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Advances in Nutrition (Bethesda, Md.) Dec 2022We studied associations between prenatal and early postnatal choline intake, brain development, and neurocognitive function of children. We conducted a systematic review... (Meta-Analysis)
Meta-Analysis
We studied associations between prenatal and early postnatal choline intake, brain development, and neurocognitive function of children. We conducted a systematic review followed by a meta-analysis and critical appraisal of human studies published from 1997 to 2021. Thirty publications were identified. The meta-analysis included 5 of 7 case-control studies studying neural tube defects (NTDs) in relation to maternal choline intakes/circulating concentrations. Low maternal choline intake/circulating concentrations were associated with a higher OR for NTDs among 1131 mothers of newborns with NTDs and 4439 control mothers (pooled estimate = 1.36; 95% CI: 1.11, 1.67). The 95% prediction intervals were 0.78, 2.36. Findings and critical evaluation of 10 publications with interventional designs showed that higher maternal choline intakes during the second half of pregnancy and early postnatal period (550 mg up to 1 g/d on top of the diet) or a child intake of 513 to 625 mg/d from supplements were safe and likely to demonstrate favorable effects on several domains of child neurocognition, such as memory, attention, and visuospatial learning versus the comparators. Findings from observational studies (n = 13) partly supported the association between maternal choline intake/serum concentrations and child neurocognition, but there was low confidence in the use of plasma choline concentrations as a choline intake marker. In conclusion, low maternal choline intakes were associated with a higher OR for NTDs. The risk could be up to 2.36-fold in some populations. Despite limitations of available trials and observational studies, higher maternal choline intake was likely to be associated with better child neurocognition/neurodevelopment. The results should be used to guide choline intake recommendations in pregnancy and lactation, especially because most young women are not achieving the reference intake of choline. This meta-analysis is registered at PROSPERO as CRD42021233790.
Topics: Pregnancy; Humans; Child; Infant, Newborn; Female; Choline; Dietary Supplements; Vitamins; Diet; Neural Tube Defects; Brain; Child Development
PubMed: 36041182
DOI: 10.1093/advances/nmac082 -
Hormone Research in Paediatrics 2023The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is...
BACKGROUND
The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial.
SUMMARY
We report the case of a patient with an unusual association of GHD due to ectopic posterior pituitary and CM-1. Our patient developed a headache and worsening of CM-1 after the initiation of rhGH therapy. Following an atlo-occipital decompression surgery, the patient was able to resume therapy with a marked growth improvement. Based on this observation, we provide a systematic review of the current literature about these two pathologies.
KEY MESSAGES
A careful follow-up of all patients with CM-1 treated with GH is mandatory, paying particular attention to the appearance of any neurological signs and symptoms.
Topics: Humans; Arnold-Chiari Malformation; Human Growth Hormone; Dwarfism, Pituitary; Recombinant Proteins
PubMed: 36001954
DOI: 10.1159/000526617 -
The Journal of Craniofacial SurgeryEncephaloceles rarely develop following traumatic skull fractures. Given their low incidence, the clinical presentations and management strategies of these lesions are...
Encephaloceles rarely develop following traumatic skull fractures. Given their low incidence, the clinical presentations and management strategies of these lesions are confined to case reports and limited case series. A systematic literature review was performed using PubMed, Ovid, and Web of Science databases to identify relevant articles using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A total of 37 articles met inclusion criteria, including the case presented herein. These articles reported 52 traumatic encephaloceles. Mean patient age was 25.3 years (range 6 mo-66 y) with a male predominance (63%, 33/52). The most common bony defects resulting in encephalocele formation were the orbital roof (52%, 27/52), ethmoid (35%, 18/52), and sphenoid (10%, 5/52). Mean time from traumatic injury to initial presentation was 21.3 months (range 0 d-36 y) with a bimodal distribution split between immediately following the traumatic injury (57%, 26/46) or in a delayed manner (43%, 20/46). Common presentations of orbital roof, frontonasal, and temporal bone encephaloceles were exophthalmos (85%, 23/27), cerebrospinal fluid rhinorrhea (71%, 17/24), and hearing loss (100%, 4/4), respectively. Operative approach, repair technique, and materials used for encephalocele reduction were highly variable. Surgical intervention afforded definitive symptomatic improvement or resolution in the majority of cases (89%, 42/47). Clinical outcomes did not differ between orbital, frontonasal, or temporal bone encephaloceles ( P =0.438). Traumatic encephaloceles are a rare entity with diverse presenting symptomatology dependent upon the location of fracture dehiscence. Surgical intervention affords symptomatic improvement in the majority of cases irrespective of encephalocele location, time to presentation, or operative approach.
