matrix of premature babies.
[18]
 The germinal matrix is a highly vascularized area within an unborn infant's brain from
which brain cells, including neurons and glial cells, originate. Infants are at most risk to
germinal matrix hemorrhages when they are born prematurely, before 32 weeks. [18] The
stresses exposed after birth, along with the fragile blood vessels, increase risk of
hemorrhage. Signs and symptoms include flaccid weakness, seizures, abnormal
posturing, or irregular respiration.[18]
CADASIL is an inherited disorder caused by mutations in the Notch 3 gene located on
chromosome 19.[19] The Notch 3 gene codes for a transmembrane protein whose
function is not well-known. However, the mutation causes accumulation of this protein
within small to medium-sized blood vessels.[19] This disease often presents in early
adulthood with migraines, stroke, mood disturbances, and cognitive deterioration. MRI
shows white matter changes in the brain and also signs of repeated strokes. The
diagnosis can be confirmed by gene testing.[20]
Acquired[edit]
Acquired cerebrovascular diseases are those that are obtained throughout a person's
life that may be preventable by controlling risk factors. The incidence of cerebrovascular
disease increases as an individual ages. [21] Causes of acquired cerebrovascular disease
include atherosclerosis, embolism, aneurysms, and arterial dissections.
[9]
 Atherosclerosis leads to narrowing of blood vessels and less perfusion to the brain,
and it also increases the risk of thrombosis, or a blockage of an artery, within the brain.
Major modifiable risk factors for atherosclerosis include: [22]

 Hypertension
 Smoking
 Obesity
 Diabetes[23][24]
Illustration of a cerebral aneurysm, demonstrating the bulge in an artery in the brain

Controlling these risk factors can reduce the incidence of atherosclerosis and stroke.
[25]
 Atrial fibrillation is also a major risk factor for strokes. Atrial fibrillation causes blood
clots to form within the heart, which may travel to the arteries within the brain and cause
an embolism. The embolism prevents blood flow to the brain, which leads to a stroke.
[citation needed]

An aneurysm is an abnormal bulging of small sections of arteries, which increases the

risk of artery rupture. Intracranial aneurysms are a leading cause of subarachnoid
hemorrhage, or bleeding around the brain within the subarachnoid space. There are
various hereditary disorders associated with intracranial aneurysms, such as Ehlers-
Danlos syndrome, autosomal dominant polycystic kidney disease, and familial
hyperaldosteronism type I.[26][27][28] However, individuals without these disorders may also
obtain aneurysms. The American Heart Association and American Stroke Association
recommend controlling modifiable risk factors including smoking and hypertension. [29]
Arterial dissections are tears of the internal lining of arteries, often associated with
trauma.[30] Dissections within the carotid arteries or vertebral arteries may compromise
blood flow to the brain due to thrombosis, and dissections increase the risk of vessel
rupture.[31]
Idiopathic[edit]
Idiopathic diseases are those that occur spontaneously without a known cause.
[32]
 Moyamoya is an example of an idiopathic cerebrovascular disorder that results in
narrowing and occlusion of intracranial blood vessels. [9] The most common presentation
is stroke or transient ischemic attack, but cognitive decline within children may also be a
presenting symptom.[9][13] The disease may begin to show symptoms beginning in
adolescence, but some may not have symptoms until adulthood. [13]

Pathophysiology[edit]
Mechanism of brain cell death[edit]
When a reduction in blood flow lasting seconds occurs, the brain tissue
suffers ischemia, or inadequate blood supply.[33][34] If the interruption of blood flow is not
restored in minutes, the tissue suffers infarction followed by tissue death.[35] When the
low cerebral blood flow persists for a longer duration, this may develop into an infarction
in the border zones (areas of poor blood flow between the major cerebral artery
distributions). In more severe instances, global hypoxia-ischemia causes widespread
brain injury leading to a severe cognitive sequelae called hypoxic-ischemic
encephalopathy.[36]
An ischemic cascade occurs where an energetic molecular problem arises due to lack
of oxygen and nutrients. The cascade results in decreased production of adenosine
triphosphate (ATP), which is a high-energy molecule needed for cells in the brain to
function.[37] Consumption of ATP continues in spite of insufficient production, this causes
total levels of ATP to decrease and lactate acidosis to become established (ionic
homeostasis in neurons is lost). The downstream mechanisms of the ischemic cascade
thus begins. Ion pumps no longer transport Ca 2+ out of cell, this triggers release of
glutamate, which in turn allows calcium into cell walls. In the end the apoptosis pathway
is initiated and cell death occurs.[38]
There are several arteries that supply oxygen to different areas of the brain, and
damage or occlusion of any of them can result in stroke. [39] The carotid arteries cover the
majority of the cerebrum. The common carotid artery divides into the internal and the
external carotid arteries. The internal carotid artery becomes the anterior cerebral artery
and the middle central artery. The ACA transmits blood to the frontal parietal. From
the basilar artery are two posterior cerebral arteries. Branches of the basilar and PCA
supply the occipital lobe, brain stem, and the cerebellum. [40] Ischemia is the loss of blood
flow to the focal region of the brain. This produces heterogeneous areas of ischemia at
the affected vascular region, furthermore blood flow is limited to a residual flow. Regions
with blood flow of less than 10 mL/100 g of tissue/min are core regions (cells here die
within minutes of a stroke). The ischemic penumbra with a blood flow of <25 ml/100g
tissue/min, remain usable for more time (hours).[41]
Types of stroke[edit]
There are two main divisions of strokes: ischemic and hemorrhagic. Ischemic stroke
involves decreased blood supply to regions of the brain, while hemorrhagic stroke is
bleeding into or around the brain.[42]
Ischemic[edit]

 Ischemic stroke, the most common is caused by a blockage of a blood vessel in

the brain, usually caused by thrombosis or emboli from a proximal arterial source or
the heart, that leads to the brain being starved of oxygen.[43] The neurologic signs
and symptoms must last longer than 24 hours or the brain infarction is
demonstrated, mainly by imaging techniques.[44]
 Transient ischemic attack (TIA) also called a mini-stroke. This is a condition in
which the blood flow to a region of the brain is blocked, but blood flow is quickly
restored and the brain tissue can fully recover. The symptoms are only transient,
leaving no sequelae, or long-term deficits.[45] In order to diagnose this entity, all
neurologic signs and symptoms must have been resolved within 24 hrs without
evidence of brain infarction on brain imaging. [46]
Hemorrhagic[edit]

 Subarachnoid haemorrhage occurs when blood leaks out of damaged vessels

into the cerebrospinal fluid in the subarachnoid space around the brain.[2] The most
common cause of a subarachnoid hemorrhage is an aneurysm rupture due to the
weakened blood vessel walls and increased wall stress. [47] The neurologic symptoms
are produced by the blood mass effect on neural structures, from the toxic effects of
blood on the brain tissue, or by the increasing of intracranial pressure.[48]
 Intracerebral haemorrhage is bleeding directly into the brain rather than around
the brain.[42] Causes and risk factors include hypertension, blood thinning
medications, trauma, and arteriovenous malformations.[49]

