Anti-NMDA Autoimmune Encephalitis Post-COVID-19 Vaccination in a Pediatric Patient: A Case Report.

Post-coronavirus disease 2019 (COVID-19) vaccination encephalitis is rarely reported particularly in the pediatric population. Herein, we report the first case of postvaccination anti-N-methyl-d-aspartate (NMDA) encephalitis in close temporal association with receiving COVID-19 vaccine in a pediatric patient. The patient is a 13-year-old female who received the first dose of the Pfizer-BioNTech COVID-19 vaccine and presented with subacute neurological and psychiatric symptoms and eventually confirmed the diagnosis of anti-NMDA autoimmune encephalitis. The patient recovered after receiving intravenous immunoglobulins and steroids.

Methylmalonic aciduria as a biochemical marker for mitochondrial DNA depletion syndrome in patients with developmental delay and movement disorders: a case series.

Background: Mitochondrial DNA (mtDNA) depletion syndromes (MDDS) are genetically and clinically variable disorders resulting from a reduction in mtDNA content in the cells, tissues, and organ systems, leading to symptoms related to energy deficits. Deficiency of the mitochondrial succinyl-CoA ligase/synthetase enzyme secondary to pathogenic variations in the SUCLG1 and SUCLA2 genes is a subtype of MDDS that presents with neurological manifestations and a specific biochemical profile. Methods: This cross-sectional series describes five patients with MDDS secondary to pathogenic variations in the SUCLG1 and SUCLA2 genes from two tertiary care centers in Canada and India. Clinical data concerning the course, investigations, and outcome were gathered through chart reviews. Results: All subjects presented in early infancy with neurological manifestations, including movement disorder, psychomotor regression, developmental delay, hearing loss, behavioral issues, or a combination thereof. Elevated methylmalonic acid metabolites, an abnormal acylcarnitine profile, and lactic acidemia were noted in the biochemical profile of each patient (n = 5/5, 100%). Molecular genetic testing disclosed the presence of pathogenic homozygous mutations in four subjects and compound heterozygosity in one subject. Conclusion: MDDS associated with SUCLG1 and SUCLA2 genes can be detected biochemically by the presence of methylmalonic aciduria besides the elevation of lactate, C3, C4DC, and C5-OH acylcarnitine. Conducting metabolic workups including MMA and acylcarnitine profiles in patients with heterogeneity of clinical symptoms associated with the presence of this biochemical marker may potentially reduce the time to diagnosis and management.

Does the duration of video-EEG recording influence diagnostic yield in pediatric epilepsy: Results from a single center study.

AIM: This exploratory study examines the association of the duration of electroencephalography (EEG) recordings to diagnostic yield in children undergoing evaluation for seizures. METHOD: Clinical and EEG data on three hundred and ten patients (167 males and 143 females) were reviewed retrospectively. 134 (43.2%) children with focal-onset seizures, and 59 (19%) children with generalized-onset seizures. The mean duration of recordings in studies interpreted as "diagnostic" was compared to studies that were interpreted as "non-diagnostic". EMU recordings were also compared to routine EEG studies to identify the relationship between routine EEG and diagnostic studies. RESULTS: The principal finding of this study indicates that a longer duration of monitoring is more likely to be associated with a positive diagnostic yield. Mean duration of recording in children with a "non-diagnostic study" was 31.05 hours versus 44.27 hours; p < 0.001 in a "diagnostic study". EMU recordings are likely to be diagnostic with longer epilepsy duration (2.6 years vs 3.7 years; p < 0.01). A diagnostic EEG from a prolonged recording is more likely to be achieved in children with abnormal routine EEG and focal-onset seizures. p < 0.001. INTERPRETATION: Tailoring the optimal duration of EEG recordings and factoring in confounding variables will reduce the need for repeated studies, improving diagnostic utility and permitting efficient utilization of resources.

Moyamoya Syndrome in an Infant with Aicardi-Goutières and Williams Syndromes: A Case Report.

