Heui-Soo Moon

Different clinical and magnetic resonance imaging features between Charcot–Marie–Tooth disease type 1A and 2A

Charcot-Marie-Tooth disease type 1A (CMT1A) is the more frequent cause of demyelinating CMT, and CMT2A is the most common cause of axonal CMT. We conducted a magnetic resonance imaging (MRI) study on 39 CMT1A and 21 CMT2A patients to compare their neuroimaging patterns and correlate with clinical features. CMT1A patients showed selective fatty infiltration with a preference for anterior and lateral compartment muscles, whereas CMT2A patients showed a preference for superficial posterior compartment muscles. Early-onset CMT2A patients showed more severe leg fatty atrophy than late-onset CMT2A patients. In late-onset CMT2A, soleus muscle was the earliest, and most severely affected than the other leg muscles. Selective involvement of intrinsic foot muscles is a characteristic pattern of minimal CMT1A and CMT2A. Our MRI study demonstrates different patterns of fatty infiltration involving superficial posterior compartment muscles in CMT2A (partial T-type), and peroneal nerve innervated muscles in CMT1A (P-type).

Cardiac troponin T elevation after stroke: relationships between elevated serum troponin T, stroke location, and prognosis.

Background and Purpose Elevation of serum cardiac troponin T (cTnT) is regarded as a specific marker of acute coronary syndrome. Serum cTnT can be increased in patients with acute ischemic stroke, but its clinical implications remain unclear. The aim of this study was to identify the relationships between elevated cTnT and stroke severity, location, and prognosis. Methods From January 2005 to December 2006, this study recruited 455 consecutive patients who were admitted to Kangbuk Samsung Hospital due to acute ischemic stroke within 3 days of onset, which was confirmed by diffusion magnetic resonance imaging. A total of 416 patients was finally included and divided into 2 groups: an elevated cTnT group (n=45) and a normal cTnT group (n=371). The short-term prognosis was assessed by 30-day modified Rankin Scale responder analysis was compared between the two groups. Results Serum cTnT was elevated in 10.8% of cases, with elevated cTnT associated with greater stroke severity, as assessed by the National Institutes of Health Stroke Scale score, Insular-lobe involvement was more common in patients with elevated cTnT than in the normal cTnT group. Short-term prognosis was more unfavorable in the elevated cTnT group than in the normal cTnT group. Multivariate regression analysis indicated that elevated cTnT was independently related to insular involvement, cardioembolism, and unfavorable outcome. Conclusions Elevated cTnT in acute ischemic stroke was associated with severe neurological deficits at stroke onset and damages to the insular lobe. The outcome of acute ischemic stroke was worse for patients with elevated cTnT than for those with normal cTnT. The pathomechanism underlying acute ischemic stroke and subclinical myocardial damage warrants further study.

Intracranial Internal Carotid Artery Calcification: A Representative for Cerebral Artery Calcification and Association with White Matter Hyperintensities

Background: Our aim was to investigate the distribution pattern of cerebral artery calcification and its association with white matter hyperintensities (WMH). Methods: We identified 159 consecutive patients with acute ischemic stroke. Calcifications of cerebral arteries and WMH were graded. Results: Cerebral artery calcification was found in 137 patients (86.2%). The intracranial internal carotid artery (I-ICA) was the most frequently affected artery with calcification (76.7%) and moderate-to-severe calcification (38.1%). Spearman’s rank test revealed that the grade of I-ICA calcification was correlated with those of periventricular WMH (r = 0.417, p < 0.001) and deep WMH (r = 0.388, p < 0.001). The adjusted ORs of I-ICA were 2.62 (p <0.05) for periventricular WMH and 3.25 (p <0.05) for deep WMH. Conclusions: Cerebral artery calcification is common in patients with ischemic stroke. I-ICA is the most frequently and most severely affected cerebral artery and its calcification is associated with WMH.

Serum Vitamin D Status as a Predictor of Prognosis in Patients with Acute Ischemic Stroke