Brain infarct

Diagnosis[edit]
Diagnosis of cerebrovascular disease is done by (among other diagnoses): [3]

 clinical history
 physical exam
 neurological examination.
 acute stroke imaging is generally performed in significant symptoms of new
onset.
It is important to differentiate the symptoms caused by a stroke from those caused
by syncope (fainting) which is also a reduction in cerebral blood flow, almost always
generalized, but they are usually caused by systemic hypotension of various
origins: cardiac arrhythmias, myocardial infarction, hemorrhagic shock, among others.[50]

Treatment[edit]
Treatment for cerebrovascular disease may include medication, lifestyle
changes and/or surgery, depending on the cause.[4]
Examples of medications are:

 antiplatelets (aspirin, clopidogrel)
 blood thinners (heparin, warfarin)

 antihypertensives:
o ACE inhibitors
o beta blockers
o calcium channel blockers - in particular Nimodipine reduces the incidence
and severity of ischemic deficits in patients with subarachnoid hemorrhage
(SAH)[51]

 anti-diabetic medications.
Surgical procedures include:

 endovascular surgery and vascular surgery (for future stroke prevention).

Prognosis[edit]
Prognostics factors: Lower Glasgow coma scale score, higher pulse rate, higher
respiratory rate and lower arterial oxygen saturation level is prognostic features of in-
hospital mortality rate in acute ischemic stroke.[52]

Epidemiology[edit]

Disability-adjusted life year for cerebrovascular disease per 100,000 inhabitants in 2004.[53]

  less than 250
  250–425
  425–600
  600–775
  775–950
  950–1125
  1125–1300
  1300–1475
  1475–1650
  1650–1825
  1825–2000
  more than 2000

Worldwide, it is estimated there are 31 million stroke survivors, though about 6 million
deaths were due to cerebrovascular disease (2nd most common cause of death in the
world and 6th most common cause of disability). [54]
Cerebrovascular disease primarily occurs with advanced age; the risk for developing it
goes up significantly after 65 years of age. CVD tends to occur earlier than Alzheimer's
Disease (which is rare before the age of 80). In some countries such as Japan, CVD is
more common than AD.[medical citation needed]
In 2012 6.4 million US individuals (adults) had a stroke, which corresponds to 2.7% in
the U.S. With approximately 129,000 deaths in 2013 (U.S.) [55]
Geographically, a "stroke belt" in the US has long been known, similar to the "diabetes
belt"which includes all of Mississippi and parts of Alabama, Arkansas, Florida, Georgia,
Kentucky, Louisiana, North Carolina, Ohio, Pennsylvania, South Carolina, Tennessee,
Texas, Virginia, and West Virginia.[56]

Demyelinating disease
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Demyelinating disease

Photomicrograph of a demyelinating MS-lesion: Immunohistochemical

staining for CD68 highlights numerous macrophages (brown). Original

magnification 10×.

Specialty Neurology 
 A demyelinating disease is any disease of the nervous system in which
the myelin sheath of neurons is damaged.[1] This damage impairs the conduction of
signals in the affected nerves. In turn, the reduction in conduction ability causes
deficiency in sensation, movement, cognition, or other functions depending on which
nerves are involved.
Demyelinating diseases can be caused
by genetics, infectious agents, autoimmune reactions, and other unknown factors.
Proposed causes for demyelination include genetics and environmental factors such as
being triggered by a viral infection or chemical exposure. Organophosphate poisoning
by commercial insecticides such as sheep dip, weed killers, and flea treatment
preparations for pets, can also result in nerve demyelination.
[2]
 Chronic neuroleptic exposure may cause demyelination.[3] Vitamin B12 deficiency may also
result in dysmyelination.[4][5]
Demyelinating diseases are traditionally classified in two kinds: demyelinating
myelinoclastic diseases and demyelinating leukodystrophic diseases. In the first
group, a normal and healthy myelin is destroyed by a toxic, chemical, or autoimmune
substance. In the second group, myelin is abnormal and degenerates. [6] The second
group was denominated dysmyelinating diseases by Poser.[7]
In the most well known example of demyelinating disease, multiple sclerosis, evidence
has shown that the body's own immune system is at least partially
responsible. Acquired immune system cells called T-cells are known to be present at
the site of lesions. Other immune-system cells called macrophages (and possibly mast
cells) also contribute to the damage.[8]

Contents

 1Signs and symptoms

 2Evolutionary considerations
 3Diagnosis
o 3.1Types
 3.1.1CNS
 3.1.2PNS
 4Treatment
 5Prognosis
 6Epidemiology
 7Research
 8In other animals
 9See also
 10References
 11External links

Signs and symptoms[edit]

Symptoms and signs that present in demyelinating diseases are different for each
condition. These symptoms and signs can present in a person with a demyelinating
disease:[9]
 Blurred double vision 
(Diplopia) Oc
 Ataxia
 Clonus
 Dysarthria
 Fatigue
 Clumsiness
 Hand paralysis
 Hemiparesis
 Genital anaesthesia
 Incoordination
 Paresthesias
ular paralysis (cranial nerve palsy)
 Impaired muscle coordination
 Weakness (muscle)
 Loss of sensation
 Impaired vision
 Neurological symptoms
 Unsteady gait
 Spastic paraparesis
 Incontinence
 Hearing problems
 Speech problems

Evolutionary considerations[edit]
The role of prolonged cortical myelination in human evolution has been implicated as a
contributing factor in some cases of demyelinating disease. Unlike other primates,
humans exhibit a unique pattern of postpubertal myelination, which may contribute to
the development of psychiatric disorders and neurodegenerative diseases that present
in early adulthood and beyond. The extended period of cortical myelination in humans
may allow greater opportunity for disruption in myelination, resulting in the onset of
demyelinating disease.[10] Furthermore, humans have significantly greater prefrontal
white matter volume than other primate species, which implies greater myelin density.
[11]
 Increased myelin density in humans as a result of a prolonged myelination may,
therefore, structure risk for myelin degeneration and dysfunction. Evolutionary
considerations for the role of prolonged cortical myelination as a risk factor for
demyelinating disease are particularly pertinent given that genetics and autoimmune
deficiency hypotheses fail to explain many cases of demyelinating disease. As has been
argued, diseases such as multiple sclerosis cannot be accounted for by autoimmune
deficiency alone, but strongly imply the influence of flawed developmental processes in
disease pathogenesis.[12] Therefore, the role of the human-specific prolonged period of
cortical myelination is an important evolutionary consideration in the pathogenesis of
demyelinating disease.

