Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 3  |  Page : 342-345  

Steele Richardson Olszewski syndrome


Department of Medicine, Padmashree Dr D Y Patil Medical College, Hospital and Research Centre, Dr D Y Patil Vidyapeeth, Pune, India

Date of Web Publication5-Jul-2013

Correspondence Address:
Vijayashree S Gokhale
Department of Medicine, Dr. D. Y. Patil Medical College, Pimpri, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.114671

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  Abstract 

Parkinson's disease and its plus syndromes are an important cause of morbidity in the geriatric age group. Its plus syndromes show a myriad of clinical features characterized by progressive symptoms. Here we present a 65-year-old woman with progressive "Parkinsonian-like features," i.e., mask-like face, slowness of all movements and tendency to fall, and difficulty in eye movements, leading to the diagnosis of Steele Richardson Olszewski Syndrome or progressive supranuclear palsy.

Keywords: Akinesia, parkinsonism, supranuclear palsy


How to cite this article:
Gokhale VS, Sharma Z, Chaudhari N. Steele Richardson Olszewski syndrome. Med J DY Patil Univ 2013;6:342-5

How to cite this URL:
Gokhale VS, Sharma Z, Chaudhari N. Steele Richardson Olszewski syndrome. Med J DY Patil Univ [serial online] 2013 [cited 2019 Jun 25];6:342-5. Available from: http://www.mjdrdypu.org/text.asp?2013/6/3/342/114671


  Introduction Top


In 1963, three Canadian Physicians, Richardson J. C., Steele J., and Olszewski J., reported eight cases with a syndrome comprising supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia, and dementia, which they titled as "a study of eight cases of heterogeneous system degeneration." [1] This degenerative syndrome is now called progressive supranuclear palsy (PSP), [2] and since it shares some features of  Parkinsonism More Details, it may be sometimes grouped in "parkinsonism plus syndromes." [3]


  Case Report Top


A 65-year-old female, hailing from Majalgaon in Beed district of Maharashtra, India, came to a Medical College-Hospital in Maharashtra with complaints of progressive slowing of all movements, stiffness of entire body, and frequent falls, mostly backward, over a period of 1 year. History was given by patient's younger sister who was normal and there was no family history of similar complaints. Relatives also complained that patient did not show any happiness or sorrow on face, spoke very slowly, ate her food also very slowly, and had urinary incontinence. There was no history of any drug intake on regular basis. There was no history of fever or altered mental status with hospitalization in the past.

Examination revealed the following: Pulse 78/min regular, blood pressure 120/80 mmHg, no orthostatic hypotension, and respiratory rate 18/min. Patient sat with a mask-like face, with minimal facial expression and neck hyperextended, and had to be supported to prevent her from falling backward. Intermittent involuntary pouting was noted.

Higher functions revealed response to simple verbal command was slow, but the same command was better performed when patient's sister repeated the "command." We interpreted this as residual emotional response [4] and evidence of some residual orientation to person. Memory and intelligence could not be tested. Speech was slurred, but patient's sister could interpret patient's speech.

On eye examination, voluntary upward, downward, and lateral movements could not be elicited. [5] Eye balls appeared as if fixed in the center with some jerky drifting movements "saccades"[Figure 1], [Figure 2], [Figure 3] and [Figure 4].
Figure 1: Ophthalmoparesis

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Figure 2: Lateral gaze palsy

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Figure 3: Lateral gaze palsy with "pouting"

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Figure 4: Upward gaze palsy

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There was no nystagmus. Pupils were mid dilated and reacting to light, and other cranial nerves were normal. Motor system showed the tone was increased in all four limbs, with no tremor. Power was 4- in all four limbs. Deep Tendon Reflexes was normal, plantar were flexors. Sensory system was normal, and there were no cerebellar signs. So, on examination of CNS, patient had rigidity, tendency to fall backward, ophthalmoparesis and social cognitive deficits, [6] early dementia, and occasional pouting.

Examination of other systems like cardiovascular, respiratory, and abdominal system was normal.

Investigations done included hemogram, liver function tests (LFT), renal function tests (RFT), X-ray Chest, and ECG, and they showed normal results. Magnetic resonance imaging (MRI) Brain showed no specific abnormality.

Invasive diagnostic procedures like brain biopsy could not be done as the patient did not consent for it, and on ethical concerns, as it would have been purely for academic purpose with not much benefit to the patient. On the basis of the clinical criteria developed by National Institute of Neurological Disorders and the Society of Progressive Supranuclear Palsy (NINDS SPSP) given in [Table 1], the patient was diagnosed as Steele Richardson Olszewski Syndrome or PSP.
Table 1: NINDS SPSP clinical criteria for diagnosis of PSP

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  Discussion Top


Steele Richardson Olszewski syndrome is a degenerative disease which presents in the age group of 60-65, with history of frequent falls, slurring of speech, difficulty in deglutition, an ophthalmoparesis, and later dementia. [5] Our patient was 65 years old and complained of falls, and on examination had ophthalmoparesis, [5] slurring of speech, and cognitive deficits. This syndrome is often diagnosed as Parkinson's disease because of stiffness and mask-like face.

