Multiple System Atrophy (MSA) is a neurodegenerative disorder and this synucleinopathy is characterized by abnormal accumulation of glial cytoplasmic inclusions (GCIs) consisting of aggregates of α-synuclein protein in the oligodendrocytes. MSA is characterized by autonomic failure and neurological signs, and patients present with urogenital symptoms, bowel dysfunction, orthostatic hypotension, parkinsonism and cerebellar ataxia. The condition may, however, be mistaken for Parkinson’s disease (PD), particularly in its early stages, and the distinction is critical as MSA is poorly responsive to antiparkinsonian treatment and has a more rapid disease progression compared to PD, being fatal within 6–10 years of the onset of symptoms. Patients with MSA often present early with significant LUT and sexual dysfunction, even prior to the onset of neurological complaints, and patients may present to a Urologist initially. Urinary incontinence is often severe and urinary retention presents early and generally progresses with advancing disease [
37••]. Neurons in the lower spinal cord such as the Onuf’s nucleus are particularly susceptible to degeneration in this condition [
38••] and therefore the anal sphincter EMG [
39,
40••] and bulbocavernosus reflex [
41••] are useful tests to detect damage, and therefore help to differentiate MSA from PD. The sensitivity and specificity of anal sphincter EMG in differentiating MSA from PD are 35 and 90%, respectively [
42] whereas, for BCR, these are 24 and 91–94%, respectively [
43•]. The value of sphincter EMG in the differential diagnosis of Parkinsonism has been debated over the years. A false-negative result may occur if the neurodegenerative process is limited and yet to affect the sacral spinal cord (Onuf’s nucleus). A false-negative result can also arise from errors in MUAP analysis. Automated MUAP analysis by the machine tends to ignore long-duration polyphasic potentials with satellite potentials and rather, analyses the individual components as separate short-duration MUAPs. Hence, it is advisable for the operator to measure MUAPs manually when MSA is suspected. Abnormal EMG findings may be seen in other neurodegenerative conditions such as long-standing Parkinson’s disease, Progressive supranuclear palsy, Dementia with Lewy Bodies and Spinocerebellar ataxia type3, following cauda equina injury, and following damage to the sphincter muscle such as haemorrhoid surgery and pelvic floor tears of traumatic childbirth. Hence, the EMG result should be interpreted in the appropriate clinical context. Nevertheless, a highly abnormal result in a patient with mild Parkinsonism is of value in establishing a diagnosis of probable MSA [
13], particularly in the first 5 years of neurological symptoms [
39]. This differentiation between MSA and PD is important not only for the Neurologist, but also for the Urologist, as incontinence rates are greater in men with MSA following prostatectomy, and should, therefore, be avoided [
44].