- Email: [email protected]
Reversible cerebral vasoconstriction following carotid endarterectomy
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Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728
We could not find a previous report in the literature of a prostate carcinoma metastasising to an atypical meningioma, as occurred in this patient. Given the propensity of prostate metastases to be durally based, at times with a dural tail on neuroimaging, the investigative challenges of the presented patient are considerable, requiring a definitive tissue diagnosis. Moreover, prostate metastases to meningioma have been rarely described,8,9 adding to the diagnostic difficulty. References
Fig. 4. Post-operative axial fluid attenuated inversion recovery (FLAIR) brain MRI showing that total macroscopic excision had been achieved.
1. Benjamin R. Neurologic complications of prostate cancer. Am Fam Physician 2002;65:1834–40. 2. Tremont-Lukats IW, Bobustuc G, Lagos GK, et al. Brain metastasis from prostate carcinoma. Cancer 2003;98:363–8. 3. Tsai V, Kim S, Clatterbuck RE, et al. Cystic prostate metastases to the brain parenchyma: report of two cases and review of the literature. J Neurooncol 2001;51:167–73. 4. Nayak L, Abrey LE, Iwamoto FM. Intracranial dural metastases. Cancer 2009;115:1947–53. 5. Behrens B, Husain MM, Schmidley JW. Cystic solitary intracerebral metastasis from prostate adenocarcinoma. Neuroradiology 2001;43:162–4. 6. Tagle P, Villanueva P, Torreabla G, et al. Intracranial metastasis or meningioma? An uncommon clinical diagnostic dilemma. Surg Neurol 2002;58:241–5. 7. Lath CO, Khanna PC, Gadewar S, et al. Intracranial metastasis from prostatic adenocarcinoma simulating a meningioma. Australas Radiol 2005;49:497–500. 8. Doering L. Metastasis of carcinoma of prostate to meningioma. Virchows Archiv – A Pathol Anat Histol 1975;366:87–91. 9. Bernstein RA, Grumet KA, Wetzel N. Metastasis of prostatic carcinoma to intracranial meningioma. J Neurosurg 1983;53:774–7.
doi:10.1016/j.jocn.2011.03.019
Reversible cerebral vasoconstriction following carotid endarterectomy Teddy Y. Wu a, Richard W. Frith b, P. Alan Barber c,⇑ a b c
Department of Neurology, Christchurch Hospital, Christchurch, New Zealand Department of Neurology, Auckland City Hospital, Auckland, New Zealand Department of Medicine, Faculty of Medicine and Health Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand
a r t i c l e
i n f o
Article history: Received 17 February 2011 Accepted 25 March 2011
a b s t r a c t Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe ‘‘thunderclap’’ headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction. We report a patient with RCVS following carotid endarterectomy and review the literature on RCVS following carotid revascularisation.
Keywords: Carotid endarterectomy Headache Reversible vasoconstriction syndrome Stroke Vasospasm
1. Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe ‘‘thunderclap’’ headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction.1 RCVS has been described in patients of all ages but most commonly occurs in the fifth decade of life and has a female preponderance.1,2 The pathophysiology of RCVS remains unknown but is thought likely to be due to a transient disturbance of
Ó 2011 Elsevier Ltd. All rights reserved.
cerebral vascular autoregulation leading to segmental and multifocal arterial constriction and dilatation.2 Primary RCVS can occur without any clear precipitant but may also be secondary; due to recent use of a vasoactive substance, pheochromocytoma, or occuring in the postpartum period.1,2 We report a patient with RCVS following carotid endarterectomy (CEA) and review the literature on RCVS following carotid revascularisation.
2. Case report ⇑ Corresponding author. Tel.: +64 9 307 4949; fax: +64 9 375 4309. E-mail addresses: [email protected], [email protected] (P.A. Barber).
A 58-year-old woman underwent an elective right CEA for asymptomatic 95% internal carotid artery (ICA) stenosis. She was
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Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728
upper and lower limb weakness. The electrocardiogram (ECG) showed sinus rhythm and a subsequent 24-hour ECG was normal. Routine blood tests including inflammatory markers, and thrombophilia and connective tissue screens were normal. A urine toxicology screen was negative for cannabis, opiates and methamphetamines. A cerebrospinal fluid (CSF) examination was normal. The patient’s MRI showed acute infarction in the right frontal and parietal regions (Fig. 1). Magnetic resonance angiography (MRA) showed that the right ICA was widely patent but there were multiple segmental diameter irregularities in the branches of the right middle cerebral artery (MCA), proximal anterior cerebral artery (ACA) and posterior cerebral artery (PCA). A dominant right posterior communicating artery (PCOM) supplied the right occipital lobe. The patient was treated with intravenous heparin but she experienced a further episode of headache with left-sided visual blurring. A repeat MRI brain scan showed new areas of ischemia in the right parietal and upper occipital region (Fig. 1, row 2). The MRA showed persistent multifocal irregular stenoses of the right ACA, MCA, PCOM and PCA thought consistent with vasoconstriction (Fig. 1, row 2). The patient was commenced on oral nimodipine 30 mg 6-hourly on day 21 and heparin was stopped. She experienced no further headaches or other neurological symptoms and made a full neurologic recovery. She was discharged directly home on oral nimodipine and aspirin. An MRI brain scan at 52 days postCEA showed no new areas of infarction and resolution of the segmental vasoconstriction (Fig. 1, row 3).
