[Downloaded free from http://www.neurologyindia.com on Thursday, March 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
pathogenesis.[1-3] Plasma exchange may be useful when there is incomplete response to steroids.[2]
Kolar Vishwanath Vinod, Madasamy Ponraj, Khened Swetharani, Tarun Kumar Dutta Department of General Medicine, JIPMER, Dhanvantari Nagar, Puducherry, India E-mail: [email protected]
References 1. 2. 3. 4.
5. 6.
Bánovcin P, Havlíceková Z, Jesenák M, Nosál S, Durdík P, Ciljaková M, et al. Severe quadriparesis caused by wasp sting. Turk J Pediatr 2009;51:485-8. L i k i t t a n a s o m b u t P, Wi t o o n p a n i c h R , Vi r a n u v a t t i K . Encephalomyeloradiculopathy associated with wasp sting. J Neurol Neurosurg Psychiatry 2003;74:134-5. Maltzman JS, Lee AG, Miller NR. Optic neuropathy occurring after bee and wasp sting. Ophthalmology 2000;107:193-5. Ridolo E, Albertini R, Borghi L, Meschi T, Montanari E, Dall’Aglio PP. Acute polyradiculoneuropathy occurring after hymenoptera stings: A clinical case study. Int J Immunopathol Pharmacol 2005;18:385-90. D’Cruz S, Chauhan S, Singh R, Sachdev A, Lehl S. Wasp sting associated with type 1 renal tubular acidosis. Nephrol Dial Transplant 2008;23:1754-5. Defer G, Cesaro P, Roualdes B, Degos JD. Acute myelitis following Hymenoptera sting. Presse Med 1984;13:227. Access this article online Quick Response Code:
limb hyporeflexia. Cerebrospinal fluid (CSF) examination revealed 303 × 106 cells/L (84% were lymphocytes), 917 mg/L protein, normal glucose and chloride and positive rapid plasma regain test (RPR). The Treponema pallidum hemagglutination test was positive and the serum RPR was positive with 1:16. Serological test for other viral infections including HIV were negative. Spinal magnetic resonance imaging (MRI) revealed swelling and high signal intensity of the spinal cord parenchyma at level of T6 through to T11 on T2-weighted images and focal gadolinium enhancement [Figure 1]. Brain MRI was normal. Treatment with ceftriaxone (2 g twice a day for 30 days) and methylprednisolone (100 mg/day, 50 mg/day and 12.5 mg/day for 3 days each) was initiated. He had symptomatic improvement by day-5. CSF examination at 1 month showed 34 × 106 cells/L, 454 mg/L protein, negative RPR. Serum RPR was positive with 1:4. Spinal MRI showed decreased abnormal signal [Figure 2]. After 3 months, neurologic examination, CSF findings and spinal MRI were normal [Figure 3]. Serum RPR titer was 1:2, indicating cure. Syphilis is a sexually transmitted infectious disease caused by spirochete T. pallidum. The incidence of neurosyphilis is estimated to be 4-10% in untreated cases and 1.5% of neurosyphilis will progress to syphilitic myelitis.[4,5]
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128346
Received: 04-01-2014 Review completed: 29-01-2014 Accepted: 29-01-2014 a
Syphilitic myelitis: Magnetic resonance imaging features
b
c
d
Figure 1: (a) Sagittal T2-weighted image of the thoracic spinal cord shows long-segment diffuse high signal intensity from T6 to T11 with cord swelling. (b) Coronal T1-weighted image with contrast shows focal enhancement at T8/T9 level. (c) Sagittal T1-weighted image with contrast. (d) Axial T1-weighted image with contrast at T8/T9 level
Sir, The incidence of syphilis has markedly declined in the post-penicillin era. However, cases of syphilis have been increasing with the emergence of human immunodeficiency virus (HIV) infection since mid-1980s world-wide.[1,2] Although approximately one-third of patients with early syphilis have central nervous system involvement, symptomatic neurosyphilis, especially syphilitic myelitis is rare,[3] hence this report. A 63-year-old male patient presented with a 12-day history of progressive lumbago and weakness of both lower extremities. Examination documented bilateral lower-limb weakness with motor power of 4-5, lower Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
a
b
