Degenerative and Latrogenic disorders

Question 1

What are the symptoms associated with pantothenate kinase deficiency (hallervorden-spatz)

Answer 1

Progressive dystonia
Oromandibular abnormalities
Mental deterioration
Pyramidal signs
Retinal degeneration

Question 2

What is the imaging with hallervorden spatz?

Answer 2

Eye of the tiger abnormality

Increased iron deposition, demyelination, reactive gliosis

Question 3

Calcification in the basal ganglia on CT

high signal (gliosos) with surrounding low signal (iron deposition) on T2 FLAIR

Answer 3

Panthothenate kinase deficiency (hallervorden spatz)

Question 4

What is the most common cause of complex partial seizures? What is the most common predisposing factor?

Answer 4

Hippocampal sclerosis

Infant febrile seizures

Question 5

What is the pathophysiology in hippocampal sclerosis?

Answer 5

Neuronal cell loss in the cornu ammonis

fields 1, 3, and 4 of the hippocampal gyrus

Question 6

What are the imaging findings in hippocampal sclerosis?

What is the treatment?

Answer 6

Hippocampal volume loss with high FLAIR signal in the hippocampus

Also see: atrophy of ipsilateral fornix and mamillary body, loss of undulations of pes hippocampus, ipsilateral temporal lobe volume loss and blurring of grey white junction, dilation of temporal horn of lateral ventricles

temporal lobe resection

Question 7

What is the pathology of huntingtons? What is the imaging?

Answer 7

Loss of GABAergic neurons

atrophy of caudate nuclei and putamen, diffuse atrophy

Can see FLAIR signal in the basal ganglia

Question 8

Caudate atrophy is seen with what?

Answer 8

Huntingtons

Question 9

What is the imaging in ALS?

Answer 9

Atrophy of the frontal lobes with widening of the central sulcus

Increased deposition of iron in the motor strip including the posterior limbs of the internal capsules

FLAIR SIGNAL IN THE CORTICOSPINAL TRACTS

Question 10

What is the pathophysiology of alzheimers?

Answer 10

Presence of neurofibrillary tangles and amyloid resulting in death of neurons leading to dementia

Question 11

What is the imaging in alzheimers?

Answer 11

Marked atrophy of the hippocampi, FLAIR signal intensity in the white matter and temporal lobes

Markedly dilated perivascular spaces

Question 12

What are the 3 subtypes of multisystem atrophy?

Answer 12

Olivopontocerebellar atrophy
Striatonigral degeneration
Shy-drager syndrome

Question 13

What is the imaging in multisystem atrophy?

Answer 13

Severe atrophy of brainstem/cerebellum

FLAIR in the pons with a “hot cross buns” appearance

Dark T2 (iron) in the basal ganglia

FLAIR in the cerebellar peduncles

Generalized atrophy

usually spares cerebrum

Question 14

Pons/cerebellar atrophy with FLAIR signal in the pons in a cross pattern

Answer 14

Multisystem atrophy

Question 15

What is the imaging in carbon monoxide?

What is the cause of death?

Answer 15

Necrosis in the globus pallidi with diffuse brain swelling

can show DWI

cardiac arrythmias

Question 16

What is the imaging in methanol intoxication?

Answer 16

Necrosis of the retina and optic disk, as well as the lateral portion of the putamen and grey white frontal matters

Cerebral edema

Question 17

What is the imaging in acute radiation injury? What is the treament?

Answer 17

Edema, responds to steroids

Question 18

What are the findings with late type radiation injury? what is the dose?

Answer 18

demyelination, focal/diffuse necrosis of the white matter

Cavernous angiomas and capillary telangectasias, mineralizing angiopathy, and vasculopathy

IRREVERSIBLE

Question 19

What is the imaging of liver insufficiency and TPN?

How long until after transplant before signal returns to normal

Answer 19

Increased signal intensity in the basal ganglia on T1 and normal signal on T2 without CT abnormality

High T1 signal in the dorsal brainstem, midbrain

3 months

Question 20

What are the symptoms of osmotic myelinolysis?

Answer 20

Lethargy

Swallowing problems

Progressive quadriparesis

Question 21

What is the imaging in CPL?

Where are the extrapontine sites?

Answer 21

FLAIR signal and DWI

Midbrain, thalami, basal ganglia, corpus callosum

Question 22

What is marchiafava-bignami?

Answer 22

Osmotic myelinolysis affecting the corpus callosum and frontal lobe white matter

Alcoholics

Question 23

What is the imaging of intracranial hypotension?

Answer 23

“Sagging” midbrain - decreased size of suprasellar, interpeduncular, and prepontine cistern

Tonsillar herniation,

Small lateral ventricles, chronic subdural collections

Significant dural enhancement

Question 24

What causes intracranial hypotension? Who gets them?

What else should be imaged?

What are the complications?

Answer 24

Chronic leak of CSF leading to low ICP

Headaches in 20-30yo women

Entire spine to look for leaks or nerve root diverticula

Venous thrombosis and infarctions, particularly cerebellum and medulla

Question 25

What is the imaging in pseudotumor cerebri?

What is associated?

Answer 25

Normal with minor changes in subarachnoid space volumes and ventricle size.

Fluid around optic nerves, flattening of the posterior globes, protrusion of the optic nerve heads into the vitrei

Empty sella

Question 26

What is seen with postshunting corpus callosum lesions?

Answer 26

Chronic compression against rigid falx by enlarged lateral ventricles and subsequent decompression of ventricles is the factor that leads to softening and stretching of corpus callosum

Glioss, demyelination, and edema can be present

Can get large callosal cysts too

Stretching of anterior cerebral artery branches can result in ischemia

Question 27

What is the imaging in postshunting corpus callosum lesions

Answer 27

low T1 and high T2 with scalloped margins in the callosum

NO enhancement