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Joubert syndrome: A classic case
Joubert syndrome is a rare autosomal recessive disorder characterized by hyperpnoea and eye movements, hypotonia, ataxia, developmental retardation with neuropathologic abnormalities of cerebellum and brainstem including inherited hypoplasia or aplasia of vermis. Cerebellar vermin anomalies are desc...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Medknow Publications & Media Pvt Ltd
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6396587/ https://www.ncbi.nlm.nih.gov/pubmed/30911530 http://dx.doi.org/10.4103/jfmpc.jfmpc_165_18 |
Sumario: | Joubert syndrome is a rare autosomal recessive disorder characterized by hyperpnoea and eye movements, hypotonia, ataxia, developmental retardation with neuropathologic abnormalities of cerebellum and brainstem including inherited hypoplasia or aplasia of vermis. Cerebellar vermin anomalies are described in other disorders such as Dandy-Walker and rhombencephalon synapsis. These disorders should be distinguished from Joubert syndrome on the basis of imaging. Comparison with typical imaging and clinical findings may be helpful for appropriate diagnosis. |
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