Figure 2: Follow-up magnetic resonance imaging performed 1 month after onset of treatment. (a) Sagittal and (b) axial gadolinium-enhanced T1-weighted images show residual focal enhancement in the thoracic cord at T8/T9 level
89
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Table 1: Cases of syphilitic myelitis reported previously
Case
Age (years) Sex
Tashiro-1987 Nabatame-1992 Tsui-2002 Kikuchi-2003 Chilver-2009
31 46 52 36 46
Male Male Female Male Male
MRI findings
Treatment
Recovery
Candle guttering appearance Candle guttering appearance Candle guttering appearance Candle guttering appearance with flip-flop sign Candle guttering appearance and abnormal enhancement in the central part of spinal cord
Penicillin G with prednisolone Antibiotic therapy Penicillin G with dexamethasone Penicillin G Penicillin G with methylprednisolone
Yes (about 1 month later) Yes Yes (about 1 month later) Yes (about 1 month later) Yes (3 months later)
MRI - Magnetic resonance imaging
a
b
Figure 3: Follow-up magnetic resonance imaging performed 3 months after onset of treatment. (a) Sagittal T2-weighted image and (b) sagittal gadolinium-enhanced T1-weighted image show normal spinal cord and the abnormal signals have disappeared
The first descriptions of MRI findings in syphilitic myelitis were short-segment high signal intensity in the thoracic cord on T2-weighted images and abnormal peripheral enhancement.[6] Since the only a few cases have been documented in the literature.[7-10] The high-signal lesions of the spinal cord parenchyma extending over multiple levels on T2-weighted imaging and the abnormal enhancement on gadolinium-enhanced T1-weighted images have been validated [Table 1]. The classic appearance on contrast studies, candle guttering appearance, was seen in our patient, but not the flip-flop sign, low signal intensity on T2-weighted imaging and high intensity on gadolinium-enhanced T1-weighted imaging. [9] The gadolinium-enhancement seen on T1-weighted images resolve with the treatment with penicillin G and prednisolone and might represent spinal cord ischemia or edema secondary to meningovascular syphilis.[6-10] However, the inflammatory nature of the MRI findings has been implied.[11] This patient had surgical resection of the lesion as the pre-operative diagnosis being spinal cord tumor and on histopathology the lesion consisted of a core of Microspironema pallidum and peripheral lymphocytic infiltrate with gliosis. The “candle guttering appearance” on MRI suggests that the pathological process of neurosyphilis involves invasion of the spinal cord from its surface. Furthermore, the high intensity area of the spinal cord on T2-weighted images may represent a spinal cord 90
edema or ischemia secondary to inflammatory process. In fact, all abnormally enhanced areas on T1-weighted images locate in the central parts of the high intensity areas on T2-weighted images. In our patient, the enhanced nodule on T1-weighted images at level of T8/T9 located centrally in high intensity area on T2-weighted images at T6-T11 level. If the lesion on gadolinium-enhanced T1-weighted images is large enough, the flip-flop sign may be observed. However, the diagnosis of syphilitic myelitis must be based on serum and CSF tests because the MR imaging features are non-specific,[12] Guidelines recommend treatment of neurosyphilis with 18-24 million units of aqueous penicillin IV per day for 10-14 days. Prednisolone may be added to prevent cord edema, ischemia, or Jarisch-Herxheimer reactions.[13] Ceftriaxone is acceptable alternative in patients who are allergic to penicillin, such as our patient. The characteristics MRI findings may suggest the diagnostic possibility of syphilitic myelitis and help in confirming the diagnosis of neurosyphilis by serological tests.
Dongmei He, Binghu Jiang1 Departments of Neurology, and 1Radiology, BenQ Medical Center, Jianye District, Nanjing 210019, China E-mail: [email protected]
References 1. 2. 3. 4. 5. 6. 7. 8. 9.
