- Email: [email protected]
Pleomorphic xanthoastrocytoma of the pineal region
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1439–1441
1439
Pleomorphic xanthoastrocytoma of the pineal region B.H. Srinivas a, Megha S. Uppin a, Manas K. Panigrahi b, M. Vijaya Saradhi a, Y. Jyotsna Rani c, Sundaram Challa a,* a b c
Department of Pathology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad 500082, Andhra Pradesh, India Department of Neurosurgery, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India Department of Radiology, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
a r t i c l e
i n f o
Article history: Received 7 January 2010 Accepted 4 February 2010
Keywords: Pleomorphic xanthoastrocytoma Pineal region Immunohistochemistry
a b s t r a c t Pleomorphic xanthoastrocytomas are indolent, astrocytic tumors usually located in the superficial cerebral cortex. They have not been described as occurring in the pineal region. We describe a patient with an astrocytic tumor arising in the pineal region that fulfilled all of the morphologic and immunohistochemical criteria of a pleomorphic xanthoastrocytoma. To our knowledge, this is the first description of a pleomorphic xanthoastrocytoma located in the pineal region. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction
2.1. Neuropathological findings
Tumors of the pineal region account for 0.4% to 1.0% of intracranial tumors in adults and 3.0% to 8.0% of brain tumors in children. They have a varied histology and are divided into germ cell and non–germ cell derivatives.1 Most tumors are the result of displaced embryonic tissue, malignant transformation of the pineal parenchymal cells or transformation of the surrounding astroglia.2 The occasional gliomas seen in the pineal region are usually pilocytic astrocytomas or glioblastoma multiformes, which are thought to arise from resident pineal astroglial cells.3,4 To the best of our knowledge, primary pleomorphic xanthoastrocytoma of the pineal region has not been described in the literature. We describe one such instance that showed the characteristic histological and immunohistochemical features of pleomorphic xanthoastrocytoma.
The tumor sections showed a cellular lesion comprised of mono- and multinucleated giant astrocytes with a high degree of pleomorphism. There were many pleomorphic giant cells arranged on a fibrillary background. The nuclei of these cells were bizarre, with prominent intranuclear inclusions. The cytoplasm was abundant and focally granular. Atypical mitotic figures or areas of necrosis were absent. The background showed numerous eosinophilic granular bodies (EGB; Fig. 2A,B). The intratumoral vessels showed perivascular lymphocytes and increased reticular deposition around the vessels. Interspersed between these pleomorphic cells, there were large xanthomatous cells with a multivacuolated appearance of the cytoplasm, pushing the nucleus to the periphery (Fig. 2C). Immunohistochemistry with glial fibrillary acidic protein (GFAP) and S-100 showed intense cytoplasmic positivity in the pleomorphic cells (Supplementary Fig. 1), whereas CD34 and synaptophysin immunohistochemistry was negative. The Ki-67% index of the tumor was less than 1%. Ultrastructural findings were not available.
2. Case report A 30-year-old man presented to our facility with gradual onset, progressive bifrontal headache for a period of 1 month. He was not hypertensive or diabetic. There was no history of vomiting, seizure or weakness. On examination, the higher motor functions and cranial nerves were normal. The gross motor and sensory system examination was also normal. Fundus examination revealed gross papilledema. A CT scan of the brain showed an isodense, wellcircumscribed tumor in the posterior third ventricle, resulting in hydrocephalus (Fig. 1A). The tumor enhanced with contrast administration and showed a speck of calcification. There was associated hydrocephalus. MRI revealed isointensity on T1weighted and hyperintensity on T2-weighted MRI, with contrast enhancement suggestive of pineocytoma (Fig. 1B). Initially, a ventriculoperitoneal shunt was inserted, followed by a transtentorial occipital craniotomy for total excision of the tumor. Operatively, the tumor was found to be grayish white in color, moderately firm, well defined and not able to be removed by suction. The tumor was entirely excised. Post-operatively, the patient developed an upward gaze palsy without other focal deficits. * Corresponding author. Tel.: +91 40 2348 9051; fax: +91 40 2331 0076. E-mail address: [email protected] (S. Challa).
