Hydrocephalus : Causes, Symptoms , Diagnosis and Treatment
Neurology / / April 20, 2016
What is hydrocephalus?
Hydrocephalus, or also called water on the brain - a disease that is typically characterized by excessive accumulation of CSF (cerebrospinal) fluid in the ventricular system of the brain, resulting in difficulty moving it from place secretion - from the brain ventricles - to the absorption site inthe circulatory system, or as a result of violations of absorption.Simply stated - a condition in which excess fluid accumulation in the brain is a major characteristic.The term "hydrocephalus" is derived from the Greek words "hydro" (water) and "tsefalus" (head).
Although hydrocephalus was once known as "water in the brain", "water" - is, in fact, cerebrospinal fluid (CSF).It is a clear liquid that surrounds the brain and spinal cord.Very large spaces of the brain in which over-accumulated cerebrospinal fluid, called "ventricles" (the ventricles of the brain -. A cavity within the different parts of the brain are derived from the cavities of the brain bubbles, from which and formed
ventricular system consists of four ventricles connected by narrow passages.Generally, cerebrospinal fluid flows through the ventricles, exits the tank at the base of the brain (the closed spaces, which serve as reservoirs) bathes the brain and spinal cord surface, and then absorbed into the bloodstream.
CSF has three important life-sustaining functions:
1) keeps the brain and spinal cord tissue as a kind of "cushion" or "shock absorber" for them;
2) acts as a "vehicle" for the delivery of nutrients to the brain and removing waste;
3) flows between the skull and spinal column, compensating for changes in intracranial blood volume (in other words, adjusts the amount of blood in the brain).
balance between production and absorption of brain and spinal cord CSF is critically important.Because cerebrospinal fluid is produced continuously.Medical conditions that block within its normal or absorption, lead to excessive accumulation of cerebrospinal fluid.The result is a strong fluid pressure at the brain tissue, causing hydrocephalus.
What are the different types of hydrocephalus?
Hydrocephalus can be congenital or acquired.
Congenital hydrocephalus is present at birth and is associated with a genetic predisposition, or malformations of critical structures inside the brain during development.For example, stenosis (narrowing) of the aqueduct of Sylvius (or the average human brain waterpipe -. A narrow, 2 cm long, the channel passing within the midbrain and connects the 3rd and 4th ventricles around the aqueduct is a central gray matter, which laid the nucleus IIIand IV pairs of cranial nerves, etc.). 10% of all cases of hydrocephalus in infants.Acquired hydrocephalus may occur at any time after the birth and is usually associated with traumatic injury, disease, tumors, intracranial hemorrhage, and infections.
Hydrocephalus can be acute, subacute, or chronic disease.There are several different forms of hydrocephalus, including - communicating, unreported and normal.
Hydrocephalus occurs when after leaving the ventricular cerebrospinal fluid flow is blocked.This form is called "communicating hydrocephalus," because the cerebrospinal fluid can flow between the ventricles, which are still open.Backlog, "obstructive" or unreported hydrocephalus (occurs as a result of the blockade, obstruction of the outflow tract of CSF in the ventricular system. It is often caused by a tumor or other bulk process, brain abscess, intraventricular hematoma, granulomatous inflammation, ependimatitom, ventriculitis, arachnoid cysts and other diseases)It occurs when the flow of cerebrospinal fluid is blocked by one or more narrow passages which connect the ventricles.One of the most common causes of hydrocephalus is "akveduktalny stenosis" in which a result of the narrowing of the aqueduct between the third and fourth ventricles formed a small passage in the middle of the brain.
There are two other forms of hydrocephalus which do not fit exactly into the above-mentioned categories and affect mainly adults: hydrocephalus ex-vacuum (when a stroke or traumatic injury can cause damage to the brain; in these cases, it may actually be the atrophy of brain tissue) andnormal pressure hydrocephalus.
