Non-inflammatory cerebral amyloid angiopathy as a cause of rapidly progressive dementia: A case study

Takada, Leonel Tadao; Camiz, Paulo; Grinberg, Lea T.; Leite, Claudia da Costa

doi:10.1590/S1980-57642009DN30400015

Abstract

A 77 year-old men developed a subacute-onset, rapidly progressive cognitive decline. After 6 months of evolution, he scored 6 on the Mini-Mental State Examination and had left hemiparesis and hemineglect. The patient died 11 months after the onset of cognitive symptoms. Brain MRI showed microhemorrhages on gradient-echo sequence and confluent areas of white matter hyperintensities on T2-weighted images. Brain biopsy revealed amyloid-b peptide deposition in vessel walls, some of them surrounded by micro-bleeds. In this case report, we discuss the role of cerebral amyloid angiopathy (CAA) in cognitive decline, due to structural lesions associated with hemorrhages and infarcts, white matter lesions and co-morbidity of Alzheimer's disease, as well as the most recently described amyloid angiopathy-related inflammation.

Key words:
dementia; cerebral amyloid angiopathy; vascular dementia.

Resumo

Um homem de setenta e sete anos desenvolveu quadro de início subagudo de demência rapidamente progressiva. Após seis meses de evolução, sua pontuação do Mini exame do estado mental foi de 6, e no exame neurológico apresentava heminegligência e hemiparesia esquerda. O paciente faleceu 11 meses após início dos sintomas. RM do encéfalo mostrou microhemorragias em sequência gradiente-echo e áreas confluentes de hiperintensidade de substância branca nas imagens pesadas em T2. Na biópsia cerebral observou-se deposição de peptídeo b-amilóide em vasos sanguíneos, alguns rodeados por microhemorragias. Neste relato de caso, discutimos o papel da angiopatia amiloide cerebral no declínio cognitivo, devido a lesões estruturais associadas a hemorragias e infartos, lesões de substância branca e comorbidade com doença de Alzheimer, assim como a mais recentemente descrita inflamação relacionada a angiopatia amiloide.

Palavras-chave:
demência; angiopatia amiloide cerebral; demência vascular.

