Research & News

Shunt Function

by Stephanie LaBandz, PT

Many children with mobility impairments due to spina bifida, intraventricular hemorrhage related to premature birth, cerebral palsy, and some genetic disorders may also have a medical history that includes hydrocephalus. Other children we see in our clinics and schools may also have hydrocephalus without other significant health issues or mobility problems.

According to UCLA Neurosurgery, hydrocephalus occurs in an average of 3-4 out of 1,000 babies born. Hydrocephalus is the result of an imbalance in the flow of cerebrospinal fluid (CSF) through the central nervous system. CSF provides protection, metabolic waste removal, and delivery of the body’s chemical messages via hormones throughout the brain and spinal cord. In a normally functioning system, CSF is produced by the brain, then gradually absorbed into the bloodstream, maintaining a constant level of pressure. In the case of hydrocephalus, excess fluid and excess pressure build up. This is usually as the result of overproduction of CSF or obstruction of the flow of CSF within the central nervous system. This places pressure on the brain and can cause damage to neural tissue.

Treatment of hydrocephalus consists of surgical placement of a ventriculoperitoneal (VP) shunt. This long, thin tube allows drainage of excess CSF from the ventricular cavities inside the brain to the abdominal cavity, where it can then be absorbed into the circulatory system.

The Hydrocephalus Association emphasizes that children with VP shunts should be treated like other children and be allowed to participate in physical activities including physical education along with their peers. The Spina Bifida Association also supports non-contact sports and regular activities including usual amounts of rolling and tumbling. That being said, it is recommend that the child and family consult with their neurosurgeon to establish guidelines on a case-by case basis. Any activity limitations should be communicated to therapists, teachers, and physical education teachers and be clearly delineated in a child’s Individualized Education Plan or Section 504 plan if applicable.

Some VP shunts are programmable to control the flow of CSF using a magnetic device. One precaution that several neurosurgeons recommend is keeping children with VP shunts away from strong magnetic fields to avoid accidental adjustment of the intended setting. Drawing with a Magna Doodle type toy or using alphabet refrigerator magnets is usually fine, but larger, stronger magnets found in some toys, electronics, and those frequently used for teaching science lessons on magnetism may pose a risk. Once again, any neurologist recommendations should be communicated to the family, health care team, and educational team and documented.

Those of us who work closely with a child and pay special attention to his or her cognitive or motor abilities may be able to help recognize early symptoms in the event of a shunt malfunction or infection.

Common symptoms include:
• Vomiting
• Headache
• Vision problems
• Irritability and/or tiredness
• Personality change
• Loss of coordination or balance
• Swelling along the shunt tract
• Difficulty in waking up or staying awake
• Decline in academic performance
• Fever
• Redness along the shunt tract

By noting these symptoms early, children can be referred to their health care providers for intervention. This may help to avoid further complications and allow them to return to learning and playing and just being kids.

References:
“A Teacher’s Guide to Hydrocephalus”. Hydrocephalus Association.
“Hydrocephalus” Spina Bifida Association.
“The Ventricular System and CSF.” University of Washington 
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Causes of Hydrocephalus After Neonatal and Adult Intraventricular Hemorrhage

Jennifer Strahle, Hugh J. L. Garton, Cormac O. Maher, Karin M. Muraszko, Richard F. Keep and Guohua Xi

From the issue entitled "Special Issue: Intracerebral Hemorrhage: Mechanisms and Therapies"

Abstract

Intraventricular hemorrhage (IVH) is a cause of significant morbidity and mortality and is an independent predictor of a worse outcome in intracerebral hemorrhage (ICH) and germinal matrix hemorrhage (GMH). IVH may result in both injuries to the brain as well as hydrocephalus. This paper reviews evidence on the mechanisms and potential treatments for IVH-induced hydrocephalus. One frequently cited theory to explain hydrocephalus after IVH involves obliteration of the arachnoid villi by microthrombi with subsequent inflammation and fibrosis causing CSF outflow obstruction. Although there is some evidence to support this theory, there may be other mechanisms involved, which contribute to the development of hydrocephalus. It is also unclear whether the causes of acute and chronic hydrocephalus after hemorrhage occur via different mechanisms: mechanical obstruction by blood in the former and inflammation and fibrosis in the latter. Management of IVH and strategies for prevention of brain injury and hydrocephalus are areas requiring further study. A better understanding of the pathogenesis of hydrocephalus after IVH may lead to improved strategies to prevent and treat post-hemorrhagic hydrocephalus.

