Cerebral amyloid angiopathy with related inflammation masquerading as crescendo transient ischaemic attacks

2021 ◽  
pp. practneurol-2021-003223
Author(s):  
Duncan Maddox ◽  
Kayla Ward ◽  
Thomas Robertson ◽  
Mike Boggild
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Neurological Disorders ◽  
Antithrombotic Therapy ◽  
Neurological Symptoms ◽  
Amyloid Angiopathy ◽  
Transient Ischaemic Attacks ◽  
Cerebral Biopsy ◽  
Cerebral Amyloid ◽  
Capsular Warning Syndrome

Cerebral amyloid angiopathy with related inflammation (CAA-RI) is an uncommon inflammatory subtype of CAA, with a variety of presentations that can mimic other focal and diffuse neurological disorders. We present a 63-year-old man with recurrent stereotyped focal neurological symptoms, who was initially diagnosed as capsular warning syndrome and treated with antithrombotic therapy. Atypical imaging led to further investigation including a cerebral biopsy, which confirmed CAA-RI; he improved clinically and radiologically with immunosuppression. This case highlights how CAA-RI is often under-recognised and that patients risk receiving inappropriate anticoagulation and delay in starting immunosuppression.

scholarly journals Slightly Symptomatic Cerebral Amyloid Angiopathy-Related Inflammation with Spontaneous Remission in Four Months

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Syuichi Tetsuka ◽  
Ritsuo Hashimoto
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Parietal Lobe ◽  
Vasogenic Edema ◽  
Brain Magnetic Resonance Imaging ◽  
Neurological Symptoms ◽  
Amyloid Angiopathy ◽  
Radiological Findings ◽  
Mri Findings ◽  
Spontaneous Improvement ◽  
Cerebral Amyloid

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare variant of CAA with autoimmune inflammation. A 77-year-old female experienced light-headedness during walking and mild ataxic gait without any other objective neuropsychological deficits. Brain magnetic resonance imaging (MRI) revealed an area of abnormal signal and mild parenchymal swelling in the right parietal lobe, indicating vasogenic edema. T2⁎-weighted gradient echo imaging revealed some subcortical microbleeds in the same lesion. Based on the proposed criteria for CAA-ri, she was diagnosed with probable CAA-ri. After 4 months, the spontaneous improvement was noted in the patient’s clinical and radiological findings. This report presents a rare and atypical case of CAA-ri in which the diagnosis was established after the patient underwent neuroimaging for only mild neurological symptoms, and the patient’s clinical and radiological findings displayed spontaneous improvement. Despite typical and striking MRI findings of CAA-ri, this patient only presented a minimal symptom; this dissociation could highlight the significance of not misinterpreting any new neurological symptoms. Thus, increased availability of MRI and growing awareness of CAA-ri might result in more incidentally diagnosed cases in the future. Furthermore, this case suggests that it would be better to strictly monitor the clinical-radiological findings of patients with probable CAA-ri who only present with minimal symptoms without the initiation of immunosuppressive therapy.

Aura attacks from acute convexity subarachnoid haemorrhage not due to cerebral amyloid angiopathy

Cephalalgia ◽  
2010 ◽  
Vol 31 (3) ◽  
pp. 368-371 ◽  
Author(s):  
DK Field ◽  
TJ Kleinig
Keyword(s):  
Subarachnoid Haemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Sinus Thrombosis ◽  
Case Series ◽  
Cerebral Venous Sinus Thrombosis ◽  
Amyloid Angiopathy ◽  
Transient Ischaemic Attacks ◽  
Cerebral Amyloid ◽  
Diagnostic Work ◽  
Work Up

