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Cerebral Amyloid Angiopathy

Congophilic Angiopathy


Presentation

  • Overall, leptomeningeal involvement at presentation was present in 70.4% of patients with ABRA or CAA-RI and in only 7.4% of patients with CAA (P   0.0001).[ncbi.nlm.nih.gov]
  • Aside from the most common subacute presentations, CAA-ri can have a stroke-like presentation and be a stroke mimic.[ncbi.nlm.nih.gov]
  • We present a unique case of CAA in a middle-aged woman with a history of cerebral cortex microhemorrhages and cortical edema. Imaging and pathology findings suggested CAA as the main etiology of this presentation.[ncbi.nlm.nih.gov]
  • We sought to determine if SVD lesion burden differed between patients with CAA first presenting with TFNEs vs cognitive symptoms.[ncbi.nlm.nih.gov]
  • However, its presentation is varied; depending upon severity, it may not be diagnosed until autopsy.[ajnr.org]
Painter
  • Shinohara M 1 , Murray ME 1 , Frank RD 2 , Shinohara M 1 , DeTure M 1 , Yamazaki Y 1 , Tachibana M 1 , Atagi Y 1 , Davis MD 1 , Liu CC 1 , Zhao N 1 , Painter MM 1 , Petersen RC 3 , Fryer JD 1 , Crook JE 2 , Dickson DW 1 , Bu G 4 , Kanekiyo T 5 .[ncbi.nlm.nih.gov]
Rigor
  • CONCLUSIONS: Subarachnoid extension and an irregular ICH border are common imaging features of CAA-associated ICH, but methodologically rigorous diagnostic test accuracy studies are required to determine the sensitivity and specificity of these features[ncbi.nlm.nih.gov]
Difficulty Walking
  • CAA-related macrohemorrhage with associated subdural hemorrhage in a 77-year-old man with severe headache and difficulty walking.[doi.org]
Generalized Seizure
  • After a generalized seizure she presented with right-side hemiparesis with signs of uncal herniation and remained unconscious.[ncbi.nlm.nih.gov]
  • A 79-year-old woman was admitted to the Neurological Department after the occurrence of generalized seizures, the first in her life. On admission she was unconscious with right hemiparesis and deviation of eyes to the left.[ncbi.nlm.nih.gov]
Sensory Seizure
  • We report a clinical case series of 3 patients with cSAH associated with probable cerebral amyloid angiopathy (CAA) who presented with focal sensory seizures and responded well to corticosteroid treatment.[ncbi.nlm.nih.gov]

Workup

  • An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic.[elynsgroup.com]
  • Consultations The following consultations are useful: Neurologic evaluation for clinical evaluation, diagnostic workup, and management Neurosurgical consultation in cases of ICH Neuropsychological assessment for cognitive impairment CT and PET Scanning[emedicine.com]
  • During her admission in May 2014, she was admitted again with acute confusion, headache, and vomiting and was hospitalized for workup of her acute encephalopathy. MRI brain showed a new right temporo-parietal ICH with surrounding vasogenic edema.[innovationscns.com]
Mass Lesion of the Brain
  • We present two cases (female and male patients, aged 64 and 38, respectively) of focal mass lesions mimicking a brain tumour: one with cognitive function deficit, memory troubles, behavioral changes and left hemiparesis, the other with difficulty in orientation[ncbi.nlm.nih.gov]

Treatment

  • RESULTS: Among patients given systemic lytic treatment for stroke, those who have microhemorrhages tend to have a higher risk of treatment-associated brain hemorrhage.[ncbi.nlm.nih.gov]
  • Previous treatment with antiplatelet drugs was discontinued. His episodes of visual aura stopped on treatment with lamotrigine and add-on treatment with verapamil.[ncbi.nlm.nih.gov]
  • Immunosuppressive therapy appears to be an appropriate treatment for CAA encephalopathy.[ncbi.nlm.nih.gov]
  • In contrast, apart from hypertension treatment, few therapeutic options exist in cerebral amyloid angiopathy.[ncbi.nlm.nih.gov]
  • The second hematoma was evacuated, and surgical treatment together with medical therapy improved patient's quality of life.[ncbi.nlm.nih.gov]

