مقاله شماره ۲۱
#Pediatric
#Neurology
#Radiology
عنوان:
MRI Findings in Pediatric Pseudotumor Cerebri Syndrome
سایتیشن:
Kohli et al.
مجله:
Pediatric Neurology
تاریخ انتشار:
17, May, 2019
چکیده:
BACKGROUND
Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children.
METHODS
As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis.
RESULTS
The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%).
CONCLUSIONS
In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
DOI: https://dx.doi.org/10.1016/j.pediatrneurol.2019.04.010
توضیحات مهم:
✅ سندرم Pseudotumor cerebri (پرفشاری خون ایدیوپاتیک intracranial) یک اختلال نسبتاً شایع است و اغلب با سردرد و papilledema بروز می کند.
✔️ معیارهای تشخیصی اصلاح شده ای بر اساس neuroimaging از جمله flat شدت غده هیپوفیز و اسکلرا خلفی ، distension غلاف عصب بینایی و تنگی سینوس وریدی عرضی وجود دارد.
✔️ نویسندگان در این مطالعه ، 101 بیمار را که از نظر بالینی مظنون به سندرم Pseudotumor cerebri بودند مورد بررسی قرار دادند و این بیماران را با 24 کنترل مقایسه کردند.
✔️ نتایج این مطالعه نشان داد که وجود سه یا بیشتر یافته در MRI حساسیت ۶۲٪ و اختصاصیت ۹۵٪ برای تشخیص این سندرم دارد.
✔ تنگی سینوس وریدی عرضی به تنهایی از حساسیت (74٪) و اختصاصیت (100٪) بالاتری برخوردار بود.
🔎 @Meditorha
#Pediatric
#Neurology
#Radiology
عنوان:
MRI Findings in Pediatric Pseudotumor Cerebri Syndrome
سایتیشن:
Kohli et al.
مجله:
Pediatric Neurology
تاریخ انتشار:
17, May, 2019
چکیده:
BACKGROUND
Revised diagnostic criteria for pseudotumor cerebri syndrome require three of four neuroimaging findings in the absence of papilledema. We examined the sensitivity and specificity of three or more of four of these magnetic resonance imaging (MRI) findings for pseudotumor cerebri syndrome in children.
METHODS
As part of clinical care, patients in whom there was suspicion for pseudotumor cerebri syndrome underwent neurological and fundoscopic examinations, lumbar puncture, MRI, or magnetic resonance venogram. For this retrospective study, we used this information to classify 119 subjects into definite (n = 66) or probable pseudotumor cerebri syndrome (n = 12), elevated opening pressure without papilledema (n = 23), or controls who had normal opening pressure without papilledema (n = 24). A neuroradiologist, unaware of the clinical findings or original MRI report, reviewed MRIs for pituitary gland flattening, flattening of the posterior sclera, optic nerve sheath distention, and transverse venous sinus stenosis.
RESULTS
The presence of three or more MRI findings has a sensitivity of 62% (95% confidence interval: 47% to 75%) and a specificity of 95% (95% confidence interval: 77% to 100%), compared with controls. Two of three (transverse venous sinus stenosis, pituitary gland flattening, flattening of the posterior sclera) had a similar sensitivity and specificity. Transverse venous sinus stenosis alone had a slightly higher sensitivity (74%, 95% confidence interval: 60% to 85%) and specificity (100%, 95% confidence interval: 80% to 100%).
CONCLUSIONS
In children, three of four of the proposed neuroimaging criteria and transverse venous sinus stenosis alone have a moderate sensitivity and robust specificity for pseudotumor cerebri syndrome. MRIs should be reviewed for these criteria, and their presence should raise suspicion for pseudotumor cerebri syndrome in children, particularly if the presence of papilledema is uncertain.
DOI: https://dx.doi.org/10.1016/j.pediatrneurol.2019.04.010
توضیحات مهم:
✅ سندرم Pseudotumor cerebri (پرفشاری خون ایدیوپاتیک intracranial) یک اختلال نسبتاً شایع است و اغلب با سردرد و papilledema بروز می کند.
✔️ معیارهای تشخیصی اصلاح شده ای بر اساس neuroimaging از جمله flat شدت غده هیپوفیز و اسکلرا خلفی ، distension غلاف عصب بینایی و تنگی سینوس وریدی عرضی وجود دارد.
✔️ نویسندگان در این مطالعه ، 101 بیمار را که از نظر بالینی مظنون به سندرم Pseudotumor cerebri بودند مورد بررسی قرار دادند و این بیماران را با 24 کنترل مقایسه کردند.
✔️ نتایج این مطالعه نشان داد که وجود سه یا بیشتر یافته در MRI حساسیت ۶۲٪ و اختصاصیت ۹۵٪ برای تشخیص این سندرم دارد.
✔ تنگی سینوس وریدی عرضی به تنهایی از حساسیت (74٪) و اختصاصیت (100٪) بالاتری برخوردار بود.
