Abnormally increased iron concentration in basal ganglia in Shy-Drager syndrome MR imaging and autonomic study: RM imagem e estudo autonômico

Maciel Jr., Jayme A.; Rocha, Cristiane M. da; Cabelho, Sandra; Pradal, Mario G.

doi:10.1590/S0004-282X1991000300020

Abstracts

Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.

É relatado o caso de unm paciente de 67 anos de idade com quadro inicial da síndrome de Shy-Drager. O diagnóstico foi possível por provas funcionais autonômicas e exames laboratoriais. A ressonância magnética cerebral (contraste baseado na densidade de prótons e em T2) objetivou proeminente hipodensidade putaminal em T2, secundária ao aumento do depósito do ferro nesta região. Esse achado da RM confirma o diagnóstico da síndrome de Shy-Drager e permite diferenciá-la da hipotensão ortostática idiopática, particularmente na fase de início da SSD quando os sinais de comprometimento do SNC são discretos ou estão ausentes.

CONTENTS CONTEÚDO

Jayme A. Maciel Jr.^I; Cristiane M. da Rocha^II; Sandra Cabelho^III; Mario G. Pradal^IV

^IProfessor Assistente Doutor - Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP)

^IIResidente de Neurologia - Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP)

^IIIEndocrinologista, CRIESP - Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP)

^IVEndocronologista CRIESP, Auxiliar de Ensino de Fisiologia da Faculdade de Medicina de Santos - Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP)

SUMMARY

Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T₂-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.

RESUMO

É relatado o caso de unm paciente de 67 anos de idade com quadro inicial da síndrome de Shy-Drager. O diagnóstico foi possível por provas funcionais autonômicas e exames laboratoriais. A ressonância magnética cerebral (contraste baseado na densidade de prótons e em T₂) objetivou proeminente hipodensidade putaminal em T₂, secundária ao aumento do depósito do ferro nesta região. Esse achado da RM confirma o diagnóstico da síndrome de Shy-Drager e permite diferenciá-la da hipotensão ortostática idiopática, particularmente na fase de início da SSD quando os sinais de comprometimento do SNC são discretos ou estão ausentes.

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Dr. Jayme Antunes Maciel Jr. - Departamento de Neurologia, Faculdade de Ciências Médicas, UNICAMP - Caixa Postal 6111 - 13081 Campinas SP - Brasil.