3. Discussion
Fig. 1. Neuroimaging of a 58-year-old woman with reversible cerebral vasoconstriction syndrome at: (row 1) day 19 post-carotid endarterectomy (CEA) – (a) axial diffusion-weighted imaging (DWI) showing acute borderzone infarction in the right frontal subcortical white matter and (b) magnetic resonance angiography (MRA) showing segmental constriction of the right anterior cerebral artery (ACA), middle cerebral artery (MCA) and posterior cerebral artery (PCA) (arrows); (row 2) at day 21 post-CEA – (a) DWI showing new regions of borderzone infarction in the right frontal, parietal and upper occipital white matter and (b) MRA showing persistent segmental constriction of the right ACA, MCA and PCA (arrows); and (row 3) at day 52 post-CEA – (a) axial T2-weighted MRI showing evidence of patchy borderzone infarction and (b) MRA showing resolution of the segmental vasoconstriction.
a heavy smoker and had a background of hypertension and depression. She had been prescribed the selective serotonergic reuptake inhibitor (SSRI) fluoxetine for depression but had stopped this medication 3 weeks prior to the CEA. Three days after the CEA she developed recurrent sudden onset, severe right fronto-temporal headaches that were associated with nausea and episodes of transient mild left-sided weakness and clumsiness. These episodes persisted and at 14 days post-CEA, she was assessed at an emergency department and was noted to have a normal neurological examination. A non-contrast CT brain scan was normal. She was discharged without specific therapy. At 18 days post-CEA, she presented to our unit with sudden onset of a severe, right-sided headache associated with weakness of the left upper and lower limbs. On examination, she was afebrile and had a pulse of 74 beats per minute and a blood pressure of 120/70 mmHg. There was moderate pyramidal distribution left
To our knowledge we report only the sixth patient with RCVS following CEA. The diagnosis of RCVS was made on the basis of recurrent severe thunderclap headaches and neurological symptoms. Cerebral infarction and vasoconstriction were isolated to the ICA territory on the same side as the CEA, including the right occipital lobe which was supplied by a dominant right PCOM. Primary central nervous system vasculitis (PCNSV) was excluded with a normal CSF examination and resolution of symptoms without immunosuppressive therapy. The patient had been taking an SSRI but had stopped this 3 weeks prior to the CEA and had not been taking other vasoactive substances. A summary of reported cases of RCVS following CEA is presented in Table 1. Patients with post-CEA RCVS develop neurological symptoms between 2 days and 8 days following surgery. Headache was present in five of six patients and all patients had neurological symptoms ipsilateral to the CEA. Cerebral infarction was present in four of six patients. The two previously reported patients without infarction did not undergo MRI or have follow-up CT scans so infarction may have been missed. Cerebral vasoconstriction was demonstrated by catheter angiogram in four patients and by serial non-invasive vascular imaging in two patients. Vasoconstriction was ipsilateral to the CEA in four patients and was bilateral in two patients. The mechanism by which CEA precipitates vasospasm is not clear but a direct mechanical relationship is suggested by the observation of ipsilateral vasospasm in four of six reported patients. We, and others, postulate that severe ICA stenosis may lead to chronic cerebral hypoperfusion and a disturbance of cerebral autoregulation. Following CEA, a relative hyperperfusion state may induce an accentuated vasoconstrictive response in susceptible individuals.5,6 This postulated direct mechanical relationship would suggest that RCVS may also follow carotid angioplasty and stenting, but to our knowledge this has not yet been reported.
