Clinical Features And Differential Diagnosis

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The most important features that characterize and differentiate PSP from other disorders are presented in Table 1 and described in more detail in the subheading that follows.

Postural Instability and Falls

Postural instability manifested as nonexplained and unexpected falls or tendency to falls is the most frequent symptom presentation in PSP (7-14). In the National Institutes of Neurological Disorders and Stroke (NINDS) study (10), 96% of 24 PSP patients had gait disorder and postural instability (83% history of falls) at the first visit to a specialized neurology center, which generally occurred 3-3.5 yr after symptom onset. Falls usually occur backward in PSP, but they can occur in any direction (7,10) (described by a patient in the video-PSP). The presence of nonexplained and unexpected falls within the first year of symptom onset is in fact one of the required criteria for the diagnosis of PSP when using the NINDS-Society for PSP (SPSP) diagnostic criteria (Table 2). PSP is the most likely diagnosis when unexplained postural instability and falls occur within the first year of symptom onset, but after that, multiple system atrophy (MSA) is equally possible. When postural instability and falls are the only features of the disease and an abnormal response to the postural reflex may be the only abnormality in patient's examination, diagnostic problems are frequent.

From: Current Clinical Neurology: Atypical Parkinsonian Disorders Edited by: I. Litvan © Humana Press Inc., Totowa, NJ

  1. 1. (Left) Neurologist J. Clifford Richardson (1909-1986) realized that a set of patients evaluated since 1955 in Toronto had an unusual combination of symptoms that seemed to correspond to a disease he was unaware of. (Middle) pathologist Jerry Olszewski (1913-1964) described in detail the pathological findings of seven cases that came to autopsy. (Right) John C. Steele (1934) joined the neurology residency in 1961 and investigated with Dr. Richardson the clinical features and progression of these patients during the following 2 yr. From Progressive Supranuclear Palsy: Clinical and Research Approaches, edited by Irene Litvan and Yves Agid, copyright 1992 by Oxford University Press, Inc. Used by permission of Oxford University Press, Inc.
  2. 1. (Left) Neurologist J. Clifford Richardson (1909-1986) realized that a set of patients evaluated since 1955 in Toronto had an unusual combination of symptoms that seemed to correspond to a disease he was unaware of. (Middle) pathologist Jerry Olszewski (1913-1964) described in detail the pathological findings of seven cases that came to autopsy. (Right) John C. Steele (1934) joined the neurology residency in 1961 and investigated with Dr. Richardson the clinical features and progression of these patients during the following 2 yr. From Progressive Supranuclear Palsy: Clinical and Research Approaches, edited by Irene Litvan and Yves Agid, copyright 1992 by Oxford University Press, Inc. Used by permission of Oxford University Press, Inc.
Fig. 2. Pattern of lesions found in the nine cases reported by Prof. Olszewski in 1963 and published in Archives of Neurology in 1964 (3), with permission.

Table 1

Comparison of Features of Various Atypical Parkinsonian Neurodegenerative Disorders

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