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INTRODUCTION: Elevated admission blood pressure (BP) and BP variability have been shown to be associated with poor outcomes in most studies, while few studies have not found such an association. We investigated the association of various BP parameters with 3-month functional outcomes, mortality, and symptomatic intracerebral hemorrhage (sICH) in patients with acute ischemic stroke (AIS) receiving intravenous thrombolysis (IVT). METHODS: Patients with AIS who received IVT between January 2012 and March 2020 were analyzed. Admission BP, 24 h mean BP, and BP variability were noted for all patients. Outcomes assessed were 3-month modified Rankin Scale (mRS), mortality, and sICH. RESULTS: Of the 237 patients, 161 were males. The mean admission systolic BP (SBP), 24 h mean SBP, and BP variability were 158.1 mm Hg (SD 30.25), 138 mm Hg (SD 16.78), 19.42 (SD 12.79), respectively. At 3 months, 147 (62%) patients had a good outcome (mRS 0-2). Multivariate analysis showed prior stroke, NIHSS >15, mean SBP ≥160 mm Hg, and SBP variability >45 to be significant predictors of poor outcome (mRS > 2) at 3 months. Eleven patients (4.6%) developed sICH. Age more than 60 years, presence of atrial fibrillation, admission blood sugar ≥180 mg/Dl, and SBP variability >45 predicted sICH. None of the above factors were predictive of 3-month mortality. CONCLUSION: An elevated mean SBP and greater SBP variability were predictive of poor functional outcomes, whereas a high SBP variability predicted sICH. Our study emphasizes the need for BP control and minimizing large fluctuations to achieve good poststroke outcomes.

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INTRODUCTION: Carotid Endarterectomy (CEA) is the standard treatment for patients with symptomatic carotid stenosis. Data from Low- and Middle-Income Countries (LMIC) is sparse on CEA and its outcomes. We aimed to describe the profile of our patients, and factors associated with periprocedural cerebral ischemic events in patients with symptomatic carotid stenosis who underwent CEA in our institute. METHODS: Retrospective review of patients with symptomatic carotid stenosis(50-99%) who underwent CEA between January 2011 and December 2021 was done. Clinical and imaging parameters and their influence on periprocedural cerebral ischemic events were analysed. RESULTS: Of the 319 patients (77% males) with a mean age of 64 years (SD ±8.6), 207 (65%) presented only after a stroke. Majority (85%) had high grade stenosis (70%) of the symptomatic carotid. The mean time to CEA was 50 days (SD ±36), however only 26 patients (8.2%) underwent surgery within 2 weeks. Minor strokes and TIA occurred in 2.2%, while major strokes and death occurred in 4.1% patients. None of the clinical or imaging parameters predicted the periprocedural cerebral ischemic events. The presence of co-existing significant (50%) tandem intracranial atherosclerosis (n=77, 24%) or contralateral occlusion (n=24, 7.5%) did not influence the periprocedural stroke risk. CONCLUSION: There is a delay in patients undergoing CEA for symptomatic carotid stenosis. Majority have high grade stenosis and present late only after a stroke reflecting a lack of awareness. CEA can be performed safely even in patients with significant intracranial tandem stenosis and contralateral carotid occlusion.

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Primary Angiitis of the Central Nervous System (PACNS) is an aggressive disease with a high rate of relapse and mortality. Majority of patients attains clinical remission with a combination of glucocorticoids and cyclophosphamide. However, there is limited evidence on further management in patients who relapse or does not achieve clinical improvement while on first line treatment. Here, we present two cases of PACNS in whom clinical course was complicated by recurrent strokes and radiological progression despite receiving optimal immunosuppression with glucocorticoids and cyclophosphamide. Rituximab, a monoclonal antibody, as second line agent was administered with which both patients had clinical improvement and was relapse free at one year followup.

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Dopa-responsive dystonia (DRD) and DRD plus are diseases of the dopamine pathway with sizeable genetic diversity and myriad presentations. DRD has onset in childhood or adolescence with focal dystonia, commonly affecting lower limb, diurnal fluctuations with evening worsening of symptoms and a demonstrable sleep benefit. DRD "plus" has "atypical features" which include infantile onset, psychomotor delay, cognitive abnormalities, oculogyric crisis, seizures, irritability, spasticity, hypotonia, ptosis, hyperthermia and cerebellar dysfunction. Neurodegeneration, however, is not a feature of either DRD or DRD-plus disorders. Tetrahydrobiopterin (BH4), a key cofactor, deficiency leads to inadequate dopamine and serotonin synthesis. Norepinephrine deficiency may coexist, depending on the enzyme defect. Hyperphenylalaninemia (HPA) is a clue for BH4 paucity. However, HPA is conspicuously absent in autosomal-dominant guanosine triphosphate cyclohydrolase 1 deficiency and sepiapterin reductase deficiency. DRD look-alike is a group of neurodegenerative disorders involving the nigrostriatal dopaminergic system, which could present with dystonia responsive to dopaminergic drugs or neurodegenerative or non-neurodegenerative disorders without involving the nigrostriatal dopaminergic system yet responsive to levodopa. Although levodopa is the mainstay of therapy, response to this drug can be unsatisfactory in DRD plus and DRD look-alike and other drugs are tried. Simultaneous management of HPA leads to remarkable improvement in both motor and cognitive functions. The aim of this review is to help neurology practitioners in treating patients with DRD, DRD-plus and DRD look-alike as many of them have excellent outcome with appropriate therapy.

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