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Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728 Table 1 Reports of patients with carotid endarterectomy (CEA) and reversible cerebral vasoconstriction syndrome (RCVS) YearRef
19903
19974
20055
20076
Our patient
Summary
No. patients Age (years), sex
1 57, M
1 62, M
1 54, F
1 58, F
6 55.8 (±3.76)*
Days post-CEA
8
5
2
3
5.5 (±2.36)**
Side of surgery
R
L
L
R
4 R/3 L
Neurologic symptoms
Confusion, paranoia, and agitation+
Recurrent transient aphasia, R arm weakness
Aphasia, R hand numbness
L sided weakness, visual blurring
Headache Imaging
Yes CT, DSA
No CT, DSA
5 of 6
No
Yes CT/CTA MRI, TCD L hemisphere borderzone
Yes CT and MRI/MRA
Acute infarction
R frontal, parietal, occipital
4 of 6
Vasoconstriction
Ipsilateral
2 Patient 1: 50, M Patient 2: 54, F Patient 1: 7 Patient 2: 8 Patient 1: R Patient 2: R and L Patient 1: L visual loss, transient L-sided numbness Patient 2: nausea, L homonymous hemianopia Yes Patient 1: CT, MRI, DSA Patient 2: CT, DSA, TCD Patient 1: R frontal and occipital Patient 2: R parietal, occipital Patient 1: bilateral Patient 2: bilateral
Ipsilateral
Ipsilateral
Ipsilateral
4 Ipsilateral, 2 bilateral
Treatment
None
Diltiazem
p.o. nimodipine
Outcome
Complete recovery
p.o. nimodipine i.v. magnesium Complete recovery
Patient Patient Patient Patient
1: 2: 1: 2:
corticosteroids verapamil not reported visual deficit
No
Complete recovery
Complete recovery
4 of 5 Complete recovery
CTA = CT angiogram, DSA = digital subtraction angiography, F = female, i.v. = intravenous, M = male, p.o. = per oral, MRA = magnetic resonance angiography, TCD = transcranial Doppler. Mean age (± standard deviation, SD). ** Mean no. of days (± SD). + Patient’s electroencephalogram (EEG) demonstrated right hemispheric periodic lateralized epileptiform discharges (PLED). Either ipsilateral or bilateral to the operated carotid artery. *
The differential diagnosis in patients with neurological symptoms following CEA includes perioperative cerebral ischemia from arterial dissection, re-stenosis, or thrombotic occlusion or distal embolisation of the operated vessel. The International Carotid Stenting Study (ICSS) MRI substudy, found that 14 of 107 (13%) patients with CEA had at least one new ipsilateral postoperative DWI lesion, which was much greater than the thirty day CEA related ischaemic stroke rate of 3%.7 Cerebral hyperperfusion syndrome (CHS), subarachnoid hemorrhage and PCNSV are the other major differential diagnoses to be considered. CHS is characterized by headaches, neurological deficits or seizures. CHS patients have evidence of vasogenic cerebral oedema or hemorrhage ipsilateral to the CEA, with a greater than 100% increase in cerebral perfusion relative to preoperative levels.8 In PCNSV there is evidence of widespread CNS inflammation and a CSF pleocytosis is usually seen.9 Investigation of patients with neurological symptoms following CEA must include neuroimaging. MRI is more sensitive for demonstrating acute infarction and vasogenic cerebral oedema and is preferred over CT scans. Imaging of the cervical and intracranial vessels with MRA, CT angiography (CTA) or digital subtraction angiography is required to rule out local complications of carotid surgery and to look for evidence of cerebral vasoconstriction. Catheter angiography is the gold standard for diagnosing cerebral vasoconstriction but MRA and CTA provide a less invasive alternative and allow assessment of the vessel lumen and wall. Repeat imaging within 12 weeks should demonstrate total or near complete resolution of vasoconstriction to confirm the diagnosis of RCVS. Patients should also have a urine drug screen looking for the presence of vasoactive substances, a vasculitis screen and a CSF examination. Evidence-based treatment for post-CEA RCVS has not been established due to the rarity of this condition. Two reported patients with RCVS following CEA were treated with nimodipine and a further two patients received non-dihydropyridine calcium
channel blockers. Our patient had recurrent headaches and neurological symptoms on most days from day 3 following the CEA and these resolved almost immediately after commencing nimodipine on day 21. While this apparent treatment response to nimodipine may well have been coincidental, the temporal relationship between starting this medication and cessation of symptoms suggests a therapeutic effect. While the symptoms of post-CEA RCVS may be dramatic, neurologic recovery was the usual outcome and four of the five reported patients, where this information was available, made a full recovery, with the fifth patient left with a mild deficit only. In summary, RVCS is a rare complication of CEA and must be considered in patients with new onset headaches, and neurological symptoms and signs, in the days following carotid surgery. It must be distinguished from benign primary headache syndromes, CHS, PCNSV and arterial dissection, and thrombotic occlusion or cerebral embolism from the operated carotid artery. Diagnosis requires vascular imaging demonstrating cerebral segmental vasoconstriction and reversibility within 12 weeks of symptom onset. Treatment should be aimed at reducing vasoconstriction and a trial of nimodipine therapy may be considered.
References 1. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101. 2. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009;9:256–67. 3. Brick JF, Dunker RO, Guitierrez AL. Cerebral vasoconstriction as a complication of carotid endarterectomy. J Neurosurg 1990;73:151–3. 4. Lopez-Valdes E, Chang HM, Pessin MS, et al. Cerebral vasoconstriction after carotid surgery. Neurology 1997;49:303–4. 5. Dagher HN, Shum MK, Campellone JV. Delayed intracranial vasospasm following carotid endarterectomy. Cerebrovasc Dis 2005;20:205–6.
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6. Rosenbloom MH, Singhal AB. CT angiography and diffusion-perfusion MR imaging in a patient with ipsilateral reversible cerebral vasoconstriction after carotid endarterectomy. AJNR Am J Neuroradiol 2007;28:920–2. 7. Bonati LH, Jongen LM, Haller S, et al. New ischaemic brain lesions on MRI after stenting or endarterectomy for symptomatic carotid stenosis: a substudy of the International Carotid Stenting Study (ICSS). Lancet Neurol 2010;9:353–62.
