Annals of Indian Academy of Neurology最新文献

Stiff-person syndrome (SPS) is a rare and complex neurologic disorder characterized by progressive muscle stiffness, painful spasms, and gait difficulties. In this report, we describe a case of SPS who presented with a relapse while on maintenance immunosuppressive treatment. In addition, we review the literature of 16 previously reported cases of SPS from India, highlighting the diverse clinical features, comorbidities, treatment response, and relapse. The occurrence of paraneoplastic SPS emphasizes the need for early recognition and diagnosis.

Carotid stump is blind remnant of occluded proximal segment of the internal carotid artery (ICA) that can become the potential source of embolism. Carotid stump syndrome is a potentially treatable cause of recurrent ischemic events in the carotid territory in the setting of occlusion of the ipsilateral ICA. It is thought to be caused by turbulent blood flow in the patent stump of the occluded ICA causing microemboli migrating in the brain through external carotid-ophthalmic anastomotic channels and retrograde flow. Here, we report a patient with known ipsilateral chronic ICA occlusion, who was on best medical management, presented on two separate occasions with recurrent embolic infarctions in ipsilateral carotid territory. She was diagnosed with carotid stump syndrome and treated through endovascular route with clinical and angiographic follow-up.

A 14-year-old girl with congenital hydrocephalus and early-onset scoliosis presented with sudden onset of severe headache on the fourth postoperative day of scoliosis correction (definitive fusion). On evaluation, she was found to have cerebellar haemorrhage on computed tomography scan with the findings of obstructive hydrocephalus. Posterior fossa bleed with hydrocephalus contributing to raised ICP was suspected initially. Headache persisted despite treating the patient with analgesics and antioedema measures. There was no history of postural variation of headache. As the drain output was not coming down, even by the sixth postoperative day, low cerebrospinal fluid pressure headache was considered and the drain was removed, which resulted in marked improvement of headache. Subsequent wound exploration revealed grade III dural tear at D10 level, which was repaired and the headache subsided completely. The linear pattern of haemorrhage in the cerebellum is classical of remote cerebellar haemorrhage, which is seen rarely following spinal surgeries with associated dural tear.

Dravet syndrome (DS) is a developmental epileptic encephalopathy, characterized by fever-triggered focal or hemiclonic seizures at onset with various associated comorbidities like intellectual disability, gait abnormalities, and behavioral issues. It typically advances to drug-refractory epilepsy with multiple seizure semiology. In this review, we give a focused narrative on the treatment aspects of DS. We searched the PubMed database for articles on DS. More than 500 articles were reviewed, of which 55 relevant articles are included in this review. ClinicalTrials.gov database was also accessed for data on ongoing trials. Majority are caused by mutations in the SCN1A gene. Valproate and clobazam are the most commonly used traditional antiseizure medications. Stiripentol, fenfluramine, and cannabidiol are recently approved drugs with promising results. Ketogenic diet and vagus nerve stimulation are commonly tried nonpharmacologic modalities that have shown significant responses. Antisense oligonucleotides and viral vector-mediated gene transfer therapies are on the horizon. This review outlines the current existing treatment rationale, evidence for newly approved drugs, and the future scope of gene therapy in DS.

Background: Bacterial meningoencephalitis presents significant diagnostic and therapeutic challenges with high morbidity and mortality in pediatric populations worldwide. The early and precise identification of the etiology of these infections is essential for effective treatment and better patient results. Traditional diagnostic methods, while effective, can be time-consuming. This manuscript aims to evaluate the accuracy of serum procalcitonin (PCT), cerebrospinal fluid (CSF) neutrophil-to-lymphocyte ratio (NLR), and CSF lactate as biomarkers in pediatric bacterial meningoencephalitis.

Methods: From March 2021 to November 2023, a cross-sectional study was conducted at Dr. Sardjito General Hospital, a tertiary referral hospital in Yogyakarta, Indonesia. One hundred ninety-seven patients underwent complete clinical and laboratory examinations before being divided into bacterial and non-bacterial groups based on CSF culture results and cytochemical profiles. The diagnostic accuracy was evaluated by the receiver operating characteristic curve using Statistical Package for the Social Sciences.

