Unknown fatal encephalomyelopolyradiculoneuritis in a child. A case report

2021 ◽  
Vol 2 (2) ◽  
pp. 100-106
Author(s):  
Aleksandra I. Pavlyuchkova ◽  
Aleksey S. Kotov

In childhood, various infectious, autoimmune, genetic diseases can manifest. We present a case of fatal encephalomyelopolyradiculoneuritis of unknown etiology in a 9-year-old child. Patient N.K. in February 2019, noted an increase in temperature to subfebrile values, received symptomatic and antibiotic therapy without effect. An increase in protein and lymphocytes was found in the cerebrospinal fluid. According to MRI data, the emergence of more and more foci of the pathological signal in the brain and spinal cord, cranial nerves and nerve roots of the lumbar plexus was noted. Known infectious and autoimmune diseases were excluded. Despite active therapy with glucocorticoids, antibiotics, antiviral drugs, immunoglobulin, the disease continued to progress, and the patient died in April 2020.

Author(s):  
Ilya Lebedev ◽  
Alexander Bragin ◽  
Yulia Boldyreva ◽  
Artem Borsukov ◽  
Alexander Tersenov ◽  
...  

The article summarizes information about the head ganglia (the sympathetic ganglia and in the sensory cranial nerves). Gives а brief historical background on the history issue and relevance of the topic. Characterized by each node with its topography and lesion clinic. The described process of treatment, and prospects for new therapies. Raised the issue of the significance of the defeat ganglia, namely, the suffering of the sick and forced treatment costs (due to the complex differential diagnosis). In a biological sense, pain first appears in chordates and during evolution, as well as transformations of the brain and spinal cord, it acquires new types, localization and significance for the performance of a living organism. And facial pain, being a nosology with a multidisciplinary approach in diagnosis and treatment, demonstrates both its complexity and importance in human life.


1930 ◽  
Vol 51 (6) ◽  
pp. 889-902 ◽  
Author(s):  
Jules Freund

1. Antibodies can be extracted from the brain and spinal cord of rabbits actively or passively immunized with typhoid bacilli. 2. The titers of the antibodies in the extracts of brain and cord depend upon the titer of the blood serum. In actively immunized rabbits the following numerical relationships exist between the titers of the serum and of these organ extracts: The ratio of the titer of the serum is to the titers of extract of brain and of the spinal cord about as 100 is to 0.8; the titer of the serum is to the titer of the cerebrospinal fluid as 100 is to 0.3. In passively immunized rabbits the titer of the serum is to the titer of brain and spinal-cord extract as 100 is to 0.7. 3. The antibodies recovered from the brain are not due to the presence of blood in it for perfusion of the brain does not reduce its antibody content appreciably. 4. Antibodies penetrate into the spinal fluid from the blood even in the absence of inflammation of the meninges. When the penetration is completed the following numerical relationship exists between the titer of the serum and that of the cerebrospinal fluid: 100 to 0.25. 5. The penetration into the cerebrospinal fluid of antibodies injected intravenously proceeds at a slow rate, being completed only several hours after the immune serum has been injected. The penetration of antibodies into the tissue of the brain occurs at a very rapid rate. It is completed within 15 minutes. 6. It is very unlikely that when the immune serum is injected intravenously the antibodies reach the brain tissue by way of the cerebrospinal fluid, for (1) the antibody titer of the cerebrospinal fluid is lower than that of the brain extract, and (2) antibodies penetrate faster into the tissue of the brain than into the cerebrospinal fluid.


2020 ◽  
Vol 16 (5) ◽  
pp. 557-569 ◽  
Author(s):  
Maryam Kharati ◽  
Sanam Foroutanparsa ◽  
Mohammad Rabiee ◽  
Reza Salarian ◽  
Navid Rabiee ◽  
...  

