The Chiari & Syringomyelia Foundation (CSF) is dedicated to “finding a cure for Chiari malformation (CM), Syringomyelia (SM) and related disorders.” CSF announcements for January 2016 include the expansion of the unite@night walks and the scheduling of two educational lectures.
unite@night walks to run from May through August
CSF’s “one-mile, casual evening walks around the country” are community activities “that bring together people who are living with the devastating effects of Chiari malformation, syringomyelia, and related disorders.” The walks help support CSF’s local chapters.
This year CSF is expanding the unite@night walk season to run from May through August. To get involved, visit unitenight@CSFinfo.org or contact Cathy Poznik at cpoznik@CSFinfo.org.
“Chiari and the Mind” educational lecture by Dr. Gerald Grant
Dr. Gerald Grant, an Associate Professor of Neurosurgery at Stanford University Medical Center, has special interest in the Chiari malformations, pediatric brain tumors, epilepsy, and trauma.
On Thursday, January 28, 2016 Dr. Grant will present Chiari and the Mind at Stanford University Medical Center. The event will be held in Room 1155 at Lucile Packard Children’s Hospital. A Meet & Greet will begin at 5:30 pm and the presentation starts at 6:30 pm.
Register here or contact Cathy Poznik at cpoznik@CSFinfo.org or 330-998-6195 for questions.
Wayne Connell to present “Invisible No More®: The Seven Realities of the Invisible becoming Visible”
Wayne Connell is founder and president of the Invisible Disabilities® Association and co-author the book, But You LOOK Good, How to Encourage and Understand People Living with Illness and Pain.
Connell will be presenting Invisible No More®: The Seven Realities of the Invisible becoming Visible at the DoubleTree by Hilton Denver Tech Center in Greenwood Village, Colorado on Wednesday, February 3. Meet & Greet begins at 5:30pm and the presentation starts at 6:30 pm.
Register here or contact Cathy Poznik at cpoznik@CSFinfo.org or 330-998-6195 for questions.
John Oró, MD
January 17, 2015
ASAP's 28th Chiari & Syringomyelia Conference announced
The American Syringomyelia & Chiari Alliance Project (ASAP) is dedicated to clinical research and education in the Chiari I malformation and syringomyelia and the support of persons affected with these neurological disorders.
ASAP just announced the 28th Chiari & Syringomyelia Conference will be held in San Francisco, California on July 21-24, 2016. The Conference will be hosted by Gerald Grant, MD, Associate Professor of Neurosurgery at Stanford School of Medicine.
Contact ASAP for more information.
Videos from the 2015 Chiari & Syringomyelia Conference are available online
The 27th Chiari & Syringomyelia Conference held in Ypsilanti, Michigan in July 2015 was hosted by Dr. Cormac Maher, Associate Professor of Neurosurgery at the University of Michigan Health System.
Over 25 specialists presented on various topics regarding the Chiari I malformation and syringomyelia. Videos of the presentations are available on the ASAP website (flashvideo) and YouTube channel (MP4).
John Oró, MD
January 17, 2016
Pseudomeningocele (PM) is one of the more common complications following posterior fossa decompression for the Chiari I malformation (CM-I). A leak of spinal fluid through the duraplasty creates a pocket of CSF in the posterior cervical muscles. If the fluid collection enlarges, it pushes the duraplasty membrane into the foramen magnum region causing crowing and recurrence of the Chiari symptoms. In a few cases, spinal fluid leaks through the incision and, if untreated, leads to infection.
While a number of Chiari centers have been able to keep the risk of pseudomenigocele very low, rates as high as 18% patients have been reported.
If the PM is small, it can be observed with follow-up MRI scans and may resolve on its own. However, large persistent PMs pose difficulties. The duraplasty can become adherent to the underlying cerebellar tonsils and block CSF flow. Once adherent, surgical revision is difficult.
Dr. Scott Parker and colleagues at the Department of Neurological Surgery at Vanderbilt University studied the effects of symptomatic PMs on the 1-year postoperative “pain, disability, and quality of life” in patients undergoing Chiari decompression.
The researchers found that “a postoperative symptomatic pseudomeningocele has lingering effects at 1 year, which significantly diminishes the overall benefit of suboccipital decompression for CM-related symptoms.”
While the authors use this finding to argue for a “less invasive approach,” I have a different view. The less invasive approaches, such as thinning of the dura by stripping its outer layer, have a higher a risk of failure than the duraplasty approach.
The goal of surgery for the Chiari malformation is adequate posterior fossa decompression with minimal surgical risks. Thus, the key is to use a duraplasty technique that markedly decreases the risk of pseudomeningocele. This is possible through the use of autologous pericranium harvested from a separate small incision in the midline occiput just above the main incision.