Diagnosis[edit]
Various methods/techniques are used to diagnose demyelinating diseases:

 Exclusion of other conditions that have overlapping symptoms [13]

 Magnetic resonance imaging (MRI) is a medical imaging technique used
in radiology to visualize internal structures of the body in detail. MRI makes use of
the property of nuclear magnetic resonance (NMR) to image nuclei of atoms inside
the body. This method is reliable because MRIs assess changes in proton density.
"Spots" can occur as a result of changes in brain water content. [13]:113
 Evoked potential is an electrical potential recorded from the nervous system
following the presentation of a stimulus as detected
by electroencephalography (EEG), electromyography (EMG), or other
electrophysiological recording method.[13]:117
 Cerebrospinal fluid analysis (CSF) can be extremely beneficial in the diagnosis of
central nervous system infections. A CSF culture examination may yield
the microorganism that caused the infection.[13]
 Quantitative proton magnetic resonance spectroscopy  (MRS) is a noninvasive
analytical technique that has been used to study metabolic changes in brain tumors,
strokes, seizure disorders, Alzheimer's disease, depression, and other diseases
affecting the brain. It has also been used to study the metabolism of other organs
such as muscles.[13]:309
 Diagnostic criteria refers to a specific combination of signs, symptoms, and test
results that the clinician uses in an attempt to determine the correct diagnosis. [13]:320
 Fluid-attenuated inversion recovery (FLAIR) uses a pulse sequence to suppress
cerebrospinal fluid and show lesions more clearly, and is used for example in
multiple sclerosis evaluation.
Types[edit]
Demyelinating diseases can be divided in those affecting the central nervous
system (CNS) and those affecting the peripheral nervous system (PNS). They can also
be classified by the presence or absence of inflammation. Finally, a division may be
made based on the underlying cause of demyelination: the disease process can
be demyelinating myelinoclastic, wherein myelin is destroyed; or dysmyelinating
leukodystrophic, wherein myelin is abnormal and degenerative.
CNS[edit]
The demyelinating disorders of the central nervous system include:

 Myelinoclastic or demyelinating disorders:

o Typical forms of multiple sclerosis
o Neuromyelitis optica, or Devic's disease
 o Idiopathic inflammatory demyelinating diseases
 Leukodystrophic or dysmyelinating disorders:
o CNS neuropathies such as those produced by vitamin B12 deficiency
o Central pontine myelinolysis
o Myelopathies such as tabes dorsalis (syphilitic myelopathy)
o Leukoencephalopathies such as progressive multifocal
leukoencephalopathy
o Leukodystrophies
The myelinoclastic disorders are typically associated with symptoms such as optic
neuritis and transverse myelitis, because the demyelinating inflammation can affect
the optic nerve or spinal cord. Many are idiopathic. Both myelinoclastic and
leukodystrophic modes of disease may result in lesional demyelinations of the central
nervous system.
PNS[edit]

Guillain–Barré syndrome – demyelination

The demyelinating diseases of the peripheral nervous system include:

 Guillain–Barré syndrome and its chronic counterpart, chronic inflammatory

demyelinating polyneuropathy
 Anti-MAG peripheral neuropathy
 Charcot–Marie–Tooth disease and its counterpart Hereditary neuropathy with
liability to pressure palsy
 Copper deficiency-associated conditions (peripheral neuropathy, myelopathy,
and rarely optic neuropathy)
 Progressive inflammatory neuropathy

Treatment[edit]
See also: Multiple sclerosis §  Medications, and Management of multiple sclerosis
Treatments are patient-specific and depend on the symptoms that present with the
disorder, as well as the progression of the condition. Improvements to the patient's life
may be accomplished through the management of symptoms or slowing of the rate of
demyelination. Treatment can include medication, lifestyle changes (i.e. smoking
cessation, increased rest, and dietary changes), counselling, relaxation, physical
exercise, patient education, and in some cases, deep brain thalamic stimulation (to
ameliorate tremors).[13]:227–248
Prognosis[edit]
Prognosis depends on the condition itself. Some conditions such as MS depend on the
subtype of the disease and various attributes of the patient such as age, sex, initial
symptoms, and the degree of disability the patient experiences. [14] Life expectancy in MS
patients is 5 to 10 years lower than unaffected people. [15] MS is an inflammatory
demyelinating disease of the central nervous system (CNS) that develops in genetically
susceptible individuals after exposure to unknown environmental trigger(s). The bases
for MS are unknown but are strongly suspected to involve immune reactions against
autoantigens, particularly myelin proteins. The most accepted hypothesis is that
dialogue between T-cell receptors and myelin antigens leads to an immune attack on
the myelin-oligodendrocyte complex. These interactions between active T cells and
myelin antigens provoke a massive destructive inflammatory response and promote
continuing proliferation of T and B cells and macrophage activation, which sustains
secretion of inflammatory mediators.[16] Other conditions such as central pontine
myelinolysis have about a third of patients recover and the other two-thirds experience
varying degrees of disability.[17] In some cases, such as transverse myelitis, the patient
can begin recovery as early as 2 to 12 weeks after the onset of the condition.

Epidemiology[edit]
Incidence of demyelinating diseases varies by disorder. Some conditions, such as tabes
dorsalis appear predominantly in males and begin in midlife. Optic neuritis, though,
occurs preferentially in females typically between the ages of 30 and 35. [18] Other
conditions such as multiple sclerosis vary in prevalence depending on the country and
population.[19] This condition can appear in children and adults. [15]

Research[edit]
Much of the research conducted on demyelinating diseases is targeted towards
discovering the mechanisms by which these disorders function in an attempt to develop
therapies and treatments for individuals affected by these conditions. For
example, proteomics has revealed several proteins which contribute to the
pathophysiology of demyelinating diseases.[20]
For example, COX-2 has been implicated in oligodendrocyte death in animal models of
demyelination.[21] The presence of myelin debris has been correlated with damaging
inflammation as well as poor regeneration, due to the presence of inhibitory myelin
components.[22][23]
N-cadherin is expressed in regions of active remyelination and may play an important
role in generating a local environment conducive to remyelination. [24] N-
cadherin agonists have been identified and observed to stimulate neurite growth and
cell migration, key aspects of promoting axon growth and remyelination after injury or
disease.[25]
Immunomodulatory drugs such as fingolimod have been shown to reduce immune-
mediated damage to the CNS, preventing further damage in patients with MS. The drug
targets the role of macrophages in disease progression.[26][27]
Manipulating thyroid hormone levels may become a viable strategy to promote
remyelination and prevent irreversible damage in MS patients. [28] It has also been shown
that intranasal administration of apotransferrin (aTf) can protect myelin and induce
remyelination.[29] Finally, electrical stimulation which activates neural stem cells may
provide a method by which regions of demyelination can be repaired. [30]