🔎 @Meditorha
مقاله شماره ۲۲
#Cardiology
#Neurology
#BasicSciences
عنوان:
Acquired Cardiac Channelopathies in Epilepsy
نوع مطالعه:
Review
مجله:
Epilepsia
تاریخ انتشار:
01, Sep, 2019
چکیده:
There is growing evidence that cardiac dysfunction in patients with chronic epilepsy could play a pathogenic role in sudden unexpected death in epilepsy (SUDEP). Recent animal studies have revealed that epilepsy secondarily alters the expression of cardiac ion channels alongside abnormal cardiac electrophysiology and remodeling. These molecular findings represent novel evidence for an acquired cardiac channelopathy in epilepsy, distinct from inherited ion channels mutations associated with cardiocerebral phenotypes. Specifically, seizure activity has been shown to alter the messenger RNA (mRNA) and protein expression of voltage-gated sodium channels (Nav 1.1, Nav 1.5), voltage-gated potassium channels (Kv 4.2, Kv 4.3), sodium-calcium exchangers (NCX1), and nonspecific cation-conducting channels (HCN2, HCN4). The pathophysiology may involve autonomic dysfunction and structural cardiac disease, as both are independently associated with epilepsy and ion channel dysregulation. Indeed, in vivo and in vitro studies of cardiac pathology reveal a complex network of signaling pathways and transcription factors regulating ion channel expression in the setting of sympathetic overactivity, cardiac failure, and hypertrophy. Other mechanisms such as circulating inflammatory mediators or exogenous effects of antiepileptic medications lack evidence. Moreover, an acquired cardiac channelopathy may underlie the electrophysiologic cardiac abnormalities seen in chronic epilepsy, potentially contributing to the increased risk of malignant arrhythmias and sudden death. Therefore, further investigation is necessary to establish whether cardiac ion channel dysregulation similarly occurs in patients with epilepsy, and to characterize any pathogenic relationship with SUDEP.
DOI: https://dx.doi.org/10.1111/epi.16301
توضیحات مهم:
✅ نویسندگان در این مرور انتقادی عالی به بررسی نقش کانال های قلبی ، به ویژه از دیدگاه SUDEP و اختلال عملکرد قلبی پرداخته اند.
✔️ نویسندگان بیشتر به نقش جهش های کانال یونی ارثی که به طور مشترک در مغز و قلب بیان می شوند و رابطه بین صرع ، اختلال عملکرد قلبی و تغییرات مولکولی را برجسته می کنند، پرداختند.
✔️ نویسندگان در این مطالعه نشان دادند که یک کانالوپاتی قلبی اکتسابی ممکن است تا حدودی حالت پیش آریتمی که در صرع دیده می شود را تسهیل کند و به خطر مرگ ناگهانی کمک کند.
مجموعه مدیتورها خواندن این مقاله مروری ارزشمند را به همه دوستان علاقمند به علوم پایه، نورو و قلب پیشنهاد می دهد.
🔎 @Meditorha
#Cardiology
#Neurology
#BasicSciences
عنوان:
Acquired Cardiac Channelopathies in Epilepsy
نوع مطالعه:
Review
مجله:
Epilepsia
تاریخ انتشار:
01, Sep, 2019
چکیده:
There is growing evidence that cardiac dysfunction in patients with chronic epilepsy could play a pathogenic role in sudden unexpected death in epilepsy (SUDEP). Recent animal studies have revealed that epilepsy secondarily alters the expression of cardiac ion channels alongside abnormal cardiac electrophysiology and remodeling. These molecular findings represent novel evidence for an acquired cardiac channelopathy in epilepsy, distinct from inherited ion channels mutations associated with cardiocerebral phenotypes. Specifically, seizure activity has been shown to alter the messenger RNA (mRNA) and protein expression of voltage-gated sodium channels (Nav 1.1, Nav 1.5), voltage-gated potassium channels (Kv 4.2, Kv 4.3), sodium-calcium exchangers (NCX1), and nonspecific cation-conducting channels (HCN2, HCN4). The pathophysiology may involve autonomic dysfunction and structural cardiac disease, as both are independently associated with epilepsy and ion channel dysregulation. Indeed, in vivo and in vitro studies of cardiac pathology reveal a complex network of signaling pathways and transcription factors regulating ion channel expression in the setting of sympathetic overactivity, cardiac failure, and hypertrophy. Other mechanisms such as circulating inflammatory mediators or exogenous effects of antiepileptic medications lack evidence. Moreover, an acquired cardiac channelopathy may underlie the electrophysiologic cardiac abnormalities seen in chronic epilepsy, potentially contributing to the increased risk of malignant arrhythmias and sudden death. Therefore, further investigation is necessary to establish whether cardiac ion channel dysregulation similarly occurs in patients with epilepsy, and to characterize any pathogenic relationship with SUDEP.
DOI: https://dx.doi.org/10.1111/epi.16301
توضیحات مهم:
✅ نویسندگان در این مرور انتقادی عالی به بررسی نقش کانال های قلبی ، به ویژه از دیدگاه SUDEP و اختلال عملکرد قلبی پرداخته اند.
✔️ نویسندگان بیشتر به نقش جهش های کانال یونی ارثی که به طور مشترک در مغز و قلب بیان می شوند و رابطه بین صرع ، اختلال عملکرد قلبی و تغییرات مولکولی را برجسته می کنند، پرداختند.
✔️ نویسندگان در این مطالعه نشان دادند که یک کانالوپاتی قلبی اکتسابی ممکن است تا حدودی حالت پیش آریتمی که در صرع دیده می شود را تسهیل کند و به خطر مرگ ناگهانی کمک کند.
مجموعه مدیتورها خواندن این مقاله مروری ارزشمند را به همه دوستان علاقمند به علوم پایه، نورو و قلب پیشنهاد می دهد.
🔎 @Meditorha
Wiley Online Library
Acquired cardiac channelopathies in epilepsy: Evidence, mechanisms, and clinical significance
There is growing evidence that cardiac dysfunction in patients with chronic epilepsy could play a pathogenic role in sudden unexpected death in epilepsy (SUDEP). Recent animal studies have revealed t...
مقاله شماره ۲۴
#Cardiology
#Neurology
#Pharmacology
#BasicSciences
#Psychology
عنوان:
No Association Between Statin Use and Memory, Cognition, or Brain Volume in the Elderly
نوع مطالعه:
Prospective observational study
مجله:
JACC
تاریخ انتشار:
Nov, 2019
چکیده:
BACKGROUND
There is widespread consumer concern that statin use may be associated with impaired memory and cognitive decline.
