Isaac Disease: Does the Clinical Spectrum Include White Matter Disease in the Brain? (P05.160)

Neurology ◽  
2012 ◽  
Vol 78 (Meeting Abstracts 1) ◽  
pp. P05.160-P05.160
Author(s):  
M. Sedarous ◽  
D. Lange
Keyword(s):  
White Matter ◽  
Clinical Spectrum ◽  
White Matter Disease ◽  
The Brain

A Prospective Analysis of Risk Factors for White Matter Disease in the Brain Stem

2003 ◽  
Vol 22 (5) ◽  
pp. 275-282 ◽  
Author(s):  
Jingzhong Ding ◽  
F. Javier Nieto ◽  
Norman J. Beauchamp ◽  
W.T. Longstreth Jr. ◽  
Teri A. Manolio ◽  
...  
Keyword(s):  
Risk Factors ◽  
White Matter ◽  
Brain Stem ◽  
Prospective Analysis ◽  
White Matter Disease ◽  
The Brain

scholarly journals The brain in myotonic dystrophy 1 and 2: evidence for a predominant white matter disease

Brain ◽  
2011 ◽  
Vol 134 (12) ◽  
pp. 3530-3546 ◽  
Author(s):  
Martina Minnerop ◽  
Bernd Weber ◽  
Jan-Christoph Schoene-Bake ◽  
Sandra Roeske ◽  
Sandra Mirbach ◽  
...  
Keyword(s):  
White Matter ◽  
Myotonic Dystrophy ◽  
White Matter Disease ◽  
The Brain

scholarly journals Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) – Still to be Considered in the Presence of Vascular Risk Factors

2020 ◽  
Vol 12 (Suppl. 1) ◽  
pp. 196-201
Author(s):  
Dinesh Naidu Ganesan ◽  
Thibault Coste ◽  
Narayanaswamy Venketasubramanian
Keyword(s):  
Risk Factors ◽  
White Matter ◽  
Autosomal Dominant ◽  
Vascular Risk Factors ◽  
Smoking History ◽  
White Matter Disease ◽  
Vascular Risk ◽  
Limb Weakness ◽  
History Of ◽  
The Brain

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL) is a rare hereditary vasculopathy that primarily affects the brain, caused mostly by missense mutations of the <i>NOTCH3</i> gene which is located on chromosome 19. Clinically, it manifests as transient ischemic attacks and strokes in individuals under the age of 60 years without vascular risk factors. We report a 46-year-old male with a 9 and 3-month history of progressive unilateral lower limb weakness and dysarthria, respectively. He had a history of diabetes mellitus but no hypertension, hyperlipidemia, or smoking history. Both parents had a stroke at the age of 65 years. Neurological examination was significant for moderate dysarthria and reduced right upper limb dexterity. Magnetic resonance imaging (MRI) of the brain revealed extensive white matter disease, lacunar infarcts, and a few microhemorrhages. Electron microscopy of his skin biopsy showed electron-dense deposits of extracellular osmiophilic granular material adjacent to smooth muscle cells. <i>NOTCH3</i> gene analysis revealed a heterozygous typical mutation in exon 6. He was commenced on aspirin and atorvastatin. Over time, he became more dysarthric and demented. MRI revealed the progression of the white matter disease and a new right subcortical infarct. His aspirin was switched to clopidogrel, and donepezil was added. CADASIL should be considered among younger stroke patients with vascular risk factors, especially in the presence of widespread white matter disease. Genetic counselling may be needed after the diagnosis is made.

White Matter Disease Severity of the Brain and Its Association With Geriatric Syndromes

2013 ◽  
Vol 125 (6) ◽  
pp. 17-23 ◽  
Author(s):  
Kannayiram Alagiakrishnan ◽  
Jenny Hsueh ◽  
Edwin Zhang ◽  
Khurshid Khan ◽  
Ambikaipakan Senthilselvan
Keyword(s):  
White Matter ◽  
Disease Severity ◽  
White Matter Disease ◽  
Geriatric Syndromes ◽  
The Brain

Fast FLAIR of the brain: the range of appearances in normal subjects and its application to quantification of white-matter disease

1997 ◽  
Vol 39 (4) ◽  
pp. 243-249 ◽  
Author(s):  
M. L. Gawne-Cain ◽  
N. C. Silver ◽  
I. F. Moseley ◽  
D. H. Miller
Keyword(s):  
White Matter ◽  
Normal Subjects ◽  
White Matter Disease ◽  
The Brain

scholarly journals Small Vessel Disease/White Matter Disease of the Brain and Its Association With Osteoporosis

2015 ◽  
Vol 7 (5) ◽  
pp. 297-302 ◽  
Author(s):  
Kannayiram Alagiakrishnan ◽  
Jenny Hsueh ◽  
Edwin Zhang ◽  
Khurshid Khan ◽  
Ambikaipakan Senthilselvan
Keyword(s):  
White Matter ◽  
Small Vessel Disease ◽  
Small Vessel ◽  
White Matter Disease ◽  
Vessel Disease ◽  
The Brain
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