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Video 1 available on www.neurologyindia.com
b
c a
d
Figure 1: (a) Patient having exaggerated forward flexion of neck with “plank-like” rigidity of abdominal muscles. (b) Needle electromyography of the patient in rectus abdominis muscle right side showing spontaneous motor unit activity. (c) Continuous hypersynchronous electromyography activity seen during spasms. (d) Improvement in the electromyography activity after diazepam
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128332
Received: 04-01-2014 Review completed: 13-01-2014 Accepted: 30-01-2014
(50 mg/day). Due to financial constraint intravenous immunoglobulins could not be administered to the patient. Instead intravenous methylprednisolone (1 g/day × 5 days) was given. Patient showed remarkable relief from painful spasms and rigidity [Video 1]. Follow-up at 2 months, pain was alleviated with diazepam and baclofen.
Diaphragma sella meningioma presenting with posterior cerebral artery infarct: Case report and review of literature
SPS is a rare immunological disorder characterized by progressive rigidity, painful spasms and continuous motor activity. [1] The hallmark of SPS is persistent muscular stiffness due to continuous co-activation of agonist and antagonist muscles, particularly “core muscles” that is the paraspinal and abdominal muscles.[2] Treatment ideally should involve symptom relief by baclofen, diazepam, and immunomodulation by intravenous immunoglubulin, plasmapheresis or in refractory cases by rituximab; but head to head trials are still lacking. [3] Corticosteroids have been used in cases which do not respond to high doses of baclofen. This was supported by results of Meinck[4] which stated a satisfactory response can be achieved by corticosteroids used as pulse therapy and as long-term maintenance in SPS.
Sir, Meningiomas are common benign intracranial tumors and being slow-growing tumors they often present with features of elevated intracranial pressure (ICP) or neurological deficit. However, it is very rare for a meningioma to present with cerebral infarction.[1-3] We report one such case.
Bhawna Sharma, Kadam Nagpal, Swayam Prakash, Pankaj Gupta Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1. 2.
3. 4.
Moersch FP, Woltman HW. Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin 1956;31:421-7. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: Clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1998;65:633-40. Holmøy T, Geis C. The immunological basis for treatment of stiff person syndrome. J Neuroimmunol 2011;231:55-60. Meinck HM. Stiff man syndrome. CNS Drugs 2001;15:515-26.
Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
A 55-year-old female patient presented with rapidly progressive worsening of vision in bilateral eyes and progressive drowsiness. She had been having localized forehead headache for many years and worsening of vision for last 6 months. On examination, she was drowsy, but obeying simple commands, in the left eye no perception of light, finger counting close to face in the right eye and no focal deficits. Computed tomography (CT) scan of the head revealed a large extra-axial tumor (4 cm × 4 cm × 3 cm) arising from the region of diaphragma sella causing mass effect on midline structures and also a hypodensity (infarct) in the entire posterior cerebral artery (PCA) territory. Magnetic resonance imaging brain showed a large tumor arising from diaphragma sella, hypointense on T1-WI, hyperintense on T2-WI, with homogenous enhancement, suggestive of diaphragm sella meningioma with mass effect on both the anterior cerebral arteries (ACA), internal carotid arteries (ICA) and PCAs [Figure 1a-c]. In addition, there was a PCA territory infarction on the left side with diffusion restriction and fluid attenuated inversion recovery hyperintensity [Figure 1d and e]. Magnetic resonance (MR)-angiography showed occlusion of left PCA [Figure 1f]. 77
[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
a
b
c
d
e
f
In this patient, there might be two mechanisms for PCA infarct. On mechanism might be direct involvement and infiltration of PCA with the tumor causing occlusion and infarct. However, there was no such evidence at surgery. The other mechanism might be possibly compression of left PCA from the tumor causing stasis and thrombosis of PCA, leading to infarct. MR-angiogram in this case showed occlusion of left PCA causing infarct. Another less likely mechanism might be meningioma causing raised ICP, leading to transtentorial herniation causing PCA infarct. However, in this case there is no such evidence on imaging.
