A long-term prospective study of optic neuritis: evaluation of risk factors.

Eighty-six patients with monosymptomatic optic neuritis of unknown cause were followed prospectively for a median period of 12.9 years. At onset, cerebrospinal fluid (CSF) pleocytosis was present in 46 patients (53%) but oligoclonal immunoglobulin in only 40 (47%) of the patients. The human leukocyte antigen (HLA)-DR2 was present in 45 (52%). Clinically definite multiple sclerosis (MS) was established in 33 patients. Actuarial analysis showed that the cumulative probability of developing MS within 15 years was 45%. Three risk factors were identified: low age and abnormal CSF at onset, and early recurrence of optic neuritis. Female gender, onset in the winter season, and the presence of HLA-DR2 antigen increased the risk for MS, but not significantly. Magnetic resonance imaging detected bilateral discrete white matter lesions, similar to those in MS, in 11 of 25 patients, 7 to 18 years after the isolated attack of optic neuritis. Nine were among the 13 with abnormal CSF and only 2 belonged to the group of 12 with normal CSF (p = 0.01). Normal CSF at the onset of optic neuritis conferred better prognosis but did not preclude the development of MS.

A serious ovarian cystadenocarcinoma metastatic to both eyes.

A patient with a stage IV ovarian cystadenocarcinoma is described. She seems to constitute the first reported case of this kind of tumour metastasizing to the eyes.

Gliomas of the anterior visual pathway in children. Tumour behaviour and effect of treatment.

An analysis of 16 cases of optic glioma in childhood is presented. All tumours were astrocytomas of the juvenile pilocytic of type low malignancy. In 7 of 13 patients with involvement of the chiasm there was a significant progression of tumour during the follow-up period, including 4 cases with fatal outcome. Patients under 5 years of age had a more unfavourable course than older children, but at the same time they had received lower doses of radiotherapy. The 5 patients treated with an absorbed dose of 39 Gy or more are alive without signs of progression 7-20 years after treatment. It is concluded from this material and from a survey of literature that many cases of optic glioma in childhood do not have a benign course. Guidelines for treatment are proposed.

Correlation of visual evoked potentials, ophthalmological and neurological findings after unilateral optic neuritis.

Forty-two patients were tested with pattern reversal visual evoked potentials (VEPs) 1--8 years after an episode of acute unilateral optic neuritis. The stimulation was produced by dot as well as checker-board pattern. An unexpectedly large proportion (12 cases) showed normal VEP latencies from the affected eye. In these patients the initial visual acuity was on the average significantly better than in cases with prolonged VEP latencies. They also had significantly fewer remaining ocular signs than the patients with abnormal VEPs. On the other hand, no correlation was found between the VEP findings and the incidence of CSF abnormalities, multiple sclerosis and HLA types. In 14 patients of which 13 belonged to the group above, repeated VEP tests were performed from the acute onset of the disease and up to 2 years afterwards confirming that a considerable normalization of VEP latencies occurs.

Automatic computerized perimetry in the detection of neurological visual field defects. A pilot study.

Forty-seven eyes from 25 patients with neurological disease were examined with an automatic computerized perimeter primarily intended for glaucoma. Manual kinetic and static perimetry with Goldmann's instrument was used for comparison. In 32 fields, the automatic method disclosed all defects that had been found by manual perimetry. Furthermore, it detected three small defects that had been missed in the initial manual examination. The remaining 12 fields were normally using both methods. Consequently, in detecting field defects, the automatic perimeter was superior to the manual method.

Diagnostic value of visual evoked response, clinical eye examination and CSF analysis in chronic myelopathy.

The results of neuro-ophthalmological examination, visual evoked responses (VER) to pattern reversal stimulation and cerebrospinal fluid (CSF) analysis were compared in 25 patients with myelopathy of unknown etiology and without subjective symptoms of involvement of CNS outside the spinal cord. Delayed latencies of VER indicating a disseminated disease were found in 76 per cent of the patients. In 64 per cent of the patients, CSF showed abnormalities similar to those found in MS. Pathological findings at the clinical eye examination consistent with such an etiology was found in 36 per cent of the cases. It is suggested that a large proportion of patients with myelopathy of unknown etiology suffer from a disseminated demyelinating disorder similar to MS. By a combination of neuro-ophthalmological, VER and CSF examinations such as etiology can be established with reasonable certainty, and more troublesome investigations such as myelographies and spinal angiographies may be restricted to patients in whom the etiology still remains unclear.

Aspects on the treatment of gliomas of the anterior visual pathway.

Partial excision of the glioma of the anterior visual pathway seemed to have no effect on the vision in 7 patients and had a negative effect in 2. These results support the opinion that no excision should be made in such tumours. However, the course of the disease in one exceptional patient raises doubts about the general validity of this rule. In this case the postulated chiasmal glioma proved to be an exophytic glioma arising in one intracranial optic nerve and compressing the other. Excision of the exophytic portion caused rapid and considerable improvement of vision in both eyes. Direct visualization by craniotomy was needed to separate this mode of growth from the common intrinsic growth of such tumours.

A portable hemianopsia tester.

A pocket-size static perimeter provided with four test lights, one for each quadrant of the visual field, has been constructed and applied to 190 visual fields in 97 patients. The main advantage of the instrument is its ability to disclose hemianopic defects in bedridden and certain sick patients unable to co-operate with other methods, including the confromtation test. This was performed in 12 fields in 7 patients.