Chiari Malformation, Trauma, CCSVI and NPH

Chiari malformation

A Chiari malformation, or cerebellar tonsillar ectopia (CTE) was once attributed to a malformation of the cerebellum and brainstem in children. It was believed that a slightly oversized brainstem caused crowding in the posterior fossa. In the picture above the posterior fossa lies beneath the cover, called the tentorium cerebelli that separates it from the rest of the brain above.

The posterior fossa contains the cerebellum. Crowding of the posterior fossa forces the inferior (lower) and medial (inner) parts of the cerebellum closest to the foramen magnum (large hole in the base of the skull) down and into it. The tonsils are the lower and inner parts of the cerebellum. Depending on how bad the situation is, it may involve much more of the cerebellum than just the tonsils as in the picture above. Click here for diagnosis and treatment of Chiari.

Later studies demonstrated that Chiari malformations can also be caused by problems in the design of the base of the skull, particularly the posterior fossa, and also an undersized foramen magnum, or tighter type design in the spinal canal of the upper cervical spine. An interesting page on skull design due to race and gender is Cerebellar Tonsillar Ectopia, Race and Gender.

In this regard some babies are born with a shorter length in the base of the skull measured from anterior to posterior, which is front to back. This can crowd the contents of the cranial vault and force them down into the foramen. It is further interesting to note that Down's syndrome babies are often found to have a short skull base. The picture below is of an infant with a short base that died of SIDS shortly after birth.

Chiari, Down's syndrome and SIDS

In addition to design problems in the brainstem, skull or spine, even more recent studies showed a significant correlation between Chiari malformations and trauma. This means that adults can get it later in life. There is also an association between Chiari, syringomyelia and trauma. Moreover, if trauma can cause it in adults, then surely cervical strains during delivery can cause it in newborns.

Lastly, there is a significant correlation between Chiari malformations and spondylosis (degenerative changes of the spine), stenosis (narrowing of the spinal canal), scoliosis,(lateral bend in the spine), tethered cord syndromes (tension in the spinal cord due a genetically short design relative to the length of the spine, or acquired shortening in its length due to degenerative changes).

Venous Compression versus Stenosis

Just recently, vascular surgeon Dr. Paulo Zamboni of the University of Ferrara in Italy has attributed the cause of multiple sclerosis to chronic cerebrospinal venous insufficiency or CCSVI. Recent studies have shown a correlation between Chiari malformations and multiple sclerosis and they share many similar signs and symptoms as well. This further solidifies the link between Chiari malformations, CCSVI and multiple sclerosis. It also lends further credence to a long recognized suspicion of a connection between multiple sclerosis and trauma.

Contact of the brainstem against the bones of the base of the skull or foramen magnum can cause compression of the venous and cerebrospinal fluid (CSF) pathways. Compression of venous pathways can cause CCSVI. Compression of CSF pathways can lead to NPH. Compression of the brainstem against the bones of the base of the skull can also compress cranial and other nearby nerve structures.

a child with a Chiari malformation

Chiari Malformations are typically graded according to the depth of penetration of the brainstem into the foramen magnum. A new term has been coined however, called Chiari 0. In a Chiari 0 the brainstem does not sink into the foramen magnum. Instead, the space typically filled with fluid between the bottom of the cerebellum and base of the skull gets compressed.

This causes the cerebellum to come in contact with the hard bones of the skull and to compress the venous dural sinus drainage routes that lie beneath it. In particular it can cause compression of the much smaller occipital marginal sinus and accessory emissary venous outlets that drain into the vertebral veins during upright posture.

Some researchers now suggest the current method of determining Chiari malformations based on descent into the foramem magnum may be inadequate. Instead, they recommend comparative analysis of CSF volume in suspected Chiari cases to normal ranges. They further suggest that herniation of the cerebellar tonsils into the foramen magnum is not the real problem. The real problem comes from blockage of CSF flow. Consequently, they maintain that a Chiari malformation occurs anytime the brain sinks in the vault sufficient enough to block CSF flow.

a Chiari 0 malformation

Chiari, CCSVI and Normal Pressure Hydrocephalus

In addition to CCSVI, compression of the brainstem in the foramen magnum also affects the subarachnoid spaces of the brainstem and spinal cord in this area, as well as in the spinal canal. The subarachnoid space contains CSF, thus, Chiari malformations can lead to entrapment of CSF resulting in normal pressure hydrocephalus (NPH) in the brain and subsequent NPH without ventriculomegaly. Ventriculomegaly means an increase in the size of the chambers of the brain called ventricles where CSF is produced. Again, some researchers now suggest that NPH occurs when CSF flow becomes blocked with or without ventriculomegaly.

