The detection of optic neuritis is of the greatest importance in the diagnosis of cerebral affections. Nevertheless, in certain cases of double optic neuritis with headache considerable caution is necessary before coming to a conclusion as to the exact nature of the disease. Though these two symptoms are present in the majority of cases of brain tumor and are so frequently due to this cause they are also met with in other diseases. In some cases of granular kidney, for example, the patient comes under treatment for headache and failure of vision; and ophthalmoscopic examination may reveal intense optic neuritis like that of cerebral tumor (neuritic form of albuminuric retinitis). At first the symptoms appear to indicate cerebral tumor, but a careful examination of the urine and cardio-vascular system will clearly reveal the cause. Limited space does not permit an enumeration of all the causes of double optic neuritis with headache. The results of the examination of one hundred cases presenting these two symptoms reveal, however, several points of interest. Most of these cases have been seen by us conjointly; some were seen separately, whilst others (in Groups I. to VIII.) were examined by one of us (R. T. W.) whilst holding the post of medical registrar at the Manchester Royal Infirmary. For permission to include the latter amongst our cases we are indebted to the medical board of that hospital.
With respect to the diagnosis and termination these one hundred cases may be grouped as follows:
I. Brain tumor, verified by necropsy, 27.
II. Cases terminating fatally; probably, majority due to brain tumor; but no necropsy obtained, 27.
III. General symptoms of brain tumor; but necropsy revealed distention of the ventricles of the brain with fluid; no tumor (serous meningitis of ventricles), 2.
IV. Cerebral abscess (fatal), 3.
V. Tuberculous meningitis (fatal), 2.
VI. Chronic interstitial nephritis; neuritic form of albuminuric retinitis (fatal), 3.
VII. Toxic conditions and blood diseases: Chronic lead poisoning, 3. Ulcerative endocarditis, (fatal), 1. Purpura hemorrhagica (fatal), 1. Henoch’s purpura (fatal), 1. Chlorosis with cerebral symptoms (recovery), 3.
VIII. Headache and double optic neuritis (without localizing symptoms); probably syphilitic (recovery, with blindness, 2; with impaired vision, 4), 6.
IX. Headache and double optic neuritis (without localizing symptoms); no evidence of syphilis; duration six and two and a quarter years, respectively. Termination still uncertain, 2.
X. Headache and double optic neuritis (without localizing symptoms); no evidence of syphilis; recovery with blindness, 8; with impaired vision, 3; with good vision, 8, 19.
The following are brief abstracts of the notes of the cases in Group X. which have come under our observation and which we have followed for a considerable period of time. The number of years during which each case has been followed is given in parentheses after the brief note.
1. A boy, aged ten years. Double optic neuritis, headache, and vomiting; slight internal strabismus of the left eye. Recovery with normal vision. (Seven years.)
2. A young woman, aged seventeen years. Headache, vomiting, and double optic neuritis. Recovery, but with impaired vision in one eye and blindness in the other. (Five and a half years.)
3. A young woman, aged eighteen years. Double optic neuritis, headache, and vomiting; several epileptic fits. Recovery, with useful vision in one eye; vision in the other is very defective. (Seven years.)
4. A young woman, aged eighteen years. Double optic neuritis, headache, and vomiting. Recovery, but complete blindness followed. (Four years.)
5. A young woman, aged nineteen years. Double optic neuritis, headache, and vomiting. Recovery with good vision. (Three years.)
6. A man, aged twenty years. Double optic neuritis, headache, and vomiting. Recovery, but complete blindness followed. (Two and a half years.)
7. A girl, aged ten years. Double optic neuritis, headache, and vomiting; knee-jerks were absent. Recovery, but complete blindness followed. (Three years.)
8. A boy at the age of thirteen years had double optic neuritis and headache; recovery ensued. At the age of fifteen years he had a return of headache and double optic neuritis; also vomiting. At a later date there was partial anæsthesia in the distribution of the right fifth cranial nerve; the right cornea was opaque; there was complete blindness in both eyes. Partial anæsthesia of the face and blindness remained, but otherwise the patient recovered and felt quite well nine months after the second attack.
9. A girl, aged sixteen years. Double optic neuritis, headache, vomiting; slight internal strabismus of the right eye. Recovery with normal vision. (Four and a half years.)
10. A boy, aged ten years. Double optic neuritis, headache, and vomiting. The head had increased in size. Recovery, but with complete blindness. (Three years.)
11. A woman, aged nineteen years. Double optic neuritis, headache, and vomiting. Recovery with normal vision. (Three years.)
12. A boy, aged fifteen years. Double optic neuritis, headache, and vomiting. Recovery. (Two and a half years.)
13. A woman, aged twenty-one years. Double optic neuritis, much swelling of the disks, headache, and vomiting. Complete recovery with normal vision. (Four and three-quarter years.)
14. A girl, aged fifteen years. Double optic neuritis, headache, and vomiting. Recovery with good vision. (Five and a quarter years.)
15. A boy, aged twelve years. Double optic neuritis, headache, and vomiting; slight internal strabismus of the right eye. Recovery with good vision. (Four years.)
16. A man, aged forty years. Double optic neuritis, headache, and vomiting. Recovery, but with complete blindness. (Eighteen months.)
17. A youth, aged seventeen years. Double optic neuritis, headache, and vomiting. Recovery, but with total blindness. (Five years.)