Rakshith Shetty, Raman Mohan Sharma, Paritosh Pandey
Figure 1: (a-c) Magnetic resonance imaging (MRI) axial, coronal and sagittal sequences showing a large diaphragm sella meningioma with retrosellar extension; (d) diffusion-weighted MRI showing acute infarct in left posterior cerebral artery (PCA) territory; (e) fluid attenuated inversion recovery images showing the tumor as well as left PCA infarct; (f) MRA showing left P1 segment occlusion
She was taken up for left sided pterional craniotomy and excision of menigioma. The optic nerves were displaced superior-laterally and were separated from the tumor. The attachment was found to be over the diaphragm sella and after detaching the same, the tumor was excised completely. She made an uneventful recovery, and her right eye vision improved. A postoperative CT scan did not show any residual lesion or hematoma. At 3-month follow-up, she was well, except for the visual deficits. Suprasellar meningiomas typically present with visual symptoms and raised ICP.[4] However, meningiomas presenting with ischemic stroke is very rare. Even when there is encasement and occlusion of large intracranial arteries by meningiomas, there is usually enough collateral supply, because the stenosis or occlusion is very gradual. There have been reports of meningiomas presenting as transient ischemic attacks.[5-7] However ischemic stroke as the presenting feature is extremely rare. Komotar et al. [2] in their study have reported two patients with meningioma presenting with infarct. Both patients had ICA encasement by the tumor and had middle cerebral artery territory infarct. They estimated the incidence of meningiomas presenting with cerebral infarct at 0.19%. Masuoka et al.[8] have reported a patient with small planum sphenoidale meningioma with ACA infarct. The right A2-segment of ACA was occluded by the tumor and caused an ACA infarct with resultant leg weakness. In all previous reported cases, the infarct was in anterior circulation. This was probably the first case of PCA in a patient with suprasellar meningioma.
78
Department of Neurosurgery, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India E-mail: [email protected]
References 1.
2. 3. 4. 5. 6. 7. 8.
Mathis S, Bataille B, Boucebci S, Jeantet M, Ciron J, Vandamme L, et al. A rare cause of stroke in young adults: Occlusion of the middle cerebral artery by a meningioma postpartum. Case Rep Neurol Med 2013;2013:652538. Komotar RJ, Keswani SC, Wityk RJ. Meningioma presenting as stroke: Report of two cases and estimation of incidence. J Neurol Neurosurg Psychiatry 2003;74:136-7. Bito S, Sakaki S. Case of middle fossa meningioma with rapid development of symptoms due to obstruction of the middle cerebral artery. No To Shinkei 1971;23:953-7. Kwancharoen R, Blitz AM, Tavares F, Caturegli P, Gallia GL, Salvatori R. Clinical features of sellar and suprasellar meningiomas. Pituitary 2013; PMID number PMID: 23975080 Published ahead of print. Oluigbo CO, Choudhari KA, Flynn P, McConnell RS. Meningioma presenting with transient ischaemic attacks. Br J Neurosurg 2004;18:635-7. Cameron EW. Transient ischaemic attacks due to meningioma – Report of 4 cases. Clin Radiol 1994;49:416-8. Ueno Y, Tanaka A, Nakayama Y. Transient neurological deficits simulating transient ischemic attacks in a patient with meningioma – Case report. Neurol Med Chir (Tokyo) 1998;38:661-5. Masuoka J, Yoshioka F, Ohgushi H, Kawashima M, Matsushima T. Meningioma manifesting as cerebral infarction. Neurol Med Chir (Tokyo) 2010;50:585-7. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128335
Received: 02-12-2013 Review completed: 18-12-2013 Accepted: 26-01-2014
Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
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.
