2. What is the nature of stiff pupils? A pupil is called stiff in the sense of the Argyll-Robertson pupil if it fails to react to illumination either of itself or of the other eye and at the same time if it reacts properly in convergence and accommodation. Of course the stiffness of a blind eye must not be regarded as an Argyll-Robertson pupil. In a case of right-sided Argyll-Robertson pupil, therefore, the left pupil reacts properly both to direct illumination of itself and to illumination of the right eye, but the right eye fails to react to illumination of either eye. Such an Argyll-Robertson right pupil will remain of the same width both in darkness and in light. Clinicians agree that the Argyll-Robertson is diagnosticated rather too frequently than too seldom, and this by reason of the fact that a sluggishness of light reaction is interpreted as stiffness. The sign, as is well known, has come to be regarded as almost pathognomonic of tabetic or paretic neurosyphilis. Nonne, however, has found among 510 cases of alcoholism, nine instances of Argyll-Robertson pupil and 19 cases of sluggish light reactions. The pathological anatomy of this sign is still doubtful although a number of schematic accounts are available; among hypotheses, one may think of an elective effect of the tabetic or paretic degeneration upon reflex collaterals. The explanation would then resemble that for absent knee-jerks and kindred reflex disorders. We should then hypothesize a loss of the finer processes of the terminal arborizations about the cells of the nucleus of sphincter nucleus iridis. However, the situation of the sphincter iridis has not yet been absolutely determined.
When a pupil is said to be entirely stiff it means that it reacts neither to light nor accommodation. This condition not infrequently follows the partial stiffness or Argyll-Robertson reaction.
3. Is the Argyll-Robertson pupil more tabetic than paretic? This has been claimed at times, but in point of fact, the Argyll-Robertson pupil is very frequent in paresis, and so also are posterior column changes. According to statistics of Bumke, 36% of tabetics fail to show the Argyll-Robertson pupil, and 38% of paretics. When, however, finer methods, such as those standardized by Weiler, with photographic records, are employed, the number of cases without at least a tendency to the Argyll-Robertson pupil becomes much smaller.
In connection with the important question as to the classical Argyll-Robertson pupil and pupillary sluggishness to light, it may be inquired what are the ocular signs in neurosyphilis? Joffroy has tabulated the signs in 300 general paretics as follows:
| Sign. | No. of cases. | Per cent. |
|---|---|---|
| Alterations of light reflex | 235 | 78 |
| Inequality | 205 | 68 |
| Abolition of light reflex (bilateral or unilateral) | 156 | 52 |
| Abolition of light reflex (bilateral) | 133 | 44 |
| Irregularity of pupil | 117 | 39 |
| Irregularity of both pupils | 109 | 36 |
| Diminution of light reflex | 108 | 36 |
| ditto (bilateral) | 79 | 26 |
| Alteration in accommodation reflex | 79 | 26 |
| Diminution of accommodation reflex | 52 | 17 |
| Mydriasis | 41 | 13 |
| Myosis | 40 | 13 |
| Diminution of light reflex (unilateral) | 35 | 11 |
| Abolition of accommodation reflex | 35 | 11 |
| Diminution of accommodation reflex (bilateral) | 29 | 9 |
| Abolition of accommodation reflex (bilateral) | 26 | 8 |
| Diminution of accommodation reflex (unilateral) | 23 | 7 |
| Fundus changes | 21 | 7 |
| Vascular changes | 16 | 5 |
| Abolition of accommodation reflex (unilateral) | 12 | 4 |
| Paresis of the third nerves | 10 | 3 |
| Ptosis | 9 | 3 |
| Irregularity of one pupil | 8 | 3 |
| Nystagmus | 7 | 2 |
| Visual acuity lost | 7 | 2 |
| Atrophy of disc | 6 | 2 |
| Total blindness | 5 | 2 |
| Paralysis of the fourth nerves | 1 | 1 |
Can neurosyphilis exist in the absence of positive findings in the spinal fluid?
Case 56. There was no great difficulty in setting up a diagnosis of general paresis in the case of James Burns, a mechanic of 31 years of age, who came voluntarily to the Psychopathic Hospital for treatment. The point in Burns’ case was that the spinal fluid proved entirely negative in all respects despite the fact that the serum W. R. was positive, and despite the following facts of history and mental examination.
The patient claimed syphilitic infection seven years before, namely, at 24 years of age, and also claimed that he had infected his wife, who was in fact at the time undergoing antisyphilitic treatment. He complained of insomnia, worry, depression, hypersensitivity to noises (such as those made by his own children), thoughts of suicide, and amnesia. The amnesia, however, might be regarded as subjective since our tests failed to show amnesia. Nor was there any diminution in arithmetical ability. Despite the patient’s claim that he had been “way off in his way of thinking,” there appeared to be no delusions. Beyond a certain flightiness in conversation, we could hardly get any evidence of psychosis unless of the neurasthenic order.
Physically, however, the left pupil failed to react to light though it was found to react to distance, and the right pupil exhibited a diminution of its reaction to light. There was no ataxia of gait, yet there was a complete Romberg reaction. There was a moderate tremor of the hands and of the tongue. Otherwise there were no reflex disorders upon systematic examination, nor was there any demonstrable disorder in the rest of the physical examination.
1. What is the diagnosis in the case of James Burns? On the whole we agree with Nonne, that negative spinal fluid findings (of course, in the absence of treatment) preclude the diagnosis of general paresis. The symptoms might possibly be explained, however, by means of a localized syphilitic involvement of the cerebrum, no cells or products of inflammation having penetrated to the spinal fluid. According to Head and Fearnsides, this condition may be found especially in the anterior or middle fossa. Accordingly, going upon these views of Nonne and of Head and Fearnsides, we should be entitled to make, perhaps, a diagnosis of cerebral syphilis.