Presentation
Clinical Presentation The clinical presentations in these five cases included confusion or cognitive decline in four patients, focal neurologic deficit in three, headache in one, and seizures in one. [ajronline.org]
Autosomal dominant — A form of inheritance in which only one copy of a gene coding for a disease need be present for that disease to be expressed. If either parent has the disease, a child has a 50% chance of inheriting the disease. [medical-dictionary.thefreedictionary.com]
Two patients developed cerebellar ataxia, three of them present transient focal neurological episodes, one dementia and, the last one, the diffuse superficial siderosis of the CNS is a radiological finding. [ncbi.nlm.nih.gov]
Here we present a case in which cerebral amyloid angiopathy (CAA) was found in a patient with RPD. [demneuropsy.com.br]
The temporal resolution of the image sampling was not compromised by the modifications and the number of presentations (four per run) and on/off frequency of the stimulus presentation (20 sec on/28 sec off) did not change from the prior reports. [onlinelibrary.wiley.com]
Entire Body System
- Swelling
A, Axial T2-weighted MR image shows translobar T2 hyperintense mass in right parietal lobe with cortical swelling and effacement. [ajronline.org]
Aggressive neurosurgical management allows increased survival following lobar hemorrhage, but as of 1998, 20-90% of patients die from the first hemorrhage or its complications, which include progression of hemorrhage, brain edema (swelling) with herniation [medical-dictionary.thefreedictionary.com]
Cardiovascular
- Hypertension
The bleeding within the brain is usually confined to a particular lobe [5] and this is slightly different compared to brain bleeds which occur as a consequence of high blood pressure ( hypertension ) - a more common cause of a hemorrhagic stroke (or bleeding [en.wikipedia.org]
White matter hyperintensity patterns in cerebral amyloid angiopathy and hypertensive arteriopathy. Neurology 2016; 86: 1-7. [ Links ] 59. Gregorie S, Charidimou A, Gadapa N, Dolan E, Antoun N, Peeters A, et al. [scielo.cl]
He had suffered from diabetes mellitus and systemic arterial hypertension for thirty years. In his past medical history, he had also undergone a right hip replacement and right transtibial amputation (for which he used a prosthesis for ambulation). [demneuropsy.com.br]
Psychiatrical
- Aggressive Behavior
By that point, he was also having delusions (such as saying his late father was home) and aggressive behavior. [demneuropsy.com.br]
Neurologic
- Headache
Description Late-onset migraine-like aura attacks without headache or with mild headache, also termed “amyloid spells”, caused by and associated with the other clinical features of cerebral amyloid angiopathy, often in the setting of convexal subarachnoid [ichd-3.org]
Seizures Stupor or coma (rarely) Vomiting If bleeding is not severe or widespread, symptoms can include: Episodes of confusion Headaches that come and go Loss of mental function (dementia) Weakness or unusual sensations that come and go, and involve [account.allinahealth.org]
Onset is at age 40-60 with headaches, brain hemorrhage often in the parietal lobe, strokes, and dementia. More than half of patients die from their first hemorrhage. [medical-dictionary.thefreedictionary.com]
Clinical Presentation The clinical presentations in these five cases included confusion or cognitive decline in four patients, focal neurologic deficit in three, headache in one, and seizures in one. [ajronline.org]
For reasons still unknown, a subset of patients with severe CAA may develop a picture of encephalopathy with subacute cognitive decline, seizures and/or headache that may respond (with both clinical and radiological improvement) to anti-inflammatory treatment19 [demneuropsy.com.br]
- Clumsiness
In some cases, rehabilitation is needed for weakness or clumsiness. This can include physical, occupational, or speech therapy. Sometimes, medicines that help improve memory, such as those for Alzheimer disease, are used. [account.allinahealth.org]
