Necrobiotic Xanthogranuloma of the Eyelids and Face, a Rare Systemic Disease: MRI Findings

Neurographics ◽  
2018 ◽  
Vol 8 (3) ◽  
pp. 218-220
Author(s):  
F.T. Pacheco ◽  
R. Correia ◽  
C.E. Iacob ◽  
H.J. Cohen ◽  
S. Jagannath ◽  
...  
Keyword(s):  
Systemic Disease ◽  
Mri Findings

Otological aspects of NLRP3-related autoinflammatory disorder focusing on the responsiveness to anakinra

Rheumatology ◽  
2020 ◽  
Author(s):  
Bong Jik Kim ◽  
Young Ho Kim ◽  
Seungmin Lee ◽  
Jin Hee Han ◽  
Sang-Yeon Lee ◽  
...  
Keyword(s):  
De Novo ◽  
Systemic Disease ◽  
Brain Mri ◽  
Hearing Threshold ◽  
Periodic Syndrome ◽  
Mri Findings ◽  
Hearing Status ◽  
Systemic Symptoms ◽  
Vitro Analysis

Abstract Objectives Gradually progressive sensorineural hearing loss (SNHL) is a prevalent sensory defect. It is generally untreatable, making rehabilitation by hearing aid or cochlear implantation the only option. However, SNHL as one of the symptoms of the hereditary autoinflammatory systemic disease cryopyrin-associated periodic syndrome, or as the only symptom of the cochlea-specific form (DFNA34), was suggested to respond to IL-1 antagonist (anakinra) therapy, which ameliorates NLRP3 variants-induced over-secretion of IL-1β. We analysed genotypic and phenotypic spectrum of cryopyrin-associated periodic syndrome or DFNA34, specifically focusing on the responsiveness of SNHL to anakinra. Methods Seventeen families diagnosed with either cryopyrin-associated periodic syndrome or DFNA34 were recruited. Genotyping and phenotyping including audiogram, MRI findings, and in vitro IL-1β assay were performed. Results Our cohort had an etiologic homogeneity of 94.1% to NLRP3 variants and a high de novo occurrence (84.6%). We identified the second DNFA34 pedigree worldwide with a novel NLRP3 variant supported by in vitro analysis. Significant improvement of hearing status against the natural course, showing response to anakinra, was identified in three probands, one of whom used to have severe SNHL. Hearing threshold worse than 60 dB at the start of anakinra and cochlear enhancement on brain MRI seemed to be related with poor audiologic prognosis and responsiveness to anakinra therapy despite stabilized systemic symptoms and inflammatory markers. Conclusion We propose a constellation of biomarkers comprising NLRP3 genotypes, hearing status at diagnosis, and cochlear radiological findings as prognostic factors of hearing status after anakinra treatment and possibly as sensitive parameters for treatment dosage adjustment.

scholarly journals MRI and clinical characteristics of suspected cerebrovascular accident in nine cats

2017 ◽  
Vol 20 (8) ◽  
pp. 674-684 ◽  
Author(s):  
Danielle E Whittaker ◽  
Randi Drees ◽  
Elsa Beltran
Keyword(s):  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Systemic Disease ◽  
Acute Onset ◽  
Short Term ◽  
Mri Findings ◽  
Mri Features ◽  
Disease Occurrence ◽  
Short And Long Term ◽  
Concurrent Disease

