Thrombolysis in acute retinal ischemia treated with tenecteplase.

Central retinal artery occlusion (CRAO), a type of acute retinal arterial ischemia, analogous to an ocular stroke, is a medical emergency that warrants immediate diagnosis and treatment. CRAO usually presents with sudden, painless, monocular vision loss. Ipsilateral carotid artery disease is an important associated finding in these patients. The primary limitation to effective treatment of CRAO is that patients are rarely seen in the acute stage. Moreover, there are no guidelines for effective treatment. We report a patient with right CRAO whose treatment with intravenous thrombolysis with tenecteplase and anterior chamber paracentesis with ocular massage resulted in a good clinical outcome.

Utility of the RIPS Scale and 2CAN Score for In-Hospital Stroke Prediction.

Background: In-hospital strokes are a small but sizeable proportion of all strokes. Identification of in-hospital strokes is confounded by stroke mimics in as many as half of in-patient stroke codes. A quick scoring system based on risk factors and clinical signs during the initial evaluation of a suspected stroke might be helpful to distinguish true strokes from mimics. Two such scoring systems based on ischemic and hemorrhagic risk factors are the risk for in-patient stroke (RIPS) and the 2CAN score. Materials and Methods: This prospective clinical study was conducted at a quaternary care hospital in Bengaluru, India. All hospitalized patients aged 18 years and above for whom a "stroke code" alert was recorded during the study period of January 2019 to January 2020 were included in the study. Results: A total of 121 in-patient "stroke codes" were documented during the study. Ischemic stroke was the most common etiological diagnosis. A total of 53 patients were diagnosed to have ischemic stroke, 4 had intracerebral hemorrhage, and the rest were mimics. Receiver operative curve analysis was performed and at a cut-off of RIPS ≥3, it predicts stroke with a sensitivity of 77% and a specificity of 73%. At a cut-off of 2CAN ≥3, it predicts stroke with a sensitivity of 67% and a specificity of 80%. RIPS and 2CAN significantly predicted stroke. Conclusions: There was no difference in the use of either RIPS or 2CAN for differentiating stroke from mimics, and hence they may be used interchangeably. They were statistically significant with good sensitivity and specificity, as a screening tool to determine in-patient stroke.

Perry Syndrome with a Novel Mutation and a Rare Presentation: First Report from India.

Objective: To characterize the first patient of Perry syndrome reported from India. Methods: A 62-year-old gentleman presented with acute encephalopathy, hypercapnia, central hypoventilation, and seizures. He required ventilatory support for persistent respiratory failure even after the resolution of the encephalopathy. History revealed symptoms of orthostatic hypotension, episodes of shallow breathing, unsteadiness of gait, anxiety and depression, and significant weight loss for the previous two years. His mother and elder brother had succumbed to a similar illness. Investigations for neuromuscular diseases, including myasthenia and Pompes disease, were negative. Genetic tests for muscular dystrophies and myopathies, investigations for infectious, autoimmune, and para-neoplastic diseases were negative. Neuroimaging and electrophysiological studies were unremarkable. During his hospital stay, he developed rigidity and bradykinesia. Results: In view of the prominent respiratory failure, Parkinsonism, unexplained weight loss, and family history, he was tested for Perry syndrome. A heterozygous missense variation in Exon 2 of the DCTN1 gene that results in the substitution of Proline for Alanine at codon 45 (pA45P) was detected. This variant was not detected in his clinically unaffected brother. The clinical presentation and genetic test indicate Perry syndrome, a rare autosomal dominant fatal disease, which has never been reported from India. The patient improved with Levodopa and neurorehabilitation but eventually succumbed to his illness three years later. Conclusion: Perry syndrome, though rare, should be considered in the differential diagnosis of patients with a family history of Parkinsonism and central hypoventilation.

COVID-19-related strokes are associated with increased mortality and morbidity: A multicenter comparative study from Bengaluru, South India.

