scholarly journals Syringomyelia Developing as an Acute Complication of Tuberculous Meningitis

1997 ◽  
Vol 24 (1) ◽  
pp. 73-76 ◽  
Author(s):  
Abdu Kader Daif ◽  
Al Rajeh ◽  
Adesola Ogunniy ◽  
Amer Al Boukai ◽  
Abdulrahman Al Tahan
Keyword(s):  
Nervous System ◽  
Tuberculous Meningitis ◽  
Early Onset ◽  
Late Onset ◽  
Acute Onset ◽  
Limb Weakness ◽  
Inflammatory Edema ◽  
Underlying Mechanisms ◽  
Tuberculosis Meningitis ◽  
Uncommon Complication

ABSTRACT:Background:Tuberculosis of the nervous system has protean manifestations. Syringomyelia, though an uncommon complication of it, is usually of late onset.Methods:We report two patients with tuberculosis meningitis who developed syringomyelia acutely. The diagnosis was supported by neuroimaging and findings at laminectomy.Results:The two patients developed syringomyelia between 11 days and 6 weeks of the onset of tuberculous meningitis. They both had cord swelling and softeningConclusions:Acute-onset syringomyelia should be suspected in any patient being treated for tuberculosis meningitis who subsequently develops limb weakness and/or sphincteric dysfunction. Inflammatory edema and cord ischemia appeared to be the underlying mechanisms in these early onset cases rather than arachnoiditis which is important in late-onset cases.

scholarly journals POS0707 SALIVARY GLAND ULTRASONOGRAPHY AND THE CLINICAL FEATURES USING ESSDAI IN PATIENTS OF EARLY-ONSET VERSUS LATE-ONSET WITH PRIMARY SJÖGREN’S SYNDROME

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 603.1-603
Author(s):  
N. Hashimoto ◽  
S. Uchiyama ◽  
T. Nakazawa ◽  
T. Iwasaki ◽  
T. Hashimoto
Keyword(s):  
Nervous System ◽  
Salivary Gland ◽  
Disease Activity ◽  
Early Onset ◽  
Late Onset ◽  
Primary Sjögren’S Syndrome ◽  
Onset Age ◽  
Primary Sjogren’S Syndrome ◽  
European League Against Rheumatism ◽  
European League

Background:Primary Sjögren’s syndrome (pSS) is a chronic inflammatory autoimmune disease characterized by lymphocyte infiltration in salivary and lacrimal glands. pSS affects primarily middle-aged and elderly patients, although younger age groups may also be involved. However, differences of etiology and pathogenesis between early-onset pSS (EOpSS) and late-onset pSS (LOpSS) are unknown. Recently, standardized outcome tools for measuring disease-specific activity and patients’ reported symptoms have been formulated by the European League Against Rheumatism (EULAR) SS study group: the EULAR SS Disease Activity Index (ESSDAI) for systemic features of pSS [1]. Also, as the new imaging techniques, salivary gland ultrasonography (SGUS) proved valuable for assessing salivary gland involvement in SS and seemed to exhibit good diagnostic properties. In addition, previous studies have demonstrated usefulness of SGUS for the prognostic stratification of patients with pSS [2], [3], [4].Objectives:The aim of this study was to examine the differences of etiology and pathogenesis between EOpSS and LOpSS using ESSDAI and SGUS.Methods:Fifty-six pSS patients who fulfilled the American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) classification criteria for SS were studied. Based on the disease onset age, all pSS patients were divided into two groups as those with the onset age of 40 years old or younger (EOpSS: n=26) and those with the onset age of older than 65 years old (LOpSS: n=30). The clinical findings were evaluated ESSDAI and OMERACT SGUS score at the first visit to our hospital. The ESSDAI (0–123) proposes the evaluation of 12 domains or organ systems (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system, muscular, hematological and biology). All patients were examined SGUS by a single investigator who was blinded to device (TUS-A300; Canon Medical Systems, Tokyo, Japan) with a linear transducer (7.5-10MHz). The OMERACT SGUS score was used for graded changes in the parenchymal homogeneity of salivary glands: grade 0, normal-appearing salivary gland parenchyma; grade 1, minimal change: mild inhomogeneity without hypo/anechoic areas; grade 2, moderate change: moderate inhomogeneity with focal hypo/anechoic areas; grade 3, severe change: diffuse inhomogeneity with hypo/anechoic areas occupying the entire gland surface [5].Results:The proportions of positive sera of RF, anti-SS-A and anti-SS-B antibodies were not different in the two groups, but the disease activities were higher in the EOpSS than in the LOpSS patients by measuring ESSDAI (7.30 vs 4.23, p=0.008), especially in constitutional domain (1.50 vs 0.60, p=0.03), articular domain (1.54 vs 0.40, p=0.0002) and biological domain (1.35 vs 0.90, p=0.04). No difference in salivary secretion was found between two groups (EOpSS: 8.02 vs LOpSS: 6.31 mL/10min.), but the OMERACT SGUS score was higher in LOpSS than in EOpSS patients (2.00 vs 2.70, p=0.0002).Conclusion:Although serological findings were not different, EOpSS patients had higher disease activity but less severe salivary gland degeneration than that in LOpSS patients, suggesting the pathogenesis of these two groups was different.References:[1]Seror R, et al. Ann Rheum Dis. 2010 Jun;69(6):1103-9.[2]Arthritis Care Res (Hoboken). 2014 Jul;66(7):1102-7.[3]Hammenfors DS, et al. Clin Exp Rheumatol. 2015 Jan-Feb;33(1):56-62.[4]Milic V, et al. PLoS One. 2019 Dec 31;14(12): e0226498.[5]Jousse-Joulin S, et al. Ann Rheum Dis. 2019 Jul;78(7):967-973.Disclosure of Interests:None declared

