Perform a neurological examination on this patient.
· Dizziness when standing up (due to postural hypotension).· Dysphagia.· Ataxia.· Symptoms of Parkinson's disease .· hnpotence, bladder disturbances .· Anhidrosis.
· 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, the hallmark of this condition (due to autonomic failure).· Signs of autonomic dysfunction (pupillary asymmetry, Homer's syndrome).
This patient has cerebellar and Parkinson's signs (lesion) due to multiple system atrophy, a degenerative disorder (aetiology), andhas marked disability including incontinence (functional status).
What are the types of multisystem atrophy?
· Striatonigral degeneration: clinical picture resembles Parkinson's disease but without tremor. These patients do not respond toanti-Parkinson medications and often develop adverse reactions to these agents.· Shy-Drager syndrome: clinical picture consists of Parkinson's disease combined with severe autonomic neuropathy (particularlypostural hypotension). Other important clinical features are impotence and bladder disturbances.· Olivopontocerebellar atrophy: combination of extrapyramidal manifestations and cerebellar ataxia. Patients may also haveautonomic neuropathy and anterior horn cell degeneration.· Parkinsonism and motor neuron disease: rare.
What is the pathology in Shy-Orager syndrome?
In 1960, Shy and Drager described changes in the brainstem and ganglia; sub-sequently, loss of neurons has been shown in theautonomic nervous system and in the cells of the intermediolateral column of the spinal cord. Positron emission tomo-graphy showsdecreased 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.Food and exercise can produce hypotensioneven in the supine position (JNeurol1990; 237(suppl 1): S24; JAm Coil Cardiol 1993; 21: 97A).
What is the morbidity of this condition?
It 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 beameliorated by antigravity stockings and fluorohydrocortisone.G.M. Shy (1919-1967), a US neurologist who obtained his MRCP in London in 1947.G.A. Drager (1917-1967), a US neurologist.Bradbury and Eggleston in 1925 first described the combination of postural hypotension, incontinence, impotence and abnormality ofsweating (anhidrosis). Neurological manifestations develop later.Christopher J. Mathias, FRCP, DSc, contemporary Professor of Medicine, St Mary's Hospital Medical School and National Hospital,Queen Square, London, whose chief interest is the autonomic control of the cardiovascular system.