Background: Low 25-hydroxyvitamin D (25(OH)D) concentrations have been shown to predict risk of cardiovascular disease and all-cause mortality. Although the prevalence of 25(OH)D deficiency is high in patients with acute stroke, the prognostic value of 25(OH)D in stroke has not been clearly established. The purpose of this study was to determine whether the baseline serum 25(OH)D level was associated with the functional outcome in patients with acute ischemic stroke. Methods: From June 2011 to January 2014, consecutive patients with acute ischemic stroke within 7 days of symptom onset were enrolled in this study from a prospectively maintained stroke registry. Serum 25(OH)D level was measured at admission. Clinical and laboratory data including stroke severity using the National Institute of Health Stroke Scale (NIHSS) score were collected during admission, and the functional outcome at 3 months was assessed by modified Rankin scale (mRS). The association between the baseline 25(OH)D level and a good functional outcome (mRS 0-2) at 3 months was analyzed by multiple logistic regression models. Results: A total of 818 patients were enrolled in this study. Mean age was 66.2 (±12.9) years, and 40.5% were female. The mean 25(OH)D level was 47.2 ± 31.7 nmol/l, and the majority of patients met vitamin D deficient status (<50 nmol/l; 68.8%), while an optimal vitamin D level (≥75 nmol/l) was present in only 13.6% of the patients, and 436 (53.3%) patients showed good functional outcome at 3 months. Serum 25(OH)D levels in patients with good outcomes were significantly higher than those with poor outcome (50.2 ± 32.7 vs. 43.9 ± 30.0 nmol/l, p = 0.007). The 3-month functional outcome was significantly associated with month-specific 25(OH)D quartiles in multivariable logistic regression analysis. After adjustment for age and sex, the highest 25(OH)D quartile group had higher tendency for good functional outcome at 3 months (odds ratio (OR) = 1.68, 95% confidence interval (CI) = 1.13-2.51). After fully adjusting for other potential confounders, such as stroke severity and vascular risk factors, the association was further strengthened with an OR (95% CI) of 1.90 (1.14-3.16). Other factors associated with good functional outcome in multivariable analysis were younger age, lower initial NIHSS score and absence of diabetes. Conclusions: This study suggests that serum 25(OH)D level is an independent predictor of functional outcome in patients with acute ischemic stroke. Further studies are required to determine whether vitamin D supplementation could improve functional outcome in patients with ischemic stroke.

A case of symptomatic hypnic headache syndrome

The hypnic headache syndrome is a rare, benign, nocturnal, non-familial headache disorder that occurs only while asleep. Since the first description by Raskin (1) in 1998, the syndrome has been characterized by a diffuse headache that awakens patients at a consistent time of the night, sometimes during a dream, and lasts for 30–180 min. This disorder mainly affects elderly subjects and both sexes equally (2). The pathophysiological mechanism of hypnic headache is not known, but its circadian periodicity and responsiveness to lithium carbonate suggest that perturbation of chronobiological rhythms causes this headache (1). We describe a patient with hypnic headache, which had developed after pontine infarction. To our knowledge, this is the first report of symptomatic hypnic headache that developed after stroke and that suggests direct evidence of a potential pathophysiological mechanism and relationship of hypnic headache to the sleep cycle.

25-Hydroxyvitamin D Status Is Associated With Chronic Cerebral Small Vessel Disease

Background and Purpose— The aim of this study was to determine the association between 25-hydroxyvitamin D (25(OH)D) and neuroimaging correlates of cerebral small vessel disease. Methods— We identified 759 consecutive patients with acute ischemic stroke or transient ischemic attack. Lacunes, white matter hyperintensity, and cerebral microbleed (CMB) were assessed using MR images. Deep CMB was defined as the presence of CMB in basal ganglia, thalamus, or brain stem. The association between 25(OH)D and small vessel disease was tested using linear and logistic regression analyses. Results— Mean age was 68 (±13) years. Mean level of 25(OH)D was 34.1±17.8 nmol/L. On bivariate analysis, a 25-nmol/L decrease in 25(OH)D was associated with lacunes (regression coefficient, 0.23; 95% confidence interval [CI], 0.02–0.45), severe white matter hyperintensity (odds ratio, 2.05; 95% CI, 1.41–3.08), and deep CMB (odds ratio, 1.28; 95% CI, 1.01–1.63). Also, 25(OH)D deficiency (⩽25 nmol/L) was associated with lacunes (regression coefficient, 0.5; 95% CI, 0.04–0.95), severe white matter hyperintensity (odds ratio, 2.74; 95% CI, 1.31–6.45), and deep CMB (odds ratio, 1.68; 95% CI, 1.03–2.78). The association remained significant even after multivariable adjustment and in the subgroup of previously healthy patients. Conclusions— 25(OH)D is inversely associated with lacunes, white matter hyperintensity, and deep CMB. Our findings suggest that 25(OH)D is linked to small vessel disease, and in future trials it should be tested whether 25(OH)D supplementation can prevent small vessel disease.

Leukoaraiosis predicts poor outcome after spontaneous supratentorial intracerebral hemorrhage.