OBJECTIVES
This study sought to examine the association between statin use and changes in memory and global cognition in the elderly population over 6 years and brain volumes over 2 years. Interactions between statin use and known dementia risk factors were examined.
METHODS
Prospective observational study of community-dwelling elderly Australians age 70 to 90 years (the MAS [Sydney Memory and Ageing Study], n = 1,037). Outcome measures were memory and global cognition (by neuropsychological testing every 2 years) and total brain, hippocampal and parahippocampal volumes (by magnetic resonance) in a subgroup (n = 526). Analyses applied linear mixed modeling, including the covariates of age, sex, education, body mass index, heart disease, diabetes, hypertension, stroke, smoking, and apolipoprotein Eε4 carriage. Interactions were sought between statin use and dementia risk factors.
RESULTS
Over 6 years there was no difference in the rate of decline in memory or global cognition between statin users and never users. Statin initiation during the observation period was associated with blunting the rate of memory decline. Exploratory analyses found statin use was associated with attenuated decline in specific memory test performance in participants with heart disease and apolipoprotein Eε4 carriage. There was no difference in brain volume changes between statin users and never users.
CONCLUSIONS
In community-dwelling elderly Australians, statin therapy was not associated with any greater decline in memory or cognition over 6 years. These data are reassuring for consumers concerned about statin use and risk of memory decline.
DOI: 10.1016/j.jacc.2019.09.041
توضیحات مهم:
✅ این مطالعه مشاهده ای آینده نگر ، نتایج شناختی و مغزی در 1037 سالمند ساکن جامعه استرالیا (سنین 70-90 ساله) طی یک دوره 6 ساله با توجه به قرار گرفتن در معرض استاتین مقایسه کرده است.
✔️ افراد شرکت کننده در این مطالعه شامل 395 نفر بودند که هرگز از استاتین (never users) استفاده نکرده بودند و 642 نفر در برخی موارد از استاتین استفاده کرده بودند (ever users).
✔️ همه شرکت کنندگان هر 2 سال یکبار تحت آزمایش neuropsychological قرار گرفتند ، و 526 نیز تحت ارزیابی MRI قرار گرفتند تا حجم مغز ، هیپوکامپ و پاراهیپوکامپ را ارزیابی کنند.
✔️ هیچ مدرکی مبنی بر تفاوت در میزان کاهش شناخت گلوبال یا حافظه بین گروه های never user و ever user مشاهده نشد.
✔️ در مقایسه با never user ها ، در 99 شرکت کننده که در طول مطالعه شروع به درمان استاتین کردند ، میزان کاهش حافظه ضعیف بود ، اما در شناخت global هیچ تفاوتی نداشتند.
✔️ تغییرات حجم مغز بین گروهها مشابه بود.
✔️✔️ با اینکه این مطالعه دارای محدودیت از نظر حجم نمونه و مشاهده ای بودن می باشد، اما به بیماران سالمند تا حدودی اطمینان خاطر می دهد که نگران اثرات #استاتین روی شناخت نباشند.
🔎 @Meditorha
#Cardiology
#Neurology
#Pharmacology
#BasicSciences
#Psychology
عنوان:
No Association Between Statin Use and Memory, Cognition, or Brain Volume in the Elderly
نوع مطالعه:
Prospective observational study
مجله:
JACC
تاریخ انتشار:
Nov, 2019
چکیده:
BACKGROUND
There is widespread consumer concern that statin use may be associated with impaired memory and cognitive decline.
OBJECTIVES
This study sought to examine the association between statin use and changes in memory and global cognition in the elderly population over 6 years and brain volumes over 2 years. Interactions between statin use and known dementia risk factors were examined.
METHODS
Prospective observational study of community-dwelling elderly Australians age 70 to 90 years (the MAS [Sydney Memory and Ageing Study], n = 1,037). Outcome measures were memory and global cognition (by neuropsychological testing every 2 years) and total brain, hippocampal and parahippocampal volumes (by magnetic resonance) in a subgroup (n = 526). Analyses applied linear mixed modeling, including the covariates of age, sex, education, body mass index, heart disease, diabetes, hypertension, stroke, smoking, and apolipoprotein Eε4 carriage. Interactions were sought between statin use and dementia risk factors.
RESULTS
Over 6 years there was no difference in the rate of decline in memory or global cognition between statin users and never users. Statin initiation during the observation period was associated with blunting the rate of memory decline. Exploratory analyses found statin use was associated with attenuated decline in specific memory test performance in participants with heart disease and apolipoprotein Eε4 carriage. There was no difference in brain volume changes between statin users and never users.
CONCLUSIONS
In community-dwelling elderly Australians, statin therapy was not associated with any greater decline in memory or cognition over 6 years. These data are reassuring for consumers concerned about statin use and risk of memory decline.
DOI: 10.1016/j.jacc.2019.09.041
توضیحات مهم:
✅ این مطالعه مشاهده ای آینده نگر ، نتایج شناختی و مغزی در 1037 سالمند ساکن جامعه استرالیا (سنین 70-90 ساله) طی یک دوره 6 ساله با توجه به قرار گرفتن در معرض استاتین مقایسه کرده است.
✔️ افراد شرکت کننده در این مطالعه شامل 395 نفر بودند که هرگز از استاتین (never users) استفاده نکرده بودند و 642 نفر در برخی موارد از استاتین استفاده کرده بودند (ever users).
✔️ همه شرکت کنندگان هر 2 سال یکبار تحت آزمایش neuropsychological قرار گرفتند ، و 526 نیز تحت ارزیابی MRI قرار گرفتند تا حجم مغز ، هیپوکامپ و پاراهیپوکامپ را ارزیابی کنند.