8. van Mook WN, Rennenberg RJ, Schurink GW, et al. Cerebral hyperperfusion syndrome. Lancet Neurol 2005;4:877–88. 9. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 1988;67:20–39.
doi:10.1016/j.jocn.2011.03.018
A clinicopathologic correlation in osteoblastoma of the spine in a child Akash J. Patel, Benjamin D. Fox, Daniel K. Fahim, Daniel H. Fulkerson, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, Andrew Jea ⇑ Division of Pediatric Neurosurgery, Texas Children’s Hospital, Department of Neurosurgery, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, Texas 77030, USA
a r t i c l e
i n f o
Article history: Received 10 March 2011 Accepted 21 March 2011
Keywords: Child Inflammatory Osteoblastoma Pathology Pediatrics Reactive bone Spine
a b s t r a c t Spinal osteoblastomas are considered benign tumors but can be locally aggressive. Patients usually present with pain and undergo radiologic and histologic work-up to establish a diagnosis. Osteoblastomas have discordant appearances on CT scans and MRI because of the inflammatory response seen on MRI that characterizes these tumors – the ‘‘flare’’ phenomenon. Solely using MRI can lead to over-resection, as signal abnormality may include areas devoid of tumor. There are a few reports of this phenomenon in the radiology literature, but to our knowledge, there are none in neurosurgery journals. We report an 11-year-old boy who presented with back pain and radiculopathy with an osteoblastoma at the L4 level. We totally excised the lesion to definitively treat the patient but also, based on MRI findings, differentially biopsied portions of the lesion and correlated them to the imaging studies, to confirm that the intense reactive portion of the lesion was devoid of tumor cells.
1. Introduction In 1932, Henry Jaffe first described an osteoblastic, osteoid-tissue forming tumor.1 It was redefined in 1935 as a lesion less than 2 cm in diameter and named ‘‘osteoid osteoma’’.2 Both Jaffe3 and Lichtenstein4 independently coined the term ‘‘benign osteoblastoma’’ in 1956. Osteoid osteomas and osteoblastomas are rare primary bone tumors that frequently involve the long bones and sometimes the spine. These tumors are histologically similar, but they are distinguished by size. Osteoid osteomas are typically lesions <2 cm in diameter. Classically, these patients present with pain. While osteoid osteomas are typically treated with pain control, osteoblastomas are reportedly capable of malignant transformation.5,6 Osteoblastomas, along with other primary bones tumors, are thought to elicit a localized inflammatory response within the bone that can lead to an aggressive MRI appearance.7–9 However, this area of marrow edema and inflammation is free of tumor cells. In the present study, we sought to differentially sample the various portions of the lesion based on MRI findings and to then correlate the results with the pathologic findings. We were able to confirm that the intense reactive portion of the lesion was devoid of tumor cells. To our knowledge, this has not been previously reported in the neurosurgical literature.
Ó 2011 Elsevier Ltd. All rights reserved.
er onset shooting pain in the right lower extremity. He did not have weakness, sensory changes, or incontinence. His physical examination, aside from an antalgic gait, was unremarkable. MRI demonstrated a focal, infiltrative lesion with contrast enhancement involving the right L4 posterior elements (Fig. 1A and B). There was extraosseous extension into the epidural space with compression of the thecal sac at L4/5 and compression of the L4 nerve root. CT scans further delineated extensive sclerotic changes in the right L4 posterior elements, pedicle, and body (Fig. 1C). 2.1. Surgery The patient initially underwent a CT-guided needle biopsy to establish a diagnosis. The biopsy trajectory was planned such that the tract could be included in a potential en bloc resection in the event of a sarcoma. Pathology was consistent with osteoblastoma. After discussion with the family, the patient underwent surgery via a lateral extracavitary approach (for piecemeal removal of the right hemilamina and pedicle) and transverse process (for gross total resection of the tumor, including sampling of the surrounding MRI signal abnormality). The patient then underwent an L3 to L5 posterior instrumented fusion. The patient’s postoperative course was unremarkable. 2.2. Pathology
2. Case report An 11-year-old boy with Asperger’s syndrome presented to the clinic with a 1.5-year history of worsening low back pain and new⇑ Corresponding author. Tel.: +1 832 822 3950; fax: +1 832 825 3072. E-mail address: [email protected] (A. Jea).
Separate specimens were sent to pathology. Biopsies taken from the discrete tumor mass based on MRI findings demonstrated areas of neoplastic, spindle-shaped cells within an interspersed network of osteoid with bone formation and fibroconnective tissue (Fig. 2A). There was no evidence of mitosis or necrosis. Samples taken from the tissue surrounding the tumor that showed signal abnormality
Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728
We could not find a previous report in the literature of a prostate carcinoma metastasising to an atypical meningioma, as occurred in this patient. Given the propensity of prostate metastases to be durally based, at times with a dural tail on neuroimaging, the investigative challenges of the presented patient are considerable, requiring a definitive tissue diagnosis. Moreover, prostate metastases to meningioma have been rarely described,8,9 adding to the diagnostic difficulty. References
Fig. 4. Post-operative axial fluid attenuated inversion recovery (FLAIR) brain MRI showing that total macroscopic excision had been achieved.