Topics: Humans; Male; Child; Female; Encephalocele; Cerebrospinal Fluid Rhinorrhea; Temporal Bone; Orbit; Hearing Loss
PubMed: 36000749
DOI: 10.1097/SCS.0000000000008955 -
Clinical Rheumatology Jan 2023Syringomyelia is an important etiology of Charcot arthropathy of the elbow. We present five interesting patients, along with a systematic literature review summarizing...
Syringomyelia is an important etiology of Charcot arthropathy of the elbow. We present five interesting patients, along with a systematic literature review summarizing the clinical profile and management of syringomyelia-induced Charcot arthropathy of the elbow. PUBMED, SCOPUS, EMBASE, and Science Direct databases were screened for English articles published between 1980 and 2022 using the search query: "Syringomyelia" AND "elbow" AND ("arthropathy" OR "neuropathic" OR "Charcot"). Articles without full text and/or lack of conclusive evidence of elbow arthropathy due to syringomyelia were excluded. The reference lists of the selected articles were reviewed to identify additional articles describing syringomyelia-induced Charcot arthropathy of the elbow. All five patients in the current series had elbow arthritis with variable motor weakness and dissociated sensory loss. The literature review included 31 reports (45 patients) and five patients from our center (n = 50). The median age at presentation was 45 (13-77) years. The median duration of arthropathy was 24 (0.5-180) months. Thirty-three patients had isolated elbow arthropathies. The other joints affected included the shoulder (n = 13), wrist (n = 7), metacarpophalangeal joints (n = 3), and interphalangeal joints (n = 1). Chiari malformations were present in 33 (66%) patients. Sensory deficits, motor deficits, and ulnar neuropathies were described in 36 (72%), 31 (62%), and 14 (28%) patients, respectively. Surgical decompression for syringomyelia was performed in 13 (26%) patients. The presence of dissociated sensory loss, with or without motor weakness, is key to the suspicion of syringomyelia-induced Charcot arthropathy of elbow. Chiari malformation and ulnar neuropathy are frequently associated with this condition. Key Points • Charcot arthropathy of elbow is not so uncommon as believed • Syringomyelia is an important etiology of Charcot arthropathy of elbow • Therefore, all patients with elbow arthropathy of unknown etiology must be evaluated for dissociative sensory loss • Chiari malformation and ulnar neuropathy are commonly associated with syringomyelia-induced Charcot arthropathy of elbow joint.
Topics: Humans; Middle Aged; Aged; Elbow Joint; Syringomyelia; Arthropathy, Neurogenic; Joints; Arnold-Chiari Malformation; Ulnar Neuropathies
PubMed: 35994178
DOI: 10.1007/s10067-022-06332-7 -
Neurology India 2022The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are... (Review)
Review
BACKGROUND
The culprit of trigeminal neuralgia (TGN) may occur at any point between the nerve's root entry zone (REZ) and Meckel's cave. Meckel's cave meningoencephaloceles are rare middle cranial fossa defects that usually remain asymptomatic but may contain prolapsed trigeminal nerve rootlets and result in TGN. Their management and surgical outcomes remain poorly understood.
OBJECTIVES
To perform a systematic review of clinical presentation and surgical outcomes of middle fossa defects presenting with trigeminal nerve-related symptoms.
MATERIALS AND METHODS
A systematic review was conducted in accordance with the PRISMA guidelines for all reports of middle cranial fossa defects causing trigeminal nerve-related symptoms. The pathophysiology, presentation, surgical management, and outcomes are discussed and illustrated with a case.
RESULTS
Initial search from inception to March 2021 identified 33 articles for screening. After applying inclusion and exclusion criteria, 6 articles were included representing a total of 8 cases in addition to our case (n = 9). All 9 patients were females and 33.3% (n = 3) presented with classic trigeminal neuralgia. "Empty sella" syndrome and radiologic signs of intracranial hypertension were present in 40%-62%. No patient presented with cerebrospinal fluid leak. The preferred treatment modality was surgical with subtemporal extradural repairs using combinations of autologous fat and muscle grafts and synthetic dura. Postoperative outcomes were only available in 55.5% (n = 5) of the cases, and nearly all reported complete symptom resolution, except for one case in which the meningoencephalocele wall was incised, along with trigeminal rootlets adhered to it. Our patient had immediate and durable symptom relief after a 4-year follow-up.
CONCLUSIONS
MEC containing prolapsed trigeminal nerve rootlets can cause typical trigeminal neuralgia from chronic pulsatile stress. This supports the hypothesis that the compressive or demyelinating culprit can locate more ventrally on the course of the trigeminal nerve. Subtemporal extradural surgical repairs can be safe, effective, and durable. Incising the MEC wall should be avoided as it may have trigeminal rootlets adhered to it.
