Letters to Editor
PSEL should be suspected in patients with spinal cord compression with imaging showing isointense, homogeneous extradural contiguous level compressive soft‑tissue lesion without bony involvement and previous history of cancer or history suggestive of any systemic involvement.
Sujit Abajirao Jagtap, Akshay S. Patil1, C. Kesavdas2, N. Radhakrishnan, Himanshu Soni, Kandraju Sai Satish Departments of Neurology, 1Neurosurgery, 2Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India E‑mail: [email protected]
References 1.
Cugati G, Singh M, Pande A, Ramamurthi R, Balasubramanyam M, Sethi SK, et al. Primary spinal epidural lymphomas. J Craniovertebr Junction Spine 2011;2:3‑11. 2. Mora J, Wollner N. Primary epidural non‑Hodgkin lymphoma: Spinal cord compression syndrome as the initial form of presentation in childhood non‑Hodgkin lymphoma. Med Pediatr Oncol 1999;32:102‑5. 3. Haque S, Law M, Abrey LE, Young RJ. Imaging of lymphoma of the central nervous system, spine, and orbit. Radiol Clin North Am 2008;46:339‑61, ix. 4. Monnard V, Sun A, Epelbaum R, Poortmans P, Miller RC, Verschueren T, et al. Primary spinal epidural lymphoma: Patients’ profile, outcome, and prognostic factors: A multicenter rare cancer network study. Int J Radiat Oncol Biol Phys 2006;65:817‑23. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: ***
syndromes are well described with CRMP‑5. We describe the first case of CRMP‑5 associated basal ganglionitis and limbic encephalitis in an Indian lady with a primary lung tumor. A 66‑year‑old housewife presented with a 3 month history of abnormal behavior, logorrhea, and visual and olfactory hallucinations. She was initially evaluated by a psychiatrist for psychosis and was started on atypical neuroleptics. During our evaluation, she also complained of patchy numbness in her arms, right hip, right side of the face and tongue, and recent memory impairment. She had recently lost 12 kg of weight. On examination, she scored 19/30 on the mini mental scale examination. She had mild choreic movements involving limbs and tongue. She had impaired position and vibration sense till her ankles bilaterally with diminished reflexes in lower limb. Plantar reflexes were flexor. She also had a wide based gait, but no Rhomberg’s sign or cerebellar signs. Routine blood tests, X‑ray chest, and ultrasound abdomen were normal. A repeat magnetic resonance imaging (MRI) brain showed a symmetric T2/fluid attenuated inversion recovery (FLAIR) hyperintensity of the putamina and caudate heads and bodies without contrast enhancement as well as bilateral mesial temporal hyperintensities on FLAIR images [Figure 1]. Cerebrospinal fluid examination showed an opening pressure of 15.5 cm of water, 6 WBCs, glucose of 62 mg, and protein of 39 mg. Polymerase chain reaction (PCR) for John Cunningham (JC) virus, acid‑fast Bacillus (AFB) stains, bacterial serology, and cytology for malignant cells were negative. Vasculitic work up and urine for heavy metals were negative. Nerve conduction studies showed low amplitude compound motor
DOI: 10.4103/0028-3886.121940
Received: 18‑07‑2013 Review completed: 03‑09‑2013 Accepted: 20‑10‑2013
Paraneoplastic CRMP‑5 basal ganglionitis and limbic encephalitis in an elderly Indian lady Sir, Collapsin response mediator proteins (CRMP) are a group of five phosphoproteins that play a vital role in neurite outgrowth and neuronal polarity.[1] Anti‑CRMP‑5 antibodies react with a 66 kDa protein found in the brain and the peripheral nerve.[2] Of these, paraneoplastic 534
a
d
c
b
e
f
Figure 1: Magnetic resonance (MR) images. Panel (a and b) T1WI plain and contrast MRI are normal. Panel (c and d) T2WI and fluid attenuated inversion recovery (FLAIR) images show bilateral symmetrical basal ganglia hyperintensities. Panel (e) FLAIR image shows bilateral mesial temporal hyperintensities. Panel (f) diffusion weighted image is normal
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Letters to Editor