1. Babinski J, Laubry C. Léçons de Séméiologie Cardio-Vasculaire. Art vertige. Paris: Doin Édit, 1924.
2. Bannister R. The diagnosis and management of chronic autonomic failure. In : Legg NJ (ed) : Neurotransmitter Systems and their Clinical Diagnosis. London: Academic Press, 1978, p 65-83.
3. Bannister R, Oppenheimer DR. Degenerative diseases of the nervous system associated with autonomic failure. Brain 1972, 95:457-474.
4. Bradbury S, Eggleston C. Postural hypotension: a report of three cases. Am Heart J 1925, 1:73-86.
5. Bradbury S, Eggleston C. Postural hypotension: autopsy on a case. Am Heart J 1927, 3:105-106.
6. Brown RT, Polinsky RJ, Di Chiro G, Pastakia B, Werner L, Simmons JT. MRI in autonomic failure. J Neurol Neurosurg Psychiat 1987, 50:913-914.
7. Burton K, Farrel K, Li D, Calne DB. Lesions of the. putamen and dystonia: CT and magnetic resonance imaging. Neurology 1984, 34:302-365.
8. Christentsen NJ. Plasma catecholamines in long-term diabetics with and without neuropathy and in hypophysectomized subjects. J Clin Invest 1972, 51:779-787.
9. Cohen J, Low L, Fealy R, Sheps S, Tiang NS. Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. Ann Neurol 1987, 22:692-699.
10. Drayer B, Olanow W, Burger P, Johnson GA, Herfkens R, Riederer S. Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron. Radiology 1986, 159:493-498.
11. Earle KM. Studies on Parkinson's disease including X-ray, fluorescent spectroscopy of formalin-fixed brain tissue. J Neuropath Exp Neurol 1968, 27:1-14.
12. Fichefet JP, Sternon JE, Franken L, Demanet JC, Vanderhaeghen JJ. Étude anatomo-clinique d'un cas d'hypotension orthostatique «idioprathique». Acta Cardiol 1966, 20:332-348.
13. Gallucci M, Cardone F, Arechi M, Splendani A, Bozzao A, Passariello F. Follow-up MR studies in Hallervorden-Spatz disease. J Comput Assist Tomogr. 1990, 14:118-120.
14. Ghrist DG, Brown GE. Postural hypotension with syncope: its successful treatment with ephedrine. Sm J med Sci 1928, 175:336-349.
15. Graham JC, Oppenheimer DR. Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psycriat 1969: 32:28-34.
16. Hallgren B, Sourander P. The effect of age on the non-haemin iron in the human brain. J Neurochem 1958, 3:41-51.
17. Johnson RH, Lee J, Oppenheimer DR, Spalding JMK. Autonomic failure with orthostatic hypotension due to intermediolateral column degeneration Q J Med 1966, 135:276-292.
18. Johnson RH, Spalding JMK. Disorders of the autonomic nervous system. Oxford: Blackwell, 1974.
19. Kennedy PGE, Duchen LW. A quantitative study of intermediolateral column cells in motor neuron disease and the Shy-Drager syndrome. J Neurol Neurosurg Psychiat 1985, 48:1103-1106.
20. Klintworth GK. Huntington chorea: morphologic contributions of a centry. Advances in Neurology. New York: Raven, 1973, p 353-368.
21. Lian C, Blondel A. Hypotension artérielle orthostatique. Paris Med 1933, 1:179-184.
22. Low PA, Dyck PJ, Lambert EH. Acute panautonomic neuropathy. Ann Neurol 1983, 13:412-417.
23. Low PA, Thomas JE, Dyck PJ. The splancnic autonomic outflow in Shy-Drager syndrome and idiopathic orthostatic hypotension. Ann Neurol 1978, 4:511-514.
24. Maciel JA Jr. Shy-Drager syndrome: a functional protocol study of the autonomic nervous system in 3 cases. M.D. Thesis, Faculty of Medical Sciences, State University of Campinas (UNICAMP), Brazil. Campinas, 1984.
25. Nanda RN, Boyle FC, Gillespie JS, Johnson RH, Keogh HJ. Idiopathic orthostatic hypotension from failure of noradrenaline release in a patient with vasomotor innervation. J Neurol Neurosurg Psychiat 1977, 40:11-19.
26. Pastakia B, Polinsky R, Di Chiro G, Simmons JT, Brown R, Werner L. Multiple system atrophy (Shy-Drager syndrome) : MR imaging. Radiology 1986, 159:499-502.
27. Piorry PA. Influence de la pesanteur sur le cours du sang, diagnostic de la syncope et de l'apoplexie. J Hebd Méd 1826, 2:292-297.
28. Polinsky RJ, Kopim IJ, Ebert MH, Weise VW. Pharmacologic distinction of different orthostatic hypotension syndromes. Neurology 1981, 31:1-7.
29. Rohmer F, Warter JM, Coquillat G, Schupp C, Maitrot D. «Maladie» de Shy et Drager: à propos d'une observation anatomo-clinique. Revue de la littérature. Ann Méd Int (Paris) 1971, 10:665-673.
30. Savoiardo M, Strada L, Girotti F, D'Incert L, Sberna M, Soliveri P, Baizarini L. MR imaging in progressive supranuclear palsy and Shy-Drager syndrome. J Comput Assist Tomogr 1989, 13:555-560.
31. Shy GM, Drager GA. Neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 1960, 2:511-527.
32. Spokes EGS, Bannister R, Oppenheimer DR. Multiple system atrophy with autonomic failure: clinical, hystological and neurochemical observations in four cases. J Neurol Sci 1979, 43:59-82.
33. Szanto J, Gallyas F. A study of iron metabolism in neuropsychiatrie patients: Haller-vorden Spatz disease. Arch Neurol 1966, 14:438-442.
34. Thomas JE, Schirger A. Neurologic manifestations in idiopathic orthostatic hypotension. Arch Neurol 1963, 8:204-208.
35. Thomas JE, Schirger A. Idiopathic orthostatic hypotension: a study of its natural history in 57 neurologically affected patients. Arch Neurol 1970, 22:289-293.
36. Thomas JE, Schirger A, Fealey RD, Sheps SG. Orthostatic hypotension. Mayo Clin Proc 1981, 56:117-125.
37. Thulesius O. Pathophysiological classification and diagnosis of orthostatic hypotension. Cardiology 1976, 61 (Suppl 1):180-190.
38. Veron JP, Escourolle R. Données actuelles sur l'hypotension orthostatique. Presse Méd 1968, 76:2343-2346.
39. Wheeler T, Watkins PJ. Cardiac denervation in diabetes. Br Med J 1973, 4:584-586.
40. Ziegler MQ, Lake CR, Kopin 1J. The sympathetic nervous system defect in primary orthostatic hypotension. N Engl J Med 1977, 296:293-297.

