Hydrocephalus

Abnormal increase in cerebrospinal fluid in the ventricles of the brain

Not to be confused with Hypocephalus or Hydranencephaly.

This article is about the medical condition. For the creature in American folklore, see Melon heads. For the trilobite, see Hydrocephalus (trilobite).

Medical condition

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.
Pronunciation|

* /ˌhaɪdroʊˈsɛfələs/[2]

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, brain bleed during birth, intraventricular hemorrhage[1]
Diagnostic method| Based on symptoms and medical imaging[1]
Treatment| Surgery[1]
Prognosis| Variable, often normal life[1]
Frequency| Varies throughout the world, from 1 per 256 live births to 1 per 9,000, depending on access to prenatal health care, prenatal tests, and abortion[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 is an option in some 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, permanent disability or death 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]

## Signs and symptoms

[edit]

Illustration showing different effects of hydrocephalus on the brain and cranium Adult showing cranial deformity from pediatric hydrocephalus

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).[8]

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.[9] 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.[10] Hearing loss is a rare but well-known sequela of procedures resulting in CSF loss.[9] Elevated ICP may result in uncal or tonsillar herniation, with resulting life-threatening brain stem compression.[11]

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.[11]

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:[11]

* Eyes that appear to gaze downward
* Irritability
* Seizures
* Separated sutures
* Sleepiness
* Vomiting

Symptoms that may occur in older children can include:[11]

* 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[12]

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 issues such as nonverbal learning disorder, difficulty understanding complex and abstract concepts, difficulty 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.[13][14] 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.[15]

## Causes

[edit]

Hydrocephalus can be caused by a combination of factors and is not fully understood. Any medical condition that interrupts the flow of cerebrospinal fluid (CSF) can cause this build-up of excess fluid. This occurs due to blocked pathways of cerebrospinal fluid (CSF), issues with CSF reabsorption, or increased CSF production[16].

Hydrocephalus can be classified as primary or secondary (acquired) based on the cause.[17]

### Primary

[edit]

Primary hydrocephalus involves congenital, developmental, and genetic factors that lead to excess CSF build-up.[17] Up to 50% of the causes of primary hydrocephalus are genetic.[18]

Congenital hydrocephalus is defined by the presence of excess CSF at birth. It occurs due to a combination of factors, with genetic causes 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.[19]

Developmental disorders including neural-tube defects, arachnoid cysts, Dandy–Walker syndrome, and Arnold–Chiari malformation can also cause primary hydrocephalus. About 80–90% of fetuses or newborn infants with the neural-tube defect spina bifida—often associated with meningocele or myelomeningocele—develop hydrocephalus.[20]

### Secondary (Acquired)

[edit]

Secondary hydrocephalus is acquired as a consequence of CNS infections, meningitis, brain tumors, head trauma, toxoplasmosis, or intracranial hemorrhage (subarachnoid or intraparenchymal).[21]

Intraventricular hemorrhage, or bleeding within the ventricles of the brain, leads to hydrocephalus in 51-89% of patients.[22] This is because the blood in the ventricles blocks the regular flow of CSF, leading to build-up of excess CSF[22]

Spontaneous intracerebral and intraventricular hemorrhage with hydrocephalus shown on CT scan[23]

Normal pressure hydrocephalus (NPH) most often occurs in elderly patients with symptoms including gait disturbance, urinary incontinence, and cognitive issues.[24] It is commonly divided into two categories, idiopathic NPH (with unknown cause) and secondary NPH (due to trauma, hemorrhage, etc.).[24]

Hydrocephalus can also be caused by overproduction of CSF (relative obstruction) (e.g., choroid plexus papilloma, villous hypertrophy).[25][26]

Bilateral ureteric obstruction is a rare, but reported, cause of hydrocephalus.

## Type

[edit]

The classification of communicating vs. noncommunicating hydrocephalus can be used to describe the mechanisms of hydrocephalus. These terms describe the nonobstructive vs. obstructive mechanisms of the excess CSF build-up.

