September 7, 2012 - Rawan Albadareen

CC: HA and word finding difficulty
A middle-aged woman who presented to the ED
of OSH with headache and speech difficulty. She
complained of a bilateral frontal headache that
has been getting progressively worse. It started
as pressure sensation that has only recently
turned into a true headache with nausea and
vomiting. Patient says she knows what she wants
to say but has trouble getting the words out.
No significant weakness or numbness.
No Hx of seizures or strokes.
No double vision, dysphagia.
No fevers, chills, or rigors.
◦ Ovarian cyst
◦ migraine
Vitamin D
◦ Mother DM
◦ Father RCC
◦ Smokes 1 ppd.
◦ Drinks 3-4oz Rum
every night
◦ Marijuana 2x per week.
Neurologic Exam
General exam: significant distress
Speech : marked aphasia (difficulty finding
words, difficulty repeating, difficulty naming
objects, and difficulty writing, copying designs
and following 3 step commands)
 CN2-12 generally intact
 Strength 5/5 in all extremities
 Reflexes brisk diffusely
 Sensory exam intact to LT/PP
 Cerebellar exam showed no ataxia
 Gait deferred due to severe HA
 Large left MCA distribution
hypoattenuation likely consistent with
subacute infarction. Diffuse left cerebral
edema, mild mass effect and 6 mm left to
right midline shift. No hyperdense
intracranial hemorrhage.
 1. Avidly enhancing 3.7 x 2.7 cm area, which
demonstrates mild restricted diffusion,
involves the left parietal and temporal region.
There is marked surrounding edema.
Differential for these findings include
primary brain neoplasm versus evolving late
subacute/early chronic infarct.
 2. Mild mass effect with 6 mm midline shift
and compression of left temporal horn.
Significant asymmetry between the sides,
with theta and delta activities being higher in
amplitude and more predominant in the left
than in the right during waking and drowsy
The finding is consistent with moderately
severe non-specific cerebral dysfunction in
the left hemisphere and mild cerebral
dysfunction in the right.
Steroids started and repeat images
Headache and Aphasia almost totally resolved
Follow-up MRI
Previously described mass like area of enhancement
within the left parietal deep white matter
demonstrates marked interval improvement, with
marked interval decrease in the extent and degree of
enhancement of marked decrease in surrounding
edema. Given the significant interval improvement,
now favor inflammatory process such tumefactive
demyelinating plaque (tumefactive MS).
Another consideration felt slightly less likely is
lymphoma, which can demonstrate decreased
enhancement following steroid administration.
Previous consideration of subacute infarct if felt
unlikely due to the current imaging appearance and
the rapid improvement is not consistent with primary
brain neoplasm.
Opening pressure19 cm H2O.
Oligoclonal bands:
◦ Identical patterns in both the CSF and serum.
IgG index within goal
Protein 53
Glucose 183
MBP within range
VDRL -ve
MS treatment started..
And steroids tapered
She presented with worsening HA, , skewed (vertical)
diplopia, aphasia, and a MVA from a seizure
1. Interval increase heterogeneous post contrast
enhancement involves the left parietal white matter with new
areas of focal enhancement in the left thalamus. There is
marked interval increase in surrounding vasogenic edema
within the adjacent subcortical and periventricular white
matter of the left parietotemporal region compared to the
last study in September . However, the edema is not as
pronounced as the initial study performed on 7/28/11. The
enhancement pattern is also not as avid as the initial exam.
Relapsing/progressive tumefactive MS is considered however,
infiltrating neoplasm or encephalitis could also have this
2. Similar appearing edematous expansion of the tectum.
Mild periaqueductal gray matter signal alteration is grossly
3. Mild mass effect on the left lateral ventricle atria without
hydrocephalous. No significant midline shift.
Brain Bx
Diffuse infiltration of the brain
parenchyma by neoplastic astrocytes.
Immunohistochemical study shows that
the tumor cells are positive for glial
fibrillary acidic protein (GFAP). Ki-67
shows a proliferation index of
approximately 15%.
Ki 67
GBM and steroids
Steroid therapy is well established in glioblastoma for
the treatment of peritumoral edema by reducing the
permeability of the blood-brain barrier. (mechanism not
Steroids have no significant tumoricidal effects on
Conversely, cerebral lymphomas are exquisitely sensitive
to the effects of steroids and will not uncommonly
shrink rapidly after commencement of treatment.
