MRI of the Optic Nerves and Chiasm in Patients With Leber Hereditary Optic Neuropathy.

BACKGROUND: The aim of this study was to characterize brain and orbital MRI features of patients with Leber hereditary optic neuropathy (LHON), with particular attention to the optic nerves and chiasm. METHOD: We studied a patient cohort with genetically confirmed LHON followed at 2 ophthalmologic hospitals in France between 2013 and 2015. High-resolution brain and orbital MRI studies were analyzed for each patient during the first 12 months after the onset of visual loss was analyzed. RESULTS: Our study included 20 men and 8 women with a mean age of 38.3 years at diagnosis, and all had genetic mutations for LHON. Nineteen patients (67.9%) had T2 hyperintensity in the posterior portion of both optic nerves and in the optic chiasm, and enlargement of the chiasm was found in 16 patients (59.3%). No enhancement of the optic nerves or chiasm was detected. The T2 hyperintensity lesions were not associated with the time between symptom onset and obtaining MRI, the mutation type, or sex of the patient. Nonspecific T2 white matter lesions were found in MRI of 6 patients, but without the characteristics of those found in patients with multiple sclerosis. CONCLUSIONS: Involvement of the posterior portions of the optic nerves has been described previously in case reports of patients with LHON. Our results support this observation with neuroimaging performed within 1 year of onset of visual loss. Enlargement of the optic chiasm also may occur in patients with LHON. The pathophysiology of the MRI changes is not yet understood.

A Puzzling Ocular Motility Disorder: Apparent Up-Gaze Fatigability in a Patient With Oculomotor Nerve Compression.

We report the case of a woman who developed right third nerve dysfunction with synkinesis and ocular neuromyotonia secondary to a compressive arterial aneurysm. Surprisingly, our examination showed a downward drift of the right eye in sustained up-gaze resulting in transient hypotropia, suggesting either fatigability of the superior rectus or contraction of the inferior rectus. We believe this ocular motility pattern is secondary to a co-contraction of the inferior rectus in up-gaze caused by synkinesis (explaining the downward drift), followed by failure of the inferior rectus to relax upon return to primary position caused by ocular neuromyotonia (explaining the hypotropia).

Temporal Trends in Sex Differences With Regard to Stroke Incidence: The Dijon Stroke Registry (1987-2012).

BACKGROUND AND PURPOSE: We evaluated temporal trends in stroke incidence between men and women to determine whether changes in the distribution of vascular risk factors have influenced sex differences in stroke epidemiology. METHODS: Patients with first-ever stroke including ischemic stroke, spontaneous intracerebral hemorrhage, subarachnoid hemorrhage, and undetermined stroke between 1987 and 2012 were identified through the population-based registry of Dijon, France. Incidence rates were calculated for age groups, sex, and stroke subtypes. Sex differences and temporal trends (according to 5-year time periods) were evaluated by calculating incidence rate ratios (IRRs) with Poisson regression. RESULTS: Four thousand six hundred and fourteen patients with a first-ever stroke (53.1% women) were recorded. Incidence was lower in women than in men (112 versus 166 per 100 000/y; IRR, 0.68; P<0.001), especially in age group 45 to 84 years, and for both ischemic stroke and intracerebral hemorrhage. From 1987 to 2012, the lower incidence of overall stroke in women was stable (IRR ranging between 0.63 and 0.72 according to study periods). When considering stroke subtype, a slight increase in the incidence of ischemic stroke was observed in both men (IRR, 1.011; 95% confidence interval, 1.005-1.016; P=0.001) and women (IRR, 1.013; 95% confidence interval, 1.007-1.018; P=0.001). The sex gap in incidence remained unchanged in ischemic stroke and intracerebral hemorrhage. Conversely, the lower subarachnoid hemorrhage incidence in women vanished with time because of an increasing incidence. CONCLUSIONS: The sex gap in stroke incidence did not change with time except for subarachnoid hemorrhage. Despite lower rates, more women than men experience an incident stroke each year because of a longer life expectancy.

[Epidemiology of strokes in pediatry]. / Épidémiologie des accidents vasculaires cérébraux en pédiatrie.

Incidence of perinatal arterial stroke (ischemic stroke, intracerebral hemorrhage and subarachnoid hemorrhage) occurring between 20 weeks of gestation and postnatal day 28 ranges between 20 and 65 cases per 100,000 living births. In children aged 29 days to 18 years old, the total incidence is 1.3 to 4.3 for 100,000 per year. In France, almost 450 children are hospitalized for a stroke each year, among them approximately half have an intracerebral hemorrhage.

Takotsubo Cardiomyopathy Following Acute Cerebral Events.

