The Piano Man: A Case Report of Anterior Thalamic Infarct with Dementia and Preserved Music Ability.

OBJECTIVE: The thalamus is the integrative hub of the brain with reciprocal connections throughout the cortex. This case report describes a right-handed 81-year-old male patient who experienced sudden onset cognitive impairment following a focal left anterior thalamic infarct. METHODS: With consent/assent, the patient was seen for a short neuropsychological assessment 6 weeks post stroke. Neuropsychological assessment included review of medical history, collateral intake, the Toronto Cognitive Assessment, Frontal Systems Behavior Scale-Family Rating Form, the Neuropsychiatric Inventory Questionnaire, and piano performance. RESULTS: The assessment revealed impaired performance on measures of orientation, memory, executive function, and language, as well as symptoms including hallucinations, apathy, and hypersomnolence, consistent with thalamic dementia. Remarkably, in this context, the patient maintained an ability to play piano and read music. CONCLUSIONS: The case has implications for understanding the complex integrative functions of the thalamus, including how profound impairment can simultaneously present with cognitive strengths that may not be captured by performance on neuropsychological testing. This case also suggests that magnetic resonance imaging may be indicated in cases presenting with vascular risk factors and sudden onset cognitive impairment, given that computed tomography may not be sensitive to small subcortical infarcts.

Combined Preconditioning Reduces the Negative Influence of Cerebral Ischemia on the Morphofunctional Condition of CNS.

We studied the influence of combined preconditioning (compound pQ-4 and moderate hypoxia) on morphometrical parameters of neuronal populations in hippocampal fields CA1 and CA3 in rats after bilateral ligation of the common carotid artery. Preconditioning produced a neuroprotective effect, improved survival of pyramidal neurons in the early and delayed periods of modeled ischemia, prevented the formation of necrotic and apoptotic neurons and hyperactivation of microglia, and protected endotheliocytes. The positive influence of preconditioning factors on the morphometric parameters of the brain under ischemic conditions agrees with the results of behavioral tests (open field and elevated plus maze) that demonstrated increased locomotor activity and exploratory behavior of animals.

Abnormal singing can identify patients with right hemisphere cortical strokes at risk for impaired prosody.

ABSTRACT: Despite lacking aphasia seen with left hemisphere (LH) infarcts involving the middle cerebral artery territory, right hemisphere (RH) strokes can result in significant difficulties in affective prosody. These impairments may be more difficult to identify but lead to significant communication problems.We determine if evaluation of singing can accurately identify stroke patients with cortical RH infarcts at risk for prosodic impairment who may benefit from rehabilitation.A prospective cohort of 36 patients evaluated with acute ischemic stroke was recruited. Participants underwent an experimental battery evaluating their singing, prosody comprehension, and prosody production. Singing samples were rated by 2 independent reviewers as subjectively "normal" or "abnormal," and analyzed for properties of the fundamental frequency. Relationships between infarct location, singing, and prosody performance were evaluated using t tests and chi-squared analysis.Eighty percent of participants with LH cortical strokes were unable to successfully complete any of the tasks due to severe aphasia. For the remainder, singing ratings corresponded to stroke location for 68% of patients. RH cortical strokes demonstrated a lower mean fundamental frequency while singing than those with subcortical infarcts (176.8 vs 130.4, P = 0.02). They also made more errors on tasks of prosody comprehension (28.6 vs 16.0, P < 0.001) and production (40.4 vs 18.4, P < 0.001).Patients with RH cortical infarcts are more likely to exhibit impaired prosody comprehension and production and demonstrate the poor variation of tone when singing compared to patients with subcortical infarcts. A simple singing screen is able to successfully identify patients with cortical lesions and potential prosodic deficits.

Exosomes containing miR-451a is involved in the protective effect of cerebral ischemic preconditioning against cerebral ischemia and reperfusion injury.