Topics: Cranial Fossa, Middle; Dura Mater; Encephalocele; Female; Humans; Male; Meningocele; Trigeminal Nerve; Trigeminal Neuralgia
PubMed: 35864609
DOI: 10.4103/0028-3886.349629 -
European Spine Journal : Official... Oct 2022Neuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit.... (Review)
Review
BACKGROUND
Neuropathic arthropathy (NA) of the shoulder is a chronic progressive process characterized by joint destruction in the presence of a neurosensory deficit. Syringomyelia, a spinal cord disease, is the leading cause of NA in the upper extremity.
OBJECTIVE
We present a systematic review of NA with syringomyelia cases alongside a case report of an adult with NA of the shoulder that occurs a few 4 years after a revelation and surgical management of a Chiari malformation with syringomyelia.
METHODS
A systematic review was conducted following PRISMA guidelines. A PubMed, Scopus, Isiknowledge, and manual search through references of relevant publications were used to identify all published case reports of NA. Data were collected from each case report on patient characteristics.
RESULTS
The systematic review identified 56 publications and 85 patients (including ours): nearly the same number of males (n = 41) and females (n = 44). The mean age was 50,69. Presentations included reduction of mobility (n = 66), swelling (n = 61) and sensory disorder (n = 63). The pain was absent in 41 cases. In the majority of reported cases 56 (65.1%), syringomyelia was revealed by neuropathic arthropathy, and eleven patients (12.9%) had a history of syringomyelia. Treatment was categorized into non-operative management (37[43.5%]), operative management (27[31.7%]). Following-up was non-reported in 31 (36%) cases. Improvement was reported more with patients who underwent a surgical approach than medical one 28.5% versus 8.1%.
CONCLUSION
Physicians need to be more aware of this destructive joint disease, rare, and often misdiagnosed. Also, it is imperative to integrate clinical, pathological, and imaging findings for accurate diagnosis and for delivering appropriate therapy.
Topics: Adult; Arnold-Chiari Malformation; Arthropathy, Neurogenic; Female; Humans; Magnetic Resonance Imaging; Male; Shoulder; Shoulder Joint; Syringomyelia; Upper Extremity
PubMed: 35841440
DOI: 10.1007/s00586-022-07299-4 -
Nutrients Jun 2022In preconception and pregnancy, women are encouraged to take folic acid-based supplements over and above food intake. The upper tolerable limit of folic acid is 1000 mcg... (Review)
Review
Women Taking a Folic Acid Supplement in Countries with Mandatory Food Fortification Programs May Be Exceeding the Upper Tolerable Limit of Folic Acid: A Systematic Review.
BACKGROUND
In preconception and pregnancy, women are encouraged to take folic acid-based supplements over and above food intake. The upper tolerable limit of folic acid is 1000 mcg per day; however, this level was determined to avoid masking a vitamin B12 deficiency and not based on folic acid bioavailability and metabolism. This review's aim is to assess the total all-source intake of folate in women of childbearing age and in pregnancy in high-income countries with folate food fortification programs.
METHODS
A systematic search was conducted in five databases to find studies published since 1998 that reported folate and folic acid intake in countries with a mandatory fortification policy.
RESULTS
Women of childbearing age do not receive sufficient folate intake from food sources alone even when consuming fortified food products; however, almost all women taking a folic acid-based supplement exceed the upper tolerable limit of folic acid intake.
CONCLUSIONS
Folic acid supplement recommendations and the upper tolerable limit of 1000 mcg set by policy makers warrant careful review in light of potential adverse effects of exceeding the upper tolerable limit on folic acid absorption and metabolism, and subsequent impacts on women's health during their childbearing years.
Topics: Dietary Supplements; Female; Folic Acid; Folic Acid Deficiency; Food, Fortified; Humans; Neural Tube Defects; Pregnancy; Women's Health
PubMed: 35807899
DOI: 10.3390/nu14132715 -
Child's Nervous System : ChNS :... Oct 2022Split cord malformation (SCM) presenting concomitant with spinal teratoma without any open spinal dysraphism has rarely been reported in the literature. We aimed to make...
PURPOSE
Split cord malformation (SCM) presenting concomitant with spinal teratoma without any open spinal dysraphism has rarely been reported in the literature. We aimed to make a systematic review and qualitative analysis of the literature about the topic and present the first case of SCM concomitant with spinal teratoma harboring papillary thyroid carcinoma (PTC) component.
METHODS
Two big search tools (Pubmed/MEDLINE) and Scopus were used. The search strategy was compatible to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). An exemplary case of ours was also presented.