In other animals[edit]
Demyelinating diseases/disorders have been found worldwide in various animals. Some
of these animals include mice, pigs, cattle, hamsters, rats, sheep, Siamese kittens, and
a number of dog breeds (including Chow Chow, Springer Spaniel, Dalmatian, Samoyed,
Golden Retriever, Lurcher, Bernese Mountain Dog, Vizsla, Weimaraner, Australian Silky
Terrier, and mixed breeds).[31][32]
Another notable animal found able to contract a demyelinating disease is the northern
fur seal. Ziggy Star, a female northern fur seal, was treated at the Marine Mammal
Center beginning in March 2014 [33] and was noted as the first reported case of such
disease in a marine mammal. She was later transported to Mystic Aquarium & Institute
for Exploration for lifelong care as an ambassador to the public.[34]

Brain Edema, Transtentorial Herniation, and Increased Intracranial Pressure BRAIN EDEMA • Brain
edema is defined as an increase in brain sodium and water content. • Brain edema occurs in many
neurological conditions such as stroke, trauma, tumors, infections, encephalopathies, and hydrocephaly.
• Brain edema is classified into three major types: vasogenic, cytotoxic, and interstitial: Vasogenic: seen
primarily in the white matter. Common causes: brain tumor, abscess, infarction, and hemorrhage.
Clinical presentation: focal neurological deficits, decreased level of consciousness, pupillary asymmetry,
and increased intracranial pressure (ICP). Cytotoxic (cellular): seen in the white and gray matter.
Common causes: ischemic/ hypoxic encephalopathies (e.g., post-cardiac arrest) and meningitis. Clinical
presentation: stupor, coma, focal or generalized seizures, and myoclonic jerks. Interstitial: increased
brain fluid due to blockage of cerebral spinal fluid absorption, seen primarily in periventricular white
matter. Common causes: pseudotumor, obstructive hydrocephalus. Clinical presentation: headache,
nausea, vomiting, mental alteration, papilledema, or gait difficulty. • Ischemic brain edema (after stroke)
begins with cellular changes to vasogenic. In meningitis, the edema begins from the cellular, and then
changes to vasogenic and may then to interstitial (hydrocephalus). TRANSTENTORIAL HERNIATION •
Brain herniation is caused by mass or any cause that increases ICP; subsequently, the brain substance
shifts from higher pressure to lower pressure. • The most common forms of herniation are subfalcine,
uncal, and cerebellar tonsilar herniation. • Clinical presentation of uncal herniation: dilation and
ophthalmoplegia (third nerve compression) followed by ipsilateral (to site of lesion) hemiplegia and
bilateral corticospinal tract signs (Kernohan’s notch phenomenon), followed by irregular respiratory
pattern, fixed and dilated pupils, coma, and cardiorespiratory collapse. INCREASED ICP • The signs of ICP
are headache, vomiting, mental dysfunction, confusion, papilledema, bradycardia, decreased respiration
(Cheyne-Stokes respirations), and increased blood pressure (Cushing’s triad). • In a patient suspected of
having increased ICP, try to answer these questions: 1. Does a space-occupying lesion exist? 2. What
 part of the brain is involved? 3. What is most likely the cause? Remember: Despite signs of increased IC
a posterior fossa tumor may not have localizing signs. WHAT TO DO • Stabilize the patient, secure vitals.
• Keep head elevated (30–45° degrees). • Keep patient moderately dehydrated. • Obtain computed
tomography scan or magnetic resonance image of head in all patients with symptoms of increased ICP. •
Eliminate or treat the underlying cause with the most effective mode of therapy. Increased ICP in many
cases requires neurosurgical procedures. • Hyperventilation (HV): Intubate and maintain pCO2 to 25–30
mmHg; HV immediately reduces the blood flow (vasoconstriction) and decreases ICP. Prolonged use of
HV causes further ischemia to the normal and damaged brain area, and therefore should be avoided. HV
takes effect within 10 minutes. HV should be used less in acute stroke or acute head trauma because of
vasoconstriction. • Hyperosmolar agents: Mannitol is most commonly used; the dose is 1–2 g/kg (25%)
given in 500 mL of 5% dextrose in water. May repeat 0.5 g/kg, every 4–6 hours. Serum osmolality is used
as a treatment guide (maintain below 320 mOsm/L) or between 295 and 305 mOsm. Monitor serum
sodium (maintain(© Humana Press Inc. 2008) Cite this chapter as:

(2008) Brain Edema, Transtentorial Herniation, and Increased

Intracranial Pressure. In: Practicing Neurology. Current Clinical
Neurology. Humana Press

Brain herniation

       
Brain herniation is the shifting of the brain tissue from one space in the brain to another through
various folds and openings.

Causes
Brain herniation occurs when something inside the skull produces pressure that moves brain tissues.
This is most often the result of brain swelling or bleeding from a head injury, stroke, or brain tumor.
Brain herniation can be a side effect of tumors in the brain, including:

 Metastatic brain tumor

 Primary brain tumor
Herniation of the brain can also be caused by other factors that lead to increased pressure inside the
skull, including:

 Collection of pus and other material in the brain, usually from a bacterial or
fungal infection (abscess)
 Bleeding in the brain (hemorrhage)

 Buildup of fluid inside the skull that leads to brain swelling (hydrocephalus)
 Strokes that cause brain swelling
 Swelling after radiation therapy

 Defect in brain structure, such as a condition called Chiari malformation

Brain herniation can occur:

 From side to side or down, under, or across rigid membrane like the tentorium or
falx

 Through a natural bony opening at the base of the skull called the foramen
magnum

 Through openings created during brain surgery

Hydrocephalus
From Wikipedia, the free encyclopedia
Jump to navigationJump to search
Not to be confused with Hypocephalus or Hydranencephaly.
For the trilobite, see Hydrocephalus (genus).

Hydrocephalus

Other names Water on the brain[1]

Hydrocephalus as seen on a CT scan of the brain. The black areas in the

middle of the brain (the lateral ventricles) are abnormally large and

filled with fluid.