AIM: To study the role of exosomes in the protective effect of cerebral ischemic preconditioning (cerebral-IPC) against cerebral I/R injury. METHOD: Mouse models of cerebral-IPC and MCAO/R were established as described previously, and their behavioral, pathological, and proteomic changes were analyzed. Neuro-2a subjected to OGD/R were treated with exosomes isolated from the plasma of sham-operated and cerebral-IPC mice. The differentially expressed miRNAs between exosomes derived from sham-operated (S-exosomes) and preconditioned (IPC-exosomes) mice were identified through miRNA array, and their targets were identified through database search. The control and OGD/R cells were treated with the IPC-exosomes, miRNA mimic or target protein inhibitor, and their viability, oxidative, stress and apoptosis rates were measured. The activated pathways were identified by analyzing the levels of relevant proteins. RESULTS: Cerebral-IPC mitigated the cerebral injury following ischemia and reperfusion, and increased the number of plasma exosomes. IPC-exosomes increased the survival of Neuro-2a cells after OGD/R. The miR-451a targeting Rac1 was upregulated in the IPC-exosomes relative to S-exosomes. The miR-451a mimic and the Rac1 inhibitor NSC23766 reversed OGD/R-mediated activation of Rac1 and its downstream pathways. CONCLUSION: Cerebral-IPC ameliorated cerebral I/R injury by inducing the release of exosomes containing miR-451a.

Concurrent diffuse dermal angiomatosis and granuloma inframammary adultorum of the breast.

Diffuse dermal angiomatosis (DDA) is a cutaneous reactive angiomatosis. Typically presenting as ulcerated, erythematous, violaceous, or purpuric plaques on the breast or lower extremities, DDA is believed to be a reaction to tissue ischemia. Granuloma inframammary adultorum (GIA) is a type of irritant dermatitis of multifactorial etiology, clinically presenting as papules and nodules. Herein, we report an interesting rash presenting as fungiform papulonodules overlying a large violaceous plaque on the left breast. Biopsy revealed an exuberant epidermal proliferation and a diffuse and deep dermal proliferation, consisting of small slit-like blood vessels in between collagen bundles. In light of these clinical and histopathologic findings in the setting of an indurated plaque on a pendulous breast of a woman with multiple risk factors for local tissue ischemia, a diagnosis of concurrent diffuse angiomatosis of the breast (DDAB) and GIA was rendered. This case highlights the critical importance of clinicopathologic correlation in the diagnosis of multiple diagnostic entities.

Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis.

BACKGROUND AND PURPOSE: Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences. METHODS: The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019. RESULTS: The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSß0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSß0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSß+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association. CONCLUSIONS: This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.

Assessing the Reliability of Reported Medical History in Older Adults.

BACKGROUND: Age-associated increases in medical complexity, frailty, and cognitive impairment may compromise reliable reporting of medical history. OBJECTIVE: To evaluate the influence of increasing age and cognitive impairment on concordance between reported history of stroke and cerebral infarction, and reported history of diabetes and elevated hemoglobinA1c in community-dwelling older adults. METHODS: The association between participant-specific factors and accurate reporting of stroke or diabetes was evaluated using multivariable logistic regression in 1,401 participants enrolled in longitudinal studies of memory and aging, including 425 participants with dementia (30.3%). Stroke and diabetes were selected as index variables as gold standard measures of both were obtained in all participants: magnetic resonance neuroimaging for cerebral infarcts and hemoglobinA1c (≥6.5%) for diabetes. RESULTS: Concordance between reported history of stroke and imaging-confirmed cerebral infarction was low (sensitivity: 17.4%, 8/46; specificity: 97.9%, 799/816). Small infarcts were strongly associated with inaccurate reporting (OR = 265.8; 95% CI: 86.2, 819.4), suggesting that occult/silent infarcts contributed to discordant reporting. Reporting accuracy was higher concerning diabetes (sensitivity: 83.5%, 147/176; specificity: 96.2%, 1100/1143). A history of hypertension (OR = 2.3; 95% CI: 1.3, 4.2), higher hemoglobinA1c (OR = 1.9; 95% CI: 1.5, 2.4), and hemoglobinA1c compatible with impaired glucose tolerance (OR = 3.1; 95% CI 1.8, 5.3) associated with increased odds of discordant reporting. Cognitive impairment and increased age were not independently associated with reliable reporting. CONCLUSION: Factors beyond advancing age and cognitive impairment appear to drive discordance in reported medical history in older participants. Objective testing for cerebral infarcts or diabetes should be performed when relevant to diagnostic or therapeutic decisions in clinical and research settings.

