Wisdom of Life

A Synopsis of MSA – Multiple System Atrophy

Background:

–          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)

Clinical features:

Motor features:

–          Bradykinesia (slowness initiating and executing movements)

–          Rigidity (predominantly akinetic-rigid parkinsonism)

–          Tremor (hands mainly, irregular, minipolymyoclonus)

–          Craniocervical dystonia

–          High-pitched dysarthria

Autonomic dysfunction:

–          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

–          Constipation

–          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

–          Anterocollis

–          Inspiratory stridor

–          Emotional incontinence

–          Severe bulbar  dysfunction

Diagnosis:

–          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.

Treatment Approach:

–          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.

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