Hook EW 3rd, Marra CM. Acquired syphilis in adults. N Engl J Med 1992;326:1060-9. Fenton KA, Breban R, Vardavas R, Okano JT, Martin T, Aral S, et al. Infectious syphilis in high-income settings in the 21st century. Lancet Infect Dis 2008;8:244-53. O’donnell JA, Emery CL. Neurosyphilis: A current review. Curr Infect Dis Rep 2005;7:277-84. Conde-Sendín MA, Amela-Peris R, Aladro-Benito Y, Maroto AA. Current clinical spectrum of neurosyphilis in immunocompetent patients. Eur Neurol 2004;52:29-35. Adams RD, Merritt HH. Meningeal and vascular syphilis of the spinal cord. Med (Baltimore) 1944;23:181-214. Tashiro K, Moriwaka F, Sudo K, Akino M, Abe H. Syphilitic myelitis with its magnetic resonance imaging (MRI) verification and successful treatment. Jpn J Psychiatry Neurol 1987;41:269-71. Nabatame H, Nakamura K, Matuda M, Fujimoto N, Dodo Y, Imura T. MRI of syphilitic myelitis. Neuroradiology 1992;34:105-6. Tsui EY, Ng SH, Chow L, Lai KF, Fong D, Chan JH. Syphilitic myelitis with diffuse spinal cord abnormality on MR imaging. Eur Radiol 2002;12:2973-6. Kikuchi S, Shinpo K, Niino M, Tashiro K. Subacute syphilitic Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
[Downloaded free from http://www.neurologyindia.com on Thursday, March 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
10. 11. 12.
13.
meningomyelitis with characteristic spinal MRI findings. J Neurol 2003;250:106-7. Chilver-Stainer L, Fischer U, Hauf M, Fux CA, Sturzenegger M. Syphilitic myelitis: Rare, nonspecific, but treatable. Neurology 2009;72:673-5. Wu M, Qian RB, Wei XP. A case of syphilitic myelitis. Shandong Med 2011;51:101. Nagappa M, Sinha S, Taly AB, Rao SL, Nagarathna S, Bindu PS, et al. Neurosyphilis: MRI features and their phenotypic correlation in a cohort of 35 patients from a tertiary care university hospital. Neuroradiology 2013;55:379-88. Centers for Disease Control and Prevention, Workowski KA, Berman SM. Sexually transmitted diseases treatment guidelines, 2006. MMWR Recomm Rep 2006;55:1-94. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128347
Received: 25-12-2013 Review completed: 27-12-2013 Accepted: 26-01-2014
An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period Sir, A 29-year-old primigravida of 34 weeks amenorrhea was referred with the complaints of severe episodic headaches, vomiting and status epilepticus after emergency cesarean section from gynecology services for evaluation. History was negative for fever, hypertension, trauma, pedal edema, abortions/fetal loss or epilepsy and she had regular antenatal care. On examination, she was stuporous with left hemiparesis and bilateral plantar extensor. Magnetic resonance imaging (MRI) brain revealed multiple large acute intrparenchymal hemorrhages: Right frontal lobe (4 × 3.8 × 2.9 cm), left superior frontal lobe (1.2 × 1 cm) and left temporo-occipital lobe (1.4 × 2 cm) with intraventricular extension [Figure 1a and b]. Fluid-attenuated inversion recovery sequence also revealed subarachnoid hemorrhage (SAH) [Figure 1c]. MR-angiography (MRA) revealed marked diffuse narrowing of supraclinoid segments of bilateral internal carotid arteries, proximal bilateral M1 segment of middle cerebral artery, A1 segment of anterior cerebral artery and P1 segment of posterior communicating artery [Figure 1d] with no evidence of cerebral aneurysm. MR-venogram (MRV) was normal. With a working diagnosis of reversible cerebral vasoconstriction Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
