842
LETTERS TO THE EDITOR
This letter was shown to the authors who reply as follows: Lymphomatoid granulomatosis (LYG), as described by Liebow et al. and subsequent reports,2'3 was defined as a distinct entity on the basis of clinicopathological features. The idea of LYG as a lymphomatous condition was suggested as early as 1977,4 and substantiated by the fact that 15% of cases were associated or evolved to lymphoma."2 This could not be demonstrated until late 80's by detection of clonal rearrangement of the beta T-cell receptor (0-TCR) gene.5 Our patient was diagnosed in 1985, and techniques for detection ofclonal rearrangement in the P-TCR gene were not available by that time in our hospital. Therefore, the diagnosis of LYG was made on clinicopathological grounds. In our patient, only an atypical presentation delayed the diagnosis. We chose for our patient the most suitable and efficacious treatment by the time the diagnosis was made.' He had an acceptable quality of life up until complications developed which required surgical procedures and a long admission in an intensive care unit. Antonio Torrelo Manuel Martin Antonio Rocamora Francisco Allegue Antonio Ledo Department of Dermatology, Hospital "Ram6n y Cajal", Apartado 37, 28034-Madrid, Spain. References 1. Liebow, A.A., Carrington, C.B. & Friedman, R.J. Lymphomatoid granulomatosis. Hwn Pathol 1972, 3: 457-558. 2. Katzenstein, A.L.A., Carrington, C.B. & Liebow, A.A. Lymphomatoid granulomatosis. A clinicopathologic study of 152 cases. Cancer 1979, 43: 360-373. 3. Patton, W.F. & Lynch, J.P. Lymphomatoid granulomatosis. Clinicopathologic study of four cases and literature review. Medicine 1982, 61: 1-12. 4. Israel, H.L., Patchefsky, A.S. & Saldana, M.J. Wegener's
A 30 year old man with clinical and biochemical evidence of phaeochromocytoma underwent meta-iodobenzyl guanidine (MIBG) scintigraphy and a computed tomographic (CT) total body scan. These techniques were non-diagnostic and selective venous sampling was performed. This showed high plasma noradrenaline concentrations in the inferior part of the right internal jugular vein and in the right atrium. Digital subtraction angiography revealed bilateral intercarotid tumours. After surgical removal of two benign intercarotid paraglangliomas, hypertension and biochemical indices of catecholamine hypersecretion were unchanged. Further MIBG scintigraphy and selective venous sampling gave similar results to those before surgery. Whereas a thoracic CT scan showed an apparently slightly enlarged left atrium and conventional two-dimensional echocardiography (2-D echo) was considered normal, a coronary angiogram revealed a mass located behind the left atrium, vascularized by the right and circumflex coronary arteries. Before operation, the lesion was differentiated from surrounding structures by magnetic resonance (MR) (performed in Grenoble) (Figure 1). At thoracotomy, an intrapericardial tumour measuring 7 x 6 x 4 cm and weighing 90 g was discovered connected to the left atrium and extending to the pulmonary vessels and the oesophagus. The resected specimen was a benign phaeochromocytoma. Now 48 months after surgery, the blood pressure and urinary levels of catecholamine and metabolites remain normal. This case describes the difficulties encountered in the topographic diagnosis of an intrapericardial phaeochromocytoma: the tumour was associated with two non-functioning paragangliomas and was not recognized by the usual non-invasive techniques. Intrapericardial phaeochromocytoma is uncommon:' thoracic paragangliomas account for approximately 2% of phaeochromocytoma,2 most of which are situated in the paravertebral gutter.2'3 Location in the middle mediastinum is exceptional.3 Intrapericardial paraganglioma are localized with great difficulty. Chest X-ray and conventional 2-D echo
granulomatosis, lymphomatoid granulomatosis, and benign lymphocytic angiitis and granulomatosis of lung: recognition and treatment. Ann Intern Med 1977, 87: 691-699. 5. Gaulard, P., Henni, T., Marolleau, J.P. et al. Lethal midline granuloma (polymorphic reticulosis) and lymphomatoid granulomatosis. Evidence for a monoclonal T-cell lymphoproliferative disorder. Cancer 1988, 62: 705-710. 6. Fauci, A.S., Haynes, B.F., Costa, J., Katz, P. & Wolff, S.M. Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. NEngl J Med 1982, 306: 68-74.
