Secondary Narcolepsy in Children

2020 ◽  
Vol 36 (2) ◽  
pp. 123-127
Author(s):  
Riya Madan ◽  
Jennifer Pitts ◽  
Marc C. Patterson ◽  
Robin Lloyd ◽  
Gesina Keating ◽  
...  
Keyword(s):  
Spontaneous Remission ◽  
Hypothalamic Region ◽  
Neurologic Disorder ◽  
Excessive Sleepiness ◽  
Specific Outcome ◽  
Type C ◽  
Niemann Pick ◽  
Multiple Sleep Latency

Secondary narcolepsy occurs as a consequence of lesions involving the hypothalamic region that subserve wakefulness. Although observations on the characteristics of secondary narcolepsy have been published in adults, information on this topic in children is sparse. This is a retrospective study of characteristics and outcome of secondary narcolepsy in children. The medical records of 10 children with this condition at Mayo Clinic, Rochester, were reviewed. Characteristics of the underlying neurologic disorder, narcolepsy subtype, multiple sleep latency tests, medications used and outcome were extracted. Age at diagnosis of narcolepsy was between 6 and 17 years. Five of 10 patients had onset of excessive sleepiness within 1 year of diagnosis of the primary neurologic disorder. Six of 10 patients had type 1 narcolepsy (with cataplexy) whereas 4/10 had type 2 (without cataplexy). The clinical course was variable, with 8/10 continuing to require treatment for sleepiness at a mean period 6.6±6.2 years after diagnosis. One patient with narcolepsy type 1 due to Niemann Pick type C disease had died. One patient with narcolepsy type 2 due to craniopharyngioma had spontaneous remission of sleepiness. The 5/10 patients surviving with narcolepsy type 1 have continued to require pharmacotherapy for both sleepiness and cataplexy. This study draws attention to an important chronic sequel of childhood brain lesions that has variable, etiology-specific outcome. The rare occurrence of spontaneous resolution of childhood narcolepsy symptoms, not previously described, is also discussed.

Hypersomnolence Disorders

2020 ◽  
pp. 134-157
Author(s):  
Sulaiman Alhifzi ◽  
Nevin Zaki ◽  
Aljohara S. Almeneesier ◽  
Ahmed S. BaHammam
Keyword(s):  
Substance Use ◽  
Psychiatric Disorder ◽  
Treatment Strategies ◽  
Classification Systems ◽  
Common Symptom ◽  
Clinical Investigations ◽  
Idiopathic Hypersomnia ◽  
Multiple Sleep Latency

Despite varied classification systems, hypersomnolence disorders (or central disorders of hypersomnolence) are a group of disorders with a common symptom of excessive daytime sleepiness. In addition to a thorough clinical interview and examination, the assessment of hypersomnolence may require clinical investigations such as polysomnography and the multiple sleep latency test. This chapter examines the disorders of hypersomnolence with an emphasis on their clinical features and treatment strategies. The authors discuss the four main types of hypersomnolence disorders, narcolepsy type 1, narcolepsy type 2, idiopathic hypersomnia and Kleine–Levin syndrome, as well as hypersomnia due to a medical disorder, medication, or substance use; a psychiatric disorder; and insufficient sleep syndrome. It also discusses the relationships between hypersomnolence and psychiatric disorders.

Effects of Three- or Four-Cortex Syndesmotic Fixation in Ankle Fractures

2007 ◽  
Vol 97 (6) ◽  
pp. 457-459 ◽  
Author(s):  
Hasan Karapinar ◽  
Onder Kalenderer ◽  
Levent Karapinar ◽  
Taskin Altay ◽  
Metin Manisali ◽  
...  
Keyword(s):  
Clinical Results ◽  
Joint Space ◽  
Ankle Fractures ◽  
Routine Procedure ◽  
Plain Radiographs ◽  
Weber Type ◽  
Type C ◽  
Type 3

Background: There is no study comparing how Weber type C ankle fractures treated with either three- or four-cortex syndesmotic fixation affects the structure of the syndesmosis. Methods: In a retrospective study, 46 patients were separated into two groups: 22 patients with three-cortex fixation and 24 patients with four-cortex fixation. All of the patients were evaluated clinically and radiographically at least 1 year after removal of the syndesmosis screws. Results: There were three types of joint space obliteration: type 1, synostosis on plain radiographs; type 2, an incomplete bony bridge on magnetic resonance imaging with normal plain radiographs; and type 3, fibrous obliteration of the joint space. Although obliteration of the joint space was significant (P < .005) after four-cortex fixation, radiologic results did not affect the clinical outcome. Conclusion: Four-cortex fixation for diastasis after an ankle fracture should not be a routine procedure. We advocate three-cortex fixation because the clinical results are no different and there is less syndesmotic space obliteration postoperatively. (J Am Podiatr Med Assoc 97(6): 457–459, 2007)

