Sr. Editor,
Paciente feminina, 83 anos, apresentando cefaleia holocraniana diária, não pulsátil e refratária a analgésicos, associada a discreta paresia de membros inferiores há cerca de um mês. Portadora de hipertensão arterial sistêmica com bom controle medicamentoso. Negava trauma, febre e viagens recentes. Hemograma normal e anti-HIV e VDRL negativos. Tomografia computadorizada (TC) de crânio demonstrou hipodensidade difusa predominando na substância branca, associada a apagamento dos sulcos e cissuras (Figura 1A). Ressonância magnética (RM) demonstrou hiperintensidade em T2 e FLAIR, sem restrição à difusão, acometendo difusamente a substância branca periventricular e profunda, predominando nos lobos frontais, associada a múltiplos focos hipointensos na sequência para suscetibilidade magnética, sugestivos de micro-hemorragias (Figuras 1B e 1C). Diante dos achados, foi proposto o diagnóstico de angiopatia amiloide associada a inflamação, posteriormente confirmada por biópsia. Iniciou-se pulsoterapia com metilprednisolona, havendo melhora dos sintomas e dos achados de imagem duas semanas depois do tratamento instituído (Figura 1D).
A: TC, sem contraste, corte axial, demonstrando acentuada hipodensidade difusa, predominando na substância branca bilateral (setas), provocando redução da amplitude dos sulcos corticais e cissuras. B: RM, FLAIR, corte axial, demonstrando hiperintensidade difusa, predominando na substância branca bilateral dos lobos frontais (setas). C: RM, T2 gradiente-eco, demonstrando múltiplos focos hipointensos dispersos, preferencialmente distribuídos na junção corticossubcortical (setas), sugestivos de micro-hemorragias. D: RM, FLAIR, corte axial, demonstrando redução da hiperintensidade depois da pulsoterapia com metilprednisolona (setas).
A literatura radiológica brasileira vem, recentemente, ressaltando a importância dos exames de RM no aprimoramento do diagnóstico do sistema nervoso central(11 Niemeyer B, Muniz BC, Gasparetto EL, et al. Congenital Zika syndrome and neuroimaging findings: what do we know so far? Radiol Bras. 2017;50:314-22.,22 Niemeyer B, Muniz BC, Ventura N, et al. Papillary tumor of the pineal region accompanied by Parinaud's syndrome: magnetic resonance imaging findings. Radiol Bras. 2018;51:202-4.). A angiopatia amiloide associada a inflamação é uma doença rara que tipicamente afeta pacientes entre 60-80 anos, sem predileção por sexo, manifestando-se clinicamente por declínio cognitivo subagudo, cefaleia, convulsão, déficits neurológicos focais e manifestações neuropsiquiátricas(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.). A sua fisiopatologia não é bem conhecida, porém, consiste na acumulação patológica de beta-amiloide nas camadas média e adventícia de pequenos e médios vasos corticais e leptomeníngeos, associada a processo inflamatório perivascular linfocítico, sendo desconhecido qual processo ocorre primeiro(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.).
Na TC, a angiopatia amiloide associada a inflamação classicamente caracteriza-se por hipodensidade cortical e subcortical, unifocal, predominando nos lobos parietais; acometimento difuso pode ocorrer, mas é menos comum e geralmente tem aspecto assimétrico(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.). Na RM, observam-se hiperintensidades em T2 e FLAIR na substância branca, sem restrição à difusão, configurando edema vasogênico, associado a focos de permeio hipointensos na sequência para suscetibilidade magnética, devido a micro-hemorragias(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.). Pode haver, ainda, realce leptomeníngeo adjacente às áreas de edema, siderose superficial, infartos e hemorragias lobares, estas, porém, sendo menos frequentes que nos pacientes com angiopatia amiloide cerebral sem inflamação(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.).
O diagnóstico diferencial de múltiplos focos de micro-hemorragias é amplo, podendo-se citar como principais causas: angiopatia amiloide sem inflamação, angeíte associada a beta-amiloide, lesão axonal difusa, hipertensão arterial mal controlada, microangiopatias trombóticas, sepse, embolia gordurosa e malária(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.
7 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.-88 Niemeyer B, Niemeyer R, Abdalla G, et al. Amyloid ß-related angiitis of the central nervous system presenting with seizures and cognitive deficit. Eur Neurol. 2017;77:173-4.).
O tratamento consiste em pulsoterapia com metilprednisolona associada ou não a imunossupressores, como metotrexato, micofenolatomofetil ou mais comumente ciclofosfamida. Contudo, quase 60% dos pacientes morrem ou permanecem com alterações(33 Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
4 Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
5 Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
6 Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.-77 Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.).
Em conclusão, apesar de rara, a angiopatia amiloide associada a inflamação deve ser lembrada no diagnóstico diferencial de múltiplos focos de micro-hemorragia associados a edema, principalmente quando achados clinicolaboratoriais excluem outras possibilidades diagnósticas.
REFERENCES
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1Niemeyer B, Muniz BC, Gasparetto EL, et al. Congenital Zika syndrome and neuroimaging findings: what do we know so far? Radiol Bras. 2017;50:314-22.
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2Niemeyer B, Muniz BC, Ventura N, et al. Papillary tumor of the pineal region accompanied by Parinaud's syndrome: magnetic resonance imaging findings. Radiol Bras. 2018;51:202-4.
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3Miller-Thomas MM, Sipe AL, Benzinger TL, et al. Multimodality review of amyloid-related diseases of the central nervous system. Radiographics. 2016;36:1147-63.
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4Tolchin B, Fantaneanu T, Miller M, et al. Status epilepticus caused by cerebral amyloid angiopathy-related inflammation. Epilepsy Behav Case Rep. 2016;6:19-22.
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5Moussaddy A, Levy A, Strbian D, et al. Inflammatory cerebral amyloid angiopathy, amyloid-ß-related angiitis, and primary angiitis of the central nervous system: similarities and differences. Stroke. 2015;46:e210-3.
-
6Salvarani C, Morris JM, Giannini C, et al. Imaging findings of cerebral amyloid angiopathy, Aß-related angiitis (ABRA), and cerebral amyloid angiopathy-related inflammation: a single-institution 25-year experience. Medicine (Baltimore). 2016;95:e3613.
-
7Crosta F, Orlandi B, De Santis F, et al. Cerebral amyloid angiopathy-related inflammation: report of a case with very difficult therapeutic management. Case Rep Neurol Med. 2015;2015:483020.
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8Niemeyer B, Niemeyer R, Abdalla G, et al. Amyloid ß-related angiitis of the central nervous system presenting with seizures and cognitive deficit. Eur Neurol. 2017;77:173-4.
Datas de Publicação
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Publicação nesta coleção
Jan-Feb 2019
Histórico
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Recebido
12 Jul 2017 -
Aceito
23 Ago 2017