2.2. Follow up The patient was alive and well 1 year after the primary operation. 3. Discussion First described by Kepes et al., pleomorphic xanthoastrocytoma is an uncommon brain tumor usually observed in the cerebral hemisphere of young adults; it is often associated with cyst formation.5,6 As per the World Health Organization (WHO) guidelines, the morphological and immunohistochemical features of our patient’s tumor were characteristic of a pleomorphic xanthoastrocytoma (WHO grade II).1 The main differential diagnostic consideration in this instance is of glioblastoma multiforme (GBM), owing to the high cellular pleomorphism. Moreover, GBM can occur in the pineal region, albeit rarely. However, the absence of mitoses and microvascular proliferation, the presence of EGB and the low proliferation index do not support the diagnosis of GBM.7 Another important consideration is that of pineal parenchymal tumors with astrocytic differentiation. These are known to occur in
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Case Reports / Journal of Clinical Neuroscience 17 (2010) 1439–1441
Fig. 1. (A) Axial brain CT scan with contrast showing an isodense lesion with a speck of calcification in the posterior third ventricle. There is hydrocephalus. (B) Sagittal T1weighted contrast enhanced brain MRI showing an enhancing homogenous lesion in the posterior third ventricle.
Fig. 2. Photographs of the histological examination of the tumor showing: (A) cells arranged on a fibrillary background with marked cellular pleomorphism (hematoxylin and eosin stain [H&E] 100); (B) bizarre giant cells with prominent intranuclear inclusions and a background showing eosinophilic granular bodies (H&E 400); and (C) xanthomatous cells with vacuolated cytoplasm (H&E 400). (This figure is available in colour at www.sciencedirect.com.)
older individuals and are relatively benign, as has been described by Borit and Blackwood, in their report on a 57-year-old man.8 Although the morphology of this type of tumor cell resembles that seen in our patient, the absence of synaptophysin immunoreactivity rules out the diagnosis of a primary pineal tumor. Nitta et al. described an atypical pleomorphic xanthoastrocytoma located in the pineal region.9 The patient was a 30-year-old woman who presented with hydrocephalus. The tumor showed marked pleomorphism and bizarre giant cells in the absence of mitosis or necrosis. However, the immunohistochemical profile was not consistent with that of pleomorphic xanthoastrocytoma. Snipes et al. have described two patients who had pleomorphic granular cell astrocytoma.10 Their findings were further strengthened by the ultrastructural findings of abundant mitochondria and neutral lipid. The histomorphology of these particular tumor cells resembles that seen in our patient, however, once again, the immunohistochemical profiles are different. The tumor we report showed distinct positivity for GFAP, which was absent in the tumor in their report. However, we could not support our diagnosis with ultrastructural findings. These tumors, under electron microscopy,
show lipid droplets, lysosomes and prominent basal lamina around the tumor cells. Malignant histiocytic tumors are also considered in the differential diagnosis of pleomorphic xanthoastrocytoma, probably because of the presence of xanthomatous cells. However, immunopositivity for S-100 protein rules out such a possibility. Despite the resemblance to a mesenchymal neoplasm, such as fibrous xanthoma, pleomorphic xanthoastrocytoma shows GFAP immunoreactivity.11 To our knowledge, this is the first report of a pleomorphic xanthoastrocytoma arising in the pineal region. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.02.022. References 1. Giannini C, Paulus W, Louis DN, et al. Pleomorphic xanthoastroma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1441–1442
2.
3. 4. 5.
classification of tumours. WHO classification of tumours of the central nervous system. 4th ed. Lyon: IARC Press. p. 22–4. Regis J, Bouillot P, Rouby-Volot F, et al. Pineal region tumors and the role of stereotactic biopsy: review of the mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery 1996;39:907–14. De Girolami U, Ambrustmache VW. Juvenile pilocytic astrocytoma of the pineal region: report of a case. Cancer 1982;50:1185–8. Vaquero J, Ramiro J, Martinez R. Glioblastoma multiforme of the pineal region. J Neurosurg Sci 1990;34:149–50. Kepes JJ, Rubinstein LJ, Eng LF. Meningocerebral xanthoastrocytoma. A distinctive glioma in young subjects, presumably originating from subpial astrocytes, with relatively favorable prognosis. Abstracts of the 8th International Congress of Neuropathology, Washington, DC. Am Assoc Neuropathol 1978;37:641.