Background: Multiple Sclerosis (MS) involves an immune-mediated response in which body’s immune system destructs the protective sheath (myelin). Part of the known MS biomarkers are discovered in cerebrospinal fluid like oligoclonal lgG (OCGB), and also in blood like myelin Oligodendrocyte Glycoprotein (MOG). The conventional MS diagnostic methods often fail to detect the disease in early stages such as Clinically Isolated Syndrome (CIS), which considered as a concerning issue since CIS highlighted as a prognostic factor of MS development in most cases. Methods: MS diagnostic techniques include Magnetic Resonance Imaging (MRI) of the brain and spinal cord, lumbar puncture (or spinal tap) that evaluate cerebrospinal fluid, evoked potential testing revealing abnormalities in the brain and spinal cord. These conventional diagnostic methods have some negative points such as extensive processing time as well as restriction in the quantity of samples that can be analyzed concurrently. Scientists have focused on developing the detection methods especially early detection which belongs to ultra-sensitive, non-invasive and needed for the Point of Care (POC) diagnosis because the situation was complicated by false positive or negative results. Results: As a result, biosensors are utilized and investigated since they could be ultra-sensitive to specific compounds, cost effective devices, body-friendly and easy to implement. In addition, it has been proved that the biosensors on physiological fluids (blood, serum, urine, saliva, milk etc.) have quick response in a non-invasive rout. In general form, a biosensor system for diagnosis and early detection process usually involves; biomarker (target molecule), bio receptor (recognition element) and compatible bio transducer. Conclusion: Studies underlined that early treatment of patients with high possibility of MS can be advantageous by postponing further abnormalities on MRI and subsequent attacks. : This Review highlights variable disease diagnosis approaches such as Surface Plasmon Resonance (SPR), electrochemical biosensors, Microarrays and microbeads based Microarrays, which are considered as promising methods for detection and early detection of MS.


2016 ◽  
Vol 9 (2) ◽  
pp. 87-89
Author(s):  
Arvind Soni ◽  
Anchal Duggal

ABSTRACT A cerebrospinal fluid (CSF) leak is an escape of the fluid that surrounds the brain and spinal cord. Any tear or hole in the membrane that surrounds the brain and spinal cord (dura) can allow the fluid that surrounds those organs to leak. Most commonly, the leak is known to occur from the nose (CSF rhinorrhea) or through the ears (CSF otorrhea). Also, etiology is posttraumatic in majority. However, idiopathic CSF leakage from the eyes is extremely uncommon. How to cite this article Soni A, Duggal A. Idiopathic Cerebrospinal Fluid Oculorrhea: An Unusual Case Report. Clin Rhinol An Int J 2016;9(2):87-89.


1998 ◽  
Vol XXX (1-2) ◽  
pp. 40-42
Author(s):  
Enrico Granieri ◽  
Ilaria Casetta

Multiple sclerosis is a disease of unknown etiology characterized by inflammory demyelination of the brain and spinal cord. Epidemiological investigations play important role in study of multiple sclerosis. Geographical distribution of the disease has been described in terms of prevalence and incidence. The possible role of environmental factors as a cause of multiple sclerosis had been hypothesized with observation of unequal geographic distribution of the disease. More interesting, in terms of their biological significance, are attempts to identify associations between multiple sclerosis and situations or events wich could cause blood-brain barrier damages, such as trauma or toxic exposures.


2021 ◽  
pp. 614-662
Author(s):  
Alastair Compston

Chapter 16: ‘Neurologie: the doctrine of the nerves: the brain and nervous stock’ summarizes Willis’s treatises in Cerebri anatome, Nervorumque descriptio et usus (1664), De motu musculari (1670) and De anima brutorum (1672). Willis’s coinage of the term ‘neurologie’, intending this as the doctrine of the nerves based on the anatomy of the cranial nerves rather than the study of diseases affecting the brain and nervous stock, is described. The chapter explains why these treatises are additionally important for assigning function to the cerebrum and cerebellum rather than the ventricles; the concept of cerebral localization; the distinction between voluntary and involuntary, or reflex, movement; Willis’s account of the autonomic nervous system; and his ideas on muscular movement. Apart from these innovative contributions, Willis’s description of the arrangement of blood vessels supplying the brain and spinal cord, for which the book is celebrated, is described. The fifteen engraved plates are included. {148 words}


