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Exam 2
Congenital Neuro Dz
44
Veterinary Medicine
Professional
02/13/2017

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Cards

Term
What is Lissencephaly? In which species is it normal?
Definition
-smooth brain w/out sulci and gyri
-rodents, reptiles, bird
Term
What is anencephaly?
Definition
-absence of brain
Term
What is microencephaly?
Definition
-small brain
Term
What is hydrocephalus?
Definition
-dilation of ventricles w/ secondary atrophy fo brain
Term
What are the three types of hydrocephalus? What causes each
Definition
-congenital: in fetus due to malformation of ventricular system
-acquired: lue to later life brain lesion that blocks CSF flow
-compensatory: secondary to loss of brain tissue
Term
What is the most common site for congenital obstruction and hydrocephalus?
Definition
-aqueduct of sylvius (mesencephalic duct)
Term
What is hydromyelia?
Definition
-obstruction in spinal cord = dilation of central canal and surrounding spinal cord atrophy
Term
What are the three types of hydromyelia?
Definition
-congenital obstruction
-acquired obstruction: tumor, inflamamtion, post trauma
-secondary to atrophy: compensaotry
Term
What is hydranencephaly?
Definition
-in utero loss of brian tissue w/ replacement by fluid
-can be nearly complete loss of cerebral hemispheres or congital cystic cavities
Term
What are some common causes of hydrancephaly?
Definition
-Bluetongue virus
-akabane virus
-idiopathic
Term
What lesions do we see w/ hydranencephaly?
Definition
-brain has cystic cavities separated from dilated lateral ventricles
Term
What is cyclops?
Definition
-congenital malformation of midline of head: fused cerebral hemispheres, mid brain anomalies, single eye
Term
What causes cyclops in sheep?
Definition
-ingestion of Veratrum californicum during 14th day of gestation
-cyclopamine is toxic alkaloid
Term
What is cerebellar hypoplasia?
Definition
-necrosis of external granule cell layer in utero or early neonatal period
-cerebellum smaller than normal due to little to no granule cell layer
Term
What are the two msot common causes of cerebellar hypoplasia?
Definition
-Panleukpenia virus
-BVDV
Term
What is lost in the brain on a microscopic level w/ cerebelalr hypoplasia? Clinical results?
Definition
-loose the granular of the brain thus granular layer cannot fine-tune movements caused by PUrkinje cells
-hypermetria
Term
True or FAlse: Kittens & calves are susceptible to PLV/BVDV in utero and for the first 10 days of life, resulting in cerebellar hypoplasia.
Definition
-FALSE, true for cats, but calves have to be infected in utero
Term
How do we definitively diagnose cerebellar hypoplasia if no visible lesions are present?
Definition
-send in for histopath, will still lack granular layer
Term
What is cerebellar abiotrphy?
Definition
-spontaneous degen of Purkinje cells due to genetic defect in metabolic or structural protein
Term
Which animals are most commonly affected by cerebellar abiotrophy?
Definition
-Arabian foals
-cattle & dogs
Term
When does cerebellar abiotrophy present clinically?
Definition
-w/in first year of life BUT NORMAL AT BIRTH
Term
What lesions do we see w/ cerebellar abiotrophy?
Definition
-grossly normal
-lack of Purkinje cells on histopath
Term
What is myelodysplasia?
Definition
-ANY developmental anomaly of the spinal cord
Term
What is syringomyelia?
Definition
-cystic cavities in the spinal cord
Term
What is spinal dysraphism?
Definition
-a form of myelodysplasia in which there is failure of neural folds to appose and close resulting in failed neural tubule closer
Term
What lesions do we see w/ spinal dysraphism?
Definition
-openings/malformatons along midline of spinal cord, usually dorsally, involing the central canal
Term
Which animals are most commonly affected by spinal dysraphism?
Definition
-Manx cats, English Bulldogs, Weimaraner dogs
Term
What clinical signs do we see w/ spinal dysraphism?
Definition
-hopping gait, ataxia, unable to stand, fecal or urinary incontinence, and/or spina bifida
Term
What causes myelodysplasia?
Definition
-spontaneously due ot unknown causes
Term
What is spina bifida/cranium bifidum?
Definition
-failure of dorsum of vertebrae or skull to develop
-can be covered by skin or open to outside
Term
What are spina bifida & cranium bifidum commonly associated w/?
Definition
-myelodysplasia, anencephaly, or meningoceles
Term
What is a meningocele?
Definition
-outpouching of fluid filled meninges through spina bifida or cranium bifidum
Term
Are meningoceles more commonly assoc w/ spina bifida or cranium bifidum?
Definition
-cranium bifidum
Term
What are meningoencephaloceles?
Definition
-meningoceles that contain CNS tissue
-aka meningomyeloceles
Term
What are lysosomal storage diseases?
Definition
-autosomal recessive-inherited enzyme defect in neuronal or oligodendroglial neuro lipid or oligosaccharide metabolism
Term
When do lysosomal storage diseases become clinical?
Definition
-progressive neuro deficits during first 6m of life BUT NORMAL AT BIRTH
Term
What lesions do we see w/ lysosomal storage diseases?
Definition
-NO GROSS LESIONS
-histo: marked vacuolation and swelling of affected cells w/ cutoplasm expanded and foamy
Term
What is alpha mannosidosis? Which spp are affected?
Definition
-lysosomal storage dz in which there is an absence of alpha-mannosidase => accumulation of mannose-rich oligosaccharides in neurons
-angus & Galloway cattle and cats
-can see induced form w/ Locoweed
Term
What is Ganlgiosidoses? Which animals are affected?
Definition
-lysosomal storage dz in which GM1 & GM3 gangliosides are neurolipids accumalting in high concentrations in neurons
-occurs in cattle, cats, dogs, pigs, and sheep
-induced form w/ choleratoxin
Term
What is the most common Lysosomal Storage Dz?
Definition
-Globoid cell keukodystrophy (Krabbe's dz)
- A NON-NEURONAL STORAGE DZ
Term
What is Krabbe's Dz?
Definition
-non-neuronal lysosomal storage dz in which atypical glycolipids accumulate and are toxic to oligos & Schwann cells causing spontaneous demylination
Term
Which animals are most commonly affected by Krabbe's dz?
Definition
-dogs, domestic cats, Dorsetsheep (both CNS and PNS)
Term
When does Krabbe's dz become clinical? Presentation?
Definition
-start at 6m of life: ataxia => paralysis
Term
What lesions do we see on histo w/ Krabbe's dz?
Definition
-globoid cells
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