18. A woman, aged twenty-two years. Double optic neuritis, headache, and vomiting. Recovery, but with total blindness. (Two and three-quarter years.)
19. A girl, aged fourteen years. Double optic neuritis, headache, and vomiting; internal strabismus (left) for fourteen days. Recovery, with normal vision. (Two years.)
The following are brief notes of the cases in Group IX.:
20. A girl, aged twelve years. Headache, vomiting, and double optic neuritis in December, 1893. Recovery in twelve months, but vision was much impaired. She remained well with the exception of occasional headache until December, 1899. Then the severe headache returned. She became ataxic and optic neuritis reappeared. In April, 1900, the headache was much less and the patient felt much better, but she was completely blind. (Six and a half years.)
21. A young woman, aged seventeen years. Headache, vomiting, and double optic neuritis. Vision was impaired. Vomiting ceased; the headache continued for over two years, but recently disappeared after lumbar puncture. (Two and a quarter years.)
In all cases of double optic neuritis a systematic and careful examination of the patients should be made. The urine and cardio-vascular system should be examined for signs of chronic interstitial nephritis; the gums should be examined for the lead line and other indications of lead poisoning should be sought for; the question of chlorosis or other “blood disease” should be considered; and the ears should be examined for signs of otitis. But when all these conditions have been excluded and when the symptoms are apparently due to a cerebral affection, there is one group of cases in which localizing brain symptoms are absent and in which the chief indications of disease are headache, double optic neuritis, and often vomiting. In most of these cases syphilis can be also excluded. A diagnosis of brain tumor is given, and the growth is thought to be situated in some region in which the localizing symptoms are at first indefinite—cerebellum, temporo-sphenoidal lobe, or prefrontal region. Such a diagnosis often proves to be correct. Localizing symptoms may develop later and a necropsy may show the accuracy of the opinion expressed. But sometimes, to the surprise of the medical man, a fatal termination does not occur; the symptoms sometimes disappear and the patient recovers, though very often impairment or loss of vision remains. The patient may continue in good health for years or for a lifetime afterwards. Most medical men who have paid much attention to cerebral diseases will have met with a case or cases of this kind. The chief object of our article is to call attention to this class of cases and to indicate the frequency of their occurrence. Nineteen out of one hundred cases of double optic neuritis with headache in the table just given could (after careful examination) be placed in this group (X.).
What is the cause of the symptoms in this group of cases? Possibly in some cases the symptoms are caused by a non-malignant tumor (or tuberculous mass) which ceases to extend and becomes quiescent and encapsuled. One of us has recorded a case in which symptoms of cerebral tumor (including Jacksonian epilepsy and hemiplegia) gradually subsided and temporary recovery ensued; but three years later symptoms of cerebellar tumor developed and death occurred. The necropsy revealed a recent large tuberculous mass in the cerebellum and an old capsuled tuberculous mass just beneath the motor cortex in the right cerebral hemisphere. The latter had evidently been the cause of the early cerebral symptoms from which the patient had recovered. An instructive case has been recorded by Dr. T. K. Monro of Glasgow. The patient, at the age of sixteen years, suffered from severe headache with failure of vision which passed on to complete blindness. For thirty-three years he was an inmate of a blind asylum, ophthalmoscopic examination showing double optic atrophy. He died at the age of sixty-three years, from cancer of the stomach, and the post-mortem examination also revealed a large myxomatous tumor in the left half of the cerebellum. In all probability the early cerebral symptoms had been associated with optic neuritis which had passed on to optic atrophy and the cause had been the myxoma in the cerebellum which had remained quiescent for forty-six years.
In some cases of double optic neuritis with headache and general cerebral symptoms, when recovery occurs the cause is probably distention of the ventricles of the brain with fluid—serous meningitis of the ventricles (Quincke). This condition was present at the necropsy, and no tumor growth could be found in two out of the one hundred cases tabulated. It is probable that a number of the cases in which a diagnosis of cerebral tumor has been made, but in which recovery has occurred, have been due to this condition—serous meningitis of the ventricles. Probably the two cases in Group IX. and possibly some of the cases in Group II., in which death did not occur for several years after the onset of symptoms, were of this nature. Other cases which recover may be due to a basal meningitis.
The table given above is instructive both as regards the diagnosis and prognosis in cases of double optic neuritis with headache. It shows the necessity for careful examination before giving either a diagnosis or prognosis, and the clinical group of cases No. X. ought always to be borne in mind whenever the diagnosis is obscure and localizing symptoms are absent.
There are two other points to which we would draw attention. In ten out of the one hundred cases the patient recovered completely from the headache and general cerebral symptoms and regained perfect health, but the optic neuritis was followed by atrophy and complete blindness. In the face of this terrible termination we cannot help thinking that simple trephining of the skull and the removal of bone, without any interference with the brain, as suggested and practiced by Mr. Victor Horsley for the relief of optic neuritis and pressure symptoms, is a method of treatment worthy of more frequent trial when vision is failing markedly. Dr. James Taylor has published cases which appear to show that this method of treatment may be of service in checking the optic neuritis and failure of vision. In the class of cases in Group X. if there should be a suspicion that the symptoms may be due to serous meningitis of the ventricles, lumbar puncture appears to be worthy of trial, since several cerebral cases are now on record in which this treatment appears to have been of great service, and in which the cause of the cerebral symptoms was probably that just mentioned.