Serum Uric Acid and High-Sensitivity C-Reactive Protein as Predictors of Cognitive Impairment in Patients with Cerebral Infarction.

BACKGROUND: Cognitive impairment induced by cerebral infarction has become a devastating health problem. More efficient predictors are required to evaluate the potential cognitive decline after cerebral infarction in clinic. Serum uric acid (UA) and high-sensitivity C-reactive protein (hs-CRP) are two factors reported to correlate with cognitive impairment. However, the understanding on serum UA and hs-CRP with cognitive dysfunction remains unclear. METHODS: Serum UA and hs-CRP were evaluated in patients with cerebral infarction (n = 197) using single factor analysis and multivariate logistic regression analysis. Clinical and pathological characteristics were analyzed by logistic regression, respectively, and the results demonstrated the correlation between the pathological characteristics and the cognitive impairment post cerebral infarction. Montreal Cognitive Assessment (MoCA) was used to evaluate the patients' cognitive function, and patients with a MoCA score <26 were recognized as with cognitive impairment. RESULTS: Clinical characteristics related to cognitive impairment, including age, gender, blood pressure, serum UA, and hs-CRP were collected and analyzed. Serum UA and hs-CRP were identified to be potential predictors for post-stroke cognitive dysfunction, with higher serum UA levels correlated with better cognitive function and higher hs-CRP levels correlated with worse cognitive impairment. CONCLUSION: Serum UA and hs-CRP are two predictors for cognitive impairment post cerebral infarction.

Relationship between recovery of motor function and neuropsychological functioning in cerebral infarction patients: the importance of social functioning in motor recovery.

Impaired motor function is a common disabling sequela after stroke. It is closely associated with the patient's quality of life and independence. Neuropsychological dysfunctions also frequently occur in stroke patients. In this paper, we evaluate the relationship between the recovery of motor function and neuropsychological functions, including cognition, language, emotion, behavior, personality, and social interaction, to provide appropriate and effective therapy for stroke patients. Motor function, neuropsychological status, social functioning, as well as emotional aspects such as depression and anxiety symptoms, were initially evaluated one month after cerebral infarction onset. The evaluations were repeated three months after the onset. Motor function was assessed with the Modified Barthel Index. The neuropsychological status was evaluated using the Mini-Mental State Examination, Global Deterioration Scale, digit span test, Korean-Boston Naming Test, Vineland Social Maturity Scale, Neuropsychiatric Inventory, Beck's Depression Inventory, and Beck Anxiety Inventory. In the results, the Modified Barthel Index, Mini-Mental State Examination, Global Deterioration Scale, digit span test, and Vineland Social Maturity Scale were significantly different between the two-time points (P < 0.05). Initial Social Maturity Scale Social Age and Social Maturity Scale Social Quotient categories of the Vineland Social Maturity Scale and Mini-Mental State Examination scores were significantly correlated with Modified Barthel Index improvement (P < 0.05). The amount of change in the Social Maturity Scale Social Age and Social Maturity Scale Social Quotient scores was significantly correlated with Modified Barthel Index improvement (P < 0.05). In multiple linear regression analysis, only the initial Social Maturity Scale Social Quotient score and the amount of score change in Social Maturity Scale Social Quotient showed a significant correlation with Modified Barthel Index improvement (P < 0.05). Social function and interaction are important in motor recovery of ischemic stroke patients.

A Rare Clinical Antity; Pure Gerstmann Syndrome.