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6. Kepes JJ. Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. Brain Pathol 1993;3:269–74. 7. Kepes JJ, Rubinstein LJ, Ansbacher L, et al. Histopathological features of recurrent pleomorphic xanthoastrocytoma: further corroboration of the glial nature of this neoplasm. Acta Neuropathol 1989;78:585–93. 8. Borit A, Blackwood W. Pineocytoma with astrocytomatous differentiation. J Neuropathol Exp Neurol 1979;38:253–8. 9. Nitta J, Tada T, Kyoshima K, et al. Atypical pleomorphic astrocytoma in the pineal gland: case report. Neurosurgery 2001;49:1458–61. 10. Snipes GJ, Horoupian DS, Shuer LM, et al. Pleomorphic granular cell astrocytoma of the pineal gland. Cancer 1992;70:2159–65. 11. Grant JW, Gallagher PJ. Pleomorphic xanthoastrocytoma: immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg Pathol 1986;10:336–41.
doi:10.1016/j.jocn.2010.02.022
Bilateral anterior opercular (Foix–Chavany–Marie) syndrome Dennis A. Nowak a,c,*, Gabriela Griebl a, Rainer Dabitz b, Günter Ochs b a b c
Neurologische Fachklinik, Klinik Kipfenberg, Kindinger Strasse 13, D-85110 Kipfenberg, Germany Neurologische Klinik, Klinikum Ingolstadt, Ingolstadt, Germany Department of Neurology, University of Erlangen, Germany
a r t i c l e
i n f o
Article history: Received 22 February 2010 Accepted 23 February 2010
Keywords: Foix–Chavany–Marie syndrome Bilateral anterior opercular syndrome Stroke
a b s t r a c t The bilateral anterior opercular (Foix–Chavany–Marie) syndrome is a rare condition most commonly encountered after bilateral middle cerebral artery stroke. Clinically, the syndrome is characterised by a loss of voluntary control of the facio-pharyngeo-glosso-masticatory muscles, while reflex movements and limb strength are preserved. We report the clinical presentation of a patient with anterior biopercular syndrome due to a bilateral middle cerebral artery stroke. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction The bilateral anterior opercular (Foix–Chavany–Marie) syndrome was first described in 1926.1 The syndrome is caused by bilateral lesions of the anterior frontoparietal opercula, most frequently due to ischaemic stroke.2 Voluntary movements of the cranial muscles innervated by the trigeminal, facial, glossopharyngeal, vagus and hypoglossus nerves are bilaterally lost.1,2 The affected individuals suffer from bilateral facial weakness and an inability to speak and chew, while the swallowing function is generally only mildly affected. There is dissociation between voluntary and automatic muscle activity: reflexes and automatic or emotional movements in those muscles that are voluntarily paralysed are well preserved,2–4 which means that the corneal reflex, the threaten reflex, and emotional laughing or crying are all possible.1,4 2. Case report A 79-year-old female with medically treated arterial hypertension and diabetes mellitus presented to our facility with bilateral facial weakness and a sudden inability to speak. She had completely recovered from an ischaemic stroke in the right middle
* Corresponding author at: Neurologische Fachklinik, Klinik Kipfenberg, Kindinger Strasse 13, D-85110 Kipfenberg, Germany. Tel.: +49 8465 175 100; fax: +49 8465 175 184. E-mail address: [email protected] (D.A. Nowak).
cerebral artery territory, which had occurred 6 years previously. Examination revealed anarthria, but well-preserved comprehension, writing and limb function. When requested, she was unable to speak or perform voluntary facial or tongue movements. She was only able to generate a groaning sound, and her swallowing function was mildly impaired. There were no deficits in somatosensory function or coordination. The deep tendon reflexes were symmetrically brisk and the Babinski sign was bilaterally negative. Swallowing reflexes and emotional facial movements were preserved. CT scans of her brain revealed a diffuse hypodensity within the right frontal and temporal lobes, the result of the middle cerebral artery stroke encompassing the frontoparietal opercular cortex, which had occurred 6 years earlier. In addition, the cortex–white matter differentiation and the gyral configuration of the left frontal opercular cortex were lost, indicative of an early middle cerebral artery stroke (Fig. 1A). CT angiography revealed occlusion of a temporal branch of the left middle cerebral artery. An acute left middle cerebral artery stroke causing bilateral anterior opercular (Foix– Chavany–Marie) syndrome was diagnosed, and intravenous recombinant tissue plasminogen activator was administered within 3 hours of the symptom onset. However, thrombolysis was ineffective and her neurological status did not change. CT scans performed 24 hours after the administration of thrombolysis revealed an unchanged area of the right-sided ischaemic stroke encompassing the frontoparietal opercular cortex and a new, leftsided ischaemic lesion of the frontoparietal opercular cortex (Fig. 1B).
