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aLateralized cognitive disturbances: sensory or visual neglect, ideomotor apraxia, aphasia, or alien limb syndrome. Cortical disturbances: memory, aphasia, apraxia, and agnosia. PSP, progressive supranuclear palsy; MSA, multiple system atrophy; CBD, corticobasal degeneration; DLB, dementia with Lewy bodies.

aLateralized cognitive disturbances: sensory or visual neglect, ideomotor apraxia, aphasia, or alien limb syndrome. Cortical disturbances: memory, aphasia, apraxia, and agnosia. PSP, progressive supranuclear palsy; MSA, multiple system atrophy; CBD, corticobasal degeneration; DLB, dementia with Lewy bodies.

Diagnostic certainty only increases with the appearance of telltale signs. Instability and falls in MSA are usually present when patients already exhibit autonomic disturbances. Early instability or falls may also rarely develop early in patients with CBD when symptoms initially occur in lower extremities, but examination shows unilateral features (15). Falls are not an early feature in Parkinson's disease (PD), but they may occur early in dementia with Lewy bodies (DLB) and usually associate to cognitive disturbances.

Table 2

Revised NINDS-SPSP Consensus Criteria for Clinical Diagnosis Definite PSP: Clinically probable or possible PSP and histologically typical PSP Clinically Definite PSP

Step 1 Mandatory Inclusion Criteria:

  1. Gradually progressive disorder with onset at age 40 or later and
  2. Vertical supranuclear ophthalmoparesis (either moderate to severe upward or any downward gaze abnormalities) and
  3. Prominent postural instability with falls (or tendency to falls) in the first year of symptom onset

Clinically Probable PSP

Step 1 Mandatory Inclusion Criteria:

  1. Gradually progressive disorder with onset at age 40 or later and either:
  2. Vertical supranuclear ophthalmoparesis (either moderate to severe upward- or any downward-gaze abnormalities) or
  3. Slowing of vertical saccades and prominent postural instability with falls (or tendency to falls) in the first year of symptom onset For Both Clinically Definite and Clinically Probable PSP

Step 2 Mandatory Exclusion Criteria:

  1. History compatible with encephalitis lethargica
  2. Alien hand syndrome, cortical sensory deficits, focal frontal or temporoparietal atrophy
  3. Hallucinations or delusions unrelated to dopaminergic therapy
  4. Cortical dementia of Alzheimer's type (severe amnesia and aphasia or agnosia, NINCDS-ADRDA criteria)
  5. Prominent cerebellar symptomatology or unexplained dysautonomia (early, prominent inconti nence, impotence, or symptomatic postural hypotension)
  6. Severe asymmetry of parkinsonian signs (bradykinesia)
  7. Neuroradiologic evidence of relevant structural abnormality (basal ganglia or brainstem infarcts, lobar atrophy)
  8. Whipple's disease, confirmed by polymerase chain reaction, if indicated Clinically Possible PSP

To be defined

Ocular Motor Abnormalities

Supranuclear vertical gaze palsy allows the diagnosis of PSP to be made and distinguishes it from all other related disorders such as CBD, MSA, PD, and DLB (see video-PSP and Chapter 15). However, vertical supranuclear gaze palsy is rarely (8%) present at symptom onset; it usually takes 3-4 yr for it to develop (10). Vertical supranuclear gaze palsy, moderate or severe postural instability, and falls during the first year after onset of symptoms classified the NINDS sample with 9% error using logistic regression analysis (16). Although supranuclear gaze palsy is key in diagnosing PSP, it may occasionally be present in patients with DLB, arteriosclerotic pseudoparkinsonism, MSA, Creutzfeldt-Jakob disease, Whipple's disease, or CBD.

Symptom progression is usually very helpful to differentiate these disorders. Whereas in PSP the vertical supranuclear gaze palsy precedes the development of the horizontal gaze palsy, in CBD the supranuclear gaze palsy, when present, usually affects both horizontal and vertical gaze and is usually preceded by ocular motor apraxia (see video-oculomotor apraxia Chapter 1). Both downward-and upward-gaze palsy can be observed in PSP, but the upward-gaze palsy needs to be differentiated from the limitation of upward gaze observed in elderly patients.

Slowing of vertical saccades (rapid eye movement between two stimuli not letting the eyeball movement be seen) usually precedes the development of the supranuclear vertical gaze palsy and allows an earlier diagnosis of the disease (4) (see video-slowing of saccades Chapter 1). In fact, marked slowing of vertical saccades should point toward the diagnosis of PSP. The saccades in CBD may have an increased latency but normal speed, and are similarly affected in the vertical and horizontal plane, whereas in MSA, the saccades have normal speed and latency. Similarly, note that the vertical saccades of elders with upward-gaze limitation are of normal speed.

