Images in Neurology
Pulvinar sign in acute disseminated encephalomyelitis Varsha S. Dabadghao, Piyush Ostwal1, Suresh K. Sharma, Subodh A. Garg Departments of Medicine, and 1Neurology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India For correspondence: Dr. Piyush Ostwal, 601, N4, Nakshatram, Premlok Park, Chinchwad, Pune - 411 033, Maharashtra, India.
E-mail: [email protected] Ann Indian Acad Neurol 2014;17:214-6
Case Report A 20-year-old lady presented with fever of 5 days duration followed by headache and drowsiness. Over next 2 days, she was noticed to have weakness of both lower limbs and distal upper limbs, tingling in the limbs and trunk below neck and retention of urine requiring catheterization. On examination, she was drowsy, had lower limb weakness of grade 3/5, upper limb distal weakness of grade 4/5, and sensory impairment below D1 (first dorsal spinal cord segment) level. Her deep tendon reflexes were normal in upper limbs and absent in lower limbs and plantar response was extensor bilaterally. She was evaluated with magnetic resonance imaging (MRI) of the brain and spinal cord which showed bilaterally symmetrical hyperintense signal changes on T2-weighted and fluid-a enuated inversion recovery (FLAIR) sequences in medial and posterior thalami without any enhancement on contrast [Figures 1 and 2]. MRI of the spinal cord showed diffuse ill-defined hyperintense signal on T2-weighted image without any cord swelling or contrast enhancement [Figures 3 and 4]. Cerebrospinal fluid analysis showed total 7 cells/mm3 predominantly lymphocytes, protein 76 mg/dl, sugar 61 mg/dl, and no oligoclonal bands. Testing for tubercular bacilli on CSF by acid fast staining and polymerase chain reaction was negative. Serological testing for antinuclear antibody (ANA), anti-Ro antibody, anti-La antibody, and antineuromylitis optica (anti-NMO) antibody was negative. Her erythrocyte sedimentation rate (ESR) was 55 mm in 1st h. Serological testing for human immunodeficiency virus (HIV), hepatitis B virus (HBsAg), and dengue virus (immunoglobulin (Ig)G, IgM, and NS1 antigen) was negative. Other parameters of blood counts and urine examination were within normal limits. The chest X-ray and ultrasonography of the abdomen and pelvis were normal. Based on the clinical picture, imaging findings, and Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.132640
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
a er ruling out other possible etiologies a final diagnosis of acute disseminated encephalomyelitis (ADEM) was made. She was given injection methylprednisolone 1 g daily for 5 days and patient showed good response to that in terms of sensorium, motor functions, sensory symptoms, and bladder dysfunction. Patient at the time of peak neurological deficits was drowsy, quadriparetic, and bed ridden with indwelling catheter. A er treatment patient was alert, self-ambulant with minimal imbalance, and had no bladder symptoms. She was put on tapering course of oral prednisolone and was maintaining improvement at 2 week follow-up.
Discussion Pulvinar nucleus is the largest nucleus in the thalamus and is situated at the caudal extremity of the thalamus. Signal changes in pulvinar on MRI were noted initially in case reports of patients with variant Creutzfeldt-Jakob disease (CJD) and subsequently consistent correlation of this finding prompted the recognition of this abnormality on MRI as pulvinar sign.[1] Pulvinar sign on MRI is considered to be a strong indicator of variant CJD in an appropriate clinical context. For the diagnosis of variant CJD, World Health Organization defined this sign as “a characteristic distribution of symmetrical hyperintensity (relative to the cortical and other deep grey ma er nuclei signal intensity) of the pulvinar nucleus (posterior nucleus) of the thalamus seen on axial images”.[2] The possible explanation for the signal change on MRI in CJD is astrocytosis.[1] The pulvinar sign has also been reported in a number of other diseases [Table 1]. In our case, in addition to pulvinar sign, our patient also had spinal cord signal changes along with clinical features of myelopathy. In view of long segment myelitis, we also considered differentials of neuromyelitis optica (NMO), Sjogren’s syndrome, systemic lupus erythematosus, and tubercular myelitis which were ruled out by appropriate investigations. Since our patient had acute onset multifocal CNS involvement with polysymptomatic clinical presentation including encephalopathy and compatible MRI findings; therefore, she met diagnostic criteria for ADEM proposed by international pediatric multiple sclerosis study group.[12] She responded well to the high dose intravenous methylprednisolone as expected in ADEM. In ADEM characteristic lesions seen on
Dabadghao, et al.: Pulvinar sign in ADEM
Figure 1: Magnetic resonance imaging (MRI) fluid-attenuated inversion recovery (FLAIR) axial image of the brain showing symmetrical hyperintensity in bilateral pulvinar nuclei of the thalamus
Figure 3: MRI T2-weighted sagittal image of the cervical spine showing hyperintense intramedullary cord signal changes throughout the cervical and upper dorsal spinal cord
Table 1: Conditions in which “pulvinar sign” has been reported in literature T2 Hyperintense pulvinar sign Creutzfeldt-Jakob disease[1] Benign intracranial hypertension[3] Alper’s disease[4] Acute encephalitis[5] Post-infectious encephalitis[6] Wernicke’s encephalopathy[7] Celiac disease[8] Paraneoplastic limbic encephalitis[9] Cat scratch disease[10] T1 hyperintense pulvinar sign
Figure 2: MRI T2-weighted axial image of the brain showing symmetrical hyperintensity in bilateral pulvinar nucluei of the thalamus
Figure 4: MRI T2-weighted sagittal image of the dorsolumbar spine showing hyperintense intramedullary cord signal changes throughout the dorsal and lumbar spinal cord
can also be affected, particularly basal ganglion, thalami, and brainstem.[13] Pulvinar sign has rarely been reported as a radiographic feature in ADEM.[6] Though the sign is not specific for ADEM, the recognition of pulvinar sign in an MRI of the brain should definitely arouse suspicion of the same apart from other differentials.
