I. M. Pepper

The incidence and prevalence of idiopathic intracranial hypertension in Sheffield, UK.

Background and purpose:  The purpose of this study was to identify the incidence and prevalence of idiopathic intracranial hypertension (IIH) in Sheffield, UK.

Early diagnosis of Horner syndrome using topical apraclonidine.

The diagnosis of Horner syndrome (HS) using apraclonidine eye drops is an alternative to the use of topical cocaine drops. A number of reports have described the efficacy of apraclonidine testing, but there is some debate over its sensitivity in the acute setting. We describe a patient with HS secondary to carotid dissection who had a positive response to apraclonidine 3 hours after the onset of symptoms. The case is made for a larger study of apraclonidine use to determine its true sensitivity and specificity, identify confounding factors, and redefine the criteria for positive testing.

Visual Outcomes from Shunting for Idiopathic Intracranial Hypertension

ABSTRACT A retrospective notes review was conducted for 50 consecutive patients who underwent shunt surgery for idiopathic intracranial hypertension (IIH). The decimal visual acuity and the mean radial degrees (MRD) of the I4e isopter of the Goldmann visual field were measured pre-operatively and after a mean follow-up period of 1123 days (range: 13–3551 days). A ventriculo-peritoneal shunt was the first procedure in 38 patients and a lumbo-peritoneal shunt in 12. The mean decimal visual acuity of the worse affected eye improved from 0.75 to 0.84, p = 0.011. The MRD score of the worse affected eye improved on average from 25.6° to 35.5°, p < 0.0001. In those with significant pre-operative visual impairment in their worse affected eye (defined as an MRD score ≤30°), the MRD score improved on average from 10.3° to 26.5°, p = 0.0008. The mean number of surgical procedures for each patient was 2.8 (range: 1–15). Taking all surgical procedures into account, post-operative complications were experienced by 30 patients. At last follow-up, 28 patients still complained of headache, 8 of whom had the intervention performed primarily for headache. Shunting can improve visual function in patients with IIH. There is significant post-operative morbidity and often the need for repeated procedures. Headache also commonly remains in these patients. There is a need for a randomised controlled trial of operative interventions in IIH. Sample size calculations for such a trial to treat significant vision loss are presented.

Decompensating esophoria as the presenting feature of myasthenia gravis

Myasthenia gravis is a disease in which antibodies directed at nicotinic acetylcholine receptors are produced, leading to a deficiency of acetylcholine receptors at the neuromuscular junction. This results in impairment of muscular excitation, which appears clinically as fatigable muscle weakness. Weakness of the extraocular muscles occurs in nearly 90% of all myasthenics at disease onset, with ptosis being the most common presenting feature. Myasthenia gravis affecting one or a combination of the extraocular muscles without ptosis is less common; however, cases such as bilateral internuclear ophthalmoplegia without ptosis have been described in the literature. The authors present a case in which decompensating esophoria was the presenting feature of myasthenia gravis.

The Evolution of an Optic Nerve Head Granuloma Due to Sarcoidosis

ABSTRACT Sarcoidosis can affect the optic nerves by means of optic disc oedema secondary to posterior uveitis, optic disc oedema secondary to raised intracranial pressure, optic neuritis, optic atrophy secondary to compression or infiltration from a primary central nervous system lesion, and primary granuloma of the optic nerve head. The authors report the use of optical coherence tomography in assessing the response to immunosuppression in a 57-year-old woman with an optic nerve head granuloma.

Neuro-ophthalmological Complications of Chronic Inflammatory Demyelinating Polyradiculoneuropathy

Abstract Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can lead to prominent nerve hypertrophy, which can mimic other forms of neuropathy radiologically. Neuro-ophthalmological complications can also occur in CIDP, either at presentation or chronically in the disorder. This can also cause diagnostic difficulties. We report three cases of neuro-ophthalmological complications of CIDP: two cases of papilloedema and one case of proptosis. In all three cases cranial nerve hypertrophy was present. CIDP should be considered in neuro-ophthalmological presentations associated with cranial/spinal nerve root hypertrophy.

Bilateral Homonymous Hemianopia with Sparing of the Vertical Meridian

A 19-year-old woman, who was 16 weeks post partum, collapsed with loss of consciousness. Following awakening she suffered abrupt loss of vision. Partial recovery occurred, although she has been left with bilateral homonymous hemianopia with sparing of vision just to the left of the vertical meridian. Magnetic resonance imaging demonstrated bilateral occipital infarcts, sparing the lips of the calcarine sulcus on the right. This is compatible with our expectation that the vertical meridian of the retinotopic map is represented at the superior and inferior lips of the calcarine sulcus.

A Stereotyped Syndrome with Retro-Ocular Pain, Photophobia, and Visual Disturbance Masquerading as Optic Neuritis: Case Series

ABSTRACT We describe here a case series of six patients referred to the Neuro-ophthalmology service in Sheffield, UK with possible acute unilateral optic neuritis. Each patient had a triad of unilateral photophobia, ipsilateral retro-ocular pain, and ipsilateral loss of vision. All patients had normal ocular examinations and investigation findings with no objective structural or functional abnormalities identified. Patients were treated by weaning-off regular analgesia and, where appropriate, commencing migraine prophylaxis. In the three patients with complete recovery of pain, there was also complete recovery of vision. We propose that this is a migraine syndrome and that the decreased visual acuity is a functional consequence of the pain and photophobia.

Do Optic Canal Dimensions Measured on CT Influence the Degree of Papilloedema and Visual Dysfunction in Idiopathic Intracranial Hypertension

ABSTRACT A recent study found that increased optic canal area on magnetic resonance imaging was associated with worse papilloedema in idiopathic intracranial hypertension (IIH). We repeated this study using more accurate computerized tomography derived measurements. Optic canal dimensions were measured from 42 IIH patients and 24 controls. These were compared with papilloedema grade. There was no correlation between any of the optic canal measurements and papilloedema grade and no significant difference in optic canal measurements between patients and controls. Our results cast doubt on the existing literature regarding the association between optic canal size and the degree of papilloedema in IIH. CT delineates bony anatomy more accurately than MRI and our CT-derived optic canal measurements cast doubt on the existing literature regarding the association between optic canal size and the degree of Papilloedema in IIH.

Relapsing-Remitting Sixth Nerve Palsy in Association with Ollier’s Disease

Abstract Relapsing-remitting sixth nerve palsy is usually due to ophthalmoplegic migraine (recurrent cranial nerve palsy) in younger patients and microvascular disease in older patients. There have been isolated reports, however, of it occurring in the presence of a skull base tumour. We report a 20-year-old woman with Ollier’s disease who presented with a relapsing-remitting sixth nerve palsy. Neuro-imaging revealed a skull base enchondroma.