Gerstmann syndrome is defined as a tetrad including agraphia, acalculia, finger agnosia, and right-left disorientation. In the case studies presented in the literature, it has been reported that Gerstmann syndrome usually appears as an incomplete tetrad of symptoms or accompanied by cognitive deficits including aphasia, alexia, apraxia and some perceptual disorders. Here, we present of the patient with left angular and supramarginal gyrus infarction affecting the parietal lobe. In addition to the symptoms mentioned above, the patient had alexia and anomic aphasia as well. We discussed the clinic appearance and reviewed the current literature.

Aparición de clínica obsesiva tras infarto cerebrovascular. a propósito de un caso / Onset of obsessive symptoms after a cerebrovascular stroke. with regard to a case

Se presenta el caso clínico de un paciente de 72 años sin antecedentes psiquiátricos previos que ingresa en el Servicio de Neurología por infarto cerebral en territorio vertebrobasilar. Durante el ingreso presenta elevada ansiedad secundaria a una ideación obsesiva de contenido erótico con intensa repercusión emocional y conductual. Se inicia tratamiento sintomático con Quetiapina, observándose mejoría progresiva del cuadro hasta la total recuperación del paciente. En el presente artículo se revisa la relación entre las diferentes áreas anatómicas cerebrales y la aparición de clínica obsesiva

A 72 year old male without previous psychiatric history is admitted to the neurology department due to vertebrobasilar stroke. During the stay, the patient manifests a high level of anxiety related to the onset of an erotic obsessive idea with an intense emotional and behavioural repercussion. Symptomatic treatment with quetiapine was started, with a progressive improvement of the symptoms until it's complete resolution. In the present article, we aim to review the relationship between the different brain anatomical areas and the onset of obsessive symptoms

The prevalence of psychiatric symptoms before the diagnosis of Parkinson's disease in a nationwide cohort: A comparison to patients with cerebral infarction.

OBJECTIVES: Psychiatric symptoms (PS) can be non-motor features in Parkinson's disease (PD) which are common even in the prodromal, untreated phase of the disease. Some PS, especially depression and anxiety recently became known predictive markers for PD. Our objective was to explore retrospectively the prevalence of PS before the diagnosis of PD. METHODS: In the framework of the Hungarian Brain Research Program we created a database from medical and medication reports submitted for reimbursement purposes to the National Health Insurance Fund in Hungary, a country with 10 million inhabitants and a single payer health insurance system. We used record linkage to evaluate the prevalence of PS before the diagnosis of PD and compared that with patients with ischemic cerebrovascular lesion (ICL) in the period between 2004-2016 using ICD-10 codes of G20 for PD, I63-64 for ICL and F00-F99 for PS. We included only those patients who got their PD, ICL and psychiatric diagnosis at least twice. RESULTS: There were 79 795 patients with PD and 676 874 patients with ICL. Of the PD patients 16% whereas of those with ischemic cerebrovascular lesion 9.7% had a psychiatric diagnosis before the first appearance of PD or ICL (p<0.001) established in psychiatric care at least twice. The higher rate of PS in PD compared to ICL remained significant after controlling for age and gender in logistic regression analysis. The difference between PD and ICL was significant for Mood disorders (F30-F39), Organic, including symptomatic, mental disorders (F00-F09), Neurotic, stress-related and somatoform disorders (F40-F48) and Schizophrenia, schizotypal and delusional disorders (F20-F29) diagnosis categories (p<0.001, for all). DISCUSSION: The higher rate of psychiatric morbidity in the premotor phase of PD may reflect neurotransmitter changes in the early phase of PD.

Long-term effects of experimental carotid stenosis on hippocampal infarct pathology, neurons and glia and amelioration by environmental enrichment.