syndromes (RCVS) she was treated with antiepileptic drugs, nemodepine and supportive therapy. Patient improved over the next 15 days and had minimal deficits when discharged. At 2 months follow-up repeat MRA was normal [Figure 1e] and patient was asymptomatic. In our patient with the clinical scenario, the differentials considered included: Eclampsia, cortical venous thrombosis (CVT), posterior reversible encephalopathy syndrome (PRES) and arterial stroke. Eclampsia was excluded as there was no history of hypertension and no albuminuria. MRI brain and MRV excluded the diagnostic possibilities of CVT and PRES. Cerebral MRA revealed bilateral multisegmental vasoconstriction, possibility of primary angiitis of central nervous system and RCVS were considered. Reversal of segmental vasoconstriction on repeat MRA confirmed the diagnosis of RCVS. RCVS comprise a group of disorders characterized by prolonged but reversible vasoconstriction of the cerebral arteries, usually associated with acute-onset, severe, recurrent headaches, with or without additional neurologic signs and symptoms.[1] Description of this rare entity dates back to 1960s[2] and was limited to case reports. Call et al. reviewed the literature and published a case series titled ‘Reversible Cerebral Segmental Vasoconstriction’ in 1988.[3] Calabrese et al.[1] brought the awareness of this entity by their reviews. It is usually a disease of young and middle aged females. The exact incidence is unknown. Approximately 60% of cases are secondary, mainly postpartum period, eclampsia, exposure to vasoactive substances [4] such as cannabis, cocaine, immunosuppressants, sympathomimetics, methergine, bromocriptine etc. Although the pathophysiology remains
a
d
c
b
e
Figure 1: Magnetic resonance imaging brain fluid-attenuated inversion recovery (FLAIR) sequence showing acute parenchymal hemorrhage in right frontal lobe (a) left temporo-occipital lobe (b) diffuse FLAIR hyperintensity of bihemispheric sulcal spaces suggestive of subarachnoid hemorrhage (c) Magnetic resonance-angiography of brain showing marked diffuse narrowing of supraclinoid segments of bilateral ICA, M1, A1 and P1 segments (d) and showing normal vessels after 2 months (e)
91
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.
pathogenesis.[1-3] Plasma exchange may be useful when there is incomplete response to steroids.[2]
Kolar Vishwanath Vinod, Madasamy Ponraj, Khened Swetharani, Tarun Kumar Dutta Department of General Medicine, JIPMER, Dhanvantari Nagar, Puducherry, India E-mail: [email protected]
References 1. 2. 3. 4.
5. 6.
Bánovcin P, Havlíceková Z, Jesenák M, Nosál S, Durdík P, Ciljaková M, et al. Severe quadriparesis caused by wasp sting. Turk J Pediatr 2009;51:485-8. L i k i t t a n a s o m b u t P, Wi t o o n p a n i c h R , Vi r a n u v a t t i K . Encephalomyeloradiculopathy associated with wasp sting. J Neurol Neurosurg Psychiatry 2003;74:134-5. Maltzman JS, Lee AG, Miller NR. Optic neuropathy occurring after bee and wasp sting. Ophthalmology 2000;107:193-5. Ridolo E, Albertini R, Borghi L, Meschi T, Montanari E, Dall’Aglio PP. Acute polyradiculoneuropathy occurring after hymenoptera stings: A clinical case study. Int J Immunopathol Pharmacol 2005;18:385-90. D’Cruz S, Chauhan S, Singh R, Sachdev A, Lehl S. Wasp sting associated with type 1 renal tubular acidosis. Nephrol Dial Transplant 2008;23:1754-5. Defer G, Cesaro P, Roualdes B, Degos JD. Acute myelitis following Hymenoptera sting. Presse Med 1984;13:227. Access this article online Quick Response Code:
