Kearns Sayre Syndrome (KSS) - A Rare Cause For Cardiac Pacing
نویسندگان
چکیده
A 22 year old male presented to our clinic with history of fatigue and breathlessness for four years. His clinical history had started during early childhood and was characterized by mental retardation. Mental retardation had prevented him from attending the school. Patient developed bilateral ptosis when he was thirteen years old. He was also giving history of double vision for the past 6 years. Unilateral hearing loss set in for the past 3 years. Patient started developing the symptoms of dysphagia and regurgitation for the past 2 years. The troubling symptoms which brought him to the hospital now were tiredness and easy fatigability with breathlessness.
منابع مشابه
Kearns Sayre Syndrome: Looking beyond A-V conduction
A 15-year-old boy was diagnosed with Kayne Sayre Syndrome. He presented with pigmentary retinopathy, progressive ophthalmoplegia and complete heart block. He received a transvenous dual chamber pacemaker. Two years later he died suddenly while at home. This case highlights the importance of recognizing mechanisms other than heart block as a cause of sudden death in a patient with KSS.
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Introduction Herein, we describe the interesting case of a 13-year-old female with Kearns-Sayre syndrome (KSS) who underwent a successful T3 ~ L3 posterior spinal instrumentation and fusion (PSIF). KSS is a rare mitochondrial myopathy with only 226 cases reported in published literature as of 1992. It is the result of deletions in mitochondrial DNA and manifests as a triad of: 1) chronic progre...
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Kearns-Sayre syndrome (KSS) was first described in 1958 as 'a rare neuromuscular disorder defined by a characteristic triad of progressive external ophthalmoplegia, pigmentary retinopathy, atrioventricular block and cerebellar ataxia'. The prevalence rate of KSS is ∼1-3 per 100 000 individuals. Here, we report a rare case of a 17-year-old Venezuelan male with KSS.
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Kearns-Sayre syndrome is a mitochondrial myopathy, which was first described by Tomas Kearn in 1958. Diagnostic symptoms of this condition include retinitis pigmentosa, chronic progressive external ophthalmoplegia, and one or more of the following factors: cardiac conduction system diseases, cerebellar ataxia, and cerebrospinal fluid (CSF) with protein content above 100 mg/dL. The nature of thi...
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