Early presentation of urinary retention in multiple system atrophy: can the disease begin in the sacral spinal cord?

Abstract

Lower urinary tract (LUT) dysfunction presents early in multiple system atrophy (MSA), usually initially as urinary urgency, frequency and incontinence, and voiding difficulties/urinary retention becomes apparent over time. We have observed a subset of patients who instead presented initially with urinary retention requiring catheterisation. At presentation, these patients had only subtle neurological signs that would not fulfil the diagnostic criteria of MSA; however, the anal sphincter electromyography (EMG) was abnormal and they reported bowel and sexual dysfunction, suggesting localisation at the level of the sacral spinal cord. They subsequently developed classical neurological signs, meeting the diagnostic criteria for probable MSA. One patient was confirmed to have MSA at autopsy. We postulate that in a subset of patients with MSA, the disease begins in the sacral spinal cord and then spreads to other regions resulting in the classical signs of MSA. The transmissibility of alpha-synuclein has been demonstrated in animal models and the spread of pathology from sacral cord to other regions of the central nervous system is therefore plausible. Patients presenting with urinary retention and mild neurological features would be an ideal group for experimental trials evaluating neuroprotection in MSA

Spacing spheroids, curl-up and coiled neuritic plexus of the sacral spinal cord of aged dog: Hypothesis based on continued NADPH diaphorase histological observations

“…The vacuolar neurons and neurites revealed in the thoracic, lumber and sacral spinal cord. This was similar other neuropathology of neurodegenerative diseases [34][35][36][37][38][39]. It could be speculated that the aging-related N-d alteration was neuropathological degeneration.…”

“…Autonomic dysfunctions of orthostatic hypotension, urinary incontinence and constipation are common symptoms in neurodegenerative demented patients [38]. Neurodegeneration of the sacral spinal cord may be relevant the onset of multiple system atrophy [39]. Onset of urinary retention in multiple system atrophy can begin in the sacral spinal cord [39].…”

“…Neurodegeneration of the sacral spinal cord may be relevant the onset of multiple system atrophy [39]. Onset of urinary retention in multiple system atrophy can begin in the sacral spinal cord [39]. Ballooned neurons can occur in the IML of the sacral spinal cord [44].…”

Possible “Premotor” Multiple System Atrophy-Cerebellar Form

“…Although he lacked a motor disorder, all these features suggested possible MSA-C [1][2][3]. We started him on clean, intermittent self-catheterization.…”

“…Multiple system atrophy (MSA) is a progressive degenerative neurological disorder clinically defined as a combination of motor (parkinsonian MSA and/or cerebellar MSA [MSA-C]) and autonomic (orthostatic and/ or bladder) disorders by the second consensus statement on the diagnosis of MSA [1]. In contrast, recent prospective cohort studies have shown that up to 18.2% of MSA patients start with bladder dysfunction alone that might last up to 7 years [2,3], suggesting that the disease process of alpha-synuclein aggregation might begin in the sacral spinal cord [4]. However, the question arises as to whether we are able to diagnose very early, "premotor" MSA.…”