Using triangular silastic templates, the appropriate size of graft can be obtained. The pericranial graft is sutured in place with a monofilament running suture in a watertight fashion. Two Valsalva maneuvers to 35 cm are performed following the repair and if any leak is visualized under microscopic magnification, the area is oversewn or a secondary patch is used.
Durplasty using a patient’s own pericranial tissue has been effective in posterior fossa decompression for patients with CM-I. The leak rate can be reduced to a minimal level: 2% or less. The use of a duraplasty results in greater expansion of the subarachnoid space at the foramen magnum than possible in non-duraplasty procedures and allows the patient the best opportunity to improve.
If a leak does develop, it is followed closely and if it enlarges, treated with lumbar drainage or surgical revision. Fortunately, the likelihood is low in centers experienced with the pericranial duraplasty technique.
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The third today
We all have them
So they say
I’m getting dizzy
My vision blurred
Your getting older
Don’t be absurd
I’m staying home
Too tired to go
Go see your doctor
She should know
Too many symptoms
You must be stressed
And get some rest
I cannot think well
Can’t find the words
My memory slipping
My speech gets slurred
We’ll get a scan
Since you insist
If we find nothing
Please don’t persist
Don’t make me laugh
Don’t make me cry
It hurts to strain
I don’t know why
Your scan is fine
By report today
So they say
What do you mean
By a doctor
I have not seen
It is anxiety
That makes you so
The world is stressful
I think you know
I pulled away
Gave up, or such
Until the pressure
Became too much
I waded through
Fearful of loosing
What I had left
It took some time
To come to know
My brain is hanging
Down too low
With every beat
The fluid flows
Deep in the brain
To the opening below
The rush of fluid
Which makes it cease
The pressure spikes
My head explodes
It makes such sense
Now that I know
We’ll find a way
To make it better
So I say
A study performed at the University of Missouri School of Medicine published recently in the Journal of Neurosurgery: Pediatrics revealed the prevalence of Chiari I malformation (CM-I) in children and adolescents with autism and discussed the difficulty of recognizing CM-I the co-existence of these two disorders.
The authors evaluated the brain MRI scans of 125 pediatric patients younger than 18 years of age seen at the Thompson Center for Autism and Developmental Disorders. The Chiari I malformation was found in 5 children, their ages ranging from 3 years 9 months to 5 years. The amount of cerebellar tonsillar herniation varied from 8 to 19 mm. All five patients presented with symptoms of autism spectrum disorder (ASD) and CM-I and underwent posterior fossa decompression by surgeons at the Division of Neurological Surgery consisting of “suboccipital craniectomy, a C-1 or a C-1 and C-2 laminectomy, and duraplasty with bovine pericardium or Type I collagen allograft.”
Outcome in these 5 autistic children with CM-I was uniformly positive:
“All patients showed symptom improvement and/or resolution of presenting symptoms, which included headache, dysphasia, speech, and irritability.”
Recognizing the presence of CM-I in children with autism can be difficult. As noted in the study, symptoms of ASD include “impairments in social interaction, communication difficulties, and the presence of restricted, repetitive, and stereotyped patterns of behavior.” Frequently these impairments make it difficult to recognize other associated disorders such as CM-I. Thus, recognizing the symptoms and findings of CM-I in autistic children of is of utmost importance. Common symptoms include “headaches, ocular disturbances, sleep apnea, lower cranial nerve compression, dysphagia, dysarthria, disequilibrium with vertigo, and motor or sensory findings caused by syringomyelia or scoliosis.”
“Features such as head holding, head banging, and difficulty in flexion and extension of the neck suggest the likelihood of headaches and neck pain. Swallowing difficulties and dysarthria may reflect lower cranial nerve dysfunction, while difficulties in balance and ambulation may reflect cerebellar or spinal cord compromise.”
The authors note the “current frequency of CM-I in the pediatric and adult population is thought to be about 1%–3.6%,” a 4% prevalence roughly similar to that in the general population.
Should routine MRI screening be performed in all children with ASD? The authors do not recommend this:
“Nevertheless, it is difficult to recommend performing cranial imaging in all patients with ASD to evaluate for CM-I, as the patient may be asymptomatic and such a practice does not represent a cost-effective form of health care delivery.”
However, they emphasize alertness on the part of evaluating physician and “clear communication” among treating specialists:
“It is therefore imperative that the physician is alert to the history given by the child’s parents or caretakers, as well as the observed clinical findings, and corroborates these aspects with the sensible utilization of further testing and subsequent management.”
“Awareness of the coexistence of symptomatic CM-I and ASD necessitates clear communication between primary care physicians, autism specialists, pediatric neurologists, and pediatric neurosurgeons.”
