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__NOTOC__
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{{Arnold-Chiari malformation}}
{{Arnold-Chiari malformation}}
{{CMG}}


== Pathophysiology ==
{{CMG}}; {{AE}} {{Fs}}
*The most widely accepted pathophysiological mechanism by which Chiari Type 1 Malformations occur is by a reduction or lack of development of the [[posterior fossa]] as a result of either congenital or acquired disorders.
==Overview==
*The [[cerebellar tonsils]] are elongated and pushed down through the opening of the base of the [[skull]] (see [[foramen magnum]]), blocking the flow of [[cerebrospinal fluid]] (CSF).  
The exact pathogenesis of [disease name] is not fully understood.
*The [[brainstem]], cranial nerves, and the lower portion of the [[cerebellum]] may be stretched or compressed.  
 
*Therefore, any of the functions controlled by these areas may be affected. The blockage of CSF flow may also cause a [[syrinx (medicine)|syrinx]] to form, eventually leading to [[syringomyelia]]. Many sufferers turn to the Chiari Institute in Long Island, NY for specialized medical attention and medication.
OR
 
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
 
OR
 
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
 
OR
 
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
 
OR
 
 
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
 
OR
 
The progression to [disease name] usually involves the [molecular pathway].
 
OR
 
The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Pathophysiology==
===Physiology===
The normal physiology of [name of process] can be understood as follows:


===Pathogenesis===
*The exact pathogenesis of [disease name] is not completely understood.
OR
*It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
*[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
*Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
*[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
*The progression to [disease name] usually involves the [molecular pathway].
*The pathophysiology of [disease/malignancy] depends on the histological subtype.


==Genetics==
[Disease name] is transmitted in [mode of genetic transmission] pattern.


Other conditions sometimes associated with Chiari Malformation include [[hydrocephalus]],<ref name="urlNeuropathology For Medical Students">{{cite web|url=http://www.pathology.vcu.edu/WirSelfInst/neuro_medStudents/devdis.html |title=Neuropathology For Medical Students |work= |accessdate=}}</ref> [[syringomyelia]], [[spinal curvature]], [[tethered spinal cord syndrome]], and connective tissue disorders<ref name="Milhorat-2007">{{Cite journal|author=Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA |title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue |journal=[[Journal of Neurosurgery|Journal of Neurosurgery: Spine]] |volume=7 |issue=6 |pages=601–9 |year=2007 |month=December |pmid=18074684 |doi=10.3171/SPI-07/12/601 |url=http://thejns.org/doi/full/10.3171/SPI-07/12/601}}</ref> such as [[Ehlers-Danlos syndrome]] and [[Marfan Syndrome]].<div align="left">
OR
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0001.jpg|Brain: Arnold-Chiari Malformation: Gross fixed tissue sagittal section brain stem
Image:Arnold-Chiari Malformation 0002.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section cerebrum brainstem and cerebellum
Image:Arnold-Chiari Malformation 0003.jpg|Brain: Arnold Chiari Malformation And Polygyria: Gross fix tissue external view
</gallery>
</div>


Genes involved in the pathogenesis of [disease name] include:
*[Gene1]
*[Gene2]
*[Gene3]


<div align="left">
OR
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0004.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brainstem close-up
Image:Arnold-Chiari Malformation 0005.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue brain stem sagittal section close-up
Image:Arnold-Chiari Malformation 0006.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem and cerebellum
</gallery>
</div>


The development of [disease name] is the result of multiple genetic mutations such as:


<div align="left">
*[Mutation 1]
<gallery heights="125" widths="125">
*[Mutation 2]
Image:Arnold-Chiari Malformation 0007.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section brain stem cerebellum and spinal column
*[Mutation 3]
Image:Arnold-Chiari Malformation 0008.jpg|Brain: Hydrocephalus Secondary To Arnold Chiari Malformation: Gross fixed tissue three coronal sections cerebral hemispheres
Image:Arnold-Chiari Malformation 0009.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue cerebellum and brainstem
</gallery>
</div>