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Pleomorphic xanthoastrocytoma of the pineal region B.H. Srinivas a, Megha S. Uppin a, Manas K. Panigrahi b, M. Vijaya Saradhi a, Y. Jyotsna Rani c, Sundaram Challa a,* a b c
Department of Pathology, Nizam’s Institute of Medical Sciences, Punjagutta, Hyderabad 500082, Andhra Pradesh, India Department of Neurosurgery, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India Department of Radiology, Nizam’s Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India
a r t i c l e
i n f o
Article history: Received 7 January 2010 Accepted 4 February 2010
Keywords: Pleomorphic xanthoastrocytoma Pineal region Immunohistochemistry
a b s t r a c t Pleomorphic xanthoastrocytomas are indolent, astrocytic tumors usually located in the superficial cerebral cortex. They have not been described as occurring in the pineal region. We describe a patient with an astrocytic tumor arising in the pineal region that fulfilled all of the morphologic and immunohistochemical criteria of a pleomorphic xanthoastrocytoma. To our knowledge, this is the first description of a pleomorphic xanthoastrocytoma located in the pineal region. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction
2.1. Neuropathological findings
Tumors of the pineal region account for 0.4% to 1.0% of intracranial tumors in adults and 3.0% to 8.0% of brain tumors in children. They have a varied histology and are divided into germ cell and non–germ cell derivatives.1 Most tumors are the result of displaced embryonic tissue, malignant transformation of the pineal parenchymal cells or transformation of the surrounding astroglia.2 The occasional gliomas seen in the pineal region are usually pilocytic astrocytomas or glioblastoma multiformes, which are thought to arise from resident pineal astroglial cells.3,4 To the best of our knowledge, primary pleomorphic xanthoastrocytoma of the pineal region has not been described in the literature. We describe one such instance that showed the characteristic histological and immunohistochemical features of pleomorphic xanthoastrocytoma.
The tumor sections showed a cellular lesion comprised of mono- and multinucleated giant astrocytes with a high degree of pleomorphism. There were many pleomorphic giant cells arranged on a fibrillary background. The nuclei of these cells were bizarre, with prominent intranuclear inclusions. The cytoplasm was abundant and focally granular. Atypical mitotic figures or areas of necrosis were absent. The background showed numerous eosinophilic granular bodies (EGB; Fig. 2A,B). The intratumoral vessels showed perivascular lymphocytes and increased reticular deposition around the vessels. Interspersed between these pleomorphic cells, there were large xanthomatous cells with a multivacuolated appearance of the cytoplasm, pushing the nucleus to the periphery (Fig. 2C). Immunohistochemistry with glial fibrillary acidic protein (GFAP) and S-100 showed intense cytoplasmic positivity in the pleomorphic cells (Supplementary Fig. 1), whereas CD34 and synaptophysin immunohistochemistry was negative. The Ki-67% index of the tumor was less than 1%. Ultrastructural findings were not available.
2. Case report A 30-year-old man presented to our facility with gradual onset, progressive bifrontal headache for a period of 1 month. He was not hypertensive or diabetic. There was no history of vomiting, seizure or weakness. On examination, the higher motor functions and cranial nerves were normal. The gross motor and sensory system examination was also normal. Fundus examination revealed gross papilledema. A CT scan of the brain showed an isodense, wellcircumscribed tumor in the posterior third ventricle, resulting in hydrocephalus (Fig. 1A). The tumor enhanced with contrast administration and showed a speck of calcification. There was associated hydrocephalus. MRI revealed isointensity on T1weighted and hyperintensity on T2-weighted MRI, with contrast enhancement suggestive of pineocytoma (Fig. 1B). Initially, a ventriculoperitoneal shunt was inserted, followed by a transtentorial occipital craniotomy for total excision of the tumor. Operatively, the tumor was found to be grayish white in color, moderately firm, well defined and not able to be removed by suction. The tumor was entirely excised. Post-operatively, the patient developed an upward gaze palsy without other focal deficits. * Corresponding author. Tel.: +91 40 2348 9051; fax: +91 40 2331 0076. E-mail address: [email protected] (S. Challa).