John Oró, MD
January 31, 2015
Another Reason to Evaluate the Foramen of Magendie during Posterior Fossa Decompression for Chiari-related Syringomyelia
When treating a patient with Chiari related syringomyelia by posterior fossa decompression, it is important for the neurosurgeon to open the dura and the arachnoid and evaluate the outlet of the fourth ventricle which drains CSF into the cisterna magna. The outlet called the foramen of Magendie after its discoverer is also known as the median aperture. Approximately 1 of 10 persons with Chiari/syringomyelia has a veil over the median aperture blocking or restricting CSF flow. Clearing the obstruction by microsurgically opening the veil facilitates syrinx decompression and helps avoid revision surgery.
In the December 2014 issue of the Journal of Neurosurgery, S. Grossaur of the Department of Neurosurgery, General Hospital of Klagenfurt, in Austria, and colleagues Koeck K, Vince GH published a report documenting significant improvement in the somatosensory evoked potentials (SSEPs) following the opening of an obstructed foramen of Mangendie. The authors describe the
case of a 32-year-old woman who underwent surgery for CM associated with extensive cervicothoracic syringomyelia and whose intraoperative somatosensory evoked potentials (SSEPs) did not notably improve after craniotomy or following durotomy; rather, they only improved after opening of the fourth ventricle and restoration of CSF flow through the foramen of Magendie. Postoperatively, the patient recovered completely from her preoperative neurological deficits. To the authors' knowledge, this is the first report of significant SSEP recovery after opening the fourth ventricle in the decompression of a CM-I.
When evaluating patients referred for persistent syringomyelia following posterior fossa decompression, who on MRI studies appear to have an adequate decompression, a careful review of the operative note should be performed to determine if the dura and arachnoid where opened during the initial procedure. If the dura or arachnoid had not been opened, the revision surgeon should consider exploration of the posterior fossa and examination for a possible median aperture veil (retained rhombic roof) as the preferred treatment strategy instead of placement of a syrinx stent or shunt.
John Oró, MD
January 2, 2015
Hans Chiari is appropriately recognized for describing and categorizing the Chiari malformations in reports published in 1891 and 1895. While others such as Jean Cruveilhier (1798-1874) and John Cleland (1835-1925) also made observations prior to Chiari, in an article published in the Journal of Neurosurgery: Pediatrics in March 2011 Martin M. Mortazavi and colleagues propose Theodor Langhans, the German pathologist remembered for describing “Langhans cells” in tuberculosis, as the first to describe the Chiari I malformation.
Mortazavi et al. review Theodor Langhans’ distinguished background:
“Theodor Langhans was born September 28, 1839, in Usingen (Nassau), Germany, and studied under Henle in Göttingen and von Recklinghausen in Berlin. He attended medical school in Heidelberg and, in 1864, completed his medical degree thesis on the structure of tendons in Würzburg. He was also a student under such names as Virchow, Trauber, and Frerichs. He served as assistant to von Recklinghausen until 1867. In Marburg, he collaborated with Lieberkölin and Wagner on anatomical research. He was later made Professor Ordinarius in Giessen and then moved to Switzerland in 1872 where he was appointed Professor and Chair of Pathological Anatomy in Bern, succeeding Klebs.”
In 1881, Langhans described a case of syringomyelia and “pyramidal tumors” of the cerebellum in the publication Über Höhlenbildung im Rückenmark als Folge Blutstauung. Mortazavi et al. translated the report (“Regarding cavity creation in the spinal cord as a consequence of obstruction to blood flow”) and believe “these were the first descriptions of what would become known as the Chiari I malformation described by Langhans as ‘pyramidal tumors.’” These selected passages are key to the description and reveal Langhans awareness of the anatomy and pathophysiology of CMI:
“In the case, which first brought to my attention the necessity to look for cavity formation in the spinal cord following a change in the cerebellar cavity, I could not find a cause for the increase in pressure; but great pressure on the pons and medulla oblongata from above was indeed apparent. Upon dissection of the cerebellum, nothing was of note except for an obvious/significant development of both tonsils, which protruded down in the form of two symmetrical pyramidal tumors and pushed the medulla oblongata in a frontal direction at almost a right angle.” (emphasis added)
“The increase in pressure in the cerebellar cavity will hinder or greatly impede the outflow of blood and cerebral spinal fluid.” (emphasis added)
Mortazavi and colleagues recognize Langhans' contribution and conclude:
“Although the association of Chiari I malformation and syringomyelia would not be commonly used until the late 20th century, it was Langhans in the 19th century who proposed this cause and effect. Therefore, appropriate recognition for this association should be given to this early pioneer who, with Chiari, helped provide us with details of hindbrain herniation that are still in use today.”
Martin M. Mortazavi, M.D., R. Shane Tubbs, M.S., P.A.-C., Ph.D., Maja Andrea Brockerhoff, M.A., Marios Loukas, M.D., Ph.D., and W. Jerry Oakes, M.D.