✔️ هیچ مدرکی مبنی بر تفاوت در میزان کاهش شناخت گلوبال یا حافظه بین گروه های never user و ever user مشاهده نشد.
✔️ در مقایسه با never user ها ، در 99 شرکت کننده که در طول مطالعه شروع به درمان استاتین کردند ، میزان کاهش حافظه ضعیف بود ، اما در شناخت global هیچ تفاوتی نداشتند.
✔️ تغییرات حجم مغز بین گروهها مشابه بود.
✔️✔️ با اینکه این مطالعه دارای محدودیت از نظر حجم نمونه و مشاهده ای بودن می باشد، اما به بیماران سالمند تا حدودی اطمینان خاطر می دهد که نگران اثرات #استاتین روی شناخت نباشند.
🔎 @Meditorha
مقاله شماره ۲۶
#Neurology
عنوان:
Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy
نوع مطالعه:
Review
مجله:
JAMA Neurology
تاریخ انتشار:
Sep, 2019
چکیده:
IMPORTANCE
Small-fiber polyneuropathy involves preferential damage to the thinly myelinated A-delta fibers, unmyelinated C sensory fibers, or autonomic or trophic fibers. Although this condition is common, most patients still remain undiagnosed and untreated because of lagging medical and public awareness of research advances. Chronic bilateral neuropathic pain, fatigue, and nausea are cardinal symptoms that can cause disability and dependence, including pain medication dependence.
OBSERVATIONS
Biomarker confirmation is recommended, given the nonspecificity of symptoms. The standard test involves measuring epidermal neurite density within a 3-mm protein gene product 9.5 (PGP9.5)-immunolabeled lower-leg skin biopsy. Biopsies and autonomic function testing confirm that small-fiber neuropathy not uncommonly affects otherwise healthy children and young adults, in whom it is often associated with inflammation or dysimmunity. A recent meta-analysis concluded that small-fiber neuropathy underlies 49% of illnesses labeled as fibromyalgia. Initially, patients with idiopathic small-fiber disorders should be screened by medical history and blood tests for potentially treatable causes, which are identifiable in one-third to one-half of patients. Then, secondary genetic testing is particularly important for familial and childhood cases. Treatable genetic causes include Fabry disease, transthyretin and primary systemic amyloidosis, hereditary sensory autonomic neuropathy-1, and ion-channel mutations. Immunohistopathologic evidence suggests that small-fiber dysfunction and denervation, especially of blood vessels, contributes to diverse symptoms, including postexertional malaise, postural orthostatic tachycardia, and functional gastrointestinal distress. Preliminary evidence implicates acute or chronic autoreactivity in some cases, particularly in female patients and otherwise healthy children and young adults. Different temporal patterns akin to Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy have been described; here, corticosteroids and immunoglobulins, which are often efficacious for inflammatory neuropathic conditions, are increasingly considered.
CONCLUSIONS AND RELEVANCE
Because small fibers normally grow throughout life, improving contributory conditions may permit regrowth, slow progression, and prevent permanent damage. The prognosis is often hopeful for improving quality of life and sometimes for abatement or resolution, particularly in the young and otherwise healthy individuals. Examples include diabetic, infectious, toxic, genetic, and inflammatory causes. The current standard of care requires prompt diagnosis and treatment, particularly in children and young adults, to restore life trajectory. Consensus diagnostic and tracking metrics should be established to facilitate treatment trials.
https://dx.doi.org/10.1001/jamaneurol.2019.2917
توضیحات مهم:
✅ نویسندگان این مطالعه ، به روزرسانی در مورد وضعیت پژوهش و رویکردهای بالینی small-fiber polyneuropathy ، فراهم کردند.
✔️ پلی نوروپاتی small-fibet یک بیماری شایع است با این وجود بسیاری از بیماران تشخیص داده نشده یا اشتباه تشخیص داده می شوند. تظاهرات بالینی شامل درد ، همراه با یافته های autonomic است ، و اریترومالالژیا stocking-and-glove یک نمایش کلاسیک محسوب می شود.
ادامه این مقاله ارزشمند را از فایل زیر بخوانید ...
🔎 @Meditorha
#Neurology
عنوان:
Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy
نوع مطالعه:
Review
مجله:
JAMA Neurology
تاریخ انتشار:
Sep, 2019
چکیده:
IMPORTANCE
Small-fiber polyneuropathy involves preferential damage to the thinly myelinated A-delta fibers, unmyelinated C sensory fibers, or autonomic or trophic fibers. Although this condition is common, most patients still remain undiagnosed and untreated because of lagging medical and public awareness of research advances. Chronic bilateral neuropathic pain, fatigue, and nausea are cardinal symptoms that can cause disability and dependence, including pain medication dependence.
OBSERVATIONS
Biomarker confirmation is recommended, given the nonspecificity of symptoms. The standard test involves measuring epidermal neurite density within a 3-mm protein gene product 9.5 (PGP9.5)-immunolabeled lower-leg skin biopsy. Biopsies and autonomic function testing confirm that small-fiber neuropathy not uncommonly affects otherwise healthy children and young adults, in whom it is often associated with inflammation or dysimmunity. A recent meta-analysis concluded that small-fiber neuropathy underlies 49% of illnesses labeled as fibromyalgia. Initially, patients with idiopathic small-fiber disorders should be screened by medical history and blood tests for potentially treatable causes, which are identifiable in one-third to one-half of patients. Then, secondary genetic testing is particularly important for familial and childhood cases. Treatable genetic causes include Fabry disease, transthyretin and primary systemic amyloidosis, hereditary sensory autonomic neuropathy-1, and ion-channel mutations. Immunohistopathologic evidence suggests that small-fiber dysfunction and denervation, especially of blood vessels, contributes to diverse symptoms, including postexertional malaise, postural orthostatic tachycardia, and functional gastrointestinal distress. Preliminary evidence implicates acute or chronic autoreactivity in some cases, particularly in female patients and otherwise healthy children and young adults. Different temporal patterns akin to Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy have been described; here, corticosteroids and immunoglobulins, which are often efficacious for inflammatory neuropathic conditions, are increasingly considered.
