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Term:
MICROANGIOPATHY AND LEUKOENCEPHALOPATHY, PONTINE, AUTOSOMAL DOMINANT (DOID:9002417)
Annotations: Rat: (1) Mouse: (1) Human: (1) Chinchilla: (1) Bonobo: (1) Dog: (1) Squirrel: (1) Pig: (1)
Parent Terms Term With Siblings Child Terms
vascular disease +     
adult-onset leukoencephalopathy with axonal spheroids and pigmented glia  
Adult-Onset Muscular Dystrophy with Leukoencephalopathy 
Aneurysm +   
angiodysplasia +   
angioedema +   
Angiomatosis +   
angiomyoma +  
anterior ischemic optic neuropathy +   
Arterial Dissection with Lentiginosis 
Arterial Injury +   
Arterial Occlusive Diseases +   
arteriovenous malformation +   
artery disease +   
capillary disease +   
Carotid Intimal Medial Thickness 2 
Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy  
Cerebroretinal Microangiopathy with Calcifications and Cysts +   
cerebrovascular disease +   
cholesterol embolism +   
COL4A1-related familial vascular leukoencephalopathy  
combined oxidative phosphorylation deficiency 12  
compartment syndrome +   
Cystic Leukoencephalopathy without Megalencephaly  
Demyelinating Autoimmune Diseases, CNS +   
diabetic angiopathy +   
Embolism and Thrombosis +   
Episodic Mitochondrial Myopathy with or without Optic Atrophy and Reversible Leukoencephalopathy  
Hand-Arm Vibration Syndrome 
hemangioma +   
hepatic vascular disease +   
hereditary arterial and articular multiple calcification syndrome +   
Hereditary Central Nervous System Demyelinating Diseases +   
Hyperemia  
hypertension +   
Hypomyelination with Brainstem and Spinal Cord Involvement and Leg Spasticity  
Hypotension +   
intravenous leiomyomatosis 
ischemia +   
ischemic colitis  
Labrune Syndrome  
Leukoencephalopathy with Ataxia  
Leukoencephalopathy with Brainstem and Spinal Cord Involvement and Lactate Elevation  
Leukoencephalopathy with Dystonia and Motor Neuropathy  
leukoencephalopathy with vanishing white matter  
LEUKOENCEPHALOPATHY, ACUTE REVERSIBLE, WITH INCREASED URINARY ALPHA-KETOGLUTARATE  
Leukoencephalopathy, Arthritis, Colitis, and Hypogammaglobulinemia 
male genital organ vascular disease 
Mesenteric Ischemia  
MICROANGIOPATHY AND LEUKOENCEPHALOPATHY, PONTINE, AUTOSOMAL DOMINANT  
A form of cerebral small vessel disease (cSVD) resulting in the onset of recurrent ischemic strokes in the thirties or forties. Affected individuals develop progressive, cognitive and motor impairment, consistent with progressive multi-infarct dementia. Brain imaging shows lacunar infarcts, often with a pontine predilection, as well as diffuse leukoencephalopathy affecting various brain regions.
Myocardial Ischemia +   
Pancytopenia and Occlusive Vascular Disease 
peripheral vascular disease +   
Posterior Leukoencephalopathy Syndrome  
Prehypertension  
Progressive Encephalopathy, Early-Onset, with Brain Edema and/or Leukoencephalopathy +   
Progressive Leukoencephalopathy, with Ovarian Failure  
progressive multifocal leukoencephalopathy 
Pulmonary Vein Stenosis 
pulmonary venoocclusive disease +   
Reperfusion Injury +   
retinal vascular disease +   
retinal vasculopathy with cerebral leukodystrophy  
Ribose 5-Phosphate Isomerase Deficiency  
scimitar syndrome +   
spastic ataxia 3  
Spinal Cord Vascular Diseases +   
splenic infarction 
Spontaneous Coronary Artery Dissection 
STING-associated vasculopathy with onset in infancy  
Superior Vena Cava Syndrome  
supine hypotensive syndrome 
telangiectasis +   
Telencephalic Leukoencephalopathy 
thoracic outlet syndrome +  
thrombosis +   
varicocele  
varicose veins +   
vascular dementia +   
Vascular Fistula +   
vascular hemostatic disease +   
Vascular Hyalinosis 
Vascular Neoplasms 
Vascular System Injuries  
Vascular Tissue Neoplasms +   
vasculitis +   
Vasoplegia 
vein disease +   
Veno-Occlusive Disease +   
venous insufficiency +   
X-linked Spondyloepimetaphyseal Dysplasia with Hypomyelinating Leukodystrophy   

Synonyms
Exact Synonyms: Autosomal dominant pontine microangiopathy and leukoencephalopathy ;   DEMENTIA, HEREDITARY MULTI-INFARCT, SWEDISH TYPE ;   PADMAL
Primary IDs: OMIM:618564

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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.