Annals of Indian Academy of Neurology最新文献

Background: Contrast-induced encephalopathy (CIE) is a rare adverse event linked to intravascular use of iodine-containing contrast media. The prevalence of CIE could increase in the future due to growing numbers of endovascular procedures. We provide insights from a case series of 7 patients.

Methods: Cases from 3 centers were collected based on existing academic collaborations, and key factors were extracted to illustrate development and management of CIE.

Results: In our retrospective case-series analysis of 7 cases from 3 countries, affected patients had an equal distribution of sex (4 women, 3 men) and a median age of 75 (IQR 63-77). Common risk factors included hypertension (5/7), hyperlipidemia (5/7), previous stroke (3/7), and type 2 diabetes (3/7). CIE developed in 3 cases after endovascular thrombectomy (EVT) for stroke, in 2 cases after aneurysm treatment, in 1 case after cardiac catheterization, and in 1 case after diagnostic computed tomography (CT) angiography without an endovascular procedure. The median procedure time was 48 min (IQR 40-81). All patients received non-ionic, low-osmolar contrast agents with volumes ranging from 100-300 ml. Symptom onset was close to contrast administration, with stroke-like neurological deficits being most common (4/7). Prednisolone was the most frequently used medication to treat the symptoms (4/7). Symptom resolution occurred in 4 out of 7 patients within two to several days, and 1 patient died, but without clear connection to CIE.

Conclusion: CIE is a rare and possibly underrecognized condition, but fortunately, with a favorable outcome in most cases.

Background: Hirayama disease (HD) is a rare benign type of cervical cord myelopathy occurring commonly in young males as unilateral or bilateral asymmetrical amyotrophy of the hand and forearm muscles in C8-T1 distribution. Magnetic resonance imaging (MRI) is the best technique for the evaluation and imaging of this entity.

Materials and methods: This is a retrospective review of cervical magnetic resonance images of patients that were taken for clinically suspected and diagnosed HD on 3T MRI in postcontrast neutral and flexion (30°-40°) positions from July 2019 to January 2024 at Shri Ram Murti Smarak Institute of Medical Sciences, Bareilly.

Results: Fourteen patients included in the study were males less than 34 years of age. MRI findings of cord atrophy in the lower cervical region/cervico-dorsal junction, abnormal cervical curvature, loss of attachment of the dorsal dural sac and subjacent laminae with anterior displacement, and a prominent intense enhancing posterior epidural space were observed in all 14 patients. The minimum anteroposterior cord diameters in the neutral and flexion positions were 2.9 and 2.8 mm, respectively (mean thickness of laminodural space on flexion - 5.2 mm). Other MRI findings showed variable representations.

Conclusions: Flexion-position MRI has emerged as the gold standard for establishing and validating the diagnosis of HD in clinically suspected cases and should be an essential part of the protocol for the screening of clinically suspected cases of HD to aid in early treatment and therapeutic intervention. Complimentary newer sequences such as the Three-dimensional (3D)-Constructive interference in Steady State (CISS)/Fast Imaging Employing Steady-state Acquisition Cycled Phases (FIESTA-C) may reinforce better appreciation of epidural flow voids.

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.

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.