CONCLUSIONS AND RELEVANCE
Because small fibers normally grow throughout life, improving contributory conditions may permit regrowth, slow progression, and prevent permanent damage. The prognosis is often hopeful for improving quality of life and sometimes for abatement or resolution, particularly in the young and otherwise healthy individuals. Examples include diabetic, infectious, toxic, genetic, and inflammatory causes. The current standard of care requires prompt diagnosis and treatment, particularly in children and young adults, to restore life trajectory. Consensus diagnostic and tracking metrics should be established to facilitate treatment trials.
https://dx.doi.org/10.1001/jamaneurol.2019.2917
توضیحات مهم:
✅ نویسندگان این مطالعه ، به روزرسانی در مورد وضعیت پژوهش و رویکردهای بالینی small-fiber polyneuropathy ، فراهم کردند.
✔️ پلی نوروپاتی small-fibet یک بیماری شایع است با این وجود بسیاری از بیماران تشخیص داده نشده یا اشتباه تشخیص داده می شوند. تظاهرات بالینی شامل درد ، همراه با یافته های autonomic است ، و اریترومالالژیا stocking-and-glove یک نمایش کلاسیک محسوب می شود.
ادامه این مقاله ارزشمند را از فایل زیر بخوانید ...
🔎 @Meditorha
Jamanetwork
Advances in Clinical Approaches to Small-Fiber Polyneuropathy
This narrative review describes the clinical characteristics and pathophysiological underpinnings of small-fiber polyneuropathy, as well as approaches to diagnosis and treatment.
مقاله شماره ۲۷
#Pain
#Neurology
عنوان:
Shooting Pain" in Lumbar Radiculopathy and Trigeminal Neuralgia and Ideas Concerning Its Neural Substrates
مجله:
Pain
تاریخ انتشار:
Oct, 2019
چکیده:
Patients with radicular low back pain (radicular LBP, sciatica) frequently describe their pain as "shooting" or "radiating". The dictionary meaning of these words implies rapid movement, and indeed, many sufferers report feeling pain moving rapidly from the lower back or buttock into the leg. But others do not. Moreover, the sensation of movement is paradoxical; it is neither predicted nor accounted for by current ideas about the pathophysiology of radicular LBP. We have employed a structured questionnaire to evaluate the sensory qualities associated with "shooting" and "radiating" in 155 patients, 98 with radicular LBP and 57 with trigeminal neuralgia (TN), a second chronic pain condition in which shooting/radiating are experienced. Results indicated a spectrum of different sensations in different people. While many sciatica patients reported rapid downward movement of their pain, even more reported downward expansion of the area of pain, some reported upward movement and for some there was no spatial dynamic at all. The velocity of movement or expansion was also variable. By cross-referencing sensations experienced in the sciatica and TN cohorts with known signal processing modes in the somatosensory system, we propose testable hypotheses concerning the pathophysiology of the various vectorial sensations reported, their direction and velocity, and the structures in which they are generated. Systematic evaluation of qualitative features of "shooting" and "radiating" pain at the time of diagnosis can shed light on the pain mechanism in the individual patient and perhaps contribute to a better therapeutic outcomes.
DOI: 10.1097/j.pain.0000000000001729
🔎 @Meditorha
#Pain
#Neurology
عنوان:
Shooting Pain" in Lumbar Radiculopathy and Trigeminal Neuralgia and Ideas Concerning Its Neural Substrates
مجله:
Pain
تاریخ انتشار:
Oct, 2019
چکیده:
Patients with radicular low back pain (radicular LBP, sciatica) frequently describe their pain as "shooting" or "radiating". The dictionary meaning of these words implies rapid movement, and indeed, many sufferers report feeling pain moving rapidly from the lower back or buttock into the leg. But others do not. Moreover, the sensation of movement is paradoxical; it is neither predicted nor accounted for by current ideas about the pathophysiology of radicular LBP. We have employed a structured questionnaire to evaluate the sensory qualities associated with "shooting" and "radiating" in 155 patients, 98 with radicular LBP and 57 with trigeminal neuralgia (TN), a second chronic pain condition in which shooting/radiating are experienced. Results indicated a spectrum of different sensations in different people. While many sciatica patients reported rapid downward movement of their pain, even more reported downward expansion of the area of pain, some reported upward movement and for some there was no spatial dynamic at all. The velocity of movement or expansion was also variable. By cross-referencing sensations experienced in the sciatica and TN cohorts with known signal processing modes in the somatosensory system, we propose testable hypotheses concerning the pathophysiology of the various vectorial sensations reported, their direction and velocity, and the structures in which they are generated. Systematic evaluation of qualitative features of "shooting" and "radiating" pain at the time of diagnosis can shed light on the pain mechanism in the individual patient and perhaps contribute to a better therapeutic outcomes.
DOI: 10.1097/j.pain.0000000000001729
🔎 @Meditorha
مقاله شماره ۳۵
#Neurology
#NeuroScience
#Genetic
عنوان:
Expansion of GGC Repeat in the Human-Specific NOTCH2NLC Gene Is Associated With Essential Tremor
مجله:
Brain: A Journal of Neurology
نوع مطالعه:
Cohort Study
تاریخ انتشار:
Dec, 09, 2019
سایتیشن:
Sun et al.
