Diagnosis and Management of Narcolepsy

2017 ◽  
Vol 37 (04) ◽  
pp. 446-460 ◽  
Author(s):  
Logan Schneider ◽  
Emmanuel Mignot
Keyword(s):  
Central Nervous System ◽  
Respiratory Infections ◽  
Genetic Studies ◽  
Upper Respiratory Infections ◽  
Idiopathic Hypersomnia ◽  
Atypical Manifestations ◽  
The Central Nervous System ◽  
Upper Respiratory

AbstractType 1 narcolepsy is caused by deficient hypocretin signaling in the central nervous system (CNS), and is distinct from other primary CNS hypersomnias, which seem to lay along a spectrum from type 2 narcolepsy to idiopathic hypersomnia. There appears to be a strong autoimmune diathesis to the development of type 1 narcolepsy, as evidenced by the near universal presence of HLA-DQB1*06:02 in patients. Growing knowledge of the immunogenetic basis of the disease is supported by genetic studies and seasonal variation of type 1 narcolepsy incidence following winter upper respiratory infections (e.g., strep throat and influenza). Despite improved diagnostic accuracy of adding cerebrospinal fluid hypocretin measurement to the traditional workup, recognition of the disorder remains limited by its moderate prevalence and atypical manifestations in different ethnic groups. Treatments are currently symptom-based, and have been extended to other hypersomnias with mixed results.

scholarly journals THE EFFECT OF FOLIC ACID ON THE CENTRAL NERVOUS SYSTEM OF NORMAL SUBJECTS AND SUBJECTS WITH ANEMIA OTHER THAN PERNICIOUS ANEMIA

Blood ◽  
1950 ◽  
Vol 5 (2) ◽  
pp. 148-154 ◽  
Author(s):  
JONAS WEISSBERG ◽  
T. H. McGAVACK ◽  
MILDRED VOGEL ◽  
S. KENIGSBERG
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Folic Acid ◽  
Pernicious Anemia ◽  
Respiratory Infections ◽  
Well Being ◽  
Normal Subjects ◽  
Upper Respiratory Infections ◽  
The Central Nervous System ◽  
Upper Respiratory

Abstract 1. The administration of 20 mg. of folic acid daily to a group of 26 normal subjects and to a group of 22 subjects with some form of anemia other than pernicious anemia for a period of six to twelve months was not harmful to the central nervous system. 2. Pre-existent neurologic changes in both groups of patients were not altered during the period of treatment with folic acid. 3. Hematologic variations in both groups were not significant during the period of treatment. 4. A sense of well being and a diminished incidence of upper respiratory infections were reported by many of the normal subjects to whom folic acid was administered.

Development and regeneration in the central nervous system

1990 ◽  
Vol 327 (1239) ◽  
pp. 127-143 ◽  
Keyword(s):  
Stem Cells ◽  
Central Nervous System ◽  
Nervous System ◽  
Progenitor Cells ◽  
Progenitor Cell ◽  
Cell Types ◽  
The Central Nervous System

As part of our attempts to understand principles that underly organism development, we have been studying the development of the rat optic nerve. This simple tissue is composed of three glial cell types derived from two distinct cellular lineages. Type-1 astrocytes appear to be derived from a monopotential neuroepithelial precursor, whereas type-2 astrocytes and oligodendrocytes are derived from a common oligodendrocyte-type-2 astrocyte (O-2A) progenitor cell. Type-1 astrocytes modulate division and differentiation of O-2A progenitor cells through secretion of platelet-derived growth factor, and can themselves be stimulated to divide by peptide mitogens and through stimulation of neurotransmitter receptors. In vitro analysis indicates that many dividing O-2A progenitors derived from optic nerves of perinatal rats differentiate symmetrically and clonally to give rise to oligodendrocytes, or can be induced to differentiate into type-2 astrocytes. O-2A perinatal progenitors can also differentiate to form a further O-2A lineage cell, the O-2A adult progenitor, which has properties specialized for the physiological requirements of the adult nervous system. In particular, O-2A adult progenitors have many of the features of stem cells, in that they divide slowly and asymmetrically and appear to have the capacity for extended self-renewal. The apparent derivation of a slowly and asymmetrically dividing cell, with properties appropriate for homeostatic maintenance of existing populations in the mature animal, from a rapidly dividing cell with properties suitable for the rapid population and myelination of central nervous system (CNS) axon tracts during early development, offers novel and unexpected insights into the possible origin of self-renewing stem cells and also into the role that generation of stem cells may play in helping to terminate the explosive growth of embryogenesis. Moreover, the properties of O-2A adult progenitor cells are consistent with, and may explain, the failure of successful myelin repair in conditions such as multiple sclerosis, and thus seem to provide a cellular biological basis for understanding one of the key features of an important human disease.

scholarly journals Vitamin D supplementation –Is it the time to stop wasting money on vitamin D?