Literature Review
Only four other similar cases of rapidly vanishing
glioblastomas after steroid treatment.
 All four cases were histologically proven GBM and were
given high doses of dexamethasone over a few weeks. Serial
imaging performed within a few weeks of commencement of
steroids showed a significant degree of resolution of the
 The recurrence of the tumor typically was very aggressive
and occurred within 1–4 weeks from the time of
 The glioblastomas that vanish rapidly are frequently
multicentric with a predilection for the corpus callosum and
are associated with rapid clinical deterioration and demise of
the patient.
Tumefactive multiple sclerosis
An acute tumor-like MS variant presents with large (>2 cm) acute
lesions, often associated with edema or ring enhancement
There may be mass effect, with compression of the lateral ventricle
and shift across the midline.
Clinical presentation
Typically, much of the T2-bright lesion volume on brain MRI is due
to edema and may be rapidly responsive to glucocorticoid
However, radiologic improvement with glucocorticoids can also
occur with glioma or with CNS lymphoma and is therefore not a
useful diagnostic criterion. Biopsy is often required.
Oligoclonal bands
Oligoclonal bands (OCBs) are found in ≥95 percent of
patients with clinically definite MS.
These represent limited classes of antibodies that are
depicted as discrete bands on agarose gel. Up to 8
percent of CSF samples from patients without MS also
contain OCBs; most are the result of chronic CNS
infections, viral syndromes, and neuropathies.
The presence of OCBs in monosymptomatic patients
predicts a significantly higher rate of progression to MS
than the absence of bands: 25 versus 9 percent at three
years follow-up in one report.
Classic Patterns of OCBs
◦ Type 1 - No OCBs in CSF and serum samples
◦ Type 2 - OCBs in CSF but not serum, indicating intrathecal IgG
◦ Type 3 - OCBs in CSF with additional identical OCBs in CSF and
serum but still indicating intrathecal IgG synthesis
◦ Type 4 - Identical OCBs in CSF and serum, indicating a systemic
immune reaction with a normal or abnormal blood-CSF barrier
and passive transfer of OCBs to the CSF
◦ Type 5 - Monoclonal bands in CSF and serum, indicating the
presence of a monoclonal gammopathy
◦ Repeating the lumbar puncture and CSF analysis is suggested if
clinical suspicion for MS is high but results are equivocal,
negative, or show only a single band on isoelectric focusing.
Take Home Message..
Not all that shines is gold.. And not all
what responds to steroids is MS or CNS
lymphoma.. GBM can also do it!!
A Smart one learns from his own
mistakes.. But a Genius learns from the
mistakes of others..
J.H. Galicich, L.A. French, J.C. Melby. Use of dexamethasone in the treatment
of cerebral oedema associated with brain tumours. Lancet, 81 (1961)pp.46–53
K. Yamada,Y. Ushio, T. Hayakawa. Effects of steroids on the blood brain barrier.
E.A. Neuwelt (Ed.), Implications of the Blood Brain Barrier and its
Manipulations, vol. 2Plenum Press, New York (1989), pp. 53–76
A. Singh, R.J. Strobos, B.M. Singh et al. Steroid-induced remissions in CNS
lymphoma. Neurology, 32 (1982), pp. 1267–1271
4. N. Buxton, N. Phillips, I. Robertson . The case of the disappearing glioma. J
Neurol Neurosurg Psychiatry, 63 (1997), pp. 520–521.
H.S. Zaki, M.D. Jenkinson, D.G. Du Plessis et al. Vanishing contrast
enhancement in malignant glioma after corticosteroid treatment. Acta
Neurochir (Wien), 146 (2004), pp. 841–845
6. Corticosteroids in Brain Cancer Patients Benefits and Pitfalls. Jörg Dietrich;
Krithika Rao; Sandra Pastorino; Santosh Kesari
7. KepesJJ. Large focal tumor-like demyelinating lesions of the brain Ann Neurol
8. Lucchinetti CF, Gavrilova RH, Metz I, et al. Clinical and radiographic spectrum
of pathologically confirmed tumefactive multiple sclerosis. Brain
Thank you

similar documents