چکیده:
Essential tremor is one of the most common movement disorders. Despite its high prevalence and heritability, the genetic aetiology of essential tremor remains elusive. Up to now, only a few genes/loci have been identified, but these genes have not been replicated in other essential tremor families or cohorts. Here we report a genetic study in a cohort of 197 Chinese pedigrees clinically diagnosed with essential tremor. Using a comprehensive strategy combining linkage analysis, whole-exome sequencing, long-read whole-genome sequencing, repeat-primed polymerase chain reaction and GC-rich polymerase chain reaction, we identified an abnormal GGC repeat expansion in the 5′ region of the NOTCH2NLC gene that co-segregated with disease in 11 essential tremor families (5.58%) from our cohort. Clinically, probands that had an abnormal GGC repeat expansion were found to have more severe tremor phenotypes, lower activities of daily living ability. Obvious genetic anticipation was also detected in these 11 essential tremor-positive families. These results indicate that abnormal GGC repeat expansion in the 5′ region of NOTCH2NLC gene is associated with essential tremor, and provide strong evidence that essential tremor is a family of diseases with high clinical and genetic heterogeneities.
توضیحات مهم:
✔ نویسندگان این مطالعه به بررسی علل ژنتیکی احتمالی Essential Tremor فمیلیال در 197 شجره چینی پرداختند.
✔ آنها 11 خانواده با یک تری نوکلئوتید GGC تکرار شده در ژن NOTCH2NLC با پیش بینی ژنتیکی essential tremor پیدا کردند. این ژن همچنین در بیماری neuronal intranuclear inclusion نقش دارد.
✔ در حال حاضر ، مشخص نیست که چگونه این ژن بر فنوتیپ تأثیر می گذارد. با این حال ، این گزارش نشان دهنده اهمیت تکنیک های جدید توالی ژنتیکی در درک پاتوفیزیولوژی پیچیده اختلالات عصبی است.
🔎 @Meditorha
#Neurology
#NeuroScience
#Genetic
عنوان:
Expansion of GGC Repeat in the Human-Specific NOTCH2NLC Gene Is Associated With Essential Tremor
مجله:
Brain: A Journal of Neurology
نوع مطالعه:
Cohort Study
تاریخ انتشار:
Dec, 09, 2019
سایتیشن:
Sun et al.
چکیده:
Essential tremor is one of the most common movement disorders. Despite its high prevalence and heritability, the genetic aetiology of essential tremor remains elusive. Up to now, only a few genes/loci have been identified, but these genes have not been replicated in other essential tremor families or cohorts. Here we report a genetic study in a cohort of 197 Chinese pedigrees clinically diagnosed with essential tremor. Using a comprehensive strategy combining linkage analysis, whole-exome sequencing, long-read whole-genome sequencing, repeat-primed polymerase chain reaction and GC-rich polymerase chain reaction, we identified an abnormal GGC repeat expansion in the 5′ region of the NOTCH2NLC gene that co-segregated with disease in 11 essential tremor families (5.58%) from our cohort. Clinically, probands that had an abnormal GGC repeat expansion were found to have more severe tremor phenotypes, lower activities of daily living ability. Obvious genetic anticipation was also detected in these 11 essential tremor-positive families. These results indicate that abnormal GGC repeat expansion in the 5′ region of NOTCH2NLC gene is associated with essential tremor, and provide strong evidence that essential tremor is a family of diseases with high clinical and genetic heterogeneities.
توضیحات مهم:
✔ نویسندگان این مطالعه به بررسی علل ژنتیکی احتمالی Essential Tremor فمیلیال در 197 شجره چینی پرداختند.
✔ آنها 11 خانواده با یک تری نوکلئوتید GGC تکرار شده در ژن NOTCH2NLC با پیش بینی ژنتیکی essential tremor پیدا کردند. این ژن همچنین در بیماری neuronal intranuclear inclusion نقش دارد.
✔ در حال حاضر ، مشخص نیست که چگونه این ژن بر فنوتیپ تأثیر می گذارد. با این حال ، این گزارش نشان دهنده اهمیت تکنیک های جدید توالی ژنتیکی در درک پاتوفیزیولوژی پیچیده اختلالات عصبی است.
🔎 @Meditorha
مقاله شماره ۳۶
#Neurology
#NeuroScience
#Psychiatry
عنوان:
Sleep duration, midday napping, and sleep quality and incident stroke
مجله:
Neurology
نوع مطالعه:
Cohort Study
تاریخ انتشار:
Dec, 11, 2019
سایتیشن:
Zhou et al.
چکیده:
Objective To investigate the associations of sleep duration, midday napping, sleep quality, and change in sleep duration with risk of incident stroke and stroke subtypes.
Methods Among 31,750 participants aged 61.7 years on average at baseline from the Dongfeng-Tongji cohort, we used Cox regression models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for incident stroke.
Results Compared with sleeping 7 to <8 hours/night, those reporting longer sleep duration (≥9 hours/night) had a greater risk of total stroke (hazard ratio [HR] 1.23; 95% confidence interval [CI] 1.07–1.41), while shorter sleep (<6 hours/night) had no significant effect on stroke risk. The HR (95% CI) of total stroke was 1.25 (1.03–1.53) for midday napping >90 minutes vs 1–30 minutes. The results were similar for ischemic stroke. Compared with good sleep quality, those with poor sleep quality showed a 29%, 28%, and 56% higher risk of total, ischemic, and hemorrhagic stroke, respectively. Moreover, we observed significant joint effects of sleeping ≥9 hours/night and midday napping >90 minutes (HR 1.85; 95% CI 1.28–2.66), and sleeping ≥9 hours/night and poor sleep quality (HR 1.82; 95% CI 1.33–2.48) on risk of total stroke. Furthermore, compared with persistently sleeping 7–9 hours/night, those who persistently slept ≥9 hours/night or switched from 7 to 9 hours to ≥9 hours/night had a higher risk of total stroke.
