Endogenous defense mechanism-based neuroprotection in large-vessel acute ischemic stroke: A hope for future.

BACKGROUND: Stroke is a leading cause of morbidity and mortality worldwide and a leading cause of disability. None of the neuroprotective agents have been approved internationally except edaravone in Japanese guidelines in acute ischemic stroke. We here discuss that there are two types of endogenous defense mechanisms (EDMs) after acute stroke for neuromodulation and neuroregeneration, and if both can be activated simultaneously, then we can have better recovery in stroke. AIMS AND OBJECTIVES: We aimed to study the effect of combination of neuroprotection therapies acting on the two wings of EDM in acute large-vessel middle cerebral artery (LMCA) ischemic stroke. METHODS: Sixty patients of LMCA stroke were enrolled and randomized within 72 h into two groups of 30 patients each. The control group received standard medical care without any neuroprotective agents while the intervention group received standard medical care combined with oral citicoline with vinpocetine for 3 months with initial 1 week intravenous and edaravone and cerebrolysin injection, started within 72 h of onset of stroke. Patients were assessed on the basis of the National Institutes of Health Stroke Scale, Fugl-Meyer Assessment Score, Glasgow Coma Scale, and Mini-Mental Status Examination at admission, discharge, and after 90 days. RESULTS: The intervention group showed significant and early improvements in motor as well as cognitive recovery. CONCLUSION: Combination therapy for neuroprotection which is acting on two pathways of EDM can be useful in functional recovery after acute ischemic stroke.

A rare report of H-partially deficient, non-secretor phenotype from India. An Ah like the rèunion and unlike a Para Bombay-Clarifying misinterpretations.

BACKGROUND: H-deficient phenotypes are classified as H-deficient non- secretors (Bombay Oh), H-deficient secretors (Para Bombay), and H-partially deficient non-secretors (O h reunion, Ah and Bh, ABh). REPORT: We report the first case of H-partially deficient non-secretor- the Ah phenotype from India. What makes this report interesting is that they do not fit into the Bombay, or the Para Bombay series of H-deficient phenotypes and these partially deficient non-secretors were exclusively found on Réunion Island, off the East Coast of Africa in 1982. These reunion type phenotypes have not been reported since then and may lead to misinterpretations and confusions when encountered in the current existing laboratory settings especially in the low income (LIC's) and low middle income (LMIC's) countries like our own. Moreover, literature from LMIC and LIC incorrectly uses Ah/Bh for parabombay phenotypes. CONCLUSIONS: H-deficient phenotypes are rare, challenging to identify and assign correct notations. Hence, we have highlighted characteristic differences between H-deficient phenotypes and illustrated a diagnostic laboratory approach to correctly identify and assign notations to them especially in the resource constrained settings.

Behavioral variations among vascular cognitive impairment subtypes - A comparative study.

Dementia of vascular origin is a distinct variety with a heterogeneous neuropsychological profile. Very few studies have compared the behavioral dysfunction in the large vessel and small vessel vascular dementia (VaD) and studied the association between executive dysfunction and behavioral dysfunction documented in these patients, between the white matter load in small vessel disease (SVD) and the behavioral dysfunction. 76 patients having a modified Hachinski Ischemic Scale score of ≥ 4 were recruited and categorized into a small vessel and large vessel VaD. The Neuropsychiatric Inventory (NPI) score ≥ 4 per domain for defining clinically relevant symptoms and the Clinical Dementia Rating Scale (CDR) for evaluating the severity of dementia were used. Behavioral and Psychological Symptoms of Dementia (BPSD) were present in 66.67% of patients with SVD and 53.57% of those having large vessel disease. Apathy, euphoria, and disinhibition were more common in SVD, while appetite alterations were more common in large vessel disease. Behavioral dysfunction was also associated with executive dysfunction in both the VaD subtypes and with white matter loads in SVD. We conclude that different VaD subtypes have different behavioral profiles. This might help in understanding the underlying pathophysiology, diagnosis and thus better management of this disorder.

Cognitive Profile of Large-Vessel Vascular Dementia-An Observational Study from a Tertiary Care Center in Kolkata.

