Síndrome extrapiramidal e hipoparatireoidismo: acerca da identidade da doença de Fahr

Vega, Marcelo Gabriel; Sousa, Atos Alves de; Lucca Jr, Francisco de; Purich, Saulo; Tenassi, Maria Lucia

doi:10.1590/S0004-282X1994000300023

Resumos

A presença de calcificações bilaterais dos gânglios da base associadas a manifestações neurológicas foi por muito tempo diagnosticada como doença de Fahr . Apresentamos os casos de dois pacientes com calcificações bilaterais de gânglios da base que apresentavam síndromes extrapiramidais e hipoparatireoidismo. Revimos a literatura pertinente e discutimos a identidade da doença de Fahr e as relações entre calcificações dos gânglios da base, manifestações neurológicas e endócrinas.

calcificações; gânglios da base; síndrome extrapiramidal; hipoparatireoidismo; doença de Fahr

Bilateral calcifications in the basal ganglia associated with neurological manifestations was diagnosed as Fahr disease for a long time. We present the cases of two patients with bilateral calcifications in the basal ganglia who presented hypoparathyroidism and extrapyramidal syndrome. Based on literature review we discuss the identity of Fahr disease and the relationship between calcifications in the basal ganglia and manifestations of endocrine system dysfunctions.

calcifications; basal ganglia; extrapiramidal syndrome; hypoparathyroidism; Fahr disease

Síndrome extrapiramidal e hipoparatireoidismo: acerca da identidade da doença de Fahr

Hypoparathyroidism and extrapyramidal syndrome: about the identity of the Fahr disease

Marcelo Gabriel Vega^I; Atos Alves de Sousa^I; Francisco de Lucca Jr^I; Saulo Purich^II; Maria Lucia Tenassi^II

^ISanta Casa de Misericórdia de Belo Horizonte: Serviço de Neurologia e Neurocirurgia

^IISanta Casa de Misericórdia de Belo Horizonte: Serviço de Endocrinologia

RESUMO

A presença de calcificações bilaterais dos gânglios da base associadas a manifestações neurológicas foi por muito tempo diagnosticada como doença de Fahr . Apresentamos os casos de dois pacientes com calcificações bilaterais de gânglios da base que apresentavam síndromes extrapiramidais e hipoparatireoidismo. Revimos a literatura pertinente e discutimos a identidade da doença de Fahr e as relações entre calcificações dos gânglios da base, manifestações neurológicas e endócrinas.

Palavras-chave: calcificações, gânglios da base, síndrome extrapiramidal, hipoparatireoidismo, doença de Fahr.

SUMMARY

Bilateral calcifications in the basal ganglia associated with neurological manifestations was diagnosed as Fahr disease for a long time. We present the cases of two patients with bilateral calcifications in the basal ganglia who presented hypoparathyroidism and extrapyramidal syndrome. Based on literature review we discuss the identity of Fahr disease and the relationship between calcifications in the basal ganglia and manifestations of endocrine system dysfunctions.

Key words: calcifications, basal ganglia, extrapiramidal syndrome, hypoparathyroidism, Fahr disease

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 16-fevereiro-1994.

Dr. Marcelo Gabriel Vega-Rua Ceará 567, 5º andar - 30150-310 Belo Horizonte MG - Brasil.

1. Babbit DP, Tang T, Osbbs J, Berk R. Idiopathic familial cerebrovascular ferrocalcinosis (Fahr's disease) and review of differencial diagnosis of intracranial calcification in children. Am J Roentgenol 1969, 105: 352-358.
2. Ben Hamida M. Maladie de Fahr. Encycl Med Chir Neurologie 17062 M10 Paris, 1972.
3. Bennet JC, Maffly RH, Steinbach HL. The significance of bilateral basal ganglia calcification. Radiology 1959, 72: 368-378.
4. Camp JD. Pathologic non neoplastic intracranial calcification. JAMA 1948, 137: 1023-1030.
5. Cohen CR, Duchesnau PM, Weintein MA. Calcification of the basal ganglia as visualised by tomography. Radiology 1980, 134:97-99.
6. Dewey RB, Jankovic J. Hemiballism-hemichorea: clinical and pharmacologic findings in 21 patients. Arch Neurol 1989, 46: 862-867.
7. Eaton LM, Camp JD, Love JL. Symmetric cerebral calcification, particularly of basal ganglia, demonstrable roentgenographicaly. Arch Neurol Psychiat 1939, 41: 921-942.
8. Eaton LM, Haines SF. Parathyroid insufficiency with simmetrical cerebral calcification. JAMA 1939, 113: 749-753.
9. Fahr TK. Idiopatische Verkalkung der Hirngefasse. Zentralbl allg Path u path anat 1930, 50: 129-133.
10. Fenelon G, Guilliard A. Maladie de Fahr et calcifications des noyaux gris centraux. Encycl Med Chir Neurologie 17062 M10. Paris, 1987.
11. Friedman JH, Chiucchinni I, Tucci J. Idiopathic hypoparathyroidism with extensive brain calcification and persistent neurologic dysfunction. Neurology 1987, 37: 307-309.
12. Harrington MG, Macpherson P, MclntoshWB, Allan BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981, 44: 1168-1170.
13. Hurst EW. On the so called calcification in the basal ganglia of the brain. J Pathol 1926, 29: 65-85.
14. Inbody S, Jankovic J. Hyperkinetic mutism, bilateral ballism and basal ganglia calcification. Neurology 1986, 36: 825-827.
15. Kahm E, Lion EG, Zimmerman HM. Cerebral cortical calcification simulating Pick's disease. Am J Psychiatry 1939, 95: 1027-1033.
16. Kasanin J, Crank RP A case of extensive calcification in the brain: selective calcification of the finer cerebral blood vessels. Arch Neurol 1962, 34: 164-178.
17. Love JG, Camp JD, Eaton LM. Symmetrical cerebral calcification, particularly of the basal ganglia, demonstrable roentgenologically, associated with cyst of the cavum septo pellucido and cavum vergae. Proc. Staff Meet Mayo Clin 1938, 13: 225-232.
18. Lowenthal A, Bruyn GW. Calcification of the striopallidodentate system. In Vinken PJ, Bruyn GW (eds). Handbook of clinical neurology. Amsterdam: North Holland, 1968, Vol 6, p 703-729.
19. Martin JP. Hemichorea (hemiballismus) without lesions in the corpus Luysii. Brain 1957, 80-I: 1-10.
20. Muenter MD, Whisnant JP. Basal ganglia calcifications, hipoparathyroidism and extrapyramidal motor manifestations. Neurology 1968, 18: 1075-1083.
21. Peters G. Neuropatologia clfnica. Barcelona: Toray, 1974.
22. Sprague RG, Haines SF, Power MH. Metabolic effects of parathyroid hormone, dihydrotachysterol and calciferol in case of pseudo-hypoparatyroidism. J Lab Clin Med 1945, 30: 363-364.
23. Wagner JA, Slager UT, Denis JM, Barnes EV. The incidence and composition of radiopaque deposits in the basal ganglia of the brain. Am J Roentgenol 1955, 74: 232-234.