scholarly journals Peripheral Nervous System Involvement in Late-Onset Cobalamin C Disease?

2020 ◽  
Vol 11 ◽  
Author(s):  
Xujun Chu ◽  
Lingchao Meng ◽  
Wei Zhang ◽  
Jinjun Luo ◽  
Zhaoxia Wang ◽  
...  
Keyword(s):  
Nervous System ◽  
Peripheral Nervous System ◽  
Methylenetetrahydrofolate Reductase ◽  
Late Onset ◽  
Hot Spot ◽  
Axonal Neuropathy ◽  
Methylmalonic Aciduria ◽  
Lower Limbs ◽  
Compound Heterozygous ◽  
Limb Weakness

Background: Cobalamin C (cblC) has a fundamental role in both central and peripheral nervous system function at any age. Neurologic manifestations may be the earliest and often the only manifestation of hereditary or acquired cblC defect. Peripheral neuropathy remains a classical but underdiagnosed complication of cblC defect, especially in late-onset cblC disease caused by mutations in the methylmalonic aciduria type C and homocysteinemia (MMACHC) gene. So the clinical, electrophysiological, and pathological characteristics of late-onset cblC disease are not well-known.Methods: A retrospective study of patients with late-onset cblC disease was conducted at our hospital on a 3-year period. The neuropathy was confirmed by the nerve conduction study. Sural biopsies were performed in 2 patients.Results: Eight patients were identified, with a mean onset age of 16.25 ± 6.07 years. All patients had methylmalonic aciduria, homocysteinemia, compound heterozygous MMACHC gene mutations were detected in all patients, and 7/8 patients with c.482G>A mutation. One patient concomitant with homozygote c.665C>T mutation in 5,10-methylenetetrahydrofolate reductase (MTHFR) gene. All patients showed limb weakness and cognitive impairment. Five patients had possible sensorimotor axonal polyneuropathy predominantly in the distal lower limbs. Sural biopsies showed loss of myelinated and unmyelinated fibers. Electro microscopy revealed crystalline-like inclusions bodies in Schwann cells and axonal degeneration.Conclusion: Late-onset cblC disease had possible heterogeneous group of distal axonal neuropathy. c.482G>A mutation is a hot spot mutation in late-onset cblC disease.

scholarly journals Visual Ratings of Medial Temporal Lobe Atrophy Correlate with CSF Tau Indices in Clinical Variants of Early-Onset Alzheimer Disease

2017 ◽  
Vol 44 (1-2) ◽  
pp. 45-54 ◽  
Author(s):  
Elias Granadillo ◽  
Pongsatorn Paholpak ◽  
Mario F. Mendez ◽  
Edmond Teng
Keyword(s):  
Alzheimer Disease ◽  
Temporal Lobe ◽  
Early Onset ◽  
Medial Temporal Lobe ◽  
Late Onset ◽  
Brain Mri ◽  
Cortical Atrophy ◽  
Medial Temporal Lobe Atrophy ◽  
Underlying Mechanisms ◽  
Lobe Atrophy