limb hyporeflexia. Cerebrospinal fluid (CSF) examination revealed 303 × 106 cells/L (84% were lymphocytes), 917 mg/L protein, normal glucose and chloride and positive rapid plasma regain test (RPR). The Treponema pallidum hemagglutination test was positive and the serum RPR was positive with 1:16. Serological test for other viral infections including HIV were negative. Spinal magnetic resonance imaging (MRI) revealed swelling and high signal intensity of the spinal cord parenchyma at level of T6 through to T11 on T2-weighted images and focal gadolinium enhancement [Figure 1]. Brain MRI was normal. Treatment with ceftriaxone (2 g twice a day for 30 days) and methylprednisolone (100 mg/day, 50 mg/day and 12.5 mg/day for 3 days each) was initiated. He had symptomatic improvement by day-5. CSF examination at 1 month showed 34 × 106 cells/L, 454 mg/L protein, negative RPR. Serum RPR was positive with 1:4. Spinal MRI showed decreased abnormal signal [Figure 2]. After 3 months, neurologic examination, CSF findings and spinal MRI were normal [Figure 3]. Serum RPR titer was 1:2, indicating cure. Syphilis is a sexually transmitted infectious disease caused by spirochete T. pallidum. The incidence of neurosyphilis is estimated to be 4-10% in untreated cases and 1.5% of neurosyphilis will progress to syphilitic myelitis.[4,5]
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128346
Received: 04-01-2014 Review completed: 29-01-2014 Accepted: 29-01-2014 a
Syphilitic myelitis: Magnetic resonance imaging features
b
c
d
Figure 1: (a) Sagittal T2-weighted image of the thoracic spinal cord shows long-segment diffuse high signal intensity from T6 to T11 with cord swelling. (b) Coronal T1-weighted image with contrast shows focal enhancement at T8/T9 level. (c) Sagittal T1-weighted image with contrast. (d) Axial T1-weighted image with contrast at T8/T9 level
Sir, The incidence of syphilis has markedly declined in the post-penicillin era. However, cases of syphilis have been increasing with the emergence of human immunodeficiency virus (HIV) infection since mid-1980s world-wide.[1,2] Although approximately one-third of patients with early syphilis have central nervous system involvement, symptomatic neurosyphilis, especially syphilitic myelitis is rare,[3] hence this report. A 63-year-old male patient presented with a 12-day history of progressive lumbago and weakness of both lower extremities. Examination documented bilateral lower-limb weakness with motor power of 4-5, lower Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
a
b
Figure 2: Follow-up magnetic resonance imaging performed 1 month after onset of treatment. (a) Sagittal and (b) axial gadolinium-enhanced T1-weighted images show residual focal enhancement in the thoracic cord at T8/T9 level
89
[Downloaded free from http://www.neurologyindia.com on Thursday, March 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
Table 1: Cases of syphilitic myelitis reported previously
Case
Age (years) Sex
Tashiro-1987 Nabatame-1992 Tsui-2002 Kikuchi-2003 Chilver-2009
31 46 52 36 46
Male Male Female Male Male
MRI findings
Treatment
Recovery
Candle guttering appearance Candle guttering appearance Candle guttering appearance Candle guttering appearance with flip-flop sign Candle guttering appearance and abnormal enhancement in the central part of spinal cord
Penicillin G with prednisolone Antibiotic therapy Penicillin G with dexamethasone Penicillin G Penicillin G with methylprednisolone
Yes (about 1 month later) Yes Yes (about 1 month later) Yes (about 1 month later) Yes (3 months later)
MRI - Magnetic resonance imaging
a
b
Figure 3: Follow-up magnetic resonance imaging performed 3 months after onset of treatment. (a) Sagittal T2-weighted image and (b) sagittal gadolinium-enhanced T1-weighted image show normal spinal cord and the abnormal signals have disappeared
