[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
extracranial, while one‑third are intracranial. Improper regression of embryonic arteries seems to be the most probable explanation for VA fenestration.[4] However, some literature suggest that intracranial VA fenestration is more likely due to the persistence of fetal anastomotic vessels. Vertebral artery fenestrations are associated with increased risk of aneurysm formation at its location. According to Drapkin, symptomatic intracranial aneurysms were found in about 20% of patients with VA fenestration.[5] Such aneurysm when found is usual site for SAH. The high preponderance of aneurysm formation in the fenestration segment may be related to abnormal tunica media and hemodynamic stress to the vessel wall at the proximal site. There are many case reports supporting the increased risk of SAH in VA fenestration due to aneurysm formation. Our patient is unique as it is the first report where perimesencephalic nonaneurysmal SAH is associated with VA fenestration. This important DSA finding should not be overlooked because it may predispose the patient to pathological conditions like SAH.
Sunil Kumar, Trilochan Srivastava, Shankar Tejwani1 Departments of Neurology, and 1Radiology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1.
U‑King‑Im JM, Koo B, Trivedi RA, Higgins NJ, Tay KY, Cross JJ, et al. Current diagnostic approaches to subarachnoid haemorrhage. Eur Radiol 2005;15:1135‑47. 2. Parmar H, Sitoh YY, Hui F. Normal variants of the intracranial circulation demonstrated by MR angiography at 3T. Eur J Radiol 2005;56:220‑8. 3. Sim E, Vaccaro AR, Berzlanovich A, Thaler H, Ullrich CG. Fenestration of the extracranial vertebral artery: Review of the literature. Spine (Phila Pa 1976) 2001;26:E139‑42. 4. Ionete C, Omojola MF. MR angiographic demonstration of bilateral duplication of the extracranial vertebral artery: Unusual course and review of the literature. AJNR. Am J Neuroradiol 2006;27:1304‑6. 5. Drapkin AJ. The double lumen: A pathognomonic angiographic sign of arterial dissection? Neuroradiology 2000;42:203‑5.
Renal small cell carcinoma presenting with solitary lumbar intradural metastasis Sir, Primary neoplastic lesions constitute a small number of patients with cauda‑equina syndrome.[1] Metastasis with cauda‑equina syndrome is even rarer with only few cases reported in literature, mostly as a result of central nervous systemdrop metastases. This report presents a case of renal small cell carcinoma with solitary lumbar intradural metastases. A 40‑year‑old male presented with progressive sensorimotor deficit in both lower limbs of six months duration and urinary incontinence. Neurologic examination revealed lower motor neuron paralysis of both lower limbs with sacral anesthesia and sphincter dysfunction. Magnetic resonance imaging of lumbosacral spine revealed contrast enhancing intradural space occupying lesion extending from L4 to S2 [Figure 1]. Patient underwent L4 to S1 laminectomy and subtotal excision of the tumor. Tumor adherent to the nerve roots was left behind. Histopathological examination of the tumor revealed tumor cells arranged in sheets, moderatelypleomorphic, negative for PAS with and without diastase and negative for CD 99, suggestive of metastatic small cell carcinoma [Figures 2 and 3]. Post operative PET‑CT revealed a focus of intense FDG uptake in the lower pole of right kidney suggestive of primary malignant lesion [Figure 4]. Patient was given palliative radiotherapy andstarted on chemotherapy with cisplatin and irinotecan. There was gradual improvement in the power of lower limbs. However during the course of his second cycle of chemotherapy, he succumbed to his illness.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144493
Received: 27-09-2014 Review completed: 28-09-2014 Accepted: 28-09-2014 Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Figure 1: Contrast MRI LS Spine showing homogenously enhancing intradural lesion extending from L4 to S2
561
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Figure 2: Synaptophysin immunohistochemistry showing strong cytoplasmic positivity in tumor cells
Figure 3: Some of the tumor cells showing cytoplasmic CD56 positivity