Compression of venous pathways and subsequent CCSVI can decrease the passive CSF pressure gradient. The job of the passive CSF pressure gradient is to increase CSF production during upright posture to compensate for the increase in outflow. This means that CCSVI could lead to a Chiari malformation and subsequent edema and NPH.

Lastly, in addition to blockage of blood and CSF flow, in severe cases of Chiari conditions in children, the blockage of CSF can cause a condition called hydrocephalus in the brain above the point of constriction, and what are called syrinxes, which are cavities in the central canal of the spinal cord, below the point of constriction.

CSF Pulsatile Flow

CSF flow is pulsatile in nature being driven by arterial pressure waves and significantly amplified by respiratory pressure waves. Inhalation sucks CSF into the skull and exhalation pushes it out.

After being produced CSF flows out of the fourth ventricle, which is the last and lowest ventricle, into the cisterns of the subarachnoid space. (Click cisterns for a picture.) Some of it also flows down into the central canal, which runs through the center of the spinal cord. All CSF in the brain and cord flows up through the subarchnoid space of the brain to be absorbed into the superior sagittal sinus (vein) at the top of the skull.

The cavities in the cord called syrinxes are caused by the pulsatile CSF jetting out at the point of constriction on one side like water out of a tight nozzle on a high pressure hose. As it jets out opposite the side of constriction it crashes into the walls of the central canal and over time wears it down like waves beating on coastal cliffs. For diagnosis and treatment go to Chiari Diagnosis and Treatment.

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Living with Chiari

On a regular basis I receive emails asking about Chiari treatments and results. There are no better people to inform others than those who have dealt with it personally.

Help others by sharing your Chiari experience. You can include, for example, the level of Chiari, the probable cause, symptoms, treatment, how you responded to the treatment, would you recommend it to others and even how the condition has affected your life!

What Other Have Contributed

Click below to see contributions from other visitors to this page...

Recently diagnosed 
I'm a 30 year old female, who was recently diagnosed. In October 2015, I was in an altercation . Needless to say my head hit a door frame. It was the left …

chiari and stenosis  
I had cranial stenosis surgery in 1980 at 3 months old. At age 35 I was dignosed with a "mild" case of chiari malformation type . My herniation is 7mm. …

To Inversion Therapy or Not to Inversion Therapy? 
On June 8, 2008, I felt like I had a heart attack. After three weeks of various symptoms they diagnosed me with pneumonia. I was given antibiotics and …

nucca is a scam 
NUCCA is far from a solution to chiari malformation or any structural malformation. The key word being structural. NUCCA is largely toted as,a gentle form …

Too late 
Hi I believe I have never been diagnosed with a Chiari malformation. I am about to be sectioned into mental health hospital. I am now aged 61 and Sixmonths …

EDS, Chiari and complete subluxation of Atlas / Axis 
Where do I start? a very long story to get to EDS diagnosis at 41 and have gone even further downhill since. After some horrific headaches, migraines and …

Surprised at 53! 
Hi, I'm Suellen. I was born with spina bifida almost 62 ears ago and I use a wheelchair for mobility. You may know that spina bifida is very often accompanied …

My name is Jennifer and I was diagnosed with Chiari Type 1 at the age of 35. I had been experiencing strange headaches since I was 18 or so. I would …

EDS, apnoea, heart rate nosediving, balance and leg-dropping 
Can you please help me identify the foramen magnum on the COR and work out if there is a CSF block, crowding of the medulla or a CSF block around the …

Am I better no... Am I a cripple no..... But I sm now disabled 
I was diagnosed with Chiari 1 20 years ago. I am now 60. Initially I went in because I would get very scary excruciating pains after yelling or coughing …

