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Stroke and Transient Ischemic Attacks of the Brain and Eye
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
A definitive diagnosis of cerebral amyloid angiopathy can only be made after a postmortem examination that proves the vasculopathy. Supportive surgical pathology fulfills a probable diagnosis. A probable diagnosis also can be made if a patient older than 55 years has multiple lobar, cortical, or cortical–subcortical hemorrhages without another cause of hemorrhage, or a single ICH with cortical superficial siderosis.20
Late Effects of Treatment for Childhood Brain and Spinal Tumors
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Ralph Salloum, Katherine Baum, Melissa Gerstle, Helen Spoudeas, Susan R. Rose
Subacute effects of RT include intralesional and perilesional white-matter reactions seen on MRI as an abnormal signal on T1- and T2-weighted images or fluid-attenuated inversion recovery images, with or without focal enhancement. Late effects of irradiation include diffuse white-matter changes varying from scattered focal white-matter lesions202 to confluent lesions involving much of the periventricular or hemispheric white matter,203 diffuse cerebral atrophy, radiation-induced vasculopathy, mineralizing microangiopathy,204,205 and focal areas of frank radiation necrosis.206 In a study investigating complications of radiotherapy in 100 survivors of childhood CNS tumors treated with radiotherapy, cerebrovascular complications were reported in 36% of the patients with a mean age at radiotherapy of 8.6 years (range: 3–17). The most common complications were microbleeds (29%) and cavernomas (19%). In seven, late cerebrovascular complications were symptomatic: epilepsy (2), motor and language deficit (2), and sensorineural hearing loss and progressive ataxia (3) associated with cavernomas, stroke, and superficial siderosis, respectively. Follow-up duration was associated with an increased diagnosis of late cerebrovascular complications. Late cerebrovascular complications occurred more commonly in children treated with whole-brain RT (P = 0.046). Interestingly, factors such as sex, chemotherapy, and histological type of tumor were not correlated with the occurrence of late cerebrovascular complications.
Reporting session
Published in Deepak Subedi, Marialena Gregoriades, En Hsun Choi, John T Murchison, Graham McKillop, A Complete Guide to the Final FRCR 2B, 2011
Deepak Subedi, Marialena Gregoriades, En Hsun Choi, John T Murchison, Graham McKillop
Superficial siderosis of the CNS occurs as a result of chronic recurrent haemorrhage. Causes include dural arteriovenous malformations, tumours or previous CNS surgery. Sensorineural hearing loss, ataxia and multiple cranial nerve palsies are the usual presenting features. MRI shows a low-signal lining of haemosiderin over the brainstem, cerebellar folia and cranial nerves. This finding is most pronounced in gradient echo sequence, due to the susceptibility effects of haemosiderin.
Comparison of clinical features in transient focal neurological episodes between hereditary transthyretin type and Aβ type cerebral amyloid angiopathy
Published in Amyloid, 2019
Makoto Nakajima, Yosuke Takeuchi, Yoichiro Nagao, Teruaki Masuda, Taro Yamashita, Toshiro Yonehara, Tadashi Terasaki, Yukio Ando
A total of 13 (7 in the ATTR group and 6 in the Aβ group) patients was analysed. In patients with hereditary amyloidosis, Tyr114Cys was (n = 4), leptomeningeal type of mutation, was the most frequently observed, followed by other types included Val30Met, Ser50Ile and Ala25Thr. Liver transplantation has been performed in five patients. Age at the first TFNE was lower in the ATTR group than in the Aß group (mean 53 vs. 72 years old). Duration of TFNE varied especially in the Aβ group (10–60 in the ATTR group, and 1–220 in the Aβ group). Most of the patients had hypertension in the Aβ group; however, no one did in the ATTR group. Speech disturbance and sensory disturbance (paresthesia) in a unilateral hand were common symptoms in both groups. Hallucinations or other vision disturbance were present in four of seven patients in the ATTR group while absent in the Aß group. As to radiological findings, cerebral microbleeds were observed in most of the patients in both groups, and cortical superficial siderosis was detected in two patients in the ATTR group and in three in the Aβ group, while convexal subarachnoid haemorrhage in two only in the Aß group. No trends were observed in distribution of microbleeds and cortical superficial siderosis. Meningeal gadolinium-enhancement was observed in four ATTR group patients, whereas no one underwent enhanced MR imaging in the other group.
Optic Neuropathy Revealing Severe Superficial Siderosis in the Setting of Long-standing Low-grade Intracranial Neoplasm
Published in Neuro-Ophthalmology, 2022
Coralie Hemptinne, Adrienne Coche, Thierry Duprez, Philippe Demaerel, Christian Raftopoulos, Antonella Boschi
Initially described in 1908 by Hamill, superficial siderosis (SS) is characterised by haemosiderin deposits within the subpial layers of the brain, spinal cord, and cranial nerves resulting from massive or repeated subarachnoid haemorrhage.1 In SS cases, irreversible neurological and cranial nerve dysfunction may occur, which are predominantly sensorineural deafness, cerebellar ataxia, and pyramidal signs.1 An increasing number of SS cases have been reported since the availability of the magnetic resonance imaging (MRI) technique in clinical settings. The technique highlights haemosiderin deposits as strongly hypointense foci, mainly on gradient-echo (GRE) and susceptibility-weighted imaging (SWI) acquisitions.
Cerebral amyloid angiopathy: diagnosis and potential therapies
Published in Expert Review of Neurotherapeutics, 2018
Stewart A. Weber, Ranish K. Patel, Helmi L. Lutsep
Although microbleeds have received the most study in CAA, cortical superficial siderosis has also been associated with recurrent ICH risk, especially with increasing multifocality [98,99]. Cortical superficial siderosis may provide another way to stratify risk in such patients and help to determine whether antithrombotics should be used.