action potentials (CMAPs) with normal velocities and normal F‑wave latencies. Needle examination showed polyphasic, short duration potentials, and scattered fibrillation potentials. An electroencephalogram (EEG) was normal. Computed tomography (CT) chest and abdomen were normal. An 18‑F fluoro‑deoxyglucose positron emission tomography (FDG‑18 PET) scan demonstrated multiple foci of increased FDG‑18 uptake in mildly enlarged lymph nodes within the mediastinum consistent with malignancy. The family was unwilling for further measures and she was discharged to domiciliary care with 40 mg/day of oral prednisolone. At 1 month, results of serum antibodies were positive for CRMP‑5 (Oxford University, Immunology lab). Other antibodies including leucine‑rich glioma inactivated 1 (LGi1), contactin associated protein‑2 (CASPR2), Hu, Ri, Yo, Tr, Ma/Tr, glutamic acid decarboxylase (GAD), amphiphysin, aquaporin‑4 (Aqp4), α-amino-3-hydroxy5-methyl-4-isoxazolepropionic acid (AMPA), and N-methyl-D-aspartate (NMDA) were negative. After 4 months, she expired at a local hospital of bronchogenic carcinoma. CRMP‑5 immunoglobulin (IgG) antibodies have been described predominantly in association with small cell lung carcinoma (SCLC; 77%) and thymoma (5%).[3] Paraneoplastic neurological associations include limbic encephalitis, chorea, cerebellar ataxia, myelopathy, radiculopathy, neuropathy, optic neuropathy, uveoretinal syndromes, myasthenia gravis, and Lambert‑Eaton syndrome. [4] Late‑onset chorea with basal ganglia hyperintensities may signal CRMP‑5 encephalitis.[5,6] Our patient had a primary lung tumor. Plasmapheresis, intravenous Ig (IVIg), corticosteroids have been used in the treatment, but the most effective treatment is eradication of the underlying tumor. In conclusion, we present probably the first case of paraneoplastic CRMP‑5 related basal ganglionitis and limbic encephalitis in an Indian lady.
Boby Varkey Maramattom Department of Neurology, Lourdes Hospital, Pachalam, Kochi, Kerala, India E‑mail: [email protected]
References 1. Brot S, Rogemond V, Perrot V, Chounlamountri N, Auger C, Honnorat J, et al. CRMP5 interacts with tubulin to inhibit neurite outgrowth, thereby modulating the function of CRMP2. J Neurosci 2010;30:10639‑54. 2. Stich O, Rauer S. Antigen‑specific oligoclonal bands in cerebrospinal fluid and serum from patients with anti‑amphiphysin‑ and anti‑CV2/ CRMP5 associated paraneoplastic neurological syndromes. Eur J Neurol 2007;14:650‑3. 3. Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP‑5 neuronal autoantibody: Marker of lung cancer and Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
thymoma‑related autoimmunity. Ann Neurol 2001;49:146‑54. 4. McKeon A, Pittock SJ. Paraneoplastic encephalomyelopathies: Pathology and mechanisms. Acta Neuropathol 2011;122:381‑400. 5. Kinirons P, Fulton A, Keoghan M, Brennan P, Farrell MA, Moroney JT. Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP‑5 neuronal antibody. Neurology 2003;61:1623‑4. 6. O'Toole O, Lennon VA, Ahlskog JE, Matsumoto JY, Pittock SJ, Bower J, et al. Autoimmune chorea in adults. Neurology 2013;80:1133‑44. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121941
Received: 22‑07‑2013 Review completed: 31‑07‑2013 Accepted: 13‑10‑2013
An unusual case of reversible cerebral vasoconstriction syndrome presenting with a large intraparenchymal haematoma Sir, Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by transient reversible multifocal arterial constrictions.[1,2] Severe headache is the characteristic presenting feature and the imaging findings include: infarcts, convexity subarachnoid hemorrhage (SAH), vasogenic edema, and sometimes intracerebral and subdural hemorrhage.[1‑5] Incidence of intracranial hemorrhage was 34% with SAH being the most common in a large study.[3] Large parenchymal hemorrhage is uncommon and usually requires cerebral angiography to rule out a vascular malformation. Furthermore, diffuse multisegmental vasoconstriction on angiogram is often mistaken for vasculitis.[6,7] We report a case of RCVS without any precipitating factor in a 43‑year‑old lady who presented acutely with a large intracerebral hematoma and in whom the digital substraction angiography (DSA) showed a reversible, multisegmental, diffuse vasoconstriction of the cerebral vessels. A 43‑year‑old lady presented with severe headache and sudden onset of right side weakness. She had mild holocranial headache for 3 days prior to this event. There was no history of use of any vasoactive substance or any other medication. Vitals were normal. Neurologic examination revealed Glasgow coma 535
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.