References 1.
2.
Fabry’s disease[11]
MRI appear as patchy areas of increased signal intensity on conventional T2-weighted images and on FLAIR sequence. Though white ma er involvement predominates grey ma er
215
3.
Zeidler M, Sellar RJ, Collie DA, Knight R, Stewart G, Macleod MA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000;355:1412-8. The Revision of the Surveillance Case Definition for Variant Creutzfeldt–Jakob Disease (vCJD): Report of a WHO Consultation [Internet]. Edinburgh: World Health Organization; 2001 May. Available from: http://whqlibdoc.who.int/hq/2002/WHO_CDS_ CSR_EPH_2001.5.pdf [Last accessed on 19 January 2014]. Yoshimura N, Soma Y. A case of benign intracranial hypertension with bilateral reversible thalamic lesions on magnetic resonance imaging. Rinsho Shinkeigaku 1992;32:327-9.
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
216 4.
5.
6.
7.
8.
9.
Dabadghao, et al.: Pulvinar sign in ADEM Harding BN, Alsanjari N, Smith SJ, Wiles CM, Thrush D, Miller DH, et al. Progressive neuronal degeneration of childhood with liver disease (Alpers’ disease) presenting in young adults. J Neurol Neurosurg Psychiatry 1995;58:320-5. Kim SS, Chang KH, Kim KW, Han MH, Park SH, Nam HW, et al. Unusual acute encephalitis involving the thalamus: Imaging features. Korean J Radiol 2001;2:68-74. Cusmai R, Bertini E, Di Capua M, Ricci S, Vigevano F, Milani L, et al. Bilateral, reversible, selective thalamic involvement demonstrated by brain MR and acute severe neurological dysfunction with favorable outcome. Neuropediatrics 1994;25:44-7. Weidauer S, Nichtweiss M, Lanfermann H, Zanella FE. Wernicke encephalopathy: MR findings and clinical presentation. Eur Radiol 2003;13:1001-9. Ryan AM, Ryan J, Wan-Ahmed M, Hardiman O, Farrell MA, McNamara B, et al. Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease. J Neurol Neurosurg Psychiatry 2007;78:98-9. Mihara M, Sugase S, Konaka K, Sugai F, Sato T, Yamamoto Y, et al. The “pulvinar sign” in a case of paraneoplastic limbic
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
10. 11.
12.
13.
encephalitis associated with non-Hodgkin’s lymphoma. J Neurol Neurosurg Psychiatry 2005;76:882-4. Hahn JS, Sum JM, Lee KP. Unusual MRI findings after status epilepticus due to cat-scratch disease. Pediatr Neurol 1994;10:255-8. Takanashi J, Barkovich AJ, Dillon WP, Sherr EH, Hart KA, Packman S. T1 hyperintensity in the pulvinar: Key imaging feature for diagnosis of Fabry disease. AJNR Am J Neuroradiol 2003;24:916-21. Young NP, Weinshenker BG, Lucchinetti CF. Acute disseminated encephalomyelitis: Current understanding and controversies. Semin Neurol 2008;28:84-94. Garg RK. Acute disseminated encephalomyelitis. Postgrad Med J 2003;79:11-7.
How to cite this article: Dabadghao VS, Ostwal P, Sharma SK, Garg SA. Pulvinar sign in acute disseminated encephalomyelitis. Ann Indian Acad Neurol 2014;17:214-6. Received: 19-12-13, Revised: 21-01-14, Accepted: 12-02-14 Source of Support: Nil, Conflict of Interest: None declared.
Copyright of Annals of Indian Academy of Neurology 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.