Intrapericardial phaeochromocytoma associated
with two intercarotid paragangliomas: diagnostic considerations Sir, Intrapericardial phaeochromocytoma is rare and, as demonstrated in the following case, a challenge inasmuch as localization is concerned.
Figure 1 Magnetic resonance imaging showing a mass (arrow) connected to the left atrium.
LETTERS TO THE EDITOR are rarely of any help.3 Transoesophageal 2-D echo could be useful for detecting retroauricular paragangliomas. Although both MIBG and CT have improved the ability to localize phaeochromocytomas,4 these imaging techniques may not detect intrathoracic forms.'2 During conventional CT of the thorax, cardiac hypervascularized phaeochromocytomas are barely differentiated from surrounding vessels and cardiac chambers. Although MIBG scintigraphy has been shown to be valuable in the localization of ectopic phaeochromocytoma," 4 false negative (5-10%) have been reported.4 When CT and MIBG fail to detect a tumour, invasive procedures may be necessary.! Cardiac paragangliomas are vascularized from the aorta or from the coronary arteries.2 They often drain to the right atrium. Therefore blood samples from superior vena cava and right atrium can be of value in assessing such phaeochromocytomas.' 5 Coronary angiography is a useful means of locating the phaeochromocytoma and assessing the vascularity of the tumour before surgery.6 In retrospect, this strategy can be discussed with the arrival of MR imaging which makes invasive techniques obsolete. In patients with negative CT and MIBG scans, MR imaging may be a useful and sensitive tool for the detection and delineation of phaeochromocytoma, and more precisely a cardiac phaeochromocytoma.6 E. Renoult N. Danchin P. Mathieu M. Kessler D. Hestin E. Legrand D. Regent J.-F. Le Bas' University Hospitals of Nancy and 'Grenoble, France. References 1. Dunn, G.D., Sapsford, R.N., Hemingway, A.P. et al. Functioning middle mediastinal paraganglioma (phaeochromocytoma) associated with intercarotid paragangliomas. Lancet 1986, i: 1061-1064. 2. Geisler, F., Barth, G., Jaeck, D. et al. Un cas de ph6ochromocytome a localisation cardiaque. Revue de la litt6rature. Presse Mid 1985, 14: 1024-1026. 3. Saad, M.F., Frazier, O.H., Hickey, R.C. & Samaan, N.A. Intrapericardial phaeochromocytoma. Am J Med 1983, 75: 371-376. 4. Chatal, J. & Charbonnel, B. Comparison of iodobenzylguanidine imaging with computed tomography in locating phaeochromocytoma. J Clin Endocrinol Metab 1985, 61: 769-772. 5. Allison, D.J., Brown, M.J., Jones, D.H. & Timmis, J.B. Role of venous sampling in locating a phaeochromocytoma. Br Med J 1983, 286: 1122-1124. 6. Fisher, M.R., Higgins, C.B. & Andereck, W. MR imaging of an intrapericardial phaeochromocytoma. J. Comput Assist Tomog 1985, 9: 1103-1105.
