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Term:
Progressive Leukoencephalopathy, with Ovarian Failure (DOID:9006658)
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Parent Terms Term With Siblings Child Terms
ovarian disease +     
Acromesomelic Dysplasia, Demirhan Type  
Acute Reversible Leukoencephalopathy with Increased Urinary Alpha-ketoglutarate  
adult-onset leukoencephalopathy with axonal spheroids and pigmented glia  
Adult-Onset Muscular Dystrophy with Leukoencephalopathy 
anovulation  
Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy  
Cerebroretinal Microangiopathy with Calcifications and Cysts +   
COL4A1-related familial vascular leukoencephalopathy  
combined oxidative phosphorylation deficiency 12  
Cystic Leukoencephalopathy without Megalencephaly  
Demyelinating Autoimmune Diseases, CNS +   
Early-Onset Progressive Encephalopathy with Brain Edema and/or Leukoencephalopathy +   
endometriosis of ovary 
Episodic Mitochondrial Myopathy with or without Optic Atrophy and Reversible Leukoencephalopathy  
Hereditary Central Nervous System Demyelinating Diseases +   
Hypomyelination with Brainstem and Spinal Cord Involvement and Leg Spasticity  
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, Arthritis, Colitis, and Hypogammaglobulinemia 
LEUKOENCEPHALOPATHY, DEVELOPMENTAL DELAY, AND EPISODIC NEUROLOGIC REGRESSION SYNDROME  
Leukoencephalopathy, Motor Delay, Spasticity, and Dysarthria Syndrome  
oophoritis +  
ovarian cyst +   
ovarian dysfunction +   
ovarian hyperstimulation syndrome  
Ovarian Neoplasms +   
ovarian stromal hyperthecosis 
Ovary Reperfusion Injury  
Pontine Microangiopathy and Leukoencephalopathy, Autosomal Dominant  
Posterior Leukoencephalopathy Syndrome  
premature menopause +   
PRIMARY OVARIAN FAILURE +   
Progressive Leukoencephalopathy, with Ovarian Failure  
An autosomal recessive neurodegenerative disorder characterized by loss of motor and cognitive skills, usually with onset in young adulthood. Neurologic decline is severe, usually resulting in gait difficulties, ataxia, spasticity, and cognitive decline and dementia. Brain MRI shows progressive white matter signal abnormalities in the deep white matter. Affected females develop premature ovarian failure. (OMIM)
progressive multifocal leukoencephalopathy 
Ribose 5-Phosphate Isomerase Deficiency  
spastic ataxia 3  
Telencephalic Leukoencephalopathy 
tuberculous oophoritis 
vascular dementia +   
X-linked Spondyloepimetaphyseal Dysplasia with Hypomyelinating Leukodystrophy   

Synonyms
Exact Synonyms: LKENP
Primary IDs: OMIM:615889
Alternate IDs: RDO:9001632

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