Theodor Langhans described the Chiari I malformation in 1881

Dr. Theodor Langhans, Professor and Chair of Pathological Anatomy in University of Bern, Switzerland.

Dr. Theodor Langhans, Professor and Chair of Pathological Anatomy in University of Bern, Switzerland.

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.” 

John Oró, MD

 

Reference

The first description of Chiari I malformation with intuitive correlation between tonsillar ectopia and syringomyelia: Historical vignette

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.

Pseudotumor cerebri a major factor in failure following surgery for the Chiari I malformation

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.

Source

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

Blood glucose, memory and dementia

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

Chiari Educational Lecture in Las Vegas, March 1, 2014

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SATURDAY MARCH 1, 2014

MEET & GREET AND EDUCATIONAL LECTURE

9:00 am Meet & Greet
10:00 am Lecture

SPEAKER: Dr. John Oró

 

TOPIC: Surgical Advances in the Management of the Chiari I Malformation

LOCATION: UNLV - 4505 S. Maryland Parkway Las Vegas, NV 89154

     Student Union; Ballroom Section A

RSVP: Cathy Poznik at cpoznik@CSFinfo.org or 330-998-6195

 

2/27/2014 Update: Thanks to CSF and the engaged and lovely audience. It is wonderful memory. - John Oró

Are Chiari & fibromyalgia related disorders?

At the 2013 Think Tank Meeting in New Orleans, LA on April 27, 2013 Dr. Harold Rekate, Director of The Chiari Institute in Long Island, NY, presented the case of a 36 year old woman with Chiari I malformation & fibromyalgia and discussed the possible relationship between these disorders. See his presentation here: CSF 2013 - Relationship Between Chiari I & Fibromyalgia

Taking Chiari to School: A Parent’s Guide & A Primer for School Nurses

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For easy access, two Conquer Chiari guides have been added to the WorkFlow section:

A positive parent-school relationship is critical when a child has a disability or health condition like Chiari. A collaborative approach, working with school staff to promote your child’s success, is in your child’s best interest. As a parent, your positive involvement at school and your relationship with the school affects your child’s cognitive, emotional, and behavioral performance at school. Taking Chiari to School - A Parent's Guide is intended to provide you with information to assist you as you navigate the school environment and help your child with Chiari succeed.

Prepared by Suzanne Oró, RN, MSN-ed.
This guide is not intended to provide health or legal advice.
Copyright 2012 C&S Patient Education Foundation

Taking Chiari to School - A Primer for School Nurses is for school nurses who may be responsible for the care of a child with the Chiari malformation type one (CM1). Chiari malformation type one (CM1), although not a common condition, may present challenges for the child in the school setting. Even though most children with CM1 are generally able to attend school with their peers, some require nursing care and accommodations in the school setting to promote their safety and success. Your familiarity with the CM1 diagnosis, symptoms, treatments, and available resources will reinforce parent efforts and provide continuity of care for the child with CM1 in school.

Prepared by Suzanne Oró, RN, MSN-ed.
This guide is not intended to provide health or legal advice.
Copyright 2012 C&S Patient Education Foundation

The Chiari Symptom Profile: Key points

  • The Chiari Symptom Profile (CSP) is the first and only patient-based Chiari outcome scale.
  • The CSP for use in measuring patient-based outcome following decompression of the Chiari I malformation with our without syringomyelia.
  • Statistical analysis was supported by a research grant from Conquer Chiari.
  • Statistical reliability and validity of the CSP assessed using PAWS resulted in a Cronbach alpha of .958 (p=.0001) “demonstrating this survey has strong reliability as a survey tool for Chiari symptoms.”
  • The CSP is comprised of 57 items that the patient rates on a Likert-type scale from 0-4, depending on the frequency/severity of the symptom.
  • The lowest possible score is 0 (no disability); highest possible score is 228 (severe disability).
  • The scale measures outcome in four realms:  Physical, Social, Functional and Psychological.
  • The CSP developed under the direction of John Oró, MD, Director of The Chiari Care Center in Aurora, Colorado.
  • Diane Mueller, ND, RN, FNP-BC took the lead in development.
  • At The Chiari Care Center, the CSP is administered and graded by an advanced practice nurse.
  • The instrument is available to any practitioner interested in its use. Contact Diane Mueller, ND, RN, FNP-BC at 303-481-0035 for more information or send a message through the Contact page.