The first descriptions of MRI findings in syphilitic myelitis were short-segment high signal intensity in the thoracic cord on T2-weighted images and abnormal peripheral enhancement.[6] Since the only a few cases have been documented in the literature.[7-10] The high-signal lesions of the spinal cord parenchyma extending over multiple levels on T2-weighted imaging and the abnormal enhancement on gadolinium-enhanced T1-weighted images have been validated [Table 1]. The classic appearance on contrast studies, candle guttering appearance, was seen in our patient, but not the flip-flop sign, low signal intensity on T2-weighted imaging and high intensity on gadolinium-enhanced T1-weighted imaging. [9] The gadolinium-enhancement seen on T1-weighted images resolve with the treatment with penicillin G and prednisolone and might represent spinal cord ischemia or edema secondary to meningovascular syphilis.[6-10] However, the inflammatory nature of the MRI findings has been implied.[11] This patient had surgical resection of the lesion as the pre-operative diagnosis being spinal cord tumor and on histopathology the lesion consisted of a core of Microspironema pallidum and peripheral lymphocytic infiltrate with gliosis. The “candle guttering appearance” on MRI suggests that the pathological process of neurosyphilis involves invasion of the spinal cord from its surface. Furthermore, the high intensity area of the spinal cord on T2-weighted images may represent a spinal cord 90
edema or ischemia secondary to inflammatory process. In fact, all abnormally enhanced areas on T1-weighted images locate in the central parts of the high intensity areas on T2-weighted images. In our patient, the enhanced nodule on T1-weighted images at level of T8/T9 located centrally in high intensity area on T2-weighted images at T6-T11 level. If the lesion on gadolinium-enhanced T1-weighted images is large enough, the flip-flop sign may be observed. However, the diagnosis of syphilitic myelitis must be based on serum and CSF tests because the MR imaging features are non-specific,[12] Guidelines recommend treatment of neurosyphilis with 18-24 million units of aqueous penicillin IV per day for 10-14 days. Prednisolone may be added to prevent cord edema, ischemia, or Jarisch-Herxheimer reactions.[13] Ceftriaxone is acceptable alternative in patients who are allergic to penicillin, such as our patient. The characteristics MRI findings may suggest the diagnostic possibility of syphilitic myelitis and help in confirming the diagnosis of neurosyphilis by serological tests.
Dongmei He, Binghu Jiang1 Departments of Neurology, and 1Radiology, BenQ Medical Center, Jianye District, Nanjing 210019, China E-mail: [email protected]
References 1. 2. 3. 4. 5. 6. 7. 8. 9.
Hook EW 3rd, Marra CM. Acquired syphilis in adults. N Engl J Med 1992;326:1060-9. Fenton KA, Breban R, Vardavas R, Okano JT, Martin T, Aral S, et al. Infectious syphilis in high-income settings in the 21st century. Lancet Infect Dis 2008;8:244-53. O’donnell JA, Emery CL. Neurosyphilis: A current review. Curr Infect Dis Rep 2005;7:277-84. Conde-Sendín MA, Amela-Peris R, Aladro-Benito Y, Maroto AA. Current clinical spectrum of neurosyphilis in immunocompetent patients. Eur Neurol 2004;52:29-35. Adams RD, Merritt HH. Meningeal and vascular syphilis of the spinal cord. Med (Baltimore) 1944;23:181-214. Tashiro K, Moriwaka F, Sudo K, Akino M, Abe H. Syphilitic myelitis with its magnetic resonance imaging (MRI) verification and successful treatment. Jpn J Psychiatry Neurol 1987;41:269-71. Nabatame H, Nakamura K, Matuda M, Fujimoto N, Dodo Y, Imura T. MRI of syphilitic myelitis. Neuroradiology 1992;34:105-6. Tsui EY, Ng SH, Chow L, Lai KF, Fong D, Chan JH. Syphilitic myelitis with diffuse spinal cord abnormality on MR imaging. Eur Radiol 2002;12:2973-6. Kikuchi S, Shinpo K, Niino M, Tashiro K. Subacute syphilitic Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
[Downloaded free from http://www.neurologyindia.com on Thursday, March 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
10. 11. 12.