1. Benjamin R. Neurologic complications of prostate cancer. Am Fam Physician 2002;65:1834–40. 2. Tremont-Lukats IW, Bobustuc G, Lagos GK, et al. Brain metastasis from prostate carcinoma. Cancer 2003;98:363–8. 3. Tsai V, Kim S, Clatterbuck RE, et al. Cystic prostate metastases to the brain parenchyma: report of two cases and review of the literature. J Neurooncol 2001;51:167–73. 4. Nayak L, Abrey LE, Iwamoto FM. Intracranial dural metastases. Cancer 2009;115:1947–53. 5. Behrens B, Husain MM, Schmidley JW. Cystic solitary intracerebral metastasis from prostate adenocarcinoma. Neuroradiology 2001;43:162–4. 6. Tagle P, Villanueva P, Torreabla G, et al. Intracranial metastasis or meningioma? An uncommon clinical diagnostic dilemma. Surg Neurol 2002;58:241–5. 7. Lath CO, Khanna PC, Gadewar S, et al. Intracranial metastasis from prostatic adenocarcinoma simulating a meningioma. Australas Radiol 2005;49:497–500. 8. Doering L. Metastasis of carcinoma of prostate to meningioma. Virchows Archiv – A Pathol Anat Histol 1975;366:87–91. 9. Bernstein RA, Grumet KA, Wetzel N. Metastasis of prostatic carcinoma to intracranial meningioma. J Neurosurg 1983;53:774–7.
doi:10.1016/j.jocn.2011.03.019
Reversible cerebral vasoconstriction following carotid endarterectomy Teddy Y. Wu a, Richard W. Frith b, P. Alan Barber c,⇑ a b c
Department of Neurology, Christchurch Hospital, Christchurch, New Zealand Department of Neurology, Auckland City Hospital, Auckland, New Zealand Department of Medicine, Faculty of Medicine and Health Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand
a r t i c l e
i n f o
Article history: Received 17 February 2011 Accepted 25 March 2011
a b s t r a c t Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe ‘‘thunderclap’’ headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction. We report a patient with RCVS following carotid endarterectomy and review the literature on RCVS following carotid revascularisation.
Keywords: Carotid endarterectomy Headache Reversible vasoconstriction syndrome Stroke Vasospasm
1. Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe ‘‘thunderclap’’ headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction.1 RCVS has been described in patients of all ages but most commonly occurs in the fifth decade of life and has a female preponderance.1,2 The pathophysiology of RCVS remains unknown but is thought likely to be due to a transient disturbance of
Ó 2011 Elsevier Ltd. All rights reserved.
cerebral vascular autoregulation leading to segmental and multifocal arterial constriction and dilatation.2 Primary RCVS can occur without any clear precipitant but may also be secondary; due to recent use of a vasoactive substance, pheochromocytoma, or occuring in the postpartum period.1,2 We report a patient with RCVS following carotid endarterectomy (CEA) and review the literature on RCVS following carotid revascularisation.
2. Case report ⇑ Corresponding author. Tel.: +64 9 307 4949; fax: +64 9 375 4309. E-mail addresses: [email protected], [email protected] (P.A. Barber).
A 58-year-old woman underwent an elective right CEA for asymptomatic 95% internal carotid artery (ICA) stenosis. She was
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Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728
upper and lower limb weakness. The electrocardiogram (ECG) showed sinus rhythm and a subsequent 24-hour ECG was normal. Routine blood tests including inflammatory markers, and thrombophilia and connective tissue screens were normal. A urine toxicology screen was negative for cannabis, opiates and methamphetamines. A cerebrospinal fluid (CSF) examination was normal. The patient’s MRI showed acute infarction in the right frontal and parietal regions (Fig. 1). Magnetic resonance angiography (MRA) showed that the right ICA was widely patent but there were multiple segmental diameter irregularities in the branches of the right middle cerebral artery (MCA), proximal anterior cerebral artery (ACA) and posterior cerebral artery (PCA). A dominant right posterior communicating artery (PCOM) supplied the right occipital lobe. The patient was treated with intravenous heparin but she experienced a further episode of headache with left-sided visual blurring. A repeat MRI brain scan showed new areas of ischemia in the right parietal and upper occipital region (Fig. 1, row 2). The MRA showed persistent multifocal irregular stenoses of the right ACA, MCA, PCOM and PCA thought consistent with vasoconstriction (Fig. 1, row 2). The patient was commenced on oral nimodipine 30 mg 6-hourly on day 21 and heparin was stopped. She experienced no further headaches or other neurological symptoms and made a full neurologic recovery. She was discharged directly home on oral nimodipine and aspirin. An MRI brain scan at 52 days postCEA showed no new areas of infarction and resolution of the segmental vasoconstriction (Fig. 1, row 3).