Stroke in infancy is a rare phenomenon but can lead to significant long-term disability. We present the story of a 6-month-old Old Order Amish infant with underlying Williams syndrome, a rare neurodevelopmental disorder caused by a microdeletion, encompassing the elastin gene that produces abnormalities in elastic fibers of the lungs and vessels. This infant presented with lethargy, irritability, and a new-onset generalized tonic-clonic seizure. Brain magnetic resonance imaging (MRI) was consistent with ischemic stroke in the supratentorial regions. MR angiogram demonstrated bilateral narrowing of the internal carotid arteries with "ivy sign," suggestive of Moyamoya. Moyamoya disease/syndrome is a cerebrovascular condition that is associated with progressive stenosis of the intracranial vessels and can cause ischemic stroke in young children. Targeted mutation analysis revealed a homozygous c.1411-2A > G splice site variant in the SAMHD1 gene, consistent with a diagnosis of Aicardi-Goutières syndrome type 5 (AGS5), an autosomal recessive condition with multisystem involvement. In our unique case of infantile stroke with Moyamoya syndrome and dual diagnosis of Williams syndrome and AGS5, both diagnoses likely contributed to the cerebrovascular pathology. This case report highlights the importance of suspecting and testing for multiple genetic abnormalities in children presenting with Moyamoya-related stroke.

Degree of Conversion of Nanoceramic and Microhybrid Composites Activated by Different Polymerization Modes at Different Intervals: An In Vitro Comparative Study.

INTRODUCTION: This is an in vitro study to evaluate the degree of conversion (DC) of nanoceramic and microhybrid composites activated by different polymerization modes at different intervals. AIM: To determine the DC of two resin composites at different time intervals and modes of polymerization on DC. MATERIALS AND METHODS: Two commercially available composite resins-nanoceramic (Ceram X, Dentsply) and microhybrid (Spectrum TPH, Dentsply)-were used in this study. A total of 80 cylindrical samples (n = 40 each) were made by packing the composites in one increment into (5 × 2 mm) Teflon molds and cured using a light-emitting diode (LED) at an irradiance of 1,200 mW/cm2. Each group was further divided into two groups (n = 20) based on the curing modes (20 seconds, 40 seconds). These 20 samples were further subdivided (n = 10) based on the time interval (2 days, 7 days). Degree of conversion was assessed by Fourier-transform infrared spectrophotometer (FTIR) using a direct technique (attenuated total reflectance) on the top surface of the samples. Degree of conversion was measured for samples cured for 20 seconds (n = 10) and 40 seconds (n = 10) after 2 days and 7 days during which the samples were stored in an incubator at 37°C, 90% ± 10% relative humidity. The results obtained were statistically analyzed using two-way and three-way analysis of variance (ANOVA) test and p value set at (<0.005) significance level. RESULTS: Degree of conversion for the tested composites varied between 44% and 55% for Ceram X and 42% and 45% for Spectrum TPH. A significant difference was observed in DC for Ceram X samples for different polymerization modes (20 seconds and 40 seconds) as well as at 2-7 days measurement, whereas Spectrum TPH did not show any significant difference for polymerization modes. CONCLUSION: Ceram X demonstrated high DC values compared with that of Spectrum TPH with regard to two different polymerization modes. CLINICAL SIGNIFICANCE: Lesser the time required for conversion and less the residual monomer left, better is the prognosis of the restoration, and better is the patient satisfaction.

Awareness regarding childhood asthma in Saudi Arabia.

OBJECTIVE: Assessing the knowledge and awareness of the Saudi society about bronchial asthma in children. METHODS: Structured questionnaires were randomly distributed to 1039 Saudi Arabians in May 2014 at Jeddah, Riyadh, and Dammam. RESULTS: The awareness of bronchial asthma questions showed that 67% of total sample thought that it could be a fatal disease, and only 13.2% thought that there is a difference between bronchial asthma and chest allergies in children. 86.1% thought that the symptoms of bronchial asthma include dyspnea and nocturnal cough, and 45.7% thought that fever, a runny nose and throat inflammation are not symptoms. 60.2% thought that infectious respiratory diseases may increase bronchial asthma progression. In addition, 40% thought that the use of antibiotics doesn't help in diminishing bronchial asthma complications, and some thought that the patient can stop medication after an acute asthma attack. 34.1% thought that inhaled medication for asthma doesn't cause addiction. Very highly significant results are shown between bronchial asthma knowledge and age, the level of education, marital status, and if the individual knows a person who suffers from bronchial asthma (P < 0.001). There are positive correlations between bronchial asthma knowledge and age, marital status, and level of education (r = 0.152, 0.150, 0.197), respectively. CONCLUSION: The study demonstrated that bronchial asthma knowledge in the Saudi Arabian population is insufficient, and efforts should be carried out to spread bronchial asthma management.

