Evaluation of the effect of Modafinil in the improvement of the level of consciousness in patients with COVID-19 encephalopathy: A randomized controlled trial.

AIM: COVID-19 can lead to encephalopathy and loss of consciousness. This double-blinded randomized clinical trial conducted in Tehran, Iran, aimed to assess the potential effectiveness of modafinil in patients with COVID-19-related encephalopathy. METHODS: Nineteen non-intubated COVID-19 patients with encephalopathy were randomized into two groups: a treatment group receiving crushed modafinil tablets and a placebo group receiving starch powder. Modafinil was administered at a dose of 100 mg every 2 h, reaching a peak dosage of 400 mg. The level of consciousness was assessed using the Glasgow Coma Score (GCS) at multiple time points on the day of medication administration. The trial was registered under IRCT20170903036041N3 on 23/5/2021. RESULTS: The average age in the modafinil and placebo groups was 75.33 and 70 years, respectively. No significant differences were observed between the two groups in terms of chronic conditions, clinical symptoms, or laboratory data. GCS scores were similar between the groups at baseline (p-value = 0.699). After four doses of modafinil, GCS scores were slightly higher in the treatment group, but this difference was not statistically significant (p-value = 0.581). GCS scores after each round of drug administration didn't significantly differ between the treatment and placebo groups (p-value = 0.908). CONCLUSION: Modafinil exhibited a slight improvement in the level of consciousness among COVID-19 patients with encephalopathy, although this improvement did not reach statistical significance when compared to the control group. Further research with larger sample sizes and longer treatment durations is recommended to explore modafinil's potential benefits in managing altered consciousness in COVID-19 patients.

A Novel Homozygous Variant in the MCOLN1 Gene Associated With Severe Oromandibular Dystonia and Parkinsonism.

BACKGROUND: Mucolipidosis type IV (MLIV) is a rare, progressive lysosomal storage disorder characterized by severe intellectual disability, delayed motor milestones and ophthalmologic abnormalities. MLIV is an autosomal recessive disease caused by mutations in the MCOLN1 gene, encoding mucolipin-1 which is responsible for maintaining lysosomal function. OBJECTIVES AND METHODS: Here, we report a family of four Iranian siblings with cognitive decline, progressive visual and pyramidal disturbances, and abnormal movements manifested by severe oromandibular dystonia and parkinsonism. MRI scans of the brain demonstrated signal abnormalities in the white matter and thinning of the corpus callosum. RESULTS AND CONCLUSIONS: Whole-exome sequencing identified a novel homozygous variant, c.362C > T:p. Thr121Met in the MCOLN1 gene consistent with a diagnosis of MLIV. The presentation of MLIV may overlap with a variety of other neurological diseases, and genetic analysis is an important strategy to clarify the diagnosis. This is an important point that clinicians should be familiar with. The novel variant c.362C > T:p. Thr121Met herein described may be related to a comparatively older age at onset. Our study also expands the clinical spectrum of MLIV associated with the MCOLN1 variants and introduces a novel likely pathogenic variant for testing in MLIV cases that remain unresolved.

Transcranial sonography in neurodegeneration with brain iron accumulation disorders.

BACKGROUND: Transcranial Sonography is a non-invasive technique that has been used as a diagnostic tool for a variety of neurodegenerative disorders. However, the utility and potential application of this technique in NBIA disorders is scarce and inconclusive. METHODS: In this cross-sectional retrospective case-control study, the echogenicity of Substantia Nigra (SN), Lentiform Nucleus (LN), and Diameter of the Third Ventricle (DTV) were assessed by TCS in genetically confirmed NBIA patients referring to the movement disorder clinic. The normal echogenicity area of SN was defined based on the 90th percentile of an age-and-gender-matched control group. NBIA patients underwent neurologic examination at each visit, but their brain magnetic resonance imaging and demographics were extracted from electronic records. RESULTS: Thirty-five NBIA patients of four subtypes with a mean disease duration of 10.54 years and 35 controls were enrolled. The normally defined SN echogenicity in controls was 0.23 cm2. DTV and SN echogenicity areas were significantly higher in patients compared to the controls (P = 0.002 and < 0.001, respectively). Around 85% and 63% of the patients showed LN and SN hyperechogenicity at least on one side, respectively. Disease duration was positively correlated with DTV (r = 0.422, p = 0.015). Cases with Pantothenate Kinase Associated Neurodegeneration (n = 23) also had significantly higher DTV and SN echogenicity area compared to the controls. CONCLUSION: Despite most NBIA patients displayed increased DVT and higher SN and LN hyperechogenicity than healthy controls, the discriminatory role of TCS on different NBIA subtypes remains to be determined.

