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Amyotonia congenita

MedGen UID:
8042
•Concept ID:
C0002735
•
Congenital Abnormality; Disease or Syndrome
Synonyms: Benign congenital hypotonia; Oppenheim Disease; Oppenheim's disease
SNOMED CT: Amyotonia congenita (63135006); Oppenheim's disease (63135006); Congenital atonic pseudoparalysis (63135006); Oppenheim's amyotonia (63135006)
 
Monarch Initiative: MONDO:0056822
OMIM®: 205000

Definition

congenital atonic pseudoparalysis observed especially in infants and characterized by absence of muscular tone only in muscles innervated by the spinal nerves; also known as Oppenheim''s disease or syndrome or congenital atonic pseudoparalysis. [from CRISP]

Clinical features

From HPO
Muscular atrophy
MedGen UID:
892680
•Concept ID:
C0541794
•
Pathologic Function
The presence of skeletal muscular atrophy (which is also known as amyotrophy).
See: Feature record | Search on this feature

Professional guidelines

PubMed

MANAGEMENT OF MYASTHENIA GRAVIS IN THE OBSTETRICAL PATIENT.
MCNALL PG, JAFARNIA MR
Am J Obstet Gynecol 1965 Jun 15;92:518-25. doi: 10.1016/s0002-9378(16)34844-x. PMID: 14295887
ELECTROMYOGRAM IN THE DIFFERENTIAL DIAGNOSIS OF THE "FLOPPY" INFANT.
MARINACCI AA
Bull Los Angel Neuro Soc 1964 Jun;29:70-5. PMID: 14169954
Differential diagnosis of infantile hypotonia. The use of the electromyograph and the developmental and neurologic examination as aids.
JEBSEN RH, JOHNSON EW, KNOBLOCH H, GRANT DK
Am J Dis Child 1961 Jan;101:8-17. PMID: 13789718

Recent clinical studies

Etiology

The future of the 'floppy infant': a follow-up study of 133 patients.
PAINE RS
Dev Med Child Neurol 1963 Apr;5:115-24. doi: 10.1111/j.1469-8749.1963.tb05010.x. PMID: 13941123

Diagnosis

Congenital myopathies with "diagnostic" pathological features.
Korényi-Both A, Korényi-Both I
J Med 1987;18(2):93-107. PMID: 3323392
THE FLOPPY INFANT.
SHY GM
Proc Inst Med Chic 1965 May;25:261-2. PMID: 14323288
CONGENITAL HYPOTONIA.
HEWSON JR
McGill Med J 1964 Apr;33:38-47. PMID: 14153581
SKELETAL MUSCLE DISEASES: RECENT ADVANCES AND SOME RELATED BASIC PROBLEMS.
BAJUSZ E, JASMIN G
Can Med Assoc J 1963 Sep 14;89(11):555-62. PMID: 14045348Free PMC Article
The limp child.
WALTON JN
J Neurol Neurosurg Psychiatry 1957 May;20(2):144-54. doi: 10.1136/jnnp.20.2.144. PMID: 13429386Free PMC Article

Prognosis

The future of the 'floppy infant': a follow-up study of 133 patients.
PAINE RS
Dev Med Child Neurol 1963 Apr;5:115-24. doi: 10.1111/j.1469-8749.1963.tb05010.x. PMID: 13941123

Clinical prediction guides

SKELETAL MUSCLE DISEASES: RECENT ADVANCES AND SOME RELATED BASIC PROBLEMS.
BAJUSZ E, JASMIN G
Can Med Assoc J 1963 Sep 14;89(11):555-62. PMID: 14045348Free PMC Article

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