- Email: [email protected]
Spinal imaging in intracranial primary pleomorphic xanthoastrocytoma with anaplastic features
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1299–1301
in patients with PD taking pramipexole. The authors found that those with a history of coronary artery disease were particularly susceptible.10 The mechanism responsible for oedema secondary to the use of pramipexole remains obscure; however, as dopamine is an important regulator of the sympathetic nervous system, secretion of aldosterone, as well as adenosine triphosphate-mediated sodium/ potassium channels, the peripheral effects of pramipexole at these levels could have a role.9 To our knowledge, this is the first report of severe chronic extensive oedema in a patient with PD, secondary to pramipexole. References 1. Shannon KM, Bennet Jr JP, Friedman JH. Efficacy of pramipexole, a novel dpaminergic agonist, as monotherapy in mild to moderate Parkinson´s disease. The Pramipexole Study Group. Neurology 1997;49:724–8. 2. Bianchi M, Castiglioni MG. Refractory generalized edema: an infrequent complication of long-term pergolide treatment for Parkinson disease. Clin Neuropharmacol 2005;28:245–6.
1299
3. Williams AF, Franks PJ, Moffatt CJ. Lymphoedema: estimating the size of the problem. Palliat Med 2005;19:300–13. 4. Husmann MJ, Roedel C, Leu AJ, et al. Lymphoedema, lymphatic microangiopathy and increased lymphatic and interstitial pressure in a patient with Parkinson’s disease. Schweiz Med Wochenschr 1999;129:410–2. 5. Opie LH. Calcium channel antagonists part III: Use and comparative efficacy in hypertensive and supraventricular arrhytmias. Minor indications. Cardiovasc Drugs Ther 1988;1:625–56. 6. Schnapp P, Hermann H, Cernak P, et al. Nifedipine monotherapy in the hypertensive elderly: a placebo-controlled clinical trial. Curr Med Res Opin 1987;10:407–13. 7. Williams SA, Rayman G, Tooke JE. Dependent oedema and attenuation of postural vasoconstriction associated with nifedipine therapy for hypertension in diabetic patients. Eur J Clin Pharmacol 1989;37:333–5. 8. Tan EK, Ondo W. Clinical characteristics of pramipexole-induced peripheral edema. Arch Neurol 2000;57:729–32. 9. Biglan KM, Holloway Jr RG, McDermott MP, et al. Risk factors for somnolence, edema, and hallucinations in early Parkinson disease. Neurology 2007;69:187–95. 10. Kleiner-Fisman G, Fisman DN. Risk factors for the development of pedal edema in patients using pramipexole. Arch Neurol 2007;64:820–4.
doi:http://dx.doi.org/10.1016/j.jocn.2011.12.012
Spinal imaging in intracranial primary pleomorphic xanthoastrocytoma with anaplastic features Christian Nern a,⇑, Jürgen Hench b, Arne Fischmann a a b
Department of Radiology and Nuclear Medicine, University Hospital Basel, Petersgraben 4, CH-4031 Basel, Switzerland Institute for Pathology, Department for Neuropathology and Ophthalmopathology, University Hospital Basel, Basel, Switzerland
a r t i c l e
i n f o
Article history: Received 27 September 2011 Accepted 6 December 2011
Keywords: Anaplastic features Brain tumor Pleomorphic xanthoastrocytoma Radiological follow-up Spinal manifestation
a b s t r a c t We present a patient with an intracranial primary pleomorphic xanthoastrocytoma (PXA) with anaplastic features that recurred repeatedly after surgery. Late in the course, radiological follow-up revealed an unresectable spinal tumor. Very few patients with PXA associated with a spinal tumor have been reported. Earlier detection of the spinal lesion would have potentially improved the therapeutic options for the patient. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic brain tumor that accounts for less than 1% of all astrocytomas and predominantly occurs in young adults. These tumors histologically correspond to the World Health Organization (WHO) grade II and have a relatively good prognosis with 72% recurrence-free survival after five years.1 However, malignant transformation has been described. PXA that show increased mitotic activity and/or necrosis at initial presentation are classified as primary PXA with anaplastic features and show an increased risk of early recurrence. According to the literature, only 27 patients with primary anaplastic PXA have been reported prior to 2009.2 Only a few of these patients developed spinal tumors.
