scholarly journals Fabry disease causes angiitis of the central nervous system:a case report

2018 ◽  
Author(s):  
De-Zheng Kong ◽  
Ya-Hui Lian ◽  
Lin-Jing Wang ◽  
Chun-Mei Wang ◽  
Yang-Yang Meng ◽  
...  

Abstract Background: Fabry disease is very rare and often delayed in diagnosis. Described herein are Fabry disease causes angiitis of the central nervous system. MRI black blood sequence has a unique advantage in showing vascular wall. It can clearly show the angiitis. Case presentation: A 27-year-old man came to our hospital for treatment because of "diplopia 6d". The patient was eventually diagnosed with Fabry disease causes angiitis of the central nervous system by a series of examinations. Then,we treated patient with hormones and the symptoms relieved. Two months later,the initial vasculitis was gone,but the new vasculitis appeared. Four months later,the last lesion disappeared,but the new lesion appeared. Conclusions: This case prompts the clinician should use MRI black blood sequence scan in time when young patients have repeated strokes and the lesions are migratory. If vasculitis is found and other systemic lesions are combined,we should think of the possibility of Fabry disease.

2019 ◽  
Author(s):  
De-Zheng Kong ◽  
Ya-Hui Lian ◽  
Lin-Jing Wang ◽  
Chun-Mei Wang ◽  
Yang-Yang Meng ◽  
...  

Abstract Background Fabry disease is rare, and often the diagnosis is delayed. Here, we describe a case of Fabry disease resulting in vasculopathy of the central nervous system. Magnetic resonance (MR) black-blood sequence (three-dimensional T1 volumetric isotropic turbo spin echo acquisition), with a unique advantage of imaging the vascular wall, facilitated a clear identification of the vasculopathy. Case presentation A 27-year-old man visited our hospital for the treatment of " double vision 6d." After a series of examinations, the patient was diagnosed with Fabry disease, which caused vasculopathy of the central nervous system. Subsequently, the patient was treated with corticosteroids and his symptoms were attenuated. Two months after the initial treatment, the initial lesion formed in the vascular vessel disappeared, however, a new lesion appeared. Similarly, four months after the initial treatment, although the previous lesion disappeared, a new lesion appeared. Conclusions This case highlights that clinicians should use MR black-blood sequence scan in a timely manner in case of young patients with repeated strokes and migratory lesions. In case of detection of a vascular lesion in combination with other systemic lesions, the possibility of Fabry disease should be considered.


2019 ◽  
Author(s):  
De-Zheng Kong ◽  
Ya-Hui Lian ◽  
Lin-Jing Wang ◽  
Chun-Mei Wang ◽  
Yang-Yang Meng ◽  
...  

Abstract Background Fabry disease is rare, and often the diagnosis is delayed. Here, we describe a case of Fabry disease resulting in vasculopathy of the central nervous system. Magnetic resonance (MR) black-blood sequence (three-dimensional T1 volumetric isotropic turbo spin echo acquisition), with a unique advantage of imaging the vascular wall, facilitated a clear identification of the vasculopathy. Case presentation A 27-year-old man visited our hospital for the treatment of " double vision 6d." After a series of examinations, the patient was diagnosed with Fabry disease, which caused vasculopathy of the central nervous system. Subsequently, the patient was treated with corticosteroids and his symptoms were attenuated. Two months after the initial treatment, the initial lesion formed in the vascular vessel disappeared, however, a new lesion appeared. Similarly, four months after the initial treatment, although the previous lesion disappeared, a new lesion appeared. Conclusions This case highlights that clinicians should use MR black-blood sequence scan in a timely manner in case of young patients with repeated strokes and migratory lesions. In case of detection of a vascular lesion in combination with other systemic lesions, the possibility of Fabry disease should be considered.


2019 ◽  
Author(s):  
De-Zheng Kong ◽  
Ya-Hui Lian ◽  
Lin-Jing Wang ◽  
Chun-Mei Wang ◽  
Yang-Yang Meng ◽  
...  

