Autonomic predominant multiple system atrophy in the context of Parkinsonian and cerebellar variants

Autonomic dysfunction is often a late feature of multiple system atrophy (MSA). Based on early features, MSAp (Parkinsonian) and MSAc (cerebellar) variants are known. We studied the severity of autonomic dysfunction at first examination in MSA patient that may have relevance in its early diagnosis.

Clinical (including autonomic), radiological and lab features of 53 MSA patients were analyzed in the context of MSAp (16 patients) and MSAc (37 patients).

Most frequent autonomic symptoms were erectile dysfunction in 30 out of 38 males (78.9%), bladder symptoms in 35 (66%), followed by blackouts/fainting attacks in 33 (62.3%). Autonomic abnormalities were detected in hand grip test (86.8%), deep breathing test (79.2%), cold pressor test (71.7%), Valsalva maneuver (75.5%), heart rate variability at 30:15 beats after standing (50.9%) and systolic BP on standing (41.5%). Six (16.2%) MSAp and 1 (6.3%) MSAc patients continued to have autonomic symptoms for several months (38.7±26.11, median 36 months) before the appearance of cerebellar (1 patient) or Parkinsonian (6 patients) features.

Autonomic dysfunction may be the only presenting feature in some patients with MSA. Routine autonomic testing may help in the early diagnosis of MSA in patients presenting with Parkinsonian or cerebellar features.