Hippocampal atrophy and pathology are common in ageing-related disorders and associated with cognitive impairment and dementia. We explored whether environmental enrichment (EE) ameliorated the pathological sequelae in the hippocampus subsequent to chronic cerebral hypoperfusion induced by bilateral common carotid artery stenosis (BCAS). Seventy-four male C57BL/6 J mice underwent BCAS or sham surgery. One-week after surgery, mice were exposed to three different degrees of EE; either standard housing conditions (std), limited 3 -h exposure to EE per day (3 h) or full-time exposure to EE (full) for 3 months. Four months after surgery, the hippocampus was examined for the extent of vascular brain injury and neuronal and glial changes. Results showed that long-term BCAS induced strokes, most often in CA1 subfield, reduced 40-50 % CA1 neurons (P < 0.01) and increased microglia/macrophage in CA1-CA3 subfields (P < 0.02). Remarkably, both 3 h and full-time EE regimes attenuated hippocampal neuronal death and repressed recurrent strokes with complete prevention of larger infarcts in mice on full-time EE (P < 0.01). Full-time EE also reduced astrocytic clasmatodendrosis and microglial/macrophage activation in all CA subfields. Our results suggest that exposure to EE differentially reduces long-term hypoperfusive hippocampal damage. The implementation of even limited EE may be beneficial for patients diagnosed with vascular cognitive impairment.

Effects of exercise timing and intensity on neuroplasticity in a rat model of cerebral infarction.

Exercise therapy plays key roles in functional improvements during neurorehabilitation. However, it may be difficult for some people to properly perform exercise because mobility and endurance might be restricted by neurological deficits due to stroke. Additionally, there is little evidence detailing the biological mechanisms underlying the most effective swimming exercise protocols for neuroplasticity after stroke. Thus, the present study investigated the effects of swimming exercise on neuroplasticity in a cerebral infarction rat model according to the timing and intensity of exercise. A total of 45 male Sprague-Dawley rats (300 ±â€¯50 g, 10 weeks old) were subjected to photothrombotic cerebral infarction and randomly divided into five groups: non-exercise (group A, n = 9); early submaximal (group B, n = 9); early maximal (group C, n = 9); late submaximal (group D, n = 9); and late maximal (group E, n = 9). Swimming exercise was performed five times a week for 4 weeks, and cognition was evaluated with the Morris water maze (MWM) test. Assessments of superoxide dismutase (SOD) activity and malondialdehyde (MDA) levels and immunohistochemical analyses of brain-derived neurotrophic factor (BDNF) were conducted in the ipsilesional hippocampus region. After 4 weeks of exercise, the escape latency was shorter and velocity was greater in group B than in groups A, C, D, and E (p = 0.046, p <  0.001, respectively). Furthermore, SOD activity was higher and MDA levels were lower in group B than in groups A, C, D, and E (p = 0.004, p = 0.019). The immunohistochemistry results revealed that the greatest BDNF immunoreactivity was in group B. Taken together, these results indicate that early submaximal swimming exercise may be the most effective protocol for the recovery of neurological deficits in a rat model of cerebral infarction.

Vision-related quality of life in patients with occipital stroke.

OBJECTIVES: The aim of this study was to detect visual field defects (VFDs) after occipital infarction, investigate the rate of recovery and the impact of VFD upon vision-related quality of life (QoL). MATERIALS AND METHODS: Multicenter, prospective study including patients with MRI verified acute occipital infarction (NOR-OCCIP project). Ophthalmological examination including perimetry was performed within 2 weeks and after 6 months. Vision-related QoL was assessed by the National Eye Institute Visual Function Questionnaire 25 (VFQ-25) at one and 6 months post-stroke. RESULTS: We included 76 patients, reliable perimetry results were obtained in 66 patients (87%) at a median of 8 days after admittance and VFD were found in 52 cases (79%). Evaluation of VFD after 6 months revealed improvement in 52%. Patients with VFD had significantly lower composite score in VFQ-25 at both test points (77 vs 96, P = .001 and 87 vs 97, P = .009), in nine out of eleven subscales of VFQ-25 at 1 month and seven subscales after 6 months, including mental health, dependency, near and distance activities. Milder VFD had better results on VFQ-25 modified composite score (95 vs 74, P = .002).VFD improvement was related to improved VFQ-25 modified composite score (9.6 vs 0.8, P = .018). About 10% of patients with VFD reported driving 1 month post-stroke and 38% after 6 months. CONCLUSION: VFD substantially reduces multiple aspects of vision-related QoL. Severity of VFD is related to QoL and VFD improvement results in better QoL. Neglecting visual impairment after stroke may result in deterioration of rehabilitation efforts. Driving post-stroke deserves particular attention.

