Neuro-ophthalmologic outcomes of standard versus hypo-fractionated stereotactic radiotherapy of AVPM.

BACKGROUND: Most anterior visual pathway meningiomas (AVPM) are benign and slow-growing, but these tumors may affect visual functions, including visual acuity (VA) and visual field (VF). Due to location, most are treated non-surgically by fractionated stereotactic radiotherapy (FSRT), aiming to prevent tumor progression and visual functions deterioration. Unfortunately, FSRT in itself may affect visual functions. The current preferred treatment regimen (in terms of safety and effectiveness) is undetermined. While most cases are treated with conventional fractionation (cFSRT)-50.4-54 Gy in 28-30 fractions of 1.8-2 Gy, advances in technology have allowed shortening of total treatment length to hypofractionation (hSRT)-25-27 Gy in 3-5 fractions of 5-9 Gy. Our aim was to evaluate the association of radiotherapy regimen for treating AVPM (cFSRT vs. hSRT) with visual function outcomes (VA, VF) at the last neuro-ophthalmologic evaluation. METHODS: We conducted a retrospective cohort study of AVPM cases treated at Sheba Medical Center during 2004-2015. We compared cFSRT and hSRT regimens regarding visual function (VA, VF) outcomes at the last neuro-ophthalmologic evaluation. VA was determined by the logarithm of the minimum angle of resolution (LogMAR). VF was determined by the mean deviation (MD). A clinically relevant change in VA was defined as 0.2 LogMAR. RESULTS: 48 patients (13 receiving hSRT, 35 receiving cFSRT) were included, with a median follow-up of 55 months. No significant difference was evident regarding LogMAR or MD of involved eyes at the last evaluation. Six (17%) patients in the cFSRT group experienced clinically relevant VA deterioration in the involved eye, compared with six (46%) in hSRT (p = 0.06). CONCLUSION: Our findings, using comprehensive and meticulous investigation of visual outcomes, suggest that hSRT may be associated with higher risk for VA and VF deterioration in AVPM especially in ONSM. We recommend the use of cFSRT for ONSM.

[Cavernous hemangioma of anterior visual pathways--a rare cause of visual loss].

OBJECTIVE: To report cases of cavernous hemangioma (CH) of the anterior visual pathways (AVP) and analyze the cLinical and radiographic presentation, as well as the surgical approach of this very rare entity. METHODS: The authors report two cases of patients presenting with headache and visual loss due to anterior visual pathways hemangioma, and conducted a PubMed search for previously reported cases. Each case was analyzed for clinical presentation, imaging characteristics, location of the lesions, treatment methods and visual outcomes. RESULTS: Loss of vision was present in all reported cases, followed by headache and periorbital pain in 60% of the patients. The onset of symptoms was apoplectic in 66% of patients. Sixteen patients suffered from previous episodes of vision loss. MRI was the most sensitive and specific imaging modality, typically demonstrating heterogeneous lesion with mixed signals suggestive of blood of different ages. The gradient echo sequence is the most sensitive to hemorrhagic components. Most patients were surgically treated (93.2%). In 82% of cases visual stabilization or improvement was achieved. Two patients were treated conservatively resulting in complete blindness in one and spontaneous recovery in the other. The two patients reported by the authors were followed-up with spontaneous visual recovery. CONCLUSIONS: Vascular lesions of the AVP are very rare, but should always be considered in the differential diagnosis of acute painful visual loss. Resection of these lesions can be associated with favorable visual outcomes; therefore, rapid diagnosis is warranted to prevent permanent damage to visual pathways.

Multiple brain infarcts and Balint syndrome in aortic arch angiosarcoma.

A 50-year-old woman presented with subacute cognitive decline, impaired eye movements, and simultanagnosia, components of the Balint syndrome. She had relatively low blood pressure in the left arm and left finger clubbing. Brain imaging identified multiple acute infarcts. Transesophageal echocardiography showed no cardiac abnormalities but demonstrated a thickened aortic wall and an intraluminal aortic arch mass. The surgical specimen revealed angiosarcoma. Of the few reported angiosarcomas involving the aorta, most have been located in the abdominal segment. This is only the second reported case of aortic arch sarcoma presenting with stroke.

Pitfalls in the diagnosis of mitochondrial encephalopathy with lactic acidosis and stroke-like episodes.