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References

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Geschwind MD, Shu H, Haman A, Sejvar JJ, Miller BL. Rapidly progressive dementia. Ann Neurol 2008;64:97-108.
²
Folstein MF, Folstein SE, McHugh PR. "Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198.
³
Maia LF, Mackenzie IR, Feldman HH. Clinical phenotypes of Cerebral Amyloid Angiopathy. J Neurol Sci 2007;257:23-30.
⁴
Yamada M. Cerebral amyloid angiopathy: an overview. Neuropathology 2000;20:8-22.
⁵
Greenberg SM, Vonsattel JP, Stakes JW, Gruber M, Finklestein SP. The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 1993;43:2073-2079.
⁶
Thal DR, Griffin WS, de Vos RA, Ghebremedhin E. Cerebral amyloid angiopathy and its relationship to Alzheimer's disease. Acta Neuropathol 2008;115:599-609.
⁷
Greenberg SM, Gurol ME, Rosand J, Smith EE. Amyloid angiopathy-related vascular cognitive impairment. Stroke 2004;35:2616-2619.
⁸
Pathological correlates of late-onset dementia in a multicentre, community-based population in England and Wales. Neuropathology Group of the Medical Research Council Cognitive Function and Ageing Study (MRC CFAS). Lancet 2001;357:169-175.
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Walker DA, Broderick DF, Kotsenas AL, Rubino FA. Routine use of gradient-echo MRI to screen for cerebral amyloid angiopathy in elderly patients. AJR Am J Roentgenol 2004;182:1547-1550.
¹⁰
Knudsen KA, Rosand J, Karluk D, Greenberg SM. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 2001;56:537-539.
¹¹
Werring DJ, Frazer DW, Coward LJ, et al. Cognitive dysfunction in patients with cerebral microbleeds on T2*-weighted gradient-echo MRI. Brain 2004;127:2265-2275.
¹²
Yakushiji Y, Nishiyama M, Yakushiji S, et al. Brain microbleeds and global cognitive function in adults without neurological disorder. Stroke 2008;39:3323-3328.
¹³
Oide T, Takahashi H, Yutani C, Ishihara T, Ikeda S. Relationship between lobar intracerebral hemorrhage and leukoencephalopathy associated with cerebral amyloid angiopathy: clinicopathological study of 64 Japanese patients. Amyloid 2003;10:136-143.
¹⁴
Attems J, Lauda F, Jellinger KA. Unexpectedly low prevalence of intracerebral hemorrhages in sporadic cerebral amyloid angiopathy: an autopsy study. J Neurol 2008;255:70-76.
¹⁵
Tian J, Shi J, Mann DM. Cerebral amyloid angiopathy and dementia. Panminerva Med 2004;46:253-264.
¹⁶
Kumar-Singh S. Cerebral amyloid angiopathy: pathogenetic mechanisms and link to dense amyloid plaques. Genes Brain Behav 2008;7(Suppl 1):67-82.
¹⁷
Smith EE, Greenberg SM. Beta-amyloid, blood vessels, and brain function. Stroke 2009;40:2601-2606.
¹⁸
Harkness KA, Coles A, Pohl U, Xuereb JH, Baron JC, Lennox GG. Rapidly reversible dementia in cerebral amyloid inflammatory vasculopathy. Eur J Neurol 2004;11:59-62.
¹⁹
Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology 2007;68:1411-1416.
²⁰
Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004;55:250-256.
²¹
Braak H, Braak E. Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol 1991;82:239-259.
²²
Mirra SS, Heyman A, McKeel D, et al. The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology 1991;41:479-486.
²³
Lippa CF, Hamos JE, Smith TW, Pulaski-Salo D, Drachman DA. Vascular amyloid deposition in Alzheimer's disease. Neither necessary nor sufficient for the local formation of plaques or tangles. Arch Neurol 1993;50:1088-1092.
²⁴
Warren JD, Schott JM, Fox NC, et al. Brain biopsy in dementia. Brain 2005;128:2016-2025.
²⁵
Josephson SA, Papanastassiou AM, Berger MS, et al. The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementia. J Neurosurg 2007;106:72-75.
²⁶
Tan EM, Feltkamp TE, Smolen JS, et al. Range of antinuclear antibodies in "healthy" individuals. Arthritis Rheum 1997;40:1601-1611.

Publication Dates

Publication in this collection
Oct-Dec 2009

History

Received
20 Aug 2009
Accepted
16 Oct 2009

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] ¹
Geschwind MD, Shu H, Haman A, Sejvar JJ, Miller BL. Rapidly progressive dementia. Ann Neurol 2008;64:97-108.

[2] ²
Folstein MF, Folstein SE, McHugh PR. "Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198.

[3] ³
Maia LF, Mackenzie IR, Feldman HH. Clinical phenotypes of Cerebral Amyloid Angiopathy. J Neurol Sci 2007;257:23-30.

[4] ⁴
Yamada M. Cerebral amyloid angiopathy: an overview. Neuropathology 2000;20:8-22.

[5] ⁵
Greenberg SM, Vonsattel JP, Stakes JW, Gruber M, Finklestein SP. The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 1993;43:2073-2079.

[6] ⁶
Thal DR, Griffin WS, de Vos RA, Ghebremedhin E. Cerebral amyloid angiopathy and its relationship to Alzheimer's disease. Acta Neuropathol 2008;115:599-609.

[7] ⁷
Greenberg SM, Gurol ME, Rosand J, Smith EE. Amyloid angiopathy-related vascular cognitive impairment. Stroke 2004;35:2616-2619.