3. Discussion
Fig. 1. Neuroimaging of a 58-year-old woman with reversible cerebral vasoconstriction syndrome at: (row 1) day 19 post-carotid endarterectomy (CEA) – (a) axial diffusion-weighted imaging (DWI) showing acute borderzone infarction in the right frontal subcortical white matter and (b) magnetic resonance angiography (MRA) showing segmental constriction of the right anterior cerebral artery (ACA), middle cerebral artery (MCA) and posterior cerebral artery (PCA) (arrows); (row 2) at day 21 post-CEA – (a) DWI showing new regions of borderzone infarction in the right frontal, parietal and upper occipital white matter and (b) MRA showing persistent segmental constriction of the right ACA, MCA and PCA (arrows); and (row 3) at day 52 post-CEA – (a) axial T2-weighted MRI showing evidence of patchy borderzone infarction and (b) MRA showing resolution of the segmental vasoconstriction.
a heavy smoker and had a background of hypertension and depression. She had been prescribed the selective serotonergic reuptake inhibitor (SSRI) fluoxetine for depression but had stopped this medication 3 weeks prior to the CEA. Three days after the CEA she developed recurrent sudden onset, severe right fronto-temporal headaches that were associated with nausea and episodes of transient mild left-sided weakness and clumsiness. These episodes persisted and at 14 days post-CEA, she was assessed at an emergency department and was noted to have a normal neurological examination. A non-contrast CT brain scan was normal. She was discharged without specific therapy. At 18 days post-CEA, she presented to our unit with sudden onset of a severe, right-sided headache associated with weakness of the left upper and lower limbs. On examination, she was afebrile and had a pulse of 74 beats per minute and a blood pressure of 120/70 mmHg. There was moderate pyramidal distribution left
To our knowledge we report only the sixth patient with RCVS following CEA. The diagnosis of RCVS was made on the basis of recurrent severe thunderclap headaches and neurological symptoms. Cerebral infarction and vasoconstriction were isolated to the ICA territory on the same side as the CEA, including the right occipital lobe which was supplied by a dominant right PCOM. Primary central nervous system vasculitis (PCNSV) was excluded with a normal CSF examination and resolution of symptoms without immunosuppressive therapy. The patient had been taking an SSRI but had stopped this 3 weeks prior to the CEA and had not been taking other vasoactive substances. A summary of reported cases of RCVS following CEA is presented in Table 1. Patients with post-CEA RCVS develop neurological symptoms between 2 days and 8 days following surgery. Headache was present in five of six patients and all patients had neurological symptoms ipsilateral to the CEA. Cerebral infarction was present in four of six patients. The two previously reported patients without infarction did not undergo MRI or have follow-up CT scans so infarction may have been missed. Cerebral vasoconstriction was demonstrated by catheter angiogram in four patients and by serial non-invasive vascular imaging in two patients. Vasoconstriction was ipsilateral to the CEA in four patients and was bilateral in two patients. The mechanism by which CEA precipitates vasospasm is not clear but a direct mechanical relationship is suggested by the observation of ipsilateral vasospasm in four of six reported patients. We, and others, postulate that severe ICA stenosis may lead to chronic cerebral hypoperfusion and a disturbance of cerebral autoregulation. Following CEA, a relative hyperperfusion state may induce an accentuated vasoconstrictive response in susceptible individuals.5,6 This postulated direct mechanical relationship would suggest that RCVS may also follow carotid angioplasty and stenting, but to our knowledge this has not yet been reported.
