A 62-year-old man presented to the clinic with a curious tale. The patient had been driving from California to Boston. When he stopped for gas, the attendant thought he was drunk and called the police. The police heard his slurred speech, found him unable to walk heel-to-toe in a straight line, and arrested him for driving under the influence. Ultimately, it was determined that he was not intoxicated, and he was released. In the clinic, the patient complained most that he had difficulty talking, and his speech was quite slow, hesitant, and dysarthric. His exam was notable for slow voluntary eye movements in all directions, including vertical gaze. Eye movements improved and became smooth by having the patient look straight ahead while his head was gently moved. He walked with a very wide-based gait and often staggered although he did not fall. He showed great difficulty with tests of executive function such as the Trail Making Test Part B and Digit Span Backward; he was unable to correctly draw a clock.
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Prevalence, Prognosis, and Definition
Progressive supranuclear palsy (often abbreviated to PSP and sometimes referred to as the Steele–Richardson–Olszewski syndrome) is a neurodegenerative disease of the brain due to the accumulation of hyperphosphorylated tau protein isoforms in the brain. Its main feature is an abnormality of vertical eye movements (supranuclear palsy), along with postural instability with falls, axial rigidity, frontal lobe signs and symptoms, and difficulty swallowing and talking (pseudobulbar palsy). It has a prevalence of 5–6 per 100,000, with approximately only 1.5 per 100,000 being accurately diagnosed. The mean age of onset of the disease is 63 years, with a usual prognosis ranging from 5 to 10 years from diagnosis to death; median 7 ( ). The disease received increased attention in the popular press when the comedian Dudley Moore was diagnosed with it in 1999.
Terminology
Supranuclear Palsy
A “supranuclear palsy” causing eye movement abnormalities means that there is dysfunction of the part of the brain that controls voluntary eye movements above ( supra in Latin) the level of the oculomotor nucleus (in the midbrain, the upper part of the brainstem). This type of palsy affects mainly voluntary eye movements, while involuntary movements are relatively spared. (Involuntary movements may be controlled by a group of neurons called the superior colliculus.) Testing for a supranuclear palsy involves comparing eye movements for voluntary and involuntary gaze. Testing for voluntary gaze may be easily accomplished by having the patient look rapidly between two points (often called “saccades”), usually between the thumb on one hand and the index finger on the other (“Look at my thumb, look at my finger,” and so on). Testing for involuntary gaze is best performed by having the patient stare at a stationary point while you gently move their head up and down and back and forth, thus moving their eyes in their head. In a supranuclear palsy the rapid, voluntary eye movements will be abnormal while the involuntary movements will be normal (or relatively normal) demonstrating that the eye movement problem is in the voluntary gaze centers, above the oculomotor nucleus.
Pseudobulbar Palsy
If a patient has a “pseudobulbar palsy,” it indicates that there is dysfunction of part of the brain that mimics dysfunction of the lower part of the brainstem, the medulla. A “pseudobulbar palsy” refers to dysfunction of the part of the brain above the medulla that controls movements of the tongue, pharynx, and larynx. Thus, the patient with a pseudobulbar palsy typically exhibits slurred and otherwise abnormal speech (dysarthria), and difficulty eating and swallowing (dysphagia).
Criteria and Diagnosis
The cardinal feature of progressive supranuclear palsy is an abnormality of vertical eye movements. Because up-gaze is sometimes impaired in normal aging, abnormalities of down-gaze are particularly sought for as confirmatory clinical signs when this diagnosis is being entertained. Although many neurologists use the criteria published by , they are less useful when confronted with the patient with cognitive impairment as they make no reference to cognition; we prefer the clinical criteria by Lees (1987; Box 9-1 ). Although not criteria per se , also useful is the progressive supranuclear palsy rating scale, which can be found at this website along with other helpful information: www.psp.org/education/professionals.html .
A degenerative disorder with onset in middle age or later with supranuclear palsy including down-gaze abnormalities and at least two of the following cardinal features:
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Postural instability and falls backward
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Axial rigidity and dystonia
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Pseudobulbar palsy
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Bradykinesia and rigidity
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Frontal lobe signs
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Slow thinking (bradyphrenia)
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Perseveration
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Grasp
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Utilization behavior
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Selected supportive signs
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Other ocular abnormalities
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Rest tremor
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Dystonia of limbs and face
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Cerebellar ataxia
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Dysphagia
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Dyspraxia
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Respiratory dyskinesias (inspiratory gasps, tachypnea)
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Echolalia and palilalia
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Myoclonus
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Emotional lability (pseudobulbar affect)
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