Neuropathologic Correlates of White Matter Hyperintensities in a Community-Based Cohort of Older Adults.

BACKGROUND: The association of white matter hyperintensities (WMH) with age-related vascular and neurodegenerative pathologies remains incompletely understood. OBJECTIVE: The objective of this work was to elucidate the neuropathologic correlates of WMH in a large community-based cohort of older adults. METHODS: Cerebral hemispheres from 603 community-based older adults were imaged with MRI ex vivo. All participants underwent annual clinical evaluation, cognitive assessment, and neuropathologic examination. WMH burden was assessed using a modified Fazekas rating scale. Multiple ordinal logistic regression was used to test the association of WMH burden with an array of age-related neuropathologies, adjusting for demographics. Mixed effects models of cognition controlling for neuropathologies and demographics were used to determine whether WMH burden contributes to cognitive decline beyond measured pathologies. RESULTS: WMH burden in the whole group was associated with both vascular and Alzheimer's disease (AD) pathologies: arteriolosclerosis (p < 10-4), gross (p < 10-4), and microscopic infarcts (p = 0.04), and amyloid-ß plaques (p = 0.028). In non-demented participants (mild or no cognitive impairment) (N = 332), WMH burden was related to gross infarcts (p = 10-4) and arteriolosclerosis (p < 10-4), but not to AD pathology. Similarly, in those with no cognitive impairment (N = 178), WMH burden was related to gross infarcts (p = 8×10-4) and arteriolosclerosis (p = 0.014). WMH burden was associated with faster decline in perceptual speed in both the whole (p = 0.038) and non-demented (p = 0.006) groups. CONCLUSION: WMH burden has independent associations with vascular pathologies in older adults regardless of clinical status, and with AD pathology later in the progression of AD. Moreover, WMH burden may reflect additional tissue injury not captured with traditional neuropathologic indices.

Effect of Unilateral Carotid Artery Stenting on Cognitive Function in Patients with Severe Bilateral Stenosis.

CASE DESCRIPTION: An 82-year-old man with dementia, gait disturbance, and a small cerebral infarction owing to severe bilateral carotid artery stenosis was successfully treated with carotid artery stenting (CAS). Preoperative cerebral vascular reactivity was reduced in the bilateral cerebral hemispheres. We performed CAS to treat right internal carotid artery stenosis. Following CAS, cerebral vascular reactivity showed an increase in the bilateral cerebral hemispheres. Memory, fluency, and attention also showed improvement. CONCLUSIONS: This case illustrates the potential benefit of single-stage CAS for cognitive function in severe bilateral carotid artery stenosis without hyperperfusion syndrome.

Neurophysiological predictors of aphasia recovery in patients with large left-hemispheric infarction: a mismatch negativity study.