Objectives Cerebrovascular accidents (CVAs) are infrequently reported in cats. To date, clinical characteristics, including lesion localisation and MRI findings, have only been reported in two cats. The aim of the current study is to document MRI findings in cats presenting with CVAs over an 11 year period. Cases were reviewed according to initial clinical presentation, subsequent physical and neurological findings, predisposing systemic disease and short- and long-term (when available) outcome with a view to identifying any typical pattern in disease occurrence. Methods Patient records of cats presenting to a single referral centre from January 2005 to September 2016 with acute onset, non-progressive (after 24 h) intracranial signs compatible with a CVA and where an MRI was performed within 72 h were retrospectively reviewed. Results Nine cats met the inclusion criteria. All cats had ischaemic CVAs (presumptively diagnosed in eight cats and confirmed in one cat following post-mortem examination). No cases of haemorrhagic CVAs were identified. Four cats presented with territorial infarcts that were confined to the territory of the rostral or caudal cerebellar arteries (n = 4). Lacunar infarcts were identified in five cats in the location of the cerebrum (n = 1), the thalamus/midbrain (n = 2) and the medulla oblongata (n = 2). Concurrent systemic disease was identified in most (n = 8/9). In the present study short-term prognosis was favourable and 8/9 cats survived to 48 h following admission. Conclusions and relevance CVAs in cats occur in the same vascular territories as in dogs and have similar MRI features. This study notes that the presenting cats had a high likelihood of concurrent disease (8/9 cases) but had a favourable short-term prognosis, if neither the clinical presentation nor concurrent disease were severe.

A series of 34 patients with Rasmussen's encephalitis: Clinical course, EEG- and MRI findings

2008 ◽  
Vol 39 (05) ◽  
Author(s):  
M Beckhaus ◽  
F Woermann ◽  
B Pohlmann-Eden
Keyword(s):  
Clinical Course ◽  
Mri Findings ◽  
Rasmussen’S Encephalitis

Preoperative localization of parathyroid glands

2004 ◽  
Vol 43 (03) ◽  
pp. 85-90 ◽  
Author(s):  
E. Lopez Hänninen ◽  
Th. Steinmüller ◽  
T. Rohlfing ◽  
H. Bertram ◽  
M. Gutberlet ◽  
...  
Keyword(s):  
Image Fusion ◽  
Single Photon ◽  
Imaging Techniques ◽  
Photon Emission ◽  
Parathyroid Glands ◽  
Preoperative Localization ◽  
Mri Findings ◽  
99Mtc Mibi ◽  
Weighted Sequences ◽  
Mibi Scintigraphy

Summary Aim: Minimally invasive resection of hyperfunctional parathyroid glands is an alternative to open surgery. However, it requires a precise preoperative localization. This study evaluated the diagnostic use of magnetic resonance (MR) imaging, parathyroid scintigraphy, and consecutive image fusion. Patients, methods: 17 patients (9 women, 8 men; age: 29-72 years; mean: 51.2 years) with primary hyperparathyroidism were included. Examination by MRI used unenhanced T1- and T2-weighted sequences as well as contrast-enhanced T1-weighted sequences. 99mTc-MIBI scintigraphy consisted of planar and SPECT (single photon emission tomography) imaging techniques. In order to improve the anatomical localization of a scintigraphic focus, SPECT-data were fused with the corresponding MR-data using a modified version of the Express 5.0 software (Advanced Visual Systems, Waltham, MA). Results of image fusion were then compared to histopathology. Results: In 14/17 patients, a single parathyroid adenoma was found. There were 3 cases with hyperplastic glands. MRI detected 10 (71%), scintigraphy 12 (86%) adenomas. Both modalities detected 1/3 patients with hyperplasia. Image fusion improved the anatomical assignment of the 13 scintigraphic foci in five patients and was helpful in the interpretation of inconclusive MR-findings in two patients. Conclusions: Both MRI and 99mTc-MIBI scintigraphy sensitively detect parathyroid adenomas but are less reliable in case of hyperplastic glands. In case of a scintigraphic focus, image fusion considerably improves its topographic assignment. Furthermore, it facilitates the evaluation of inconclusive MRI findings.

MRI FINDINGS INFORM NEURODEVELOPMENTAL OUTCOMES IN INDIVIDUALS WITH AGENESIS OF THE CORPUS CALLOSUM (ACC): ANALYSIS OF A PROSPECTIVE COHORT

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
A Phillips ◽  
S Gobuty ◽  
S Hetts ◽  
M Shevell ◽  
A Majnemer ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
Prospective Cohort ◽  
Mri Findings ◽  
Neurodevelopmental Outcomes

INITIAL CLINICAL AND MRI FINDINGS IN SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE)

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
N Jovic ◽  
V Milic-Rasic ◽  
D Puric
Keyword(s):  
Subacute Sclerosing Panencephalitis ◽  
Mri Findings
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