spread from cerebral secondary sites.[2] It represents roughly 2-6% of all spinal metastases, [2] and can befound in 2% of cancer patients during autopsy.[2]In the majority the primary lesion in lung, followed by breast.[2] Intradural metastases are commonly thought tooriginate from cerebrospinal fluid (CSF) seeding. First, tumor cells are transferredto the brain (secondary drop metastasis), and then they enterthe CSF. This spreading causes either multifocal or diffuse infiltration of the leptomeninges and is known as leptomeningeal carcinomatosis. Finally, metastatic tumors arise in intradural extra‑medullary (IDEM) lesions (tertiary drop metastasis). In the literature review, the development of IDEM metastasis was divided into earlier and later cases. The delayed metastasis may be related with tertiary dropmetastasis. However, early metastasis could not be explained by tertiary drop hypothesis. It may also be due to concurrent tumors from cell rests of neurulation.[3] Primary renal small cell carcinomas are extremely rare with less than 30 cases reported in literature.[4] A review of patients with small cell carcinoma of the kidney revealed a median age at diagnosis of 62 years, women more commonly affected than men. The diagnosis of a small cell carcinoma, whether of pulmonary or extrapulmonary origin, can be established by light microscopic and immunohistochemical examination of tumor tissue. Extra pulmonary small cell carcinomas are invariably aggressive, with a tendency to develop early nodal and disseminated metastatic disease. Because of the unfavorable prognosis, multimodal therapy has been used increasingly to prolong life.[5] Chemotherapeutic regimens used in treating extrapulmonary small cell carcinomas are the same as those employed in small cell carcinomas of the lung. The severity of disease at diagnosis represents the most sensitive predictor of survival.[5] Early diagnosis is important as it reducessurgical invasion and helps adequate recovery from neurological sequelae. Surgical treatment of metastatic cancer involvingintraduralcompartment is associated with highmorbidity and mortalityaccording to the primary lesion. Surgical intervention is appropriatewhen there is diagnostic uncertainty or for healthy patientswith good quality of survival or for patients with progressive neurological deficit.
Figure 4: Whole body PET CT showing evidence of abnormal metabolism in the lower pole of right kidney suggestive of primary
Spinal metastases are predominantly extradural and rarely breach the dura and may be the first manifestation of malignancy in ~20%, mostly representing tertiary 562
Anirudh Srinivasan, Sivashanmugam Dhandapani, Debajyoti Chatterjee1, Vijai Simha2 Departments of Neurosurgery, 1Pathology, 2Radiotherapy, Post Graduate Institute of Medical Education and Research, Chandigarh, India E‑mail: [email protected] Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
References 1. 2. 3.
4. 5.
Dhandapani S, Anirudh S, Garg R, Vasishta RK, Vyas S. Subependymoma causing conus‑cauda syndrome: Cured by total excision. Neurol India 2013;61:675‑7. Grant R, Papadopoulos SM, Greenberg HS. Metastaticepidural spinal cord compression. Neurol Clin 1991;9:825‑41. Dhandapani S, Mehta VS, Sharma BS. "Horseshoe cord terminus" sans filum around a bone spur: a rare composite of faulty gastrulation with agenesis of secondary neurulation: Case report. J Neurosurg Pediatr 2013;12:411-3.. Si Q, Dancer J, Stanton ML, Tamboli P, Ro JY, Czerniak BA, et al. Small cell carcinoma of the kidney: A clinicopathologic study of 14 cases. Hum Pathol 2011;42:1792‑8. Masuda T, Oikawa H, Yashima A, Sugimura J, Okamoto T, Fujioka T. Renal small cell carcinoma (neuroendorine carcinoma) without features of transitional cell carcinoma. Pathol Int 1998;48:412‑5. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144494
Received: 11-09-2014 Review completed: 14-09-2014 Accepted: 28-09-2014
Corpus callosal hemorrhage due to deep cerebral venous sinus thrombosis Sir, Hemorrhage in corpus callosum (CCH) (largest association fiber) is rare and is it is usually due to trauma, hypertension arteriovenous malformation and neoplasm.[1,2] Deep cerebral venous thrombosis (DCVT) producing CCH is a rare entity with only one case noted so far.[3] We report two patients of CCH seen in study of 63 DCVT patients admitted at stroke ward of our institute from January 2008 till December 2013. The institute ethical board approved the study protocol and informed consent was taken. Case 1: A 33 year old female presented with ten days history of progressively increasing holocranial headache and two episodes of generalized seizures with no other significant history. Her general physical examination and neurological examination including fundus were normal. Computerized tomographic (CT) scan of the brain showed hemorrhage in the genu of corpus callosum [Figure 1a]; with contrast scan showing filling defects in internal cerebral veins, vein of Galen and straight sinus [Figure 1b]. Her Magnetic Resonance Imaging (MRI) of the brain Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