13.
meningomyelitis with characteristic spinal MRI findings. J Neurol 2003;250:106-7. Chilver-Stainer L, Fischer U, Hauf M, Fux CA, Sturzenegger M. Syphilitic myelitis: Rare, nonspecific, but treatable. Neurology 2009;72:673-5. Wu M, Qian RB, Wei XP. A case of syphilitic myelitis. Shandong Med 2011;51:101. Nagappa M, Sinha S, Taly AB, Rao SL, Nagarathna S, Bindu PS, et al. Neurosyphilis: MRI features and their phenotypic correlation in a cohort of 35 patients from a tertiary care university hospital. Neuroradiology 2013;55:379-88. Centers for Disease Control and Prevention, Workowski KA, Berman SM. Sexually transmitted diseases treatment guidelines, 2006. MMWR Recomm Rep 2006;55:1-94. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128347
Received: 25-12-2013 Review completed: 27-12-2013 Accepted: 26-01-2014
An unusual case of reversible cerebral vasoconstriction syndrome presenting during antepartum period Sir, A 29-year-old primigravida of 34 weeks amenorrhea was referred with the complaints of severe episodic headaches, vomiting and status epilepticus after emergency cesarean section from gynecology services for evaluation. History was negative for fever, hypertension, trauma, pedal edema, abortions/fetal loss or epilepsy and she had regular antenatal care. On examination, she was stuporous with left hemiparesis and bilateral plantar extensor. Magnetic resonance imaging (MRI) brain revealed multiple large acute intrparenchymal hemorrhages: Right frontal lobe (4 × 3.8 × 2.9 cm), left superior frontal lobe (1.2 × 1 cm) and left temporo-occipital lobe (1.4 × 2 cm) with intraventricular extension [Figure 1a and b]. Fluid-attenuated inversion recovery sequence also revealed subarachnoid hemorrhage (SAH) [Figure 1c]. MR-angiography (MRA) revealed marked diffuse narrowing of supraclinoid segments of bilateral internal carotid arteries, proximal bilateral M1 segment of middle cerebral artery, A1 segment of anterior cerebral artery and P1 segment of posterior communicating artery [Figure 1d] with no evidence of cerebral aneurysm. MR-venogram (MRV) was normal. With a working diagnosis of reversible cerebral vasoconstriction Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
syndromes (RCVS) she was treated with antiepileptic drugs, nemodepine and supportive therapy. Patient improved over the next 15 days and had minimal deficits when discharged. At 2 months follow-up repeat MRA was normal [Figure 1e] and patient was asymptomatic. In our patient with the clinical scenario, the differentials considered included: Eclampsia, cortical venous thrombosis (CVT), posterior reversible encephalopathy syndrome (PRES) and arterial stroke. Eclampsia was excluded as there was no history of hypertension and no albuminuria. MRI brain and MRV excluded the diagnostic possibilities of CVT and PRES. Cerebral MRA revealed bilateral multisegmental vasoconstriction, possibility of primary angiitis of central nervous system and RCVS were considered. Reversal of segmental vasoconstriction on repeat MRA confirmed the diagnosis of RCVS. RCVS comprise a group of disorders characterized by prolonged but reversible vasoconstriction of the cerebral arteries, usually associated with acute-onset, severe, recurrent headaches, with or without additional neurologic signs and symptoms.[1] Description of this rare entity dates back to 1960s[2] and was limited to case reports. Call et al. reviewed the literature and published a case series titled ‘Reversible Cerebral Segmental Vasoconstriction’ in 1988.[3] Calabrese et al.[1] brought the awareness of this entity by their reviews. It is usually a disease of young and middle aged females. The exact incidence is unknown. Approximately 60% of cases are secondary, mainly postpartum period, eclampsia, exposure to vasoactive substances [4] such as cannabis, cocaine, immunosuppressants, sympathomimetics, methergine, bromocriptine etc. Although the pathophysiology remains
a
d
c
b
e
Figure 1: Magnetic resonance imaging brain fluid-attenuated inversion recovery (FLAIR) sequence showing acute parenchymal hemorrhage in right frontal lobe (a) left temporo-occipital lobe (b) diffuse FLAIR hyperintensity of bihemispheric sulcal spaces suggestive of subarachnoid hemorrhage (c) Magnetic resonance-angiography of brain showing marked diffuse narrowing of supraclinoid segments of bilateral ICA, M1, A1 and P1 segments (d) and showing normal vessels after 2 months (e)
91
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.