A 57-year-old male patient presented with focal anopsia and headaches for eight weeks. Brain MRI (Fig. 1A, B) showed a tumor mass with cystic and solid portions in the right temporal lobe with enlargement of the anterior part of the temporal horn of the right lateral ventricle. During surgical resection, the right temporal horn was opened and complete macroscopic resection was achieved. The intraoperative appearance of the tumor was xanthomatous. The histopathological examination (Supplementary Fig. 1) showed highly pleomorphic tumor cells with astrocytic differentiation, induction of reticulin fibers and focal expression of CD34. Prominent lymphocytic infiltration and a xanthomatous tumor component were also observed. In addition, signs of malignancy, including focal necrosis and focally increased mitotic activity, were found. Vascular proliferation was absent. Mutation analysis revealed IDH1/2 and BRAF wild-type alleles. The tumor was classified as PXA with anaplastic features.
⇑ Corresponding author. Tel.: +41 762 151035; fax: +41 32 5129798. E-mail address: [email protected] (C. Nern).
1300
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1299–1301
Fig. 1. (A, B) Preoperative brain MRI of a patient with intracranial primary pleomorphic xanthoastrocytoma with anaplastic features: (A) axial T2-weighted MRI showing a large tumor within the right temporal lobe with solid and cystic portions – the temporal horn of the right lateral ventricle is expanded (asterisk); (B) coronal contrast enhanced (CE) T1-weighted MRI showing enhancement of the tumor border with focal nodular enhancement (arrow); (C) sagittal and (D) axial CE-T1-weighted MRI spinal MRI showing an intramedullary tumor mass in continuity with an intradural extramedullary component (arrowheads). TH1 = first thoracic vertebral body.
The patient received postoperative fractionated radiotherapy (30 2 Gy) and exhibited complete remission of symptoms. An MRI follow-up 10 months later showed recurrence of the lesion. Thus, surgical resection was performed, during which the trigone of the right lateral ventricle was opened. Ependymal tumor infiltration was observed, therefore only subtotal resection could be achieved. Histology again confirmed PXA with anaplastic features, without progression to glioblastoma. The disease progressed despite chemotherapy with temozolomide (three cycles) followed by bevacizumab (15 cycles). One year after the second resection, a temporary weakness of the lower extremities was observed. These symptoms were initially ameliorated with stereotactic radiotherapy (10 3.5 Gy) combined with topotecan. Four months later, the patient presented with apparent paresis of both legs and the left arm. An MRI of the whole spine showed a single contrast-enhancing intramedullary lesion at C4–TH1 with focal extension to the meninges (Fig. 1C, D). The patient received palliative radiotherapy to the spine (10 3 Gy) combined with dexamethasone (24 mg per day), which temporarily reduced the symptoms. Two months after detection to the spinal lesion, the patient died from respiratory insufficiency, which was probably caused by progressive involvement of the spinal cord and brain stem.
3. Discussion A histopathological distinction between PXA with anaplastic features and glioblastoma multiforme can be challenging since both entities share high cellular pleomorphism and meet morphological criteria of malignancy.