a
b
c
d
Figure 1: In Patient No. 1: CT scan of brain plain shows hemorrhage in the genu of corpus callosum (a), contrast scan showing filling defect in vein of Galen (b). Arrows pointing to the abnormalities.: In Patient No. 2: (c) – Hemorrhage in the genu of corpus callosum (white arrow), hyperdense bilateral internal cerebral veins (Black arrow) (d)- Filling defect in Vein of Galen (white arrow)
done four days later [Figure 2a‑f] showed additional parenchymal lesions with better visualisation of the hemorrhage. Re‑examination for the presence of any disconnection syndrome and parietal lobe dysfunction was noncontributory. She was also evaluated for risk factors for venous thrombosis including procagulant work up, which were negetive. She was managed with subcutaneous heparin, antiedema measures and later oral anticoagulants for two years. She is asymptomatic from last three years. Case 2: A 50 year old female was brought with five days history of generalized headache and two days history of drowsiness with no other significant history. Examination showed pallor; neurologically she was in altered sensorium with left hemiparesis. Investigations showed microcytic hypochromic anemia (Hb 7.5 gm%), with other work up being negative. Her CT brain showed hypodensities in the bilateral thalami, internal capsule and left basal ganglia and hemorrhage in the genu of the corpus callosum [Figure 1c] hyperdense bilateral internal cerebral veins with filling defect in contrast [Figure 1d]. MRI Brain showed thrombosis of deep venous system and left transverse sinus along with corpus colossal hemorrhage which was well appreciated on sequential weighted images [Figure 3]. She was managed with subcutaneous heparin followed by oral anticoagulation, antiedema measures and anemia was corrected. She improved subsequently and two years in to her follow up she is asymptomatic. In the present study of 63 DCVT patients, we had two patients (3.2%) with CCH. These patients presented with either headache, seizures due to associated cortical lesions or encephalopathy resulting from the 563
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.
extracranial, while one‑third are intracranial. Improper regression of embryonic arteries seems to be the most probable explanation for VA fenestration.[4] However, some literature suggest that intracranial VA fenestration is more likely due to the persistence of fetal anastomotic vessels. Vertebral artery fenestrations are associated with increased risk of aneurysm formation at its location. According to Drapkin, symptomatic intracranial aneurysms were found in about 20% of patients with VA fenestration.[5] Such aneurysm when found is usual site for SAH. The high preponderance of aneurysm formation in the fenestration segment may be related to abnormal tunica media and hemodynamic stress to the vessel wall at the proximal site. There are many case reports supporting the increased risk of SAH in VA fenestration due to aneurysm formation. Our patient is unique as it is the first report where perimesencephalic nonaneurysmal SAH is associated with VA fenestration. This important DSA finding should not be overlooked because it may predispose the patient to pathological conditions like SAH.
Sunil Kumar, Trilochan Srivastava, Shankar Tejwani1 Departments of Neurology, and 1Radiology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1.
U‑King‑Im JM, Koo B, Trivedi RA, Higgins NJ, Tay KY, Cross JJ, et al. Current diagnostic approaches to subarachnoid haemorrhage. Eur Radiol 2005;15:1135‑47. 2. Parmar H, Sitoh YY, Hui F. Normal variants of the intracranial circulation demonstrated by MR angiography at 3T. Eur J Radiol 2005;56:220‑8. 3. Sim E, Vaccaro AR, Berzlanovich A, Thaler H, Ullrich CG. Fenestration of the extracranial vertebral artery: Review of the literature. Spine (Phila Pa 1976) 2001;26:E139‑42. 4. Ionete C, Omojola MF. MR angiographic demonstration of bilateral duplication of the extracranial vertebral artery: Unusual course and review of the literature. AJNR. Am J Neuroradiol 2006;27:1304‑6. 5. Drapkin AJ. The double lumen: A pathognomonic angiographic sign of arterial dissection? Neuroradiology 2000;42:203‑5.