1974 ◽  
Vol 11 (1) ◽  
pp. 87-96 ◽  
Author(s):  
Jill Beech ◽  
D. C. Dodd

Eight horses with progressive neurologic signs had encephalomyelitis associated with toxoplasma-like protozoan bodies. There were scattered hemorrhagic, malacic lesions in white and grey matter in the brain and spinal cord. Microscopically there was malacia, mononuclear cell infiltration, especially perivascularly, gliosis, and various degrees of necrosis and hemorrhage. Other tissues were normal, except for the lung of one horse that had focal bronchopneumonia. The cerebrospinal fluid did not contain measurable amounts of IgM, IgG, or IgA. Serum from one horse was negative at 1:64 by the hemagglutination-inhibition test for toxoplasma antibodies.


2021 ◽  
Vol 14 ◽  
Author(s):  
Thomas Leth Jensen ◽  
Casper René Gøtzsche ◽  
David P. D. Woldbye

In recent years, gene therapy has been raising hopes toward viable treatment strategies for rare genetic diseases for which there has been almost exclusively supportive treatment. We here review this progress at the pre-clinical and clinical trial levels as well as market approvals within diseases that specifically affect the brain and spinal cord, including degenerative, developmental, lysosomal storage, and metabolic disorders. The field reached an unprecedented milestone when Zolgensma® (onasemnogene abeparvovec) was approved by the FDA and EMA for in vivo adeno-associated virus-mediated gene replacement therapy for spinal muscular atrophy. Shortly after EMA approved Libmeldy®, an ex vivo gene therapy with lentivirus vector-transduced autologous CD34-positive stem cells, for treatment of metachromatic leukodystrophy. These successes could be the first of many more new gene therapies in development that mostly target loss-of-function mutation diseases with gene replacement (e.g., Batten disease, mucopolysaccharidoses, gangliosidoses) or, less frequently, gain-of-toxic-function mutation diseases by gene therapeutic silencing of pathologic genes (e.g., amyotrophic lateral sclerosis, Huntington's disease). In addition, the use of genome editing as a gene therapy is being explored for some diseases, but this has so far only reached clinical testing in the treatment of mucopolysaccharidoses. Based on the large number of planned, ongoing, and completed clinical trials for rare genetic central nervous system diseases, it can be expected that several novel gene therapies will be approved and become available within the near future. Essential for this to happen is the in depth characterization of short- and long-term effects, safety aspects, and pharmacodynamics of the applied gene therapy platforms.


2015 ◽  
Vol 112 (31) ◽  
pp. 9704-9709 ◽  
Author(s):  
Yuxuan Wang ◽  
Simeon Springer ◽  
Ming Zhang ◽  
K. Wyatt McMahon ◽  
Isaac Kinde ◽  
...  

Cell-free DNA shed by cancer cells has been shown to be a rich source of putative tumor-specific biomarkers. Because cell-free DNA from brain and spinal cord tumors cannot usually be detected in the blood, we studied whether the cerebrospinal fluid (CSF) that bathes the CNS is enriched for tumor DNA, here termed CSF-tDNA. We analyzed 35 primary CNS malignancies and found at least one mutation in each tumor using targeted or genome-wide sequencing. Using these patient-specific mutations as biomarkers, we identified detectable levels of CSF-tDNA in 74% [95% confidence interval (95% CI) = 57–88%] of cases. All medulloblastomas, ependymomas, and high-grade gliomas that abutted a CSF space were detectable (100% of 21 cases; 95% CI = 88–100%), whereas no CSF-tDNA was detected in patients whose tumors were not directly adjacent to a CSF reservoir (P < 0.0001, Fisher’s exact test). These results suggest that CSF-tDNA could be useful for the management of patients with primary tumors of the brain or spinal cord.


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