Showing posts with label neurology. Show all posts
Showing posts with label neurology. Show all posts

Wednesday, November 12, 2014

Infant Neurodevelopment After Congenital HHV-6 Infection

By: Lewis First, MD, MS; Editor-in-Chief 

HHV-6 Inclusion bodies. Photo via the US National Cancer Institute
While we usually think of human herpesvirus 6 (HHV-6) as being the virus responsible for roseola, there is much more to this virus than a simple viral exanthem. About 1 percent of infants are born with vertical transmission of HHV-6 and when this happens, the results are far more worrisome than just being at risk for a viral exanthem.

Dr. Mary Caserta and her colleagues in Rochester (doi: 10.1542/peds. 2014-0886) have been actively studying congenital HHV-6 and this month release their findings on early developmental outcomes of children who have received the virus through vertical transmission from mother to baby. The authors performed developmental testing in a prospective double-blind controlled study at four points between 4 months and 30 months of age.

The results suggest that by 1 year old, some detrimental effects on neurodevelopment can be seen in those infants infected congenitally with HHV-6 compared to controls.

Have you thought about HHV-6 as a cause for developmental delay in your patients? After reading this study, you will want to learn more about what this virus can potentially do if it is present at the time of birth. We suspect more studies will be forthcoming at a faster rate given what we are learning about this virus—and if you haven’t read much about it, this is a great study to start with. Check out the study and learn more!

Related Reading:

Monday, September 15, 2014

Post-Vaccination Seizures: Though Infrequent, Why do They Occur?

Photo by the World Health Organization
By: Lewis First, MD, MS; Editor-in-Chief 

Much anti-vaccine spin has been attributed to the risk of seizures and subsequent epilepsy occurring after administration of some vaccines, and while ongoing epilepsy has not been proven to be a common complication after a post-vaccine seizure, one wonders why epilepsy occurs.

Is it something about the vaccine or is it something about the wiring of a child’s brain that predisposes it to an increased risk of ongoing seizure activity? As it turns out, the latter appears to be true—at least according to an article by Verbeek et al. (doi:10.1542/ peds.2014-0690) being early released online this week.

The investigators studied a cohort of almost 1,000 children in the Netherlands who experienced seizure activity 24 hours after getting an inactivated vaccine or five to 12 days after a live attenuated vaccine and followed those who were given the diagnosis of epilepsy to see if an etiology is found other than the vaccine for ongoing seizures. As it turns out, genetic and anatomic structural defects are identified in the majority of these children, thus making those without such predisposing factors at minimal if any risk for ongoing epilepsy after receiving a vaccine.

This study can be very useful in helping convince families to vaccinate their children in the absence of neurologic underlying abnormalities—but don’t take our word—read this study yourself and see if you don’t agree.

Related Reading:

Thursday, September 4, 2014

75 Years Later, What Does Birth Size Have to Do with Brain Function?

By: Lewis First, MD, MS; Editor-in-Chief 

Photo by Adrian Dressler via Flickr
If a baby is born with a smaller birth size, did you ever think it might influence brain tissue volume and function in that person’s later elderly life?

We certainly did not until we read the manuscript by Muller et al. (doi:10.1542/peds.2014-1108) being early released this week. The investigators studied an Icelandic birth cohort of more than 1,200 men and women born between 1907 and 1935 and then studied that cohort at an average age of 75 years via a brain MRI and cognitive assessment.

The results show an association with lower ponderal index (the ratio of body weight to length) at birth with late-life changes in brain tissue volume and function. Just how does this association play out and were there risk factors beyond birth weight that showed even stronger associations between birth size and elderly brain function?

If this blog doesn't intrigue you to read and learn more, you haven’t really read it. Open your mind to learning more from a cohort that has been studied for three-quarters of a century and offers many thought-provoking ideas as to the very long-term neurodevelopmental outcomes of being born smaller than average.

Related Reading:

Friday, August 15, 2014

Heads Up: Impact Location & Concussion Outcomes in High School Football Collisions

By: Lewis First, MD, MS; Editor-in-Chief 

Photo by Erik Drost via Flickr
It seems that concussion studies are appearing more and more frequently in pediatric peer review journals given the seriousness of these head injuries. Yet few, if any, have looked at the importance of impact location until Kerr et al. (doi: 10.1542/peds.2014-0770) examined rates and outcomes of football concussions by location (front, back, side, and top of head).

So does location matter when it comes to where the head is hit? The authors tackled this question using five years’ worth of national sports-related injury surveillance data to determine that although player-to-player collisions were mostly likely to result in front-of-the head concussions, top-of-the-head concussions most frequently caused a lack of consciousness.

The researchers go for the extra point by exploring the importance of proper tackling technique to reduce concussion rates. So read this study and huddle with your football-playing patients to make sure they are doing all they can to reduce their risk of concussion.

Related Reading:

Thursday, July 31, 2014

Outcomes in Multifocal Neuroblastoma as Part of the Neurocristopathy Syndrome

Our Case Report Editor, Dr. Jeffery Malatack shares a case report we early released this month from our upcoming August issue.

By: Jeffrey Malatack, MD

Neuroblastoma rosettes
Photo by Maria Tsokos, US National Cancer Institute
Neuroblastoma has been appreciated for many years to have patient outcomes related to a variety of unique tumor features, including presence of stage 4S subtype, presence of opsoclonus/myoclonus and association with ROHHAD syndrome (Rapid onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation).