### 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 reabsorption of CSF, causing diffuse ventricular dilatation.[27]

An adult with congenital hydrocephalus in the Philippines

### Noncommunicating

[edit]

Noncommunicating hydrocephalus, or obstructive hydrocephalus, is caused by an obstruction to the flow of CSF.[28]

* 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.[29]
* 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.[30] 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.[30]

## Mechanism

[edit]

Diagram showing CSF flow and neuroanatomy.

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.[31]

CSF is formed by the choroid plexus (in the lateral ventricles) and the ependymal lining of the ventricular system. It flows through the ventricular system through the following pathway:[32]

Lateral Ventricles → Interventricular Foramen of Monro → Third Ventricle → Cerebral Aqueduct → Fourth Ventricle

The CSF then exits the fourth ventricle through the median aperture and lateral aperture. It goes into the subarachnoid space or central canal of the spinal cord. Then, it is absorbed by the vili of arachnoid granulations into the blood circulation[32].

There is also theory about CSF drainage into the lymphatic vessel system[32].

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.[33]

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.[34][35] The cochlear aqueduct connects the Perilymphatic space of the inner ear with the subarachnoid space of the posterior cranial fossa.[36] 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.[34]

CSF can accumulate within the ventricles, this condition is called internal hydrocephalus and may result 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.[37]

## Treatments

[edit]

### Procedures

[edit]

Baby recovering from shunt surgery Ventriculoperitoneal shunt placement in child with hydrocephalus

Hydrocephalus is treated through surgery by creating creating a way for the excess fluid to drain away. An external ventricular drain (EVD), also known as an extraventricular drain or ventriculostomy, provides relief in the short term.[38] In the long term, some people will need any of the various types of cerebral shunts. It involves the placement of a ventricular catheter (a tube made of silastic) into the cerebral ventricles. This creates a way to bypass the flow obstruction/malfunctioning arachnoidal granulations. The excess fluid drains into other body cavities where it can be resorbed. Most shunts drain the fluid into the peritoneal cavity (ventriculoperitoneal shunt). Other shunts drain the fluid into the right atrium (ventriculoatrial shunt), pleural cavity (ventriculopleural shunt), and gallbladder.[39]

Further information on the non-invasive diagnostic medical device: ShuntCheck

A shunt system can also be placed in the lumbar space of the spine. This allows the excess fluid to be redirected to the peritoneal cavity (lumbar-peritoneal shunt).[40] Another treatment for obstructive hydrocephalus is an endoscopic third ventriculostomy (ETV). This surgery creates an opening in the floor of the third ventricle so that CSF flows directly to the basal cisterns. This treatment can shortcut any obstruction like aqueductal stenosis. This may or may not be appropriate based on individual anatomy. Some infants can be treated with ETV and choroid plexus cauterization. Choroid plexus cauterization 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.[41][42]

### External hydrocephalus

[edit]

External hydrocephalus is generally seen in infants. It involves enlarged fluid spaces or subarachnoid spaces outside of the brain. The most common sign is a head circumference above the 90th percentile. In most cases, no other signs or symptoms are reported.[43] Rarely reported symptoms include a tense anterior fontanel, developmental delay, seizures, irritability, and vomiting.[44] Usually, this condition is benign. It resolves spontaneously by two to three years of age.[45] Thus, it usually does not need insertion of a shunt. If surgical treatment is required, a ventriculoperitoneal shunt is usually preferred.[44] Other treatment options include using medications like acetazolamide.[43] The condition can be diagnosed and monitored with brain sonography and CT/MRI. These tests and a good medical history can help to identify external hydrocephalus from similar conditions: subdural hemorrhages or symptomatic chronic extra-axial fluid collections which are accompanied by vomiting, headaches, and seizures.[46][47]

### Shunt complications

[edit]

Some risk exists of infection being introduced into the brain through cerebral shunts, as they must be replaced as the person grows.[48][49] 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.[50]

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,[36] 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.[51]

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.[36] 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.[52]

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.[53]

Standardized protocols for inserting cerebral shunts have been shown to reduce shunt infections.[54][55] There is tentative evidence that preventative antibiotics may decrease the risk of shunt infections.[56]