Hypoglycaemiia masquerading as a stroke Sir, Cerebrovascular events. account for a significant morbidity and mortality in the elderly. IDiabetes mellitus and
843
its treatment can cause similar clinical findings with an altered level of consciousness. Sulphonylurea therapy, such as glibenclamide, is a recognized cause of this when precipitating hypoglycaemia.2'3 An 81 year old woman was admitted drowsy with complete aphasia but some motor response to verbal commands. Upper motor neurone signs were present in the left arm, and both legs, with bilaterally up going plantars. Her pupils reacted equally to light with loss of vertical eye movements but intact horizontal conjugate eye movements. Pulse rate was 80 beats per minute regular, supine blood pressure 190/90 mmHg with normal cardiac auscultation. A left carotid bruit was audible. A history was later obtained that on the day of admission she had become confused with a 3 hour history of sudden onset left-sided limb weakness and complete aphasia. Medication on admission included prednisolone 6 mg once daily, for polymyalgia rheumatica and glibenclamide 5 mg once daily for diabetes mellitus diagnosed 2 years previously. She lived with her husband in a ground floor rented flat and, prior to this episode, walked with a stick and received no social service support. She was an ex-smoker and drank no alcohol. Capillary blood BM Stix was 2-4 mmol/l, blood glucose was 1.6 mmol/l. Plasma urea and electrolytes, liver function tests, electrocardiogram and chest X-ray were normal. Following an intravenous bolus of 50% dextrose she immediately regained the use of all limbs and her speech returned. Repeat neurological examination was normal except for a right extensor plantar response. Repeat blood glucose estimation was 7.3 mmol/l. Capillary blood BM Stix were monitored and remained in the 7-11 mmol/l range overnight. This case highlights the importance of performing BM Stix and formal blood sugars on admission in all patients receiving glibenclamide therapy, particularly if there is an altered level of consciousness or focal neurology. The significant mortality of glibenclamide-associated hypoglycaemia should also be remembered.3
Kevin Shotliff Abi Prasad Peter Millard Division of Geriatric Medicine, St George's Hospital Medical School, Cranmere Terrace, London SWJ7 ORE, UK. Referenoes 1. Weatherall, D.J., Ledingham, L.G.G. & Warrell, D.A. Oxford Textbook of Medicine, 2nd ed. Oxford University Press, Oxford, pp. 21.155-21.156. 2. Jennings, A.M., Wilson, R.W. & Ward, J.D. Symptomatic hypoglycaemia in NIDDM patients treated with oral hypoglycaemic agents. Diabetes Care 1989, 12: 203-208. 3. Aspuld, K., Wilholm, B.E. & Lithner, F. Glibenclamideassociated hypoglycaemia: a report on 57 cases. Diabetologia 1983, 24: 412-417.
LETTERS TO THE EDITOR
This letter was shown to the authors who reply as follows: Lymphomatoid granulomatosis (LYG), as described by Liebow et al. and subsequent reports,2'3 was defined as a distinct entity on the basis of clinicopathological features. The idea of LYG as a lymphomatous condition was suggested as early as 1977,4 and substantiated by the fact that 15% of cases were associated or evolved to lymphoma."2 This could not be demonstrated until late 80's by detection of clonal rearrangement of the beta T-cell receptor (0-TCR) gene.5 Our patient was diagnosed in 1985, and techniques for detection ofclonal rearrangement in the P-TCR gene were not available by that time in our hospital. Therefore, the diagnosis of LYG was made on clinicopathological grounds. In our patient, only an atypical presentation delayed the diagnosis. We chose for our patient the most suitable and efficacious treatment by the time the diagnosis was made.' He had an acceptable quality of life up until complications developed which required surgical procedures and a long admission in an intensive care unit. Antonio Torrelo Manuel Martin Antonio Rocamora Francisco Allegue Antonio Ledo Department of Dermatology, Hospital "Ram6n y Cajal", Apartado 37, 28034-Madrid, Spain. References 1. Liebow, A.A., Carrington, C.B. & Friedman, R.J. Lymphomatoid granulomatosis. Hwn Pathol 1972, 3: 457-558. 2. Katzenstein, A.L.A., Carrington, C.B. & Liebow, A.A. Lymphomatoid granulomatosis. A clinicopathologic study of 152 cases. Cancer 1979, 43: 360-373. 3. Patton, W.F. & Lynch, J.P. Lymphomatoid granulomatosis. Clinicopathologic study of four cases and literature review. Medicine 1982, 61: 1-12. 4. Israel, H.L., Patchefsky, A.S. & Saldana, M.J. Wegener's
