Degenerative and Latrogenic disorders Flashcards
What are the symptoms associated with pantothenate kinase deficiency (hallervorden-spatz)
Progressive dystonia Oromandibular abnormalities Mental deterioration Pyramidal signs Retinal degeneration
What is the imaging with hallervorden spatz?
Eye of the tiger abnormality
Increased iron deposition, demyelination, reactive gliosis
Calcification in the basal ganglia on CT
high signal (gliosos) with surrounding low signal (iron deposition) on T2 FLAIR
Panthothenate kinase deficiency (hallervorden spatz)
What is the most common cause of complex partial seizures? What is the most common predisposing factor?
Hippocampal sclerosis
Infant febrile seizures
What is the pathophysiology in hippocampal sclerosis?
Neuronal cell loss in the cornu ammonis
fields 1, 3, and 4 of the hippocampal gyrus
What are the imaging findings in hippocampal sclerosis?
What is the treatment?
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
What is the pathology of huntingtons? What is the imaging?
Loss of GABAergic neurons
atrophy of caudate nuclei and putamen, diffuse atrophy
Can see FLAIR signal in the basal ganglia
Caudate atrophy is seen with what?
Huntingtons
What is the imaging in ALS?
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
What is the pathophysiology of alzheimers?
Presence of neurofibrillary tangles and amyloid resulting in death of neurons leading to dementia
What is the imaging in alzheimers?
Marked atrophy of the hippocampi, FLAIR signal intensity in the white matter and temporal lobes
Markedly dilated perivascular spaces
What are the 3 subtypes of multisystem atrophy?
Olivopontocerebellar atrophy
Striatonigral degeneration
Shy-drager syndrome
What is the imaging in multisystem atrophy?
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
Pons/cerebellar atrophy with FLAIR signal in the pons in a cross pattern
Multisystem atrophy
What is the imaging in carbon monoxide?
What is the cause of death?
Necrosis in the globus pallidi with diffuse brain swelling
can show DWI
cardiac arrythmias
What is the imaging in methanol intoxication?
Necrosis of the retina and optic disk, as well as the lateral portion of the putamen and grey white frontal matters
Cerebral edema
What is the imaging in acute radiation injury? What is the treament?
Edema, responds to steroids
What are the findings with late type radiation injury? what is the dose?
demyelination, focal/diffuse necrosis of the white matter
Cavernous angiomas and capillary telangectasias, mineralizing angiopathy, and vasculopathy
IRREVERSIBLE
What is the imaging of liver insufficiency and TPN?
How long until after transplant before signal returns to normal
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
What are the symptoms of osmotic myelinolysis?
Lethargy
Swallowing problems
Progressive quadriparesis
What is the imaging in CPL?
Where are the extrapontine sites?
FLAIR signal and DWI
Midbrain, thalami, basal ganglia, corpus callosum
What is marchiafava-bignami?
Osmotic myelinolysis affecting the corpus callosum and frontal lobe white matter
Alcoholics
What is the imaging of intracranial hypotension?
“Sagging” midbrain - decreased size of suprasellar, interpeduncular, and prepontine cistern
Tonsillar herniation,
Small lateral ventricles, chronic subdural collections
Significant dural enhancement
What causes intracranial hypotension? Who gets them?
What else should be imaged?
What are the complications?
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
What is the imaging in pseudotumor cerebri?
What is associated?
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
What is seen with postshunting corpus callosum lesions?
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
What is the imaging in postshunting corpus callosum lesions
low T1 and high T2 with scalloped margins in the callosum
NO enhancement
