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CC: HA and word finding difficulty

CC: HA and word finding difficulty.

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CC: HA and word finding difficulty

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  1. CC: HA and word finding difficulty • Amiddle-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.

  2. PMHx: • Ovarian cyst • migraine • GERD • FHx: • Mother DM • Father RCC • PSHx: • TAH BSO. • MEDICATION: • Wellbutrin • Relpax • Nexium • Vitamin D • SHx: • Smokes 1 ppd. • Drinks 3-4oz Rum every night • Marijuana 2x per week.

  3. 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

  4. CT • Impression: • Large left MCA distribution hypoattenuationlikely consistent with subacute infarction. Diffuse left cerebral edema, mild mass effect and 6 mm left to right midline shift. No hyperdense intracranial hemorrhage.

  5. MRI • Impression: • 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.

  6. EEG • 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 stages. • The finding is consistent with moderately severe non-specific cerebral dysfunction in the left hemisphere and mild cerebral dysfunction in the right.

  7. Steroids started and repeat images showed • Headache and Aphasia almost totally resolved

  8. 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.

  9. LP • Opening pressure19 cm H2O. • Oligoclonal bands: • Identical patterns in both the CSF and serum. • IgG index within goal • Protein 53 • Glucose 183 • WBC 1 • RBC 0 • MBP within range • VDRL -ve

  10. MS treatment started.. And steroids tapered She presented with worsening HA, , skewed (vertical) diplopia, aphasia, and a MVA from a seizure

  11. MRI • 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 appearance.2. Similar appearing edematous expansion of the tectum. Mild periaqueductal gray matter signal alteration is grossly stable. 3. Mild mass effect on the left lateral ventricle atria without hydrocephalous. No significant midline shift.

  12. 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%. • NO IMMUNOPHENOTYPIC EVIDENCE FOR NON-HODGKIN LYMPHOMA DETECTED WITH THIS HYPOCELLULAR SPECIMEN

  13. H&E 10x Ki 67 H&E 40x GFAP

  14. 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 understood). • Steroids have no significant tumoricidal effects on glioblastomas. • Conversely, cerebral lymphomas are exquisitely sensitive to the effects of steroids and will not uncommonly shrink rapidly after commencement of treatment.

  15. 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 tumor. • The recurrence of the tumor typically was very aggressive and occurred within 1–4 weeks from the time of disappearance. • 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.

  16. 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 treatment. • 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.

  17. 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 CSFsamples 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.

  18. Classic Patterns of OCBs • Type 1 - No OCBs in CSF and serum samples • Type 2 - OCBs in CSF but not serum, indicating intrathecal IgG synthesis • 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.

  19. 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..

  20. References • 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 • 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 • Corticosteroids in Brain Cancer Patients Benefits and Pitfalls. Jörg Dietrich; Krithika Rao; Sandra Pastorino; Santosh Kesari • KepesJJ. Large focal tumor-like demyelinating lesions of the brain Ann Neurol 1993;33:18 • Lucchinetti CF, Gavrilova RH, Metz I, et al. Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 2008;131:1759

  21. Thank you

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