## Epidemiology

[edit]

It is estimated that congenital hydrocephalus occurs in 8.5 out of 10,000 live births globally. It was found that disease burden is more concentrated in Africa, Asia, and South America.[57] The hydrocephalus disease burden are concentrated in the developing world while North America has the fewest number of cases. A systematic review in 2019 estimated that there are 180,000 childhood hydrocephalus cases from the African continent per year, followed by 90,000 cases from Southeast Asia and the Western Pacific. Latin America also has a high prevalence of hydrocephalus. Congenital hydrocephalus was found to be associated with the health of the affected individual's mother, exposure to medications in the prenatal period such as antibiotice, the affected individuals environment like altitude, and low socioeceonomic status.[57] In adults that are 18-64 years-old, about 11 in 100,000 cases of hydrocephalus is estimated. For adults over 65 years of age, 175 in 100,000 cases is estimated.[58]

## History

[edit]

Skull of a hydrocephalic child (1800s)

In the pre-historic area, there were various paintings or artifacts depicting children or adults with macrocephaly (large head) or clinical findings of hydrocephalus.[59] The earliest scientific description of hydrocephalus was written by the ancient Greek physician Hippocrates, who coined the word 'hydrocephalus' from the Greek ὕδωρ, hydōr meaning 'water' and κεφαλή, kephalē meaning 'head'.[60] A more accurate description was later given by the Roman physician Galen in the second century AD.[60]

The first clinical description of an operative procedure for hydrocephalus appears in the Al-Tasrif (1,000 AD) by the Arab surgeon Abulcasis, who described the evacuation of superficial intracranial fluid in hydrocephalic children.[60] He described it in his chapter on neurosurgical disease, describing infantile hydrocephalus as being caused by mechanical compression. He wrote:[60]

> 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.[60] It remained an intractable condition until the 20th century, when cerebral shunt and other neurosurgical treatment modalities were developed.[citation needed]

The first hydrocephalus shunt was placed in 1956.[61]

## 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][62]

### 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, such as the One Small Voice Foundation, promote awareness and fundraising activities.[citation needed]

### 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."[63] Intelligence tests showed the person had an IQ of 75, considered "Borderline intellectual functioning", just above what would be officially classified as intellectually disabled.[64][65]

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."[66][67][68]

### Notable cases

[edit]

* Ice hockey player Colby Cave had acute obstructive hydrocephalus due to a colloid cyst.[69]
* 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.[70]
* Prince William, Duke of Gloucester (1689–1700), probably contracted meningitis at birth, which resulted in this condition.[71]
* Emperor Ferdinand I of Austria (1793–1875) became emperor in 1835 despite various health issues including hydrocephalus and epilepsy.[citation needed]
* In the American folklore of the states of Ohio, Michigan, and Connecticut, an urban legend exists about the melon heads, the inbred descendants of families of people born with hydrocephaly.
* Masato Kudo, a professional soccer player, died of hydrocephalus on October 21, 2022.

## References

[edit]

