Literature DB >> 10948580

[Carlevoix-Saguenay type recessive spastic ataxia. A report of a Spanish case].

I Pascual-Castroviejo1, S I Pascual-Pascual, J Viaño, V Martínez.   

Abstract

OBJECTIVE: To present the first Spanish case, perhaps the first non-Canadian, with ataxia type Charlevoix-Saguenay. CLINICAL CASE: A patient with important psychomotor delay and non-progressive ataxia has been studied in our Service during the first years of life and his follow-up has been carried out until 30 years of age. He has been studied from the clinical, ophthalmological and neuroradiological with magnetic resonance point of views.
RESULTS: Ataxia and psychomotor delay showed a non-progressive evolution. The patient showed myelinated retinal fibers and atrophy of the superior half of the cerebellum.
CONCLUSION: Neurological, ophthalmological and magnetic resonance images showing atrophy of the superior part of the cerebellum are the three main signs of the ataxia type Charlevoix-Saguenay. This patient could be the first case reported out Canada.

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Mesh:

Year:  2000        PMID: 10948580

Source DB:  PubMed          Journal:  Rev Neurol        ISSN: 0210-0010            Impact factor:   0.870


  3 in total

1.  Private SACS mutations in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) families from Turkey.

Authors:  Andrea M Richter; Riza Koksal Ozgul; Virginie C Poisson; Haluk Topaloglu
Journal:  Neurogenetics       Date:  2004-05-20       Impact factor: 2.660

Review 2.  Autosomal recessive cerebellar ataxias.

Authors:  Francesc Palau; Carmen Espinós
Journal:  Orphanet J Rare Dis       Date:  2006-11-17       Impact factor: 4.123

Review 3.  Genetics of Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) and Role of Sacsin in Neurodegeneration.

Authors:  Jaya Bagaria; Eva Bagyinszky; Seong Soo A An
Journal:  Int J Mol Sci       Date:  2022-01-04       Impact factor: 5.923

  3 in total

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