PSEL should be suspected in patients with spinal cord compression with imaging showing isointense, homogeneous extradural contiguous level compressive soft‑tissue lesion without bony involvement and previous history of cancer or history suggestive of any systemic involvement.
Sujit Abajirao Jagtap, Akshay S. Patil1, C. Kesavdas2, N. Radhakrishnan, Himanshu Soni, Kandraju Sai Satish Departments of Neurology, 1Neurosurgery, 2Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India E‑mail: [email protected]
References 1.
Cugati G, Singh M, Pande A, Ramamurthi R, Balasubramanyam M, Sethi SK, et al. Primary spinal epidural lymphomas. J Craniovertebr Junction Spine 2011;2:3‑11. 2. Mora J, Wollner N. Primary epidural non‑Hodgkin lymphoma: Spinal cord compression syndrome as the initial form of presentation in childhood non‑Hodgkin lymphoma. Med Pediatr Oncol 1999;32:102‑5. 3. Haque S, Law M, Abrey LE, Young RJ. Imaging of lymphoma of the central nervous system, spine, and orbit. Radiol Clin North Am 2008;46:339‑61, ix. 4. Monnard V, Sun A, Epelbaum R, Poortmans P, Miller RC, Verschueren T, et al. Primary spinal epidural lymphoma: Patients’ profile, outcome, and prognostic factors: A multicenter rare cancer network study. Int J Radiat Oncol Biol Phys 2006;65:817‑23. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: ***
syndromes are well described with CRMP‑5. We describe the first case of CRMP‑5 associated basal ganglionitis and limbic encephalitis in an Indian lady with a primary lung tumor. A 66‑year‑old housewife presented with a 3 month history of abnormal behavior, logorrhea, and visual and olfactory hallucinations. She was initially evaluated by a psychiatrist for psychosis and was started on atypical neuroleptics. During our evaluation, she also complained of patchy numbness in her arms, right hip, right side of the face and tongue, and recent memory impairment. She had recently lost 12 kg of weight. On examination, she scored 19/30 on the mini mental scale examination. She had mild choreic movements involving limbs and tongue. She had impaired position and vibration sense till her ankles bilaterally with diminished reflexes in lower limb. Plantar reflexes were flexor. She also had a wide based gait, but no Rhomberg’s sign or cerebellar signs. Routine blood tests, X‑ray chest, and ultrasound abdomen were normal. A repeat magnetic resonance imaging (MRI) brain showed a symmetric T2/fluid attenuated inversion recovery (FLAIR) hyperintensity of the putamina and caudate heads and bodies without contrast enhancement as well as bilateral mesial temporal hyperintensities on FLAIR images [Figure 1]. Cerebrospinal fluid examination showed an opening pressure of 15.5 cm of water, 6 WBCs, glucose of 62 mg, and protein of 39 mg. Polymerase chain reaction (PCR) for John Cunningham (JC) virus, acid‑fast Bacillus (AFB) stains, bacterial serology, and cytology for malignant cells were negative. Vasculitic work up and urine for heavy metals were negative. Nerve conduction studies showed low amplitude compound motor
DOI: 10.4103/0028-3886.121940
Received: 18‑07‑2013 Review completed: 03‑09‑2013 Accepted: 20‑10‑2013
Paraneoplastic CRMP‑5 basal ganglionitis and limbic encephalitis in an elderly Indian lady Sir, Collapsin response mediator proteins (CRMP) are a group of five phosphoproteins that play a vital role in neurite outgrowth and neuronal polarity.[1] Anti‑CRMP‑5 antibodies react with a 66 kDa protein found in the brain and the peripheral nerve.[2] Of these, paraneoplastic 534
a
d
c
b
e
f
Figure 1: Magnetic resonance (MR) images. Panel (a and b) T1WI plain and contrast MRI are normal. Panel (c and d) T2WI and fluid attenuated inversion recovery (FLAIR) images show bilateral symmetrical basal ganglia hyperintensities. Panel (e) FLAIR image shows bilateral mesial temporal hyperintensities. Panel (f) diffusion weighted image is normal
Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
Letters to Editor
