Page 1 of 1: steel richardson synd

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steel richardson syndrome is

a variant of parkinsonism

a variant of neuropathy

a variant of cerebrovasular accident

a variant of alzimer disease

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Degenerative conditions of the nervous system, which may have Parkinsonian features, include:

Progressive supranuclear palsy (Steel Richardson's)
Corticobascal degeneration
Creutzfeld-Jacob disease
Primary pallidal atrophy
Idiopathic dystonia-parkinsonism
Hemiatrophy-hemiparkinsonism
Parkinsonism-ALS-dementia complex of Gaum
Atherosclerotic or senile Parkinsonism
Alzheimer's and Pick's diseases

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Ref- Oxford Manual of Medicine Pg 452
Steel- Richardson- Olszewski Synd is one of the rare causes of Parkinsonnism.It is a progressive supranuclear palsy with
1.absent vertical gaze, both upward & downward, and
2.dementia

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PROGRESSIVE SUPRANUCLEAR PALSY
Definition
In 1964 Steele, Richardson, and Olszewski described a syndrome with similarities to parkinsonism, but with an additional characteristic and diagnostic abnormality of voluntary eye movement

. The illness is progressive and is characterized by akinesia, axial rigidity, postural imbalance, dysarthria, a supranuclear paralysis of voluntary vertical eye movements, and mental changes, with onset in middle or late life.

Aetiology
The cause of progressive supranuclear palsy is not known. It is a rare sporadic disease. Males are affected more than females, and no familial cases have been described. Onset usually is in the fifth to seventh decades.

Pathology
There is widespread neuronal loss and gliosis in the brain-stem, affecting particularly the globus pallidus, substantia nigra, subthalamus, red nucleus, tectum and periaqueductal grey matter, and dentate nucleus. Such affected nerve cells contain neurofibrillary tangles. The cerebral cortex, however, is unaffected and senile plaques do not occur.

Symptoms
The illness most commonly presents either with imbalance or visual symptoms. The latter consist of difficulty with reading or coming down stairs, because both demand voluntary downward gaze, and vertical gaze characteristically is impaired. The patient cannot voluntarily look up and down, nor follow an object moved in the vertical plane (Fig. 6) 2947. But a full range of vertical eye movements can be elicited reflexly by rapid posturing of the head (the doll's eye manoeuvre). Horizontal eye movement also is similarly affected, but usually later than vertical eye movement. The earliest signs of impairment of eye movement is a breakdown of swift, smooth, rapid voluntary movement into short, jerky, repetitive saccades. The characteristic profound and early involvement of vertical gaze is accompanied by a typical dystonic extension of the neck, and a fixed wide-eyed stare. The patient looks around by moving the head, rather than the eyes.

Imbalance and unexplained falls are a common early complaint. At this stage the patient also will exhibit characteristic axial rigidity of neck, trunk, and proximal limb muscles, and poverty of movement, particularly of whole-body movement. Speech is dysarthric, as in a pseudobulbar palsy, and swallowing may be impaired. Dystonic postures and other abnormal movements, including tremor, may occur. Akinesia and rigidity are evident, but power is preserved, as is sensation; the tendon reflexes are brisk and the plantar responses may be extensor.

Mental function is impaired. The patient may give an impression of dementia, with difficulty in memory and loss of intellectual power. Careful examination, however, reveals that memory and intellect are better than is apparent at first sight, provided the patient is given adequate time to formulate answers. This impairment of mental processes, characterized by mental slowness and inefficiency on tests sensitive to frontal lobe dysfunction, has been termed ‘subcortical dementia’.

Natural history
The disease is relentlessly progressive, the patient becoming bedridden and anarthric, leading to death in 5 to 7 years.

Diagnosis
The crucial diagnostic feature is the supranuclear gaze palsy. However, eye movements may also be abnormal in patients with other types of parkinsonism. Progressive supranuclear palsy is diagnosed with confidence only when down-gaze is affected, but this may occur late in the illness, and such a disorder of eye movements is not specific for Steele-Richardson-Olszewski disease.

Treatment
There is no effective treatment for this condition, which usually does not respond to levodopa or other dopamine agonists, although such drugs are worth trying. Amantadine and anticholinergic drugs also occasionally may help.

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abdoel-sayed · Credits: 751 My Scrapbook

thanks forr all

arrii · Credits: 311 My Scrapbook

keep on asking questions they will help to clear your path of all doubts. keep it up

bksnitin · Credits: 473 My Scrapbook

tats a good information... It helped me a lot...

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