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Risk Factors Associated with Perinatal Stroke, Neonatal Stroke or Stroke in Infants

Perinatal Stroke involves an often poorly understood neurological events affecting the fetus and the newborn with a potential for serious neurological outcome. 

Perinatal arterial ischemic stroke is the most common known cause of cerebral palsy in term and near-term infants.  Perinatal stroke is seventeen times more common than pediatric stroke beyond the newborn period.  Arterial ischemia occurring during the 3 days surround birth is reported to be responsible for 50% to 70% of congenital hemiplegic cerebral palsy.

What causes a Perinatal Stroke (between 28 weeks gestation to 28 days after birth)?

When we discuss causes of perinatal or infant stroke, we're actually discussing risk factors for perinatal or infant stroke.  A risk factor is something that may increase the likelihood of a disease, condition, or injury.   Listed below are risk factors that may be associated with perinatal or infant stroke.  Even though a risk factor may be identified, this does not necessarily mean that the risk factor is what caused the stroke.

Cardiac disorders including congenital heart disease, patent ductus arteriosus and pulmonary valve atresia.

Blood Disorders due to deficiencies or mutations in coagulation factors and hyperviscocity syndromes which make an infant's blood more likely to clot or hemorrhage.  Blood, homocysteine, and lipid disorders include polycythaemia, disseminated intravascular coagulopathy, Factor-V Leiden mutation, Protein-S deficiency, Protein-C deficiency, Prothrombin mutation, Homocysteine, Lipoprotein (a), and Factor VIII.

Infection leads to a hypercoagulable state and has been found to be a risk factor for cerebrovascular disease.  CNS infection and systemic infection are perinatal risk factors.

Maternal disorders including autoimmune disorders, coagulation disorders, anticardiolipin antibodies, twin to twin transfusion syndrome, in utero cocaine exposure and infection.

Placental disorders including placental thrombosis, placental abruption, placental infection, and fetomaternal hemorrhage.

Vasculopathy including vascular maldevelopment

Trauma and catheterization procedures

Birth asphyxia

Dehydration

Extracorporeal membrane oxygenation

No cause can be detected

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The severity of strokes

Published May 14, 2012

NewsCore

Brain cells start to die when the blood flow to one's brain is disrupted, depriving the brain vital nutrients and oxygen. This interruption may be caused by a clot (ischemic stroke) or a blood vessel that has burst (hemorrhagic stroke).

In the United States, stroke is the leading cause of serious long-term disability, and the fourth most common cause of death, reports the American Heart Association.

Types

Ninety percent of strokes are ischemic. When the arteries leading to the brain are blocked or narrowed, blood flow is halted or reduced, causing an immediate and dire loss of brain cells. Thrombotic and embolic stroke are two common types of ischemic strokes.

Thrombotic strokes occur when a blood clot (typically from fatty deposits) develops in one of the two carotid arteries, which bring blood to your brain. An embolic stroke happens when a blood clot develops away from your brain and travels through the bloodstream, eventually getting stuck in the brain arteries. The clot typically develops in the heart.

A hemorrhage stroke occurs when a blood vessel in the brain leaks or bursts. High blood pressure or weak blood vessel walls (intracerebral hemorrhage) or aneurysms (subarachnoid hemorrhage) can spur this kind of stroke.

Symptoms

The Stroke Awareness Foundation reports that stroke symptoms include confusion, difficulty understanding speech, vision troubles, loss of coordination or balance and severe headaches.

J Mocco, associate professor of neurological surgery at Vanderbilt University, said that "sudden onset of weakness of half the body or face (inability to move one's face, arm or leg)" is another common warning sign.

Shalini Bansil, stroke neurologist at Overlook Medical Center, said that every moment a stroke goes untreated can have devastating neurological effects, potentially leading to disability or fatality.

Although most people have never heard of it, aphasia (the sudden inability to speak, read, write and/or understand language) affects 25 to 40 percent of all stroke survivors. "The lack of awareness is almost as devastating as the disorder since people do not know where to access resources and services," explains Ellayne Ganzfried, executive director of the National Aphasia Association.

Diagnosis

Doctors will screen for a stroke if a patient demonstrates symptoms or suffers a transient ischemic attack (TIA), which is also called a mini-stroke. TIAs occur when one suffers through stroke symptoms for less than five minutes due to a temporary decrease of blood to the brain.

Along with reviewing the patient's medical history and administering physical and neurological exams, the doctor can conduct brain-centric diagnostic tests: Imaging tests (MRI, CT scan), electrical tests to understand electrical impulses (EEG, Evoked Response) and blood flow tests (B-mode imaging, Doppler testing,duplex scanning).