However, there are some differences. In Parkinson's disease, patient tends to have a forward bending stooped posture while the opposite is true in Steele Richardson Olszewski syndrome. Our patient had neck and body leaning backward, with tendency to fall backward. Ophthalmoparesis or gaze palsies present in our patient are not a feature of Parkinson's disease.

Steele Richardson Olszewski syndrome is now classified as PSP.

In 2003, the NINDS SPSP and the Scientific Issues Committee of the Movement Disorders Society formed a Task Force to evaluate the current diagnostic criteria [Table 1]. [7]

Our patient was diagnosed as PSP on the basis of the following:

  • Inclusion criteria "probable": Our patient had vertical supranuclear palsy and prominent postural instability with falls within first year of disease onset
  • Exclusion criteria: Our patient had no history suggestive of encephalitis, no cortical sensory deficits, hallucinations, delusions, cerebellar symptoms, and evidence of dysautonomia
  • Supportive criteria: Our patient had symmetrical rigidity, akinesia, retrocollis, early cognitive impairment, dysarthria, and frontal release signs in the form of "pouting".


PSP can be further differentiated into two phenotypes: [8]

  • PSP-Richardson or the classical type, where supranuclear gaze palsy appears early and
  • PSP-Parks, where there is asymmetrical involvement with extra-axial dystonia and tremor, and patient may respond better to Levodopa.
This difference corresponds to the different areas of brain affected and to the difference in tau protein deposition.

Pathophysiology of PSP: Both neuronal and glial cells are affected, besides showing neurofibrillary tangles containing tau protein. However, this tau protein is different chemically from the tau protein of Alzheimer's disease.

On MRI brain, selective atrophy of mid-brain, thinning of quadrigeminal plate, and atrophy of region around 3 rd ventricle may be seen. [9]

Our patient's MRI was reported normal.

Treatment: Levodopa may improve rigidity, akinesia to some extent.

Rivastigmine [10] and acetylcholinesterase inhibitor therapy [11] has been tried.

Follow-up

Patient was discharged on request and followed up in OPD after 1 week. There was no change in her condition. Later she was lost to follow-up.


  Conclusion Top


We conclude that our case was PSP: The Steele Richardson Olszewski syndrome on the basis of clinical criteria. She had rigidity, akinesia, upward, downward, and lateral gaze palsy, and social cognition deficits, all within a year of onset of disease. Hence, by NINDS SPSP "Special Task force ask Force Criteria" "clinically definite," and since her gaze palsy was of a severe degree and her response to levodopa was minimal, she probably had PSP-Richardson, the classic type.

 
  References Top

1.Richardson JC, Steele J, Olszewski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of "heterogeneous system degeneration". Trans Am Neurol Assoc 1963;88:25-9.  Back to cited text no. 1
    
2.Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy: A heterogeneous degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-59.  Back to cited text no. 2
    
3.Litvan I, Campbell G, Mangone CA, Verny M, McKee A, Chaudhuri KR, et al. Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study. Brain 1997;120:65-74.  Back to cited text no. 3
    
4.Ghosh BC, Rowe JB, Calder AJ, Hodges JR, Bak TH. Emotion recognition in progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 2009;80:1143-5.  Back to cited text no. 4
    
5.Rehman HU. Progressive supranuclear palsy. Postgrad Med J 2000;76:333-6.  Back to cited text no. 5
    
6.Ghosh BC, Calder AJ, Peers PV, Lawrence AD, Acosta-Cabronero J, Pereira JM, et al. Social cognitive deficits and their neural correlates in progressive supranuclear palsy. Brain 2012;135(Pt 7):2089-102.  Back to cited text no. 6
    
7.Litvan I, Bhatia KP, Burn DJ, Goetz CG, Lang AE, McKeith I, et al. Movement Disorders Society Scientific Issues Committee. Movement Disorders Society Issues Committee report: SIC Task Force apparaisal of clinical diagnostic criteria for Parkinsonian disorders. Mov Disord 2003;18:467-86.  Back to cited text no. 7
    
8.Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism. Brain 2005;128(Pt 6):1247-58.  Back to cited text no. 8
    
9.Rizzo G, Martinelli P, Manners D, Scaglione C, Tonon C, Cortelli P, et al. Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and Parkinson's disease. Brain 2008;131:2690-700.  Back to cited text no. 9
    
10.Liepelt I, Gaenslen A, Godau J, Di Santo A, Schweitzer KJ, Gasser T, et al. Rivastigmine for the treatment of dementia in patients with progressive supranuclear palsy: Clinical observations as a basis for power calculations and safety analysis. Alzheimers Dement 2010;6:70-4.  Back to cited text no. 10
    
11.Nijboer H, Dautzenberg PL. Progressive supranucleair palsy: Acetylcholineesterase-inhibitor a possible therapy? Tijdschr Gerontol Geriatr 2009;40:133-7.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

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