Abstract Background: Fabry disease is rare, and the diagnosis is often delayed. Here, we describe a case of Fabry disease resulting in vasculopathy of the central nervous system. Magnetic resonance (MR) black-blood sequence (three-dimensional T1 volumetric isotropic turbo spin echo acquisition), with the unique advantage of imaging the vascular wall, facilitated a clear identification of the vasculopathy. Case presentation: A 27-year-old man visited our hospital for the treatment of " double vision 6d." After a series of examinations, the patient was diagnosed with Fabry disease, which caused vasculopathy of the central nervous system. Subsequently, the patient was treated with corticosteroids and his symptoms were attenuated. Two months after the initial treatment, the initial lesion in the vascular vessel disappeared, however, a new lesion appeared. Similarly, four months after the initial treatment, although the previous lesion disappeared, a new lesion appeared. Conclusions: This case highlights that clinicians should use MR black-blood sequence scan in a timely manner in case of young patients with migratory lesions of brain. In case of detection of a vascular lesion in combination with other systemic lesions, the possibility of Fabry disease should be considered.


2019 ◽  
Author(s):  
De-Zheng Kong ◽  
Ya-Hui Lian ◽  
Lin-Jing Wang ◽  
Chun-Mei Wang ◽  
Yang-Yang Meng ◽  
...  

Abstract Background: Fabry disease is rare, and the diagnosis is often delayed. Here, we describe a case of Fabry disease resulting in vasculopathy of the central nervous system. Magnetic resonance (MR) black-blood sequence (three-dimensional T1 volumetric isotropic turbo spin echo acquisition), with the unique advantage of imaging the vascular wall, facilitated a clear identification of the vasculopathy. Case presentation: A 27-year-old man visited our hospital for the treatment of " double vision 6d." After a series of examinations, the patient was diagnosed with Fabry disease, which caused vasculopathy of the central nervous system. Subsequently, the patient was treated with corticosteroids and his symptoms were attenuated. Two months after the initial treatment, the initial lesion in the vascular vessel disappeared, however, a new lesion appeared. Similarly, four months after the initial treatment, although the previous lesion disappeared, a new lesion appeared. Conclusions: This case highlights that clinicians should use MR black-blood sequence scan in a timely manner in case of young patients with migratory lesions of brain. In case of detection of a vascular lesion in combination with other systemic lesions, the possibility of Fabry disease should be considered.


2021 ◽  
Vol 21 ◽  
pp. S437-S438
Author(s):  
Carine Ribeiro Franzon ◽  
Andressa Oliveira Martin Wagner ◽  
Annelise Correa Wengerkievicz Lopes ◽  
Douglas Gebauer Bona ◽  
Talita Bertazzo Schmitz

2014 ◽  
Vol 54 (9) ◽  
pp. 709-714
Author(s):  
Taro Okunomiya ◽  
Takashi Kageyama ◽  
Kanta Tanaka ◽  
Daisuke Kambe ◽  
Akiyo Shinde ◽  
...  

1998 ◽  
Vol 84 (3) ◽  
pp. 408-411 ◽  
Author(s):  
Maria Laura Del Basso De Caro ◽  
Antonella Siciliano ◽  
Paolo Cappabianca ◽  
Alessandra Alfieri ◽  
Enrico de Divitiis

Paragangliomas are usually benign tumors which can be found in many sites of the body, from the base of the skull down to the pelvic floor. In the central nervous system the sellar region is very rarely involved; only three well studied cases have been reported to date. We present the cytological, histological, histochemical, immunocytochemical and ultrastructural features of an intrasellar and suprasellar paraganglioma in an 84-year-old man.


2014 ◽  
Vol 4 (1) ◽  
pp. 52-55
Author(s):  
R Habib ◽  
SB Mizan ◽  
A Rahman ◽  
NB Bhowmik ◽  
A Haque

Most clinicians consider a peripheral nerve lesion in patients with drop foot. However, causes stemming from the central nervous system represent rare, important, and underappreciated differential etiologies. Central causative lesions usually occur at locations where pyramidal tract connections are condensed and specific and the function is somatotopically organized. Here we report case presenting as central foot drop or spastic foot-drop and other myriad clinical features which after investigations was found due to bilateral parasagittal meningiomas. DOI: http://dx.doi.org/10.3329/birdem.v4i1.18559 Birdem Med J 2014; 4(1): 52-55


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