Specific types of ultrasounds may help as well. Murray Flaster, a neurologist and stroke specialist at Loyola University Medical Center, explained, "B-mode imaging is one mode of ultrasound. Doppler testing is a second mode of ultrasound. Duplex scanning is a combination of B-mode and doppler mode."

Treatment

The Mayo Clinic reports that treatments vary depending on the type of stroke. For ischemic strokes, the goal is to return blood flow to the head. Aspirin helps thin the blood and tissue plasminogen activator, injected intravenously, can break up blood clots. Sometimes, a doctor must remove the clot manually through a catheter.

With a carotid endarterectomy, a surgeon opens the blocked artery to remove the plaque buildup.

Angioplasty entails moving a balloon-tipped catheter into the clogged artery. By inflating the balloon, the plaque is pushed against the artery walls, opening the passage for blood flow. A stent is left behind to keep the passage wide.

Hemorrhagic strokes are treated differently because the goal is to decrease bleeding rather than increase blood flow. Therefore, drugs like aspirin do not help. Doctors provide blood pressure-lowering drugs. To prevent clots, warfarin or clopidogrel may be provided. Surgical procedures for hemorrhagic strokes include aneurysm clipping, coiling and surgical AVM removal. Bed rest is prolonged medical attention are necessary for recovery.

Read more: http://www.foxnews.com/health/2012/05/14/severity-strokes/#ixzz1xGETRBZV

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Real Mom: My Baby Had a Stroke

It happened just hours after I gave birth to my little boy. Now that he's a healthy 4-year-old, I'm finally ready to tell our story.

By Lisa Applegate

Originally published in the September 2010 issue of Parents magazine

In my darkest moment -- 24 hours after my son, Luc, was born and 12 hours after he first turned blue in my arms -- I sat on a worn sofa between my husband, Mike, and the hospital chaplain. Mike was crying, the chaplain was praying, and I was feeling utterly, nauseatingly helpless.

I had never felt such shock, such numbing fear. My vision was blurred, my hearing seemed muffled, and I couldn't comprehend what was happening in the intensive care unit. Was that nurse really pumping air into Luc's lungs while his beautiful body stiffened with seizures? Did I really hear the doctor say "spinal tap," "CT scan," and "possible brain damage?" Why was my healthy son -- who was born full-term at just over 7 pounds, after an uncomplicated pregnancy -- in the same unit as fragile preemies?

After praying for an answer for three endless days and nights, we received one. Unfortunately, that answer came in the form of an MRI image. On the left half of Luc's brain scan, what looked like a large black hole seared through his otherwise healthy gray matter. Our newborn son had suffered a stroke. One-sixth of his neurons had been destroyed, and his body reacted by having seizures.

Mike and I were dumbfounded. A baby can have a stroke? One of my first thoughts was whether I caused this. But most of all I wondered what this meant for Luc.

No Easy Answers

The neurologist couldn't give us any assurances. No one could tell us what perfect storm of biological factors caused oxygen to be momentarily cut off from Luc's brain. That was just the first of many maddening unknowns.

However, this is what we do know about pediatric stroke: It occurs in the first month of life in roughly one in every 4,000 births (whether premature or full-term) and is one of the top 10 killers of children. It happens when a blood vessel that carries oxygen and nutrients to the brain either bursts or is blocked by a clot. (About 80 percent of strokes in babies are caused by a clot.) At that point, part of the brain cannot get the blood and oxygen it needs, so it starts to die.

I went numb yet again when our neurologist told us that, based on where the stroke was located, Luc could develop cerebral palsy, learning disabilities, and epilepsy. But I also remember his telling us, "A baby's brain is extremely elastic." Much of its wiring -- especially the areas involving thinking, emotions, and behavior -- is established during the first months and years after birth. This means that a newborn's brain can build new neuron connections despite stroke damage, finding alternate routes around the injury. But he needs age-appropriate rehabilitation and therapy. Doctors said that with this therapy, Luc's brain could adjust and function fine. Or not.

How do parents make future plans with a question mark as big as this? At first, we simply worried. I agonized over the past, questioning every aspect of my pregnancy even though I had no scientific proof that my actions resulted in Luc's stroke. Should I have eaten those french fries that miraculously quelled my nausea? Was it the mild depression I experienced in those early months? Did it happen because I tripped while I was jogging one day?

Mike chose to torture himself with thoughts of the future: What if Luc can't play sports with me? What if the seizures come back? What if we go broke paying for his doctor visits, leg braces, or special schools?