1727
Case Reports / Journal of Clinical Neuroscience 18 (2011) 1725–1728 Table 1 Reports of patients with carotid endarterectomy (CEA) and reversible cerebral vasoconstriction syndrome (RCVS) YearRef
19903
19974
20055
20076
Our patient
Summary
No. patients Age (years), sex
1 57, M
1 62, M
1 54, F
1 58, F
6 55.8 (±3.76)*
Days post-CEA
8
5
2
3
5.5 (±2.36)**
Side of surgery
R
L
L
R
4 R/3 L
Neurologic symptoms
Confusion, paranoia, and agitation+
Recurrent transient aphasia, R arm weakness
Aphasia, R hand numbness
L sided weakness, visual blurring
Headache Imaging
Yes CT, DSA
No CT, DSA
5 of 6
No
Yes CT/CTA MRI, TCD L hemisphere borderzone
Yes CT and MRI/MRA
Acute infarction
R frontal, parietal, occipital
4 of 6
Vasoconstriction
Ipsilateral
2 Patient 1: 50, M Patient 2: 54, F Patient 1: 7 Patient 2: 8 Patient 1: R Patient 2: R and L Patient 1: L visual loss, transient L-sided numbness Patient 2: nausea, L homonymous hemianopia Yes Patient 1: CT, MRI, DSA Patient 2: CT, DSA, TCD Patient 1: R frontal and occipital Patient 2: R parietal, occipital Patient 1: bilateral Patient 2: bilateral
Ipsilateral
Ipsilateral
Ipsilateral
4 Ipsilateral, 2 bilateral
Treatment
None
Diltiazem
p.o. nimodipine
Outcome
Complete recovery
p.o. nimodipine i.v. magnesium Complete recovery
Patient Patient Patient Patient
1: 2: 1: 2:
corticosteroids verapamil not reported visual deficit
No
Complete recovery
Complete recovery
4 of 5 Complete recovery
CTA = CT angiogram, DSA = digital subtraction angiography, F = female, i.v. = intravenous, M = male, p.o. = per oral, MRA = magnetic resonance angiography, TCD = transcranial Doppler. Mean age (± standard deviation, SD). ** Mean no. of days (± SD). + Patient’s electroencephalogram (EEG) demonstrated right hemispheric periodic lateralized epileptiform discharges (PLED). Either ipsilateral or bilateral to the operated carotid artery. *
The differential diagnosis in patients with neurological symptoms following CEA includes perioperative cerebral ischemia from arterial dissection, re-stenosis, or thrombotic occlusion or distal embolisation of the operated vessel. The International Carotid Stenting Study (ICSS) MRI substudy, found that 14 of 107 (13%) patients with CEA had at least one new ipsilateral postoperative DWI lesion, which was much greater than the thirty day CEA related ischaemic stroke rate of 3%.7 Cerebral hyperperfusion syndrome (CHS), subarachnoid hemorrhage and PCNSV are the other major differential diagnoses to be considered. CHS is characterized by headaches, neurological deficits or seizures. CHS patients have evidence of vasogenic cerebral oedema or hemorrhage ipsilateral to the CEA, with a greater than 100% increase in cerebral perfusion relative to preoperative levels.8 In PCNSV there is evidence of widespread CNS inflammation and a CSF pleocytosis is usually seen.9 Investigation of patients with neurological symptoms following CEA must include neuroimaging. MRI is more sensitive for demonstrating acute infarction and vasogenic cerebral oedema and is preferred over CT scans. Imaging of the cervical and intracranial vessels with MRA, CT angiography (CTA) or digital subtraction angiography is required to rule out local complications of carotid surgery and to look for evidence of cerebral vasoconstriction. Catheter angiography is the gold standard for diagnosing cerebral vasoconstriction but MRA and CTA provide a less invasive alternative and allow assessment of the vessel lumen and wall. Repeat imaging within 12 weeks should demonstrate total or near complete resolution of vasoconstriction to confirm the diagnosis of RCVS. Patients should also have a urine drug screen looking for the presence of vasoactive substances, a vasculitis screen and a CSF examination. Evidence-based treatment for post-CEA RCVS has not been established due to the rarity of this condition. Two reported patients with RCVS following CEA were treated with nimodipine and a further two patients received non-dihydropyridine calcium
channel blockers. Our patient had recurrent headaches and neurological symptoms on most days from day 3 following the CEA and these resolved almost immediately after commencing nimodipine on day 21. While this apparent treatment response to nimodipine may well have been coincidental, the temporal relationship between starting this medication and cessation of symptoms suggests a therapeutic effect. While the symptoms of post-CEA RCVS may be dramatic, neurologic recovery was the usual outcome and four of the five reported patients, where this information was available, made a full recovery, with the fifth patient left with a mild deficit only. In summary, RVCS is a rare complication of CEA and must be considered in patients with new onset headaches, and neurological symptoms and signs, in the days following carotid surgery. It must be distinguished from benign primary headache syndromes, CHS, PCNSV and arterial dissection, and thrombotic occlusion or cerebral embolism from the operated carotid artery. Diagnosis requires vascular imaging demonstrating cerebral segmental vasoconstriction and reversibility within 12 weeks of symptom onset. Treatment should be aimed at reducing vasoconstriction and a trial of nimodipine therapy may be considered.
References 1. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain 2007;130:3091–101. 2. Ducros A, Bousser MG. Reversible cerebral vasoconstriction syndrome. Pract Neurol 2009;9:256–67. 3. Brick JF, Dunker RO, Guitierrez AL. Cerebral vasoconstriction as a complication of carotid endarterectomy. J Neurosurg 1990;73:151–3. 4. Lopez-Valdes E, Chang HM, Pessin MS, et al. Cerebral vasoconstriction after carotid surgery. Neurology 1997;49:303–4. 5. Dagher HN, Shum MK, Campellone JV. Delayed intracranial vasospasm following carotid endarterectomy. Cerebrovasc Dis 2005;20:205–6.
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6. Rosenbloom MH, Singhal AB. CT angiography and diffusion-perfusion MR imaging in a patient with ipsilateral reversible cerebral vasoconstriction after carotid endarterectomy. AJNR Am J Neuroradiol 2007;28:920–2. 7. Bonati LH, Jongen LM, Haller S, et al. New ischaemic brain lesions on MRI after stenting or endarterectomy for symptomatic carotid stenosis: a substudy of the International Carotid Stenting Study (ICSS). Lancet Neurol 2010;9:353–62.
