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Mai > metabolic and degenerative encephalopathies > Flashcards

metabolic and degenerative encephalopathies Flashcards

1
Q

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

A

d

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2
Q

what is A
a) Basal nuclei
b) Caudate nucleus
c) Internal capsule
d) Corpus callosum

A

b

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3
Q

what is B
a) Basal nuclei
b) Internal capsule
c) Corona radiata
d) Claustrum

A

c

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4
Q

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

A

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.

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5
Q

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

a

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6
Q

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

A

6) A, B, C (subdural haematoma formation)

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7
Q

what is the structure labelled below?
a) Cerebellum
b) Caudal colliculus
c) Tegmentum of the midbrain
d) Pons

A

b

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8
Q

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

A

d

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9
Q

what is thr structure labelled c?
a) Putamen
b) Claustrum
c) Lentiform nucleus
d) Amygdala

A

c

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10
Q

10) Kernicterus is caused by deposition of what substance in the brain?
a) Bilirubin
b) Manganese
c) Glutamine- glutamate
d) lipofuscin

A

a

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11
Q

which of these is not a lysosomal storage disease?
a) mucopolysaccharidosis
b) neuronal ceroid lipofuscinosis
c) L-2-hyroxyglutaric aciduria
d) globoid cell leukodystrophy

A

c

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12
Q

what type of lysosomal storage disease is neuronal ceroid lipofuscinosis?
a) glycoproteinoses
b) sphingolipidoses
c) mucopolysaccharidoses
d) proteinoses

A

d

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13
Q

mucopolysaccharidosis is caused by a disorder of metabolism of what compound?

A

glycosaminoglycan

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14
Q

most lysosomal storage diseases are inherited in what way?
autosomal dominant
autosomal recessive
x linked dominant
x linked recessive

A

autosomal recessive

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15
Q

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

A

b and c most common, the others have also been reported.

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15
Q

which two diseases have not been documented to have a small corpus callosum?
MPS I
MPS III
gangliosidosis
globoid cell leukodystrophy

A

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

16
Q

true or false, cerebellar cortical abiotrophy is a type of neuronal cell lipofuscinosis?

A

true

17
Q

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

A

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

18
Q

what disease is most likely with this appearance?
a) l2 hydroxyglutaric aciduria
b) MPS I
c) neuronal ceroid lipofuscinosis
d) gangliosidosis

A

A

19
Q

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

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

20
Q

what are the common mri findings in hepatic encephalopathy?

A

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.

21
Q

In this t1w image, which nucleus is hyperintense?
a) lentiform
b) putamen
c)claustrum
d) thalamus

A

A

22
Q

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?

A

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

23
Q

what disease process and which regions are affected in this dog with hepatic fibrosis?

A

HE, lentiform nuclei. one of the basal ganglia

24
Q

what is the asterisk over?
lentiform nuclei
caudate nuclei
putamen
geniculate nuclei

A

caudate nuclei

25
Q

which of the following is not a feature of kernicterus?
a) Bilaterally symmetric T2 and T2-FLAIR hyperintensity of thalamus, lateral and medial geniculate nuclei,
caudate nuclei, regions of other basal nuclei, deep cerebellar nuclei, and throughout the cerebral cortical
gray matter
b) Slight decreases in T2 signal intensity in regions of subthalamic nuclei, substantia nigra, and hippocampus.
c) Mild T1 hyperintensity of caudate nuclei, region of the globus pallidus, and deep cerebellar nuclei, with
no evidence of contrast enhancement.

A

b- increased t2w intensity

26
Q

which regions of the brain can be t1w, t2w and flair hyperintense with thiamine deficiency?

A

bilaterally symmetrical, multifocal thalamus- lateral geniculate nuclei, red nuclei, caudal colliculi, vestibular nuclei, cerebellar nodulus.
t2w hyprintense only- caudate nuclei and rostral colliculi.
oculomotor nuclei can also be affected in recent abstract.

27
Q

what nucleus is shown here?
a) lentiform
b) basil
c) medial vestibular
d) caudal colliculus

A

c. in the medulla oblongata

28
Q

Other than cerebellar abiotrophy, what other diseases can cause cerebellar atrophy?
a) neospora
b) panleukopaenia
c) leptospirosis
d) distemper

A

a and b

29
Q

globoid cell leukodystrophy predominantly affects the:
grey matter
white matter
both
neither

A

white matter.
– Increased signal intensity in the corpus callosum on T1W images.
– Symmetric T2 hyperintensity of corpus callosum, centrum semiovale, internal capsule, corona radiata,
and cerebellar white matter.
– Decreased T2 signal intensity of thalamus and caudate nucleus.
– Mild hydrocephalus.
– Symmetric contrast enhancement of the corpus callosum, internal capsule, and corona radiata.
* Hypointensity of white matter tracts on magnetization transfer-weighted images consistent with demyelination

Mai (2 decks)

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