Background/Aims: Prior studies of late-onset Alzheimer disease (AD) have reported that cerebrospinal fluid (CSF) tau levels correlate with hippocampal/medial temporal lobe atrophy. These findings suggest that CSF tau indices in AD may reflect tau-related neurodegeneration in the medial temporal lobe. However, it remains uncertain whether elevated CSF tau levels in the clinically heterogeneous subtypes of early-onset AD (EOAD; amnestic, posterior cortical atrophy [PCA], and logopenic progressive aphasia [LPA]) are attributable to similar underlying mechanisms. Methods: We identified 41 EOAD patients (18 amnestic, 14 with LPA, and 9 with PCA) with CSF and brain MRI data. Semiquantitative ratings were used to assess medial temporal lobe atrophy and PCA, which were compared to CSF biomarker indices. Results: Lower CSF tau levels were seen in PCA relative to amnestic EOAD and LPA, but similar ratings for medial temporal lobe atrophy and PCA were seen across the groups. After adjustments for demographics and cognitive performance, both total (p = 0.004) and hyperphosphorylated (p = 0.026) tau levels correlated with medial temporal lobe atrophy across this EOAD cohort. Conclusions: These results replicate prior findings in late-onset AD and support the hypothesis that CSF tau levels primarily reflect tau-related neurodegenerative changes in the hippocampus/medial temporal lobe across the clinical subtypes of EOAD.

scholarly journals Anti-MuSK-Positive Myasthenia Gravis in a Patient with Parkinsonism and Cognitive Impairment

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
S. Lanfranconi ◽  
S. Corti ◽  
P. Baron ◽  
G. Conti ◽  
L. Borellini ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cognitive Impairment ◽  
Myasthenia Gravis ◽  
Early Onset ◽  
Late Onset ◽  
Shoulder Girdle ◽  
Cholinergic Transmission ◽  
Central Nervous System Dysfunction ◽  
Seronegative Myasthenia Gravis

Muscle-specific tyrosine kinase- (MuSK-) antibodies-positive Myasthenia Gravis accounts for about one third of Seronegative Myasthenia Gravis and is clinically characterized by early onset of prominent bulbar, neck, shoulder girdle, and respiratory weakness. The response to medical therapy is generally poor. Here we report a case of late-onset MuSK-antibodies-positive Myasthenia Gravis presenting with signs of cognitive impairment and parkinsonism in addition to bulbar involvement and external ophthalmoplegia. The pattern of involvement of both peripheral and central nervous system dysfunction might suggest a common pathogenic mechanism, involving impaired cholinergic transmission.

NEONATAL SEPSIS AND IDENTIFICATION OF RISK FACTORS: STUDY IN AVBRH HOSPITAL SAWANGI

2019 ◽  
Vol 3 (6) ◽  
Author(s):  
Pramod P. Singhavi
Keyword(s):  
Risk Factors ◽  
Neonatal Sepsis ◽  
Early Onset ◽  
Late Onset ◽  
Signs And Symptoms ◽  
Premature Rupture ◽  
Maternal Risk ◽  
Late Onset Sepsis ◽  
Early Onset Sepsis ◽  
Meconium Stained Amniotic Fluid

Introduction: India has the highest incidence of clinical sepsis i.e.17,000/ 1,00,000 live births. In Neonatal sepsis septicaemia, pneumonia, meningitis, osteomyelitis, arthritis and urinary tract infections can be included. Mortality in the neonatal period each year account for 41% (3.6 million) of all deaths in children under 5 years and most of these deaths occur in low income countries and about one million of these deaths are due to infectious causes including neonatal sepsis, meningitis, and pneumonia. In early onset neonatal sepsis (EOS) Clinical features are non-specific and are inefficient for identifying neonates with early-onset sepsis. Culture results take up to 48 hours and may give false-positive or low-yield results because of the antenatal antibiotic exposure. Reviews of risk factors has been used globally to guide the development of management guidelines for neonatal sepsis, and it is similarly recommended that such evidence be used to inform guideline development for management of neonatal sepsis. Material and Methods: This study was carried out using institution based cross section study . The total number neonates admitted in the hospital in given study period was 644, of which 234 were diagnosed for neonatal sepsis by the treating pediatrician based on the signs and symptoms during admission. The data was collected: Sociodemographic characteristics; maternal information; and neonatal information for neonatal sepsis like neonatal age on admission, sex, gestational age, birth weight, crying immediately at birth, and resuscitation at birth. Results: Out of 644 neonates admitted 234 (36.34%) were diagnosed for neonatal sepsis by the paediatrician based on the signs and symptoms during admission. Of the 234 neonates, 189 (80.77%) infants were in the age range of 0 to 7 days (Early onset sepsis) while 45 (19.23%) were aged between 8 and 28 days (Late onset sepsis). Male to female ratio in our study was 53.8% and 46% respectively. Out of total 126 male neonates 91(72.2%) were having early onset sepsis while 35 (27.8%) were late onset type. Out of total 108 female neonates 89(82.4%) were having early onset sepsis while 19 (17.6%) were late onset type. Maternal risk factors were identified in 103(57.2%) of early onset sepsis cases while in late onset sepsis cases were 11(20.4%). Foul smelling liquor in early onset sepsis and in late onset sepsis was 10(5.56%) and 2 (3.70%) respectively. In early onset sepsis cases maternal UTI, Meconium stained amniotic fluid, Multipara and Premature rupture of membrane was seen in 21(11.67%), 19 (10.56%), 20(11.11%) and 33 (18.33%) cases respectively. In late onset sepsis cases maternal UTI, Meconium stained amniotic fluid, Multipara and Premature rupture of membrane was seen in 2 (3.70%), 1(1.85%), 3 (5.56%) and 3 (5.56%) cases respectively. Conclusion: Maternal risk identification may help in the early identification and empirical antibiotic treatment in neonatal sepsis and thus mortality and morbidity can be reduced.

