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HomePediatric Specialties > Neurosurgery > Neurosurgery Conditions

Neurosurgery Conditions

Arachnoid cysts

Arachnoid cysts are present at birth and are the result of developmental abnormalities in the brain and spinal cord that arise during the early weeks of gestation.  Secondary arachnoid cysts are not as common as primary cysts and develop as a result of head injury, meningitis, or tumors, or as a complication of brain surgery.  The majority of arachnoid cysts form outside the temporal lobe of the brain in an area of the skull known as the middle cranial fossa.  Arachnoid cysts involving the spinal cord are rarer.  The location and size of the cyst determine the symptoms and when those symptoms begin.  Most individuals with arachnoid cysts develop symptoms before the age of 20, and especially during the first year of life, but some people with arachnoid cysts never have symptoms.  Males are four times more likely to have arachnoid cysts than females. 

Symptoms

Typical symptoms of an arachnoid cyst around the brain include headache, nausea and vomiting, seizures, hearing and visual disturbances, vertigo, and difficulties with balance and walking.  Arachnoid cysts around the spinal cord compress the spinal cord or nerve roots and cause symptoms such as progressive back and leg pain and tingling or numbness in the legs or arms.  Diagnosis usually involves a brain scan or spine scan using diffusion-weighted MRI (magnetic resonance imaging) which helps distinguish fluid-filled arachnoid cysts from other types of cysts.   

Untreated, arachnoid cysts may cause permanent severe neurological damage when progressive expansion of the cyst(s) or bleeding into the cyst injures the brain or spinal cord.  Symptoms usually resolve or improve with treatment. 

Treatment

There has been active debate about how to treat arachnoid cysts.  The need for treatment depends mostly upon the location and size of the cyst.  If the cyst is small, not disturbing surrounding tissue, and not causing symptoms, some doctors will refrain from treatment.  In the past, doctors placed shunts in the cyst to drain its fluid.  Now with microneurosurgical techniques and endoscopic tools that allow for minimally invasive surgery, more doctors are opting to surgically remove the membranes of the cyst or open the cyst so its fluid can drain into the cerebrospinal fluid and be absorbed. 

NIH: National Institute of Neurological Disorders and Stroke

AVMs

Arteriovenous malformations (AVMs) are abnormal, snarled tangles of blood vessels that cause multiple irregular connections between the arteries and veins.  These malformations most often occur in the spinal cord and in any part of the brain or on its surface, but can develop elsewhere in the body.

Normally, arteries carry oxygen-rich blood away from the heart to the body’s cells, organs, and tissues; veins return blood with less oxygen to the lungs and heart.  But in an AVM, the absence of capillaries—a network of small blood vessels that connect arteries to veins and deliver oxygen to cells—creates a shortcut for blood to pass directly from arteries to veins and bypass tissue, which can lead to tissue damage and the death of nerve cells and other cells.  Over time, some AVMs get progressively larger as the amount of blood flow increases.

In some cases, a weakened blood vessel may burst, spilling blood into the brain (hemorrhage) that can cause stroke and brain damage.  Other neurological problems include headache, weakness, seizures, pain, and problems with speech, vision, or movement.  In most cases, people with neurological AVMs experience few, if any, significant symptoms.

It is unclear why AVMs form.  Most often AVMs are congenital, but they can appear sporadically.  In some cases the AVM may be inherited, but it is more likely that other inherited conditions increase the risk of having an AVM.  The malformations tend to be discovered only incidentally, usually during treatment for an unrelated disorder or at autopsy.  It is estimated that brain AVMs occur in less than one percent of the general population; each year about one percent of those with AVMs will die as a direct result of the AVM.

Symptoms

Seizures and headaches that may be severe are the most generalized symptoms of AVMs.  Seizures can be focal (meaning they involve a small part of the brain) or generalized (widespread), involving convulsions, a loss of control over movement, or a change in a person’s level of consciousness.  Headaches can vary greatly in frequency, duration, and intensity, sometimes becoming as severe as migraines.  Pain may be on either one side of the head or on both sides.  Sometimes a headache consistently affecting one side of the head may be closely linked to the site of an AVM.  Most often, the location of the pain is not specific to the malformation and may encompass most of the head.

AVMs also can cause a wide range of more specific neurological symptoms that vary from person to person, depending primarily upon the location of the AVM.  Such symptoms may include:

  • Muscle weakness or paralysis in one part of the body
  • A loss of coordination (ataxia) that can lead to such problems as gait disturbances
  • Difficulties carrying out tasks that require planning (apraxia)
  • Back pain or weakness in the lower extremities caused by a spinal AVM
  • Dizziness
  • Visual problems such as a loss of part of the visual field, inability to control eye movement, or swelling of a part of the optic nerve
  • Difficulty speaking or understanding language (aphasia)
  • Abnormal sensations such as numbness, tingling, or spontaneous pain
  • Memory deficits
  • Confusion, hallucinations, or dementia.

AVMs may also cause subtle learning or behavioral disorders in some people during their childhood or adolescence, long before more obvious symptoms become evident.

