Rahsan Gocmen

The detrimental effect of aging on leptomeningeal collaterals in ischemic stroke.

BACKGROUND Aging is associated with decreased penumbral salvage in patients with ischemic stroke. Another critical factor that determines the fate of penumbra tissue is the degree of collateral circulation, which decreases significantly with aging in experimental models of stroke. In this study, we sought to identify whether these observations could be translated to humans and, therefore, analyzed the effect of patient age on extent of leptomeningeal collaterals in patients with ischemic stroke. METHODS Computed tomography angiography (CTA) source images were used to assess the degree of collateral circulation in a retrospective series of patients with proximal middle cerebral artery (MCA) occlusion. Bivariate and multivariate analyses were used to explore the relationship between patient age and degree of collateral circulation. RESULTS A total of 70 patients were included into the study. Older age (P = .005), history of hypertension (P = .036), higher admission National Institutes of Health Stroke Scale (NIHSS) scores (P = .013), and increased time to CTA (P = .013) were associated with inadequate collaterals in bivariate analyses. In multivariate analysis, older age (P = .008) and higher NIHSS scores (P = .032) remained as the only significant independent variables that were associated with inadequate collaterals. A 10-year increment in patient age increased the odds of inadequate collateral circulation by 1.87 (95% confidence interval: 1.18-2.97). CONCLUSION Our findings show that there is a significant interplay between patient age and adequacy of leptomeningeal collateral circulation in patients with proximal MCA occlusion. The relationship could contribute to adverse tissue outcome and thereby to unfavorable clinical outcome observed in elderly patients with ischemic stroke.

Percutaneous Placement of Peritoneal Port-Catheter in Patients with Malignant Ascites

We report our experience with a radiologically placed peritoneal port-catheter in palliation of malignant ascites. Port-catheters were successfully placed under ultrasonographic and fluoroscopic guidance in seven patients (five women, two men) who had symptomatic malignant ascites. The long-term primary patency rate was 100%. The mean duration of catheter function was 148 days. Seven patients had a total of 1040 port-days. Two patients received intraperitoneal chemotherapy via the port-catheter. There were no procedure-related mortality and major complications. Minor complications such as ascitic fluid leakage from the peritoneal entry site, migration of the catheter tip to the right upper quadrant, and reversal of the port reservoir occurred in four patients. None of these complications affected the drainage and required port explantation. In patients with symptomatic malignant ascites, a peritoneal port-catheter can provide palliation and eliminate multiple hospital visits for repeated paracentesis with high patency and low complication rates.

Congenital absence of the portal vein associated with congenital hepatic fibrosis

The radiological features of a 7-year-old boy with congenital absence of the portal vein, pathologically proven congenital hepatic fibrosis, double inferior vena cava, ventricular septal defect, vertebral anomalies, crossed fused renal ectopia, and facial anomalies with pathological correlation are reported. This association between congenital absence of the portal vein and congenital hepatic fibrosis is unique.

Fulminant Central Plus Peripheral Nervous System Demyelination without Antibodies to Neurofascin.

BACKGROUND Combined central and peripheral nervous system demyelination is a rare and poorly described phenomenon. Recently, anti-neurofascin antibodies were reported to be positive in 86% of these patients in a Japanese cohort. Yet, there seems to be a clinical, radiological, and serological heterogeneity among these patients. In this report, our aim is to describe characteristics of our patients with this entity and compare with others in the literature. METHODS We report clinical, electrophysiological, radiological, and laboratory characteristics of five patients with both multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy from our institutional database containing 1890 MS patients. RESULTS Three patients presented with extensive, active demyelination of both central nervous system and peripheral nervous system with hypertrophic peripheral nerves. Plexuses, trunks, division and cords were involved in the process. Oligoclonal band was negative. Conduction block was not detected. Corticosteroid treatment was not adequate. Others had a slowly progressive clinical course. Serum anti-neurofascin antibody was negative. Review of the literature revealed similar cases with active disease, early-onset hypertrophic peripheral nerves, and central demyelination, in addition to other cases with an insidious course. CONCLUSIONS Patients with combined central and peripheral demyelination form a spectrum. Some patients may have an antibody-mediated syndrome with or without anti-neurofascin antibodies and others seem to represent a coincidence.

