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Congenital neuronal ceroid lipofuscinosis

disorder
SNOMED 720830009CUI C1864669

Overview

Source: MedlinePlus.gov, National Library of Medicine. Not a substitute for medical advice.

Signs & Symptoms

Based on Human Phenotype Ontology (HPO) disease-phenotype annotations.

Apnea
Always present (100%)HP:0002104
Ataxia
Always present (100%)HP:0001251
Atrophic cerebellum
Always present (100%)HP:0001272
Cerebral atrophy
Always present (100%)HP:0002059
Intellectual deterioration
Always present (100%)HP:0001268
Mental retardation, severe
Always present (100%)HP:0010864
Retinal atrophy
Always present (100%)HP:0001105
Retinitis pigmentosa
Always present (100%)HP:0000510
Decreased size of cranium
Frequent (30-79%)HP:0000252
Epilepsy
Frequent (30-79%)HP:0001250
Involuntary muscle stiffness, contraction, or spasm
Frequent (30-79%)HP:0001257
Low-set ears
Frequent (30-79%)HP:0000369
Peripheral sensory axonal neuropathy
Frequent (30-79%)HP:0003390
Vascular granular osmiophilic material deposition
Frequent (30-79%)HP:0003657
Increased width of bridge of nose
Occasional (5-29%)HP:0000431
Myoclonic seizure
Occasional (5-29%)HP:0032794
Respiratory failure
Occasional (5-29%)HP:0002878
Sloping forehead
Occasional (5-29%)HP:0000340
Split hand
Occasional (5-29%)HP:0001171
Early closure of the cranial sutures
HP:0005458
Mental-retardation
HP:0001249
Muscle rigidity
HP:0002063
Neuronal lipopigments
HP:0002074
Neuronal loss in CNS
HP:0002529
Prolonged seizure
HP:0002133
Respiratory function loss
HP:0002093
Visual loss
HP:0000572

Quick Facts

SNOMED CT
720830009
UMLS CUI
C1864669
Fully Specified Name
Neuronal ceroid lipofuscinosis due to deficiency of cathepsin D (disorder)
Specialists
0
Diagnostic Biomarkers
0
HPO Phenotypes
27
Medical Disclaimer

This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always consult with a qualified healthcare provider regarding any medical condition or treatment plan.

Clinical content is derived from the SNOMED CT clinical ontology and curated medical knowledge graphs.