Read it on a X-Ray report-questioning what is going on! 
I will try and keep this quick. I was in a car accident in April 2012. Month after car wreck arms went numb and had a spinal fusion surgery. I was told …

They think i am crazy. 
I was born premature in 1975 with some sort of synostotic plagiocephaly or lambdoid synostosis.this looks like posterior in nature but my cheekbone,wisdom …

He is much better since surgery (to correct Chiari). 
A very dear friend was diagnosed with MS ten years ago. He was in a wheel chair, eyes looking in opposite directions, and on his way down when they realized …

Healthy until car accident 
I was just diagnosed with a "forme fruste" Chiari malformation of 3.5 mm revealed on an upright MRI. My MRI also revealed a right lateral subluxation of …

But you don't look sick 
I was diagnosed with Chiari 1 in 2004 after 2 seizures. I had suffered with headaches for years. Since childhood. I worked at a maximum security prison …

But you don't look sick 
I was diagnosed with Chiari 1 in 2004 after 2 seizures. I had suffered with headaches for years. Since childhood. I worked at a maximum security prison …

Idiopathic intracranial Hypertension, chiari and possible csf leak? 
Hi I'm Kim! I've been spending much of the last year in bed due to major head pressure upon being upright. Upon looking for a csf leak my spinal tap …

Chiari misdiagnosed 
Originally I was diagnosed with Chiari. Later it was found to be Intracranial Hypotension or CSF Leaks which have not yet been located. Being upright …

I have Multiple Sclerosis and Chiara Malformation 
MUSC in Charleston, SC says that they have never seen or heard of anyone that has both MS and Chiari Malformation until they saw me today. Is there anyone …

I was diagnosed with Arnold Chiari Malformation after a car accident. It took a while to diagnose after breast went numb. I had the operation and healing …

My story. 
I was just diganosed with chiari malformation. It scared the hell out of me. My left arm is really week, and I've been off balance forever. I just need …

Too Much Brain to Contain 
After misdiagnosis, bad doctors ("do something nice for yourself, like dye your hair, and you'll be fine!"), I finally received my diagnosis of Chiari, …

Born with "something" Not rated yet
For as many years as I can remember I was told I was the clumsy one, the sickly one, etc. I can remember the family joke being I could trip over a hair …

Looking for the truth Not rated yet
I was diagnosed with Arnold Chiari after 2 years of therapy after a car accident. I had no pain at all until the accident and after researching the malformation …

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Alzheimer's Disease
Arachnoid Granulations
Basal Ganglia
Body Building
Brain Anatomy
Brain Cooling and the Cranial Veins
Cerebellar Tonsillar Ectopia Race and Gender
Cerebrospinal Fluid
Cervical Spine and Cord
Cervical Spondylosis
and Neurodegenerative Disease

Chiari Malformations
Chiari Diagnosis and Treatment
Chiropractic Upper Cervical
Cranial Nerves
Cysts, Syrinxes and CSF
Diffuse Hyperintensity Signals
Dysautonomia, Cerebellar Signs and Multisystem Atrophy
Ehlers Danlos
Foramen Magnum
The Fourth Ventricle
Hyperintensity Signals
Lateral Ventricles
Limbic System
Martial Arts
Multiple Sclerosis
MS Lesions
Multiple Sclerosis Treatment
Neurovascular Tunnels
Normal Pressure Hydrocephalus
Optic Neuritis
Orthogonal Corrective Care
Parkinson's Disease
Parkinson's, Dementia and Neck Injuries
Pelvic Anatomy
Physical Anthropology
The Pituitary Gland and Hypothalamus
Posterior Fossa and Chiari Malformation
Racial Skull Design
Site Search
Skull Anatomy
Skull Base
Skull Deformation and Correction
Skull Diploe
Skull Shape
Spinal Cord Diseases
Spine Anatomy
Spine Injuries
Substantial Nigra
Tethered Cord
Third Ventricle
Thoracic Outlet Syndrome
Tonsillar Ectopia and Chiari Malformations
Treatments and Cures
The Upper Cervical Angle
Upper Cervical Strain
Venous Inversion Flows and Skull Shape
Vertebral Arteries
Vertebral Veins