RADIOLOGY REPORT

September 29, 2017

PGI John Christopher luces
 HISTORY:

A 14-month-old male with macrocephaly, developmental

delay, and seizures
 Non-Contrast CT Scan

symmetric diffuse hypoattenuation of the

hyperdense caps along the bilateral frontal
bilateral frontal lobe white matter and
horns
bilateral Candate heads
MRI

Symmetric T1 hypointenslty and T2

hyperintenslty within the bilateral frontal
lobe white matter (in the regions of
hypoattenuation on CT) with characteristic
involvement of the subcortical
U fibers as well as of the deep white-matter
tracts
MRI

There is mild T2 hypointensity and T1

hyperintensity of the periventricular white-
matter rim anterior to the bilateral
frontal horns. Postcontrast images
demonstrate intense enhancement along the
tips of bilateral frontal horns
 DIAGNOSIS:
ALEXANDERS DISEASE
(Infantile form)
 ALEXANDERS DISEASE

mutation to the gene Demyelination and rarefaction of

for glial fibrillary the subependymal, subpial, and
acidic protein
(GFAP)
perivascular white matter with a
frontal predominance.
 Subgroups

INFANTILE JUVENILE ADULT

most common onset 7 and 14 years onset between the 2nd and
characterized by early onset of of age 7th decades.
macrocephaly, developmental Progressive bulbar The symptoms and disease
delay, and seizures symptoms with course can be
Death occurs within 23 years. spasticity are indistinguishable from those
Definite diagnosis usually requires common of classic multiple sclerosis
brain biopsy or autopsy. in the adult subgroup.
CHARACTERSTIC IMAGING FINDINGS
(Infantile form)
 HISTORY:

A 16-year-old male with long-standing history of

partial complex seizures
 well-demarcated,
multilobulated, and markedly
hyperintense lesion in the
posterior left frontal lobe
with multiple internal cystic
appearing areas, giving
it a "bubbly" appearance
The mass is hypointense on
the T1-weighted sequence
and does not enhance after
the administration of
contrast
 DIAGNOSIS:
Dysembryoplastic
neuroepithelial tumor
 Dysembryoplastic neuroepithelial tumor

is a benign (WHO grade I), mixed glial neuronal tumor arising from the
supratentorial cortex

The most common clinical presentation:

long-standing, drug-resistant partial complex seizures in a child or a
young adult

LOCATION: are most commonly found in the temporal lobe

Treatment:
requires surgical resection, which can be curative, even if incomplete.
Cortically based, wedge shaped, multilobulated,
"bubbly' lesion without mass effect in a young
patient with long-standing history of seizures
 HISTORY:

A 14-year-old boy with seizure disorder


gyriform cortical caldfi.cations in the
temporal and ocdpital lobes
Gyriform Low signal corresponding to the
calcifications seen on the CT and volume
loss of the left hemisphere, mostly within
the occipital lobe.
 DIAGNOSIS:
Sturg-Weber syndrome
 Sturg-Weber syndrome

Sporadically occurring, neurocutaneous syndrome

The hallmark of the disease:

vascular angiomatous lesion (i.e., port-wine stain or news tlammeus)
involving the face in the distribution of the trigeminal nerve and
ipsilateral brain and meninges.

Treatment:
requires surgical resection, which can be curative, even if incomplete.

severe atrophy of the left hemisphere
with diffuse hypointensity
and magnetic susceptibility artifact
throughout the cortex
extensive leptomeningeal enhancement
throughout the left hemisphere
Note the compensatory enlargement
of the left frontal sinuses.
Thank you!