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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 467-469

Peri-ictal pseudoprogression [pipg] in an operated case of right frontal glioma; case report and review of literature


1 Department of Neurology, Aster Medcity, Cheranelloor, Kochi, Kerala, India
2 Department of Neurosurgery, Aster Medcity, Cheranelloor, Kochi, Kerala, India

Date of Web Publication19-Sep-2014

Correspondence Address:
Boby Varkey Maramattom
Department of Neurology, Aster Medcity, Cheranelloor, Kochi, Kerala
India
Boby Varkey Maramattom
Department of Neurology, Aster Medcity, Cheranelloor, Kochi, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.141291

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How to cite this article:
Maramattom BV, Panikar D, Maramattom BV, Panikar D. Peri-ictal pseudoprogression [pipg] in an operated case of right frontal glioma; case report and review of literature. Neurol India 2014;62:467-9

How to cite this URL:
Maramattom BV, Panikar D, Maramattom BV, Panikar D. Peri-ictal pseudoprogression [pipg] in an operated case of right frontal glioma; case report and review of literature. Neurol India [serial online] 2014 [cited 2020 Oct 20];62:467-9. Available from: https://www.neurologyindia.com/text.asp?2014/62/4/467/141291


Sir,

Peri-ictal pseudo-progression [PIPG] is a rare delayed sequela of cranial irradiation after brain tumors. It is a newly described phenomenon and less than 19 cases have been described, worldwide. [1],[2],[3],[4],[5] Herein, we present the first case of PIPG from India and review the literature on PIPG and other delayed post-radiation vasculopathies.

A 55-year-old man presented with difficulty in speaking. He had undergone complete resection of right frontal low grade glioma [WHO Grade II oligoastrocytoma] in 2008, followed by 27 fractions of radiotherapy [200cGy/#, total dose of 5400cGy]. Interval magnetic resonance imaging (MRI) scans had shown only focal encephalomalacia in the right frontal region. He was asymptomatic in the interim. Ten days prior to presentation he developed word finding difficulty and severe continuous right sided headache. He was admitted with history of four episodes of vomiting and a generalized seizure. Contrast MRI done outside was normal. Examination revealed global aphasia, left lower facial palsy and a left arm pronator drift. He had five more complex partial seizures in the hospital. Electroencephalograph (EEG) was normal. He was treated with intravenous anticonvulsants, levetiracetam, sodium valproate and phenytoin sodium. Repeat contrast magnetic resonance imaging (MRI) showed new changes of unilateral cortical thickening and gyriform hyperintensity in right frontal lobe, right insular cortex and transverse temporal gyri with borderline reduction in ADC. Patchy gyriform enhancement was seen involving the leptomeninges over the right fronto-temporal region [Figure 1]. Cerebrospinal fluid (CSF) examination was normal. The clinico-radiological picture was suggestive of PIPG and dexamethasone 24 mg/day was added to his regimen. He improved over 10 days and repeat contrast MRI after 2 weeks showed total resolution of contrast enhancement. After 14 days, he had only mild residual anomia.
Figure 1: Contrast MRI brain sections. Panels (a-c) post-op scans after 3 years show only residual gliotic cavity. Panels (d-f) show contrast enhancement of the uncus, opercular cortex and leptomeninges [arrows]. Panels (g-i) show resolution of contrast enhancement and return to prior status

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Peri-ictalpseudoprogression lies along a spectrum of delayed post-radiation vasculopathies (DPRV) occurring in survivors of brain tumors that include other entities such as SMART syndrome [stroke-like migraine attacks after radiation therapy], ALERT syndrome [acute late-onset encephalopathy after radiotherapy] and focal radionecrosis. Patients with a remote history of brain tumor and cranial irradiation present 3-20 years later with frequent partial seizures. [1] The EEG may show focal epileptiform discharges. The MRI focal cortical and/or leptomeningeal enhancing lesions. Leptomeningeal enhancement may differentiate it from post-ictal changes. SMART syndrome is a similar entity, however stroke like deficits and headaches are more frequent. [6] MRI often shows transient patchy gyriform enhancement and cortical swelling in the parieto-occipital area which evolves over 2-7 days and resolves in 4-6 weeks. [7] Nevertheless, both PIPG and SMART have many overlapping clinical features. ALERT syndrome is a recently described entity with steroid responsive alteration of sensorium and diffuse or multifocal brain dysfunction, after a remote history of whole-brain irradiation. [8] The MRI shows multiple bilateral areas of subcortical patchy enhancement or focal leptomeningeal enhancement. These 3 vasculopathies bear a pathophysiological similarities with the PRES syndrome [posterior reversible encephalopathy syndrome] [Table 1]. Other differential diagnosis includes recurrent tumor, ischemic stroke, leptomeningeal disease, cerebral venous thrombosis, duralarterio-venous fistula and infections. [6] Clinical improvement in PIPG occurs after treatment with steroids and anticonvulsants after a few weeks with radiological resolution over a period of 1-3 months. Relapses may occur in a stereotyped fashion. In conclusion, PIPG is an unusual delayed sequalae of radiation with distinctive clinical and radiological patterns that should help the clinician to immediately recognize it.
Table 1: Features of different radiation vasculopathies

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  References Top

1.Rheims S, Ricard D, van den Bent M, Taillandier L, Bourg V, Désestret V, et al. Peri-ictal pseudo progression in patients with brain tumor. Neuro Oncol 2011;13:775-82.  Back to cited text no. 1
    
2.Finn MA, Blumenthal DT, Salzman KL, Jensen RL. Transient postictal MRI changes in patients with brain tumors may mimic disease progression. Surg Neurol 2007;67:246-50.  Back to cited text no. 2
    
3.Hattingen E, Franz K, Pilatus U, Weidauer S, Lanfermann H. Postictal spectroscopy and imaging findings mimicking brain tumor recurrence. J Magn Reson Imaging 2006;24:226-30.  Back to cited text no. 3
    
4.Hormigo A, Liberato B, Lis E, DeAngelis LM. Nonconvulsive status epilepticus in patients with cancer: Imaging abnormalities. Arch Neurol 2004;61:362-5.  Back to cited text no. 4
    
5.Quan D, Hackney DB, Pruitt AA, Lenkinski RE, Cecil KM. Transient MRI enhancement in a patient with seizures and previously resected glioma: Use of MRS. Neurology 1999;53:211-3.  Back to cited text no. 5
    
6.Kerklaan JP, Lycklama á Nijeholt GJ, Wiggenraad RG, Berghuis B, Postma TJ, Taphoorn MJ. SMART syndrome: A late reversible complication after radiation therapy for brain tumours. J Neurol 2011;258:1098-104.  Back to cited text no. 6
    
7.Black DF, Morris JM, Lindell EP, Krecke KN, Worrell GA, Bartleson JD, et al. Stroke-like migraine attacks after radiation therapy (SMART) syndrome is not always completely reversible: A case series. AJNR Am J Neuroradiol 2013;34:2298-303.  Back to cited text no. 7
    
8.Di Stefano AL, Berzero G, Vitali P, Galimberti CA, Ducray F, Ceroni M, et al. Acute late-onset encephalopathy after radiotherapy: An unusual life-threatening complication. Neurology 2013;81:1014-7.  Back to cited text no. 8
    


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