Renal small cell carcinoma presenting with solitary lumbar intradural metastasis Sir, Primary neoplastic lesions constitute a small number of patients with cauda‑equina syndrome.[1] Metastasis with cauda‑equina syndrome is even rarer with only few cases reported in literature, mostly as a result of central nervous systemdrop metastases. This report presents a case of renal small cell carcinoma with solitary lumbar intradural metastases. A 40‑year‑old male presented with progressive sensorimotor deficit in both lower limbs of six months duration and urinary incontinence. Neurologic examination revealed lower motor neuron paralysis of both lower limbs with sacral anesthesia and sphincter dysfunction. Magnetic resonance imaging of lumbosacral spine revealed contrast enhancing intradural space occupying lesion extending from L4 to S2 [Figure 1]. Patient underwent L4 to S1 laminectomy and subtotal excision of the tumor. Tumor adherent to the nerve roots was left behind. Histopathological examination of the tumor revealed tumor cells arranged in sheets, moderatelypleomorphic, negative for PAS with and without diastase and negative for CD 99, suggestive of metastatic small cell carcinoma [Figures 2 and 3]. Post operative PET‑CT revealed a focus of intense FDG uptake in the lower pole of right kidney suggestive of primary malignant lesion [Figure 4]. Patient was given palliative radiotherapy andstarted on chemotherapy with cisplatin and irinotecan. There was gradual improvement in the power of lower limbs. However during the course of his second cycle of chemotherapy, he succumbed to his illness.
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144493
Received: 27-09-2014 Review completed: 28-09-2014 Accepted: 28-09-2014 Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Figure 1: Contrast MRI LS Spine showing homogenously enhancing intradural lesion extending from L4 to S2
561
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
Figure 2: Synaptophysin immunohistochemistry showing strong cytoplasmic positivity in tumor cells
Figure 3: Some of the tumor cells showing cytoplasmic CD56 positivity
spread from cerebral secondary sites.[2] It represents roughly 2-6% of all spinal metastases, [2] and can befound in 2% of cancer patients during autopsy.[2]In the majority the primary lesion in lung, followed by breast.[2] Intradural metastases are commonly thought tooriginate from cerebrospinal fluid (CSF) seeding. First, tumor cells are transferredto the brain (secondary drop metastasis), and then they enterthe CSF. This spreading causes either multifocal or diffuse infiltration of the leptomeninges and is known as leptomeningeal carcinomatosis. Finally, metastatic tumors arise in intradural extra‑medullary (IDEM) lesions (tertiary drop metastasis). In the literature review, the development of IDEM metastasis was divided into earlier and later cases. The delayed metastasis may be related with tertiary dropmetastasis. However, early metastasis could not be explained by tertiary drop hypothesis. It may also be due to concurrent tumors from cell rests of neurulation.[3] Primary renal small cell carcinomas are extremely rare with less than 30 cases reported in literature.[4] A review of patients with small cell carcinoma of the kidney revealed a median age at diagnosis of 62 years, women more commonly affected than men. The diagnosis of a small cell carcinoma, whether of pulmonary or extrapulmonary origin, can be established by light microscopic and immunohistochemical examination of tumor tissue. Extra pulmonary small cell carcinomas are invariably aggressive, with a tendency to develop early nodal and disseminated metastatic disease. Because of the unfavorable prognosis, multimodal therapy has been used increasingly to prolong life.[5] Chemotherapeutic regimens used in treating extrapulmonary small cell carcinomas are the same as those employed in small cell carcinomas of the lung. The severity of disease at diagnosis represents the most sensitive predictor of survival.[5] Early diagnosis is important as it reducessurgical invasion and helps adequate recovery from neurological sequelae. Surgical treatment of metastatic cancer involvingintraduralcompartment is associated with highmorbidity and mortalityaccording to the primary lesion. Surgical intervention is appropriatewhen there is diagnostic uncertainty or for healthy patientswith good quality of survival or for patients with progressive neurological deficit.
Figure 4: Whole body PET CT showing evidence of abnormal metabolism in the lower pole of right kidney suggestive of primary
Spinal metastases are predominantly extradural and rarely breach the dura and may be the first manifestation of malignancy in ~20%, mostly representing tertiary 562
Anirudh Srinivasan, Sivashanmugam Dhandapani, Debajyoti Chatterjee1, Vijai Simha2 Departments of Neurosurgery, 1Pathology, 2Radiotherapy, Post Graduate Institute of Medical Education and Research, Chandigarh, India E‑mail: [email protected] Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
References 1. 2. 3.
