5. The Defias Brotherhood of Neurodegeneration

Question 1

Answer 1

Fahr Disease (Syndrome)

Question 2

Answer 2

Hallervorden Spatz so called PKAN (pantothenate kinase associated neuropathy)

Iron in globus pallidus

T2 Dark globus + central bright area of necrosis

No enhancenent or diffusion restriction

Question 3

Upper motor neuro loss in the brain and
spine. Most people die within 5 years
(unless you are really good at physics).

• Does NOT show gross volume loss.
• T2/FLA1R tends to be Normal (rarely can
  be bright in the posterior internal capsule).
• Motor Band sign on GRE/SWI sequences.

Answer 3

Amyotrophic Lateral Sclerosis

Question 4

Tauopathy (whatever the F that means).
Awesome clinical manifestations like the
“Alien limb phenomenon ” -50% of cases.

• Asymmetric frontoparietal atrophy

Answer 4

Cortico-basal Degeneration

Question 5

Answer 5

T2/FLAIR bright lesions in the Brainstem,
Basal Ganglia , and Cerebral Peduncles.

They can restrict, but do NOT enhance

Mitochondrial Disorder
Elevated Lactate peak at 1.3 ppm

Question 6

Answer 6

Huntington Disease

Question 7

Caudate Atrophy

Answer 7

Huntington Disease

Question 8

Answer 8

MELAS Syndrome

Mitochondrial Disorder
Lactic Acidosis, Seizures, and Strokes
Elevated Lactate “doublet” at 1.3 ppm

Atypical strokes in the cortical gray matter
with a nonvascular distribution (usually
occipital and parietal).
Underlying WM is nonnal

Question 9

Answer 9

Question 10

Answer 10

Multipie System Atropliy (MSA)

The highest yield pearl is the appearance of the Cerebellar subtype MSA-C

Question 11

Answer 11

Progressive supranuclear palsy

Micky Mouse Sign: Tegmentum Atrophy
with Sparing of the Tectum & Peduncles.

Hummingbird Sign: Midbrain volume loss
with a concave upper surface + relative
sparing of the Pons.

Question 12

Answer 12

Wilson Disease

AR copper metabolism malfunction. Once the liver fills up with copper it starts spilling over into other organs including the brain.