Background and purpose: Convexity subarachnoid haemorrhage (cSAH) has recently been recognised as a cause of recurrent aura-like symptoms, mimicking transient ischaemic attacks (TIAs). Subarachnoid haemorrhage and recurrent aura-like episodes can occur in patients with cerebral amyloid angiopathy (CAA), which has been the presumed cause in the majority of reported cases. However, this syndrome can occur following cSAH secondary to other conditions, and it is important for clinicians to investigate and manage such patients appropriately. Method: Case series. Results: We describe two patients who presented with recurrent stereotyped transient neurological symptoms in the setting of acute cSAH identified on MRI. In one patient, SAH occurred secondary to cerebral venous sinus thrombosis. In the other, SAH was due to extension of a traumatic subdural haematoma. Conclusions: Conditions other than CAA can cause the clinicoradiological syndrome of cSAH with recurrent TIA-like events. Gradient echo or susceptibility-weighted imaging should be included in the diagnostic work-up of patients presenting with such events. When cSAH is detected, the full differential diagnosis for this should be considered. Aetiologies other than CAA can cause this syndrome and management can vary greatly depending on the underlying cause.

Cerebral amyloid angiopathy: an underdiagnosed cause of recurrent neurological symptoms

2021 ◽  
Vol 14 (4) ◽  
pp. e235949
Author(s):  
Fakharunisa Shah ◽  
Shayda Yazdani ◽  
Mark I'anson ◽  
Tahir Nazir
Keyword(s):  
Cerebral Amyloid Angiopathy ◽  
Brain Injuries ◽  
Hospital Admissions ◽  
Treatment Options ◽  
Functional Limitation ◽  
Amyloid Β ◽  
Neurological Symptoms ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid ◽  
The Brain

Cerebral amyloid angiopathy (CAA) is a common, yet frequently underdiagnosed pathology characterised by accumulation of amyloid β proteins in the small blood vessels of the brain. As a result, cerebrovascular dysregulation follows, leading to cerebral microbleeds, lobar intracerebral haematomas and sulcal subarachnoid haemorrhages. Gradual motor and cognitive decline due to these brain injuries leads to significant functional limitation in patients. We describe the case of a 69-year-old man requiring multiple hospital admissions with a variety of neurological symptoms. Following imaging of the brain, he was eventually diagnosed with CAA. We present a brief up-to-date literature review on epidemiology, pathophysiology, clinical features, diagnosis and treatment options for CAA.

Cerebral amyloid angiopathy with multiple intracerebral hemorrhages

1982 ◽  
Vol 57 (2) ◽  
pp. 286-289 ◽  
Author(s):  
Kenneth L. Tyler ◽  
Charles E. Poletti ◽  
Roberto C. Heros
Keyword(s):  
Intracerebral Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Neurological Disorders ◽  
Brain Parenchyma ◽  
Amyloid Angiopathy ◽  
Content Type ◽  
Intracerebral Hemorrhages ◽  
Cerebral Amyloid ◽  
Unclear Etiology ◽  
Adjacent Brain

✓ Cerebral amyloid angiopathy (CAA) has been associated with atypical dementia and a variety of other neurological disorders. Intracerebral hemorrhage is the most striking manifestation of CAA. A 63-year-old patient is described who sustained four intracerebral hemorrhages although he had no predisposing factors other than CAA. The neurological and pathological features of CAA are reviewed, with particular emphasis on the problem of CAA-associated intracerebral hemorrhage. On the basis of the experience with this case, it is recommended that adjacent brain parenchyma be biopsied in all patients undergoing evacuation of intracerebral hematomas of unclear etiology.

EVALUATION OF THE UNITED KINGDOM NATIONAL EXTERNAL QUALITY ASSESSMENT SERVICE 2008 (UK NEQAS 2008) GUIDELINES FOR THE DIAGNOSIS OF SUBARACHNOID HEMORRHAGE USING SPECTROPHOTOMETRIC XANTHOCHROMIA: A RETROSPECTIVE STUDY

2009 ◽  
Vol 32 (6S) ◽  
pp. 5
Author(s):  
A Gangloff ◽  
L Nadeau
Keyword(s):  
Subarachnoid Hemorrhage ◽  
Cerebral Amyloid Angiopathy ◽  
Viral Encephalitis ◽  
Objective Evaluation ◽  
Final Diagnosis ◽  
Amyloid Angiopathy ◽  
The United Kingdom ◽  
Cerebral Amyloid ◽  
The One ◽  
The Uk

