Clinical Neurosciences conference

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Michael Solle MD in collaboration with Thomas
Bouldin MD Neuroradiology and Neuropathology,
UNC-CH
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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 gt200's,
  and patient had headache, then a seizure, and
  bilateral blindness.
• Head CT Brain MRI obtained.

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• Additional History
• Patients 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.

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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.
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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.
Merritts Neurology, 12th ed., 2009, p. 1000.
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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.

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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.
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Follow up study shows development of diffuse
leptomeningeal metastases.
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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

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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.

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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 HE-stained section. Right panel shows
eosinophilic granular bodies (arrow) in a
PAS-stained section.
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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.
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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.

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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.
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Spinal cord ependymoma

• Most common spinal cord tumor of adults
• Most common in patients with NF-2
• Rarely high grade (anaplastic) ependymoma

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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.

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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.
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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. 

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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.

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Biopsy. Low power view shows a circumscribed
adenocarcinoma within the cerebellum. The
immunohistochemical profile of this metastatic
adenocarcinoma was consistent with a colonic
primary.
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