Background and Aims: Leukoaraiosis (LA) is associated with aging and vascular risk factors, and is a risk factor of intracerebral hemorrhage (ICH) after treatment with warfarin or thrombolytic treatment for ischemic stroke. In this study, we sought to examine whether LA is a predictor of outcome after spontaneous ICH. Methods: We retrospectively analyzed 238 consecutive patients with spontaneous supratentorial ICH identified by a database search. Patients were divided into two groups according to neurological outcome at 90 days: patients with good outcomes (Glasgow Outcome Scale ≧4) and patients with poor outcomes. Demographic features, ICH characteristics, and LA severity as assessed by van Swieten score on brain CT were compared between the two groups. Results: Overall, 105 (44.1%) of the patients analyzed had poor outcomes. In univariate analysis, LA severity, ICH volume on initial brain CT, initial Glasgow Coma Scale (GCS), presence of intraventricular hemorrhage (IVH), old age, surgical treatment, and higher admission serum glucose level were associated with poor outcome. Multiple logistic regression analysis showed that severity of LA, initial GCS score, hematoma volume, presence of IVH, and surgical treatment were independent predictors of poor outcome. Conclusion: LA is an independent predictor of poor neurological outcome in patients with spontaneous supratentorial ICH.

Association between Cerebral Arterial Calcification and Brachial-Ankle Pulse Wave Velocity in Patients with Acute Ischemic Stroke

Background/Aims: Vascular calcification is known to be associated with cardiovascular mortality, and arterial stiffness measured by pulse wave velocity is associated with major cardiovascular risk factors. The aim of the present study was to elucidate the correlation between arterial stiffness and cerebral arterial calcification. Methods: Arterial stiffness, as measured by brachial-ankle pulse wave velocity (baPWV), and cerebral arterial calcification, as measured by CT angiography using a 40-multidetector scanner, were examined in patients with acute ischemic stroke. Sixty-seven subjects who were free of renal disease or peripheral arterial disease were included in the analysis. Results: Univariate analysis revealed that baPWV was significantly correlated with cerebral arterial calcification (r = 0.524, p < 0.001) and age (r = 0.452, p < 0.001), and multiple linear regression analysis indicated that age and cerebral arterial calcification were independent determinants of baPWV. Conclusion: We report that increased baPWV is closely associated with the degree of cerebral arterial calcification in patients with acute ischemic stroke. Our results suggest that the severity of cerebral arterial calcification is representative of the degree of systemic arterial stiffness.

Increased Pulsatility Index Is Associated with Intracranial Arterial Calcification

Background/Aims: An increase in the pulsatility index (PI) has been suggested to reflect distal vascular resistance. The purpose of the present study was to investigate the association between intracranial arterial calcification and intracranial PIs. Methods: Consecutive patients with acute ischemic stroke or transient ischemic attack were included. The PIs of both middle cerebral arteries (MCAs) were measured by transcranial Doppler ultrasonography. Intracranial carotid artery calcification (ICAC) was assessed on computed tomography angiography, and then compared with the mean PI of both MCAs. Patients with internal carotid artery steno-occlusion were excluded from this study. Results: A total of 156 patients were finally enrolled. The prevalence of diabetes increased as the PI value increased (p for trends; p = 0.025). PI was correlated with ICAC score (r = 0.413, p < 0.001) and age (r = 0.507, p < 0.001). Multiple linear regression analysis indicated that aging and ICAC were independent determinants of the PI of MCA after adjusting for sex, systolic blood pressure, smoking, and the presence of diabetes. Conclusions: This study shows that an increase in PI was correlated with the severity of ICAC, which suggests calcification-related vascular resistance might have a role in the elevation of PI.

Association between Small Deep Cerebellar Ischemic Lesion and Small-Vessel Disease.

Background: Pathologic investigations showed that lacunar infarction could develop in the deep cerebellar region. However, the etiology of small deep cerebellar ischemic lesions (SDCI) has not been adequately studied. The aim of this study was to investigate the relationship between SDCI and small-vessel disease. Methods: We studied 100 consecutive patients who had both (1) acute ischemic stroke (index stroke) confirmed by diffusion-weighted MRI and (2) evidence of a chronic small cerebellar ischemic lesion (<15 mm). Small-vessel-related MRI findings, risk factors and subtype of index stroke were compared between the patients with SDCI and those with small cortical cerebellar ischemic lesions (SCCI). Results: Eighty patients had SCCI, and 20 patients had SDCI. Cardioembolic sources (38.8 vs. 5%, p = 0.003) and vertebrobasilar artery stenosis (61.3 vs. 35%, p = 0.034) were more frequent in patients with SCCI. In a multivariate model including traditional risk factors, patients with SDCI had significantly more lacunar infarcts (odds ratio, 1.18; 95% confidence interval, 1.02–1.37) and cerebral microbleeds (odds ratio, 10.92; 95% confidence interval, 2.16–55.32) than those with SCCI. Patients with SDCI frequently had the small-artery disease subtype of index stroke (odds ratio, 5.84; 95% confidence interval, 1.71–19.9). Conclusions: The results suggest that SDCI are frequently associated with small-vessel disease.