Blink rate usually becomes profoundly sparse in PSP, although often diminished in PD and MSA. The combination of rare blinking, facial dystonia, and gaze abnormalities leads to the development of a particular "staring and nonblinking faces." Eyelid apraxia (a difficulty or slowness with opening or closing the eyelids accompanied by compensatory elevation of the eyebrows and frontalis overactivity giving a furrowed brow) or blepharospasm (a forceful contraction of orbicularis oculi squeezing the eyes shut and making the eyebrows descend) are also features observed in PSP (14,17,18), but they hardly help in the diagnosis as they may be observed in other parkinsonian disorders (i.e., CBD).

Behavioral and Cognitive Frontal Features

Florid frontal lobe symptomatology (impaired abstract thought, decreased verbal fluency) including motor perseveration and frontal behavioral disturbances, primarily apathy, but also disinhibition, depression, and anxiety, usually manifests at early stages in PSP, whereas it is typically less evident or manifests later in the other parkinsonian disorders (19-25). Apathy, but not depression, is frequently observed in patients with PSP and may be the initial symptom (24). Almost all PSP patients examined neuropsychologically demonstrate an early and prominent executive dysfunction that includes difficulty with planning, problem solving, concept formation, and social cognition. Moreover, executive dysfunction may be the presenting symptom in some PSP patients and is a frequent feature throughout the disease.

In PD and MSA, in contrast to PSP, frontal lobe features are usually mild, and revealed only on detailed neuropsychological testing (26). Because patients with PSP usually exhibit early frontal lobe cognitive and/or behavioral disturbances, they occasionally are confused with patients with Pick's disease or AD. However in PSP, "cortical" dementia is rare or only mild.

Extrapyramidal Signs

PSP patients usually exhibit axial more than limb muscle involvement (10,27) (see video-PSP). In the NINDS study, at the first visit to a specialized neurology center, 88% had bilateral bradykinesia; 63% a predominant akinetic-rigid disease course, and 63% axial rigidity (10). An absent, poor, or waning response to levodopa is a characteristic feature defining the atypical parkinsonian disorders (see Chapter 1) and is a feature of PSP. For practical purposes, all PD patients have a good or excellent response to dopaminergic agents given in appropriate doses for an adequate period of time. If they do not respond, they almost certainly do not have PD. A few PSP patients show a moderate transient response from dopaminergic agents, but most do not. Indeed, this may be because many PSP patients have little or no limb parkinsonism to respond to. In addition, PSP patients uncommonly develop levodopa-induced involuntary movements and if they do, they usually develop dystonia (i.e., blepharospasm). The disproportionate retrocollis that used to be thought of as characteristic of PSP is infrequently observed, but other types of dystonia such as oromandibular, blepharospasm, and more rarely limb dystonia, have been reported (18). In those cases dopaminergic medication should be cautiously reduced or discontinued to rule out the possibility of treatment-induced symptoms.

Speech, Swallowing, and Other Neurological Features

Patients with PSP may also present prominent early, or severe, speech and swallowing difficulties, but these features may also be present in CBD. PSP patients classically have a hypokinetic-spastic dysarthria (see video-PSP, and also Chapter 16). Speech perseveration, where whole words or phrases are repeated, and anomia, but not true aphasia may be observed in PSP.

Swallowing disturbances occur early in PSP. PD rarely causes early dysphagia, and even later in the disease, severe dysphagia is more common in PSP and MSA. Severe early sialorrhoea may be observed in PSP, but is rarely observed early in PD. Drooling of saliva and the voluntary down-gaze palsy causing food to fall from the fork may lead to the reported "sloppy tie or shirt sign."

Even at early stages of the disease, PSP patients may have difficulty judging the amount of food they can swallow and tend to take oversize mouthfuls or overstuff their mouths when eating (see video-PSP). This type of change in eating behavior is infrequent in patients with other atypical parkinsonian disorders or PD.

Pyramidal signs, usually bilateral, are features that present later in PSP than in MSA or CBD. About one-third of patients with PSP and one-half of those with MSA and CBD develop pyramidal signs. In these disorders, it may be hard to differentiate a Babinski sign from a "striatal" toe owing to dystonia.

Early or late insomnia and difficulties in maintaining sleep have all been reported in this disorder, but REM sleep behavioral abnormalities are infrequently reported. Sleep disturbances are thought to be a result of abnormalities in sleep patterns and in motor function.

In general, symptoms and signs apparent early in the course of PSP progress steadily. Most PSP patients eventually require a wheelchair and a feeding tube, and speech may become unintelligible, palilalic, or mute. Goetz et al. reported that these three milestones (wheelchair, unintelligible speech, and feeding tube) occur rapidly in PSP and can be monitored with standardized rating scales (28). In their study, 88% of the sample (50 patients) met at least one of the three milestones, but the need for nasogastric tube was never the first. The median time from symptom onset to the first key motor impairment was 48 mo; gait disturbances occurred at a median symptom duration of 57 mo, and unintelligible speech at 71 mo. As a composite end point, speech and gait accounted for 98% of this sample first key motor impairment. These indices could be used as outcome measures in clinical trials to assess how interventions alter anticipated disease progression (29).

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