/ˌhaɪdroʊˈsɛfələs/[2]
Pronunciation

Specialty Neurosurgery

Symptoms Babies: rapid head growth, vomiting,

sleepiness, seizures[1]

Older people: Headaches, double vision, poor

balance, urinary incontinence, personality

changes, mental impairment[1]

Causes Neural tube defects, meningitis, brain

tumors, traumatic brain injury, intraventricular

hemorrhage[1]

Diagnostic method Based on symptoms and medical imaging[1]

Treatment Surgery[1]

Prognosis Variable, often normal life[1]

 Frequency 1.5 per 1,000 (babies)[1][3]

Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF)

occurs within the brain.[1] This typically causes increased pressure inside the skull. Older
people may have headaches, double vision, poor balance, urinary incontinence,
personality changes, or mental impairment. In babies, it may be seen as a rapid
increase in head size. Other symptoms may include vomiting, sleepiness, seizures,
and downward pointing of the eyes.[1]
Hydrocephalus can occur due to birth defects or be acquired later in life.[1] Associated
birth defects include neural tube defects and those that result in aqueductal stenosis.[1]
[4]
 Other causes include meningitis, brain tumors, traumatic brain injury, intraventricular
hemorrhage, and subarachnoid hemorrhage. The four types of hydrocephalus are
communicating, noncommunicating, ex vacuo, and normal pressure. Diagnosis is
typically made by physical examination and medical imaging.[1]
Hydrocephalus is typically treated by the surgical placement of a shunt system.[1] A
procedure called a third ventriculostomy may be an option in a few people.
[1]
 Complications from shunts may include overdrainage, underdrainage, mechanical
failure, infection, or obstruction.[1] This may require replacement.[1] Outcomes are
variable, but many people with shunts live normal lives. [1] Without treatment, death or
permanent disability may occur.[1]
About one to two per 1,000 newborns have hydrocephalus. [1][3] Rates in the developing
world may be higher.[5] Normal pressure hydrocephalus is estimated to affect about 5 per
100,000 people, with rates increasing with age. [6] Description of hydrocephalus
by Hippocrates dates back more than 2,000 years.[5] The word hydrocephalus is from the
Greek ὕδωρ, hydōr, meaning 'water' and κεφαλή, kephalē, meaning 'head'.[7]

Contents

 1Signs and symptoms

 2Cause
o 2.1Congenital
o 2.2Acquired
 3Type
o 3.1Communicating
o 3.2Noncommunicating
o 3.3Other
 4Mechanism
 5Treatments
o 5.1Procedures
o 5.2External hydrocephalus
o 5.3Shunt complications
 6History
 7Society and culture
o 7.1Name
 o 7.2Awareness campaign
o 7.3Exceptional case
o 7.4Notable cases
 8References
 9External links

Signs and symptoms[edit]

Illustration showing different effects of hydrocephalus on the brain and cranium

The clinical presentation of hydrocephalus varies with chronicity. Acute dilatation of

the ventricular system is more likely to manifest with the nonspecific signs and
symptoms of increased intracranial pressure (ICP). By contrast, chronic dilatation
(especially in the elderly population) may have a more insidious onset presenting, for
instance, with Hakim's triad (Adams' triad).
Symptoms of increased ICP may
include headaches, vomiting, nausea, papilledema, sleepiness, or coma. With
increased levels of CSF, there have been cases of hearing loss due to CSF creating
pressure on the auditory pathways or disrupting the communication of inner ear fluid.
[8]
 Elevated ICP of different etiologies have been linked to sensorineural hearing loss
(SNHL). Transient SNHL has been reported after the loss of CSF with shunt surgeries.
[9]
 Hearing loss is a rare but well-known sequela of procedures resulting in CSF loss.
[8]
 Elevated ICP may result in uncal or tonsillar herniation, with resulting life-
threatening brain stem compression.
Hakim's triad of gait instability, urinary incontinence, and dementia is a relatively typical
manifestation of the distinct entity normal-pressure hydrocephalus. Focal neurological
deficits may also occur, such as abducens nerve palsy and vertical gaze
palsy (Parinaud syndrome due to compression of the quadrigeminal plate, where the
neural centers coordinating the conjugated vertical eye movement are located). The
symptoms depend on the cause of the blockage, the person's age, and how much brain
tissue has been damaged by the swelling.
In infants with hydrocephalus, CSF builds up in the central nervous system (CNS),
causing the fontanelle (soft spot) to bulge and the head to be larger than expected.
Early symptoms may also include:
 Eyes that appear to gaze downward
 Irritability
 Seizures
 Separated sutures
 Sleepiness
 Vomiting
Symptoms that may occur in older children can include:

 Brief, shrill, high-pitched cry

 Changes in personality, memory, or the ability to reason or think
 Changes in facial appearance and eye spacing (craniofacial disproportion)
 Crossed eyes or uncontrolled eye movements
 Difficulty feeding
 Excessive sleepiness
 Headaches
 Irritability, poor temper control
 Loss of bladder control (urinary incontinence)
 Loss of coordination and trouble walking
 Muscle spasticity (spasm)
 Slow growth (child 0–5 years)
 Delayed milestones
 Failure to thrive
 Slow or restricted movement
 Vomiting[10]
Because hydrocephalus can injure the brain, thought and behavior may be adversely
affected. Learning disabilities, including short-term memory loss, are common among
those with hydrocephalus, who tend to score better on verbal IQ than on performance
IQ, which is thought to reflect the distribution of nerve damage to the brain.
[1]
 Hydrocephalus that is present from birth can cause long-term complications with
speech and language. Children can have difficulties such as nonverbal learning
disorder, understanding complex and abstract concepts, retrieving stored information,
and spatial/perceptual disorders. Children with hydrocephalus are often known in having
the difficulty in understanding the concepts within conversation and tend to use words
they know or have heard.[11][12] However, the severity of hydrocephalus can differ
considerably between individuals, and some are of average or above-average
intelligence. Someone with hydrocephalus may have coordination and visual problems,
or clumsiness. They may reach puberty earlier than the average child (this is
called precocious puberty). About one in four develops epilepsy.

Cause[edit]
Congenital[edit]
A one-year-old girl with hydrocephalus showing "sunset eyes", before shunt surgery

Congenital hydrocephalus is present in the infant prior to birth, meaning the fetus
developed hydrocephalus in utero during fetal development. The most common cause
of congenital hydrocephalus is aqueductal stenosis, which occurs when the narrow
passage between the third and fourth ventricles in the brain is blocked or too narrow to
allow sufficient cerebral spinal fluid to drain. Fluid accumulates in the upper ventricles,
causing hydrocephalus.[13]
Other causes of congenital hydrocephalus include neural-tube defects, arachnoid
cysts, Dandy–Walker syndrome, and Arnold–Chiari malformation. The cranial
bones fuse by the end of the third year of life. For head enlargement to occur,
hydrocephalus must occur before then. The causes are usually genetic, but can also be
acquired and usually occur within the first few months of life, which include
intraventricular matrix hemorrhages in premature infants, infections, type II Arnold-
Chiari malformation, aqueduct atresia and stenosis, and Dandy-Walker malformation.
In newborns and toddlers with hydrocephalus, the head circumference is enlarged
rapidly and soon surpasses the 97th percentile. Since the skull bones have not yet
firmly joined together, bulging, firm anterior and posterior fontanelles may be present
even when the person is in an upright position.
The infant exhibits fretfulness, poor feeding, and frequent vomiting. As the
hydrocephalus progresses, torpor sets in, and infants show lack of interest in their
surroundings. Later on, their upper eyelids become retracted and their eyes are turned
downwards ("sunset eyes") (due to hydrocephalic pressure on the mesencephalic
tegmentum and paralysis of upward gaze). Movements become weak and the arms
may become tremulous. Papilledema is absent, but vision may be reduced. The head
becomes so enlarged that they eventually may be bedridden.
About 80–90% of fetuses or newborn infants with spina bifida—often associated
with meningocele or myelomeningocele—develop hydrocephalus.[14]
Acquired[edit]
This condition is acquired as a consequence of CNS infections, meningitis, brain
tumors, head trauma, toxoplasmosis, or intracranial hemorrhage (subarachnoid or
intraparenchymal), and is usually painful.[15]

Type[edit]
The cause of hydrocephalus is not known with certainty and is probably multifactorial. It
may be caused by impaired CSF flow, reabsorption, or excessive CSF production.