Conclusions Long sleep duration, long midday napping, and poor sleep quality were independently and jointly associated with higher risks of incident stroke. Persistently long sleep duration or switch from average to long sleep duration increased the risk of stroke.
https://doi.org/10.1212/WNL.0000000000008739
توضیحات مهم:
✔ خواب طولانی ریسک سکته ی مغزی را افزایش می دهد !
یک مطالعه با بررسی ۳۱۷۵۰ نفر به این نتیجه رسیده است که خواب شبانه بیش از ۹ ساعت (در مقایسه با خواب بین ۷ تا کمتر از ۸ ساعت) ریسک سکته ی مغزی را افزایش می دهد. این در حالی است که خواب کمتر از ۶ ساعت چنین تاثیری ندارد. از سوی دیگر این مطالعه نشان داده است که خواب نیمروزی بیش از ۹۰ دقیقه نیز می تواند ریسک سکته ی مغزی را افزایش دهد.
🔎 @Meditorha
#Neurology
#NeuroScience
#Psychiatry
عنوان:
Sleep duration, midday napping, and sleep quality and incident stroke
مجله:
Neurology
نوع مطالعه:
Cohort Study
تاریخ انتشار:
Dec, 11, 2019
سایتیشن:
Zhou et al.
چکیده:
Objective To investigate the associations of sleep duration, midday napping, sleep quality, and change in sleep duration with risk of incident stroke and stroke subtypes.
Methods Among 31,750 participants aged 61.7 years on average at baseline from the Dongfeng-Tongji cohort, we used Cox regression models to estimate hazard ratios (HRs) and 95% confidence intervals (CIs) for incident stroke.
Results Compared with sleeping 7 to <8 hours/night, those reporting longer sleep duration (≥9 hours/night) had a greater risk of total stroke (hazard ratio [HR] 1.23; 95% confidence interval [CI] 1.07–1.41), while shorter sleep (<6 hours/night) had no significant effect on stroke risk. The HR (95% CI) of total stroke was 1.25 (1.03–1.53) for midday napping >90 minutes vs 1–30 minutes. The results were similar for ischemic stroke. Compared with good sleep quality, those with poor sleep quality showed a 29%, 28%, and 56% higher risk of total, ischemic, and hemorrhagic stroke, respectively. Moreover, we observed significant joint effects of sleeping ≥9 hours/night and midday napping >90 minutes (HR 1.85; 95% CI 1.28–2.66), and sleeping ≥9 hours/night and poor sleep quality (HR 1.82; 95% CI 1.33–2.48) on risk of total stroke. Furthermore, compared with persistently sleeping 7–9 hours/night, those who persistently slept ≥9 hours/night or switched from 7 to 9 hours to ≥9 hours/night had a higher risk of total stroke.
Conclusions Long sleep duration, long midday napping, and poor sleep quality were independently and jointly associated with higher risks of incident stroke. Persistently long sleep duration or switch from average to long sleep duration increased the risk of stroke.
https://doi.org/10.1212/WNL.0000000000008739
توضیحات مهم:
✔ خواب طولانی ریسک سکته ی مغزی را افزایش می دهد !
یک مطالعه با بررسی ۳۱۷۵۰ نفر به این نتیجه رسیده است که خواب شبانه بیش از ۹ ساعت (در مقایسه با خواب بین ۷ تا کمتر از ۸ ساعت) ریسک سکته ی مغزی را افزایش می دهد. این در حالی است که خواب کمتر از ۶ ساعت چنین تاثیری ندارد. از سوی دیگر این مطالعه نشان داده است که خواب نیمروزی بیش از ۹۰ دقیقه نیز می تواند ریسک سکته ی مغزی را افزایش دهد.
🔎 @Meditorha
Neurology
Sleep duration, midday napping, and sleep quality and incident stroke
Objective To investigate the associations of sleep duration, midday napping, sleep quality, and change in sleep duration with risk of incident stroke and stroke subtypes.
Methods Among 31,750 participants aged 61.7 years on average at baseline from the Dongfeng…
Methods Among 31,750 participants aged 61.7 years on average at baseline from the Dongfeng…
Forwarded from Meditorha.com | مدیتورها
مقاله شماره ۲۴
#Cardiology
#Neurology
#Pharmacology
#BasicSciences
#Psychology
عنوان:
No Association Between Statin Use and Memory, Cognition, or Brain Volume in the Elderly
نوع مطالعه:
Prospective observational study
مجله:
JACC
تاریخ انتشار:
Nov, 2019
چکیده:
BACKGROUND
There is widespread consumer concern that statin use may be associated with impaired memory and cognitive decline.
OBJECTIVES
This study sought to examine the association between statin use and changes in memory and global cognition in the elderly population over 6 years and brain volumes over 2 years. Interactions between statin use and known dementia risk factors were examined.
METHODS
Prospective observational study of community-dwelling elderly Australians age 70 to 90 years (the MAS [Sydney Memory and Ageing Study], n = 1,037). Outcome measures were memory and global cognition (by neuropsychological testing every 2 years) and total brain, hippocampal and parahippocampal volumes (by magnetic resonance) in a subgroup (n = 526). Analyses applied linear mixed modeling, including the covariates of age, sex, education, body mass index, heart disease, diabetes, hypertension, stroke, smoking, and apolipoprotein Eε4 carriage. Interactions were sought between statin use and dementia risk factors.