Autologous bone marrow transplanation for extramedullary plasmacytoma presenting as adrenal incidentaloma.

Extramedullary adrenal plasmacytoma (EMP) involving the adrenal glands is rarely encountered clinically. We report a A 47-year-old male who presented with bilateral adrenal incidentalomas. After confirming EMP, the patient received two consecutive autologous hematopoietic stem cell transplants (HSCT) using high-dose melphalan. Following HSCT, a serial follow-up helical CT revealed a substantial decrease in the size of both adrenal masses. Serial periodic serum protein and urine electrophoresis and immunofixation showed abrogation of a previously noted monoclonal band. At 50 months follow-up the patient was alive and well. Our patient is the first with EMP to have received an autologous HSCT, which may prove to have a role in therapy due to the immunological effect of the infused donor marrow T-lymphocytes against the clonal proliferation of abnormal plasma cells in extrammedullary sites.This case indicates that an EMP should be added to the differential diagnosis of adrenal incidentalomas.

Dental caries experience in patients with aggressive periodontitis compared to those with chronic periodontitis.

OBJECTIVE: It has been reported that caries prevalence in patients with aggressive periodontitis (ie, juvenile periodontitis) might be higher than that in matched controls. However, the available results are inconclusive. The aim of this study was to assess this relationship by comparing caries experience among subjects with generalized aggressive periodontitis (AgP) and those with chronic periodontitis (CP) and to identify reasons for tooth loss in both groups. METHOD AND MATERIALS: A convenient sample of subjects with AgP (210) and CP (332) was evaluated for decayed, missing, and filled teeth (DMFT) by both clinical and radiographic methods. Statistical analysis was performed by t test and ANOVA. RESULTS: The AgP subjects have significantly less caries experience than the CP subjects older than 20 (P < .001). Tooth loss was mainly due to periodontal disease in AgP subjects and caries in CP subjects. CONCLUSION: Subjects with AgP have significantly less caries experience than subjects with CP over 20 years of age (P < .001).

Clinicopathologic features of hepatic neoplasms in explanted livers: a single institution experience.

BACKGROUND: Hepatic neoplasms can be the primary indication for hepatic transplantation. The tumors can also be incidentally identified in explanted livers. We explored the clinicopathologic features of hepatic neoplasms identified in explanted livers. MATERIALS AND METHODS: All explanted livers resected between 2001 and 2006 were evaluated for the presence of neoplasms and their clinicopathologic features were examined. RESULTS: In 98 liver transplants, 15 neoplasms (15.3%) were identified. Patient ages ranged from 5 to 63 years (median, 56 years). The primary etiology of hepatic disease was hepatitis C virus in 12 cases, hepatitis B virus in 1 case, cryptigenic cirrhosis in 1 case and congenital hepatic fibrosis in 1 case. Serum alpha-fetoprotein was significantly elevated (>400 U/L) in only 2 cases. CA19-9 was not elevated in any of the cases. The tumors included hepatocellular carcinoma (HCC) in 13 cases, 1 case of choloangiocarcinoma and 1 case of combined HCC and hepatoblastoma. The tumors in size from 0.5 to 5 cm (median 1.4 cm) and were multifocal in 5 of the cases (33%). Tissue alpha-fetoprotein expression was only seen in the cases associated with elevated serum levels. CONCLUSION: In our institution hepatic neoplasms are seen in more than 15% explanted livers. They can be incidentally indentified, are frequently not associated with elevated serum levels of alpha-fetoprotein and CA19-9, are commonly multifocal but small, and are associated with good prgonosis. Elevated serum alpha-fetoprotein, albeit specific, is not a very sensitive marker in the detection of hepatic neoplasms.