Existence of host-related DNA sequences in the schistosome genome

Parasitology ◽  
1991 ◽  
Vol 102 (3) ◽  
pp. 397-403 ◽  
Author(s):  
Y. Iwamura ◽  
Y. Irie ◽  
R. Kominami ◽  
T. Nara ◽  
K. Yasuraoka
Keyword(s):  
Dna Sequences ◽  
Extra Chromosome ◽  
Repetitive Sequences ◽  
Dna Fingerprints ◽  
Alu Sequence ◽  
Type C ◽  
Almost All ◽  
Intracisternal A Particle

DNA sequences homologous to the mouse intracisternal A particle and endogenous type C retrovirus were detected in the DNAs ofSchistosoma japonicumadults andS. mansonieggs. Furthermore, other kinds of repetitive sequences in the host genome such as mouse type 1 Alu sequence (B1), mouse type 2 Alu sequence (B2) and mo-2 sequence, a mouse mini-satellite, were also detected in the DNAs from adults and eggs ofS. japonicumand eggs ofS. mansoni. Almost all of the sequences described above were absent in the DNAs ofS. mansoniadults. The DNA fingerprints of schistosomes, using the mo-2 sequence, were indistinguishable from each other and resembled those of their murine hosts. Moreover, the mo-2 sequence was hypermethylated in the DNAs of schistosomes and its amount was variable in them. These facts indicate that host-related sequences are actually present in schistosomes and that the mo-2 repetitive sequence exists probably in extra-chromosome.

scholarly journals Crista Terminalis, Musculi Pectinati, and Taenia Sagittalis: Anatomical Observations and Applied Significance

ISRN Anatomy ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-6
Author(s):  
Abu Ubaida Siddiqui ◽  
Syed Rehan Hafiz Daimi ◽  
Kusum Rajendra Gandhi ◽  
Abu Talha Siddiqui ◽  
Soumitra Trivedi ◽  
...  
Keyword(s):  
Right Atrium ◽  
Cardiac Conduction ◽  
Type B ◽  
Crista Terminalis ◽  
Type C ◽  
The Right ◽  
Complex Architecture ◽  
Complex Arrangement

Background. The complex architecture of the right atrium, crista terminalis (CT), and the musculi pectinati (MP) poses enormous challenges in electrophysiology and cardiac conduction. Few studies have been undertaken to substantiate the gross features of MP, in relation to the CT, but there is still scarcity of data regarding this. We tried to reinvestigate the gross arrangement of muscle bundles in the right atrium. Methods. Utilizing 151 human hearts and orientation of MP and its variations and relationship to the CT were investigated along with taenia sagittalis (TS). Patterns of MP were grouped in 6 categories and TS under three groups. Result. A plethora of variations were observed. Analysis of all the specimen revealed that 68 samples (45%) were of type 1 category and 27 (18%) fell into type 2 category. Prominent muscular columns were reported in 12 samples (8%). 83 samples (55%) presented with a single trunk of TS. Multiple trunks of TS were reported in 38 samples (25%). Conclusion. Samples with type 6 MP and type B/type C TS, which have a more complex arrangement of fibers, have a tendency to be damaged during cardiac catheterization. Nonetheless, the area as a whole is extremely significant considering the pragmatic application during various cardiac interventions.

scholarly journals Multiple sleep latency test in narcolepsy type 1 and narcolepsy type 2: A 5-year follow-up study

2018 ◽  
Vol 27 (5) ◽  
pp. e12700 ◽  
Author(s):  
Yu-Shu Huang ◽  
Christian Guilleminault ◽  
Cheng-Hui Lin ◽  
Chia-Hsiang Chen ◽  
Wei-Chih Chin ◽  
...  
Keyword(s):  
Sleep Latency ◽  
Multiple Sleep Latency Test ◽  
Follow Up Study ◽  
Multiple Sleep Latency

scholarly journals Deficiency of Niemann-Pick Type C-1 Protein Impairs Release of Human Immunodeficiency Virus Type 1 and Results in Gag Accumulation in Late Endosomal/Lysosomal Compartments

2009 ◽  
Vol 83 (16) ◽  
pp. 7982-7995 ◽  
Author(s):  
Yuyang Tang ◽  
Ihid Carneiro Leao ◽  
Ebony M. Coleman ◽  
Robin Shepard Broughton ◽  
James E. K. Hildreth
Keyword(s):  
Human Immunodeficiency Virus ◽  
Human Immunodeficiency Virus Type ◽  
Virus Type ◽  
Cholesterol Trafficking ◽  
Immunodeficiency Virus ◽  
Type C ◽  
Intracellular Cholesterol ◽  
Niemann Pick ◽  
Hiv 1

