Transcript Clinical Neurosciences conference

Michael Solle MD in collaboration with Thomas Bouldin MD
Neuroradiology and Neuropathology, UNC-CH
Case # 1

History:
 25 yo female with PMH of TTP (Dx 4-2008)
transferred with 2 days of bloody emesis and
diarrhea. She had thrombocytopenia,
microangiopathic hemolytic anemia, and renal
failure. Treated with plasma exchange & steroids.
 1 week later:
○ After single treatment with rituxan as part of NIH trial,
BP was elevated with systolic >200's, and patient had
headache, then a seizure, and bilateral blindness.
 Head CT & Brain MRI obtained.

Additional History:
 Patient’s vision improved after aggressive blood




pressure control measures were taken.
TTP failed to respond to IV steroids and PLEX,
and vincristine chemotherapy to prevent further
hemolysis was initiated. Renal function
continued to improve, but she had worsening
abdominal pain in the afternoon after
chemotherapy; ultrasound showed an inflamed
pancreas but no signs of gallstones.
She had increasing somnolence and tachypnea
overnight.
Apnea and arrest.
Autopsy performed.
Postmortem brain. Left panel: low power view shows small
hemorrhage (arrow) in cerebral cortex. Right panel: high power
view shows thrombus (arrow) within a small cortical vessel.
Perivascular ischemic necrosis of cortex and hemorrhage are
associated with the thrombosed vessel.
Idiopathic thrombotic thrombocytopenic purpura (TTP)
• Classically presents with the five findings of microangiopathic hemolytic anemia,
thrombocytopenia, renal abnormalities, neurologic manifestations, and fever.
• Neurologic manifestations include headache, confusion, stupor, focal neurologic signs, seizures,
and coma.
• Peak incidence is middle-aged adults, with a female predominance.
• With improved recognition of TTP and better therapies, mortality rate has dropped from 90% to
20%.
• Pathogenesis of idiopathic TTP involves autoantibodies to ADAMTS13, a metalloprotease that
normally cleaves the large multimers of von Willebrand factor (vWF) produced by the vascular
endothelial cells. Persistence of these large vWF multimers leads to the formation of platelet-rich
thrombi in small vessels.
• TTP may be associated with HIV infection, pregnancy, or drug therapy.
• MRI may show a posterior reversible leukoencephalopathy syndrome (PRES) and/or edema of
basal ganglia.
• Plasma exchange (PLEX) is the current treatment of choice for TTP.
Rowland, Lewis P.; Pedley, Timothy A, eds. Merritt’s Neurology, 12 th ed., 2009, p. 1000.
Case # 2

History:
 9 mo female previously healthy, 3-day history of
not being able to sit up, and has "not been
acting herself" over the last week. Mother noted
that child becomes wobbly and falls to the right
upon sitting up. Further history: approximately
5-10 staring spells/day that last 2-3 min each.
During these episodes child does not respond to
movement in front of her eyes. She had 1
episode of emesis this AM when awakening.
 MRI obtained.
 Neurosurgical procedure and biopsy performed.
Biopsy. Left panel: high power view shows densely cellular small blue
cell tumor. Right panel: low power view shows the pale-staining
nodules (arrow) that characterize the desmoplastic/nodular variant of
medulloblastoma.
Follow up study shows development of
diffuse leptomeningeal metastases.
Medulloblastoma
 Mostly
solid & show enhancement
 Often dense on CT
 Can have restricted diffusion
○ Most do not have cysts or calcium
 Hydrocephalus
at presentation
 Arise from roof of fourth ventricle
 CSF spread at diagnosis is assumed
Case # 3

History:
 13 yo female referred for a posterior fossa
mass. She had persistent and worsening
headaches which were accompanied by fatigue,
double vision, and nausea. Headaches were
initially attributed to menstrual migraines and
became continuous for past week. Headaches
described as throbbing and pressure over
occiput, worsened by touching chin to chest and
improved by sitting up.
 CT and brain and spine MRI were done.
Biopsy. Left panel shows astrocytic proliferation with moderate
cellularity, mild atypia, and no mitotic figures. Center panel shows
numerous brightly eosinophilic Rosenthal fibers (arrow) in an H&Estained section. Right panel shows eosinophilic granular bodies
(arrow) in a PAS-stained section.
Pilocytic astrocytoma
Low T1 and high T2 cystic mass
 Homogeneous enhancement of a mural
nodule


Together with medulloblastoma, most
common infratentorial neoplasms in
pediatric age group

DDX:
 Child: medulloblastoma, ependymoma
 Adult: hemangioblastoma (flow voids due to
hypervascularity) and metastases
Syrinx resolved after
resection of mass.
Case # 4

History:
 40 yo male with sensory symptoms and
weakness in hands starting 4-5 years ago.
Symptoms spread to toes, left then right. Feels
unsteady when walking and thinks symptoms
are steadily progressing, especially during the
last year. He cannot run anymore.
 MRI of spine was done.
 Neurosurgical procedure and biopsy performed.
Biopsy. Left panel: low power view shows glial proliferation with
pseudorosette formation around blood vessels (arrow).
Right panel: high power view shows true ependymal rosette.
Spinal cord ependymoma
Most common spinal cord tumor of adults
 Most common in patients with NF-2
 Rarely high grade (anaplastic) ependymoma

Case # 5

History:
 53 yo male transferred with dystonic
movements of left arm and leg concerning
for partial seizures.
 CT and brain MRI were done.
 Neurosurgical procedure and biopsy
performed.
Biopsy. Left panel: low power view shows brain with multiple
areas of inflammation (encephalitis).
Right panel: high power view shows fungal hyphae (arrow),
neutrophils, and multinucleated giant cells.
Fungal encephalitis

Cultures grew Scedosporium apiospermum.
S. apiospermum is the asexual form of
Pseudallescheria boydii and is a ubiquitous
environmental mold. Infection is typically
described in immunocompromised hosts, but
localized infections are also well described in
immunocompetent individuals.
Case # 6

History:
 74 yo female with a history of hypertension
and colon cancer with possible metastases
to the lung. She presents with a 1-week
history of difficulty with gait, dizziness, and
nausea.
 Head CT and brain MRI were done.
 A neurosurgical procedure and biopsy were
performed.
Biopsy. Low power view shows a circumscribed adenocarcinoma
within the cerebellum. The immunohistochemical profile of this
metastatic adenocarcinoma was consistent with a colonic primary.