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Infantile axial hypotonia

MedGen UID:
812934
Concept ID:
C3806604
Finding
Synonym: Hypotonia, axial, in infancy
 
HPO: HP:0009062

Definition

Muscular hypotonia (abnormally low muscle tone) affecting the musculature of the trunk and with onset in infancy. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Infantile axial hypotonia

Conditions with this feature

Intellectual disability, autosomal dominant 42
MedGen UID:
934741
Concept ID:
C4310774
Mental or Behavioral Dysfunction
GNB1 encephalopathy (GNB1-E) is characterized by moderate-to-severe developmental delay / intellectual disability, structural brain abnormalities, and often infantile hypotonia and seizures. Other less common findings include dystonia, reduced vision, behavior issues, growth delay, gastrointestinal (GI) problems, genitourinary (GU) abnormalities in males, and cutaneous mastocytosis.
Intellectual disability, autosomal dominant 53
MedGen UID:
1623344
Concept ID:
C4540481
Mental or Behavioral Dysfunction
Myopathy, congenital, progressive, with scoliosis
MedGen UID:
1684769
Concept ID:
C5231417
Disease or Syndrome
Congenital myopathy-19 (CMYO19) is an autosomal recessive skeletal muscle disorder characterized by infantile-onset of progressive muscle weakness and atrophy associated with scoliosis, variably impaired walking, and dysmorphic facial features (Feichtinger et al., 2019). For a discussion of genetic heterogeneity of congenital myopathy, see CMYO1A (117000).

Recent clinical studies

Etiology

Prasad M, Glueck M, Ceballos-Saenz D, Zapata-Aldana E, Johnson N, Campbell C, Nguyen CE
J Neuromuscul Dis 2019;6(3):341-347. doi: 10.3233/JND-180345. PMID: 31227653

Clinical prediction guides

Prasad M, Glueck M, Ceballos-Saenz D, Zapata-Aldana E, Johnson N, Campbell C, Nguyen CE
J Neuromuscul Dis 2019;6(3):341-347. doi: 10.3233/JND-180345. PMID: 31227653

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