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==Introduction==
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#redirect[[:Category:Central Nervous System - Congenital Abnormalities]]
 
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* Congenital malformations may involve the spinal cord, brain, meninges, calvaria or vertebral column.
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* Abnormalities typically result from defects in neural tube development. 
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* Congenital malformations are present at or before birth, and manifest as either:
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** Morphological problems
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** Functional problems
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*** Tend to arise as biochemical abnormalities, such as the lysosomal diseases or leukodystrophies.
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* Causes of congenital abnormalities include:
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** Environmental causes
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*** For example, these may be toxic, infectious, nutritional, or physical (e.g. radiation)
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*** This is by far the most common cause.
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** Inherited traits.
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==[[Meningoencephalocoele]]==
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* A defect in the cranium allows protrustion of the brain and meninges.
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** There is always crania bifida (a split skull).
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* Meningoencephalocoele may be inherited in pigs and cats.
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[[Category:Central Nervous System - Congenital Abnormalities]]
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==[[Hydrocephalus]]==
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[[Image:hydrocephalus.jpg|thumb|right|150px|Hydrocephalus in situ]] [[Image:hydrocephalussection.jpg|thumb|right|150px|Hydrocephalus. Image courtesy of BioMed Archive.]] [[Image:aqueductstenosis.jpg|thumb|right|150px|Aqueduct stenosis. Image courtesy of BioMed Archive.]]
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* Hydrocephalus is an increased accumulation of fluid in the cranial cavity.
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* There are several different types of hydrocephalus relating to where the fluid is located within the cavity.
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** '''Internal''' hydrocephalus - fluid is within the <u>ventricles</u>
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*** The most common type.
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*** There is unilateral or bilateral dilation of the ventricles.
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** '''External''' hydrocephalus - fluid is within the <u>arachnoid space</u>
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** '''Communicating''' hydrocephalus - fluid is within the <u>ventricles and arachnoid space</u>
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** '''Hydrocephalus ex vacuo''' - the <u>ventricle is dilated secondary to loss of cerebral tissue</U>
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*** Also known as compensatory hydrocephalus.
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[http://w3.vet.cornell.edu/nst/nst.asp?Fun=F_KSsrch&kw=HYDROCEPHALUS View images courtesy of Cornell Veterinary Medicine]
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===Causes===
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* Hydrocephalus may be '''congenital'''.
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** Congenital hydrocephalus is common in brachycephalic and small breed dogs.
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*** It occurs sporadically in cattle.
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** Obstructive lesions are often not found.
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** Amalformed mesencephalic aqueduct may be involved.
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** The cranium may be abnormal, for example, doming.
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* '''Acquired''' hydrocephalus is  the result of obstruction, usually due to inflammation or compression.
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** Space-occupying lesions include :
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*** Neoplasms
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*** Abscesses
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*** Cholesteatomas
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** Inflammation of meninges and/or ependymal cells can lead to hydrocephalus.
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*** For example,  FIP causes ependymitis which can obstruct flow if it affects the mesencephalic aqueduct.
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* Hydrocephalus may also arise with:
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** Increased ventricle size due to loss of brain parenchyma.
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** Overproduction of CSF
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*** Rare
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*** Associated with choroid plexus tumours.
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===Breed predisposition===
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* Some breeds are predisposed to hydrocephalus.
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** Chihuahua, pomeranian, yorkshire terrier, english bulldog, lhasa apso, toy poodle, cairn terrier, boston terrier, pug, pekingese, maltese terrier.
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** Hydrocephalus has also been described in
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*** Bullmastiffs, in association with cerebellar ataxia.
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*** Siamese cats, as an autosomal recessive trait.
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===Clinical Signs===
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* Clinical signs are the result of:
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** Loss of neurons or neuronal function
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** Alterations in intercranial pressure
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** Pathophysiological effects of intracranial disease
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* Signs reflect the anatomical level of disease involvement.
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** Rostrotentorial, vestibular and cerebellar signs are most common.
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* Signs are not related to the degree of ventricular dilation. They are more associated with other damage caused by the disease proces:
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** Intracranial pressure changes
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** Intraventricular haemorrhage
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** Speed of ventricular obstruction
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* Hydrocephalus may contribute to abnormalities of skull development, where it is a congenital disease.
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** Thinning of the bone structure
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** Dome-shape head
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** Persistent fontanelles
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* Ventral or lateral strabismus may occur, although the reason for this is unknown.
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** May be related to distortion of the orbits, due to skill deformity.
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** May be realted with pressue on the mesencephalic tegementum.
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===Diagnosis===
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* Diagnosis is by a variety of imaging and electrophysiologic methods.
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** MRI allows examination of the ventricular system, and give better resolution of the brain parenchyma than CT.
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===Treatment===
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* Prognosis is generally poor.
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* Medical treatment:
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** General supportive care
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** Medications to limit CSF production and reduce intracranial pressure.
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* Surgical treatment:
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** Aims to allow drainage of CSF from the brain to another site for absorption.
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[[Category:Central Nervous System - Congenital Abnormalities]]
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==Cerebellar Defects==
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* Cerebellar defects include:
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** [[Cerebellar Pathology - Pathology#Cerebellar Hypoplasia|Cerebellar hypoplasia]]
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** [[Cerebellar Pathology - Pathology#Cerebellar Abiotrophy|Cerebellar abiotrophy]]
 
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