So hydrocephalus ex-vacuo occurs when stroke or injuries cause brain damage.In these cases, brain tissue may actually decline.Normal (normal) Hydrocephalus most often occurs in older people.It can be the result of subarachnoid hemorrhage (SAH - bleeding into the cavity between the arachnoid and pia mater, and often occurs spontaneously, as a result of rupture of an arterial aneurysm or due to traumatic brain injury), infection, tumor, or complications after surgery, although many people have normal pressure hydrocephalusIt develops without obvious causes, when none of the above factors are not present.
who develop hydrocephalus?
number of people who develop hydrocephalus or who are currently living with them, it is difficult to establish because there is no national registry or database of people with this disease.However, experts estimate that hydrocephalus affects approximately one out of every five hundred children.
What causes hydrocephalus?
- age: there are two peaks associated with morbidity and age of onset hydrocephalus.The first peak in childhood and is associated with a number of congenital malformations.The second peak occurs in adulthood and is mainly due to normal hydrocephalus.Approximately 60% of the total number of cases of hydrocephalus is congenital or acquired in childhood.Hydrocephalus is a common and significant problem of pediatric;
- floor: there is no difference in the incidence of hydrocephalus between men and women.One exception is Bickers-Adams syndrome (or Hydrocephalus due to congenital stenosis of aqueduct of Sylvius, X-linked hydrocephalus, X-linked stenosis aqueduct - the most common form of hereditary congenital hydrocephalus, clinical core of which - the expansion of the brain ventricles and mental retardation, frequent spastic paraparesisand bringing the thumbs of hands), which is an X-linked recessive hydrocephalus, which occurs only in males and affects approximately 1/30000 males at birth.Normal pressure hydrocephalus is slightly more common in men;
- genetics: there are a number of rare genetic causes of hydrocephalus, the most important of which is Bickers-Adams syndrome.
Causes and risk factors
Congenital causes in babies and infants:
- Akveduktalnye anomaly: unreported hydrocephalus develops most often in infants and children because of the violations of thework of the aqueduct of Sylvius or defeat in the fourth ventricle.Results - by abnormally narrow to completely occluded (in medicine, this term refers to the periodic violation of patency, or obturation, hollow anatomical structures due to the destruction of their walls) aqueduct;
-. Anomaly Arnold Chiari , types I and II (a congenital abnormality hindbrain development, manifested mismatch PCF sizes, posterior fossa and brain structures in this area This leads to the omission of the tonsils of the cerebellum and brain stem in large magnum. opening and infringement on this level incidence of the disease ranges from 3.3 to 8.2 observations per 100,000 population anomaly of Arnold-Chiari type I -.. omission of PCF structures in the spinal canal below the plane of the foramen magnum When anomalies Arnold-Chiari type II usually occurs caudaldislocation of the lower divisions of the worm, the medulla oblongata, and the IV ventricle and often develop hydrocephalus. The disease manifests symptoms of varying severity of lesions of the medulla oblongata, cerebellum, the symptoms of spinal cord lesions, etc.) is characterized by progressive hydrocephalus and myelomeningocele (hernial protrusion of the spinal cord through a spina bifida.Hernial bag is made of leather and the pia mater, and its contents filled the spinal cord and cerebrospinal fluid.Often the defect is localized in the lumbosacral area, and cervical spine);
- Congenital infection (eg, cytomegalovirus, toxoplasmosis, rubella);spina bifida and neural tube defects;hemorrhage or internal bleeding in the brain and other reasons.
Acquired causes in babies and infants:
- tumors of the brain (benign or malignant);
- cysts, abscesses, hematomas;
- intraventricular hemorrhage (IVH - the most common problem among premature infants, occurs in the ventricles of the child's brain when small blood vessels burst, causing bleeding in most cases gradually bleeding stops, the blood vessels heal Most often, surgery to eliminate..IVH can not be performed. If the brain tissue is damaged, the child may have problems with future development), which most often affects preterm birth.It may also be due to head injuries or abnormal vessel rupture;
- Subarachnoid hemorrhage (SAH - bleeding into the cavity between the arachnoid and pia mater, and often occurs spontaneously, as a result of rupture of an arterial aneurysm or due to traumatic brain injury): it is most often followed by communicating hydrocephalus and can block spider villi leadingobstruction to the flow of cerebrospinal fluid.This is usually the result of intraventricular hemorrhage in premature baby;
- infections, particularly bacterial meningitis (fulminant, often fatal pyogenic infection of the meninges, inflammation of the brain or spinal cord, is caused by a bacterial infection) and cerebral abscess (brain abscess, CNS abscess - appears in the brain and is caused by the accumulationand inflammation of infected material formed by a local or a remote source of infection and immune cells);
- Increased venous sinus pressure;
- leukemic infiltrates the CNS (infiltration - congestion in the tissues of the body of cellular elements with an admixture of blood and lymph, leukemic infiltration of the CNS - the accumulation in the central nervous system, blood cells that is affected by leukemia, or leukemia nerve fibers) can fill in the subarachnoid space and cause hydrocephalus.
