metabolic and degenerative encephalopathies Flashcards
1) Which of the below is not a common feature of metabolic encephalopathy on MRI?
a) Bilaterally symmetric signal changes to deep grey matter nuclei
b) Brain atrophy
c) Symmetric and diffuse changes to grey and/ or subcortical white matter
d) Signs of increased intracranial pressure
d
what is A
a) Basal nuclei
b) Caudate nucleus
c) Internal capsule
d) Corpus callosum
b
what is B
a) Basal nuclei
b) Internal capsule
c) Corona radiata
d) Claustrum
c
4) Which of the following is not a finding in dogs with MPS I?
a) Cerebral ventricular enlargement and a small corpus callosum
b) Cardiac valvular disease
c) Hiatal hernias
d) Bone deformities
c, MPS I in dogs causes enlargement of the internal organs,
cardiac valvular disease, arteriosclerotic-like lesions in
large blood vessels, umbilical hernias, corneal clouding,
bony deformities, poor growth, and a shortened life span
(<3 years).
MRI findings have been reported in a Plott Hound
research colony18 and include:
* Cerebral ventricular enlargement and cortical atrophy
at 12 months of age.
* Abnormally small corpus callosum.
5) What MRI changes are seen in dogs with MPS III?
a) Unremarkable MRIs of the brain in these patients
b) Small corpus callosum
c) White matter changes
d) Grey matter changes
a
6) What MRI changes can be seen in dogs with neuronal ceroid lipofuscinosis (select all that apply)?
a) Signs of brain atrophy
b) Small corpus callosum
c) Lack of grey/ white matter distinction in T2w images in some patients
d) Epidural haematoma formation
6) A, B, C (subdural haematoma formation)
what is the structure labelled below?
a) Cerebellum
b) Caudal colliculus
c) Tegmentum of the midbrain
d) Pons
b
8) Which of the following is false regarding mri features of hereditary polioencephalomyelopathies?
a) Symmetrical T2 hyperintensities of brain nuceli
b) Symmetrical T2 hyperintensities of brainstem nuceli
c) Symmetrical grey matter lesions in the spinal cord
d) T1w contrast enhancement of the grey matter lesions
d
what is thr structure labelled c?
a) Putamen
b) Claustrum
c) Lentiform nucleus
d) Amygdala
c
10) Kernicterus is caused by deposition of what substance in the brain?
a) Bilirubin
b) Manganese
c) Glutamine- glutamate
d) lipofuscin
a
which of these is not a lysosomal storage disease?
a) mucopolysaccharidosis
b) neuronal ceroid lipofuscinosis
c) L-2-hyroxyglutaric aciduria
d) globoid cell leukodystrophy
c
what type of lysosomal storage disease is neuronal ceroid lipofuscinosis?
a) glycoproteinoses
b) sphingolipidoses
c) mucopolysaccharidoses
d) proteinoses
d
mucopolysaccharidosis is caused by a disorder of metabolism of what compound?
glycosaminoglycan
most lysosomal storage diseases are inherited in what way?
autosomal dominant
autosomal recessive
x linked dominant
x linked recessive
autosomal recessive
which or these are the 2 most common findings with neuronal ceroid lipofuscinosis?
a) enhancement and thickening of the meninges
b)signs of brain atrophy- wide sulci and ventriculomegaly
c) small corpus callosum
d) subdural haematoma formation
e) lack of grey/ white matter distinction on t2w images
b and c most common, the others have also been reported.
which two diseases have not been documented to have a small corpus callosum?
MPS I
MPS III
gangliosidosis
globoid cell leukodystrophy
MPS III, globoid cell leukodystrophy, also fucosidosis, alpha manosidosis. other diseases with small corpus callosum reported (can be small or partially absent) include GM1 and GM2- gangliosidosis, neuronal ceroid lipofuscinosis
true or false, cerebellar cortical abiotrophy is a type of neuronal cell lipofuscinosis?
true
which of these has not been reported in l2 hydroxyglutaric aciduria?
a) bilateral symmetric grey matter abnormalities affecting the cerebrum, cerebellum, diencephalon, midbrain, pons including swelling and intensity changes on mri
b) t2w hyperintensity of the peripheral subcortical white matter
c) the cerebral cortex and thalamus are commonly affected
d) the caudal coliculi and lentiform nuclei are commonly affected
d, the lentiform nuclei are not mentioned specifically. from recent abstract- L-2-hydroxyglutaric aciduria had the characteristic T2W hyperintense
swelling of the cerebral and cerebellar cortices in all cases
what disease is most likely with this appearance?
a) l2 hydroxyglutaric aciduria
b) MPS I
c) neuronal ceroid lipofuscinosis
d) gangliosidosis
A
what disease is most likely in this Alaskan husky? (T2w images)
a) polioencephalopathy
b) neuronal ceroid lipofuscinosis
c) l2 hydroxyglutaric aciduria
d) hepatic encephalopathy
a. MRI findings include:
* Bilaterally symmetric abnormalities (T2 hyperintensity and T1 iso- or hypointensity without evidence of contrast enhancement) of various brain and brainstem nuclei. often the thalamus is effected.
* Symmetric spinal cord lesion(s) affecting the gray matter (same signal characteristics as brain lesions)
also called necrotizing encephalopathy and can effect yorkies
what are the common mri findings in hepatic encephalopathy?
brain atrophy
on spectroscopy- high glutamine- glutamate and low myoinositol
less common findings include- t1w hyperintense lentiform nuclei and bilateral extensive t2w hyperintense lesions along the cerebral cortex
in recent abstract- t2w hyperintensity of the white matter, specicially the corona radiata.
In this t1w image, which nucleus is hyperintense?
a) lentiform
b) putamen
c)claustrum
d) thalamus
A
6-year-old domestic short hair cat presenting with a recent history of seizures, ataxia, and vestibular signs. which regions are affected and what is the likely disease process?
There is bilaterally symmetrical T2 hyperintensity at the level of the thalamus (lateral geniculate nuclei) (A, white arrows) and also in the colliculi (B, white arrows) due to thiamine deficiency
what disease process and which regions are affected in this dog with hepatic fibrosis?
HE, lentiform nuclei. one of the basal ganglia