In each of these settings the prognosis of the malignancy is improved by what is believed to be an antitumor immune response. Neuroblastoma occurring as part of neurocristopathy syndrome has not previously been considered another special setting with an improved outcome. The neurocristopathy syndrome occurs due to a germline mutation of a neurogenesis regulator gene. The result is abnormal neural crest cell development resulting in congenital central hypoventilation syndrome, Hirschprung’s disease, and neuroblastoma which is often multifocal and disseminated in its presentation. Such widespread neuroblastoma has been considered highly aggressive and treated with either palliative intent or with very intense high dose chemotherapy.

Dr. Williams and colleagues (doi:10.1542/peds.2013-3340) writing from Australia report a patient with neurocristopathy syndrome who had multifocal neuroblastoma associated with the underlying germline mutation. The reported patient was treated conservatively with surgery and low dose chemotherapy. Following treatment he had extensive residual disease that has continued to mature from malignant to nonmalignant neural tumor despite no further treatment.

A literature review also provided in Dr. Williams’ report identified 26 similar patients presenting with multifocal neuroblastoma as part of the neurocristopathy syndrome. In all cases the neuroblastoma behaved in an indolent manner with no deaths from tumor reported where patients received appropriate treatment. These provocative findings suggest neurocristopathy-associated neuroblastoma should be treated conservatively, despite the aggressive appearance of the disease.

Related Reading:

Tuesday, June 3, 2014

CT or Rapid MRI for Diagnosing a Ventricular Shunt Malfunction: Which Would You Choose?

By: Lewis First, MD, MS

Given the concerns of radiation exposure from CT scans that have been written about in our journal and others, it is great to see other modalities like rapid MRI come on the scene to offer imaging alternatives. One situation where this is occurring is in the diagnosing of a ventricular shunt malfunction.

Photo by Aimun AB Jamjoom 
via Wikimedia Commons
Cerebrospinal fluid ventricular shunts, implanted surgically, are the preferred method for treating pediatric hydrocephalus. But, whether due to mechanical obstruction, over-drainage or equipment failure, malfunctions are commonplace and frequently require imaging to evaluate the potential malfunction. This frequent need to image and use of cranial CT can expose children with ventricular shunts to a large cumulative dose of ionizing radiation. But is rapid MRI accurate enough, compared to CT, to help avoid excess CT scans?

Boyle et al. (doi: 10.1542/peds. 2013-3739) performed a retrospective cohort study of almost 300 children and teens under 21 years of age seen in the emergency department with a possible shunt malfunction to determine the non-inferiority of the accuracy of rapid cranial MRI when compared to CT. The results are worth reading about and may change your approach or your discussion with your local pediatric emergency specialists as to what imaging to recommend.

This study will likely get some nice exposure once it is officially published next month (just not radiation exposure). Check it out and learn more.

Related Reading:

Tuesday, May 27, 2014

Best Imaging Strategy for Evaluation of Neonatal Encephalopathy via the Vermont Oxford Network

By: Lewis First, MD, MS

You don’t have to read far into a journal table of contents nowadays (ours included) to often find studies showing the risks of computed tomography (CT) radiation in children—especially if there are same or better ways to get the information needed besides CT scans that come with increased risk of radiation to the brain. Nowhere is this more of a concern than in our smallest high-risk infants and their immature brains that need to be examined in the setting of a known or suspected disorder such as neonatal encephalopathy.

Photo by denver kid via Flickr
Fortunately, Barnette et al. (doi: 10.1542/peds. 2013-4247), with the help of the large number of babies enrolled in the Vermont Oxford Network (VON), looked at imaging modalities used in the first three days of life in more than 4,000 infants over 36 weeks gestation and/or treated by therapeutic hypothermia to determine the modality, timing and neuroimaging results obtained by CT, ultrasound and MRI. While CT could show hemorrhage and deep brain structural abnormalities better than ultrasound, ultrasound still had a role in screening for problems with backup from MRI, perhaps shifting CT out of the first line imaging strategy if you haven’t shifted it already.

To get a much better handle on the robustness and import of the findings shared in this study, Dr. Donald Frush (doi: 10.1542/peds. 2014-0733) has written a commentary to accompany this study that should not be missed. Scan, or better yet read, this study carefully and learn more.

If you do take care of high risk newborns with potential encephalopathy, what imaging strategy do you use—and would this study change what you are currently doing? Share your thoughts with us by responding to the blog, sending us an e-letter, or posting your thoughts via our Facebook page or Twitter.

Related Reading:

Friday, April 18, 2014

Injury Risk in Children with Epilepsy: New Opportunities for Prevention Strategies

Photo by Amanda Mills via the CDC
Having a seizure disorder is certainly a challenge for our patients, but does having such a disorder further predispose them to accidental fractures, burns, and overdoses of medications?

Prasad et al. (doi: 10.1542/peds. 2013-2554) opted to look into the prevalence of various injuries in
almost 12,000 patients with epilepsy between the ages of 1 to 24 years and compared these patients to a comparison group of more than 46,000 patients without seizures using a large longitudinal data base of primary care records.

Do you counsel your patients with seizures in regard to injury and poison prevention strategies? After reading this study, I suspect you will, based on the results and the discussion of these results by the authors. Check out this early-released study and learn more.

Related Reading:

Wednesday, September 25, 2013

State of the Art Care for Late Preterm Infants

In the last few years, the pediatric community has developed a growing awareness about late preterm infants. These children were previously considered "near term", which meant that they could be managed, for the most part, like term infants.

It is now very clear that they are at risk for long-term neurodevelopment and pulmonary disabilities, perhaps due to interruption of development at a crucial period, as proposed by Kugelman and Colin (doi: 10.1542/peds.2013-1131) in the September State of the Art Review. Pediatric practitioners will encounter many of these infants in their practices as they constitute over 75% of all premature infants. Educators will also need to be aware of the subtle cognitive deficits for which these infants are at risk.