[Downloaded free from http://www.neurologyindia.com on Thursday, September 25, 2014, IP:]  ||  Click here to download free Android application for this journal Letters to Editor

in this report suggest that malignant cell infiltration of the peripheral nerve may be the presenting feature of lymphoreticular malignancy and should be included in the diagnostic algorithm of peripheral neuropathy.

Meena Angamuthu Kannan, Megha Shantiveer Uppin1, Sheik Afshan Jabeen, Rukmini K. Mridula, Sundaram Challa1, Rupam Borgohain Departments of Neurology and 1Pathology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad, India

E‑mail: [email protected]

References 1. Ramchandren S, Dalmau J. Metastases to the peripheral nervous system. J Neurooncol 2005;75:101‑10. 2. Aregawi DG, Sherman JH, Douvas MG, Burns TM, Schiff D. Neuroleukemiosis: Case report of leukemic nerve infiltration in acute lymphoblastic leukemia. Muscle Nerve 2008;38:1196‑200. 3. Reddy CG, Mauermann ML, Solomon BM, Ringler MD, Jerath NU, Begna KH, et al. Neuroleukemiosis: An unusual cause of peripheral neuropathy. Leuk Lymphoma 2012;53:2405‑11. 4. Hughes R, Sharrack B, Rubens R. Carcinoma and the peripheral nervous system. J Neurol 1996;243:371‑6. 5. Denier C, Lozeron P, Adams D, Decaudin D, Isnard‑Grivaux F, Lacroix C, et al. Multifocal neuropathy due to plasma cell infiltration of peripheral nerves in multiple myeloma. Neurology 2006;66:917‑8. Access this article online Quick Response Code:

Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141288

Received: 13‑02‑2014 Review completed: 09‑03‑2014 Accepted: 27‑06‑2014

Peri‑ictal pseudoprogression [pipg] in an operated case of right frontal glioma; case report and review of literature 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.[1‑5] Herein, we present the first case of PIPG from India and review Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4

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. 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 461

[Downloaded free from http://www.neurologyindia.com on Thursday, September 25, 2014, IP:]  ||  Click here to download free Android application for this journal Letters to Editor










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

Table 1: Features of different radiation vasculopathies




Incidence Clinical features

Rare Seizures [partial] predominate

Rare (only 5 cases reported) Focal neurological deficits, Stupor, coma

MRI findings

Focal cortical and/or leptomeningeal enhancing lesions Superficial lesions Steroids, anticonvulsants

Rare Focal neurological deficits Migraine like headache Seizures rare Diffuse unilateral cortical gadolinium enhancement of the cerebral gyri especially over the parieto‑occipital cortex Leptomeningeal enhancement rare Steroids, anticonvulsants, Verapamil


Unilateral or bilateral multiple subcortical patchily enhancing lesions Steroids, Anticonvulsants

SMART - Stroke-like migraine attacks after radiation therapy, PIPG - Peri-ictal pseudo-progression, ALERT syndrome - Acute late-onset encephalopathy after radiotherapy

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.

Boby Varkey Maramattom, Dilip Panikar1 Departments of Neurology, and 1Neurosurgery, Aster Medcity, Cheranelloor, Kochi, Kerala, India E-mail: [email protected] 462

References 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. 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. 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. 4. Hormigo A, Liberato B, Lis E, DeAngelis LM. Nonconvulsive status epilepticus in patients with cancer: Imaging abnormalities. Arch Neurol 2004;61:362‑5. 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. 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. 7. Black DF, Morris JM, Lindell EP, Krecke KN, Worrell GA, Bartleson JD, Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4

[Downloaded free from http://www.neurologyindia.com on Thursday, September 25, 2014, IP:]  ||  Click here to download free Android application fo this journal Letters to Editor


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. 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. Access this article online Quick Response Code:

Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.141291

Received: 10-07-2014 Review completed: 13-07-2014 Accepted: 19-08-2014

Vasospasm causing reversible cortical deafness in subarachnoid hemorrhage Sir, A 32‑year‑old male presented with sudden onset of holocranial severe headache of 2‑day duration. Magnetic resonance imaging (MRI) of brain showed subarachnoid hemorrhage (SAH) in the left sylvian fissure [Figure 1a‑d]. Cerebral computed tomographic angiography (CTA) revealed an aneurysm at bifurcation of the left‑middle cerebral artery [Figure 1e]. He was advised surgery but he did not turn up for five days due to some family issues. On the eighth day, he developed bilateral deafness of sudden onset. Pure tone audiometry (PTA) confirmed presence of bilateral sensorineural hearing loss [Figure 2a]. Brainstem auditory evoked potentials demonstrated normal patterns bilaterally. Transcranial doppler (TSD) showed elevated blood flow velocities (~280 cm/s) involving both middle cerebral arteries. Patient refused surgery in that hospital due to economic constraints and got admitted to our facility. Neurologic examination revealed bilateral sensorineural hearing loss. Patient had left pterional craniotomy and clipping of the aneurysm. Patient improved in sensorium and deafness after five days of surgery. Postoperative pure tone audiometry confirmed resolution of the sensorineural deafness [Figure 2b]. At follow‑up of 12 months the patient is doing well. Severe auditory deficit due to bilateral cerebral lesions is called as “cortical deafness”. In cortical deafness, auditory signals cannot be perceived in the cortex and audiometry reveals severe bilateral hearing loss. It results from damage to both temporal or temporoparietal lobes including the primary auditory cortex (Broadmann areas Neurology India | Jul-Aug 2014 | Vol 62 | Issue 4

41 and 42) on both transverse gyri (Heschl). The clinical syndrome of cortical deafness with bitemporal infarction was described by Wernicke and Friedlander in 1883.[1] Different causes of cortical deafness include congenital lesions, cerebral infarction or cerebral hemorrhage.[1] We could find only four cases of cortical deafness caused by vasospasm in subarachnoid hemorrhage [Table 1]. Peripheral hearing loss due to aneurysmal bleed is most commonly caused by antero‑inferior cerebellar artery (AICA) aneurysm [Table 2]. Rhoton divided AICA in to four segments  ‑  anterior pontine, lateral pontine, floculonodular, and cortical. The lateral pontine segment is divided into premeatal, meatal, and postmeatal parts. Mazzoni found the meatal segment was medial to porus in 33%, reaching the porus in 27% and entered the canal in 40%. All the AICA aneurysm causing deafness belong to the latter group.[4]






Figure 1: T1 weighted axial image (a), FLAIR image (b), GRE sequence (c and d) of MRI showing blood in the left sylvain fissure. (e) CT angiography of brain showing aneurysm at bifurcation of left middle cerebral artery


b Figure 2: (a) Preoperative pure tone audiogram (PTA) of patient suggestive of bilateral sensorineural hearing loss. (b) Postoperative PTA of patient showing normal findings


Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.

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

Peri-ictal pseudoprogression [pipg] in an operated case of right frontal glioma; case report and review of literature. - PDF Download Free
1MB Sizes 0 Downloads 3 Views