Video 1 available on www.neurologyindia.com
b
c a
d
Figure 1: (a) Patient having exaggerated forward flexion of neck with “plank-like” rigidity of abdominal muscles. (b) Needle electromyography of the patient in rectus abdominis muscle right side showing spontaneous motor unit activity. (c) Continuous hypersynchronous electromyography activity seen during spasms. (d) Improvement in the electromyography activity after diazepam
Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128332
Received: 04-01-2014 Review completed: 13-01-2014 Accepted: 30-01-2014
(50 mg/day). Due to financial constraint intravenous immunoglobulins could not be administered to the patient. Instead intravenous methylprednisolone (1 g/day × 5 days) was given. Patient showed remarkable relief from painful spasms and rigidity [Video 1]. Follow-up at 2 months, pain was alleviated with diazepam and baclofen.
Diaphragma sella meningioma presenting with posterior cerebral artery infarct: Case report and review of literature
SPS is a rare immunological disorder characterized by progressive rigidity, painful spasms and continuous motor activity. [1] The hallmark of SPS is persistent muscular stiffness due to continuous co-activation of agonist and antagonist muscles, particularly “core muscles” that is the paraspinal and abdominal muscles.[2] Treatment ideally should involve symptom relief by baclofen, diazepam, and immunomodulation by intravenous immunoglubulin, plasmapheresis or in refractory cases by rituximab; but head to head trials are still lacking. [3] Corticosteroids have been used in cases which do not respond to high doses of baclofen. This was supported by results of Meinck[4] which stated a satisfactory response can be achieved by corticosteroids used as pulse therapy and as long-term maintenance in SPS.
Sir, Meningiomas are common benign intracranial tumors and being slow-growing tumors they often present with features of elevated intracranial pressure (ICP) or neurological deficit. However, it is very rare for a meningioma to present with cerebral infarction.[1-3] We report one such case.
Bhawna Sharma, Kadam Nagpal, Swayam Prakash, Pankaj Gupta Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1. 2.
3. 4.
Moersch FP, Woltman HW. Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin 1956;31:421-7. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: Clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry 1998;65:633-40. Holmøy T, Geis C. The immunological basis for treatment of stiff person syndrome. J Neuroimmunol 2011;231:55-60. Meinck HM. Stiff man syndrome. CNS Drugs 2001;15:515-26.
Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
A 55-year-old female patient presented with rapidly progressive worsening of vision in bilateral eyes and progressive drowsiness. She had been having localized forehead headache for many years and worsening of vision for last 6 months. On examination, she was drowsy, but obeying simple commands, in the left eye no perception of light, finger counting close to face in the right eye and no focal deficits. Computed tomography (CT) scan of the head revealed a large extra-axial tumor (4 cm × 4 cm × 3 cm) arising from the region of diaphragma sella causing mass effect on midline structures and also a hypodensity (infarct) in the entire posterior cerebral artery (PCA) territory. Magnetic resonance imaging brain showed a large tumor arising from diaphragma sella, hypointense on T1-WI, hyperintense on T2-WI, with homogenous enhancement, suggestive of diaphragm sella meningioma with mass effect on both the anterior cerebral arteries (ACA), internal carotid arteries (ICA) and PCAs [Figure 1a-c]. In addition, there was a PCA territory infarction on the left side with diffusion restriction and fluid attenuated inversion recovery hyperintensity [Figure 1d and e]. Magnetic resonance (MR)-angiography showed occlusion of left PCA [Figure 1f]. 77
[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
a
b
c
d
e
f
In this patient, there might be two mechanisms for PCA infarct. On mechanism might be direct involvement and infiltration of PCA with the tumor causing occlusion and infarct. However, there was no such evidence at surgery. The other mechanism might be possibly compression of left PCA from the tumor causing stasis and thrombosis of PCA, leading to infarct. MR-angiogram in this case showed occlusion of left PCA causing infarct. Another less likely mechanism might be meningioma causing raised ICP, leading to transtentorial herniation causing PCA infarct. However, in this case there is no such evidence on imaging.