A 30 year old man with clinical and biochemical evidence of phaeochromocytoma underwent meta-iodobenzyl guanidine (MIBG) scintigraphy and a computed tomographic (CT) total body scan. These techniques were non-diagnostic and selective venous sampling was performed. This showed high plasma noradrenaline concentrations in the inferior part of the right internal jugular vein and in the right atrium. Digital subtraction angiography revealed bilateral intercarotid tumours. After surgical removal of two benign intercarotid paraglangliomas, hypertension and biochemical indices of catecholamine hypersecretion were unchanged. Further MIBG scintigraphy and selective venous sampling gave similar results to those before surgery. Whereas a thoracic CT scan showed an apparently slightly enlarged left atrium and conventional two-dimensional echocardiography (2-D echo) was considered normal, a coronary angiogram revealed a mass located behind the left atrium, vascularized by the right and circumflex coronary arteries. Before operation, the lesion was differentiated from surrounding structures by magnetic resonance (MR) (performed in Grenoble) (Figure 1). At thoracotomy, an intrapericardial tumour measuring 7 x 6 x 4 cm and weighing 90 g was discovered connected to the left atrium and extending to the pulmonary vessels and the oesophagus. The resected specimen was a benign phaeochromocytoma. Now 48 months after surgery, the blood pressure and urinary levels of catecholamine and metabolites remain normal. This case describes the difficulties encountered in the topographic diagnosis of an intrapericardial phaeochromocytoma: the tumour was associated with two non-functioning paragangliomas and was not recognized by the usual non-invasive techniques. Intrapericardial phaeochromocytoma is uncommon:' thoracic paragangliomas account for approximately 2% of phaeochromocytoma,2 most of which are situated in the paravertebral gutter.2'3 Location in the middle mediastinum is exceptional.3 Intrapericardial paraganglioma are localized with great difficulty. Chest X-ray and conventional 2-D echo
granulomatosis, lymphomatoid granulomatosis, and benign lymphocytic angiitis and granulomatosis of lung: recognition and treatment. Ann Intern Med 1977, 87: 691-699. 5. Gaulard, P., Henni, T., Marolleau, J.P. et al. Lethal midline granuloma (polymorphic reticulosis) and lymphomatoid granulomatosis. Evidence for a monoclonal T-cell lymphoproliferative disorder. Cancer 1988, 62: 705-710. 6. Fauci, A.S., Haynes, B.F., Costa, J., Katz, P. & Wolff, S.M. Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. NEngl J Med 1982, 306: 68-74.
Intrapericardial phaeochromocytoma associated
with two intercarotid paragangliomas: diagnostic considerations Sir, Intrapericardial phaeochromocytoma is rare and, as demonstrated in the following case, a challenge inasmuch as localization is concerned.
Figure 1 Magnetic resonance imaging showing a mass (arrow) connected to the left atrium.
LETTERS TO THE EDITOR are rarely of any help.3 Transoesophageal 2-D echo could be useful for detecting retroauricular paragangliomas. Although both MIBG and CT have improved the ability to localize phaeochromocytomas,4 these imaging techniques may not detect intrathoracic forms.'2 During conventional CT of the thorax, cardiac hypervascularized phaeochromocytomas are barely differentiated from surrounding vessels and cardiac chambers. Although MIBG scintigraphy has been shown to be valuable in the localization of ectopic phaeochromocytoma," 4 false negative (5-10%) have been reported.4 When CT and MIBG fail to detect a tumour, invasive procedures may be necessary.! Cardiac paragangliomas are vascularized from the aorta or from the coronary arteries.2 They often drain to the right atrium. Therefore blood samples from superior vena cava and right atrium can be of value in assessing such phaeochromocytomas.' 