action potentials (CMAPs) with normal velocities and normal F‑wave latencies. Needle examination showed polyphasic, short duration potentials, and scattered fibrillation potentials. An electroencephalogram (EEG) was normal. Computed tomography (CT) chest and abdomen were normal. An 18‑F fluoro‑deoxyglucose positron emission tomography (FDG‑18 PET) scan demonstrated multiple foci of increased FDG‑18 uptake in mildly enlarged lymph nodes within the mediastinum consistent with malignancy. The family was unwilling for further measures and she was discharged to domiciliary care with 40 mg/day of oral prednisolone. At 1 month, results of serum antibodies were positive for CRMP‑5 (Oxford University, Immunology lab). Other antibodies including leucine‑rich glioma inactivated 1 (LGi1), contactin associated protein‑2 (CASPR2), Hu, Ri, Yo, Tr, Ma/Tr, glutamic acid decarboxylase (GAD), amphiphysin, aquaporin‑4 (Aqp4), α-amino-3-hydroxy5-methyl-4-isoxazolepropionic acid (AMPA), and N-methyl-D-aspartate (NMDA) were negative. After 4 months, she expired at a local hospital of bronchogenic carcinoma. CRMP‑5 immunoglobulin (IgG) antibodies have been described predominantly in association with small cell lung carcinoma (SCLC; 77%) and thymoma (5%).[3] Paraneoplastic neurological associations include limbic encephalitis, chorea, cerebellar ataxia, myelopathy, radiculopathy, neuropathy, optic neuropathy, uveoretinal syndromes, myasthenia gravis, and Lambert‑Eaton syndrome. [4] Late‑onset chorea with basal ganglia hyperintensities may signal CRMP‑5 encephalitis.[5,6] Our patient had a primary lung tumor. Plasmapheresis, intravenous Ig (IVIg), corticosteroids have been used in the treatment, but the most effective treatment is eradication of the underlying tumor. In conclusion, we present probably the first case of paraneoplastic CRMP‑5 related basal ganglionitis and limbic encephalitis in an Indian lady.
Boby Varkey Maramattom Department of Neurology, Lourdes Hospital, Pachalam, Kochi, Kerala, India E‑mail: [email protected]
References 1. Brot S, Rogemond V, Perrot V, Chounlamountri N, Auger C, Honnorat J, et al. CRMP5 interacts with tubulin to inhibit neurite outgrowth, thereby modulating the function of CRMP2. J Neurosci 2010;30:10639‑54. 2. Stich O, Rauer S. Antigen‑specific oligoclonal bands in cerebrospinal fluid and serum from patients with anti‑amphiphysin‑ and anti‑CV2/ CRMP5 associated paraneoplastic neurological syndromes. Eur J Neurol 2007;14:650‑3. 3. Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP‑5 neuronal autoantibody: Marker of lung cancer and Neurology India | Sep-Oct 2013 | Vol 61 | Issue 5
thymoma‑related autoimmunity. Ann Neurol 2001;49:146‑54. 4. McKeon A, Pittock SJ. Paraneoplastic encephalomyelopathies: Pathology and mechanisms. Acta Neuropathol 2011;122:381‑400. 5. Kinirons P, Fulton A, Keoghan M, Brennan P, Farrell MA, Moroney JT. Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP‑5 neuronal antibody. Neurology 2003;61:1623‑4. 6. O'Toole O, Lennon VA, Ahlskog JE, Matsumoto JY, Pittock SJ, Bower J, et al. Autoimmune chorea in adults. Neurology 2013;80:1133‑44. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.121941
Received: 22‑07‑2013 Review completed: 31‑07‑2013 Accepted: 13‑10‑2013
An unusual case of reversible cerebral vasoconstriction syndrome presenting with a large intraparenchymal haematoma Sir, Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by transient reversible multifocal arterial constrictions.[1,2] Severe headache is the characteristic presenting feature and the imaging findings include: infarcts, convexity subarachnoid hemorrhage (SAH), vasogenic edema, and sometimes intracerebral and subdural hemorrhage.[1‑5] Incidence of intracranial hemorrhage was 34% with SAH being the most common in a large study.[3] Large parenchymal hemorrhage is uncommon and usually requires cerebral angiography to rule out a vascular malformation. Furthermore, diffuse multisegmental vasoconstriction on angiogram is often mistaken for vasculitis.[6,7] We report a case of RCVS without any precipitating factor in a 43‑year‑old lady who presented acutely with a large intracerebral hematoma and in whom the digital substraction angiography (DSA) showed a reversible, multisegmental, diffuse vasoconstriction of the cerebral vessels. A 43‑year‑old lady presented with severe headache and sudden onset of right side weakness. She had mild holocranial headache for 3 days prior to this event. There was no history of use of any vasoactive substance or any other medication. Vitals were normal. Neurologic examination revealed Glasgow coma 535
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.