Rakshith Shetty, Raman Mohan Sharma, Paritosh Pandey
Figure 1: (a-c) Magnetic resonance imaging (MRI) axial, coronal and sagittal sequences showing a large diaphragm sella meningioma with retrosellar extension; (d) diffusion-weighted MRI showing acute infarct in left posterior cerebral artery (PCA) territory; (e) fluid attenuated inversion recovery images showing the tumor as well as left PCA infarct; (f) MRA showing left P1 segment occlusion
She was taken up for left sided pterional craniotomy and excision of menigioma. The optic nerves were displaced superior-laterally and were separated from the tumor. The attachment was found to be over the diaphragm sella and after detaching the same, the tumor was excised completely. She made an uneventful recovery, and her right eye vision improved. A postoperative CT scan did not show any residual lesion or hematoma. At 3-month follow-up, she was well, except for the visual deficits. Suprasellar meningiomas typically present with visual symptoms and raised ICP.[4] However, meningiomas presenting with ischemic stroke is very rare. Even when there is encasement and occlusion of large intracranial arteries by meningiomas, there is usually enough collateral supply, because the stenosis or occlusion is very gradual. There have been reports of meningiomas presenting as transient ischemic attacks.[5-7] However ischemic stroke as the presenting feature is extremely rare. Komotar et al. [2] in their study have reported two patients with meningioma presenting with infarct. Both patients had ICA encasement by the tumor and had middle cerebral artery territory infarct. They estimated the incidence of meningiomas presenting with cerebral infarct at 0.19%. Masuoka et al.[8] have reported a patient with small planum sphenoidale meningioma with ACA infarct. The right A2-segment of ACA was occluded by the tumor and caused an ACA infarct with resultant leg weakness. In all previous reported cases, the infarct was in anterior circulation. This was probably the first case of PCA in a patient with suprasellar meningioma.
78
Department of Neurosurgery, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India E-mail: [email protected]
References 1.
2. 3. 4. 5. 6. 7. 8.
Mathis S, Bataille B, Boucebci S, Jeantet M, Ciron J, Vandamme L, et al. A rare cause of stroke in young adults: Occlusion of the middle cerebral artery by a meningioma postpartum. Case Rep Neurol Med 2013;2013:652538. Komotar RJ, Keswani SC, Wityk RJ. Meningioma presenting as stroke: Report of two cases and estimation of incidence. J Neurol Neurosurg Psychiatry 2003;74:136-7. Bito S, Sakaki S. Case of middle fossa meningioma with rapid development of symptoms due to obstruction of the middle cerebral artery. No To Shinkei 1971;23:953-7. Kwancharoen R, Blitz AM, Tavares F, Caturegli P, Gallia GL, Salvatori R. Clinical features of sellar and suprasellar meningiomas. Pituitary 2013; PMID number PMID: 23975080 Published ahead of print. Oluigbo CO, Choudhari KA, Flynn P, McConnell RS. Meningioma presenting with transient ischaemic attacks. Br J Neurosurg 2004;18:635-7. Cameron EW. Transient ischaemic attacks due to meningioma – Report of 4 cases. Clin Radiol 1994;49:416-8. Ueno Y, Tanaka A, Nakayama Y. Transient neurological deficits simulating transient ischemic attacks in a patient with meningioma – Case report. Neurol Med Chir (Tokyo) 1998;38:661-5. Masuoka J, Yoshioka F, Ohgushi H, Kawashima M, Matsushima T. Meningioma manifesting as cerebral infarction. Neurol Med Chir (Tokyo) 2010;50:585-7. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.128335
Received: 02-12-2013 Review completed: 18-12-2013 Accepted: 26-01-2014
Neurology India | Jan-Feb 2014 | Vol 62 | Issue 1
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.