Pulvinar sign in acute disseminated encephalomyelitis Varsha S. Dabadghao, Piyush Ostwal1, Suresh K. Sharma, Subodh A. Garg Departments of Medicine, and 1Neurology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India For correspondence: Dr. Piyush Ostwal, 601, N4, Nakshatram, Premlok Park, Chinchwad, Pune - 411 033, Maharashtra, India.
E-mail: [email protected] Ann Indian Acad Neurol 2014;17:214-6
Case Report A 20-year-old lady presented with fever of 5 days duration followed by headache and drowsiness. Over next 2 days, she was noticed to have weakness of both lower limbs and distal upper limbs, tingling in the limbs and trunk below neck and retention of urine requiring catheterization. On examination, she was drowsy, had lower limb weakness of grade 3/5, upper limb distal weakness of grade 4/5, and sensory impairment below D1 (first dorsal spinal cord segment) level. Her deep tendon reflexes were normal in upper limbs and absent in lower limbs and plantar response was extensor bilaterally. She was evaluated with magnetic resonance imaging (MRI) of the brain and spinal cord which showed bilaterally symmetrical hyperintense signal changes on T2-weighted and fluid-a enuated inversion recovery (FLAIR) sequences in medial and posterior thalami without any enhancement on contrast [Figures 1 and 2]. MRI of the spinal cord showed diffuse ill-defined hyperintense signal on T2-weighted image without any cord swelling or contrast enhancement [Figures 3 and 4]. Cerebrospinal fluid analysis showed total 7 cells/mm3 predominantly lymphocytes, protein 76 mg/dl, sugar 61 mg/dl, and no oligoclonal bands. Testing for tubercular bacilli on CSF by acid fast staining and polymerase chain reaction was negative. Serological testing for antinuclear antibody (ANA), anti-Ro antibody, anti-La antibody, and antineuromylitis optica (anti-NMO) antibody was negative. Her erythrocyte sedimentation rate (ESR) was 55 mm in 1st h. Serological testing for human immunodeficiency virus (HIV), hepatitis B virus (HBsAg), and dengue virus (immunoglobulin (Ig)G, IgM, and NS1 antigen) was negative. Other parameters of blood counts and urine examination were within normal limits. The chest X-ray and ultrasonography of the abdomen and pelvis were normal. Based on the clinical picture, imaging findings, and Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.132640
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
a er ruling out other possible etiologies a final diagnosis of acute disseminated encephalomyelitis (ADEM) was made. She was given injection methylprednisolone 1 g daily for 5 days and patient showed good response to that in terms of sensorium, motor functions, sensory symptoms, and bladder dysfunction. Patient at the time of peak neurological deficits was drowsy, quadriparetic, and bed ridden with indwelling catheter. A er treatment patient was alert, self-ambulant with minimal imbalance, and had no bladder symptoms. She was put on tapering course of oral prednisolone and was maintaining improvement at 2 week follow-up.
Discussion Pulvinar nucleus is the largest nucleus in the thalamus and is situated at the caudal extremity of the thalamus. Signal changes in pulvinar on MRI were noted initially in case reports of patients with variant Creutzfeldt-Jakob disease (CJD) and subsequently consistent correlation of this finding prompted the recognition of this abnormality on MRI as pulvinar sign.[1] Pulvinar sign on MRI is considered to be a strong indicator of variant CJD in an appropriate clinical context. For the diagnosis of variant CJD, World Health Organization defined this sign as “a characteristic distribution of symmetrical hyperintensity (relative to the cortical and other deep grey ma er nuclei signal intensity) of the pulvinar nucleus (posterior nucleus) of the thalamus seen on axial images”.[2] The possible explanation for the signal change on MRI in CJD is astrocytosis.[1] The pulvinar sign has also been reported in a number of other diseases [Table 1]. In our case, in addition to pulvinar sign, our patient also had spinal cord signal changes along with clinical features of myelopathy. In view of long segment myelitis, we also considered differentials of neuromyelitis optica (NMO), Sjogren’s syndrome, systemic lupus erythematosus, and tubercular myelitis which were ruled out by appropriate investigations. Since our patient had acute onset multifocal CNS involvement with polysymptomatic clinical presentation including encephalopathy and compatible MRI findings; therefore, she met diagnostic criteria for ADEM proposed by international pediatric multiple sclerosis study group.[12] She responded well to the high dose intravenous methylprednisolone as expected in ADEM. In ADEM characteristic lesions seen on
Dabadghao, et al.: Pulvinar sign in ADEM
Figure 1: Magnetic resonance imaging (MRI) fluid-attenuated inversion recovery (FLAIR) axial image of the brain showing symmetrical hyperintensity in bilateral pulvinar nuclei of the thalamus
Figure 3: MRI T2-weighted sagittal image of the cervical spine showing hyperintense intramedullary cord signal changes throughout the cervical and upper dorsal spinal cord
Table 1: Conditions in which “pulvinar sign” has been reported in literature T2 Hyperintense pulvinar sign Creutzfeldt-Jakob disease[1] Benign intracranial hypertension[3] Alper’s disease[4] Acute encephalitis[5] Post-infectious encephalitis[6] Wernicke’s encephalopathy[7] Celiac disease[8] Paraneoplastic limbic encephalitis[9] Cat scratch disease[10] T1 hyperintense pulvinar sign
Figure 2: MRI T2-weighted axial image of the brain showing symmetrical hyperintensity in bilateral pulvinar nucluei of the thalamus
Figure 4: MRI T2-weighted sagittal image of the dorsolumbar spine showing hyperintense intramedullary cord signal changes throughout the dorsal and lumbar spinal cord
can also be affected, particularly basal ganglion, thalami, and brainstem.[13] Pulvinar sign has rarely been reported as a radiographic feature in ADEM.[6] Though the sign is not specific for ADEM, the recognition of pulvinar sign in an MRI of the brain should definitely arouse suspicion of the same apart from other differentials.