Tuberculosis of the meninges and central nervous system. Experience of clinical diagnostics in St. Petersburg for 50 years

MedAlliance ◽  
2020 ◽  
Vol 8 (1) ◽  
pp. 14-24
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Medical Errors ◽  
Clinical Diagnostics ◽  
Lethal Outcome ◽  
Significant Difference ◽  
Group 2 ◽  
Allergic Vasculitis ◽  
Tuberculosis Meningitis ◽  
Group 1

The clinic and diagnostics of tuberculosis meningitis (TM) in 926 patients treated in St. Petersburg hospitals in 1965–1994 (group 1) and in 1995–2018 (group 2) is presented. The TM clinic is demonstrated to be determined by the form of tuberculosis and its characteristic generalization nature in the presence of repeated waves of bacteremia and allergic vasculitis of greater or lesser severity. There is clinical peculiarity of TM in primary pulmonary tuberculosis and its early large-focal and late miliar generalization, as well as in hematogenous tuberculosis. In patients of the 1st and 2nd groups the TM clinic shows in some respects a noticeable similarity, in others — a significant difference. Despite the typical symptoms, early diagnosis of TM took place in less than 20% of patients. Clinical examples illustrating the unusual development of TM, contrasting with its usual course, are given. A number of objective and subjective factors contributing to the adverse evolution of TM and its lethal outcome are discussed. These include the peculiarity of modern tuberculosis, especially when associated with HIV infection, as well as medical errors associated with ignorance of the pathogenesis of tuberculosis and failure to comply with the minimum examination for tuberculosis.

Exploring the Role of Aggregated Proteomes in the Pathogenesis of Alzheimer’s Disease

2020 ◽  
Vol 21 (12) ◽  
pp. 1164-1173
Author(s):  
Siju Ellickal Narayanan ◽  
Nikhila Sekhar ◽  
Rajalakshmi Ganesan Rajamma ◽  
Akash Marathakam ◽  
Abdullah Al Mamun ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Amyloid Precursor Protein ◽  
Early Onset ◽  
Late Onset ◽  
Precursor Protein ◽  
Brain Disorder ◽  
Amyloid Aβ ◽  
T Tau

: Alzheimer’s disease (AD) is a progressive brain disorder and one of the most common causes of dementia and death. AD can be of two types; early-onset and late-onset, where late-onset AD occurs sporadically while early-onset AD results from a mutation in any of the three genes that include amyloid precursor protein (APP), presenilin 1 (PSEN 1) and presenilin 2 (PSEN 2). Biologically, AD is defined by the presence of the distinct neuropathological profile that consists of the extracellular β-amyloid (Aβ) deposition in the form of diffuse neuritic plaques, intraneuronal neurofibrillary tangles (NFTs) and neuropil threads; in dystrophic neuritis, consisting of aggregated hyperphosphorylated tau protein. Elevated levels of (Aβ), total tau (t-tau) and phosphorylated tau (ptau) in cerebrospinal fluid (CSF) have become an important biomarker for the identification of this neurodegenerative disease. The aggregation of Aβ peptide derived from amyloid precursor protein initiates a series of events that involve inflammation, tau hyperphosphorylation and its deposition, in addition to synaptic dysfunction and neurodegeneration, ultimately resulting in dementia. The current review focuses on the role of proteomes in the pathogenesis of AD.

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