 Obstruction to CSF flow hinders its free passage through the ventricular system
and subarachnoid space (e.g., stenosis of the cerebral aqueduct or obstruction of
the interventricular foramina secondary
to tumors, hemorrhages, infections or congenital malformations) and can cause
increases in ICP.
 Hydrocephalus can also be caused by overproduction of CSF (relative
obstruction) (e.g., choroid plexus papilloma, villous hypertrophy).[16][17]
 Bilateral ureteric obstruction is a rare, but reported, cause of hydrocephalus.
Based on its underlying mechanisms, hydrocephalus can be classified into
communicating and noncommunicating (obstructive). Both forms can be either
congenital or acquired.[citation needed]
Communicating[edit]
Communicating hydrocephalus, also known as nonobstructive hydrocephalus, is caused
by impaired CSF reabsorption in the absence of any obstruction of CSF flow between
the ventricles and subarachnoid space. This may be due to functional impairment of
the arachnoidal granulations (also called arachnoid granulations or Pacchioni's
granulations), which are located along the superior sagittal sinus, and is the site of CSF
reabsorption back into the venous system. Various neurologic conditions may result in
communicating hydrocephalus, including subarachnoid/intraventricular hemorrhage,
meningitis, and congenital absence of arachnoid villi. Scarring and fibrosis of the
subarachnoid space following infectious, inflammatory, or hemorrhagic events can also
prevent resorption of CSF, causing diffuse ventricular dilatation. [18]
Noncommunicating[edit]
Noncommunicating hydrocephalus, or obstructive hydrocephalus, is caused by a CSF-
flow obstruction.

 Foramen of Monro obstruction may lead to dilation of one, or if large enough

(e.g., in colloid cyst), both lateral ventricles.
 The aqueduct of Sylvius, normally narrow, may be obstructed by a number of
genetic or acquired lesions (e.g., atresia, ependymitis, hemorrhage, or tumor) and
lead to dilation of both lateral ventricles, as well as the third ventricle.
 Fourth ventricle obstruction leads to dilatation of the aqueduct, as well as the
lateral and third ventricles (e.g., Chiari malformation).
 The foramina of Luschka and foramen of Magendie may be obstructed due to
congenital malformation (e.g., Dandy-Walker malformation).
Other[edit]
Hydrocephalus ex vacuo from vascular dementia as seen on MRI

 Normal pressure hydrocephalus (NPH) is a particular form of chronic

communicating hydrocephalus, characterized by enlarged cerebral ventricles, with
only intermittently elevated cerebrospinal fluid pressure. Characteristic triad of
symptoms are; dementia, apraxic gait and urinary incontinence. The diagnosis of
NPH can be established only with the help of continuous intraventricular pressure
recordings (over 24 hours or even longer), since more often than not instant
measurements yield normal pressure values. Dynamic compliance studies may be
also helpful. Altered compliance (elasticity) of the ventricular walls, as well as
increased viscosity of the cerebrospinal fluid, may play a role in the pathogenesis.
 Hydrocephalus ex vacuo also refers to an enlargement of cerebral ventricles and
subarachnoid spaces, and is usually due to brain atrophy (as it occurs
in dementias), post-traumatic brain injuries, and even in some psychiatric disorders,
such as schizophrenia.[19] As opposed to hydrocephalus, this is a compensatory
enlargement of the CSF-spaces in response to brain parenchyma loss; it is not the
result of increased CSF pressure.[19]

Mechanism[edit]
Spontaneous intracerebral and intraventricular hemorrhage with hydrocephalus shown on CT scan[20]

3D cast of lateral ventricles in hydrocephalus

Hydrocephalus is usually due to blockage of CSF outflow in the ventricles or in the

subarachnoid space over the brain. In a person without hydrocephalus, CSF
continuously circulates through the brain, its ventricles and the spinal cord and is
continuously drained away into the circulatory system. Alternatively, the condition may
result from an overproduction of the CSF, from a congenital malformation blocking
normal drainage of the fluid, or from complications of head injuries or infections. [21]
Compression of the brain by the accumulating fluid eventually may cause neurological
symptoms such as convulsions, intellectual disability, and epileptic seizures. These
signs occur sooner in adults, whose skulls are no longer able to expand to
accommodate the increasing fluid volume within. Fetuses, infants, and young children
with hydrocephalus typically have an abnormally large head, excluding the face,
because the pressure of the fluid causes the individual skull bones—which have yet to
fuse—to bulge outward at their juncture points. Another medical sign, in infants, is a
characteristic fixed downward gaze with whites of the eyes showing above the iris, as
though the infant were trying to examine its own lower eyelids. [22]
The elevated ICP may cause compression of the brain, leading to brain damage and
other complications. A complication often overlooked is the possibility of hearing loss
due to ICP. The mechanism of ICP on hearing loss is presumed that the transmission of
CSF pressure to and from the Perilymphatic space through a patent cochlear aqueduct.
[23][24]
 The cochlear aqueduct connects the Perilymphatic space of the inner ear with the
subarachnoid space of the posterior cranial fossa. [25] A loss of CSF pressure can induce
Perilymphatic loss or endolymphatic hydrops resembling the clinical presentation
of Ménière's disease associated hearing loss in the low frequencies. [23] Conditions
among affected individuals vary widely.
If the foramina of the fourth ventricle or the cerebral aqueduct are blocked, CSF can
accumulate within the ventricles. This condition is called internal hydrocephalus and it
results in increased CSF pressure. The production of CSF continues, even when the
passages that normally allow it to exit the brain are blocked. Consequently, fluid builds
inside the brain, causing pressure that dilates the ventricles and compresses
the nervous tissue. Compression of the nervous tissue usually results in irreversible
brain damage. If the skull bones are not completely ossified when the hydrocephalus
occurs, the pressure may also severely enlarge the head. The cerebral aqueduct may
be blocked at the time of birth or may become blocked later in life because of a tumor
growing in the brainstem.