In the patient reported here, histological examination revealed features of PXA with anaplastic features. Mutation analysis can be helpful in differential diagnosis of gliomas. Two recent studies3,4 describe a high frequency, approximately 65%, of BRAF V600E mutations in World Health Organization (WHO) grade II PXA. These mutations are found only very rarely in glioblastoma multiforme. The frequency of BRAF mutations in anaplastic PXA varies in the literature from 17%4 to 65%.3 IDH1/2 mutations are typically not observed in PXA and therefore may be helpful in discriminating these tumors from diffuse astrocytomas, which frequently show IDH1/2 mutations. The finding of wild-type alleles for these three genes in anaplastic PXA is in line with the studies mentioned above. In MRI and CT scans, PXA most commonly present as well-circumscribed cystic tumors with an enhancing mural nodule attached to the meninges, predominantly located in the temporal lobes.5 In our patient, the intracranial tumor showed most of these hallmarks with the exception of distinct meningeal attachment. The spinal intramedullary lesion showed focal contact with the meninges. The detection of an intradural extramedullary tumor component (as illustrated in Fig. 1D) may support the hypothesis that the spinal cord was invaded secondarily after leptomeningeal spread. However, no further lesions were observed along the rest of the spine, which suggests that this may be a true, initially intramedullary metastasis, although these are very rare. In the absence of a biopsy or autopsy examination of the spinal lesion, an entity different from PXA with anaplastic features cannot be excluded. However, infiltration of the lateral ventricles and the time course of the onset of the lesion argue for an associated spinal manifestation, potentially with progression to a more malignant tumor type. Spinal spread of secondary PXA with anaplastic features is not uncommon and occurs in approximately one-third of patients.6 Very few patients with primary PXA with anaplastic features associated with spinal tumor have been reported. Thus, the prevalence might be underestimated, and it is important to be aware of this potentially fatal complication. Adverse effects of treatment with bevacizumab can include increased tumor infiltration and distant tumor recurrence.7 It remains possible that bevacizumab contributed to the increased infiltration of the ventricular system and concomitant spinal spread. Opening of the ventricular system during surgery, as performed in this patient, may also have triggered spinal spread. Due to the poor understanding of the biological behavior of PXA with anaplastic features, a WHO grade has not yet been defined. However, a WHO grade of III has been suggested. Current therapeutic options are limited to surgery and palliative radio-chemotherapy. Recently, a patient with long-term control of secondary PXA with anaplastic features by means of stereotactic irradiation of intracranial and spinal tumor masses has been reported.6 4. Conclusion Our report illustrates the unpredictable biological behavior of primary PXA with anaplastic features. The late detection of an unresectable spinal tumor mass underlines the importance of expanding radiological follow-up to imaging of the spine. Earlier detection of the spinal involvement might have improved the therapeutic options and potentially ameliorated both quality of life and clinical outcome of this patient. Disclosure/Conflict of Interest The authors acknowledge financial support from Bracco, Switzerland. The study sponsor had no role in the study design, collec-
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1301–1302
tion, analysis, or interpretation of data; writing of the report, or decision to submit this work for publication. Acknowledgements The brain-MRI-dataset was generously provided by the Medical Radiologic Institute, Zürich, Switzerland. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.12.010. References
1301
2. Okazaki T, Kageji T, Matsuzaki K, et al. Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis–a pediatric case report and review of the literature. J Neurooncol 2009;94:431–7. 3. Schindler G, Capper D, Meyer J, et al. Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol 2011;121:397–405. 4. Dias-Santagata D, Lam Q, Vernovsky K, et al. BRAF V600E mutations are common in pleomorphic xanthoastrocytoma: diagnostic and therapeutic implications. PLoS ONE 2011;6:e17948. 5. Tonn JC, Paulus W, Warmuth-Metz M, et al. Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls. Surg Neurol 1997;47:162–9. 6. Koga T, Morita A, Maruyama K, et al. Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation. Neuro Oncol 2009;11:446–51. 7. Zuniga RM, Torcuator R, Jain R, et al. Efficacy, safety and patterns of response and recurrence in patients with recurrent high-grade gliomas treated with bevacizumab plus irinotecan. J Neurooncol 2009;91:329–36.
1. Louis DN, Ohgaki H, Wiestler OD, et al. WHO Classification of Tumors of the Central Nervous System. fourth ed. Lyon: IARC; 2007. doi:http://dx.doi.org/10.1016/j.jocn.2011.12.010
Brachial neuritis with phrenic nerve involvement Allison Barraclough a,c,⇑, James Triplett a,c, Philip Tuch a,b a
Department of Neurology, Hollywood Private Hospital, Nedlands, Western Australia, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia c Department of Neurology, Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, Western Australia 6009, Australia b
a r t i c l e
i n f o
Article history: Received 4 December 2011 Accepted 10 December 2011
Keywords: Brachial neuritis Neuralgic amyotrophy Phrenic nerve Parsonage-Turner syndrome
a b s t r a c t We report a 73-year-old woman with a complicated occurrence of brachial neuritis (BN) with phrenic nerve involvement. Our patient developed shortness of breath (ShOB) post coronary artery bypass graft secondary to phrenic nerve palsy and in the following year developed right arm pain and weakness. Electromyography confirmed the diagnosis of BN. Despite the passage of time, the ShOB worsened. This was initially attributed to the ongoing post-operative phrenic nerve palsy but on further investigation it was found to be related to the BN process. BN is an uncommon entity and when associated with distant nerve involvement, diagnostic confusion can ensue. Further difficulties arise when the patient has underlying co-morbidities that also affect nerve function such as diabetes. This article aims to explore these issues and examine the literature for prognosis and management considerations. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Brachial neuritis (BN) is an uncommon condition which classically presents with an acute or subacute onset of unilateral shoulder and arm pain followed by weakness and atrophy of the upper arm. Sensory symptoms occur commonly and generally manifest as hyperesthesia and/or paraesthesia. While any nerve in the brachial plexus can be affected, the disease process is most commonly focused around the upper and middle trunk distribution. Simultaneous involvement of other peripheral nerves has been described.1 We report a patient with BN with phrenic nerve involvement.