Datas de Publicação

Publicação nesta coleção
19 Jan 2011
Data do Fascículo
Set 1994

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

[1] 1. Babbit DP, Tang T, Osbbs J, Berk R. Idiopathic familial cerebrovascular ferrocalcinosis (Fahr's disease) and review of differencial diagnosis of intracranial calcification in children. Am J Roentgenol 1969, 105: 352-358.

[2] 2. Ben Hamida M. Maladie de Fahr. Encycl Med Chir Neurologie 17062 M10 Paris, 1972.

[3] 3. Bennet JC, Maffly RH, Steinbach HL. The significance of bilateral basal ganglia calcification. Radiology 1959, 72: 368-378.

[4] 4. Camp JD. Pathologic non neoplastic intracranial calcification. JAMA 1948, 137: 1023-1030.

[5] 5. Cohen CR, Duchesnau PM, Weintein MA. Calcification of the basal ganglia as visualised by tomography. Radiology 1980, 134:97-99.

[6] 6. Dewey RB, Jankovic J. Hemiballism-hemichorea: clinical and pharmacologic findings in 21 patients. Arch Neurol 1989, 46: 862-867.

[7] 7. Eaton LM, Camp JD, Love JL. Symmetric cerebral calcification, particularly of basal ganglia, demonstrable roentgenographicaly. Arch Neurol Psychiat 1939, 41: 921-942.

[8] 8. Eaton LM, Haines SF. Parathyroid insufficiency with simmetrical cerebral calcification. JAMA 1939, 113: 749-753.

[9] 9. Fahr TK. Idiopatische Verkalkung der Hirngefasse. Zentralbl allg Path u path anat 1930, 50: 129-133.

[10] 10. Fenelon G, Guilliard A. Maladie de Fahr et calcifications des noyaux gris centraux. Encycl Med Chir Neurologie 17062 M10. Paris, 1987.

[11] 11. Friedman JH, Chiucchinni I, Tucci J. Idiopathic hypoparathyroidism with extensive brain calcification and persistent neurologic dysfunction. Neurology 1987, 37: 307-309.

[12] 12. Harrington MG, Macpherson P, MclntoshWB, Allan BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981, 44: 1168-1170.

[13] 13. Hurst EW. On the so called calcification in the basal ganglia of the brain. J Pathol 1926, 29: 65-85.

[14] 14. Inbody S, Jankovic J. Hyperkinetic mutism, bilateral ballism and basal ganglia calcification. Neurology 1986, 36: 825-827.

[15] 15. Kahm E, Lion EG, Zimmerman HM. Cerebral cortical calcification simulating Pick's disease. Am J Psychiatry 1939, 95: 1027-1033.

[16] 16. Kasanin J, Crank RP A case of extensive calcification in the brain: selective calcification of the finer cerebral blood vessels. Arch Neurol 1962, 34: 164-178.

[17] 17. Love JG, Camp JD, Eaton LM. Symmetrical cerebral calcification, particularly of the basal ganglia, demonstrable roentgenologically, associated with cyst of the cavum septo pellucido and cavum vergae. Proc. Staff Meet Mayo Clin 1938, 13: 225-232.

[18] 18. Lowenthal A, Bruyn GW. Calcification of the striopallidodentate system. In Vinken PJ, Bruyn GW (eds). Handbook of clinical neurology. Amsterdam: North Holland, 1968, Vol 6, p 703-729.

[19] 19. Martin JP. Hemichorea (hemiballismus) without lesions in the corpus Luysii. Brain 1957, 80-I: 1-10.

[20] 20. Muenter MD, Whisnant JP. Basal ganglia calcifications, hipoparathyroidism and extrapyramidal motor manifestations. Neurology 1968, 18: 1075-1083.

[21] 21. Peters G. Neuropatologia clfnica. Barcelona: Toray, 1974.

[22] 22. Sprague RG, Haines SF, Power MH. Metabolic effects of parathyroid hormone, dihydrotachysterol and calciferol in case of pseudo-hypoparatyroidism. J Lab Clin Med 1945, 30: 363-364.

[23] 23. Wagner JA, Slager UT, Denis JM, Barnes EV. The incidence and composition of radiopaque deposits in the basal ganglia of the brain. Am J Roentgenol 1955, 74: 232-234.