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57. ^ a b Gili, Juan Antonio; López‐Camelo, Jorge Santiago; Nembhard, Wendy N.; Bakker, Marian; de Walle, Hermien E. K.; Stallings, Erin B.; Kancherla, Vijaya; ECEMC Peripheral Group; Contiero, Paolo; Dastgiri, Saeed; Feldkamp, Marcia L.; Nance, Amy; Gatt, Miriam; Martínez, Laura; Canessa, María Aurora (2022-07-15). "Analysis of early neonatal case fatality rate among newborns with congenital hydrocephalus, a 2000–2014 multi‐country registry‐based study". Birth Defects Research. 114 (12): 631–644\. doi:10.1002/bdr2.2045. ISSN 2472-1727. PMC 9288486. PMID 35633200.631-644&rft.date=2022-07-15&rft_id=https://www.ncbi.nlm.nih.gov/pmc/articles/PMCPMC9288486#id-name=PMC&rft.issn=2472-1727&rft_id=info:pmid/35633200&rft_id=info:doi/10.1002/bdr2.2045&rft.aulast=Gili&rft.aufirst=Juan+Antonio&rft.au=López‐Camelo,+Jorge+Santiago&rft.au=Nembhard,+Wendy+N.&rft.au=Bakker,+Marian&rft.au=de+Walle,+Hermien+E.+K.&rft.au=Stallings,+Erin+B.&rft.au=Kancherla,+Vijaya&rft.au=ECEMC+Peripheral+Group&rft.au=Contiero,+Paolo&rft.au=Dastgiri,+Saeed&rft.au=Feldkamp,+Marcia+L.&rft.au=Nance,+Amy&rft.au=Gatt,+Miriam&rft.au=Martínez,+Laura&rft.au=Canessa,+María+Aurora&rft_id=https://onlinelibrary.wiley.com/doi/10.1002/bdr2.2045&rfr_id=info:sid/en.wikipedia.org:Hydrocephalus" class="Z3988">`{{cite journal}}`: CS1 maint: PMC format (link)
58. ^ Tullberg, Mats; Toma, Ahmed K.; Yamada, Shigeki; Laurell, Katarina; Miyajima, Masakazu; Watkins, Laurence D.; Wikkelsø, Carsten (2024-03-01). "Classification of Chronic Hydrocephalus in Adults: A Systematic Review and Analysis". World Neurosurgery. 183: 113–122\. doi:10.1016/j.wneu.2023.12.094. ISSN 1878-8750.113-122&rft.date=2024-03-01&rft_id=info:doi/10.1016/j.wneu.2023.12.094&rft.issn=1878-8750&rft.aulast=Tullberg&rft.aufirst=Mats&rft.au=Toma,+Ahmed+K.&rft.au=Yamada,+Shigeki&rft.au=Laurell,+Katarina&rft.au=Miyajima,+Masakazu&rft.au=Watkins,+Laurence+D.&rft.au=Wikkelsø,+Carsten&rft_id=https://linkinghub.elsevier.com/retrieve/pii/S1878875023018260&rfr_id=info:sid/en.wikipedia.org:Hydrocephalus" class="Z3988">
59. ^ Cinalli, G (2019). "History of Hydrocephalus and Its Surgical Treatment". In Cinalli, Giuseppe; Özek, M. Memet; Sainte-Rose, Christian (eds.). Pediatric Hydrocephalus. Cham: Springer International Publishing. p. 3\. doi:10.1007/978-3-319-27250-4. ISBN 978-3-319-27248-1. S2CID 128359318.
60. ^ a b c d e Aschoff A, Kremer P, Hashemi B, Kunze S (October 1999). "The scientific history of hydrocephalus and its treatment". Neurosurgical Review. 22 (2–3): 67–93, discussion 94–5. doi:10.1007/s101430050035. PMID 10547004. S2CID 10077885.2–3&rft.pages=67-93,+discussion+94-5&rft.date=1999-10&rft_id=https://api.semanticscholar.org/CorpusID:10077885#id-name=S2CID&rft_id=info:pmid/10547004&rft_id=info:doi/10.1007/s101430050035&rft.aulast=Aschoff&rft.aufirst=A&rft.au=Kremer,+P&rft.au=Hashemi,+B&rft.au=Kunze,+S&rfr_id=info:sid/en.wikipedia.org:Hydrocephalus" class="Z3988">
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## External links

[edit]

Wikimedia Commons has media related to Hydrocephalus.

* Guidelines for pediatric hydrocephalus

Classification|

* ICD-10: G91, Q03
* ICD-9-CM: 331.3, 331.4, 741.0, 742.3
* OMIM: 236600
* MeSH: D006849
* DiseasesDB: 6123

|

External resources|

* MedlinePlus: 001571
* eMedicine: neuro/161
* Patient UK: Hydrocephalus

* v
* t
* e

Diseases of the nervous system, primarily CNS

Inflammation|

| Brain|

* Encephalitis
* Viral encephalitis
* Herpesviral encephalitis
* Limbic encephalitis
* Encephalitis lethargica
* Cavernous sinus thrombosis
* Brain abscess
* Amoebic

|

Brain and spinal cord|

* Encephalomyelitis
* Acute disseminated
* Meningitis
* Meningoencephalitis

Brain/
encephalopathy|

| Degenerative|

| Extrapyramidal and
movement disorders|

* Basal ganglia disease
* Parkinsonism
* PD
* Postencephalitic
* NMS
* NBIA
* PKAN
* Tauopathy
* PSP
* Striatonigral degeneration
* Hemiballismus
* HD
* OA