Symptoms caused by AVMs can appear at any age.  Because the abnormalities tend to result from a slow buildup of neurological damage over time, they are most often noticed when people are in their twenties or older.  If AVMs do not become symptomatic by the time people reach their late forties or early fifties, they tend to remain stable and are less likely to produce symptoms. 

Although most neurological AVMs have very few, if any, significant symptoms, one particularly severe type of AVM causes symptoms to appear at, or very soon after, birth.  Called a vein of Galen defect after the major blood vessel involved, this lesion is located deep inside the brain.  It is frequently associated with hydrocephalus (an accumulation of fluid within certain spaces in the brain, often with visible enlargement of the head), swollen veins visible on the scalp, seizures, failure to thrive, and congestive heart failure.  Children born with this condition who survive past infancy often remain developmentally impaired.

Treatment

There are several options for treating AVMs.  Although medication can often lessen general symptoms such as headache, back pain, and seizures caused by AVMs and other vascular lesions, the definitive treatment for AVMs is either surgery or focused radiation therapy.  Venous malformations and capillary telangiectases rarely require surgery.  Cavernous malformations are usually well defined enough for surgical removal, but surgery on these lesions is less common than for AVMs because they do not pose the same risk of hemorrhage.

Because so many variables are involved in treating AVMs, doctors must assess the danger posed to individuals largely on a case-by-case basis. A hemorrhage from an untreated AVM can cause serious neurological deficits or death, leading many clinicians to recommend surgical intervention whenever the physical characteristics of an AVM appear to indicate a greater-than-usual likelihood of significant bleeding and subsequent neurological damage.

Three surgical options are used to treat AVMs:  conventional surgeryendovascular embolization, and radiosurgery.  The choice of treatment depends largely on the size and location of an AVM.   Endovascular embolization and radiosurgery are less invasive than conventional surgery and offer safer treatment options for some AVMs located deep inside the brain.

  • Conventional surgery involves entering the brain or spinal cord and removing the central portion of the AVM, including the fistula, while causing as little damage as possible to surrounding neurological structures.  This surgery is most appropriate when an AVM is located in a superficial portion of the brain or spinal cord and is relatively small in size.  AVMs located deep inside the brain generally cannot be approached through conventional surgical techniques because there is too great a possibility that functionally important brain tissue will be damaged or destroyed.
  • In endovascular embolization the surgeon guides a catheter though the arterial network until the tip reaches the site of the AVM.  The surgeon then injects a substance (such as fast-drying glue-like substances, fibered titanium coils, and tiny balloons) that will travel through blood vessels and create an artificial blood clot in the center of an AVM.  Since embolization usually does not permanently obliterate the AVM, it is usually used as an adjunct to surgery or to radiosurgery to reduce the blood flow through the AVM and make the surgery safer.
  • Radiosurgery is an even less invasive therapeutic approach often used to treat small AVMs that haven’t ruptured.  A beam of highly focused radiation is aimed directly on the AVM and damages the walls of the blood vessels making up the lesion.  Over the course of the next several months, the irradiated vessels gradually degenerate and eventually close, leading to the resolution of the AVM.

NIH: National Institute of Neurological Disorders and Stroke

Aneurysms

A cerebral aneurysm is a weak or thin spot on a blood vessel in the brain that balloons out and fills with blood.  An aneurysm can press on a nerve or surrounding tissue, and also leak or burst, which lets blood spill into surrounding tissues (called a hemorrhage). Cerebral aneurysms can occur at any age, although they are more common in adults than in children and are slightly more common in women than in men.

The prognosis for a individual with a ruptured cerebral aneurysm depends on the location of the aneurysm, extent of bleeding or rebleeding, the person's age, general health, pre-existing neurological conditions and time between rupture and medical attention. Early diagnosis and treatment are important. A burst cerebral aneurysm may be fatal or could lead to hemorrhagic stroke, vasospasm (in which other blood vessels in the brain contract and limit blood flow), hydrocephalus, coma, or short-term and/or permanent brain damage. Recovery from treatment or rupture may take weeks to months.

Symptoms

The signs and symptoms of an unruptured cerebral aneurysm will partly depend on its size and rate of growth. For example, a small, unchanging aneurysm will generally produce no symptoms, whereas a larger aneurysm that is steadily growing may produce symptoms such as headache, numbness, loss of feeling in the face or problems with the eyes. Immediately after an aneurysm ruptures, an individual may experience such symptoms as a sudden and unusually severe headache, nausea, vision impairment, vomiting, and loss of consciousness.

Treatment

For unruptured aneurysms, treatment may be recommended for large or irregularly shaped aneurysms or for those causing symptoms. Emergency treatment for individuals with a ruptured cerebral aneurysm may be required to restore deteriorating respiration and reduce abnormally high pressure within the brain.  Treatment is necessary to prevent the aneurysm from rupturing again.  Surgical treatment prevents repeat aneurysm rupture by placing a metal clip at the base of the aneurysm.  Individuals deemed too risky for surgery may be treated by inserting the tip of a catheter into an artery in the groin and advancing it through the blood stream to the site of the aneurysm, where it is used to insert metal coils that induce clot formation within the aneurysm. 