BACKGROUND: Although the rehabilitation of aphasia has been extensively studied, the prediction of language outcome still has not received sufficient attention. The aim of this study was to predict the language outcome using mismatch negativity (MMN) in patients with large left-hemispheric infarction. METHODS: MMN was elicited by an oddball paradigm in which a standard tone (1000 Hz) and deviant tone (1500 Hz) were presented at 90% and 10% of the number of tones, respectively. The mean amplitudes and laterality indexes (LIs) of MMN were measured over the prefrontal, frontal, central, parietal, temporal, and perisylvian electrodes and both hemispheres during the first 7 days (session 1) and 10 to 20 days (session 2) post-onset. Mixed three-way analysis of variance (ANOVA) was used to investigate differences in these factors between two aphasia groups (the good recovery group and poor recovery group). The predictive value of the most significant LI was also compared with the score of National Institutes of Health Stroke Scale score and low-density volume on computed tomography. RESULTS: A total of 18 patients were enrolled in this study. Mixed three-way ANOVA showed no interaction effect of session × region of interest (ROI) × group (F [3.59, 57.38] = 1.301, P = 0.282) and no interaction effect of ROI × group (F [1.81, 29.01] = 0.71, P = 0.487) and session × group (F [1.00, 16.00] = 0.084, P = 0.776) for MMN amplitude. No interaction effect of session × ROI × group (F [1.79, 28.58] = 0.62, P = 0.530), but an interaction effect of session × group (F [1.00, 16.00] = 5.21, P = 0.036) was found for LIs. In the poor recovery group, the LIs of MMN over all the ROIs, except the parietal area, became more negative at session 2 than those at session 1 (P < 0.05), but this effect was not observed in the good recovery group. Additionally, significant differences were observed in the LIs at session 2 between the two groups (P < 0.05). The LI over the perisylvian area at session 2 had the highest predictive value with an area under the curve of 0.963 (95% confidence interval: 0.884-1.000). An LI score >-0.36 over the perisylvian area suggested good recovery, but a score <-0.36 suggested poor recovery. The LI cut-off value of -0.36 had the highest sensitivity (90.0%) and specificity (87.5%) for predicting a good language outcome at 3 months post-stroke. CONCLUSION: LIs of MMN amplitudes at approximately 2 weeks post left-hemisphere stroke serve as more sensitive predictors of language outcome, among which the LI over the perisylvian area exhibits the best predictive value.

A Case of Leukoencephalopathy and Small Vessels Disease Caused by a Novel HTRA1 Homozygous Mutation.

Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a heritable, rare small vessel disease, which is caused by HTRA1 mutations and mostly reported Japanese and Chinese population. CARASIL is an orphan disease, which presents with progressive motor and cognitive impairment, alopecia, and spondylosis. The disease typically starts with lumbago at early twenties. Ischemic strokes start at mid-twenties. Patients have no cardiovascular or any other risk factors. Multiple lacunar infarcts and leukoencephalopathy cause progressive neurologic involvement. Leukoencephalopathy and small vessel disease without any risk factors is a significant finding for the differential diagnosis of HTRA1 gene pathology. This report presents clinical and genetic features of a rare case of typical CARASIL from Turkey who was followed with uncertain diagnoses for years.

Anemia predicts lower white matter volume and cognitive performance in sickle and non-sickle cell anemia syndrome.

Severe chronic anemia is an independent predictor of overt stroke, white matter damage, and cognitive dysfunction in the elderly. Severe anemia also predisposes to white matter strokes in young children, independent of the anemia subtype. We previously demonstrated symmetrically decreased white matter (WM) volumes in patients with sickle cell disease (SCD). In the current study, we investigated whether patients with non-sickle anemia also have lower WM volumes and cognitive dysfunction. Magnetic Resonance Imaging was performed on 52 clinically asymptomatic SCD patients (age = 21.4 ± 7.7; F = 27, M = 25; hemoglobin = 9.6 ± 1.6 g/dL), 26 non-sickle anemic patients (age = 23.9 ± 7.9; F = 14, M = 12; hemoglobin = 10.8 ± 2.5 g/dL) and 40 control subjects (age = 27.7 ± 11.3; F = 28, M = 12; hemoglobin = 13.4 ± 1.3 g/dL). Voxel-wise changes in WM brain volumes were compared to hemoglobin levels to identify brain regions that are vulnerable to anemia. White matter volume was diffusely lower in deep, watershed areas proportionally to anemia severity. After controlling for age, sex, and hemoglobin level, brain volumes were independent of disease. WM volume loss was associated with lower Full Scale Intelligence Quotient (FSIQ; P = .0048; r2 = .18) and an abnormal burden of silent cerebral infarctions (P = .029) in males, but not in females. Hemoglobin count and cognitive measures were similar between subjects with and without white-matter hyperintensities. The spatial distribution of volume loss suggests chronic hypoxic cerebrovascular injury, despite compensatory hyperemia. Neurocognitive consequences of WM volume changes and silent cerebral infarction were strongly sexually dimorphic. Understanding the possible neurological consequences of chronic anemia may help inform our current clinical practices.