Sigmoid Sinus Diverticulum, Dehiscence, and Venous Sinus Stenosis: Potential Causes of Pulsatile Tinnitus in Patients with Idiopathic Intracranial Hypertension?

Background And Purpose: Pulsatile tinnitus is experienced by most patients with idiopathic intracranial hypertension. The pathophysiology remains uncertain; however, transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence have been proposed as potential etiologies. We aimed to determine whether the prevalence of transverse sinus stenosis and sigmoid sinus diverticulum/dehiscence was increased in patients with idiopathic intracranial hypertension and pulsatile tinnitus relative to those without pulsatile tinnitus and a control group.

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.

Subacute peripheral and optic neuropathy syndrome with no evidence of a toxic or nutritional cause.

Background: The syndrome of subacute simultaneous peripheral neuropathy and bilateral optic neuropathy is known to occur in tropical countries, probably due to malnutrition or toxicity, but not often seen in developed countries. We report seven patients in London who were not malnourished or alcoholic, and in whom no clear cause was found.

Methods: We retrospectively reviewed the case notes and arranged some further investigations.

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.

Temozolomide in the management of dopamine agonist-resistant prolactinomas.

Background: The majority of prolactinomas respond to dopamine agonist therapy, but a proportion are resistant, requiring other treatments including surgery and/or radiotherapy. Temozolomide is an oral chemotherapy agent, which has been used as a salvage therapy to treat aggressive pituitary adenomas and carcinomas, including prolactinomas, unresponsive to all conventional treatment.

Case Series: We report three patients where temozolomide was used in the treatment of refractory prolactinomas.

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.

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.

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.

Clinical assessment of optic nerve disorders.

Optic nerve disorders range from the manifestations of life-threatening intracranial or systemic disease, to minor congenital anomalies. Careful clinical assessment, which relies upon a thorough evaluation of symptoms as well as signs, is essential for effective and timely investigation and treatment. Examination methods and pitfalls to be avoided are discussed.

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.

EFNS task force--therapy of nystagmus and oscillopsia.

An overview of possible treatment options for oculomotor disorders that prevent clear vision is given. Downbeat nystagmus, upbeat nystagmus, seesaw nystagmus, periodic alternating nystagmus, acquired pendular nystagmus, and saccadic oscillations such as opsoclonus/ocular flutter are discussed. In addition, superior oblique myokymia and vestibular paroxysmia are reviewed.

Pupil perimetry in the diagnosis of functional visual field loss.

The diagnosis of functional visual loss-reduced visual performance in the absence of an organic cause-is usually made by exclusion. We conducted a pilot study to evaluate pupil perimetry in three patients (aged 14, 43 and 50) with visual field loss presumed to be functional on clinical grounds and having no cause identified by visual electrophysiology or magnetic resonance imaging. A modified automated perimeter was used to examine visual and pupil responses to a light stimulus (size 1.

Recognizing periodic alternating nystagmus.

Congenital or acquired periodic alternating nystagmus (PAN) is characterized by nystagmus occurring in a cycle. The cycle consists of a left-beating nystagmus, a transition phase, a right-beating nystagmus, and a further transition phase. The purpose of this review is to assist the clinician in the recognition of periodic alternating nystagmus (PAN), either as a type of congenital nystagmus or in its acquired form, and to highlight why such identification is important.