2. Case report A 73-year-old female awoke with neck stiffness gradually worsening in intensity. It became more concentrated to the right side
⇑ Corresponding author. Tel.: +61 8 9346 3088. E-mail address: [email protected] (A. Barraclough).
and radiated down the same arm like an ‘‘electric shock’’. There was corresponding numbness over the right shoulder with decreased strength in her right hand. Two months later the neck and arm pain resolved but weakness remained. The patient’s medical history includes well-controlled type 2 diabetes (on insulin), hypertension, sleep apnoea and ischemic heart disease. There was a past history of low-grade, left-sided breast cancer. A curative mastectomy was performed and no radiation or chemotherapy was required. A further breast cancer was discovered on the right two years later and was managed in the same way. MRI of the cervical spine and right brachial plexus was performed with no abnormality detected. Initial electromyography (EMG) was performed very early in the disease process, at a time where the syndrome was still evolving. It showed patchy mild involvement of C5–7 innervated muscles. In the year preceding her symptom onset, the patient underwent a coronary artery bypass graft (CABG) and developed shortness of breath (ShOB) post-operatively. She was found to have paralysis of the left hemidiaphragm secondary to a phrenic nerve injury sustained during surgery. With the development of the right arm weakness her ShOB worsened and she complained
in patients with PD taking pramipexole. The authors found that those with a history of coronary artery disease were particularly susceptible.10 The mechanism responsible for oedema secondary to the use of pramipexole remains obscure; however, as dopamine is an important regulator of the sympathetic nervous system, secretion of aldosterone, as well as adenosine triphosphate-mediated sodium/ potassium channels, the peripheral effects of pramipexole at these levels could have a role.9 To our knowledge, this is the first report of severe chronic extensive oedema in a patient with PD, secondary to pramipexole. References 1. Shannon KM, Bennet Jr JP, Friedman JH. Efficacy of pramipexole, a novel dpaminergic agonist, as monotherapy in mild to moderate Parkinson´s disease. The Pramipexole Study Group. Neurology 1997;49:724–8. 2. Bianchi M, Castiglioni MG. Refractory generalized edema: an infrequent complication of long-term pergolide treatment for Parkinson disease. Clin Neuropharmacol 2005;28:245–6.
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3. Williams AF, Franks PJ, Moffatt CJ. Lymphoedema: estimating the size of the problem. Palliat Med 2005;19:300–13. 4. Husmann MJ, Roedel C, Leu AJ, et al. Lymphoedema, lymphatic microangiopathy and increased lymphatic and interstitial pressure in a patient with Parkinson’s disease. Schweiz Med Wochenschr 1999;129:410–2. 5. Opie LH. Calcium channel antagonists part III: Use and comparative efficacy in hypertensive and supraventricular arrhytmias. Minor indications. Cardiovasc Drugs Ther 1988;1:625–56. 6. Schnapp P, Hermann H, Cernak P, et al. Nifedipine monotherapy in the hypertensive elderly: a placebo-controlled clinical trial. Curr Med Res Opin 1987;10:407–13. 7. Williams SA, Rayman G, Tooke JE. Dependent oedema and attenuation of postural vasoconstriction associated with nifedipine therapy for hypertension in diabetic patients. Eur J Clin Pharmacol 1989;37:333–5. 8. Tan EK, Ondo W. Clinical characteristics of pramipexole-induced peripheral edema. Arch Neurol 2000;57:729–32. 9. Biglan KM, Holloway Jr RG, McDermott MP, et al. Risk factors for somnolence, edema, and hallucinations in early Parkinson disease. Neurology 2007;69:187–95. 10. Kleiner-Fisman G, Fisman DN. Risk factors for the development of pedal edema in patients using pramipexole. Arch Neurol 2007;64:820–4.
