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EDITORIAL COMMENTARY
Year : 2021  |  Volume : 24  |  Issue : 1  |  Page : 3-4
 

Commentary on PSP subtypes in India


Department of Neurology, Flt. Lt. Rajan Dhall Fortis Hospital, Vasant Kunj, New Delhi, India

Date of Submission12-Oct-2020
Date of Acceptance12-Oct-2020
Date of Web Publication16-Feb-2021

Correspondence Address:
Dr. Madhuri Behari
Director & Head, Department of Neurology, Flt. Lt. Rajan Dhall Fortis Hospital, Vasant Kunj, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aian.AIAN_1047_20

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How to cite this article:
Behari M. Commentary on PSP subtypes in India. Ann Indian Acad Neurol 2021;24:3-4

How to cite this URL:
Behari M. Commentary on PSP subtypes in India. Ann Indian Acad Neurol [serial online] 2021 [cited 2021 Jun 15];24:3-4. Available from: https://www.annalsofian.org/text.asp?2021/24/1/3/309457




Fifty years ago, Canadian neurologist J. Clifford Richardson described five patients with supranuclear vertical gaze palsy, pseudobulbar palsy, axial rigidity-in-extension and cognitive impairment.[1] He along with Olszewski and Steele described the neuro-pathological findings showing loss of nerve cells and gliosis in the pallidum, subthalamic and red nucleus, substantia nigra and locus coeruleus, the superior colliculi, periaqueductal grey matter, and pretectal regions, the vestibular nuclei, various nuclei of the reticular formation and the dentate nuclei. In these regions neurofibrillary tangles without senile plaques were present. Granulovacuolar changes were seen in the red nucleus and nuclei pontis. PSP is a neuro-degenerative disease which is 4R tauopathy.[2]

The National Institute of Neurological Disorders and Stroke (NINDS-SPSP)[3] criteria have specificity of 96–100% but sensitivity is low.[4],[5],[6]

Since the description of NINDS-SPSP criteria, clinical heterogeneity of PSP has been described with autopsy proven PSP pathology.[5],[7],[8],[9],[10],[11] This prompted MDS to setup the task force on PSP which was mandated to provide all inclusive, yet robust, and highly sensitive and specific clinical diagnostic criteria for PSP. Ideally, diagnostic investigations should be added to the clinical diagnostic criteria of PSP to increase both specificity and sensitivity. The positive predictive value of a clinical diagnosis of PSP in life in a patient with the Richardson's syndrome, especially with falls in the first year of life (fulfilling NINDS-SPSP clinical diagnostic criteria is quite high although many patients with the pathological changes of PSP are missed (i.e. low sensitivity).

The MDS in the new diagnostic criteria,[12] included four core clinical domains (ocular motor dysfunction [O], postural instability [P], akinesia [A], and cognitive dysfunction [C]. In each domain, they proposed three characteristic core clinical feature.

Supportive features to indicate the presence of sporadic, adult-onset, gradually progressive neurodegenerative disease.

Mandatory exclusion criteria to rule out PSP should be applied only in patients presenting with suggestive, unusual clinical features justifying further investigation.

As in the NINDS-SPSP criteria three certainty levels are included in the MDS diagnostic criteria. They are definite which is the neuropathological gold standard proved at autopsy, regardless of its clinical presentation. Probable PSP is diagnosed in the presence of a combination of clinical features with high specificity. Possible PSP is diagnosed in the presence of clinical features considered to substantially increase the sensitivity for PSP.


   Predominant Types of PSP Top


Clinical predominance types are determined based on the combination of clinical features (These include PSP-RS (Richardson), PSP-OM (oculo-motor), PSP-PI (postural instability), PSP-P (parkinsonism), PSP-F (Frontal dysfunction), PSP-PGF (gait freezing), PSP-CBS (corticobasal), and PSP-SL (speech and language).[13]

The importance of making a correct diagnosis of PSP, will become critical with the development of effective disease modifying therapies that are specifically directed at the influence of tau aggregation in the pathogenesis of the disorder.