==Associated Conditions==
Conditions associated with [disease name] include:


<div align="left">
*[Condition 1]
<gallery heights="125" widths="125">
*[Condition 2]
Image:Arnold-Chiari Malformation 0010.jpg|Spinal cord: Malformation Vertebral Bodies: Gross natural color sagittal section spinal column with malformation in region C7 T1 associated with Arnold Chiari malformation
*[Condition 3]
Image:Arnold-Chiari Malformation 0011.jpg|Brain: Arnold Chiari Malformation; with Hydrocephalus, Type I
Image:Arnold-Chiari Malformation 0012.jpg|Brain: Arnold Chiari Malformation; Type II
</gallery>
</div>


==Gross Pathology==
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].


<div align="left">
==Microscopic Pathology==
<gallery heights="125" widths="125">
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Image:Arnold-Chiari Malformation 0013.jpg|Brain: Arnold Chiari Type II with Meningomyelocele
Image:Arnold-Chiari Malformation 0014.jpg|Brain: Arnold Chiari Malformation; Mid Sagittal
Image:Arnold-Chiari Malformation 0015.jpg|Brain: Arnold Chiari Malformation with Myelocele
</gallery>
</div>




<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0016.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0017.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0018.jpg|Brain: Arnold Chiari Malformation
</gallery>
</div>




<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0019.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0020.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0021.jpg|Brain: Arnold Chiari Malformation, a close up view
</gallery>
</div>




<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0022.jpg|Brain: Arnold Chiari Malformation; Tonsilar Herniation of Cerebellum Compressing Medulla
Image:Arnold-Chiari Malformation 0023.jpg|Brain: Arnold Chiari Malformation; Meningomyelocele, Type II, Meningitis
Image:Arnold-Chiari Malformation 0024.jpg|Brain: Arnold Chiari Malformation, Type II; Meningomyelocele, Meningitis, Close-up of Previous one
</gallery>
</div>




<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0025.jpg|Brain: Arnold Chiari Malformation, Intramedullary Hemorrhage
Image:Arnold-Chiari Malformation 0026.jpg|Brain: Arnold Chiari Malformation, Type II
Image:Arnold-Chiari Malformation 0027.jpg|Spinal Cord: Arnold Chiari Malformation; Type II, Meningomyelitis
</gallery>
</div>


*The most widely accepted pathophysiological mechanism by which Chiari Type 1 Malformations occur is by a reduction or lack of development of the [[posterior fossa]] as a result of either congenital or acquired disorders.
*The [[cerebellar tonsils]] are elongated and pushed down through the opening of the base of the [[skull]] (see [[foramen magnum]]), blocking the flow of [[cerebrospinal fluid]] (CSF).
*The [[brainstem]], cranial nerves, and the lower portion of the [[cerebellum]] may be stretched or compressed.
*Therefore, any of the functions controlled by these areas may be affected. The blockage of CSF flow may also cause a [[syrinx (medicine)|syrinx]] to form, eventually leading to [[syringomyelia]]. Many sufferers turn to the Chiari Institute in Long Island, NY for specialized medical attention and medication.


<div align="left">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0028.jpg|Spinal Cord: Arnold Chiari Malformation; Type II, Meningomyelitis
Image:Arnold-Chiari Malformation 0029.jpg|Brain: Arnold Chiari Malformation
Image:Arnold-Chiari Malformation 0030.jpg|Brain: Arnold Chiari Malformation; Note Z-Shaped Kink in Cervical Spinal Cord
</gallery>
</div>