NIH: National Institute of Neurological Disorders and Stroke

Brain and spinal cord tumors

Tumors of the brain and spinal cord are abnormal growths of tissue found inside the skull or the bony spinal column.  The brain and spinal cord are the primary components of the central nervous system (CNS). Benign tumors are noncancerous, and malignant tumors are cancerous. The CNS is housed within rigid, bony quarters (i.e., the skull and spinal column), so any abnormal growth, whether benign or malignant, can place pressure on sensitive tissues and impair function. Tumors that originate in the brain or spinal cord are called primary tumors. Most primary tumors are caused by out-of-control growth among cells that surround and support neuron, specific genetic disease (such as neurofibromatosis type 1 and tuberous sclerosis), or from exposure to radiation or cancer-causing chemicals. Metastatic, or secondary, tumors in the CNS are caused by cancer cells that break away from a primary tumor located in another region of the body. Tumors can place pressure on sensitive tissues and impair function.

Symptoms

Symptoms of brain tumors include headaches, seizures, nausea and vomiting, poor vision or hearing, changes in behavior, unclear thinking, and unsteadiness.  Spinal cord tumor symptoms include pain, numbness, and paralysis. Diagnosis is made after a neurological examination, special imaging techniques (computed tomography, and magnetic resonance imaging, positron emission tomography), laboratory tests, and a biopsy (in which a sample of tissue is taken from a suspected tumor and examined).

Symptoms of brain and spinal cord tumors generally develop slowly and worsen over time unless they are treated. The tumor may be classified as benign or malignant and given a numbered score that reflects its rate of malignancy. This score can help doctors determine how to treat the tumor and predict the likely outcome, or prognosis, for the individual.

Treatment

The three most commonly used treatments are surgery, radiation, and chemotherapy. Doctors also may prescribe steroids to reduce the tumor-related swelling inside the CNS.

NIH: National Institute of Neurological Disorders and Stroke

Cerebral cavernous malformation

Cerebral cavernous malformations (CCMs) are vascular lesions comprised of clusters of tightly packed, abnormally thin-walled small blood vessels (capillaries) that displace normal neurological tissue in the brain or spinal cord. The vessels are filled with slow-moving or stagnant blood that is usually clotted or in a state of decomposition. Cavernous malformations can occur in the brain, spinal cord, and some other body regions. In the brain and spinal cord these cavernous lesions are quite fragile and are prone to bleeding, causing hemorrhagic strokes (bleeding into the brain), seizures, and neurological deficits. CCMs can range in size from a few fractions of an inch to several inches in diameter, depending on the number of blood vessels involved. Some people develop multiple lesions while others never experience related medical problems. Hereditary forms of CCM are caused by mutations in one of three CCM disease genes: CCM1, CCM2, and CCM3. A large population with hereditary CCM disease is found in New Mexico and the Southwestern United States, in which the disease is caused by mutations in the gene CCM1 (or KRIT1).

Rebleeding from a cavernous angioma is common, it is not predictable, and individuals frequently have multiple CCMs found via magnetic resonance imaging. Individuals with CCM are faced with a diagnosis that imparts risk of multiple future hemorrhages that occur seemingly at random and without any preventative therapy except surgical removal.

Treatment

The primary treatment option for a CCM is surgical removal. Radiation therapy has not been shown to be effective. The decision to operate is made based upon the risk of approaching the lesion. For example, symptomatic lesions close to the brain surface in “non eloquent” brain (areas for example, those areas not involved with motor function, speech, vision, hearing, memory, and learning) are very likely to be candidates for removal. On the other hand, lesions located in deep brain areas are associated with higher surgical risk and are often not candidates for surgery until the lesion has bled multiple times. Medications can often lessen general symptoms such as headache, back pain, and seizures.

NIH: National Institute of Neurological Disorders and Stroke

Cerebral palsy/spasticity/dystonia

The term cerebral palsy refers to a group of neurological disorders that appear in infancy or early childhood and permanently affect body movement, muscle coordination, and balance. CP affects the part of the brain that controls muscle movements.  The majority of children with cerebral palsy are born with it, although it may not be detected until months or years later. The early signs of cerebral palsy usually appear before a child reaches three years of age. The most common are a lack of muscle coordination when performing voluntary movements (ataxia); stiff or tight muscles and exaggerated reflexes (spasticity); walking with one foot or leg dragging; walking on the toes, a crouched gait, or a “scissored” gait; and muscle tone that is either too stiff or too floppy. Other neurological symptoms that commonly occur in individuals with CP include seizures, hearing loss and impaired vision, bladder and bowel control issues, and pain and abnormal sensations. A small number of children have CP as the result of brain damage in the first few months or years of life, brain infections such as bacterial meningitis or viral encephalitis, or head injury from a motor vehicle accident, a fall, or child abuse. The disorder isn't progressive, meaning that the brain damage typically doesn't get worse over time. Risk factors associated with CP do not cause the disorder but can increase a child's chance of being born with the disorder.CP is not hereditary.