8. van Mook WN, Rennenberg RJ, Schurink GW, et al. Cerebral hyperperfusion syndrome. Lancet Neurol 2005;4:877–88. 9. Calabrese LH, Mallek JA. Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 1988;67:20–39.
doi:10.1016/j.jocn.2011.03.018
A clinicopathologic correlation in osteoblastoma of the spine in a child Akash J. Patel, Benjamin D. Fox, Daniel K. Fahim, Daniel H. Fulkerson, William E. Whitehead, Daniel J. Curry, Thomas G. Luerssen, Andrew Jea ⇑ Division of Pediatric Neurosurgery, Texas Children’s Hospital, Department of Neurosurgery, Baylor College of Medicine, 6621 Fannin Street, CCC 1230.01, 12th Floor, Houston, Texas 77030, USA
a r t i c l e
i n f o
Article history: Received 10 March 2011 Accepted 21 March 2011
Keywords: Child Inflammatory Osteoblastoma Pathology Pediatrics Reactive bone Spine
a b s t r a c t Spinal osteoblastomas are considered benign tumors but can be locally aggressive. Patients usually present with pain and undergo radiologic and histologic work-up to establish a diagnosis. Osteoblastomas have discordant appearances on CT scans and MRI because of the inflammatory response seen on MRI that characterizes these tumors – the ‘‘flare’’ phenomenon. Solely using MRI can lead to over-resection, as signal abnormality may include areas devoid of tumor. There are a few reports of this phenomenon in the radiology literature, but to our knowledge, there are none in neurosurgery journals. We report an 11-year-old boy who presented with back pain and radiculopathy with an osteoblastoma at the L4 level. We totally excised the lesion to definitively treat the patient but also, based on MRI findings, differentially biopsied portions of the lesion and correlated them to the imaging studies, to confirm that the intense reactive portion of the lesion was devoid of tumor cells.
1. Introduction In 1932, Henry Jaffe first described an osteoblastic, osteoid-tissue forming tumor.1 It was redefined in 1935 as a lesion less than 2 cm in diameter and named ‘‘osteoid osteoma’’.2 Both Jaffe3 and Lichtenstein4 independently coined the term ‘‘benign osteoblastoma’’ in 1956. Osteoid osteomas and osteoblastomas are rare primary bone tumors that frequently involve the long bones and sometimes the spine. These tumors are histologically similar, but they are distinguished by size. Osteoid osteomas are typically lesions <2 cm in diameter. Classically, these patients present with pain. While osteoid osteomas are typically treated with pain control, osteoblastomas are reportedly capable of malignant transformation.5,6 Osteoblastomas, along with other primary bones tumors, are thought to elicit a localized inflammatory response within the bone that can lead to an aggressive MRI appearance.7–9 However, this area of marrow edema and inflammation is free of tumor cells. In the present study, we sought to differentially sample the various portions of the lesion based on MRI findings and to then correlate the results with the pathologic findings. We were able to confirm that the intense reactive portion of the lesion was devoid of tumor cells. To our knowledge, this has not been previously reported in the neurosurgical literature.
Ó 2011 Elsevier Ltd. All rights reserved.
er onset shooting pain in the right lower extremity. He did not have weakness, sensory changes, or incontinence. His physical examination, aside from an antalgic gait, was unremarkable. MRI demonstrated a focal, infiltrative lesion with contrast enhancement involving the right L4 posterior elements (Fig. 1A and B). There was extraosseous extension into the epidural space with compression of the thecal sac at L4/5 and compression of the L4 nerve root. CT scans further delineated extensive sclerotic changes in the right L4 posterior elements, pedicle, and body (Fig. 1C). 2.1. Surgery The patient initially underwent a CT-guided needle biopsy to establish a diagnosis. The biopsy trajectory was planned such that the tract could be included in a potential en bloc resection in the event of a sarcoma. Pathology was consistent with osteoblastoma. After discussion with the family, the patient underwent surgery via a lateral extracavitary approach (for piecemeal removal of the right hemilamina and pedicle) and transverse process (for gross total resection of the tumor, including sampling of the surrounding MRI signal abnormality). The patient then underwent an L3 to L5 posterior instrumented fusion. The patient’s postoperative course was unremarkable. 2.2. Pathology
2. Case report An 11-year-old boy with Asperger’s syndrome presented to the clinic with a 1.5-year history of worsening low back pain and new⇑ Corresponding author. Tel.: +1 832 822 3950; fax: +1 832 825 3072. E-mail address: [email protected] (A. Jea).
Separate specimens were sent to pathology. Biopsies taken from the discrete tumor mass based on MRI findings demonstrated areas of neoplastic, spindle-shaped cells within an interspersed network of osteoid with bone formation and fibroconnective tissue (Fig. 2A). There was no evidence of mitosis or necrosis. Samples taken from the tissue surrounding the tumor that showed signal abnormality