Introduction Vascular dementia is the second leading cause of dementia worldwide. Its heterogenous presentation along with potential for reversibility at earlier stages makes it unique among all dementias. Objectives We aimed to study the cognitive dysfunction in large-vessel vascular dementia. Second, we tried to study the cognitive dysfunction in large-vessel vascular dementia as per the arterial territory involvement. Additionally, we also tried to study the contribution of hemispheric involvement to the dementia severity as evidenced by clinical dementia rating (CDR) scale. Materials and Methods We recruited 28 patients of large-vessel vascular dementia and categorized them on the basis of the arterial territories and hemisphere involved. The groups were later studied for the type of cognitive and behavioral dysfunctions as well as the dementia severity. Results Among 28 patients of large-vessel vascular dementia, attention (100%), executive function (100%), and behavior (100%) were more impaired in anterior cerebral artery territory infarcts ( p < 0.05). Language (53.8%) and memory (53.8%) were more impaired in middle cerebral artery territory infarcts, while visuoperceptual (33.3%) domains were more impaired in posterior cerebral artery territory infarcts ( p > 0.05). The mean CDR was lower in patients of right-sided lesions (1.292) than in those with left-sided (1.750) or bilateral lesions (2.000). Conclusion Different arterial territory lesions have different patterns of cognitive impairment in large-vessel vascular dementia. The dementia severity is less in right-sided lesions when compared with left-sided or bilateral lesions.

Comparative Study of Risk Factors and Cognitive Profile of Small- and Large-Vessel Vascular Dementia - A Clinic Based Study.

Background: Vascular dementia (VaD) is a clinically heterogeneous entity. There is a dearth of studies for comparison of the cognitive profile of cerebral small-vessel disease (SVD) with large-vessel disease. Objective: We planned to evaluate and compare the cognitive profile of SVD and large-vessel VaD and evaluate various risk factors associated with them. Materials and Methods: Patients of VaD were recruited after excluding mixed and ambiguous cases. Patients were classified into SVD and large-vessel VaD and analyzed for their clinic-epidemiological and cognitive profiles. Results: Among 76 patients, 48 (62.5%) have SVD and 28 (37.5%) have large-vessel disease. Hypertension (93.4%) was the commonest risk factor, followed by smoking (34.21%), hyperlipidemia (26.31%), and diabetes mellitus (DM, 22.36%). Hypertension (P < 0.05) and DM were common in SVD, whereas smoking, hyperlipidaemia, and cardiac diseases were common in large-vessel disease. Attention (77.1% vs 25%), executive function (68.8% vs 28.6%), and calculation (58.3% vs 32.1%) were significantly more impaired in SVD compared to large-vessel disease, whereas visuoperceptual (21.4% vs 6.3%), praxis (28.6% vs 4.2%), and gnosis (14.3% vs 2.1%) were significantly more impaired in large-vessel disease than in SVD. Disruption of frontal-subcortical connection was responsible for the cognitive profile in SVD, but in large-vessel disease, it resulted from the cumulative loss of function from different lesions. Conclusions: Despite having common vascular risk factors, few are more common in SVD than in large-vessel disease. The different clinical and cognitive profile is due to the diverse anatomical lesions in these two subclasses of VaD.

Young-onset sporadic Creutzfeldt-Jakob disease with atypical phenotypic features: a case report.

BACKGROUND: Sporadic Creutzfeldt-Jakob disease, with a mean survival of 6 months, is duly considered among the most fatal neurological disorders. Rapidly progressive dementia with multi-axial involvement of the nervous system is the known presentation. Although, the peak age at onset is between sixth and eighth decades, cases of young-onset sporadic Creutzfeldt-Jakob disease have also been reported in the literature. Interestingly, these young-onset cases were reported to have some features distinct from their older age group counterparts, such as slower progression as well as longer duration of illness, dominance of psychiatric manifestations at the onset, and relatively less prevalence of radiological and electroencephalographic abnormalities. CASE PRESENTATION: We describe here the case of a 42-year-old Asian woman from India who presented with cerebellar ataxia, pyramidal and extrapyramidal involvement, followed by rapidly progressive dementia along with myoclonus, all within a span of 1 month. Probable infective, metabolic, autoimmune, and paraneoplastic etiologies were ruled out. Magnetic resonance imaging of her brain revealed bilateral caudate nucleus hyperintensity in T2/fluid-attenuated inversion recovery sequence. Diffusion-weighted imaging revealed bilateral caudate and putaminal diffusion restriction plus ribbon pattern in bilateral parieto-occipital and insular cortex. Serial electroencephalography revealed diffuse slowing of background activity along with triphasic waves in short periodic interval. Cerebrospinal fluid was tested positive for 14-3-3 protein. Based on these findings, a diagnosis of sporadic Creutzfeldt-Jakob disease was made. CONCLUSION: Our patient represents an atypical clinical situation as she is much younger than the usual presentation of Creutzfeldt-Jakob disease and it progressed far too rapidly. Cognitive decline came late in the temporal sequence of clinical events; rather, the onset was dominated by features consistent with cerebellar ataxia and basal ganglia involvement. The presence of magnetic resonance imaging abnormality and electroencephalography changes are other rare findings in young-onset sporadic Creutzfeldt-Jakob disease.