Prognosis

  • This case reminds us early recognition of brain abscess formation at the site of intracerebral hemorrhage is very important for prompt and appropriate treatment to improve the overall prognosis.[ncbi.nlm.nih.gov]
  • To the best of our knowledge, there has been only one previous case of pathology-proven CAA-I without CMBs reported and this was associated with a good prognosis.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Patients with HCHWA-D have worse long-term prognosis after a first ICH than patients with sCAA.[ncbi.nlm.nih.gov]
  • Diagnosis and Prognosis: The diagnosis requires a multidisciplinary approach by neurologists and ophthalmologists. No treatment is available other than cataract surgery if necessary and most patients do not live beyond about the 5 th or 6 th decades.[disorders.eyes.arizona.edu]
  • Diagnosis - Cerebral Amyloid Angiopathy Prognosis - Cerebral Amyloid Angiopathy Not supplied. Treatment - Cerebral Amyloid Angiopathy Resources - Cerebral Amyloid Angiopathy[checkorphan.org]

Etiology

  • Spontaneous convexity SAH in the elderly has heretofore been of uncertain origin until several recent case reports have suggested cerebral amyloid angiopathy (CAA) as the etiology.[ncbi.nlm.nih.gov]
  • Imaging and pathology findings suggested CAA as the main etiology of this presentation. This is a very rare case of CAA coexisting with aneurysmal subarachnoid hemorrhage.[ncbi.nlm.nih.gov]
  • Although the etiology of the white matter lesion was not specified, the lesion and symptoms improved with steroid pulse therapy.[jstage.jst.go.jp]
  • An extensive workup including HIV testing, CSF examination for infectious etiology including protein 14-3-3 and demylineating disease was negative. Paraneoplastic and autoimmune workup was also non-diagnostic.[elynsgroup.com]

Epidemiology

  • In this article, we aimed to present a systematic review of cerebral amyloid angiopathy related disorders with regard to their epidemiology, pathophysiology, clinical features, neuroimaging biomarkers, diagnosis criteria, and treatment.[ingentaconnect.com]
  • But he said there are no good epidemiological data to put an absolute number on it.[medpagetoday.com]
  • Epidemiology Occurrence in the United States The true incidence and prevalence of cerebral amyloid angiopathy (CAA) are hard to specify, as definite CAA is a pathologic diagnosis typically obtained postmortem.[emedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • Its precise pathophysiology remains uncertain and we currently have limited evidence on which immunosuppressive agents are the most effective in its treatment.[ncbi.nlm.nih.gov]
  • In this article we aim to cover the pathophysiology, clinical findings, radiological appearances and approach to management of cerebral amyloid angiopathy.[ncbi.nlm.nih.gov]
  • The pathophysiology of microbleeds and ICH in CAA is not well understood, but it is thought to be the result of vessel weakening and rupture secondary to amyloid deposition.[ncbi.nlm.nih.gov]
  • This study provides evidence for heterogeneous pathologic substrates and possibly different pathophysiologic mechanisms underlying MRI-observed cortical microbleeds in the context of advanced CAA and Alzheimer disease.[ncbi.nlm.nih.gov]
  • Over the past few years, it has become clear that, at the pathophysiological level, cerebral amyloid angiopathy appears to be in part a protein elimination failure angiopathy and that this dysfunction is a feed-forward process, which potentially leads[ncbi.nlm.nih.gov]

Prevention

  • In this review, we summarize the risks linked to CAA with respect to the treatment and prevention of stroke.[ncbi.nlm.nih.gov]
  • General Hospital of Tianjin Medical University, 154 An Shan Road, Tianjin 300052, PR China. 3 Department of Laboratory, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention[ncbi.nlm.nih.gov]
  • Our data indicate that the selective up-regulation of P-gp could enhance the efficacy of Aβ immunization in the treatment or prevention of AD. Copyright Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.[ncbi.nlm.nih.gov]
  • In conclusion, taxifolin prevents amyloid-β oligomer assembly and fully sustains cognitive and cerebrovascular function in a CAA model mice. Taxifolin thus appears a promising therapeutic approach for CAA.[ncbi.nlm.nih.gov]
  • Causes - Cerebral Amyloid Angiopathy Prevention - Cerebral Amyloid Angiopathy Not supplied. Diagnosis - Cerebral Amyloid Angiopathy Prognosis - Cerebral Amyloid Angiopathy Not supplied.[checkorphan.org]

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