Quickly, though, we both grabbed onto the promise of therapy. The neurologist had mentioned that babies are born with millions of brain cells, but that thousands of those eventually die off because they're not needed. So Mike and I focused on keeping hold of every remaining cell.

We enrolled Luc in Illinois' Early Intervention program, and soon several talented therapists started coming to our house every other week for more than a year. They identified subtle weaknesses in Luc's body that I, as a new mother, might not have even noticed, and they empowered me to help him overcome them.

For example, most 3-month-olds have learned to open and close their hands. But as Luc approached 4 months, his right hand remained in a fist. His occupational therapist showed me how to rub his palm to get his hand to open, and to move toys strategically so he'd stretch his fingers for them. We placed him on an exercise ball to strengthen his core and shoulder muscles, so he would no longer lean to one side when sitting. When Luc learned to walk, his physical therapist played all kinds of jumping and kicking games to fix the slight drag in his right leg. The developmental and speech therapists kept their eyes on him, too. They visited every three months or so to see whether Luc could babble, swallow baby food, make eye contact, and show signs of separation anxiety when I left the room, among other things.

Meanwhile, I scoured the Internet for what little research there was on pediatric stroke and reached out to a statewide support group. That's when I realized we'd actually been quite lucky so far. Luc's seizures -- which, thankfully, have never returned -- were actually a blessing because they alerted us to a problem. Otherwise, we might not have realized he'd suffered a stroke until weeks or months later. The symptoms of stroke in babies are different and more subtle than in adults, and even doctors can miss the clues. One mom told us her pediatrician wasted months of precious time by dismissing her infant daughter's inability to use one hand, even though weakness on one side is a sign of stroke. As a result, the girl didn't receive therapy until she was almost 1.

Compared with those of other young stroke victims we met and read about, Luc's challenges were mild. Some children must wear braces or endure Botox injections to strengthen muscles weakened by cerebral palsy. We met preschoolers who were still learning to talk. One child had tried several medications to treat his stroke-related epilepsy, but nothing was easing his daily seizures.

We wanted to be informed about the possible outcomes of stroke, but I have to admit that hearing these stories terrified us. We skipped some support-group meetings because we couldn't handle the thought of being in the same situation as some of the other members. I stopped Googling the word "stroke," and instead read child-development books. Fortunately, as the months passed, Mike and I had more to celebrate than to fear.

Healing Together

By the time he was 2, Luc had "graduated" from therapy, and his neurologist could no longer find physical evidence of impairment from his stroke. From picking up a Cheerio with his fingers to speaking a full sentence and reading simple words, he has met every developmental milestone on time. We believe that having Luc in therapy at such an early age made a big difference. Does this mean that we no longer have to worry about the consequences of his stroke? Unfortunately, no. Luc's brain probably developed differently because it had to accommodate the injury, and those differences might not appear until he's older.

I've shed a few tears recently about research from the University of California, San Diego that found that 60 percent of infants and children who'd had strokes showed signs in their teen years of psychiatric disorders, particularly ADHD. The branches of my family tree are laden with anxiety and depression, and I worry that Luc's stroke will place him at further risk for such disorders.

I wish that I could be certain that Luc won't be part of any of those statistics. I like to think that the way his doctors, therapists, and family have nurtured him has permanently improved his odds. I want brilliant scientists to find the cause of infant stroke so that my grandchildren won't be at risk. I want to know in my heart what doctors keep telling me: Luc's stroke wasn't my fault.

I might never know any of this, and I still find such mysteries to be maddening. But then I look at Luc today. At 4, he imagines exploring the planet Jupiter and loves playing baseball with his dad. I dream about his future and all of the exciting opportunities that he might enjoy. Isn't that what all parents do, no matter the circumstance? Simply by bringing children into this world, we must accept that the unexpected will occur -- and dream anyway.

The Facts About Stroke

Who's Most Likely to Be Affected?
Stroke is a risk factor for babies with congenital heart disease, blood disorders such as sickle cell disease, and infections including meningitis and encephalitis. Premature babies, whose blood vessels are more fragile, can suffer bleeding in the brain known as hemorrhagic strokes. Stroke can also occur when mothers have a history of infertility, or preeclampsia, or antiphospholipid syndrome, an immune-system disorder that can cause recurrent miscarriage. But in 10-20 percent of cases, the cause of stroke is unknown.

What Are the Signs?
Infants might have seizures or twitches in only one arm or one leg, show a weakness on one side of the body, or have delays with their speech as well as with crawling and walking. Young children might limit their arm or leg movements or show a preference for one hand, and they might also have language delays.