We describe a patient with genetically- and biochemically-proven mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS) who was initially misdiagnosed as having had multiple ischemic strokes in part because the clinical presentation appeared to be acute, the MRI of lesions showed restricted diffusion, and the brain biopsy showed features suggestive of stroke. This report emphasizes the pitfalls in the diagnosis of MELAS and points out the similarities and differences between MELAS and ischemic stroke.

Papilledema in the assessment of ventriculomegaly.

To determine whether ventriculomegaly is associated with ongoing increased intracranial pressure (ICP), physicians often rely on corroborative imaging features such as altered periependymal signal, distortion of ventricular shape, subarachnoid space flattening, and an increase in ventricular size over time. In 2 patients with new headache and altered mental status, symptoms and ventriculomegaly were dismissed as long-standing and not reflective of current ICP elevation. In the first patient, ICP was considered normal because there were no corroborative imaging features of elevated ICP. In the second patient, ICP was considered normal because ventricular size was stable over a 1-year period. The diagnosis of ICP elevation was finally made by ICP monitoring after papilledema was recognized. Ventriculoperitoneal shunting rapidly resolved the papilledema and markedly improved mentation. Brain imaging may often be an unreliable guide to the presence of elevated ICP. In such patients, the finding of papilledema is a critical determinant of management.

Intraocular pressure is low in eyes with giant cell arteritis.

BACKGROUND: Although ocular ischemia occurs in giant cell arteritis (GCA), intraocular pressure (IOP) has not been systematically evaluated as a diagnostic sign. METHODS: We conducted a retrospective, case-controlled, observational study of IOP in patients with ocular manifestations of GCA (GCA patients), age-matched patients diagnosed with nonarteritic ischemic optic neuropathy (NAION patients), and age-matched patients with cataract (control patients). Medical records were examined for all consecutive patients with the diagnosis of GCA from 1995 to 2004 (n = 16) and NAION from 2002 to 2004 (n = 16) and for patient candidates for cataract extraction (n = 16). The eye intended for cataract extraction was chosen as the "affected eye" in the control patients. RESULTS: The mean IOP in the affected eye of 16 GCA patients was 11.9 mm Hg, significantly lower than the 15.1 mm Hg in affected eyes of age-matched NAION patients and 15.8 mm Hg in control patients (P = 0.002). At presentation, 5 GCA patients had IOP < 10 mm Hg (mean 6.8 mm Hg) without other signs of anterior segment ischemia. None of the NAION or control patients displayed such low IOPs. CONCLUSIONS: IOP was significantly lower in the patients with GCA than in patients with NAION or cataract. Hypotony occurred in one third of GCA patients without other signs of anterior ocular ischemia. These findings suggest that low IOP may be a distinguishing factor between GCA and NAION in patients with ischemic optic neuropathy, but evaluation of a larger group of patients is needed for confirmation.

Homonymous hemianopia caused by occipital lobe infarction in heparin-induced thrombocytopenia and thrombosis syndrome.

A 73-year-old woman developed mental confusion and finger pain after treatment with enoxaparin following arthroplasty. A platelet count was 163,000/microL. Because digital embolism was suspected, she was emergently treated with heparin and recombinant tissue plasminogen activator (rTPA). During rTPA infusion, she reported sudden hemifield loss, so the infusion was aborted. Brain CT disclosed a non-hemorrhagic occipital infarct. Platelets had fallen to 63,000 over eight days, and antibodies against a complex of heparin and platelet factor 4 were detected. These findings led to the diagnosis of heparin-induced thrombocytopenia and thrombosis syndrome (HITTS), an immune-mediated disorder in which venous and arterial thromboses occur. Right lower extremity deep venous thromboses were later diagnosed, and an MRI disclosed multiple cerebral infarcts of recent onset but different ages. Previous reports have documented brain arterial strokes in HITTS, mostly in the distribution of the middle cerebral artery, but clinical documentation is sparse, and there have been no imaging reports. This is the first report to document the clinical and imaging features of a HITTS stroke and the first to describe a stroke presumptively caused by a low molecular weight heparin. It emphasizes that HITTS may cause stroke even when the platelet count is normal. Diagnosis of HITTS should prompt immediate cessation of heparin treatment and substitution of a direct thrombin inhibitor or fondaparinux.