Introduction
Although involvement of the autonomic nervous system in the skin and gastrointestinal tract has been described in Sandhoff disease, no reports of autonomic changes of the cardiovascular system, such as dysautonomia, have been documented in the literature reviewed. On the other hand, the neurally mediated cardioinhibitory syncope that is refractory to medical management can be treated with pacemakers with closed loop sensors.
Closed-loop pacemakers in dysautonomia secondary to Sandhoff disease
 
  Keywords
 
 
  
   Pacemakers
  
 
 
  
   Syncope
  
 
 
  
   Autonomic nervous system
  
 
Introduction
Although involvement of the autonomic nervous system in the skin and gastrointestinal tract has been described in Sandhoff disease, no reports of autonomic changes of the cardiovascular system, such as <dis>dysautonomia</dis>, have been documented in the literature reviewed. 
On the other hand, the neurally mediated cardioinhibitory syncope that is refractory to medical management can be treated with pacemakers with closed loop sensors.
Materials and methods
The case is described of a 42 year-old patient who was diagnosed with Sandhoff disease. 
Associated to this condition, he presented with <dis>dysautonomia</dis> with daily episodes of syncope, with no response to medical treatment, which warranted the implant of a pacemaker with a closed loop sensor. 
The existing literature on this topic was subsequently reviewed.
Conclusions
Up until now, there have been no reports on the relationship between Sandhoff disease and <dis>dysautonomia</dis>. 
In the case of this patient, the deterioration in the quality of life was significant. 
The use of the closed loop stimulation (CLS) algorithm in neurally mediated cardioinhibitory syncope has been shown to reduce the number of syncopes in this type of patient and improve their quality of life.
Introduction
Although involvement of the autonomic nervous system in the skin and gastrointestinal tract has been described in Sandhoff disease, no reports of autonomic changes of the cardiovascular system, such as dysautonomia, have been documented in the literature reviewed. 
On the other hand, the neurally mediated cardioinhibitory syncope that is refractory to medical management can be treated with pacemakers with closed loop sensors.
Materials and methods
The case is described of a 42 year-old patient who was diagnosed with Sandhoff disease. 
Associated to this condition, he presented with dysautonomia with daily episodes of syncope, with no response to medical treatment, which warranted the implant of a pacemaker with a closed loop sensor. 
The existing literature on this topic was subsequently reviewed.
Conclusions
Up until now, there have been no reports on the relationship between Sandhoff disease and dysautonomia. 
In the case of this patient, the deterioration in the quality of life was significant. 
The use of the closed loop stimulation (CLS) algorithm in neurally mediated cardioinhibitory syncope has been shown to reduce the number of syncopes in this type of patient and improve their quality of life.
Introduction
Although involvement of the autonomic nervous system in the skin and gastrointestinal tract has been described in Sandhoff disease, no reports of autonomic changes of the cardiovascular system, such as <dis>dysautonomia</dis>, have been documented in the literature reviewed. On the other hand, the neurally mediated cardioinhibitory syncope that is refractory to medical management can be treated with pacemakers with closed loop sensors.
dysautonomia
DISABILITY