OBJECTIVE: Takotsubo cardiomyopathy (TCM) is characterized by transient apical ventricular dysfunction typically induced by acute stress. Acute cerebral events including ischemic stroke (IS) or epileptic events (EEs) may be associated with massive catecholamine release. We aimed to identify the characteristics and outcomes of patients who experienced the Takotsubo syndrome complicated by IS or EE. METHODS: Between 2008 and 2013, 87 patients were admitted to our intensive care unit for TCM. Of these, 6 had previously experienced acute cerebral symptoms within 2 days of experiencing either IS or EE. Takotsubo syndrome was diagnosed on cardiac MRI, echocardiography, electrocardiography (ECG), biology and coronary angiography data. RESULTS: Five women and 1 man were included in the study. The mean age was 63.7 ± 20.1 years (range 44-84). Four of them (67%) initially presented an acute IS and 2 (33%) had EE. The suspected brain injury was found in the insular cortex for 4 patients and the posterior fossa for 2 patients. Hemiparesis, aphasia and cerebellar symptoms were the main neurological signs. Abnormal ECG findings including ST-segment elevation (33%) or T-wave inversion (50%) developed between a few hours and 48 h after the onset of the IS or EE. Peak troponin was 1.8 (0.79-14.11) µg/l. A transient reduction in the left ventricular ejection fraction (46 ± 12%) with apical hypokinesis was found using echocardiography. Two (33%) patients went on to develop acute heart failure. Coronary angiography confirmed the lack of significant coronary stenosis for all 6 patients. CONCLUSION: TCM can develop within the first few days after an acute cerebral event. It occurs predominantly in women with insular or posterior fossa lesions and is possibly induced by vegetative reactions.

Increasing early ambulation disability in spontaneous intracerebral hemorrhage survivors.

OBJECTIVE: To evaluate temporal trends in early ambulatory status in patients with spontaneous intracerebral hemorrhage (ICH). METHODS: All patients with ICH between 1985 and 2011 were prospectively registered in a population-based registry in Dijon, France, and included in the study. Outcomes of ICH survivors were assessed at discharge from their stay in an acute care ward with the use of a 4-grade ambulation scale. Time trends in ambulation disability and place of discharge were analyzed in 3 periods (1985-1993, 1994-2002, and 2003-2011). Multivariable ordinal and logistic regression models were applied. RESULTS: Five hundred thirty-one patients with ICH were registered, of whom 200 (37.7%) died in the acute care ward. While the proportion of deaths decreased over time, that of patients with ambulation disability increased (odds ratio [OR] 1.67, 95% confidence interval [CI] 0.87-3.23, p = 0.124 for 1994-2002; and OR 1.97, 95% CI, 1.08-3.60, p = 0.027 for 2003-2011 vs 1985-1993 in ordinal logistic regression). The proportion of patients dependent in walking rose (OR 2.11, 95% CI 1.16-3.82, p = 0.014 for 1994-2002; and OR 2.73; 95% CI 1.54-4.84, p = 0.001 for 2003-2011), and the proportion of patients discharged to home decreased (OR 0.49, 95% CI 0.24-0.99, p = 0.048 for 1994-2002; and OR 0.32, 95% CI 0.16-0.64, p = 0.001 for 2003-2011). CONCLUSION: The decrease in in-hospital mortality of patients with ICH translated into a rising proportion of patients with ambulation disability at discharge. A lower proportion of patients returned home. These results have major implications for the organization of postacute ICH care.

Temporal trends in the premorbid use of preventive treatments in patients with acute ischemic cerebrovascular events and a history of vascular disease: The Dijon Stroke Registry (1985-2010).

INTRODUCTION: Although secondary prevention in patients with arterial vascular diseases has improved, a gap between recommendations and clinical practice may exist. OBJECTIVES: We aimed to evaluate temporal trends in the premorbid use of preventive treatments in patients with ischemic cerebrovascular events (ICVE) and prior vascular disease. METHODS: Patients with acute ICVE (ischemic stroke/TIA) were identified through the population-based stroke registry of Dijon, France (1985-2010). Only those with history of arterial vascular disease were included and were classified into four groups: patients with previous coronary artery disease only (CAD), previous peripheral artery disease only (PAD), previous ICVE only, and patients with at least two different past vascular diseases (polyvascular group). We assessed trends in the proportion of patients who were treated with antihypertensive treatments and antithrombotics at the time of their ICVE using multivariable logistic regression models. RESULTS: Among the 5309 patients with acute ICVE, 2128 had a history of vascular disease (mean age 77.3±11.9, 51% men; 25.1% CAD 7.5% PAD, 39.8% ICVE, and 27.5% poylvascular). A total of 45.8% of them were on antithrombotics, 64.1% on antihypertensive treatment, and 34.4% on both. Compared with period 1985-1993, periods 1994-2002 and 2003-2010 were associated with a greater frequency of prior-to-ICVE use of antithrombotics (adjusted OR=5.94; 95% CI: 4.61-7.65, P<0.01, and adjusted OR=6.92; 95% CI: 5.33-8.98, P<0.01, respectively) but not of antihypertensive drugs. Consistent results were found when analyses were stratified according to the type of history of arterial vascular disease. CONCLUSION: Patients with ICVE and previous vascular disease were still undertreated with recommended preventive therapies.