BACKGROUND: COVID-19-related strokes are increasingly being diagnosed across the world. Knowledge about the clinical profile, imaging findings, and outcomes is still evolving. Here we describe the characteristics of a cohort of 62 COVID-19-related stroke patients from 13 hospitals, from Bangalore city, south India. OBJECTIVE: To describe the clinical profile, neuroimaging findings, interventions, and outcomes in COVID-19-related stroke patients. METHODS: This is a multicenter retrospective study of all COVID-19-related stroke patients from 13 hospitals from south India; 1st June 2020-31st August 2020. The demographic, clinical, laboratory, and neuroimaging data were collected along with treatment administered and outcomes. SARS-CoV-2 infection was confirmed in all cases by RT-PCR testing. The data obtained from the case records were entered in SPSS 25 for statistical analysis. RESULTS: During the three-month period, we had 62 COVID-19-related stroke patients, across 13 centers; 60 (97%) had ischemic strokes, while 2 (3%) had hemorrhagic strokes. The mean age of patients was 55.66 ± 13.20 years, with 34 (77.4%) males. Twenty-six percent (16/62) of patients did not have any conventional risk factors for stroke. Diabetes mellitus was seen in 54.8%, hypertension was present in 61.3%, coronary artery disease in 8%, and atrial fibrillation in 4.8%. Baseline National Institutes of Health Stroke Scale score was 12.7 ± 6.44. Stroke severity was moderate (National Institutes of Health Stroke Scale 5-15) in 27 (61.3%) patients, moderate to severe (National Institutes of Health Stroke Scale 16-20) in 13 (20.9%) patients and severe (National Institutes of Health Stroke Scale 21-42) in 11 (17.7%) patients. According to TOAST classification, 48.3% was stroke of undetermined etiology, 36.6% had large artery atherosclerosis, 10% had small vessel occlusion, and 5% had cardioembolic strokes. Three (5%) received intravenous thrombolysis with tenecteplase 0.2 mg/kg and 3 (5%) underwent mechanical thrombectomy, two endovascular and one surgical. Duration of hospital stay was 16.16 ± 6.39 days; 21% (13/62) died in hospital, while 37 (59.7%) had a modified Rankin score of 3-5 at discharge. Hypertension, atrial fibrillation, and higher baseline National Institutes of Health Stroke Scale scores were associated with increased mortality. A comparison to 111 historical controls during the non-COVID period showed a higher proportion of strokes of undetermined etiology, higher mortality, and higher morbidity in COVID-19-related stroke patients. CONCLUSION: COVID-19-related strokes are increasingly being recognized in developing countries, like India. Stroke of undetermined etiology appears to be the most common TOAST subtype of COVID-19-related strokes. COVID-19-related strokes were more severe in nature and resulted in higher mortality and morbidity. Hypertension, atrial fibrillation, and higher baseline National Institutes of Health Stroke Scale scores were associated with increased mortality.

Etiologic spectrum of biopsy-proven peripheral neuropathies in childhood from a resource-poor setting.

There are only a few studies describing the etiologic spectrum of biopsy-proven peripheral neuropathies in children. This study reviewed the clinical, electrophysiological, and pathologic profile of 239 children (≤18 years of age) who have undergone nerve biopsy in a tertiary care centre for neurologic disorders and analyzed the etiologic spectrum and utility of nerve biopsy. The clinical profile, neuropathologic findings, and other investigations were combined to infer the final diagnosis. Neuropathy was detected in 199 biopsies; axonal pathology in 43%; demyelination in 41%; mixed pattern in 8%; and nonspecific findings in 8%. The major diagnostic categories included hereditary neuropathies (48%), heredodegenerative and metabolic disorders (27%), and inflammatory neuropathies (12%). Nerve biopsy proved most helpful in diagnosis of demyelinating and inflammatory neuropathies, reiterating its usefulness in specific situations.

Altered polysomnographic profile in juvenile myoclonic epilepsy.