JMS SKIMS ◽  
2019 ◽  
Vol 22 (1) ◽  
Author(s):  
Shariq R Masoodi
Keyword(s):  
Vitamin D ◽  
Bone Health ◽  
Respiratory Infections ◽  
Vitamin D Supplementation ◽  
Increased Risk ◽  
Wide Range ◽  
Upper Respiratory ◽  
The Relationship

In recent years, vitamin D has received increased attention, as a number of studies have shown its link to the pathogenesis of various diseases. Apart from its benefits on bone health, vitamin D supplementation has been shown beneficial in reducing risk for many chronic diseases including autoimmune diseases, type 2 diabetes, heart disease, many cancers and infectious diseases. Vitamin deficiency has been associated with increased risk of elevated blood pressure, heart attack and stroke in studies. The relationship between vitamin D deficiency and the increased incidence of upper respiratory infections, asthma and eczema, among children, has been observed in several studies. Recent meta-analyzes showed a relationship between vitamin D administration during the early months of life and a lower incidence of type 1 diabetes later in life. Therefore, it is not surprising that Vitamin D supplementation is used to prevent and treat a wide range of diseases, and the use has increased considerably in the last decade.

scholarly journals Prion type 2 selection in sporadic Creutzfeldt–Jakob disease affecting peripheral ganglia

2021 ◽  
Vol 9 (1) ◽  
Author(s):  
Anna Hofmann ◽  
Arne Wrede ◽  
Wiebke M. Jürgens-Wemheuer ◽  
Walter J. Schulz-Schaeffer
Keyword(s):  
Central Nervous System ◽  
Prion Protein ◽  
Trigeminal Ganglion ◽  
Dorsal Root ◽  
Pathological Changes ◽  
Jakob Disease ◽  
The Central Nervous System ◽  
Dependent Property

AbstractIn sporadic Creutzfeldt–Jakob disease (sCJD), the pathological changes appear to be restricted to the central nervous system. Only involvement of the trigeminal ganglion is widely accepted. The present study systematically examined the involvement of peripheral ganglia in sCJD utilizing the currently most sensitive technique for detecting prions in tissue morphologically. The trigeminal, nodose, stellate, and celiac ganglia, as well as ganglia of the cervical, thoracic and lumbar sympathetic trunk of 40 patients were analyzed with the paraffin-embedded tissue (PET)-blot method. Apart from the trigeminal ganglion, which contained protein aggregates in five of 19 prion type 1 patients, evidence of prion protein aggregation was only found in patients associated with type 2 prions. With the PET-blot, aggregates of prion protein type 2 were found in all trigeminal (17/17), in some nodose (5 of 7) and thoracic (3 of 6) ganglia, as well as in a few celiac (4 of 19) and lumbar (1 of 5) ganglia of sCJD patients. Whereas aggregates of both prion types may spread to dorsal root ganglia, more CNS-distant ganglia seem to be only involved in patients accumulating prion type 2. Whether the prion type association is due to selection by prion type-dependent replication, or due to a prion type-dependent property of axonal spread remains to be resolved in further studies.

Neurofibromatosis Type 1 and Type 2: review of the central nervous system and related structures

1997 ◽  
Vol 19 (1) ◽  
pp. 1-12 ◽  
Author(s):  
Yuichi Inoue ◽  
Yutaka Nemoto ◽  
Takahiko Tashiro ◽  
Keiko Nakayama ◽  
Tetsuo Nakayama ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
The Central Nervous System

scholarly journals Update in the CNS Response to Hypoglycemia

2012 ◽  
Vol 97 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Rory J. McCrimmon
Keyword(s):  
Type 2 Diabetes ◽  
Central Nervous System ◽  
Type 1 Diabetes ◽  
Nervous System ◽  
Animal Studies ◽  
Human Subjects ◽  
Diabetes Research ◽  
The Central Nervous System