Cerebral palsy doesn’t always cause profound disabilities and for most people with CP the disorder does not affect life expectancy. Many children with CP have average to above average intelligence and attend the same schools as other children their age. Supportive treatments, medications, and surgery can help many individuals improve their motor skills and ability to communicate with the world. While one child with CP might not require special assistance, a child with severe CP might be unable to walk and need extensive, lifelong care.

Treatment

Cerebral palsy can’t be cured, but treatment will often improve a child's capabilities. In general, the earlier treatment begins the better chance children have of overcoming developmental disabilities or learning new ways to accomplish the tasks that challenge them. Early intervention, supportive treatments, medications, and surgery can help many individuals improve their muscle control. Treatment may include physical and occupational therapy, speech therapy, drugs to control seizures, relax muscle spasms, and alleviate pain; surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; wheelchairs and rolling walkers; and communication aids such as computers with attached voice synthesizers. 

What is dystonia?

Dystonia is a disorder characterized by involuntary muscle contractions that cause slow repetitive movements or abnormal postures. The movements may be painful, and some individuals with dystonia may have a tremor or other neurologic features. There are several different forms of dystonia that may affect only one muscle, groups of muscles, or muscles throughout the body. Some forms of dystonia are genetic but the cause for the majority of cases is not known.

Symptoms of dystonia

Dystonia can affect many different parts of the body, and the symptoms are different depending upon the form of dystonia. Early symptoms may include a foot cramp or a tendency for one foot to turn or drag—either sporadically or after running or walking some distance—or a worsening in handwriting after writing several lines.  In other instances, the neck may turn or pull involuntarily, especially when the person is tired or under stress. Sometimes both eyes might blink rapidly and uncontrollably; other times, spasms will cause the eyes to close.  Symptoms may also include tremor or difficulties speaking.  In some cases, dystonia can affect only one specific action, while allowing others to occur unimpeded.  For example, a musician may have dystonia when using her hand to play an instrument, but not when using the same hand to type.  The initial symptoms can be very mild and may be noticeable only after prolonged exertion, stress, or fatigue.  Over a period of time, the symptoms may become more noticeable or widespread; sometimes, however, there is little or no progression. Dystonia typically is not associated with problems thinking or understanding, but depression and anxiety may be present.

Dystonia can occur at any age, but is often described as either early, or childhood, onset versus adult onset. 

Early-onset dystonia often begins with symptoms in the limbs and may progress to involve other regions. Some symptoms tend to occur after periods of exertion and/or fluctuate over the course of the day. 

Dystonias often progress through various stages. Initially, dystonic movements may be intermittent and appear only during voluntary movements or stress. Later, individuals may show dystonic postures and movements while walking and ultimately even while they are relaxed. Dystonia can be associated with fixed postures and shortening of tendons.

Treatment

Currently, there are no medications to prevent dystonia or slow its progression.  There are, however, several treatment options that can ease some of the symptoms of dystonia, so physicians can select a therapeutic approach based on each individual’s symptoms. 

  • Botulinum toxin – Botulinum injections often are the most effective treatment for the focal dystonias.  Injections of small amounts of this chemical into affected muscles prevents muscle contractions and can provide temporary improvement in the abnormal postures and movements that characterize dystonia.
  • Medications – Several classes of drugs that affect different neurotransmitters may be effective for various forms of dystonia. These medications are used “off-label”, meaning they are approved by the U.S. Food and Drug Administration to treat different disorders or conditions but have not been specifically approved to treat dystonia.  The response to drugs varies among individuals and even in the same person over time.  
  • Deep brain stimulation (DBS) – may be recommended for some individuals with dystonia, especially when medications do not sufficiently alleviate symptoms or the side effects are too severe. DBS involves surgically implanting small electrodes that are connected to a pulse generator into specific brain regions that control movement.  Controlled amounts of electricity are sent into the exact region of the brain that generates the dystonic symptoms and interfere with and block the electrical signals that cause the symptoms.
  • Other surgeries – aim to interrupt the pathways responsible for the abnormal movements at various levels of the nervous system. Some operations purposely damage small regions of the thalamus (thalamotomy), globus pallidus (pallidotomy), or other deep centers in the brain. Other surgeries include cutting nerves leading to the nerve roots deep in the neck close to the spinal cord (anterior cervical rhizotomy) or removing the nerves at the point they enter the contracting muscles (selective peripheral denervation).  Some patients report significant symptom reduction after surgery.

NIH: National Institute of Neurological Disorders and Stroke

Chiari malformation

Chiari malformations (CMs) are structural defects in the base of the skull and the cerebellum, the part of the brain that controls balance. When part of the cerebellum extends through the opening at the base of the skull, the cerebellum and brain stem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (CSF, the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, headache, and problems with hearing, balance, and coordination. Symptoms may change for some individuals depending on buildup of CSF and any resulting pressure on tissue and nerves. CMs are classified by the severity of the disorder and the parts of the brain that protrude into the spinal canal. The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition. Type II (also called classic CM and Arnold-Chiari malformation) is usually accompanied by a myelomeningocele--a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening. Symptoms of Type III--the most serious form of CM--include those seen in Type II, in addition to additional severe neurological defects. In CM Type IV, parts of the cerebellum are missing, and portions of the spinal cord may be visible. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia (a fluid-filled cyst in the spinal cord), and spinal curvature.