[8] ⁸
Pathological correlates of late-onset dementia in a multicentre, community-based population in England and Wales. Neuropathology Group of the Medical Research Council Cognitive Function and Ageing Study (MRC CFAS). Lancet 2001;357:169-175.

[9] ⁹
Walker DA, Broderick DF, Kotsenas AL, Rubino FA. Routine use of gradient-echo MRI to screen for cerebral amyloid angiopathy in elderly patients. AJR Am J Roentgenol 2004;182:1547-1550.

[10] ¹⁰
Knudsen KA, Rosand J, Karluk D, Greenberg SM. Clinical diagnosis of cerebral amyloid angiopathy: validation of the Boston criteria. Neurology 2001;56:537-539.

[11] ¹¹
Werring DJ, Frazer DW, Coward LJ, et al. Cognitive dysfunction in patients with cerebral microbleeds on T2*-weighted gradient-echo MRI. Brain 2004;127:2265-2275.

[12] ¹²
Yakushiji Y, Nishiyama M, Yakushiji S, et al. Brain microbleeds and global cognitive function in adults without neurological disorder. Stroke 2008;39:3323-3328.

[13] ¹³
Oide T, Takahashi H, Yutani C, Ishihara T, Ikeda S. Relationship between lobar intracerebral hemorrhage and leukoencephalopathy associated with cerebral amyloid angiopathy: clinicopathological study of 64 Japanese patients. Amyloid 2003;10:136-143.

[14] ¹⁴
Attems J, Lauda F, Jellinger KA. Unexpectedly low prevalence of intracerebral hemorrhages in sporadic cerebral amyloid angiopathy: an autopsy study. J Neurol 2008;255:70-76.

[15] ¹⁵
Tian J, Shi J, Mann DM. Cerebral amyloid angiopathy and dementia. Panminerva Med 2004;46:253-264.

[16] ¹⁶
Kumar-Singh S. Cerebral amyloid angiopathy: pathogenetic mechanisms and link to dense amyloid plaques. Genes Brain Behav 2008;7(Suppl 1):67-82.

[17] ¹⁷
Smith EE, Greenberg SM. Beta-amyloid, blood vessels, and brain function. Stroke 2009;40:2601-2606.

[18] ¹⁸
Harkness KA, Coles A, Pohl U, Xuereb JH, Baron JC, Lennox GG. Rapidly reversible dementia in cerebral amyloid inflammatory vasculopathy. Eur J Neurol 2004;11:59-62.

[19] ¹⁹
Kinnecom C, Lev MH, Wendell L, et al. Course of cerebral amyloid angiopathy-related inflammation. Neurology 2007;68:1411-1416.

[20] ²⁰
Eng JA, Frosch MP, Choi K, Rebeck GW, Greenberg SM. Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol 2004;55:250-256.

[21] ²¹
Braak H, Braak E. Neuropathological stageing of Alzheimer-related changes. Acta Neuropathol 1991;82:239-259.

[22] ²²
Mirra SS, Heyman A, McKeel D, et al. The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology 1991;41:479-486.

[23] ²³
Lippa CF, Hamos JE, Smith TW, Pulaski-Salo D, Drachman DA. Vascular amyloid deposition in Alzheimer's disease. Neither necessary nor sufficient for the local formation of plaques or tangles. Arch Neurol 1993;50:1088-1092.

[24] ²⁴
Warren JD, Schott JM, Fox NC, et al. Brain biopsy in dementia. Brain 2005;128:2016-2025.

[25] ²⁵
Josephson SA, Papanastassiou AM, Berger MS, et al. The diagnostic utility of brain biopsy procedures in patients with rapidly deteriorating neurological conditions or dementia. J Neurosurg 2007;106:72-75.

[26] ²⁶
Tan EM, Feltkamp TE, Smolen JS, et al. Range of antinuclear antibodies in "healthy" individuals. Arthritis Rheum 1997;40:1601-1611.

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