<div align="left">
Other conditions sometimes associated with Chiari Malformation include [[hydrocephalus]],<ref name="urlNeuropathology For Medical Students">{{cite web|url=http://www.pathology.vcu.edu/WirSelfInst/neuro_medStudents/devdis.html |title=Neuropathology For Medical Students |work= |accessdate=}}</ref> [[syringomyelia]], [[spinal curvature]], [[tethered spinal cord syndrome]], and connective tissue disorders<ref name="Milhorat-2007">{{Cite journal|author=Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA |title=Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue |journal=[[Journal of Neurosurgery|Journal of Neurosurgery: Spine]] |volume=7 |issue=6 |pages=601–9 |year=2007 |month=December |pmid=18074684 |doi=10.3171/SPI-07/12/601 |url=http://thejns.org/doi/full/10.3171/SPI-07/12/601}}</ref> such as [[Ehlers-Danlos syndrome]] and [[Marfan Syndrome]].<div align="left">
<gallery heights="125" widths="125">
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0031.jpg|Brain: Arnold Chiari Malformation with Hydrocephalus
Image:Arnold-Chiari Malformation 0001.jpg|Brain: Arnold-Chiari Malformation: Gross fixed tissue sagittal section brain stem
Image:Arnold-Chiari Malformation 0002.jpg|Brain: Arnold Chiari Malformation: Gross fixed tissue sagittal section cerebrum brainstem and cerebellum
Image:Arnold-Chiari Malformation 0003.jpg|Brain: Arnold Chiari Malformation And Polygyria: Gross fix tissue external view
</gallery>
</gallery>
</div>
</div>




<div align="left">
 
<gallery heights="125" widths="125">
Image:Arnold-Chiari Malformation 0033.jpg|Brain: Cerebellum Arnold Chiari: Gross fixed tissue flattened cerebellum
Image:Arnold-Chiari Malformation 0034.jpg|Brain: Polymicrogyria: Gross fixed brain lateral view of left cerebral hemisphere case of Arnold Chiari cerebellum
</gallery>
</div>


==References==
==References==

Revision as of 16:26, 8 August 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.

Overview

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR


[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

Physiology

The normal physiology of [name of process] can be understood as follows:

Pathogenesis

  • The exact pathogenesis of [disease name] is not completely understood.

OR

  • It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
  • [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
  • Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
  • [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
  • The progression to [disease name] usually involves the [molecular pathway].
  • The pathophysiology of [disease/malignancy] depends on the histological subtype.

Genetics

[Disease name] is transmitted in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

  • [Gene1]
  • [Gene2]
  • [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

  • [Mutation 1]
  • [Mutation 2]
  • [Mutation 3]

Associated Conditions

Conditions associated with [disease name] include:

  • [Condition 1]
  • [Condition 2]
  • [Condition 3]

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].





  • The most widely accepted pathophysiological mechanism by which Chiari Type 1 Malformations occur is by a reduction or lack of development of the posterior fossa as a result of either congenital or acquired disorders.
  • The cerebellar tonsils are elongated and pushed down through the opening of the base of the skull (see foramen magnum), blocking the flow of cerebrospinal fluid (CSF).
  • The brainstem, cranial nerves, and the lower portion of the cerebellum may be stretched or compressed.
  • Therefore, any of the functions controlled by these areas may be affected. The blockage of CSF flow may also cause a syrinx to form, eventually leading to syringomyelia. Many sufferers turn to the Chiari Institute in Long Island, NY for specialized medical attention and medication.


Other conditions sometimes associated with Chiari Malformation include hydrocephalus,[1] syringomyelia, spinal curvature, tethered spinal cord syndrome, and connective tissue disorders[2] such as Ehlers-Danlos syndrome and Marfan Syndrome.



References

  1. "Neuropathology For Medical Students".
  2. Milhorat TH, Bolognese PA, Nishikawa M, McDonnell NB, Francomano CA (2007). "Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and chiari malformation type I in patients with hereditary disorders of connective tissue". Journal of Neurosurgery: Spine. 7 (6): 601–9. doi:10.3171/SPI-07/12/601. PMID 18074684. Unknown parameter |month= ignored (help)


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