Symptoms

Headache is the hallmark sign of Chiari malformation, especially after sudden coughing, sneezing, or straining.  Other symptoms may vary among individuals and may include:

  • Neck pain
  • Hearing or balance problems
  • Muscle weakness or numbness
  • Dizziness
  • Difficulty swallowing or speaking
  • Vomiting
  • Ringing or buzzing in the ears (tinnitus)
  • Curvature of the spine (scoliosis)
  • Insomnia
  • Depression
  • Problems with hand coordination and fine motor skills. 

Some individuals with CM may not show any symptoms.  Symptoms may change for some individuals, depending on the compression of the tissue and nerves and on the buildup of CSF pressure. 

Infants with a Chiari malformation may have difficulty swallowing, irritability when being fed, excessive drooling, a weak cry, gagging or vomiting, arm weakness, a stiff neck, breathing problems, developmental delays, and an inability to gain weight.

Treatment

Medications may ease certain symptoms, such as pain, when present. In many cases, surgery is the only treatment available to improve or stabilize symptoms or halt the progression of damage to the central nervous system. More than one surgery may be needed to treat the condition. Surgery may include a procedure to create more space for the cerebellum or removing part of the cerebellar tonsils that may reach through the skull opening (the cerebellar tonsils do not have a recognized function and can be removed without causing any known neurological problems).

NIH: National Institute of Neurological Disorders and Stroke

Concussions

Traumatic brain injury (TBI) is a sudden injury from an external force that affects the functioning of the brain. It can be caused by a bump or blow to the head (closed head injury) or by an object penetrating the skull (called a penetrating injury).

The most common form of TBI is concussion. A concussion can happen when the head or body is moved back and forth quickly, such as during a motor vehicle accident or sports injury.

Concussions are often called "mild TBI" because they are usually not life threatening. However, they still can cause serious problems, and research suggests that repeated concussions can be particularly dangerous.

A person who has a TBI may have some of the same symptoms as a person who has a non-traumatic brain injury. Unlike TBI, this type of injury is not caused by an external force, but is caused by an internal problem, such as a stroke or infection.

Both types of injury can have serious, long-term effects on a person's cognition and functioning.

Symptoms

TBI symptoms vary depending on the extent of the injury and the area of the brain affected. Some symptoms appear immediately; others may appear several days or even weeks later. A person with TBI may or may not lose consciousness—loss of consciousness is not always a sign of severe TBI.

Symptoms of Mild TBI

A person with a mild TBI may experience:

  • Headache
  • Confusion
  • Lightheadedness
  • Dizziness
  • Blurred vision
  • Ringing in the ears
  • Tiredness or sleepiness
  • A bad taste in the mouth
  • A change in sleep habits
  • Behavior or mood changes
  • Trouble with memory, concentration, attention, or thinking
  • Loss of consciousness lasting a few seconds to minutes1
  • Sensitivity to light or sound
  • Nausea or vomiting

Symptoms of Moderate or Severe TBI

A person with moderate or severe TBI may have some of the symptoms listed above. In addition, the person may experience any of the following:

  • Headache that gets worse or won’t go away
  • Repeated vomiting or nausea
  • Slurred speech
  • Convulsions or seizures
  • An inability to wake up from sleep
  • Enlargement of the pupil (dark center) of one or both eyes
  • Numbness or tingling of arms or legs
  • Loss of coordination
  • Increased confusion, restlessness, or agitation
  • Loss of consciousness lasting a few minutes to hours

A person who suffers a blow to the head or another trauma that may have caused a TBI should seek medical attention.

Treatment for severe TBI

In most cases, emergency care focuses on stabilizing the patient and promoting survival. This care may include ensuring adequate oxygen flow to the brain, controlling blood pressure, and preventing further injury to the head or neck.3 Once the patient is stable, other types of care for TBI and its effects can begin.

Surgery may be needed as part of emergency care to reduce additional damage to the brain tissues. Surgery may include:

  • Removing clotted blood. Bleeding in the brain or between the brain and skull can lead to large areas of clotted blood, sometimes called hematomas (pronounced hee-ma-TOH-muhz), that put pressure on the brain and damage brain tissues.
  • Repairing skull fractures. Setting severe skull fractures or removing pieces of skull or other debris from the brain can help start the healing process of the skull and surrounding tissues.
  • Relieving pressure in the skull. Making a hole in the skull or adding a shunt or drain can relieve pressure inside the skull and allow excess fluid to drain.

NIH: National Institute of Neurological Disorders and Stroke

Craniosynostosis/plagiocephaly

Craniosynostosis is a birth defect of the skull characterized by the premature closure of one or more of the fibrous joints between the bones of the skull (called the cranial sutures) before brain growth is complete. Closure of a single suture is most common. Normally the skull expands uniformly to accommodate the growth of the brain; premature closure of a single suture restricts the growth in that part of the skull and promotes growth in other parts of the skull where sutures remain open. This results in a misshapen skull but does not prevent the brain from expanding to a normal volume. However, when many sutures close prematurely, the skull cannot expand to accommodate the growing brain, which leads to increased pressure within the skull and impaired development of the brain. Craniosynostosis can be gene-linked or caused by metabolic diseases (such as rickets )or an overactive thyroid. Some cases are associated with other disorders such as microcephaly (abnormally small head) and hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain). The first sign of craniosynostosis is an abnormally shaped skull. Other features can include signs of increased intracranial pressure, developmental delays, or impaired cognitive development, which are caused by constriction of the growing brain. Seizures and blindness may also occur.

Treatment

Treatment for craniosynostosis generally consists of surgery to improve the symmetry and appearance of the head and to relieve pressure on the brain and the cranial nerves. For some children with less severe problems, cranial molds can reshape the skull to accommodate brain growth and improve the appearance of the head.

NIH: National Institute of Neurological Disorders and Stroke

Plagiocephaly

Deformational plagiocephaly is cranial asymmetry occurring as a result of forces that deform skull shape in the supine position. The risk of plagiocephaly may be modified by positioning the baby on alternate days with the head to the right or the left side, and by increasing time spent in the prone position during awake periods. When deformational plagiocephaly is already present, physiotherapy (including positioning equivalent to the preventive positioning, and exercises as needed for torticollis and positional preference) has been shown to be superior to counselling about preventive positioning only. Helmet therapy (moulding therapy) has been utilized to reduce skull asymmetry.

NIH: National Institute of Neurological Disorders and Stroke 

Epilepsy

The epilepsies are a spectrum of brain disorders ranging from severe, life threatening and disabling, to ones that are much more benign. In epilepsy, the normal pattern of neuronal activity becomes disturbed, causing strange sensations, emotions, and behavior or sometimes convulsions, muscle spasms, and loss of consciousness. The epilepsies have many possible causes and there are several types of seizures. Anything that disturbs the normal pattern of neuron activity—from illness to brain damage to abnormal brain development—can lead to seizures. Epilepsy may develop because of an abnormality in brain wiring, an imbalance of nerve signaling chemicals called neurotransmitters, changes in important features of brain cells called channels, or some combination of these and other factors. Having a single seizure as the result of a high fever (called febrile seizure) or head injury does not necessarily mean that a person has epilepsy. Only when a person has had two or more seizures is he or she considered to have epilepsy. A measurement of electrical activity in the brain and brain scans such as magnetic resonance imaging or computed tomography are common diagnostic tests for epilepsy.

While epilepsy cannot be cured, for some people the seizures can be controlled with medication, diet, devices, and/or surgery. Most seizures do not cause brain damage, but ongoing uncontrolled seizures may cause brain damage. It is not uncommon for children with epilepsy to develop behavioral and emotional problems in conjunction with seizures. Issues may also arise as a result of the stigma attached to having epilepsy, which can lead to embarrassment and frustration or bullying, teasing, or avoidance in school and other social settings.

Epilepsy can be a life-threatening condition. Some people with epilepsy are at special risk for abnormally prolonged seizures or sudden unexplained death in epilepsy.

Treatment

Once epilepsy is diagnosed, it is important to begin treatment as soon as possible. For about 70 percent of those diagnosed with epilepsy, seizures can be controlled with modern medicines and surgical techniques. Some drugs are more effective for specific types of seizures. An individual with seizures, particularly those that are not easily controlled, may want to see a neurologist specifically trained to treat epilepsy. In some children, special diets may help to control seizures when medications are either not effective or cause serious side effects.

Deep brain stimulation (DBS) therapy aims to reduce seizures that are not controlled with medication, and where surgery to treat the cause of seizures is not possible.

NIH: National Institute of Neurological Disorders and Stroke

Hydrocephalus

The primary characteristic of hydrocephalus is excessive accumulation of cerebrospinal fluid (CSF), the clear fluid that surrounds the brain and spinal cord. This excessive accumulation results in an abnormal dilation of the spaces in the brain called ventricles. This dilation causes potentially harmful pressure on the tissues of the brain. Hydrocephalus may be congenital or acquired. Congenital hydrocephalus is present at birth and may be caused by genetic abnormalities or developmental disorders such as spina bifida and encephalocele.

Symptoms of hydrocephalus vary with age, disease progression, and individual differences in tolerance to CSF. In infancy, the most obvious indication of hydrocephalus is often the rapid increase in head circumference or an unusually large head size. In older children and adults, symptoms may include headache followed by vomiting, nausea, papilledema (swelling of the optic disk, which is part of the optic nerve), downward deviation of the eyes (called "sunsetting"), problems with balance, poor coordination, gait disturbance, urinary incontinence, slowing or loss of development (in children), lethargy, drowsiness, irritability, or other changes in personality or cognition, including memory loss.