Treatments[edit]
Procedures[edit]

Baby recovering from shunt surgery

Hydrocephalus treatment is surgical, creating a way for the excess fluid to drain away.
In the short term, an external ventricular drain (EVD), also known as an extraventricular
drain or ventriculostomy, provides relief. In the long term, some people will need any of
various types of cerebral shunt. It involves the placement of a ventricular catheter (a
tube made of silastic) into the cerebral ventricles to bypass the flow
obstruction/malfunctioning arachnoidal granulations and drain the excess fluid into other
body cavities, from where it can be resorbed. Most shunts drain the fluid into
the peritoneal cavity (ventriculoperitoneal shunt), but alternative sites include the right
atrium (ventriculoatrial shunt), pleural cavity (ventriculopleural shunt), and gallbladder. A
shunt system can also be placed in the lumbar space of the spine and have the CSF
redirected to the peritoneal cavity (lumbar-peritoneal shunt).[26] An alternative treatment
for obstructive hydrocephalus in selected people is the endoscopic third
ventriculostomy (ETV), whereby a surgically created opening in the floor of the third
ventricle allows the CSF to flow directly to the basal cisterns, thereby shortcutting any
obstruction, as in aqueductal stenosis. This may or may not be appropriate based on
individual anatomy. For infants, ETV is sometimes combined with choroid plexus
cauterization, which reduces the amount of cerebrospinal fluid produced by the brain.
The technique, known as ETV/CPC, was pioneered in Uganda by
neurosurgeon Benjamin Warf and is now in use in several U.S. hospitals. [27]
  Hydrocephalus can be successfully treated by placing a drainage tube (shunt)
[28]

between the brain ventricles and abdominal cavity. Some risk exists of infection being
introduced into the brain through these shunts, however, and the shunts must be
replaced as the person grows.
External hydrocephalus[edit]
External hydrocephalus is a condition generally seen in infants which involves enlarged
fluid spaces or subarachnoid spaces around the outside of the brain. This is generally
a benign condition that resolves spontaneously by two years of age [29] and therefore
usually does not require insertion of a shunt. Imaging studies and a good medical
history can help to differentiate external hydrocephalus from subdural hemorrhages or
symptomatic chronic extra-axial fluid collections which are accompanied by vomiting,
headaches, and seizures.
Shunt complications[edit]
Examples of possible complications include shunt malfunction, shunt failure, and shunt
infection, along with infection of the shunt tract following surgery (the most common
reason for shunt failure is infection of the shunt tract). Although a shunt generally works
well, it may stop working if it disconnects, becomes blocked (clogged) or infected, or it is
outgrown. If this happens, the CSF begins to accumulate again and a number of
physical symptoms develop (headaches, nausea, vomiting, photophobia/light
sensitivity), some extremely serious, such as seizures. The shunt failure rate is also
relatively high (of the 40,000 surgeries performed annually to treat hydrocephalus, only
30% are a person's first surgery) and people not uncommonly have multiple shunt
revisions within their lifetimes.
Another complication can occur when CSF drains more rapidly than it is produced by
the choroid plexus, causing symptoms of listlessness, severe headaches, irritability,
light sensitivity, auditory hyperesthesia (sound sensitivity), hearing loss[25], nausea,
vomiting, dizziness, vertigo, migraines, seizures, a change in personality, weakness in
the arms or legs, strabismus, and double vision to appear when the person is vertical. If
the person lies down, the symptoms usually vanish quickly. A CT scan may or may not
show any change in ventricle size, particularly if the person has a history of slit-like
ventricles. Difficulty in diagnosing over-drainage can make treatment of this
complication particularly frustrating for people and their families. Resistance to
traditional analgesic pharmacological therapy may also be a sign of shunt overdrainage
or failure.[30]
Following placement of a ventriculoperitoneal shunt there have been cases of a
decrease in post-surgery hearing. It is presumed that the cochlea aqueduct is
responsible for the decrease in hearing thresholds. The cochlea aqueduct has been
considered as a probable channel where CSF pressure can be transmitted. Therefore,
the reduced CSF pressure could cause a decrease in Perilymphatic pressure and cause
secondary endolymphatic hydrops.[25] In addition to the increased hearing loss, there
have also been findings of resolved hearing loss after ventriculoperitoneal shunt
placement, where there is a release of CSF pressure on the auditory pathways. [31]
The diagnosis of CSF buildup is complex and requires specialist expertise. Diagnosis of
the particular complication usually depends on when the symptoms appear, that is,
whether symptoms occur when the person is upright or in a prone position, with the
head at roughly the same level as the feet. [32]
Standardized protocols for inserting cerebral shunts have been shown to reduce shunt
infections.[33][34] Prophylatic treatment with antibiotics may ahve a postive effect on the
number of people who get shunt infections, but due to the low quality or lack of large
scale randomised controlled trials it is unclear how variability in outcomes is effected by
factors such as the use of different antibiotic agents, administration routes, timing and
doses; or by the characteristics of patients. [35]

History[edit]

Skull of a hydrocephalic child (1800s)

References to hydrocephalic skulls can be found in ancient Egyptian medical literature

from 2,500 BC to 500 AD.[36] Hydrocephalus was described more clearly by the ancient
Greek physician Hippocrates in the fourth century BC, while a more accurate
description was later given by the Roman physician Galen in the second century AD.[36]
The first clinical description of an operative procedure for hydrocephalus appears in
the Al-Tasrif (1,000 AD) by the Arab surgeon Abulcasis, who clearly described the
evacuation of superficial intracranial fluid in hydrocephalic children. [36] He described it in
his chapter on neurosurgical disease, describing infantile hydrocephalus as being
caused by mechanical compression. He wrote: [36]
The skull of a newborn baby is often full of liquid, either because the matron has
compressed it excessively or for other, unknown reasons. The volume of the skull then
increases daily, so that the bones of the skull fail to close. In this case, we must open
the middle of the skull in three places, make the liquid flow out, then close the wound
and tighten the skull with a bandage.
Historical specimen of an infant with severe hydrocephalus, probably untreated

In 1881, a few years after the landmark study of Retzius and Key, Carl

Wernicke pioneered sterile ventricular puncture and external drainage of CSF for the
treatment of hydrocephalus.[36] It remained an intractable condition until the 20th century,
when cerebral shunt and other neurosurgical treatment modalities were developed.
It is a lesser-known medical condition; relatively little research is conducted to improve
treatment, and still no cure has been found. In developing countries, the condition often
goes untreated at birth. Before birth, the condition is difficult to diagnose, and access to
medical treatment is limited. However, when head swelling is prominent, children are
taken at great expense for treatment. By then, brain tissue is undeveloped and
neurosurgery is rare and difficult. Children more commonly live with undeveloped brain
tissue and consequential intellectual disabilities and restrictions. [37]

