Parkinson’s disease with Onuf’s nucleus involvement mimicking multiple system atrophy
- S S O’Sullivan1,2,
- J L Holton1,
- L A Massey1,3,
- D R Williams1,4,
- T Revesz1,
- A J Lees1,2
- 1Queen Square Brain Bank, Department of Molecular Neuroscience, Institute of Neurology, University College London, London, UK
- 2Reta Lila Weston Institute of Neurological Studies, University College London, London, UK
- 3Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK
- 4Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), Melbourne, Australia
- Prof Andrew J Lees, FRCP, Reta Lila Weston Institute of Neurological Studies, University College London, 1 Wakefield Street, London WC1N 1PJ, UK; alees{at}ion.ucl.ac.uk
- Received 24 August 2007
- Revised 24 September 2007
- Accepted 12 October 2007
- Published Online First 19 October 2007
Urinary frequency, urgency and nocturia are common complaints in Parkinson’s disease (PD). The hypothesis most widely proposed to explain neurogenic bladder symptoms in PD is that cell loss in the substantia nigra may cause detrusor hyperactivity due to a loss in the D1 receptor-mediated tonic inhibition of the micturition reflex, although other causes including anti-parkinsonian medication cortical effects have been considered.1 We present the clinical and pathological findings of a patient with parkinsonism who presented with prominent dysautonomia and a poor response to dopaminergic medications and was considered to have possible multiple system atrophy parkinsonism (MSA-P). Pathological examination revealed that the patient had PD with α-synuclein pathology in the Onuf’s nucleus (ON).
CASE REPORT
A 52-year-old male presented with a 12-month history of urinary frequency and urgency in 1999. Examination revealed no prostate enlargement. Urodynamic studies showed evidence of bladder instability and obstruction, with flexible cystoscopy not showing any abnormalities. Two years later, he started to slow down in his movements, shuffle and complain of stiffness. He had a stooped posture, a frozen facial appearance, reduced blinking and mild limb rigidity. Deep tendon reflexes were brisk, with equivocal plantar responses. Orthostatic hypotension was found with a …
