Medically unexplained visual loss in a specialist clinic: a retrospective case-control comparison.

PURPOSE: To compare the clinical and demographic characteristics of adult patients with nonorganic or medically unexplained visual loss (MUVL) to those with other common conditions presenting to a neuro-ophthalmology clinic. METHODS: Case-control design: a retrospective review of medical notes on a consecutive case series of 49 patients assessed at the King's College Hospital neuro-ophthalmology clinic with unexplained visual loss and matched with the next assessed patient identified from clinic records. Patients presented post-symptom onset with a mean clinical course of 30 months (SD=67 months) and standard clinical examination used to confirm diagnoses, alongside ancillary investigations if required. RESULTS: Seventy-two percent (n=36) of MUVL patients were female. In comparison with patients with organic visual disorders, MUVL cases presented with significantly higher rates of bilateral (cf. unilateral) visual impairment (41%, n=20), premorbid psychiatric (27%, n=13) as well as functional (24%, n=12) diagnoses and psychotropic medication usage (22%, n=11). Medically unexplained cases were significantly more likely to report preceding psychological stress (n=9; 18%). CONCLUSIONS: Medically unexplained visual impairment may be regarded as part of the spectrum of medically unexplained disorders seen in the general hospital setting. Research is needed to determine long-term outcomes and effective tailored interventions.

Sheehan syndrome associated with raised intracranial pressure.

UNLABELLED: Intracranial hypertension (IH) has been associated with hypocortisolism caused by either primary adrenocortical insufficiency or corticosteroid withdrawal. METHOD: The authors describe a case of IH in association with Sheehan syndrome (SS) in a postpartum 29-year-old woman. RESULTS: The clinical manifestations of IH resolved with corticosteroid replacement. CONCLUSIONS: This case supports a causal role of hypocortisolism in IH. The authors are unaware of previous reports of hypocortisolism caused by SS leading to IH.

Canomad presenting without ophthalmoplegia and responding to intravenous immunoglobulin.

The acronym CANOMAD encompasses chronic ataxic neuropathy combined with ophthalmoplegia, M protein, cold agglutinins, and anti-disialosyl antibodies.Herein we describe 2 patients presenting with progressive ataxic neuropathy who only developed ophthalmoplegia after a significant delay post-presentation, which in 1 case had features indicative of brainstem dysfunction. Both patients were found to have an IgM paraprotein and anti-disialosyl antibodies. They responded to treatment with intravenous immunoglobulin, thus illustrating the importance of diagnosing this condition.

Idiopathic intracranial hypertension: the association between weight loss and the requirement for systemic treatment.

BACKGROUND: To determine whether weight loss is significantly associated with a discontinuation of treatment for idiopathic intracranial hypertension METHODS: The notes of 36 patients with idiopathic intracranial hypertension under regular review for at least 12 months by a single neuro-ophthalmologist were retrospectively reviewed. Weight was recorded at each assessment and weight loss recommended. Treatment was adjusted according to symptoms, visual function including visual fields and optic disc appearance only. Patients were divided according to duration of continuous follow-up, and then sub-divided as to whether they were on or not on treatment at most recent review and whether weight loss had been achieved compared to presentation. Survival analysis was performed to assess the probability of remaining on treatment having lost weight. RESULTS: Considering the patients as 3 groups, those with at least 12 months follow-up (n = 36), those with at least 18 months follow-up (n = 24) and those with 24 months or more follow-up (n = 19), only the group with 24 months or more follow-up demonstrated a significant association between weight loss and stopping systemic treatment (Fisher's exact test, p = 0.04). Survival analysis demonstrated that the probability of being on treatment at 5 years having gained weight was 0.63 and having lost weight was 0.38 (log rank test, p = 0.04). The results suggest that final absolute body mass index is more important than the change in body mass index for patients who stop treatment (Mann Whitney U, p = 0.05). CONCLUSION: This is the first study to demonstrate that weight loss is associated with discontinuation of treatment. Unlike previous studies, our results suggest that final absolute body mass index is more important for stopping treatment than a proportional reduction in weight.

Raised intracranial pressure complicating cryptococcal meningitis: immune reconstitution inflammatory syndrome or recurrent cryptococcal disease?

We report the case of a patient with advanced HIV disease and cryptococcal meningitis, who after an initially good clinical and mycological response to systemic anti-fungal treatment developed symptomatic raised intracranial pressure 10 days after initiation of highly active anti-retroviral therapy. We describe the subsequent clinical management and the features that suggest that this persistently raised ICP was more likely due to an immune reconstitution syndrome (IRIS) following HAART rather than relapse of cryptococcal disease or failure of anti-fungal therapy.

Persistent visual loss after retinochoroidal infarction in pregnancy-induced hypertension and disseminated intravascular coagulation.

A 40-year-old woman had pregnancy-induced hypertension, disseminated intravascular coagulation (DIC), choroidal infarction, and magnetic resonance imaging (MRI) high-signal abnormalities in the occipital regions. With successful treatment of the hypertension and spontaneous resolution of the DIC, the MRI signal abnormalities resolved, but visual acuity remained decreased because of damage to the retina and choroid. This case demonstrates that pregnancy-induced hypertension, particularly if combined with DIC, may produce infarction of the retina and choroid and persistent visual loss even if the effect of this condition on the occipital lobes is limited to reversible vasogenic edema.

Maximum angle of ocular duction during visual fixation as a function of age.

PURPOSE: To measure and compare the maximum angle of ocular duction in healthy individuals as a function of age. METHODS: A calibrated arc perimeter was modified to display one of six randomly presented targets (high contrast Snellen equivalent letters), in both vertical (supra/infraduction) and horizontal (ab/adduction) gaze to the dominant eye of 204 healthy volunteers with best-corrected visual acuity. A bite-bar and headrest were employed to prevent head movement. Using a modified method of limits for discrimination threshold, a maximum mean angle of ocular duction was determined by stepping a target out in 5 degrees steps until an error was reported and thereafter bracketing around the limits of the target identification in 1 degrees steps. A mean threshold value was determined as the angle at which a subject obtained a correct response 75% of the time in two and as many as the trails in each of the four randomly presented directions of gaze (abduction, adduction, supraduction and infraduction). RESULTS: A decrease in mean maximum duction angle was found over all age groups in al four directions (p < 0.001), with a step decline beginning in the sixth decade and almost doubling in the oldest age group tested (80-95 years-olds). The percent change in mean maximum angle of duction due to age from the 14-19 to the 80-95 year-olds was: abduction 21%, adduction 24%, supraduction 35%, infraduction 26%. CONCLUSION: Baseline data are useful to differentiate normal changes occurring with age from early signs of disease. AdditIonally, disease progression and effects of treatment can be monitored.

Bilateral orbital metastases from a neuroendocrine tumor.

When neuroendocrine tumors metastasize to the orbit, they usually do so as solitary lesions, sometimes involving an extraocular muscle. A 70-year-old woman with a known neuroendocrine tumor had bilateral painful proptosis, orbital soft tissue swelling, and ophthalmoplegia. Imaging showed masses within all the extraocular muscles. Orbital biopsy disclosed metastatic neuroendocrine tumor cells within the connective tissue.