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Warburg micro syndrome type 1 associated with peripheral neuropathy and cardiomyopathy.
- Source :
-
Folia neuropathologica [Folia Neuropathol] 2016; Vol. 54 (3), pp. 273-281. - Publication Year :
- 2016
-
Abstract
- The Warburg micro syndrome (WARBM) is a genetically heterogeneous syndrome linked to at least 4 loci. At the clinical level, WARBM is characterized by microcephaly, microphthalmia, microcornea, congenital cataracts, corpus callosum hypoplasia, severe mental retardation, and hypogonadism. In some families additional clinical features have been reported. The presence of uncommon clinical features (peripheral neuropathy, cardiomyopathy) may result in misdirected molecular diagnostics. Using the next generation sequencing approach (NGS), we were able to diagnose WARBM1 syndrome by detection of a new mutation within the RAB3GAP1 gene. We have detected some DNA variants which may be responsible for cardiomyopathy. We did not find any obvious pathogenic mutation within a set of genes known to be responsible for hereditary motor and sensory neuropathy (HMSN). We conclude that: (i) in clinically delineated syndromes, a classical single-gene oriented approach may be not conclusive especially in the presence of rare clinical features, (ii) peripheral neuropathy and cardiomyopathy are rare additional symptoms coexisting with WARBM1, (iii) a pleiotropic effect of a single point mutation is sufficient to be causative for WARBM1 and (iv) more WARBM-affected patients should be reported to delineate a complete phenotype.
- Subjects :
- Abnormalities, Multiple diagnosis
Agenesis of Corpus Callosum diagnosis
Agenesis of Corpus Callosum genetics
Cardiomyopathies diagnosis
Cardiomyopathies genetics
Cataract complications
Cataract diagnosis
Cataract genetics
Child, Preschool
Female
High-Throughput Nucleotide Sequencing methods
Humans
Hypogonadism complications
Hypogonadism diagnosis
Infant
Intellectual Disability complications
Intellectual Disability diagnosis
Microcephaly complications
Microcephaly diagnosis
Optic Atrophy complications
Optic Atrophy diagnosis
Peripheral Nervous System Diseases diagnosis
Peripheral Nervous System Diseases etiology
Phenotype
Abnormalities, Multiple genetics
Cataract congenital
Cornea abnormalities
Hypogonadism genetics
Intellectual Disability genetics
Microcephaly genetics
Mutation genetics
Optic Atrophy genetics
Peripheral Nervous System Diseases genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1509-572X
- Volume :
- 54
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Folia neuropathologica
- Publication Type :
- Academic Journal
- Accession number :
- 27764520
- Full Text :
- https://doi.org/10.5114/fn.2016.62537