Angiopathia amyloido cerebralis

Angiopathia amyloido cerebralis (AAC, abbreviatura Anglica: CAA) sive angiopathia congophila (ICD-10: I68.0) est morbus depositionibus in cerebralium parietibus arteriarum minorum amyloidi manifestus^[1]. In cursu morbi eruptiones sanguinis e vasis haemorrhagiae in lobis cerebri oriuntur. Apparent plures genera, distributione enim amyloidi varia ac communiter cum factoribus geneticis, ut polymorphismo apolipoproteini E (ApoE)^[2].

Cave: notitiae huius paginae nec praescriptiones nec consilia medica sunt.

Tomographia cerebralis haemorrhagias intracerebrales, et corticales et subcorticales, ostendit. Periculum apoplexiae cerebri et dementiae et encephalopathiae augmentatur.

Symptomata morbi tantisper, in parte funditus, occulta restant, dum detrimentum parenchymatis cerebri gravius est. Tum demum symptomata apoplectica, dementiva, encephalopathica agnosci queant.

Indoles morbi

Natura pathologica

AAC: Amyloidum beta sub microsopio post immunocolorationem intra vasorum parietes parva vista.

Essentia angiopathiae amyloido cerebralis est aggregatio proteinorum in parietibus vasorum cerebralium minorum (arteriolarum, vasorum capillarium, venularum) saepe certorum cerebri loborum leptomeningumque, quo accumulatio intra parietem vascularem illam detrimentum patitur adeo, ut microthromboses, ruptura vasorum et haemorrhagias sequantur. Quamquam damnum toto in neocortice vel meningibus obtegentibus observatur, in senectute imprimis lobus occipitalis affectus videtur^[3].

Investigata sunt diversa genera proteinorum aggregatorum, sed semper singulari typus exprimi videtur. Plerumque copia amyloidi beta, quod etiam in morbo Alzheimeriano invenitur, conseritur, sed etiam, rarius, alia, nimiopere cerebrum temptant, ut^[4]:

Cystatinum C (ACys, "typus Islandicus", OMIM: 105150),
ITM2B ("typus Britannicus", OMIM: 176500)
Transthyretinum (ATTR),
Gelsolinum (AGel).

Aggregatio amyloidi

Duo genera angiopathiarum histopathologice descripta sunt: angiopathia congophilica et angiopathia dysphorica, quamquam utraque semper communiter invenitur.

Symptomata et signa

In principio morbum hunc dignosci non facile est idcirco, quia non raro symptomata morbi et longe et indistincte restat. In cursu morbi symptomata eruptione causa sanguinis e vaso oriuntur, minoria vel maiora, cum malis paucis, sed etiam cum gravioria ut convulsiones, doloribus capitis, atque tardius dementia^[5]. Generaliter dictum, angiopathiae amyloido cerebrales quattuor modis promulgare solent^[6]:

Symptomata haemorrhagia intracerebrali lobulari
Symptomata dementiae
Symptomata casuum neurologicorum transeuntium
Symptomata encephalopathiae

Diagnosis

Cerebralis imago resonantia magnetica Tesla 1.5 collocans focos signis levibus, quae eruptiones sanguinis multiplices (haemorrhagias) repraesentant. Sinstra imago T2* ponderata, medio imago susceptibilitate ponderata (SWI), atque dextra imago phasis SWI.

Usque dum technologiae tomographicae aberant, diagnosis excisione equidem partis parvae telae cerebri (biopsia) pendebat. Imagines hodie et tomographiae radiis X operantis ope et tomographiae resonantia magnetica multiplices sanguinis eruptiones parvas ("microhaemorrhagias") intracerebrales collocare possunt. Etiam amyloidi PET adhibetur^[7]. In liquore cerebrospinali valor Aβ40 altior est.

Therapia

Adhuc tractatio causarum ignota est, quamquam deminuere valores hypertensivos effectus beneficium dare possunt. Si adest haemorrhagia intracerebralis maior, tractatatio neurochirurgica necesse possit, quamquam prognosis in senectute dubius videtur^[8].

Notae

↑ Definitio morbi apud MedlinePlus. (Anglice).
↑ Thal D. R., Ghebremedhin E., Rüb U., Yamaguchi H., Del Tredici K., Braak H. (2002). "Two types of sporadic cerebral amyloid angiopathy". Journal of neuropathology and experimental neurology 61 (3): 282-93 .
↑ Yamada M., Tsukagoshi H., Otomo E., Hayakawa M. (1987). "Cerebral amyloid angiopathy in the aged". Journal of neurology 234 (6): 371-6 .
↑ Yamada M. (2004). "Cerebral amyloid angiopathy and gene polymorphisms". Journal of the neurological sciences 226 (1-2): 41-1
↑ Yoshimura M., Yamanouchi H., Kuzuhara S., Mori H., Sugiura S., Mizutani T., Shimada H., Tomonaga M., Toyokura Y. (1992). "Dementia in cerebral amyloid angiopathy: a clinicopathological study". Journal of neurology 239 (8): 441-50 .
↑ Werring D., Banerjee G. (2016). "Cerebral amyloid angiopathy and intracerebral haemorrhage". ACNR 16 (2): 8-12
↑ Banerjee G., Carare R., Cordonnier C., Greenberg S. M., Schneider J. A., Smith E. E., Buchem M. V., Grond J. V., Verbeek M. M., Werring D. J. (2017). "The increasing impact of cerebral amyloid angiopathy: essential new insights for clinical practice". Journal of neurology, neurosurgery, and psychiatry 88 (11): 982-94 .
↑ Zhang Y., Wang X., Schultz C., Lanzino G., Rabinstein A. A. (2012). "Postoperative outcome of cerebral amyloid angiopathy-related lobar intracerebral hemorrhage: case series and systematic review". Neurosurgery 70 (1): 125-30 .

Nexus interni

[1] Definitio morbi apud MedlinePlus. (Anglice).

[2] Thal D. R., Ghebremedhin E., Rüb U., Yamaguchi H., Del Tredici K., Braak H. (2002). "Two types of sporadic cerebral amyloid angiopathy". Journal of neuropathology and experimental neurology 61 (3): 282-93 .

[3] Yamada M., Tsukagoshi H., Otomo E., Hayakawa M. (1987). "Cerebral amyloid angiopathy in the aged". Journal of neurology 234 (6): 371-6 .

[4] Yamada M. (2004). "Cerebral amyloid angiopathy and gene polymorphisms". Journal of the neurological sciences 226 (1-2): 41-1

[5] Yoshimura M., Yamanouchi H., Kuzuhara S., Mori H., Sugiura S., Mizutani T., Shimada H., Tomonaga M., Toyokura Y. (1992). "Dementia in cerebral amyloid angiopathy: a clinicopathological study". Journal of neurology 239 (8): 441-50 .

[6] Werring D., Banerjee G. (2016). "Cerebral amyloid angiopathy and intracerebral haemorrhage". ACNR 16 (2): 8-12

[7] Banerjee G., Carare R., Cordonnier C., Greenberg S. M., Schneider J. A., Smith E. E., Buchem M. V., Grond J. V., Verbeek M. M., Werring D. J. (2017). "The increasing impact of cerebral amyloid angiopathy: essential new insights for clinical practice". Journal of neurology, neurosurgery, and psychiatry 88 (11): 982-94 .

[8] Zhang Y., Wang X., Schultz C., Lanzino G., Rabinstein A. A. (2012). "Postoperative outcome of cerebral amyloid angiopathy-related lobar intracerebral hemorrhage: case series and systematic review". Neurosurgery 70 (1): 125-30 .

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