Objective: Evaluation of the UK NEQAS 2008 guidelines for the interpretation of spectrophotometric xanthochromia. Method: A search of the laboratory database for all the xanthochromia test results between May 1st 2008 and May 1st 2009 was performed. Medical charts were reviewed for patients of Hôpital de l’Enfant-Jésus (HEJ) that had at least one detectable pigment (bilirubin, oxyhemoglobin, or methemoglobin). Xanthochromia results obtained with 4 different criteria (Chalmers original, Modified Chalmers, Duiser and UK NEQAS 2008) were compared. Results: We reviewed 41 medical charts (2 patients with duplicate lumbar punctures (LP) for a total of 43 LP). For these 41 patients there were 11 positive xanthochromia results, 5 of which were in concordance with a final diagnosis of subarachnoid hemorrhage (SAH). The diagnosis of the 6 other positive xanthochromia results were as follow: meningeal spread of a lymphoma, cerebral amyloid angiopathy, exertional headache, viral encephalitis with a possibility of petechiaes on the cerebral CT and second LP. Interpretation (negative/positive) of 40/43 LP was identical for the 4 methods. 2 LP were positive with Duiser and UK NEQAS 2008 but negative with Chalmers approaches (final diagnosis: SAH and cerebral amyloid angiopathy). 1 LP was positive only by the Duiser method (viral encephalitis). Conclusions: UK NEQAS 2008 guidelines identified all SAH but are sensitive to traumatic and pathologic meningeal lesions. Except for a case of viral encephalitis with a suspicion of cerebral petechiaes on CT, UK NEQAS 2008 gave xanthochromia results similar to the one in use at HEJ (Duiser). Chalmers original and Modified Chalmers methods missed one of the five SAH.

Cerebral Amyloid Angiopathy in Combination with Paroxysmal Atrial Fibrillation

2020 ◽  
Vol 25 (4) ◽  
pp. 31-37
Author(s):  
A. A. Kornilova ◽  
O. V. Lagoda ◽  
M. M. Tanashyan
Keyword(s):  
Atrial Fibrillation ◽  
Cerebral Amyloid Angiopathy ◽  
Paroxysmal Atrial Fibrillation ◽  
Past History ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Cerebral Haemorrhage ◽  
Exclusion Criterion ◽  
Amyloid Angiopathy ◽  
Cerebral Amyloid

The present article addresses the definition of cerebral amyloid angiopathy (CAA) and its symptoms based on the analysis of the medical case; the issues of diagnosis and treatment of this pathology are discussed. The Boston criteria, which became the basis for diagnosis, study of clinical manifestations and progression of CAA and approaches to its therapy, are presented. Methods and modes of neuroimaging, including magnetic resonance imaging (MRI), which verify micro cerebral haemorrhage, are described. At the same time, the role and significance of cardiac arrhythmias in the genesis of ischemic stroke are discussed, and scales for assessing the risk of its occurrence are presented. The observation of the neurological, somatic, neuroimaging, neuropsychological status of a 62-year-old patient confirms quite rare combination of probable CAA, paroxysmal atrial fibrillation and repeated hemorrhagic functional apoplexy (FA). The relevance of the case described, is a complex clinical dilemma based on mutually exclusive recommendations for the pharmacological correction of such conditions. It is emphasized that in many multicenter clinical studies on the effectiveness of antithrombotic medication (antiaggregants, anticoagulants) in the treatment and prevention of ischaemic functional apoplexy , an important exclusion criterion is a hemorrhagic stroke in past history (including the multiple changes in haemostasis indicators). Taking into account the obtained clinical and laboratory data in the dynamics, the tactics of treating the described patient were determined. The results of studies related to the treatment of comorbid pathology that should become the subject of the development of a personalized algorithm for managing patients in each specific case, are discussed.

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