Multiple system atrophy

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 02/04/2015

Print this page

rate 1 star rate 2 star rate 3 star rate 4 star rate 5 star
Your rating: none, Average: 0 (0 votes)

This article have been viewed 1036 times

95 Multiple system atrophy

Instruction

Perform a neurological examination on this patient.

Salient features

History

• Dizziness when standing up (caused by postural hypotension)

• Dysphagia

• Ataxia

• Symptoms of Parkinson’s disease (p. 191)

• Impotence, bladder disturbances (p. 346)

• Anhidrosis.

Examination

• Mask-like facies and other features of bradykinesia

• Increased tone (rigidity)

• Cerebellar signs.

Proceed as follows:

• Tell the examiner that you would like to:

• look for postural hypotension (a fall in systolic BP of at least 20 mmHg or in diastolic BP of at least 10 mmHg within 3 min after standing), the hallmark of this condition (caused by autonomic failure)
• look for signs of autonomic dysfunction (pupillary asymmetry, Horner syndrome).

Diagnosis

This patient has cerebellar and Parkinson’s signs (lesion) caused by multiple system atrophy, a degenerative disorder (aetiology), and has marked disability including incontinence (functional status).

Advanced-level questions

What are the types of multisystem atrophy?

Striatonigral degeneration. Clinical picture resembles Parkinson’s disease but without tremor. These patients do not respond to antiparkinsonian medications and often develop adverse reactions to these agents.

Shy–Drager syndrome. Clinical picture consists of Parkinson’s disease combined with severe autonomic neuropathy (particularly postural hypotension). Other important clinical features are impotence and bladder disturbances.

Olivopontocerebellar atrophy. Combination of extrapyramidal manifestations and cerebellar ataxia. Patients may also have autonomic neuropathy and anterior horn cell degeneration.

Parkinsonism and motor neuron disease. Rare.

What is the pathology in Shy–Drager syndrome?

In 1960, Shy and Drager described changes in the brainstem and ganglia; subsequently, loss of neurons has been shown in the autonomic nervous system and in the cells of the intermediolateral column of the spinal cord. PET shows decreased uptake of dopamine in the putamen and caudate lobe.

What factors can lower blood pressure in these patients?

Standing up: orthostatic hypotension, a hallmark of this condition.

Food and exercise can produce hypotension even in the supine position (J Neurol 1990;237(suppl 1):S24, J Am Coll Cardiol 1993;21:97A).

What is the morbidity of this condition?

It is tends to disable most patients severely by the end of 5–7 years.

How would you treat these patients?

Treatment is symptomatic and supportive for hypotension and neurological deficits. Symptoms of postural hypotension may be ameliorated by antigravity stockings and fluorohydrocortisone.

How does pure autonomic failure differ from multisystem atrophy?

Buy Membership for Internal Medicine Category to continue reading. Learn more here

Related posts:

Default ThumbnailSixth cranial nerve palsy Osteoarthrosis Optic atrophy Default ThumbnailHepatomegaly Carotid artery aneurysm Felty’s syndrome