Reversible transverse sinus collapse in a patient with idiopathic intracranial hypertension

The association of idiopathic intracranial hypertension (IIH) with stenosis or narrowing of the transverse sinuses (TSs) is well known. However, there is debate as to whether the stenosis is a cause or consequence. Here we describe a case of IIH and narrowing of the TSs, with four relapses and recoveries after repeated CSF diversions with lumbar puncture (LP) over 2 months. Subsequently, implantation of a lumboperitoneal shunt (LPrS) ensured recovery. MR venography 20 months after LPrS showed normally calibrated TSs. We show repeated MR venography findings before and after the LPs, and discuss the pathogenesis of IIH in terms of the cause and effect relationship between IIH and sinus collapse.

Remission with fingolimod in a case of demyelinating polyneuropathy

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The association of cognitive impairment with gray matter atrophy and cortical lesion load in clinically isolated syndrome

BACKGROUND Multiple sclerosis can impair cognition from the early stages and has been shown to be associated with gray matter damage in addition to white matter pathology. OBJECTIVES To investigate the profile of cognitive impairment in clinically isolated syndrome (CIS), and the contribution of cortical inflammation, cortical and deep gray matter atrophy, and white matter lesions to cognitive decline. METHODS Thirty patients with clinically isolated syndrome and twenty demographically- matched healthy controls underwent neuropsychologic assessment through the Rao Brief Repeatable Battery, and brain magnetic resonance imaging with double inversion recovery using a 3T scanner. RESULTS Patients with clinically isolated syndrome performed significantly worse than healthy controls on tests that evaluated verbal memory, visuospatial learning and memory, and verbal fluency. Significant deep gray matter atrophy was found in the patients but cortical volume was not lower than the controls. Visual memory tests correlated with the volume of the hippocampus, cerebral white matter and deep gray matter structures and with cerebellar cortical atrophy. Cortical or white matter lesion load did not affect cognitive test results. CONCLUSION In our patients with CIS, it was shown that cognitive impairment was mainly related to cerebral white matter, cerebellar cortical and deep gray matter atrophy, but not with cortical inflammation, at least in the early stage of disease.

Blood-retina-barrier disruption accompanying blood-brain-barrier dysfunction in posterior reversible encephalopathy syndrome.

Blood-brain-barrier dysfunction is well known to accompany hypertensive posterior reversible encephalopathy syndrome (PRES) and is considered as the culprit of vasogenic edema and cerebral hemorrhage observed as part of this syndrome. An 84-year-old female was admitted with a diagnosis of PRES in the setting of malignant hypertension. The clinical course was further complicated by ischemic stroke and seizures. Contrast enhanced fluid attenuated inversion recovery (FLAIR) studies revealed diffuse enhancement within the subarachnoid space extending to regions without evidence of cytotoxic or vasogenic edema. These findings suggestive of increased permeability were not only confined to the blood-brain-barrier, but also involved the blood-retina-barrier interface. Our observations suggest that pathologic conditions that disrupt the integrity of blood-brain-barrier might concomitantly affect retinal microcirculation, which highly resembles cerebral microcirculation both anatomically and functionally. Imaging modalities sensitive for detection of blood-brain-barrier dysfunction, such as contrast enhanced FLAIR, might be helpful in identifying these abnormalities.

Nonsustained Atrial Fibrillation in Ischemic Stroke Patients and Stroke‐Free Controls From the Perspective of Stroke Pathophysiology