Intravenous thrombolysis in a patient with acute ischemic stroke associated with a ruptured sinus Valsalva aneurysm: The first case report.

Sinus Valsalva aneurysms (SVA) are rare asymptomatic cardiac anomalies, which can rupture and cause heart failure, myocardial infarction and also, they can be a potential source for embolic strokes. We report the first case of a patient with acute ischemic stroke associated with a ruptured SVA, who was treated with intravenous thrombolysis (tPA) without further complications.

Cervical longitudinally extensive myelitis after vaccination with inactivated virus-based COVID-19 vaccine.

Myelitis, including longitudinally extensive transverse myelitis (LTEM), is reported in more than forty patients after coronavirus disease 2019 (COVID-19). Among COVID-19 vaccines, only Oxford-AstraZeneca COVID-19 vaccine (AZD1222) has been associated with few cases of myelitis (1 LTEM). We report the first case of myelitis/LTEM after BBIBP-CorV/Sinopharm vaccine, interestingly presented as a hemicord syndrome. A 71-year-old male presented with left-side diplegia, right-side hemihyposthesis with facial sparing and impaired position sensation in left foot after vaccination with BBIBP-CorV. MRI revealed a longitudinal signal in left cervical hemicord. This is the first reported myelitis and LTEM with COVID-19 vaccines other than AZD1222.

Correlates of memory complaints and personality, depression, and anxiety in a memory clinic.

The aim of the study was to find whether there is an association between subjective memory complaint and memory impairment and probable underlying psychological conditions. A total of 90 patients with subjective memory complaint enrolled in this study. Short history and demographic information were obtained and then the patients underwent memory and mental health assessments, using Wechsler Memory Scale (WMS), Hospital Anxiety and Depression Scale (HADS) and Minnesota Multiphasic Personality Inventory (MMPI) test tools. The mean age of the participants was 52.31 ± 17.97. Forty patients out of 90 (44.4%) were male. The prevalence of depression, anxiety and memory impairment was 10%, 12.2%, and 28.8%, respectively. Memory impairment has only shown a significant association with the presence of anxiety disorder according to the HADS findings (P=0.001). Regarding the MMPI, considerable differences were observed in the average grade of hysteria among patients with and without memory impairment: 8.38 ± 2.27 vs. 4.35 ± 1.96. There was also significant statistical association between the average score of depression on the MMPI in patients with and without memory impairment that were 13.7 ± 3.33 and 8.31 ±3.86, (P=0.03). The result of the current study shows that underlying psychological conditions such as anxiety, depression, and histrionic personality are associated with memory impairment.

Depression and Anxiety in a Cardiovascular Outpatient Clinic: A descriptive study.

OBJECTIVE: Cardiac diseases are psycho-somatic disorders, and psychological aspects play an essential role in their initiation and exacerbation. The aim of this study was to gain appropriate knowledge in the epidemiology of co-morbid depression and anxiety disorder in cardiovascular outpatients. METHOD: This study is descriptive with a sample of patients attending a cardio-vascular clinic. 238 individuals were included in this study using a consecutive sampling method. The study instrument was Hospital Anxiety and Depression Scale (HADS) questionnaire, which is a clinical scale for assessing anxiety and depression. RESULTS: Of the 238 participants in this study, 93(38.7%) were male and 146 (61.3%) female. 28.5% of patients suffered from anxiety disorders, and 41.9% had depression. Regarding comorbid diseases such as diabetes mellitus, hyperlipidemia and hypertension, the severity of depression was just related to hypertension. There was a meaningful relationship between gender and symptoms of anxiety so that symptoms were more severe in women. CONCLUSION: Considering the high prevalence of depression and anxiety in patients suffering from cardio-vascular diseases, it is necessary to screen psychological disorders in patients with cardio-vascular diseases and improve their cardio-vascular health and quality of life as mush as possible.