It has been known for some time that some dog breeds such as the Cavalier King Charles spaniels can have the Chiari-like malformation (CLM). Their small skulls crowd the brain and result in herniation of the cerebellum and brain stem through the foramen magnum. About 50% also have syringomyelia.
Among the clinical signs, as described by the Canine Inherited Disorders Database, is “phantom” scratching:
“The most important and consistent sign of CM/SM is pain, which your dog may show through crying out, withdrawn behavior, reluctance to exercise, intolerance of a neck collar or touch about the head and neck, and sleeping with the head raised. Affected dogs may have scoliosis (curvature of the spine), weakness and poor coordination and they may scratch on one side without touching the skin – called “phantom” scratching.”
Chiari-like malformation has also been found in other "toy" breeds such as Chihuahuas and the Brussels griffon. In order to determine the skull anatomy that results in CLM, a team of investigators from the United Kingdom, U.S. and Canada performed MRI measurements on skulls of “Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables.” In the study, published recently in PLOS ONE, the authors
"... found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes."
"Our latest discoveries will be significant in driving this research forward. Our next steps will be to apply our technique to other breeds with Chiari malformation such as the Cavalier King Charles spaniel and Chihuahua."
"We also want to investigate more sensitive ways of screening so that risk of disease can be detected easier, at an earlier age and with a single MRI scan."
Improving treatment outcome in the Chiari I malformation (CM1) depends on many factors; among the most important is the recognition of associated co-morbidities. A common associated condition is idiopathic intracranial hypertension, also known as pseudotumor cerebri (PTC).
In 2006, Fagan et al. of The University of Chicago Children’s Hospital in Chicago, Illinois published a milestone study recognizing the importance of considering this disorder in patients with CM1, especially as the body mass index of the U.S. population continues to increase.
The authors defined the problem and goal of their study –
“The etiology of Chiari malformation type I (CM1) as well as other anomalies associated with CM1 remains poorly defined. We have noted the presence of elevated CSF pressures with small ventricles, consistent with the pseudotumor cerebri (PTC) syndrome in a group of CM1 patients that did not respond over the long term to posterior fossa decompression. In order to better understand this association, we reviewed a series of CM1 patients treated by posterior fossa decompression to define the prevalence and nature of post-Chiari PTC.”
The records of 192 patients with CMI previously treated by posterior fossa decompression were reviewed. The 36 patients failing surgery had a lumbar puncture performed to measure their spinal fluid pressure. Of these, 15 patients (42%) were found to have elevated intracranial pressure consistent with pseudotumor cerebri; a high number that should serve to get this disorder on the radar of all healthcare providers caring with persons with CM1.
Of the 15 patients found to have the Chiari pseudotumor cerebri syndrome, 14 required subsequent treatment with a CSF shunt. Outcome following shunt placement varied depending on age of the patient: “Seven of 9 pediatric patients had significant symptom resolution while 6/6 adult patients remained variably symptomatic.”
The author’s concluded:
“CM1 and PTC co-exist in a surprising percentage of failed operative CM1 patients and present with a syndrome that is difficult to treat.” (emphasis added)
Future posts will look further at this important syndrome including its presentation, diagnosis and treatment.
Fagan LH, Ferguson S, Yassari R, Frim DM.
The Chiari pseudotumor cerebri syndrome: symptom recurrence after decompressive surgery for Chiari malformation type I
Pediatr Neurosurg. 2006;42(1):14-9
Currently at the Las Vegas airport waiting for my return flight to Denver. This morning I attended the Chiari & Syringomyelia Foundation public meeting at the University of Nevada, Las Vegas. A section of my talk was on the Chiari/Pseudotumor Cerebri Syndrome, a challenging condition in which the Chiari I malformation and pseudotumor cerebri occur together.
In 2006, Dr. David Frim and colleagues at The University of Chicago described the syndrome in a study of 36 patients that had failed Chiari surgery. The study revealed 42% of these patients had associated pseudotumor cerebri (AKA: idiopathic intracranial hypertension), a condition of raised brain pressure of uncertain cause.
Since one of the suspected causes of pseudotumor cerebri is inflammation, a question at the end of the presentation led to a discussion of the role of nutrition. While inflammation is a complex and not yet fully understood condition, information is developing on the neurological effects of sugar in our diet. I mentioned two recent studies warning us that even mild elevation of blood glucose (above normal but not to the diabetic level) can hurt the brain.
One study showed that mildly elevated blood glucose can shrink the hippocampus, the key brain center for memory storage. The other study revealed mildly elevated blood glucose increases the risk of dementia. To learn more about these two studies, see my posts on PaleoTerran.com: