Progressive Supranuclear Palsy

In 1963, Steele, Richardson, and Olszewski presented a report at the American Neurological Association of a new syndrome typified by parkinsonism, marked vertical gaze paresis, dementia, and axial rigidity (10,11). Though they felt they were describing a new syndrome, they referred colleagues to similar cases from the recent past (12-14) (Fig. 4). H. Houston Merritt opened the discussion, commenting that he had not seen similar cases, that the involved areas all related to cell populations of similar phylogenetic age, and that dementia was of particular interest. F. McNaughton acclaimed: "I believe that the authors have described a clear-cut neurological syndrome and to judge from the pathological studies, it may, in fact, represent a new disease entity." D. Denny-Brown was less sure and ascribed the cases to variants of Jakob's "spastic pseudosclerosis" (10).

Prior to these 20th-century descriptions, several cases of "Parkinson's disease with extended posture" can be identified with characteristics suggestive of progressive supranuclear palsy. Charcot presented a man, named Bachere, to his students on several occasions (see Fig. 2). Commenting on June 12, 1888, Charcot mentioned that Bachere did not have marked tremor and emphasized the issue of extension posture:

There is something else unusual here worth noting. Look how he stands. I present him in profile so you can see the inclination of the head and trunk, well described by Parkinson. All this is typical. What is atypical, however, is that Bachere's forearms and legs are extended, making the extremities like rigid bars, whereas in the ordinary case, the same body parts are partly flexed. One can say then that in the typical case of Parkinson's disease, flexion is the predominant feature, whereas here, extension predominates and accounts fro this unusual presentation. The difference is even more evident when the patient walks (3).

In addition to extended posture, this patient had particular facial bradykinesia and contracted forehead muscles (15). Charcot commented that the patient had the perpetual look of surprise because the eyes remained widely opened and the forehead continually wrinkled (Fig. 5). In a modern setting, Jankovic has detailed similar facial morphology in Parkinsonism-plus patients, specifically those with progressive supranuclear palsy (16). The extended truncal posture of this patient would be compatible with the posture of progressive supranuclear palsy, although Charcot did not comment on specific supranuclear eye movement abnormalities. Another Salpetriere patient with "Parkinson's disease in extension" was described by Dutil in 1889 and eye movement abnormalities are mentioned, although a supranuclear lesion is not documented clinically (17,18) (see Fig. 3). This case also had highly asymmetric rigidity of the extremities, a feature more reminiscent of corticobasal degeneration than progressive supranuclear palsy (see next subheading). In this case, the extended neck posture was graphically emphasized:

The face is masked, the forehead wrinkled, the eyebrows raised, the eyes immobile. This facies, associated with the extended posture of the head and trunk, gives the patient a singularly majestic air (17,18).

With clinical features reminiscent of both progressive supranuclear palsy and corticobasal degeneration, this patient was mentioned in several articles from the Salpetriere school, although no autopsy was apparently performed.

In their studies of tremor and Parkinson's disease, Charcot and contemporary colleagues described several other cases of parkinsonian patients who never suffered with either prominent resting or postural tremor. Although the descriptive details are often cursory, several of these cases may well represent cases of progressive supranuclear palsy. Bourneville published two cases in 1876 (19), and later French students chose this subclass of patients for special clinical emphasis (20). In his thesis on

Fig. 4. Photograph from 1951 article by Chavany (12) showing a patient with extended posture suggestive of progressive supranuclear palsy prior to the definitive description by Steele and colleagues in 1964.

atypical forms of parkinsonism, Compin specifically noted that parkinsonian cases without tremor showed especially marked rigidity and often fall (20). His first case history documented several additional features typical of the group of parkinsonism-plus syndromes, including early age of onset (age 45), prominent gait and balance difficulty within the first years of illness, and other midline dysfunction such as marked and early speech impairment.

Historical research on early medical diagnoses has occasionally benefited from nonmedical sources, especially literary descriptions. Because movement disorders are particularly visual in their character, it is reasonable to search the writings of celebrated authors known for their picturesque descriptive

Vertical Supranuclear Gaze Palsy

Fig. 5. Four pictures drawn by Charcot of a patient with likely progressive supranuclear palsy. The top two sketches emphasize the superior orbicularis contracture of the forehead, whereas the lower sketches capture the activation of the palpebral portion of the orbicularis (muscle of reflection, left) and the combined activation of the frontalis superior portion of the orbicularis and platysma, giving a frightened expression (right) (4).

Fig. 5. Four pictures drawn by Charcot of a patient with likely progressive supranuclear palsy. The top two sketches emphasize the superior orbicularis contracture of the forehead, whereas the lower sketches capture the activation of the palpebral portion of the orbicularis (muscle of reflection, left) and the combined activation of the frontalis superior portion of the orbicularis and platysma, giving a frightened expression (right) (4).

writings. In this context, Larner proposed that Charles Dickens captured the essential features of progressive supranuclear palsy in his description of a character in The Lazy Tour of Two Idle Apprentices (21). Dickens wrote:

A chilled, slow, earthy, fixed old man. A cadaverous man of measured speech. An old man who seemed as unable to wink, as if his eyelids had been nailed to his forehead. An old man whose eyes—two spots of fire—had no more motion than if they had been connected with the back of his skull by screws driven through it and riveted and bolted outside, among his grey hair. He had come in and shut the door, and he now sat down. He did not bend himself to sit, as other people do, but seemed to sink bold upright, as if in water until the chair stopped him (22).

For the medical reader with a knowledge of progressive supranuclear palsy, this description provides images compatible with the medical diagnosis. On the other hand, the passage falls short of the more convincing descriptions of sleep apnea in the Pickwick Papers or torticollis in Little Dorrit.

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