Abstract:  Mueller DM, Oro' JJ.  The Chiari Symptom Profile: development and validation of a Chiari-/syringomyelia-specific questionnaire. J Neurosci Nurs. 2013 Aug;45(4):205-10.

Neurosurgical vs. Orthopedic Management of Chiari associated Syringomyelia

I must admit, I was not aware that orthopedic surgeons treated Chiari associated syringomyelia. They do in Japan. They perform extradural decompression (dura not opened). The results of a study published in the Journal of Neurosurgery: Spine are not surprising: foramen magnum decompression with duraplasty was superior to an extradural decompression. 

The results of the study:

"A total of 232 patients with syringomyelia caused by Chiari I malformation were included in this study. Two-thirds of patients were treated in a neurosurgery department and the other third in an orthopedics department. Neurosurgeons preferred foramen magnum decompression (FMD) with dural patch grafting, and orthopedists preferred FMD with dural dissection. Foramen magnum decompression with dural patch grafting was associated with better outcomes than was dural dissection with regard to the following: motor impairment (66% vs 39%, p < 0.05), sensory disturbance (60% vs 43%, p = 0.051), pain (67% vs 47%, p < 0.05), and cavity size (74% vs 58%, p < 0.05). Improved motor function was associated more with cavity size reduction than with sensory disturbance and pain."

The authors conclusion:

"Neurosurgeons and orthopedists preferred different surgical procedures for the treatment of syringomyelia. Better outcomes were achieved by FMD with dural patch grafting."

John Oró, MD

Source: Different surgical treatment techniques used by neurosurgeons and orthopedists for syringomyelia caused by Chiari I malformation in Japan (Clinical article)

Partially empty sella in pseudotumor cerebri

Patient with Chiari I malformation, syringomyelia, and a partially empty sella (*)

Patient with Chiari I malformation, syringomyelia, and a partially empty sella (*)

Patients with Chiari I malformation, especially if obese, may have associated intracranial hypertension, a condition known as the Chiari pseudotumor cerebri syndrome. Surgical decompression of the Chiari malformation without recognition of pseudotumor cerebri can result in persistent headaches and the development of a post-operative pseudomeningocele.

Recognition of partially empty sella prior to posterior fossa decompression is important. The classic appearance is a gland flattened along the bottom of the sella turcica as in the image. (The condition was initially termed empty sella because the apparently compressed gland was not easily visible on diagnostic studies prior to magnetic resonance imaging. Since MRI allows visualization of the flattened pituitary gland, the term partially empty sella is more accurate.)

While the usual conception is that the gland is compressed into a flattened shape due to increased intracranial pressure, a recent study published online in the Journal of Neurosurgery  provides evidence that the primary cause of a partially empty sella is enlargement of the bony sella and less so a compression of the gland. The authors measured the cross-sectional area of the sella turcica and pituitary on sagittal T1-weighted MR images in "48 patients with pseudotumor cerebri and 48 age-matched controls" and reported:

“Our data showed a mean 38% increase in the sagittal cross-sectional area of the sella turcica in pseudotumor cerebri patients compared with control subjects. This difference in area corresponds to a 63% increase in volume if the sella is modeled as a spherical chamber. The larger the sella, the emptier it appears. This correlation supports Kaufman's hypothesis that the sella becomes enlarged by chronic elevation of intracranial pressure in patients with pseudotumor cerebri. Expansion occurs in all dimensions, but descent of the floor is most prominent.”

“The mean gland area was 42 ± 13 mm2 (95% CI 39–46 mm2) in controls and 34 ± 14 m m2 (95% CI 30–38 mm2) in patients with pseudotumor cerebri. The pituitary gland had a smaller midline cross-sectional area in patients with pseudotumor cerebri (2-tailed t-test, p < 0.05). However… this small reduction in gland cross-sectional area does not necessarily signify a reduction in gland volume.”

Even if the mean gland size is smaller due to “compression or atrophy … the reduction is slight… This explains why tests of pituitary function in patients with pseudotumor cerebri usually show no deficiency.” The authors conclude:

“Our results indicate that chronic elevation of intracranial pressure causes expansion of the pituitary fossa. Little or no reduction occurs in the size of the actual gland. Because the pituitary tissue becomes molded to the walls of a larger container, the sella turcica appears partially empty.” (emphasis added)

John Oró, MD

Source: Enlargement of the sella turcica in pseudotumor cerebri: Clinical article