2.2. Follow up The patient was alive and well 1 year after the primary operation. 3. Discussion First described by Kepes et al., pleomorphic xanthoastrocytoma is an uncommon brain tumor usually observed in the cerebral hemisphere of young adults; it is often associated with cyst formation.5,6 As per the World Health Organization (WHO) guidelines, the morphological and immunohistochemical features of our patient’s tumor were characteristic of a pleomorphic xanthoastrocytoma (WHO grade II).1 The main differential diagnostic consideration in this instance is of glioblastoma multiforme (GBM), owing to the high cellular pleomorphism. Moreover, GBM can occur in the pineal region, albeit rarely. However, the absence of mitoses and microvascular proliferation, the presence of EGB and the low proliferation index do not support the diagnosis of GBM.7 Another important consideration is that of pineal parenchymal tumors with astrocytic differentiation. These are known to occur in
1440
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1439–1441
Fig. 1. (A) Axial brain CT scan with contrast showing an isodense lesion with a speck of calcification in the posterior third ventricle. There is hydrocephalus. (B) Sagittal T1weighted contrast enhanced brain MRI showing an enhancing homogenous lesion in the posterior third ventricle.
Fig. 2. Photographs of the histological examination of the tumor showing: (A) cells arranged on a fibrillary background with marked cellular pleomorphism (hematoxylin and eosin stain [H&E] 100); (B) bizarre giant cells with prominent intranuclear inclusions and a background showing eosinophilic granular bodies (H&E 400); and (C) xanthomatous cells with vacuolated cytoplasm (H&E 400). (This figure is available in colour at www.sciencedirect.com.)
older individuals and are relatively benign, as has been described by Borit and Blackwood, in their report on a 57-year-old man.8 Although the morphology of this type of tumor cell resembles that seen in our patient, the absence of synaptophysin immunoreactivity rules out the diagnosis of a primary pineal tumor. Nitta et al. described an atypical pleomorphic xanthoastrocytoma located in the pineal region.9 The patient was a 30-year-old woman who presented with hydrocephalus. The tumor showed marked pleomorphism and bizarre giant cells in the absence of mitosis or necrosis. However, the immunohistochemical profile was not consistent with that of pleomorphic xanthoastrocytoma. Snipes et al. have described two patients who had pleomorphic granular cell astrocytoma.10 Their findings were further strengthened by the ultrastructural findings of abundant mitochondria and neutral lipid. The histomorphology of these particular tumor cells resembles that seen in our patient, however, once again, the immunohistochemical profiles are different. The tumor we report showed distinct positivity for GFAP, which was absent in the tumor in their report. However, we could not support our diagnosis with ultrastructural findings. These tumors, under electron microscopy,
show lipid droplets, lysosomes and prominent basal lamina around the tumor cells. Malignant histiocytic tumors are also considered in the differential diagnosis of pleomorphic xanthoastrocytoma, probably because of the presence of xanthomatous cells. However, immunopositivity for S-100 protein rules out such a possibility. Despite the resemblance to a mesenchymal neoplasm, such as fibrous xanthoma, pleomorphic xanthoastrocytoma shows GFAP immunoreactivity.11 To our knowledge, this is the first report of a pleomorphic xanthoastrocytoma arising in the pineal region. Appendix A. Supplementary material Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2010.02.022. References 1. Giannini C, Paulus W, Louis DN, et al. Pleomorphic xanthoastroma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. World Health Organization
Case Reports / Journal of Clinical Neuroscience 17 (2010) 1441–1442
2.
3. 4. 5.
classification of tumours. WHO classification of tumours of the central nervous system. 4th ed. Lyon: IARC Press. p. 22–4. Regis J, Bouillot P, Rouby-Volot F, et al. Pineal region tumors and the role of stereotactic biopsy: review of the mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery 1996;39:907–14. De Girolami U, Ambrustmache VW. Juvenile pilocytic astrocytoma of the pineal region: report of a case. Cancer 1982;50:1185–8. Vaquero J, Ramiro J, Martinez R. Glioblastoma multiforme of the pineal region. J Neurosurg Sci 1990;34:149–50. Kepes JJ, Rubinstein LJ, Eng LF. Meningocerebral xanthoastrocytoma. A distinctive glioma in young subjects, presumably originating from subpial astrocytes, with relatively favorable prognosis. Abstracts of the 8th International Congress of Neuropathology, Washington, DC. Am Assoc Neuropathol 1978;37:641.