The symptoms of normal pressure hydrocephalus usually get worse over time if the condition is not treated, although some people may experience temporary improvements. If left untreated, progressive hydrocephalus is fatal, with rare exceptions. The parents of children with hydrocephalus should be aware that hydrocephalus poses risks to both cognitive and physical development. Treatment by an interdisciplinary team of medical professionals, rehabilitation specialists, and educational experts is critical to a positive outcome. Many children diagnosed with the disorder benefit from rehabilitation therapies and educational interventions, and go on to lead normal lives with few limitations.

Treatment

Hydrocephalus is most often treated with the surgical placement of a shunt system. This system diverts the flow of CSF from a site within the central nervous system to another area of the body where it can be absorbed as part of the circulatory process. A limited number of individuals can be treated with an alternative procedure called third ventriculostomy. In this procedure, a small hole is made in the floor of the third ventricle, allowing the CSF to bypass the obstruction and flow toward the site of resorption around the surface of the brain.

NIH: National Institute of Neurological Disorders and Stroke

Moyamoya disease

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage.  Moyamoya disease was first described in Japan in the 1960s and it has since been found in individuals in the other countries around the world; its incidence is higher in Asian countries than in Europe or North America. The disease primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures.

Without surgery, the majority of individuals with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries. Without treatment, Moyamoya disease can be fatal as the result of intracerebral hemorrhage (bleeding within the brain).

Symptoms

Individuals with this disorder may have disturbed consciousness, problems with speaking and understanding speech, sensory and cognitive impairments, involuntary movements, and vision problems. About one in 10 individuals with Moyamoya disease has a close relative who is also affected; in these cases researchers think that Moyamoya disease is the result of inherited genetic abnormalities. 

Treatment

There are several types of surgery that can restore blood flow (revascularization) to the brain by opening narrowed blood vessels or by bypassing blocked arteries.  Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery.

NIH: National Institute of Neurological Disorders and Stroke

Pseudotumor cerebri

Pseudotumor cerebri literally means "false brain tumor." It is likely due to high pressure within the skull caused by the buildup or poor absorption of cerebrospinal fluid (CSF). The disorder is most common in women between the ages of 20 and 50. Symptoms of pseudotumor cerebri, which include headache, nausea, vomiting, and pulsating sounds within the head, closely mimic symptoms of large brain tumors.

The disorder may cause progressive, permanent visual loss in some patients. In some cases, pseudotumor cerebri recurs.

Treatment

If a diagnosis of pseudotumor cerebri is confirmed, close, repeated ophthalmologic exams are required to monitor any changes in vision. Drugs may be used to reduce fluid buildup and to relieve pressure. Weight loss through dieting or weight loss surgery and cessation of certain drugs (including oral contraceptives, tetracycline, and a variety of steroids) may lead to improvement. Surgery may be needed to remove pressure on the optic nerve. Therapeutic shunting, which involves surgically inserting a tube to drain CSF from the lower spine into the abdominal cavity, may be needed to remove excess CSF and relieve CSF pressure.

NIH: National Institute of Neurological Disorders and Stroke

Spina bifida

Spina bifida (SB) is a neural tube defect (a disorder involving incomplete development of the brain, spinal cord, and/or their protective coverings) caused by the failure of the fetus's spine to close properly during the first month of pregnancy. Infants born with SB sometimes have an open lesion on their spine where significant damage to the nerves and spinal cord has occurred. Although the spinal opening can be surgically repaired shortly after birth, the nerve damage is permanent, resulting in varying degrees of paralysis of the lower limbs. Even when there is no lesion present there may be improperly formed or missing vertebrae and accompanying nerve damage. In addition to physical and mobility difficulties, most individuals have some form of learning disability.

Types of SB

  • Myelomeningocele, the severest form, in which the spinal cord and its protective covering (the meninges) protrude from an opening in the spine.
  • Meningocele in which the spinal cord develops normally but the meninges and spinal fluid) protrude from a spinal opening.
  • Closed neural tube defects, which consist of a group of defects in which development of the spinal cord is affected by malformations of the fat, bone, or meninges.
  • Occulta, the mildest form, in which one or more vertebrae are malformed and covered by a layer of skin. SB may also cause bowel and bladder complications, and many children with SB have hydrocephalus (excessive accumulation of cerebrospinal fluid in the brain).

Symptoms

The symptoms of spina bifida vary from person to person, depending on the type and level of involvement.   Closed neural tube defects are often recognized early in life due to an abnormal tuft or clump of hair or a small dimple or birthmark on the skin at the site of the spinal malformation.

Meningocele and myelomeningocele generally involve a fluid-filled sac – visible on the back – protruding from the spinal canal.  In meningocele, the sac may be covered by a thin layer of skin. In most cases of myelomeningocele, there is no layer of skin covering the sac and an area of abnormally developed spinal cord tissue is usually exposed.