Abnormally increased iron concentration in basal ganglia in Shy-Drager syndrome MR imaging and autonomic study

Concentração de ferro anormalmente aumentada nos núcleos da base na síndrome de Shy-Drager: RM imagem e estudo autonômico.

Publication Dates

Publication in this collection
22 Feb 2011
Date of issue
Sept 1991

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Babinski J, Laubry C. Léçons de Séméiologie Cardio-Vasculaire. Art vertige. Paris: Doin Édit, 1924.

[2] 2. Bannister R. The diagnosis and management of chronic autonomic failure. In : Legg NJ (ed) : Neurotransmitter Systems and their Clinical Diagnosis. London: Academic Press, 1978, p 65-83.

[3] 3. Bannister R, Oppenheimer DR. Degenerative diseases of the nervous system associated with autonomic failure. Brain 1972, 95:457-474.

[4] 4. Bradbury S, Eggleston C. Postural hypotension: a report of three cases. Am Heart J 1925, 1:73-86.

[5] 5. Bradbury S, Eggleston C. Postural hypotension: autopsy on a case. Am Heart J 1927, 3:105-106.

[6] 6. Brown RT, Polinsky RJ, Di Chiro G, Pastakia B, Werner L, Simmons JT. MRI in autonomic failure. J Neurol Neurosurg Psychiat 1987, 50:913-914.

[7] 7. Burton K, Farrel K, Li D, Calne DB. Lesions of the. putamen and dystonia: CT and magnetic resonance imaging. Neurology 1984, 34:302-365.

[8] 8. Christentsen NJ. Plasma catecholamines in long-term diabetics with and without neuropathy and in hypophysectomized subjects. J Clin Invest 1972, 51:779-787.

[9] 9. Cohen J, Low L, Fealy R, Sheps S, Tiang NS. Somatic and autonomic function in progressive autonomic failure and multiple system atrophy. Ann Neurol 1987, 22:692-699.

[10] 10. Drayer B, Olanow W, Burger P, Johnson GA, Herfkens R, Riederer S. Parkinson plus syndrome: diagnosis using high field MR imaging of brain iron. Radiology 1986, 159:493-498.

[11] 11. Earle KM. Studies on Parkinson's disease including X-ray, fluorescent spectroscopy of formalin-fixed brain tissue. J Neuropath Exp Neurol 1968, 27:1-14.

[12] 12. Fichefet JP, Sternon JE, Franken L, Demanet JC, Vanderhaeghen JJ. Étude anatomo-clinique d'un cas d'hypotension orthostatique «idioprathique». Acta Cardiol 1966, 20:332-348.

[13] 13. Gallucci M, Cardone F, Arechi M, Splendani A, Bozzao A, Passariello F. Follow-up MR studies in Hallervorden-Spatz disease. J Comput Assist Tomogr. 1990, 14:118-120.

[14] 14. Ghrist DG, Brown GE. Postural hypotension with syncope: its successful treatment with ephedrine. Sm J med Sci 1928, 175:336-349.

[15] 15. Graham JC, Oppenheimer DR. Orthostatic hypotension and nicotine sensitivity in a case of multiple system atrophy. J Neurol Neurosurg Psycriat 1969: 32:28-34.

[16] 16. Hallgren B, Sourander P. The effect of age on the non-haemin iron in the human brain. J Neurochem 1958, 3:41-51.

[17] 17. Johnson RH, Lee J, Oppenheimer DR, Spalding JMK. Autonomic failure with orthostatic hypotension due to intermediolateral column degeneration Q J Med 1966, 135:276-292.

