Friday, October 26, 2012

Multiple System Atrophy causes of death

Cardiopulmonary arrest7 (33.3)
 Urinary tract infection5 (23.8)
 Aspiration pneumonia2 (9.5)
 Infectious pneumonia2 (9.5)
 Acute aspiration1 (4.8)
 Wasting syndrome3 (14.3)

"Occurrence of sudden death is a common cause of mortality in MSA and may happen in the early stages while disability remains acceptable."
Citation to: Shimohata, T., Ozawa, T., Nakayama, H., Tomita, M., Shinoda, H. and Nishizawa, M. (2008) Frequency of nocturnal sudden death in patients with multiple system atrophy. J Neurol 255: 1483-1485.
Sudden death has been reported in patients with multiple system atrophy (MSA), although the frequency of this event has not been well delineated. We investigated the frequency and potential causes of sudden death in patients with MSA. During the 5-year observation period, 10 of 45 patients with probable MSA died. The causes of death included sudden death of unknown etiology (seven patients), aspiration pneumonia (one patient), asphyxia after vomiting (one patient), and lung cancer (one patient). The mean survival time of patients with sudden death was 63.0 ± 24.7 months (range, 39–116 months). Among seven patients who experienced sudden death, six were found to have died during sleep. Among these patients, two had been treated with tracheostomy and three with continuous positive airway pressure (CPAP) or noninvasive positive pressure ventilation (NPPV) during sleep, suggesting that these treatments do not always prevent sudden death in patients with MSA. Nocturnal sudden death should be recognized as the most common mechanism of death in patients with MSA.
The close relationship between life-threatening breathing disorders and urine storage dysfunction in multiple system atrophy. K Deguchi, K Ikeda, R Goto, M Tsukaguchi, Y Urai… - Journal of Neurology, 2010 - Springer
Survival of multiple system atrophy (MSA) depends on whether a variety of sleep-related breathing problems as well as autonomic failure (AF) occur. Since the brainstem lesions that cause respiratory and autonomic dysfunction overlap with each other, these critical manifestations might get worse in parallel. If so, the detection of AF, which is comparatively easy, might be predictive of a latent life-threatening breathing disorder. In 15 patients with MSA, we performed autonomic function tests composed of postural challenges and administered a questionnaire on bladder condition, as well as polysomnography and laryngoscopy during wakefulness and under anesthesia. Polysomnographic variables such as the apnea-hypopnea index (AHI) and oxygen saturation (SpO2) and the findings of laryngoscopy were compared with the degree of cardiac and urinary autonomic dysfunction. AHI, mean SpO2 and the lowest SpO2 showed significant correlations with urine storage dysfunction. In addition, patients with vocal cord abductor paralysis (VCAP) or central sleep apnea (CSA) contributing to nocturnal sudden death had more severe storage disorders than those without. On the other hand, no significant relationship between polysomnographic variables and orthostatic hypotension was observed except in the case of mean SpO2. These results indicate that life-threatening breathing disorders have a close relationship with AF, and especially urine storage dysfunction. Therefore, longitudinal assessment of deterioration of the storage function might be useful for predicting the latent progress of VCAP and CSA.

1 comment:

Mike Loshe said...

Thanks for the share. I am curious if there is anyway to detect such a disease. I know for many autonomic disorders physicians use an ANS test to detect various abnormalities, maybe it could be used here as well.