doi:http://dx.doi.org/10.1016/j.jocn.2011.12.012
Spinal imaging in intracranial primary pleomorphic xanthoastrocytoma with anaplastic features Christian Nern a,⇑, Jürgen Hench b, Arne Fischmann a a b
Department of Radiology and Nuclear Medicine, University Hospital Basel, Petersgraben 4, CH-4031 Basel, Switzerland Institute for Pathology, Department for Neuropathology and Ophthalmopathology, University Hospital Basel, Basel, Switzerland
a r t i c l e
i n f o
Article history: Received 27 September 2011 Accepted 6 December 2011
Keywords: Anaplastic features Brain tumor Pleomorphic xanthoastrocytoma Radiological follow-up Spinal manifestation
a b s t r a c t We present a patient with an intracranial primary pleomorphic xanthoastrocytoma (PXA) with anaplastic features that recurred repeatedly after surgery. Late in the course, radiological follow-up revealed an unresectable spinal tumor. Very few patients with PXA associated with a spinal tumor have been reported. Earlier detection of the spinal lesion would have potentially improved the therapeutic options for the patient. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic brain tumor that accounts for less than 1% of all astrocytomas and predominantly occurs in young adults. These tumors histologically correspond to the World Health Organization (WHO) grade II and have a relatively good prognosis with 72% recurrence-free survival after five years.1 However, malignant transformation has been described. PXA that show increased mitotic activity and/or necrosis at initial presentation are classified as primary PXA with anaplastic features and show an increased risk of early recurrence. According to the literature, only 27 patients with primary anaplastic PXA have been reported prior to 2009.2 Only a few of these patients developed spinal tumors.
A 57-year-old male patient presented with focal anopsia and headaches for eight weeks. Brain MRI (Fig. 1A, B) showed a tumor mass with cystic and solid portions in the right temporal lobe with enlargement of the anterior part of the temporal horn of the right lateral ventricle. During surgical resection, the right temporal horn was opened and complete macroscopic resection was achieved. The intraoperative appearance of the tumor was xanthomatous. The histopathological examination (Supplementary Fig. 1) showed highly pleomorphic tumor cells with astrocytic differentiation, induction of reticulin fibers and focal expression of CD34. Prominent lymphocytic infiltration and a xanthomatous tumor component were also observed. In addition, signs of malignancy, including focal necrosis and focally increased mitotic activity, were found. Vascular proliferation was absent. Mutation analysis revealed IDH1/2 and BRAF wild-type alleles. The tumor was classified as PXA with anaplastic features.
⇑ Corresponding author. Tel.: +41 762 151035; fax: +41 32 5129798. E-mail address: [email protected] (C. Nern).
1300
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1299–1301
Fig. 1. (A, B) Preoperative brain MRI of a patient with intracranial primary pleomorphic xanthoastrocytoma with anaplastic features: (A) axial T2-weighted MRI showing a large tumor within the right temporal lobe with solid and cystic portions – the temporal horn of the right lateral ventricle is expanded (asterisk); (B) coronal contrast enhanced (CE) T1-weighted MRI showing enhancement of the tumor border with focal nodular enhancement (arrow); (C) sagittal and (D) axial CE-T1-weighted MRI spinal MRI showing an intramedullary tumor mass in continuity with an intradural extramedullary component (arrowheads). TH1 = first thoracic vertebral body.
The patient received postoperative fractionated radiotherapy (30 2 Gy) and exhibited complete remission of symptoms. An MRI follow-up 10 months later showed recurrence of the lesion. Thus, surgical resection was performed, during which the trigone of the right lateral ventricle was opened. Ependymal tumor infiltration was observed, therefore only subtotal resection could be achieved. Histology again confirmed PXA with anaplastic features, without progression to glioblastoma. The disease progressed despite chemotherapy with temozolomide (three cycles) followed by bevacizumab (15 cycles). One year after the second resection, a temporary weakness of the lower extremities was observed. These symptoms were initially ameliorated with stereotactic radiotherapy (10 3.5 Gy) combined with topotecan. Four months later, the patient presented with apparent paresis of both legs and the left arm. An MRI of the whole spine showed a single contrast-enhancing intramedullary lesion at C4–TH1 with focal extension to the meninges (Fig. 1C, D). The patient received palliative radiotherapy to the spine (10 3 Gy) combined with dexamethasone (24 mg per day), which temporarily reduced the symptoms. Two months after detection to the spinal lesion, the patient died from respiratory insufficiency, which was probably caused by progressive involvement of the spinal cord and brain stem.