[18] 18. Johnson RH, Spalding JMK. Disorders of the autonomic nervous system. Oxford: Blackwell, 1974.

[19] 19. Kennedy PGE, Duchen LW. A quantitative study of intermediolateral column cells in motor neuron disease and the Shy-Drager syndrome. J Neurol Neurosurg Psychiat 1985, 48:1103-1106.

[20] 20. Klintworth GK. Huntington chorea: morphologic contributions of a centry. Advances in Neurology. New York: Raven, 1973, p 353-368.

[21] 21. Lian C, Blondel A. Hypotension artérielle orthostatique. Paris Med 1933, 1:179-184.

[22] 22. Low PA, Dyck PJ, Lambert EH. Acute panautonomic neuropathy. Ann Neurol 1983, 13:412-417.

[23] 23. Low PA, Thomas JE, Dyck PJ. The splancnic autonomic outflow in Shy-Drager syndrome and idiopathic orthostatic hypotension. Ann Neurol 1978, 4:511-514.

[24] 24. Maciel JA Jr. Shy-Drager syndrome: a functional protocol study of the autonomic nervous system in 3 cases. M.D. Thesis, Faculty of Medical Sciences, State University of Campinas (UNICAMP), Brazil. Campinas, 1984.

[25] 25. Nanda RN, Boyle FC, Gillespie JS, Johnson RH, Keogh HJ. Idiopathic orthostatic hypotension from failure of noradrenaline release in a patient with vasomotor innervation. J Neurol Neurosurg Psychiat 1977, 40:11-19.

[26] 26. Pastakia B, Polinsky R, Di Chiro G, Simmons JT, Brown R, Werner L. Multiple system atrophy (Shy-Drager syndrome) : MR imaging. Radiology 1986, 159:499-502.

[27] 27. Piorry PA. Influence de la pesanteur sur le cours du sang, diagnostic de la syncope et de l'apoplexie. J Hebd Méd 1826, 2:292-297.

[28] 28. Polinsky RJ, Kopim IJ, Ebert MH, Weise VW. Pharmacologic distinction of different orthostatic hypotension syndromes. Neurology 1981, 31:1-7.

[29] 29. Rohmer F, Warter JM, Coquillat G, Schupp C, Maitrot D. «Maladie» de Shy et Drager: à propos d'une observation anatomo-clinique. Revue de la littérature. Ann Méd Int (Paris) 1971, 10:665-673.

[30] 30. Savoiardo M, Strada L, Girotti F, D'Incert L, Sberna M, Soliveri P, Baizarini L. MR imaging in progressive supranuclear palsy and Shy-Drager syndrome. J Comput Assist Tomogr 1989, 13:555-560.

[31] 31. Shy GM, Drager GA. Neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 1960, 2:511-527.

[32] 32. Spokes EGS, Bannister R, Oppenheimer DR. Multiple system atrophy with autonomic failure: clinical, hystological and neurochemical observations in four cases. J Neurol Sci 1979, 43:59-82.

[33] 33. Szanto J, Gallyas F. A study of iron metabolism in neuropsychiatrie patients: Haller-vorden Spatz disease. Arch Neurol 1966, 14:438-442.

[34] 34. Thomas JE, Schirger A. Neurologic manifestations in idiopathic orthostatic hypotension. Arch Neurol 1963, 8:204-208.

[35] 35. Thomas JE, Schirger A. Idiopathic orthostatic hypotension: a study of its natural history in 57 neurologically affected patients. Arch Neurol 1970, 22:289-293.

[36] 36. Thomas JE, Schirger A, Fealey RD, Sheps SG. Orthostatic hypotension. Mayo Clin Proc 1981, 56:117-125.

[37] 37. Thulesius O. Pathophysiological classification and diagnosis of orthostatic hypotension. Cardiology 1976, 61 (Suppl 1):180-190.

[38] 38. Veron JP, Escourolle R. Données actuelles sur l'hypotension orthostatique. Presse Méd 1968, 76:2343-2346.

[39] 39. Wheeler T, Watkins PJ. Cardiac denervation in diabetes. Br Med J 1973, 4:584-586.

[40] 40. Ziegler MQ, Lake CR, Kopin 1J. The sympathetic nervous system defect in primary orthostatic hypotension. N Engl J Med 1977, 296:293-297.