Oral disease status of a sample of Jordanian people ages 10 to 28 with cleft lip and palate.

OBJECTIVE: To determine the levels of periodontal disease and dental caries in subjects with cleft lip and palate and to compare them with matched noncleft control subjects. DESIGN: A total of 32 subjects with cleft lip and palate, ages 10 to 28 years, and a similar number of noncleft control subjects were examined for plaque biofilm deposits, gingivitis, periodontitis, and dental caries by using the Silness and Löe plaque index (PI), Löe and Silness gingival index (GI), probing pocket depth (PPD), and the decayed-missing-filled-teeth (DMFT) index, respectively. SETTING: Faculty of Dentistry, Jordan University of Science and Technology, and Prince Rashed Hospital, Royal Medical Services, northern Jordan. RESULTS: Scores for PI, GI, PPD, and DMFT were significantly higher in subjects with cleft lip and palate than in control subjects. CONCLUSION: Subjects with cleft lip and palate are at an increased risk for dental caries and periodontal disease when compared with a noncleft population.

Dental diseases in a Jordanian population on renal dialysis.

OBJECTIVE: The aim of this study was to examine the levels of periodontal disease and dental caries in individuals on renal dialysis in a Jordanian population. METHOD AND MATERIALS: A total of 47 individuals (mean age = 42.9 years, standard deviation [SD] 12.5) were examined for plaque deposits, gingivitis, periodontitis, and dental caries using the Silness and Löe plaque index (PII), Löe and Silness gingival index (GI), probing pocket depth (PPD), and decayed, missing, or filled teeth (DMFT), respectively. Gingival recession also was recorded using the distance from the cementoenamel junction to the gingival margin. Patients were categorized into three subgroups based on their renal dialysis histories: on dialysis for less than 1 year; on dialysis for 1 to 3 years; or on dialysis longer than 3 years. RESULTS: There were no statistically significant differences in PII, GI, PPD, and gingival recession among the three subgroups examined. The GI revealed that 55% scored more than 2, indicating moderate to severe gingivitis. None of the individuals in this study displayed an absence of gingival inflammation. DMFT index showed that there were statistically significant differences between subjects on renal dialysis for less than 1 year and subjects on dialysis for 1 to 3 years and more than 3 years. The group DMFT index mean was 8.5 (SD 2.88). CONCLUSION: Gingival and dental diseases are prevalent in the renal dialysis population. The dental community's awareness of the implication of poor oral health within this population should be elevated and daily oral health maintenance should be reinforced by the dialysis team.

Cephalometric measurements and facial deformities in subjects with beta-thalassaemia major.

This study was performed to identify cephalometric and facial features of patients with beta-thalassaemia major. A total of 54 thalassaemic subjects were examined for craniofacial deformities, including 37 patients (24 males and 13 females, aged 5-16 years) who had lateral cephalometric radiographs. The thalassaemic groups were compared with a normal control group matched for sex and dental age, using a t-test. All thalassaemic patients had a Class II skeletal base relationship. The average ANB angle was significantly larger than the controls in dental stages 2 and 3 (P < 0.05). Mandibular base length (Ar-Gn) was significantly less in thalassaemic patients than in controls, with the greatest differences (P < 0.001) found in the younger age group. The maxilla was of normal length (PNS-ANS, Ptm'-ANS') and appeared prominent (3.3 mm in males and 5.1 mm in females) due to a reduced cranial base length (Ar'-S') and a short mandible (Ar'-P'). Vertically, thalassaemic patients showed a significantly increased maxillary/mandibular planes angle in all groups, with differences ranging between 6.19 and 12.55 degrees (P < 0.001). Thalassaemic patients also showed a reduced posterior facial height (S-Go, Ar-Go) and increased anterior facial proportions. Of the 54 thalassaemic patients examined, 17 per cent had severe facial disfigurements (grade 3).