References 1.
2.
Fabry’s disease[11]
MRI appear as patchy areas of increased signal intensity on conventional T2-weighted images and on FLAIR sequence. Though white ma er involvement predominates grey ma er
215
3.
Zeidler M, Sellar RJ, Collie DA, Knight R, Stewart G, Macleod MA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet 2000;355:1412-8. The Revision of the Surveillance Case Definition for Variant Creutzfeldt–Jakob Disease (vCJD): Report of a WHO Consultation [Internet]. Edinburgh: World Health Organization; 2001 May. Available from: http://whqlibdoc.who.int/hq/2002/WHO_CDS_ CSR_EPH_2001.5.pdf [Last accessed on 19 January 2014]. Yoshimura N, Soma Y. A case of benign intracranial hypertension with bilateral reversible thalamic lesions on magnetic resonance imaging. Rinsho Shinkeigaku 1992;32:327-9.
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
216 4.
5.
6.
7.
8.
9.
Dabadghao, et al.: Pulvinar sign in ADEM Harding BN, Alsanjari N, Smith SJ, Wiles CM, Thrush D, Miller DH, et al. Progressive neuronal degeneration of childhood with liver disease (Alpers’ disease) presenting in young adults. J Neurol Neurosurg Psychiatry 1995;58:320-5. Kim SS, Chang KH, Kim KW, Han MH, Park SH, Nam HW, et al. Unusual acute encephalitis involving the thalamus: Imaging features. Korean J Radiol 2001;2:68-74. Cusmai R, Bertini E, Di Capua M, Ricci S, Vigevano F, Milani L, et al. Bilateral, reversible, selective thalamic involvement demonstrated by brain MR and acute severe neurological dysfunction with favorable outcome. Neuropediatrics 1994;25:44-7. Weidauer S, Nichtweiss M, Lanfermann H, Zanella FE. Wernicke encephalopathy: MR findings and clinical presentation. Eur Radiol 2003;13:1001-9. Ryan AM, Ryan J, Wan-Ahmed M, Hardiman O, Farrell MA, McNamara B, et al. Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease. J Neurol Neurosurg Psychiatry 2007;78:98-9. Mihara M, Sugase S, Konaka K, Sugai F, Sato T, Yamamoto Y, et al. The “pulvinar sign” in a case of paraneoplastic limbic
Annals of Indian Academy of Neurology, April-June 2014, Vol 17, Issue 2
10. 11.
12.
13.
encephalitis associated with non-Hodgkin’s lymphoma. J Neurol Neurosurg Psychiatry 2005;76:882-4. Hahn JS, Sum JM, Lee KP. Unusual MRI findings after status epilepticus due to cat-scratch disease. Pediatr Neurol 1994;10:255-8. Takanashi J, Barkovich AJ, Dillon WP, Sherr EH, Hart KA, Packman S. T1 hyperintensity in the pulvinar: Key imaging feature for diagnosis of Fabry disease. AJNR Am J Neuroradiol 2003;24:916-21. Young NP, Weinshenker BG, Lucchinetti CF. Acute disseminated encephalomyelitis: Current understanding and controversies. Semin Neurol 2008;28:84-94. Garg RK. Acute disseminated encephalomyelitis. Postgrad Med J 2003;79:11-7.
How to cite this article: Dabadghao VS, Ostwal P, Sharma SK, Garg SA. Pulvinar sign in acute disseminated encephalomyelitis. Ann Indian Acad Neurol 2014;17:214-6. Received: 19-12-13, Revised: 21-01-14, Accepted: 12-02-14 Source of Support: Nil, Conflict of Interest: None declared.
Copyright of Annals of Indian Academy of Neurology 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.