ABSTRACT Human immunodeficiency virus type 1 (HIV-1) relies on cholesterol-laden lipid raft membrane microdomains for entry into and egress out of susceptible cells. In the present study, we examine the need for intracellular cholesterol trafficking pathways with respect to HIV-1 biogenesis using Niemann-Pick type C-1 (NPC1)-deficient (NPCD) cells, wherein these pathways are severely compromised, causing massive accumulation of cholesterol in late endosomal/lysosomal (LE/L) compartments. We have found that induction of an NPC disease-like phenotype through treatment of various cell types with the commonly used hydrophobic amine drug U18666A resulted in profound suppression of HIV-1 release. Further, NPCD Epstein-Barr virus-transformed B lymphocytes and fibroblasts from patients with NPC disease infected with a CD4-independent strain of HIV-1 or transfected with an HIV-1 proviral clone, respectively, replicated HIV-1 poorly compared to normal cells. Infection of the NPCD fibroblasts with a vesicular stomatitis virus G-pseudotyped strain of HIV-1 produced similar results, suggesting a postentry block to HIV-1 replication in these cells. Examination of these cells using confocal microscopy showed an accumulation and stabilization of Gag in LE/L compartments. Additionally, normal HIV-1 production could be restored in NPCD cells upon expression of a functional NPC1 protein, and overexpression of NPC1 increased HIV-1 release. Taken together, our findings demonstrate that intact intracellular cholesterol trafficking pathways mediated by NPC1 are needed for efficient HIV-1 production.

Diagnosis and Treatment of the Central Disorders of Hypersomnolence

2021 ◽  
pp. 487-508
Author(s):  
Caroline Maness ◽  
Lynn Marie Trotti
Keyword(s):  
Behavioral Therapy ◽  
Sodium Oxybate ◽  
Idiopathic Hypersomnia ◽  
Diagnostic Lumbar Puncture ◽  
Multiple Sleep Latency ◽  
Dietary Modifications ◽  
Ancillary Features ◽  
Novel Therapeutic

The central disorders of hypersomnolence (CDH) are lifelong conditions that cause an irrepressible need for sleep during waking hours. Some of these disorders are due to medications or other conditions, but narcolepsy type 1 (NT1), narcolepsy type 2 (NT2), and idiopathic hypersomnia (IH) occur as primary conditions. In each disorder excessive daytime sleepiness (EDS) may be accompanied by ancillary features such as sleep paralysis, hypnagogic/hypnopompic hallucinations, sleep drunkenness, or, in the case of NT1, cataplexy. Polysomnography and a multiple sleep latency test are used for diagnosis. Diagnostic lumbar puncture may be performed to differentiate NT1 from NT2. Although the mainstay of treatment for CDH is currently pharmacotherapy, management may also involve behavioral approaches such as sleep and nap scheduling, dietary modifications, and cognitive-behavioral therapy. Pharmacologic treatment can include traditional medications such as modafinil, methylphenidate, or sodium oxybate. Novel therapeutic agents such as pitolisant, clarithromycin, flumazenil, and melatonin may be useful in relieving EDS and associated symptoms.

The Branching Pattern of the Inferior Pancreaticoduodenal Artery in Patients with a Replaced Right Hepatic Artery

2018 ◽  
Vol 84 (6) ◽  
pp. 796-800 ◽  
Author(s):  
Yoshito Tomimaru ◽  
Kaishu Tanaka ◽  
Kozo Noguchi ◽  
Shunji Morita ◽  
Hiroshi Imamura ◽  
...  
Keyword(s):  
Type A ◽  
Multidetector Row Computed Tomography ◽  
Branching Pattern ◽  
Pancreaticoduodenal Artery ◽  
Replaced Right Hepatic Artery ◽  
Artery First ◽  
Type C ◽  
Type 3

Understanding the anatomy of the inferior pancreaticoduodenal artery (IPDA) is important in patients undergoing pancreaticoduodenectomy, especially in an artery-first approach, resulting in some studies focusing on IPDA anatomy. However, the studies have covered only cases without variation in hepatic arterial anatomy, a common arterial variant, suggesting the necessity of the investigation of IPDA anatomy in cases with the variant. Using images of multidetector row computed tomography, cases with replaced right hepatic artery (rRHA) were picked out among 714 patients undergoing multidetector row computed tomography for determining arteries of the pancreatic head at our institution. IPDA branching pattern was investigated in the rRHA cases. Three-dimensional reconstruction was performed to visually understand the branching pattern in representative cases. rRHA was identified in 139 cases (19.5%); rRHA originating from the superior mesenteric artery (SMA) (type 1; 74.1%), celiac axis (type 2; 18.0%), and others (type 3; 7.9%). IPDA branching pattern was categorized; IPDA originated from SMA (type A), posterior and anterior IPDA originated from rRHA and SMA, respectively (type B), or IPDA originated from rRHA (type C). Of type 1 cases, 69, 23, and 11 cases showed type A, B, and C pattern, respectively. Of type 2 cases, 16 and 9 cases showed type A and B, respectively. All 11 type 3 cases showed type C. IPDA branching pattern was determined in the rRHA cases. This would help identification of rRHA cases where the artery-first approach is technically less feasible at pancreaticoduodenectomy (type 1-B, 1-C, and 2-B).

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