Pseudoprogeria syndrome

Orpha code: 2985OMIM code: 200130

Definition

A rare syndromic intellectual deficiency characterized by psychomotor delay, severe progressive spastic quadriplegia, microcephaly, and a Hallerman-Streiff-like phenotype including absence of eyebrows and eyelashes, glaucoma, and small, beaked nose. Structural central nervous system abnormalities (cervical spinal cyst, occipital cranium bifidum occulatum) were additional findings. There have been no further descriptions in the literature since 1974.

Disease data
Classification

Malformation syndrome

Synonyms
Absent eyebrows and eyelashes-intellectual disability syndrome
Brak brwi i rzęs - niepełnosprawność intelektualna
Zespół Hala, Berga i Rudolpha
Hal-Berg-Rudolph syndrome
ORPHA code
2985
OMIM code
200130
ICD10 code
Q87.8
ICD11 code
-

No additional description.

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