Society and culture[edit]

Name[edit]
The word hydrocephalus is from the Greek ὕδωρ, hydōr meaning 'water'
and κεφαλή, kephalē meaning 'head'.[7] Other names for hydrocephalus include "water
on the brain", a historical name, and "water baby syndrome". [1][38]
Awareness campaign[edit]
Hydrocephalus awareness ribbon

September was designated National Hydrocephalus Awareness Month in July 2009 by

the U.S. Congress in H.Res. 373. The resolution campaign is due in part to the
advocacy work of the Pediatric Hydrocephalus Foundation. Prior to July 2009, no
awareness month for this condition had been designated. Many hydrocephalus
organizations promote awareness and fundraising activities.
Exceptional case[edit]
One case of hydrocephalus was a man whose brain shrank to a thin sheet of tissue,
due to a buildup of cerebrospinal fluid in his skull. As a child, the man had a shunt, but it
was removed when he was 14. In July 2007, at age 44, he went to a hospital due to
mild weakness in his left leg. When doctors learned of the man's medical history, they
performed a CT and MRI scan, and were astonished to see "massive enlargement" of
the lateral ventricles in the skull. Dr. Lionel Feuillet of Hôpital de la Timone
in Marseille said, "The images were most unusual... the brain was virtually
absent."[39] Intelligence tests showed the person had an IQ of 75, considered "borderline
intellectual functioning", just above what would be officially considered mentally
challenged.
The person was a married father of two children, and worked as a civil servant, leading
an at least superficially normal life, despite having enlarged ventricles with a decreased
volume of brain tissue. "What I find amazing to this day is how the brain can deal with
something which you think should not be compatible with life", commented Dr. Max
Muenke, a pediatric brain-defect specialist at the National Human Genome Research
Institute. "If something happens very slowly over quite some time, maybe over decades,
the different parts of the brain take up functions that would normally be done by the part
that is pushed to the side."[40][41][42]
Notable cases[edit]

 Author Sherman Alexie, born with the condition, wrote about it in his semi-
autobiographical junior fiction novel The Absolutely True Diary of a Part-Time Indian.
[43]
 Prince William, Duke of Gloucester (1689–1700) probably
contracted meningitis at birth, which resulted in this condition.[44]

 Emperor Ferdinand I of Austria (1793–1875) became emperor in 1835 despite

various health issues including hydrocephalus and epilepsy.

 Congenital Brain Malformations

 Congenital brain malformations are a group of brain defects or disorders that develop
in the womb and are present at birth.

 The fetal brain begins to develop shortly after conception and continues to grow
throughout pregnancy. Billions of neurons, or nerve cells, undergo a critical
development process in which they form brain regions. If anything interrupts this
process, particularly during the first 12 weeks of pregnancy, brain malformations can
result. Brain malformations may also develop if the skull doesn't form properly.

 While the cause is not always known, congenital brain malformations may result from
inherited genetic defects, spontaneous mutations within the embryo's genes, or
damage to the fetus caused by the mother's exposure to toxins, infection, trauma or
drug use.

 The symptoms and prognosis for congenital brain malformations vary, depending on
their type and severity. Some brain malformations are diagnosed at birth, while others
may go undetected until adolescence or adulthood.

 Babies born with congenital brain malformations are cared for in our Neuro-Intensive
Care Nursery or NICN, a component of the UCSF William H. Tooley Intensive Care
Nursery. The NICN provides specialized, high-level care for babies at risk for
neurological injury. We are also at the forefront of research studying brain
malformations in newborns, particularly premature babies.

 What are congenital brain and spine malformations?

 Congenital abnormalities, called malformations, are conditions affecting the form
and function of the nervous system.

 There are numerous variations of congenital malformations of the bone and soft
tissue of the head and spine, including neural tube defects, such as spina bifida,
encephaloceles, Chiari malformations and arachnoid cysts.

 Some congenital malformations are mild, and some are severe but correctable
with surgery by a pediatric neurosurgeon.
 Types of Congenital Brain and Spine Malformations

 Chiari Malformations
 This is a condition in which portions of the brain — the cerebellar tonsils —
protrude through the bottom opening of the skull into the upper spine, which can
put pressure on the brain or spinal cord. Chiari malformations may block the flow
of cerebrospinal fluid, leading to hydrocephalus.

 Treatments often focus on removing portions of the bone and soft tissue to
relieve pressure on the spinal cord and brain, as well as providing new pathways
to drain cerebrospinal fluid. Surgeons have several different methods for treating
these malformations, including decompression, with or without duraplasty
(opening the dura, the thick membrane covering the brain).

 Encephaloceles
 Encephaloceles are a type of neural tube defect characterized by the brain being
exposed to the outside instead of being covered by the skull and skin. It can lead
to infections and hydrocephalus.

 Surgical treatment of this condition involves removing bone and soft tissue,
draining cerebrospinal fluid, and surgically repairing or closing the
encephalocele.

 Children who have developed hydrocephalus as a result of an encephalocele will

require treatment for that condition, often with a cerebrospinal fluid shunt.
Shunting is the placement of a tube into the open area (ventricle) of the brain that
allows cerebrospinal fluid to drain to the child’s abdomen or other location where
it can be safely reabsorbed into the body.

 Arachnoid Cysts
 Arachnoid cysts  are the most common type of brain cyst. They are congenital
(present at birth) lesions that occur as a result of the splitting of the arachnoid
 membrane. The cysts are fluid-filled sacs, not tumors, appearing in one of the
three layers of tissue covering the central nervous system.

 Surgical treatment of this condition involves draining the cyst by drilling a small
hole or by opening the skull and making small openings in the cyst to open the
natural fluid pathways in the brain (fenestrating).

 Diagnosis of Congenital Brain and Spine Malformations

 If a child is born with any of the above malformations, a thorough evaluation by a
pediatrician or neurologist is needed to diagnose the problem and recommend a
plan for addressing it.

 After a thorough physical and detailed family and patient history, your doctor may
order imaging of the brain and/or spine through an MRI scan. If the MRI scan
shows any evidence of these malformations, a neurosurgical consultation is
recommended to develop the best treatment plan.

 Congenital Brain and Spine Malformations: Treatment

 A multidisciplinary approach is often beneficial for addressing children with
congenital brain and spine malformations. Neurosurgeons, craniofacial plastic
specialists and geneticists, among others, may be called upon to develop your
child’s treatment plan and determine what kind of surgery may be appropriate.

 If a congenital brain or spine malformation is mild and not causing any signs or
symptoms in your child, the neurosurgeon may recommend observation, which
means regular visits and testing to monitor your child’s condition.

 If your child does undergo surgery, follow-up care is extremely important in

tracking the progress of your child’s recovery. Your pediatric neurosurgeon will
schedule follow-up appointments to ensure your child is making the best
recovery possible.