What Are the Long-Term Effects?
In addition to weakness or paralysis on one side of the body, a stroke can cause a child to have trouble with swallowing or speech; he might also find it hard to control his emotions, remember things, or problem-solve. The long-term complications of a stroke might include cerebral palsy, epilepsy, and psychiatric disorders such as ADHD.

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Research Synopsis

I have been doing much research on Neonatal Stroke, causes, and prognosis.  From the scholarly articles and studies I have read, the following is a short synopsis of what I have found thus far.  This is not an exhaustive literature review and is certainly not complete, but it is a starting point.

Fetal stroke man represent an under-reported cause of neurodevelopmental handicap.  These stokes normally occur between 28 weeks gestation and 28 days of life and are common in Neonates (babies born before 33 weeks gestation). Perinatal stroke is reported to occur in 1 in 4000 live births.

Grading System
Intraventricular Hemorrhage (IVH) is graded by the site of bleeding and the presence or absence of ventricular (the ventricles in the brain which hold and circulate Cerebral Spinal Fluid [CSF]). Grade I is the least serious and Grade IV are the most serious and pervasive of bleeds.  About 70% of the bleeds are Grade I - II, 20% are Grade IIII, ad 10% are Grade IV:

Grade I: Is isolated hemorrhage int he germinal matrix (a highly cellular and highly vascularized region in the brain from which cells migrate out during brain development and form neurons) next to the ventricle and there is no bleeding inside the ventricle.

Grade II: The germinal matrix hemorrhage extends into the ventricle but the ventricles are not enlarged.

Grade III: Intraventricular hemorrhage associated with ventricular enlargement.

Grade IV: In addition to Grade III intraventricular hemorrhage there is bleeding within the brain tissue.

The causes of IVH bleeding are not completely clear, but it seems to be related to the regulation of blood flow and pressure within the fragile blood vessels of the Germinal Matrix.  Wide fluctuations in blood flow and inability of the neonatal brain to regulate blood flow probably cause the bleeding.  Abrupt elevations in baby's blood pressure, deprivation of oxygen, trauma to the mother, or some other insult to the developing brain can result in the rupture of the delicate blood vessels of the Germinal Matrix.

Long Term Outcome

Grades I and II bleeds are minor and do little if any harm.  The incidence of neurological problems increases as the severity of the bleeding increases.  Long term neurological problems for babies with this grade of bleed may include slight weakness on one side of the body, Cerebral Palsy (CP), and abnormalities in muscle tone and movement.

Infants with Grade III and IV bleeds normally result in death in approximately 70% of cases. If these children survive, they have little chance of being completely normal.  Problems include pervasive CP, mental retardation, Hydrocephalus, and seizures.

Fetal Stroke Study Review
One study, completed in 2004, reviewed 54 cases of reported IVH.  The mean age of diagnosis was 31.5 weeks gestation. Seventy-eight percent of these resulted in either death or adverse neurodevelopmental outcome at ages 3 months to 6 years.  In half of these cases a definitive cause could not be attributed to the condition.  Trauma and placental hemorrhage or abruption, and maternal trauma was associated with fetal stroke in three of the cases reviewed. 

Prognosis results are as follows: Thirty-two of the 54 fetuses were found to have hydrocephalus (an enlargement of the brain ventricles due to build-up of CSF).  Of these 32, only 25 survived.  Fifteen of the surviving 25 required a shunt to divert the build-up of the CSF into the abdominal cavity and relieve pressure on the brain. 

Twenty-three of the 45 cases resulted in fetal or neonatal death, and 12 of the 22 survivors were handicapped at follow-up ages of 3 months to 6 years.  Many suffered from mental retardation, motor disabilities, visual impairment or seizure disorders. 

Existing data are limited, but indicate that the recurrence risk for perinatal stroke recurrence is 3 - 5%.

Fetal Stroke Study Review

Forty-six children were followed up for a mean of 42.1 months.  Neurodevelopmental outcome was normal in 15 and abnormal in 31.  A single disability was present in 8, and multiple disabilities were present in 23. Cerebral Palsy was present in 22 and cognitive impairment in 19.  Abnormal findings on neurologic examination at discharge and seizures in the neonatal period were associated with the presence of one or more childhood disabilities.

Resources:

Ozduman MD, Koray. Fetal Stroke. Pediatric Neurology 2004; 30: 151-162.

Puri MD, Asha. Cedars-Sinai Medical Center.