3. Discussion A histopathological distinction between PXA with anaplastic features and glioblastoma multiforme can be challenging since both entities share high cellular pleomorphism and meet morphological criteria of malignancy.
In the patient reported here, histological examination revealed features of PXA with anaplastic features. Mutation analysis can be helpful in differential diagnosis of gliomas. Two recent studies3,4 describe a high frequency, approximately 65%, of BRAF V600E mutations in World Health Organization (WHO) grade II PXA. These mutations are found only very rarely in glioblastoma multiforme. The frequency of BRAF mutations in anaplastic PXA varies in the literature from 17%4 to 65%.3 IDH1/2 mutations are typically not observed in PXA and therefore may be helpful in discriminating these tumors from diffuse astrocytomas, which frequently show IDH1/2 mutations. The finding of wild-type alleles for these three genes in anaplastic PXA is in line with the studies mentioned above. In MRI and CT scans, PXA most commonly present as well-circumscribed cystic tumors with an enhancing mural nodule attached to the meninges, predominantly located in the temporal lobes.5 In our patient, the intracranial tumor showed most of these hallmarks with the exception of distinct meningeal attachment. The spinal intramedullary lesion showed focal contact with the meninges. The detection of an intradural extramedullary tumor component (as illustrated in Fig. 1D) may support the hypothesis that the spinal cord was invaded secondarily after leptomeningeal spread. However, no further lesions were observed along the rest of the spine, which suggests that this may be a true, initially intramedullary metastasis, although these are very rare. In the absence of a biopsy or autopsy examination of the spinal lesion, an entity different from PXA with anaplastic features cannot be excluded. However, infiltration of the lateral ventricles and the time course of the onset of the lesion argue for an associated spinal manifestation, potentially with progression to a more malignant tumor type. Spinal spread of secondary PXA with anaplastic features is not uncommon and occurs in approximately one-third of patients.6 Very few patients with primary PXA with anaplastic features associated with spinal tumor have been reported. Thus, the prevalence might be underestimated, and it is important to be aware of this potentially fatal complication. Adverse effects of treatment with bevacizumab can include increased tumor infiltration and distant tumor recurrence.7 It remains possible that bevacizumab contributed to the increased infiltration of the ventricular system and concomitant spinal spread. Opening of the ventricular system during surgery, as performed in this patient, may also have triggered spinal spread. Due to the poor understanding of the biological behavior of PXA with anaplastic features, a WHO grade has not yet been defined. However, a WHO grade of III has been suggested. Current therapeutic options are limited to surgery and palliative radio-chemotherapy. Recently, a patient with long-term control of secondary PXA with anaplastic features by means of stereotactic irradiation of intracranial and spinal tumor masses has been reported.6 4. Conclusion Our report illustrates the unpredictable biological behavior of primary PXA with anaplastic features. The late detection of an unresectable spinal tumor mass underlines the importance of expanding radiological follow-up to imaging of the spine. Earlier detection of the spinal involvement might have improved the therapeutic options and potentially ameliorated both quality of life and clinical outcome of this patient. Disclosure/Conflict of Interest The authors acknowledge financial support from Bracco, Switzerland. The study sponsor had no role in the study design, collec-
Case Reports / Journal of Clinical Neuroscience 19 (2012) 1301–1302
tion, analysis, or interpretation of data; writing of the report, or decision to submit this work for publication. Acknowledgements The brain-MRI-dataset was generously provided by the Medical Radiologic Institute, Zürich, Switzerland. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2011.12.010. References
1301
2. Okazaki T, Kageji T, Matsuzaki K, et al. Primary anaplastic pleomorphic xanthoastrocytoma with widespread neuroaxis dissemination at diagnosis–a pediatric case report and review of the literature. J Neurooncol 2009;94:431–7. 3. Schindler G, Capper D, Meyer J, et al. Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol 2011;121:397–405. 4. Dias-Santagata D, Lam Q, Vernovsky K, et al. BRAF V600E mutations are common in pleomorphic xanthoastrocytoma: diagnostic and therapeutic implications. PLoS ONE 2011;6:e17948. 5. Tonn JC, Paulus W, Warmuth-Metz M, et al. Pleomorphic xanthoastrocytoma: report of six cases with special consideration of diagnostic and therapeutic pitfalls. Surg Neurol 1997;47:162–9. 6. Koga T, Morita A, Maruyama K, et al. Long-term control of disseminated pleomorphic xanthoastrocytoma with anaplastic features by means of stereotactic irradiation. Neuro Oncol 2009;11:446–51. 7. Zuniga RM, Torcuator R, Jain R, et al. Efficacy, safety and patterns of response and recurrence in patients with recurrent high-grade gliomas treated with bevacizumab plus irinotecan. J Neurooncol 2009;91:329–36.