5 Coronary angiography is a useful means of locating the phaeochromocytoma and assessing the vascularity of the tumour before surgery.6 In retrospect, this strategy can be discussed with the arrival of MR imaging which makes invasive techniques obsolete. In patients with negative CT and MIBG scans, MR imaging may be a useful and sensitive tool for the detection and delineation of phaeochromocytoma, and more precisely a cardiac phaeochromocytoma.6 E. Renoult N. Danchin P. Mathieu M. Kessler D. Hestin E. Legrand D. Regent J.-F. Le Bas' University Hospitals of Nancy and 'Grenoble, France. References 1. Dunn, G.D., Sapsford, R.N., Hemingway, A.P. et al. Functioning middle mediastinal paraganglioma (phaeochromocytoma) associated with intercarotid paragangliomas. Lancet 1986, i: 1061-1064. 2. Geisler, F., Barth, G., Jaeck, D. et al. Un cas de ph6ochromocytome a localisation cardiaque. Revue de la litt6rature. Presse Mid 1985, 14: 1024-1026. 3. Saad, M.F., Frazier, O.H., Hickey, R.C. & Samaan, N.A. Intrapericardial phaeochromocytoma. Am J Med 1983, 75: 371-376. 4. Chatal, J. & Charbonnel, B. Comparison of iodobenzylguanidine imaging with computed tomography in locating phaeochromocytoma. J Clin Endocrinol Metab 1985, 61: 769-772. 5. Allison, D.J., Brown, M.J., Jones, D.H. & Timmis, J.B. Role of venous sampling in locating a phaeochromocytoma. Br Med J 1983, 286: 1122-1124. 6. Fisher, M.R., Higgins, C.B. & Andereck, W. MR imaging of an intrapericardial phaeochromocytoma. J. Comput Assist Tomog 1985, 9: 1103-1105.
Hypoglycaemiia masquerading as a stroke Sir, Cerebrovascular events. account for a significant morbidity and mortality in the elderly. IDiabetes mellitus and
843
its treatment can cause similar clinical findings with an altered level of consciousness. Sulphonylurea therapy, such as glibenclamide, is a recognized cause of this when precipitating hypoglycaemia.2'3 An 81 year old woman was admitted drowsy with complete aphasia but some motor response to verbal commands. Upper motor neurone signs were present in the left arm, and both legs, with bilaterally up going plantars. Her pupils reacted equally to light with loss of vertical eye movements but intact horizontal conjugate eye movements. Pulse rate was 80 beats per minute regular, supine blood pressure 190/90 mmHg with normal cardiac auscultation. A left carotid bruit was audible. A history was later obtained that on the day of admission she had become confused with a 3 hour history of sudden onset left-sided limb weakness and complete aphasia. Medication on admission included prednisolone 6 mg once daily, for polymyalgia rheumatica and glibenclamide 5 mg once daily for diabetes mellitus diagnosed 2 years previously. She lived with her husband in a ground floor rented flat and, prior to this episode, walked with a stick and received no social service support. She was an ex-smoker and drank no alcohol. Capillary blood BM Stix was 2-4 mmol/l, blood glucose was 1.6 mmol/l. Plasma urea and electrolytes, liver function tests, electrocardiogram and chest X-ray were normal. Following an intravenous bolus of 50% dextrose she immediately regained the use of all limbs and her speech returned. Repeat neurological examination was normal except for a right extensor plantar response. Repeat blood glucose estimation was 7.3 mmol/l. Capillary blood BM Stix were monitored and remained in the 7-11 mmol/l range overnight. This case highlights the importance of performing BM Stix and formal blood sugars on admission in all patients receiving glibenclamide therapy, particularly if there is an altered level of consciousness or focal neurology. The significant mortality of glibenclamide-associated hypoglycaemia should also be remembered.3
Kevin Shotliff Abi Prasad Peter Millard Division of Geriatric Medicine, St George's Hospital Medical School, Cranmere Terrace, London SWJ7 ORE, UK. Referenoes 1. Weatherall, D.J., Ledingham, L.G.G. & Warrell, D.A. Oxford Textbook of Medicine, 2nd ed. Oxford University Press, Oxford, pp. 21.155-21.156. 2. Jennings, A.M., Wilson, R.W. & Ward, J.D. Symptomatic hypoglycaemia in NIDDM patients treated with oral hypoglycaemic agents. Diabetes Care 1989, 12: 203-208. 3. Aspuld, K., Wilholm, B.E. & Lithner, F. Glibenclamideassociated hypoglycaemia: a report on 57 cases. Diabetologia 1983, 24: 412-417.