Acquired causes in adults:
hydrocephalus can cause :
- benign or malignant tumors can cause blockage of cerebrospinal fluid.For example, these types of tumors, such as: ependymoma, astrocytoma cells, choroid plexus papilloma craniopharyngioma, pituitary adenoma, or hypothalamic glioma of the optic nerve, hamartoma and metastatic tumors - most commonly associated with hydrocephalus;
- subarachnoid hemorrhage , which accounts for one-third of cases of hydrocephalus in adults.Gossamer villi block hemorrhage, but communicating hydrocephalus persists between the ventricles and subarachnoid space, creating a form of hydrocephalus, which is at the same time and obstructive, and communicating;
- traumatic brain injury: hydrocephalus is usually caused by subarachnoid hemorrhage, which most often occurs due to traumatic head injury;
- idiopathic hydrocephalus: is about one third of cases of hydrocephalus;
- posterior fossa as a consequence of surgery: normal cerebrospinal fluid path may be blocked as a result of the operation;
- akveduktalny congenital stenosis: hydrocephalus may be asymptomatic until adulthood;
- infection: such as bacterial meningitis, cerebral abscess;
- drugs: for example, the infusion of infliximab.
Causes normal hydrocephalus:
- subarachnoid hemorrhage;
- traumatic brain injury;
- infection, especially meningitis;
- surgery of the posterior fossa;
idiopathic hydrocephalus: approximately one third of cases of hydrocephalus.
Rare causes of hydrocephalus
- Bickers-Adams syndrome;
- excessive production of cerebrospinal fluid (CSF) in the choroid plexus papilloma - a very rare, but the real cause of hydrocephalus.
- recent head injury;
- premature infants who were born at gestational age less than 34 weeks or weighing less than 1.8 kg at birth, there is a high risk of intraventricular hemorrhage, which can lead to hydrocephalus.
symptoms of hydrocephalus
Symptoms of acquired hydrocephalus may include:
- nausea and vomiting;
- blurry vision;
- balance problems;
- problems with bladder control;
- problems with thinking and memory.
Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to this state (ie, the ability to tolerate it).For example, the ability to compensate for increased pressure baby excess cerebrospinal fluid ventricles and expansion - is different from the ability of an adult.Skull baby may grow more or less easy to accommodate the accumulated cerebrospinal fluid because the sutures (the fibrous joints that connect the bones of the skull) are soft and not yet closed.
Infants often the most obvious sign of hydrocephalus - the rapid growth of head circumference or an unusually large head size.Other symptoms may include: vomiting, sleepiness, irritability, downward strabismus and seizures.
Older children and adults can experience different symptoms because their skulls can not expand to accommodate the accumulated cerebrospinal fluid.The symptoms they may include: headache, accompanied by vomiting, nausea, papilledema, which is part of the optic nerve, vision disorder, including double vision, strabismus down, problems with balance, poor coordination, gait disturbance,urinary incontinence, slowing or loss of development progress, lethargy, drowsiness, irritability, as well as other changes in personality or consciousness, including memory loss.
normal hydrocephalus Symptoms include problems with walking, bladder control violation, leading to frequent urination, mental disorders and progressive dementia.A person with this type of hydrocephalus may have a general slowing of movements or may complain that his or her feet as if the "stuck".Some of these symptoms may also be part or other serious disorders - such as for example Alzheimer's disease (this progressive memory paralysis; the most common form of dementia, an incurable degenerative disease of the central nervous system characterized by progressive loss of memory), Parkinson's disease (or parkinsonism -