Background Short‐lasting (<30 s), nonsustained episodes of atrial fibrillation (NS‐AF) are considered a risk factor for future development of paroxysmal or persistent AF. Nonetheless, their causal role in stroke pathogenesis is currently unknown. In this study we determined the frequency of NS‐AF, together with the associated clinical and imaging features, in stroke‐free controls and ischemic stroke patients. Methods and Results A total of 332 controls, ≥50 years of age and no prior history of stroke or AF, were evaluated with 24‐hour Holter monitoring for the presence of <30‐s‐long AF episodes. The demographic and cardiovascular features of this cohort, together with imaging finding on magnetic resonance imaging, were compared to a consecutive series of ≥50‐year‐old ischemic stroke patients without AF (n=498). The prevalence of NS‐AF was significantly higher among ischemic stroke patients in comparison to controls (37% versus 27%; P=0.002). In multivariable analyses, after adjustment for demographic and cardiovascular risk factors, patients with ischemic stroke were more likely to harbor NS‐AF episodes (odds ratio 1.43; 95% CI 1.01–2.02; P=0.041). The association between ischemic stroke and NS‐AF weakened when the analyses were restricted to cryptogenic stroke patients (odds ratio 1.31; 95% CI 0.82–2.08). No significant association was observed between the presence of chronic cortical infarcts and NS‐AF. Conclusions Our study shows a higher prevalence of NS‐AF episodes in ischemic stroke patients in comparison to controls. Nonetheless, the lack of a stronger association with cryptogenic strokes and absence of a relationship with chronic cortical infarcts brings into question the causal influence of NS‐AF in the ischemic stroke setting.

Central pontine and extra-pontine myelinolysis after correction of severe hypoglycemia

A 73-year-old man with diabetes mellitus, hypertension, and recent Whipple’s operation for pancreatic cancer was admitted due to fever, cough and weight loss. His hospitalization was complicated by progressive malnutrition despite total parenteral nutrition, difficulties in controlling blood glucose levels and recurrent pneumonia. Two months after his admission, he developed sudden-onset coma attributed to severe hypoglycemia (deep to 13 mg/ dL). Blood glucose level was 214 mg/dL at 3 h prior to the incident and 238 mg/dL at 2 h after prompt replacement. Subsequently, glucose levels were stabilized between 150 and 250 mg/dL. Of note, no imbalance of electrolytes including sodium was noted during the hospital course. Because his status did not recover following glucose replacement, a brain magnetic resonance imaging (MRI) was obtained on the same day, and did not reveal any specific pathology. MRI was repeated after metabolically uneventful 11 days in which he stayed comatose. This MRI was typical for central pontine myelinolysis (CPM), characterized by restricted diffusion (Fig. 1a) and T2hyperintensity involving mid-pontine transverse fibers (Fig. 1b) with capsular (extra-pontine) extension (Fig. 1c). CPM is typically seen in connection with rapid correction of chronic and severe hyponatremia. However, it is well recognized that CPM can also occur in a variety of clinical diseases or conditions resulting in rapid and significant shifts in plasma osmolality in the absence of abnormalities of sodium homeostasis [1]. Fluctuations of blood glucose concentrations can be so brisk and profound as to cause serum tonicity changes wide enough for CPM development. In accordance, case reports, albeit quite rare, connecting CPM to the episodes of severe hypoglycemia [2], severe hyperglycemia [3, 4] or fast transition between hyperglycemia and hypoglycemia [5] can be found in the literature. We add another example, wherein CPM triggered by either profound hypoglycemia or, more likely, its rapid correction, supporting the theory that the pathogenesis of CPM is primarily dependent on tonicity changes, not independently from sodium abnormalities itself. Another feature that warrants further discussion pertaining to this case is unusual extra-pontine lesion location. It is well known that extra-pontine myelinolysis is frequently seen in the setting of CPM. Therefore, ‘‘osmotic demyelination syndrome’’ has recently been coined as an alternative description for the syndrome. Indeed, many parts of the central nervous system can be involved in CPM in addition to basis pontis. Frequently involved extrapontine areas are the thalamus, sub-thalamic nucleus, external geniculate body, putamen, globus pallidum, cerebellar and subcortical white matter. Internal capsule involvement, as documented in the case presented herein, is an exceptional feature for CPM [6]. An MRI documentation of unilateral involvement of the internal capsule has recently been reported in the setting of extra-pontine H. Onder E. M. Arsava M. A. Topcuoglu (&) Neurosonology Laboratory, Neurointensive Care Unit, Department of Neurology, Faculty of Medicine, Hacettepe University Hospitals, Hacettepe University, Sihhiye, 06100 Ankara, Turkey e-mail: mat@hacettepe.edu.tr; matopcuoglu@yahoo.com