* Dyskinesia
* Dystonia
* Status dystonicus
* Spasmodic torticollis
* Meige's
* Blepharospasm
* Athetosis
* Chorea
* Choreoathetosis
* Myoclonus
* Myoclonic epilepsy
* Akathisia

* Tremor
* Essential tremor
* Intention tremor
* Restless legs
* Stiff-person

|

Dementia|

* Tauopathy
* Alzheimer's
* Early-onset
* Primary progressive aphasia
* Frontotemporal dementia/Frontotemporal lobar degeneration
* Pick's
* Lewy bodies dementia
* Posterior cortical atrophy

Creutzfeldt–Jakob disease

* Vascular dementia

Mitochondrial disease|

* Leigh syndrome

Demyelinating|

* Autoimmune
* Inflammatory
* Multiple sclerosis
* For more detailed coverage, see Template:Demyelinating diseases of CNS

Episodic/
paroxysmal|

| Seizures and epilepsy|

* Focal
* Generalised
* Status epilepticus
* For more detailed coverage, see Template:Epilepsy

|

Headache|

* Migraine
* Cluster
* Tension
* For more detailed coverage, see Template:Headache

Cerebrovascular|

* TIA
* Stroke
* For more detailed coverage, see Template:Cerebrovascular diseases

Other|

* Sleep disorders
* For more detailed coverage, see Template:Sleep

CSF|

* Intracranial hypertension
* Hydrocephalus
* Normal pressure hydrocephalus
* Choroid plexus papilloma
* Idiopathic intracranial hypertension
* Cerebral edema
* Intracranial hypotension

Other|

* Brain herniation
* Reye syndrome
* Hepatic encephalopathy
* Toxic encephalopathy
* Hashimoto's encephalopathy
* Static encephalopathy

Both/either|

| Degenerative|

| SA|

* Friedreich's ataxia
* Ataxia–telangiectasia

|

MND|

* UMN only:
* Primary lateral sclerosis
* Pseudobulbar palsy
* Hereditary spastic paraplegia

* LMN only:
* Distal hereditary motor neuronopathies
* Spinal muscular atrophies
* SMA
* SMAX1
* SMAX2
* DSMA1
* Congenital DSMA
* Spinal muscular atrophy with lower extremity predominance (SMALED)
* SMALED1
* SMALED2A
* SMALED2B
* SMA-PCH
* SMA-PME
* Progressive muscular atrophy
* Progressive bulbar palsy
* Fazio–Londe
* Infantile progressive bulbar palsy

* both:
* Amyotrophic lateral sclerosis

* v
* t
* e

Congenital malformations and deformations of nervous system

Brain|

| Neural tube defect|

* Anencephaly
* Acephaly
* Acrania
* Acalvaria
* Iniencephaly
* Encephalocele
* Chiari malformation

|

Other|

* Microcephaly
* Congenital hydrocephalus
* Dandy–Walker syndrome

* other reduction deformities
* Holoprosencephaly
* Lissencephaly
* Microlissencephaly
* Pachygyria
* Hydranencephaly

* Agenesis of the corpus callosum
* Septo-optic dysplasia
* Megalencephaly
* Hemimegalencephaly

* CNS cyst
* Porencephaly
* Schizencephaly

* Polymicrogyria
* Bilateral frontoparietal polymicrogyria

Spinal cord|

| Neural tube defect|

* Spina bifida
* Rachischisis

|

Other|

* Currarino syndrome
* Diastomatomyelia
* Syringomyelia

Authority control databases: National |

* Germany
* United States
* France
* BnF data
* Japan
* Czech Republic
* Latvia
* Israel

|

*[v]: View this template
*[t]: Discuss this template
*[e]: Edit this template