RESULTS
There were 30 patients (15 pediatric and 15 adult). Female and male distribution was even. Median age of the patients was 18 years (range = 0-66 years). The most common presenting symptoms were back pain and lower limb weakness. Spinal teratoma and SCM mostly presented at thoracic/thoracolumbar region in children and lumbar region in adults. Surgical outcome was better in the children compared to the adults.
CONCLUSION
Thoracolumbar region is the most common location for such entity in children, whereas lumbar region for the adults. Surgical resection should be done as much as possible under neuromonitorization. The resected material should be evaluated thoroughly not to miss any malign pathology. Surgical outcome is better when it is done at an early age.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Middle Aged; Muscle Weakness; Neural Tube Defects; Spinal Cord; Spinal Dysraphism; Spine; Teratoma; Young Adult
PubMed: 35687168
DOI: 10.1007/s00381-022-05578-5 -
BMC Neurology Jun 2022Neural tube defects (NTDs) are associated with high rates of neonatal mortality and morbidity worldwide. The promotion of folic acid fortification and supplementation... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Neural tube defects (NTDs) are associated with high rates of neonatal mortality and morbidity worldwide. The promotion of folic acid fortification and supplementation in pregnant women by the Food and Drug Administration significantly decreased the incidence of NTDs in the United States. This practice is not widely adopted in Eastern Africa countries. We hypothesized that these countries experience a higher burden of NTDs than countries that promote the use of folic acid. We aimed to estimate the birth prevalence of NTDs in the United Nations (UN) Eastern African region.
METHODS
PubMed (Medline), Embase, and Cochrane Library databases were systematically searched from inception to December 17, 2021. We included randomized controlled trials or observational studies that reported the prevalence estimates of NTDs in Eastern Africa. Random effects model was used to pool the effect estimates. The GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach was used to assess the certainty of the evidence. Outcome measures were overall and specific (spina bifida, anencephaly, encephalocele) rates of NTDs per 10,000 births, including live and stillborn cases.
RESULTS
The meta-analysis included 20 studies consisting of 752,936 individuals. The pooled prevalence of all NTDs per 10,000 births in Eastern Africa was 33.30 (95% CI: 21.58 to 51.34). Between-study heterogeneity was high (I = 97%, p < 0.0001), The rate was highest in Ethiopia (60 per 10,000). Birth prevalence of spina bifida (20 per 10,000) was higher than anencephaly (9 per 10,000) and encephalocele (2.33 per 10,000). No studies on NTDs were identified in 70% of the UN Eastern Africa region. Birth prevalence increased by 4% per year from 1983 to 2018. The level of evidence as qualified with GRADE was moderate.
CONCLUSION
The birth prevalence of NTDs in the United Nations region of Eastern Africa is 5 times as high as observed in Western countries with mandatory folic acid supplementation in place. Therefore, mandatory folic acid supplementation of stable foods may decrease the risk of NTDs in Eastern Africa.
Topics: Anencephaly; Encephalocele; Female; Folic Acid; Humans; Infant, Newborn; Neural Tube Defects; Pregnancy; Prevalence; Spinal Dysraphism; United States
PubMed: 35650541
DOI: 10.1186/s12883-022-02697-z -
Seminars in Reproductive Medicine Jul 2022Preconception care (PCC) involves a wide-ranging set of interventions to optimize health prior to pregnancy. These interventions seek to enhance conception rates,...
Preconception care (PCC) involves a wide-ranging set of interventions to optimize health prior to pregnancy. These interventions seek to enhance conception rates, pregnancy outcomes, childhood health, and the health of future generations. To assist health care providers to exercise high-quality clinical care in this domain, clinical practice guidelines from a range of settings have been published. This systematic review sought to identify existing freely accessible international guidelines, assess these in terms of their quality using the AGREE II tool, and assess the summary recommendations and the evidence level on which they are based. We identified 11 guidelines that focused on PCC. Ten of these were classified as moderate quality (scores ranging from 3.5 to 4.5 out of 7) and only one was classified as very high quality, scoring 6.5. The levels of evidence for recommendations ranged from the lowest possible level of evidence (III) to the highest (I-a): the highest quality evidence available is for folic acid supplementation to reduce risk of neural tube defects and the role of antiviral medication to prevent HIV transmission. This systematic review identified that high-quality guidelines on PCC are lacking and that few domains of PCC recommendations are supported by high-quality evidence.
Topics: Child; Exercise; Female; Fertilization; Health Personnel; Humans; Preconception Care; Pregnancy; Quality of Health Care
PubMed: 35576970
DOI: 10.1055/s-0042-1748190