Hypoglycemia remains a major clinical issue in the management of people with type 1 and type 2 diabetes. Research in basic science is only beginning to unravel the mechanisms that: 1) underpin the detection of hypoglycemia and initiation of a counterregulatory defense response; and 2) contribute to the development of defective counterregulation in both type 1 and type 2 diabetes, particularly after prior exposure to repeated hypoglycemia. In animal studies, the central nervous system has emerged as key to these processes. However, bench-based research needs to be translated through studies in human subjects as a first step to the future development of clinical intervention. This Update reviews studies published in the last 2 yr that examined the central nervous system effects of hypoglycemia in human subjects, largely through neuroimaging techniques, and compares these data with those obtained from animal studies and the implications for future therapies. Based on these studies, it is increasingly clear that our understanding of how the brain responds and adapts to recurrent hypoglycemia remains very limited. Current therapies have provided little evidence that they can prevent severe hypoglycemia or improve hypoglycemia awareness in type 1 diabetes. There remains an urgent need to increase our understanding of how and why defective counterregulation develops in type 1 diabetes in order for novel therapeutic interventions to be developed and tested.

scholarly journals Non-Oncological Neuroradiological Manifestations in NF1 and Their Clinical Implications

Cancers ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 1831
Author(s):  
Camilla Russo ◽  
Carmela Russo ◽  
Daniele Cascone ◽  
Federica Mazio ◽  
Claudia Santoro ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Structural Organization ◽  
Neurofibromatosis Type ◽  
Review Article ◽  
Tumor Suppressor Protein ◽  
Clinical Implications ◽  
Nf1 Gene ◽  
The Central Nervous System

Neurofibromatosis type 1 (NF1), the most frequent phakomatosis and one of the most common inherited tumor predisposition syndromes, is characterized by several manifestations that pervasively involve central and peripheral nervous system structures. The disorder is due to mutations in the NF1 gene, which encodes for the ubiquitous tumor suppressor protein neurofibromin; neurofibromin is highly expressed in neural crest derived tissues, where it plays a crucial role in regulating cell proliferation, differentiation, and structural organization. This review article aims to provide an overview on NF1 non-neoplastic manifestations of neuroradiological interest, involving both the central nervous system and spine. We also briefly review the most recent MRI functional findings in NF1.

scholarly journals Cerebrospinal fluid monoamine levels in central hypersomnolence disorders

SLEEP ◽  
2021 ◽  
Author(s):  
Lucie Barateau ◽  
Isabelle Jaussent ◽  
Julien Roeser ◽  
Claudio Ciardiello ◽  
Thomas S Kilduff ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Daytime Sleepiness ◽  
Direct Consequence ◽  
Trace Amines ◽  
Idiopathic Hypersomnia ◽  
National Reference Center ◽  
Drug Free ◽  
Sleep Propensity

Abstract Study objectives Whether the cause of daytime sleepiness in narcolepsy type 1 (NT1) is a direct consequence of the loss of orexin neurons or whether low orexin reduces the efficacy of the monoaminergic systems to promote wakefulness is unclear. The neurobiology underlying sleepiness in other central hypersomnolence disorders, narcolepsy type 2 (NT2) and idiopathic hypersomnia (IH), is currently unknown. Methods Eleven biogenic amines including the monoaminergic neurotransmitters and their metabolites and five trace amines were measured in the cerebrospinal fluid (CSF) of 94 drug-free subjects evaluated at the French National Reference Center for Narcolepsy: 39 NT1(orexin-deficient) patients, 31 patients with objective sleepiness non-orexin deficient (NT2 and IH), and 24 patients without objective sleepiness. Results Three trace amines were undetectable in the sample: Tryptamine, Octopamine, and 3-iodothyronamine. No significant differences were found among the three groups for quantified monoamines and their metabolites in crude and adjusted models; however, CSF 5-HIAA levels tended to increase in NT1 compared to other patients after adjustment. Most of biomarkers were not associated with ORX-A levels, clinical or neurophysiological parameters, but a few biomarkers (e.g., MHPG and norepinephrine) correlated with daytime sleepiness and high REM sleep propensity. Conclusion We found no striking differences among CSF monoamines, their metabolites and trace amine levels, and few associations between them and key clinical or neurophysiological parameters in NT1,NT2/IH and patients without objective sleepiness. Although mostly negative, these findings are a significant contribution to our understanding of the neurobiology of hypersomnolence in these disorders that remain mysterious and deserve further exploration.

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