RESULTS
Over 6 years there was no difference in the rate of decline in memory or global cognition between statin users and never users. Statin initiation during the observation period was associated with blunting the rate of memory decline. Exploratory analyses found statin use was associated with attenuated decline in specific memory test performance in participants with heart disease and apolipoprotein Eε4 carriage. There was no difference in brain volume changes between statin users and never users.
CONCLUSIONS
In community-dwelling elderly Australians, statin therapy was not associated with any greater decline in memory or cognition over 6 years. These data are reassuring for consumers concerned about statin use and risk of memory decline.
DOI: 10.1016/j.jacc.2019.09.041
توضیحات مهم:
✅ این مطالعه مشاهده ای آینده نگر ، نتایج شناختی و مغزی در 1037 سالمند ساکن جامعه استرالیا (سنین 70-90 ساله) طی یک دوره 6 ساله با توجه به قرار گرفتن در معرض استاتین مقایسه کرده است.
✔️ افراد شرکت کننده در این مطالعه شامل 395 نفر بودند که هرگز از استاتین (never users) استفاده نکرده بودند و 642 نفر در برخی موارد از استاتین استفاده کرده بودند (ever users).
✔️ همه شرکت کنندگان هر 2 سال یکبار تحت آزمایش neuropsychological قرار گرفتند ، و 526 نیز تحت ارزیابی MRI قرار گرفتند تا حجم مغز ، هیپوکامپ و پاراهیپوکامپ را ارزیابی کنند.
✔️ هیچ مدرکی مبنی بر تفاوت در میزان کاهش شناخت گلوبال یا حافظه بین گروه های never user و ever user مشاهده نشد.
✔️ در مقایسه با never user ها ، در 99 شرکت کننده که در طول مطالعه شروع به درمان استاتین کردند ، میزان کاهش حافظه ضعیف بود ، اما در شناخت global هیچ تفاوتی نداشتند.
✔️ تغییرات حجم مغز بین گروهها مشابه بود.
✔️✔️ با اینکه این مطالعه دارای محدودیت از نظر حجم نمونه و مشاهده ای بودن می باشد، اما به بیماران سالمند تا حدودی اطمینان خاطر می دهد که نگران اثرات #استاتین روی شناخت نباشند.
🔎 @Meditorha
#Cardiology
#Neurology
#Pharmacology
#BasicSciences
#Psychology
عنوان:
No Association Between Statin Use and Memory, Cognition, or Brain Volume in the Elderly
نوع مطالعه:
Prospective observational study
مجله:
JACC
تاریخ انتشار:
Nov, 2019
چکیده:
BACKGROUND
There is widespread consumer concern that statin use may be associated with impaired memory and cognitive decline.
OBJECTIVES
This study sought to examine the association between statin use and changes in memory and global cognition in the elderly population over 6 years and brain volumes over 2 years. Interactions between statin use and known dementia risk factors were examined.
METHODS
Prospective observational study of community-dwelling elderly Australians age 70 to 90 years (the MAS [Sydney Memory and Ageing Study], n = 1,037). Outcome measures were memory and global cognition (by neuropsychological testing every 2 years) and total brain, hippocampal and parahippocampal volumes (by magnetic resonance) in a subgroup (n = 526). Analyses applied linear mixed modeling, including the covariates of age, sex, education, body mass index, heart disease, diabetes, hypertension, stroke, smoking, and apolipoprotein Eε4 carriage. Interactions were sought between statin use and dementia risk factors.
RESULTS
Over 6 years there was no difference in the rate of decline in memory or global cognition between statin users and never users. Statin initiation during the observation period was associated with blunting the rate of memory decline. Exploratory analyses found statin use was associated with attenuated decline in specific memory test performance in participants with heart disease and apolipoprotein Eε4 carriage. There was no difference in brain volume changes between statin users and never users.
CONCLUSIONS
In community-dwelling elderly Australians, statin therapy was not associated with any greater decline in memory or cognition over 6 years. These data are reassuring for consumers concerned about statin use and risk of memory decline.
DOI: 10.1016/j.jacc.2019.09.041
توضیحات مهم:
✅ این مطالعه مشاهده ای آینده نگر ، نتایج شناختی و مغزی در 1037 سالمند ساکن جامعه استرالیا (سنین 70-90 ساله) طی یک دوره 6 ساله با توجه به قرار گرفتن در معرض استاتین مقایسه کرده است.
✔️ افراد شرکت کننده در این مطالعه شامل 395 نفر بودند که هرگز از استاتین (never users) استفاده نکرده بودند و 642 نفر در برخی موارد از استاتین استفاده کرده بودند (ever users).
✔️ همه شرکت کنندگان هر 2 سال یکبار تحت آزمایش neuropsychological قرار گرفتند ، و 526 نیز تحت ارزیابی MRI قرار گرفتند تا حجم مغز ، هیپوکامپ و پاراهیپوکامپ را ارزیابی کنند.
✔️ هیچ مدرکی مبنی بر تفاوت در میزان کاهش شناخت گلوبال یا حافظه بین گروه های never user و ever user مشاهده نشد.
✔️ در مقایسه با never user ها ، در 99 شرکت کننده که در طول مطالعه شروع به درمان استاتین کردند ، میزان کاهش حافظه ضعیف بود ، اما در شناخت global هیچ تفاوتی نداشتند.
✔️ تغییرات حجم مغز بین گروهها مشابه بود.
✔️✔️ با اینکه این مطالعه دارای محدودیت از نظر حجم نمونه و مشاهده ای بودن می باشد، اما به بیماران سالمند تا حدودی اطمینان خاطر می دهد که نگران اثرات #استاتین روی شناخت نباشند.
🔎 @Meditorha