1. Louis DN, Ohgaki H, Wiestler OD, et al. WHO Classification of Tumors of the Central Nervous System. fourth ed. Lyon: IARC; 2007. doi:http://dx.doi.org/10.1016/j.jocn.2011.12.010
Brachial neuritis with phrenic nerve involvement Allison Barraclough a,c,⇑, James Triplett a,c, Philip Tuch a,b a
Department of Neurology, Hollywood Private Hospital, Nedlands, Western Australia, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia c Department of Neurology, Sir Charles Gairdner Hospital, Hospital Avenue, Nedlands, Western Australia 6009, Australia b
a r t i c l e
i n f o
Article history: Received 4 December 2011 Accepted 10 December 2011
Keywords: Brachial neuritis Neuralgic amyotrophy Phrenic nerve Parsonage-Turner syndrome
a b s t r a c t We report a 73-year-old woman with a complicated occurrence of brachial neuritis (BN) with phrenic nerve involvement. Our patient developed shortness of breath (ShOB) post coronary artery bypass graft secondary to phrenic nerve palsy and in the following year developed right arm pain and weakness. Electromyography confirmed the diagnosis of BN. Despite the passage of time, the ShOB worsened. This was initially attributed to the ongoing post-operative phrenic nerve palsy but on further investigation it was found to be related to the BN process. BN is an uncommon entity and when associated with distant nerve involvement, diagnostic confusion can ensue. Further difficulties arise when the patient has underlying co-morbidities that also affect nerve function such as diabetes. This article aims to explore these issues and examine the literature for prognosis and management considerations. Ó 2012 Elsevier Ltd. All rights reserved.
1. Introduction Brachial neuritis (BN) is an uncommon condition which classically presents with an acute or subacute onset of unilateral shoulder and arm pain followed by weakness and atrophy of the upper arm. Sensory symptoms occur commonly and generally manifest as hyperesthesia and/or paraesthesia. While any nerve in the brachial plexus can be affected, the disease process is most commonly focused around the upper and middle trunk distribution. Simultaneous involvement of other peripheral nerves has been described.1 We report a patient with BN with phrenic nerve involvement.
2. Case report A 73-year-old female awoke with neck stiffness gradually worsening in intensity. It became more concentrated to the right side
⇑ Corresponding author. Tel.: +61 8 9346 3088. E-mail address: [email protected] (A. Barraclough).
and radiated down the same arm like an ‘‘electric shock’’. There was corresponding numbness over the right shoulder with decreased strength in her right hand. Two months later the neck and arm pain resolved but weakness remained. The patient’s medical history includes well-controlled type 2 diabetes (on insulin), hypertension, sleep apnoea and ischemic heart disease. There was a past history of low-grade, left-sided breast cancer. A curative mastectomy was performed and no radiation or chemotherapy was required. A further breast cancer was discovered on the right two years later and was managed in the same way. MRI of the cervical spine and right brachial plexus was performed with no abnormality detected. Initial electromyography (EMG) was performed very early in the disease process, at a time where the syndrome was still evolving. It showed patchy mild involvement of C5–7 innervated muscles. In the year preceding her symptom onset, the patient underwent a coronary artery bypass graft (CABG) and developed shortness of breath (ShOB) post-operatively. She was found to have paralysis of the left hemidiaphragm secondary to a phrenic nerve injury sustained during surgery. With the development of the right arm weakness her ShOB worsened and she complained