Efforts by several authors have been made to identify several phenotypic variants based on autopsy studies. They have shown PSP-RS to form the major proportion, followed by PSP-P, PSP-OM, PSP-PIGF followed by the rest.

In this issue an article from a tertiary care teaching hospital categorized 334 PSP patients collected from 1996 to 2017, diagnosis based on NINDS-SPSP criteria, were re-classified according to MDS PSP criteria. Though the study lacks supporting evidence of autopsy, it is still a major landmark study.[14]



 
   References Top

1.
Richardson JC, Steele J, Olszewski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of “heterogenous system degeneration”. Trans Am Neurol Assoc 1963;88:25-9.  Back to cited text no. 1
    
2.
Williams, DR, Lees AJ, Wherrett JR, Steele JC. J. Clifford Richardson and 50 years of progressive supranuclear palsy. Neurology 2008;70:566-73.  Back to cited text no. 2
    
3.
Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): Report of the NINDS-SPSP international workshop. Neurology 1996;47:1-9.  Back to cited text no. 3
    
4.
Respondek G, Roeber S, Kretzschmar H, Troakes C, Al-Sarraj S, Gelpi E, et al. Accuracy of the National Institute for Neurological Disorders and Stroke/Society for Progressive Supranuclear Palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy. Mov Disord 2013;28:504-9.  Back to cited text no. 4
    
5.
Birdi S, Rajput AH, Fenton M, Donat JR, Rozdilsky B, Robinson C, et al. Progressive supranuclear palsy diagnosis and confounding features: Report on 16 autopsied cases. Mov Disord 2002;17:1255-64.  Back to cited text no. 5
    
6.
Osaki Y, Ben-Shlomo Y, Lees AJ, Daniel SE, Colosimo C, Wenning G, et al. Accuracy of clinical diagnosis of progressive supranuclear palsy. Mov Disord 2004;19:181-9.  Back to cited text no. 6
    
7.
Hassan A, Parisi JE, Josephs KA. Autopsy-proven progressive supranuclear palsy presenting as behavioral variant frontotemporal dementia. Neurocase 2012;18:478-88.  Back to cited text no. 7
    
8.
Compta Y, Valldeoriola F, Tolosa E, Rey MJ, Marti MJ, Valls-Sole J. Long lasting pure freezing of gait preceding progressive supranuclear palsy: A clinicopathological study. Mov Disord 2007;22:1954-8.  Back to cited text no. 8
    
9.
Facheris MF, Maniak S, Scaravilli F, Schule B, Klein C, Pramstaller PP. Pure akinesia as initial presentation of PSP: A clinicopathological study. Parkinsonism Relat Disord 2008;14:517-9.  Back to cited text no. 9
    
10.
Williams DR, Holton JL, Strand K, Revesz T, Lees AJ. Pure akinesia with gait freezing: A third clinical phenotype of progressive supranuclear palsy. Mov Disord 2007;22:2235-41.  Back to cited text no. 10
    
11.
Boeve B, Dickson D, Duffy J, Bartleson J, Trenerry M, Petersen R. Progressive nonfluent aphasia and subsequent aphasic dementia associated with atypical progressive supranuclear palsy pathology. Eur Neurol 2003;49:72-8.  Back to cited text no. 11
    
12.
Hoglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, et al. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord 2017;32:853-64.  Back to cited text no. 12
    
13.
Grimm MJ, Respondek G, Stamelou M, Arzberger T, Ferguson L, Gelpi E, et al. How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy. Mov Disord 2019;34:1228-32.  Back to cited text no. 13
    
14.
Mahale RR, Krishnan S, Divya KP, Jisha VT, Kishore A. Subtypes of PSP and prognosis: A retrospective analysis. Ann Indian Acad Neurol 2020;24:56-62.  Back to cited text no. 14
    




 

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