Literature DB >> 29062563

Segmental Spinal Muscular Atrophy Localised to the Lower Limbs: First case from Oman.

Roshan Koul1, Amna Al-Futaisi1, Khalid Al-Thihli2, Zandre Bruwer2, Patrick Scott2.   

Abstract

Spinal muscular atrophy (SMA) is a genetic lower motor neuron disease. It usually involves all of the skeletal muscles innervated by the anterior horn cells of the spinal cord. In rare cases, there is also localised involvement of the spinal cord. We report a 10-year-old boy who presented to the Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with muscle weakness restricted to the lower limbs. The presence of a homozygous deletion within the survival of motor neuron 1 gene confirmed the diagnosis of SMA. To the best of the authors' knowledge, this is the first report of an Omani patient with segmental SMA involving only the lower limbs. Treatment for this rare and relatively benign form of SMA is symptomatic and includes physiotherapy.

Entities:  

Keywords:  Case Report; Muscle Weakness; Oman; Pes Cavus; Segmental; Spinal Muscular Atrophies of Childhood; Spinal Muscular Atrophy

Mesh:

Substances:

Year:  2017        PMID: 29062563      PMCID: PMC5642370          DOI: 10.18295/squmj.2017.17.03.018

Source DB:  PubMed          Journal:  Sultan Qaboos Univ Med J        ISSN: 2075-051X


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1.  Generalized Hypotonia Revealing Spinal Muscular Atrophy Type 2: The First Case Reported From the Dominican Republic and a Review of the Literature.

Authors:  Rubén Blanco; Jessie Pichardo; Hassan Abdullah
Journal:  Cureus       Date:  2020-11-12
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