- Neurologic Manifestation
CAA may manifest clinically not only as lobar hemorrhage - the most recognized manifestation - but also as ischemic infarctions, subarachnoid hemorrhage, transient focal neurological manifestations (caused by focal seizures or transient ischemic attacks [demneuropsy.com.br]
Treatment
There were no deaths or treatment–related serious adverse events, as judged by the site investigator and central medical monitor, in the ponezumab treatment group. [onlinelibrary.wiley.com]
There is no known effective treatment. [account.allinahealth.org]
Treatment Although there is no effective treatment for the underlying disease process of CAA, measures can be taken to prevent brain hemorrhage in patients diagnosed with CAA. [medical-dictionary.thefreedictionary.com]
Furthermore, three of our patients responded to treatment with steroids, and the conditions of two improved without specific treatment. [ajronline.org]
^ Advances in Dementia Research and Treatment: 2012 Edition. ScholarlyEditions. 2012-12-26. ISBN 9781464991721. ^ Mittal, S.; Wu, Z.; Neelavalli, J.; Haacke, E. M. (2009). [en.wikipedia.org]
Prognosis
Imaging tests of the head that may be done include: CT scan or MRI scan to check for bleeding in the brain MRA scan to check for large bleeds and rule out other causes of bleeding PET scan to check for amyloid deposits in the brain Outlook (Prognosis) [account.allinahealth.org]
Prognosis Since CAA is associated with progressive blood vessel degeneration, and since there is no effective treatment, most patients have a poor prognosis. [medical-dictionary.thefreedictionary.com]
Incidence, 30 days case-fatality rate and prognosis of stroke in Iquique, Chile: results of a two years community based prospective study (PISCIS Proyect). [scielo.cl]
Etiology
Atraumatic convexal subarachnoid hemorrhage clinical presentation, imaging patterns, and etiologies. Neurology 2010; 74: 893-9. [ Links ] 26. Beitzke M, Gattringer T, Enzinger C, Wagner G, Niederkorn K, Fazekas F. [scielo.cl]
Rapidly progressive dementia (RPD), although more typically associated with prion diseases, can be caused by various conditions.1 These include neurodegenerative, autoimmune, toxic/metabolic and vascular etiologies, which must therefore be sought whenever [demneuropsy.com.br]
Epidemiology
Stroke epidemiology: a review of population-based studies of incidence, prevalence, and case-fatality in the late 20th century. Lancet Neurol 2003; 2 (1): 43-53. [ Links ] 3. Tapia J, Corbalán R, Huete I, Valdés F, Villanueva C, Córdoba J, et al. [scielo.cl]
Pathophysiology
Sporadic cerebral amyloid angiopathy revisited: recents insights into pathophysiology and clinical spectrum. J Neurol Neurosurg Psychiatry 2012; 83: 124-37. [ Links ] 9. Scholz W. [scielo.cl]
Prevention
We recently demonstrated that in a mouse model of CAA, taxifolin improved cerebral blood flow, promoted amyloid-β removal from the brain, and prevented cognitive dysfunction when administered orally. [pnas.org]
Anti-epileptic drugs are sometimes given to patients with large lobar hemorrhage in an attempt to prevent seizures, although the benefit of this is unclear. [medical-dictionary.thefreedictionary.com]
^ "Brain Basics: Preventing Stroke: National Institute of Neurological Disorders and Stroke (NINDS)". www.ninds.nih.gov. Retrieved 2015-05-27. ^ Sirven, Joseph I.; Malamut, Barbara L. (2008). Clinical Neurology of the Older Adult. [en.wikipedia.org]
Although there are no known prevention strategies, appropriate diagnosis of tumefactive CAA may help prevent morbidity resulting from unnecessary aggressive therapy. 1. Gandhi D, Wee R, Goyal M. [ajronline.org]
As A β 1–40 is the predominant species present in blood vessel walls, ponezumab was developed to prevent or reverse β ‐amyloid aggregation and deposition and thus prevent or reduce CAA progression with minimal ARIA risk. 15 The current study sought to [onlinelibrary.wiley.com]