Perioptic Cerebrospinal Fluid Dynamics in Idiopathic Intracranial Hypertension

Purpose: To examine the cerebrospinal fluid (CSF) dynamics along the entire optic nerve in patients with idiopathic intracranial hypertension (IIH) and papilledema by computed tomographic (CT) cisternography.Methods: Retrospective analysis of CT cisternographies in 16 patients with a history of IIH and papilledema (14 females and 2 males, mean age: 49 ± 16 years). Contrast loaded CSF (CLCSF) was measured in Hounsfield Units (HU) at three defined regions of interest (ROI) along the optic nerve (orbital optic nerve portion: bulbar and mid-orbital segment, intracranial optic nerve portion) and additionally in the basal cistern. The density measurements in ROI 1, ROI 2, and ROI 3 consist of measurements of the optic nerve complex: optic nerve sheath, CLCSF filled SAS and optic nerve tissue. As controls served a group of patients (mean age: 60 ± 19 years) without elevated intracranial pressure and without papilledema.Results: In IIH patients the mean CLCSF density in the bulbar segment measured 65 ± 53 HU on the right and 63 ± 35 HU on the left side, in the mid-orbital segment 68 ± 37 HU right and 60 ± 21 HU left. In the intracranial optic nerve portion 303 ± 137 HU right and 323 ± 169 HU left and in the basal cistern 623 ± 188 HU. Within the optic nerve the difference of CLCSF density showed a highly statistical difference (p < 0.001) between the intracranial optic nerve portion and the mid-orbital segment. CLCSF density was statistically significantly (p < 0.001) reduced in both intraorbital optic nerve segments in patients with IIH compared to controls.Conclusions: The current study demonstrates reduced CLCSF density within the orbital optic nerve segments in patients with IIH and papilledema compared to 12 controls without elevated intracranial pressure and without papilledema. Impaired CSF dynamics could be involved in the pathophysiology of optic nerve damage in PE in IIH

Meningothelial Cells React to Elevated Pressure and Oxidative Stress

BACKGROUND: Meningothelial cells (MECs) are the cellular components of the meninges enveloping the brain. Although MECs are not fully understood, several functions of these cells have been described. The presence of desmosomes and tight junctions between MECs hints towards a barrier function protecting the brain. In addition, MECs perform endocytosis and, by the secretion of cytokines, are involved in immunological processes in the brain. However, little is known about the influence of pathological conditions on MEC function; e.g., during diseases associated with elevated intracranial pressure, hypoxia or increased oxidative stress. METHODS: We studied the effect of elevated pressure, hypoxia, and oxidative stress on immortalized human as well as primary porcine MECs. We used MTS (3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium) bioreduction assays to assess the proliferation of MECs in response to treatment and compared to untreated control cells. To assess endocytotic activity, the uptake of fluorescently labeled latex beads was analyzed by fluorescence microscopy. RESULTS: We found that exposure of MECs to elevated pressure caused significant cellular proliferation and a dramatic decrease in endocytotic activity. In addition, mild oxidative stress severely inhibited endocytosis. CONCLUSION: Elevated pressure and oxidative stress impact MEC physiology and might therefore influence the microenvironment of the subarachnoid space and thus the cerebrospinal fluid within this compartment with potential negative impact on neuronal function

'Spontaneous' periocular hemorrhage and macrohematuria

PURPOSE: To report on a 19-year-old man with 'spontaneous' periocular hemorrhage and macrohematuria as the first signs of a bone-marrow-infiltrating rhabdomyosarcoma of the left superior rectus muscle. PROCEDURES: Clinical eye examination, magnetic resonance imaging, laboratory workup and bone marrow biopsy were performed. RESULTS: Magnetic resonance imaging showed a mass lesion in the left superior rectus muscle. While funduscopy had initially been normal, optic disc swelling, retinal hemorrhage and vitreous cell infiltration could be seen in the further course of disease. Laboratory work-up showed macrohematuria, thrombocytopenia and decreased concentration levels of plasma fibrinogen. Thromboplastin time was decreased. A bone marrow biopsy revealed small cell infiltration; using immunohistochemistry, a rhabdomyosarcoma was diagnosed. Unfortunately, the patient died few months later, in spite of chemotherapy. CONCLUSION: 'Spontaneous' periocular hemorrhage and macrohematuria were the first signs of a bone-marrow-infiltrating rhabdomyosarcoma in this young and otherwise healthy patient

Bilateral Papilledema Without Elevated Cranial Pressure: A New Clinical Syndrome?

Papilledema is defined as swelling of the optic nerve head due to axon swelling caused by elevated intracranial pressure. Spontaneous venous pulsation (SVP) as a rule are absent. We report the case of a 72 year old women who was referred with papilledema

Cerebrospinal Fluid Composition: a Clue to Optic Nerve Compartment Syndrome

Unlike other cranial nerves, the optic nerve (ON) is covered by the meninges and surrounded by cerebrospinal fluid (CSF). Its cul-de-sac anatomy, with unidirectional CSF influx from the chiasmal cistern into the subarachnoid space (SAS), renders its axons vulnerable to damage from elevated pressure or toxic substances

Posterior Ischemic Optic Neuropathy Treated with Optic Nerve Sheath Decompression; Report of One Case

Posterior ischemic optic neuropathy is a diagnosis of exclusion. We report one such case of a 61 year old patient who experienced sudden painless loss of vision in his right eye

New Onset Asymmetric Bilateral Visual Field Defect After Sequential Retrobulbar Neuritis in a Patient with Multiple Sclerosis

A 36 year old female patient presented with a sensomotor hemisymptomatic deficiendy and was hospitalized for work up and treatment. Magnetic resonance imaging (MRI) of the brain revealed multiple white matter lesions, compatible with the diagnosis of demyelinating disease such as multiple sclerosis

Bilateral Nonarteritic Anterior Ischemic Optic Neuropathy and Autoimmune Thrombocytopenia. What is the Role of the Thrombocytes?

A 68 year old man presented with bilateral nonarteritic anterior ischemic optic neuropathy (NAION). Except for a very low platelet count of 15'000 the laboratory findings were within normal range. Treatment with steroids was started to improve the platelet count

Ptosis, Diplopia and Hepatitis Presenting Signs and Symptoms in Biopsy Proven Giant Cell Arteritis

Giant cell arteritis (GCA) presents either as localized or systemic vasculitis and is found typically in individuals older than 50 years. It is often associated with polymyalgia. GCA involves frequently the temporal artery and may be bilateral