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6. Kepes JJ. Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. Brain Pathol 1993;3:269–74. 7. Kepes JJ, Rubinstein LJ, Ansbacher L, et al. Histopathological features of recurrent pleomorphic xanthoastrocytoma: further corroboration of the glial nature of this neoplasm. Acta Neuropathol 1989;78:585–93. 8. Borit A, Blackwood W. Pineocytoma with astrocytomatous differentiation. J Neuropathol Exp Neurol 1979;38:253–8. 9. Nitta J, Tada T, Kyoshima K, et al. Atypical pleomorphic astrocytoma in the pineal gland: case report. Neurosurgery 2001;49:1458–61. 10. Snipes GJ, Horoupian DS, Shuer LM, et al. Pleomorphic granular cell astrocytoma of the pineal gland. Cancer 1992;70:2159–65. 11. Grant JW, Gallagher PJ. Pleomorphic xanthoastrocytoma: immunohistochemical methods for differentiation from fibrous histiocytomas with similar morphology. Am J Surg Pathol 1986;10:336–41.
doi:10.1016/j.jocn.2010.02.022
Bilateral anterior opercular (Foix–Chavany–Marie) syndrome Dennis A. Nowak a,c,*, Gabriela Griebl a, Rainer Dabitz b, Günter Ochs b a b c
Neurologische Fachklinik, Klinik Kipfenberg, Kindinger Strasse 13, D-85110 Kipfenberg, Germany Neurologische Klinik, Klinikum Ingolstadt, Ingolstadt, Germany Department of Neurology, University of Erlangen, Germany
a r t i c l e
i n f o
Article history: Received 22 February 2010 Accepted 23 February 2010
Keywords: Foix–Chavany–Marie syndrome Bilateral anterior opercular syndrome Stroke
a b s t r a c t The bilateral anterior opercular (Foix–Chavany–Marie) syndrome is a rare condition most commonly encountered after bilateral middle cerebral artery stroke. Clinically, the syndrome is characterised by a loss of voluntary control of the facio-pharyngeo-glosso-masticatory muscles, while reflex movements and limb strength are preserved. We report the clinical presentation of a patient with anterior biopercular syndrome due to a bilateral middle cerebral artery stroke. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction The bilateral anterior opercular (Foix–Chavany–Marie) syndrome was first described in 1926.1 The syndrome is caused by bilateral lesions of the anterior frontoparietal opercula, most frequently due to ischaemic stroke.2 Voluntary movements of the cranial muscles innervated by the trigeminal, facial, glossopharyngeal, vagus and hypoglossus nerves are bilaterally lost.1,2 The affected individuals suffer from bilateral facial weakness and an inability to speak and chew, while the swallowing function is generally only mildly affected. There is dissociation between voluntary and automatic muscle activity: reflexes and automatic or emotional movements in those muscles that are voluntarily paralysed are well preserved,2–4 which means that the corneal reflex, the threaten reflex, and emotional laughing or crying are all possible.1,4 2. Case report A 79-year-old female with medically treated arterial hypertension and diabetes mellitus presented to our facility with bilateral facial weakness and a sudden inability to speak. She had completely recovered from an ischaemic stroke in the right middle
* Corresponding author at: Neurologische Fachklinik, Klinik Kipfenberg, Kindinger Strasse 13, D-85110 Kipfenberg, Germany. Tel.: +49 8465 175 100; fax: +49 8465 175 184. E-mail address: [email protected] (D.A. Nowak).
cerebral artery territory, which had occurred 6 years previously. Examination revealed anarthria, but well-preserved comprehension, writing and limb function. When requested, she was unable to speak or perform voluntary facial or tongue movements. She was only able to generate a groaning sound, and her swallowing function was mildly impaired. There were no deficits in somatosensory function or coordination. The deep tendon reflexes were symmetrically brisk and the Babinski sign was bilaterally negative. Swallowing reflexes and emotional facial movements were preserved. CT scans of her brain revealed a diffuse hypodensity within the right frontal and temporal lobes, the result of the middle cerebral artery stroke encompassing the frontoparietal opercular cortex, which had occurred 6 years earlier. In addition, the cortex–white matter differentiation and the gyral configuration of the left frontal opercular cortex were lost, indicative of an early middle cerebral artery stroke (Fig. 1A). CT angiography revealed occlusion of a temporal branch of the left middle cerebral artery. An acute left middle cerebral artery stroke causing bilateral anterior opercular (Foix– Chavany–Marie) syndrome was diagnosed, and intravenous recombinant tissue plasminogen activator was administered within 3 hours of the symptom onset. However, thrombolysis was ineffective and her neurological status did not change. CT scans performed 24 hours after the administration of thrombolysis revealed an unchanged area of the right-sided ischaemic stroke encompassing the frontoparietal opercular cortex and a new, leftsided ischaemic lesion of the frontoparietal opercular cortex (Fig. 1B).