Treatment

There is no cure for SB because the nerve tissue cannot be replaced or repaired. Treatment for the variety of effects of SB may include surgery, medication, and physiotherapy. Many individuals with SB will need assistive devices such as braces, crutches, or wheelchairs. Ongoing therapy, medical care, and/or surgical treatments may be necessary to prevent and manage complications throughout the individual's life. Surgery to close the newborn's spinal opening is generally performed within 24 hours after birth to minimize the risk of infection and to preserve existing function in the spinal cord.

NIH: National Institute of Neurological Disorders and Stroke

Syrinx

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a fluid-filled cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, damaging the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage may cause pain, weakness, and stiffness in the back, shoulders, arms, or legs. Symptoms vary among individuals. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari malformation, which causes brain tissue to protrude from its normal location in the back of the head and into the cervical or neck portion of the spinal canal. Syringomyelia may also occur as a complication of trauma, inflammation, spinal cord injury, hemorrhage, spinal cord tumors, or other conditions. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare.

Symptoms

Symptoms usually begin in young adulthood, with symptoms of one form usually beginning between the ages of 25 and 40. If not treated surgically (when needed), syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. Symptoms may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate. Some individuals may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most individuals. Delay in treatment may result in irreversible spinal cord injury.

Treatment

The type of treatment for syringomyelia depends on the severity and progression of an individual’s symptoms. Surgery is usually recommended for individuals with symptomatic or progressive syringomyelia.  There are two general forms of treatment: restoration of normal CSF flow around the spinal cord, and direct drainage of the syrinx. The type of treatment depends on what is causing the symptoms. 

  • Treating the Chiari malformation. The main goal of Chiari surgery is to provide more space at the base of the skull and upper neck.  This reduces pressure on the brain and spinal cord and restores the normal flow of CSF.  Surgery can allow the syrinx to drain, sometimes becoming smaller or even disappearing entirely.  Symptoms may improve even if the syrinx remains the same size or is reduced only slightly.  Individuals should get treated sooner rather than later since delaying treatment can cause irreversible spinal cord damage.  Syringomyelia can reoccur after surgery, making additional operations necessary.
  • Preventing a syrinx from forming or expanding after an injury. In the case of trauma-related syringomyelia, the primary strategy is to prevent a syrinx from developing or growing in the first place.  This somewhat controversial procedure (called expansive duraplasty) is done in a surgical operation on the spine that involves removing scar tissue around the spinal cord and sometimes adding a patch to expand the “dura,” the membrane that surrounds the spinal cord. By clearing and expanding the space around the spinal cord, the normal flow of CSF may be restored. 
  • Removing the obstruction. Surgical removal of obstructions such as scar tissue, bone from the spinal canal, or tumors can help restore the normal flow of CSF. If a tumor is causing syringomyelia, removing the tumor almost always eliminates the syrinx.  Occasionally, radiation may also be used to shrink the tumor. 
  • Draining the syrinx. Especially if there is no associated Chiari malformation or tumor, it may be necessary to drain the syrinx.  This is usually done in cases where the syrinx is growing or the cause of the CSF obstruction cannot be identified.  To perform this procedure a surgeon inserts a drain called a stent or shunt.  A stent consists of a small tube that is inserted into the syrinx fluid and allows fluid from within the cord to drain into the space just outside the spinal cord.  Similarly, a shunt consists of a flexible tube and a valve that drains the syrinx fluid into another part of the body, usually the abdomen.  By draining the syrinx, both of these procedures can halt the progression of symptoms and relieve headache.  However, these procedures carry the risk of spinal cord injury, infection, or bleeding, and may not help all individuals.

NIH: National Institute of Neurological Disorders and Stroke

Tethered cord

Tethered spinal cord syndrome is a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.  Attachments may occur congenitally at the base of the spinal cord (conus medullaris) or they may develop near the site of an injury to the spinal cord.  These attachments cause an abnormal stretching of the spinal cord.  The course of the disorder is progressive.

With treatment, individuals with tethered spinal cord syndrome have a normal life expectancy.  However, some neurological and motor impairments may not be fully correctable.  Surgery soon after symptoms emerge appears to improve chances for recovery and can prevent further functional decline.

Symptoms

In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence.  This type of tethered spinal cord syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida.

Tethered spinal cord syndrome may go undiagnosed until adulthood, when pain, sensory and motor problems, and loss of bowel and bladder control emerge.  This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time and may be exacerbated during sports or pregnancy, or may be due to narrowing of the spinal column (stenosis) with age. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord.  Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia.  This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.

Treatment

MRI imaging is often used to evaluate individuals with these symptoms, and can be used to diagnose the location of the tethering, lower than normal position of the conus medullaris, or presence of a tumor or fatty mass (lipoma).  In children, early surgery is recommended to prevent further neurological deterioration. Regular follow-up is important: retethering may occur in some individuals during periods of rapid growth and may be seen between five to nine years of age.  If surgery is not advisable, spinal cord nerve roots may be cut to relieve pain.  In adults, surgery to free (detether) the spinal cord can reduce the size and further development of cysts in the cord and may restore some function or alleviate other symptoms.  Other treatment is symptomatic and supportive.

NIH: National Institute of Neurological Disorders and Stroke

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