– A progressive neurodegenerative disorder
– Hallmarks: autonomic dysfunction and motor symptoms (parkinsonism and cerebellar ataxia)
– Prevalence: 1 – 4.9 per 100, 000
– Commoner: > 50 years; mean onset in 6th decade
– Mean survival: 7 to 9 years
– Common causes of death: sudden death, pneumonia
– Slight male preponderance (55% of cases are men)
– Classified based on initial predominant motor symptom into 2 main forms: MSA-P (parkinsonian subtype – 80%) and MSA-C (cerebellar subtype -20%)
– Pathology: widespread neuronal cell loss and gliosis in the nigrostraital olivopontocerebellar, medullary and spinal cord autonomic pathways; glial cytoplasmic inclusions (Papp-Lantos bodies)
– Bradykinesia (slowness initiating and executing movements)
– Rigidity (predominantly akinetic-rigid parkinsonism)
– Tremor (hands mainly, irregular, minipolymyoclonus)
– Craniocervical dystonia
– High-pitched dysarthria
– Urogenital dysfunction: impotence in men
– Postural hypotension (dizziness or fainting)
– Respiratory difficulties: inspiratory stridor, vocal cord paralysis, loud snoring)
– Other sleep problems: obstructive sleep apunoea, REM BD
– Urinary incontinence, incomplete bladder emptying, urgency, frequency, retention
– Thermoregulatory difficulties; sweating abnormalities, dry mouth and skin.
Distinguishing from PD:
– Early postural instability (early falls 1 in 5 in first year)
– Akinetic rigid parkinsonism
– Early and severe autonomi dysfunction (bldder, GUS)
– Rapid progression
– Inspiratory stridor
– Emotional incontinence
– Severe bulbar dysfunction
– Clinical diagnosis
– Assessment of autonomic function: Cardiac autonomic function, Urodynamic testing.
– Histopathology (at autopsy): Cell loss and gliosis or a proliferation of astrocytes in damaged areas of CNS; Formation of glial scar.
– Requires specialist care: recognition, early referral
– Symtomatc treatment: postural hypotension, parkinsonian features, urinary complaints, constipation, sleep and breathing
– Physical therapy and rehabilitation (walking, speech, mobility, daily tasks, gait training, mobility aids, transfer training, home adaptations)
– Social Support: referral to social workers to provide support for carers, patients, family care givers.
– Homecare services: long term; help with daily activities and general care.