Tag Archives: age of death myotonic dystrophy

Why do Myotonic Dystrophy Patients Die?

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Myotonic Dystrophy patients have a shorter lifespan according to this study from 2016. A comprehensive review shows an average age of death at about 60 years. Lung issues and Heart Issues are the major casues of death. Sudden cardiac failure occurs in 27% of patients.

 

April 05, 2016; 86 (16 Supplement) APRIL 20, 2016

Causes and Predictors of Mortality in a Large U.S. Myotonic Dystrophy Type 1 Adult Cohort (P5.077)

Julian Duda, Yedatore Venkatesh, William Groh
First published April 8, 2015,
 
 

Abstract

Background: There is limited data on the causes and predictors of mortality in patients (pts) with myotonic dystrophy type 1 (DM1) evaluated and treated with modern medical therapy in the U.S. Objective: To determine the epidemiology of mortality in U.S. patients with DM1. Methods: Analysis from a U.S. registry with clinically- and genetically-verified adult DM1 pts (age at entry≥18 yrs) enrolled at MDA clinics (1997-2005) and prospectively followed (study entry-N=406; age: 42±12 yrs; male: 205 (50.5[percnt]); CTG repeats: 629±386; muscular impairment rating score (MIRS): 3.2±1.0). Causes of death were adjudicated by death certificate and medical records review. Results: By last follow-up (11.2±4.2 yrs), 170 (41.9[percnt]) of pts had died with a median age at death of 55.4 yrs. Causes of death in the 170 pts were respiratory failure (90, 52.9[percnt]), sudden unexpected possibly cardiac (47, 27.6[percnt]), non-sudden cardiac (8, 4.7[percnt]), non-sudden other (21, 12.4[percnt]), and uncertain cause (4, 2.4[percnt]). The median survival age was 60.5 yrs. Study entry characteristics predicting all-cause mortality using survival analysis included age (per decile, RR 1.5; 95[percnt] CI 1.3-1.7, p<0.001), MIRS (per 1-level increase, RR 1.7; 95[percnt] CI 1.5-2.1, p<0.001), CTG repeat length (per1-log increase, RR 1.9; 95[percnt] CI 1.2-3.0, p=0.006), cardiac diagnoses (if present, RR 2.7; 95[percnt] CI 2.0-3.7, p<0.001), and an abnormal EKG (if present, RR 2.4; 95[percnt] CI 1.7-3.3, p<0.001). Conclusions: Despite modern therapy, adult DM1 pts in the U.S. have a shortened lifespan. The most common causes of death are respiratory failure followed by cardiac causes. Predictors of death include older age, worsened muscular disability, greater CTG repeat length, and presence of cardiac issues either a diagnoses or abnormal EKG. Study Supported by: Research grant with Biogen, Inc. and Isis pharmaceuticals

Disclosure: Dr. Duda has nothing to disclose. Dr. Venkatesh has nothing to disclose. Dr. Groh has received personal compensation for activities with Isis Pharmaceuticals.

Causes of Death Myotonic Dystrophy – Survival to age 65 lower

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This is an older study from 1998 but many folks ask about the longevity with this disease. How long will I live with myotonic dystrophy?

Age and causes of death in adult-onset myotonic dystrophy.

Brain

(1998) 121 (8): 1557-1563. doi: 10.1093/brain/121.8.1557

  1. C E de Die-Smulders,
  2. C J Höweler,
  3. C Thijs,
  4. J F Mirandolle,
  5. H B Anten,
  6. H J Smeets,
  7. K E Chandler and
  8. J P Geraedts

+ Author Affiliations


  1. Department of Clinical Genetics, Academic Hospital Maastricht, The Netherlands. christine.dedie@gen.unimaas.nl

Summary

Myotonic dystrophy is a relatively common type of muscular dystrophy, associated with a variety of systemic complications. Long term follow-up is difficult because of the slow progression. The objective of this study was to determine survival, age at death and causes of death in patients with the adult-onset type of myotonic dystrophy. A register of myotonic dystrophy patients was set up in Southern Limburg (the Netherlands), using data longitudinally collected over a 47-year period (1950-97). Survival for 180 patients (from the register) with adult-onset type myotonic dystrophy was established by the Kaplan-Meier method. The median survival was 60 years for males and 59 years for females. Survival of the patients was also estimated from the age of 15 years to the ages of 25, 45 and 65 years and compared with the expected survival of age- and sex-matched birth cohorts from the normal Dutch population. The observed survival to the ages of 25, 45 and 65 years was 99%, 88% and 18% compared with an expected survival of 99%, 95% and 78%, respectively. Thus, survival to the age of 65 in patients with adult-onset myotonic dystrophy is markedly reduced. A weak positive correlation between the CTG repeat length and younger age at death was found in the 13 patients studied (r = 0.50, P = 0.08). The cause of death could be determined in 70 of the 83 deceased patients. Pneumonia and cardiac arrhythmias were the most frequent primary causes of death, each occurring in approximately 30%, which was far more than expected for the general Dutch population. In addition, we assessed mobility in the years before death in a subgroup of 18 patients, as a reflection of the long-term physical handicap in myotonic dystrophy patients. Half of the patients studied were either partially or totally wheelchair-bound shortly before their death.