Results: Serum PCT, CSF NLR, and CSF lactate levels showed a notable increase in the bacterial meningoencephalitis group (mean = 4.63 ± 5.52 ng/ml, 4.39 ± 6.68, and 3.59 ± 2.38 mmol/l, respectively) compared to the viral/aseptic group (mean = 0.51 ± 0.88 ng/ml, 0.33 ± 0.95, and 2.25 ± 2.33 mmol/l, respectively) ( P < 0.001). Serum PCT and CSF NLR combined measurement had high sensitivity (86.4%) and specificity (88.6%), with an area under the curve of 0.929 (95% confidence interval, 0.873-0.985), surpassing other tested biomarkers.

Conclusion: The findings suggest that combining serum PCT and CSF NLR could be beneficial for early diagnosis, potentially allowing timely, targeted treatment and differentiating between bacterial and non-bacterial infections, ultimately improving patient outcomes.

Background: Acute kidney injury (AKI) is prevalent in patients with acute stroke. Although AKI is linked to poor clinical outcomes, data about its incidence and effect on stroke outcomes is limited.

Methods: This was a prospective observational study carried out at a single tertiary care center that analyzed the data of 204 consecutive subjects with acute ischemic stroke and intracerebral hemorrhage. Considering serum creatinine at admission as the baseline, AKI was defined as a rise in serum creatinine value of 0.3 mg/dl over 48 h or a percentage increase of at least 50% from baseline over 7 days during hospitalization. The primary outcome was to measure the prevalence of AKI in patients with acute stroke. Secondary outcome measures were all-cause mortality, duration of hospital stay, need for dialysis, and comparison of outcomes in ischemic and hemorrhagic stroke. For both the stroke subtypes, we employed a multivariate logistic regression model, with AKI and hospital mortality being the outcomes. Covariates included gender, age, ventilatory requirement, duration of hospital stay, and National Institutes of Health Stroke Scale score at admission.

Results: There were 144 cases of ischemic stroke with 12 deaths (8.3%) and 60 cases of intracranial hemorrhage (ICH) with 22 deaths (36.7%). The mean age was 55 years, 72.6% were males, and AKI complicated 34% of ischemic stroke and 66.7% of ICH hospitalizations. AKI was linked to increased hospital mortality from ischemic stroke (odds ratio [OR] 27.21, 95% CI 3.39-218.13) and hemorrhagic stroke (OR 5.12, 95% CI 1.29-20.28) in multivariate analysis stratified by stroke type.

Conclusions: AKI complicates stroke frequently and increases hospital mortality. Additional studies are required to assess if the association is causal and if remedies to prevent AKI would decrease mortality.

Background and aims: Tenecteplase is used as the standard of care treatment for thrombolysis in acute ischemic stroke (AIS) patients within 4.5 h of symptom onset. Documented reports were less certain to claim the benefits of it in an extended window period. EAST-AIS (CTRI/2022/03/040718) trial is designed to determine the success rate of thrombolysis in an extended window period for good clinical outcomes.

Study design: It is a randomized, placebo-controlled trial of tenecteplase administered within 4.5-24 h of stroke onset (with or without large vessel occlusion) based on evidence of salvageable tissue through baseline computed tomography perfusion (CTP) or magnetic resonance imaging (MRI) scan. Criteria of patient inclusion are as follows: patients of both genders (male and female), age >18 years, pre-stroke modified Ranking Scale (mRS) <2, baseline NIHSS >5, CTP showing penumbra-ischemic core ratio >1.8, absolute difference in volume >10 ml, and ischemic core volume <70 ml. The sample size for the study is 100 patients: 50 in the tenecteplase arm (0.25 mg/kg body weight; maximum- 25 mg) and 50 in the placebo arm (controls).

Study outcomes: The study's primary objective is safety endpoints along with the efficacy of tenecteplase assessed using the mRS score at 90 days of stroke onset.

Conclusion: The result obtained from EAST-AIS will determine the safety and efficacy of tenecteplase injection administered 4.5-24 h following the symptom onset for AIS patients within the territory of Internal Carotid Artery (ICA), Middle Cerebral Artery (MCA), or Anterior Cerebral Artery (ACA) occlusion.