4. 5.
Dhandapani S, Anirudh S, Garg R, Vasishta RK, Vyas S. Subependymoma causing conus‑cauda syndrome: Cured by total excision. Neurol India 2013;61:675‑7. Grant R, Papadopoulos SM, Greenberg HS. Metastaticepidural spinal cord compression. Neurol Clin 1991;9:825‑41. Dhandapani S, Mehta VS, Sharma BS. "Horseshoe cord terminus" sans filum around a bone spur: a rare composite of faulty gastrulation with agenesis of secondary neurulation: Case report. J Neurosurg Pediatr 2013;12:411-3.. Si Q, Dancer J, Stanton ML, Tamboli P, Ro JY, Czerniak BA, et al. Small cell carcinoma of the kidney: A clinicopathologic study of 14 cases. Hum Pathol 2011;42:1792‑8. Masuda T, Oikawa H, Yashima A, Sugimura J, Okamoto T, Fujioka T. Renal small cell carcinoma (neuroendorine carcinoma) without features of transitional cell carcinoma. Pathol Int 1998;48:412‑5. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144494
Received: 11-09-2014 Review completed: 14-09-2014 Accepted: 28-09-2014
Corpus callosal hemorrhage due to deep cerebral venous sinus thrombosis Sir, Hemorrhage in corpus callosum (CCH) (largest association fiber) is rare and is it is usually due to trauma, hypertension arteriovenous malformation and neoplasm.[1,2] Deep cerebral venous thrombosis (DCVT) producing CCH is a rare entity with only one case noted so far.[3] We report two patients of CCH seen in study of 63 DCVT patients admitted at stroke ward of our institute from January 2008 till December 2013. The institute ethical board approved the study protocol and informed consent was taken. Case 1: A 33 year old female presented with ten days history of progressively increasing holocranial headache and two episodes of generalized seizures with no other significant history. Her general physical examination and neurological examination including fundus were normal. Computerized tomographic (CT) scan of the brain showed hemorrhage in the genu of corpus callosum [Figure 1a]; with contrast scan showing filling defects in internal cerebral veins, vein of Galen and straight sinus [Figure 1b]. Her Magnetic Resonance Imaging (MRI) of the brain Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
a
b
c
d
Figure 1: In Patient No. 1: CT scan of brain plain shows hemorrhage in the genu of corpus callosum (a), contrast scan showing filling defect in vein of Galen (b). Arrows pointing to the abnormalities.: In Patient No. 2: (c) – Hemorrhage in the genu of corpus callosum (white arrow), hyperdense bilateral internal cerebral veins (Black arrow) (d)- Filling defect in Vein of Galen (white arrow)
done four days later [Figure 2a‑f] showed additional parenchymal lesions with better visualisation of the hemorrhage. Re‑examination for the presence of any disconnection syndrome and parietal lobe dysfunction was noncontributory. She was also evaluated for risk factors for venous thrombosis including procagulant work up, which were negetive. She was managed with subcutaneous heparin, antiedema measures and later oral anticoagulants for two years. She is asymptomatic from last three years. Case 2: A 50 year old female was brought with five days history of generalized headache and two days history of drowsiness with no other significant history. Examination showed pallor; neurologically she was in altered sensorium with left hemiparesis. Investigations showed microcytic hypochromic anemia (Hb 7.5 gm%), with other work up being negative. Her CT brain showed hypodensities in the bilateral thalami, internal capsule and left basal ganglia and hemorrhage in the genu of the corpus callosum [Figure 1c] hyperdense bilateral internal cerebral veins with filling defect in contrast [Figure 1d]. MRI Brain showed thrombosis of deep venous system and left transverse sinus along with corpus colossal hemorrhage which was well appreciated on sequential weighted images [Figure 3]. She was managed with subcutaneous heparin followed by oral anticoagulation, antiedema measures and anemia was corrected. She improved subsequently and two years in to her follow up she is asymptomatic. In the present study of 63 DCVT patients, we had two patients (3.2%) with CCH. These patients presented with either headache, seizures due to associated cortical lesions or encephalopathy resulting from the 563
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.