PURPOSE: To study the spectra of sleep profile using PSG in a cohort of patients with JME attending a University hospital. METHODOLOGY: This prospective cross-sectional case-control study involved 25 patients of JME (age: 22.0±6.3 years; M:F=13:12) on valproic acid (VPA) and 25 matched healthy controls (age: 23.2±3.04 years; M:F=16:9) were recruited. All patients underwent clinical assessment, electroencephalogram (EEG), and evaluation with sleep questionnaire and PSG. RESULTS: PSG analysis revealed significant alterations in sleep architecture in the JME group in the form of reduced mean sleep efficiency (p=<0.035) and number of patients with reduced sleep efficiency (p=0.001), increased mean sleep onset latency (p=0.04) and number of patients with increased sleep latency (p=0.023), reduced mean N2 sleep percentage (p=0.005) and reduced mean total NREM (non-rapid eye movement) sleep (p=0.001) and increased mean wake percentage (p=0.001). The frequency of arousals, involuntary limb movements, and event related arousals in the JME groups was not different from the controls. Patients >20 years had reduced total sleep time compared to those <20 years (p=0.012). Patients with seizures for >5 years had reduced NREM sleep percentage (p=0.042) and those on VPA therapy >1 year had a longer stage 2 (p=0.03) and N3 latency (p=0.03). Patients on ≤600mg/day of VPA had a higher prevalence of isolated limb movements (p=0.01). CONCLUSIONS: PSG revealed significant alterations in sleep architecture in JME despite adequate seizure control. There was variable degree of PSG-phenotypic correlation.

Sleep disturbances in juvenile myoclonic epilepsy: a sleep questionnaire-based study.

Sleep and epilepsy share a complex pathophysiological association. Juvenile myoclonic epilepsy (JME) is a common sleep-sensitive epilepsy in which the effect of seizures could have therapeutic implications in terms of sleep disturbances and seizure control. This study aimed to analyze the effect of epilepsy on sleep in patients with JME. Fifty patients on valproic acid (VPA) monotherapy, and age- and gender-matched controls were recruited into this prospective, hospital-based, case-control study after informed consent and screening for inclusion criteria. They underwent a detailed clinical assessment, electroencephalogram (EEG) and neuroimaging, and were administered validated sleep questionnaires, which included the Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI) and NIMHANS Sleep Disorders Questionnaire. The patient and control groups had identical numbers of males and females (M: F=22: 28), without any significant difference in the age and body mass index (BMI). The clinical profile of JME was similar to published literature while the prevalence of EEG abnormalities was less compared to similar studies. The mean ESS and PSQI scores and the number of subjects with abnormal scores on one or both questionnaires were significantly more in patients. Patients had a higher prevalence of sleep disturbances, insomnia and excessive daytime somnolence. No significant seizure- or treatment-related factors influencing sleep could be identified. This study, the first of its kind, revealed that patients with JME have significant sleep disturbances characterized by excessive daytime sleepiness and disturbed night sleep, despite adequate medications and good seizure control. The role of VPA in the genesis of these symptoms needs clarification.

Spectrum of Neuro-Sjogren's syndrome in a tertiary care center in south India.

BACKGROUND: Neurological affection in Sjogren's syndrome (SS) can occur in the central and peripheral nervous system. Literature describing the neurological involvement in SS among Indian patients is lacking. MATERIALS AND METHODS: Six patients of SS fulfilling the histological or serological criteria of the American European Consensus Group for SS were studied prospectively. The patients underwent clinical examination and laboratory investigations. Their clinical and investigation features are described. RESULTS: The age of the patients ranged from 26 to 48 years, with a male to female ratio of 2:4. In our series, peripheral sensori-motor neuropathy and sensory ataxic neuropathy was seen in 3/6, mononeuritis multiplex in 2/6, cranial neuropathy in 2/6, autonomic neuropathy in 1/6, myelopathy in 4/6, optic neuropathy in 2/6, with presence of classical sicca features in 5/6 patients. Positive lip biopsy was seen in three, altitudinal field defect in one and positive Schirmer's test in five patients. Nerve conduction study abnormalities were seen in three and evidence of vasculitis was seen in nerve biopsy of one patient and chronic nonuniform axonopathy was seen in another. Antibody to Ro (SSA) or La (SSB) was positive in five patients. CONCLUSIONS: SS involves different parts of the nervous system with varied presentations. Clinical suspicion and adequate laboratory testing helps to diagnose and manage this disorder that is relatively rare in Indian patients.