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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuroimaging and management advances require review of indications for excluding cerebral venous sinus (sinovenous) thrombosis (CSVT) in children. Our goals were to examine (i) clinical presentations of CSVT, (ii) prothrombotic risk factors and other predisposing events, (iii) clinical and radiological features of brain lesions in CSVT compared with arterial stroke, and (iv) predictors of outcome. We studied 42 children with CSVT from five European paediatric neurology stroke registries. Patients aged from 3 weeks to 13 (median 5.75) years (27 boys; 64%) presented with lethargy, anorexia, headache, vomiting, seizures, focal signs or coma and with CSVT on neuroimaging. Seventeen had prior chronic conditions; of the 25 previously well patients, 23 had recent infections, eight became dehydrated and six had both. Two children had a history compatible with prior CSVT. Anaemia and/or microcytosis (21 probable iron deficiency, five haemolytic, including two with sickle cell disease and one with beta-thalassaemia) was as common (62%) as prothrombotic disorder (13/21 screened). High factor VIII and homozygosity for the thermolabile methylene tetrahydrofolate reductase polymorphism were the commonest prothrombotic disorders. The superficial venous system was involved in 32 patients, the deep in six, and both in four. Data on the 13 children with bland infarction and the 12 with haemorrhage in the context of CSVT were compared with those from 88 children with ischaemic (AIS) and 24 with haemorrhagic (AHS) arterial stroke. In multiple logistic regression, iron deficiency, parietal infarction and lack of caudate involvement independently predicted CSVT rather than arterial disease. Five patients died, three acutely, one after recurrence and one after 6 months being quadriparetic and blind. Follow-up ranged from 0.5 to 10 (median 1) years. Twenty-six patients (62%) had sequelae: pseudotumour cerebri in 12 and cognitive and/or behavioural disabilities in 14, associated with epilepsy in three, hemiparesis in two and visual problems in two. Eighteen patients, including six with haemorrhage, were anticoagulated. Older age [odds ratio (OR) 1.54, 95% confidence limits (CI) 1.12, 2.13, P = 0.008], lack of parenchymal abnormality (OR 0.17, 95% CI 0.02, 1.56, P = 0.1), anticoagulation (OR 24.2, 95% CI 1.96, 299) and lateral and/or sigmoid sinus involvement (OR 16.2, 95% CI 1.62, 161, P = 0.02) were independent predictors of good cognitive outcome, although the last predicted pseudotumour cerebri. Death was associated with coma at presentation. Of 19 patients with follow-up magnetic resonance (MR) venography, three had persistent occlusion, associated with anaemia and longer prodrome. A low threshold for CT or MR venography in children with acute neurological symptoms is essential. Nutritional deficiencies may be modifiable risk factors. A paediatric anticoagulation trial may be required, after the natural history has been further established from registries of cases with and without treatment.
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PMID:Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. 1569 61

The purpose of this study was to examine the relationship between pain symptoms, daily functioning, health-related quality of life (HRQOL), and subjectively reported sleep disturbances in adolescents with chronic pain. Depressive symptoms were tested as a general risk factor for increased sleep problems. During routine subspecialty clinic visits, 86 adolescents (mean age, 14.75 years; 67% female) diagnosed with chronic headaches, juvenile idiopathic arthritis, or sickle cell disease completed measures to assess pain, sleep disturbances, functional disability, depression, and HRQOL. Across pain diagnoses, adolescents experienced similar sleep patterns and sleep behaviors with the exception of daytime sleepiness, which was higher in adolescents with headache compared to adolescents with sickle cell disease. Bivariate correlations showed low relationships between pain and sleep and moderate to high relationships between depressive symptoms, daily functioning, HRQOL, and sleep. In multivariate analyses, as hypothesized, depressive symptoms were predictive of the severity of sleep disturbances after controlling for the effect of all other demographic, pain, and functional impact variables. Results suggest that a relationship between the experience of recurrent and chronic pain and sleep disturbances exists for adolescents, and these sleep disturbances are linked to mood disturbances and reductions in daily functioning and quality of life. Sleep disturbances have been described in adult patients with chronic pain, but little is known about sleep in adolescents with chronic pain. This study examined the complex interrelationship between sleep, pain, mood, functioning, and HRQOL. Findings suggest that mood is strongly related to sleep and might share common pathophysiologic or behavioral origins in adolescents with chronic pain.
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PMID:Subjective sleep disturbances in adolescents with chronic pain: relationship to daily functioning and quality of life. 1577 14

Although the prevalence of seizures in children with sickle cell disease (SCD) is 10 times that of the general population, there are few prospectively collected data on mechanism. With transcranial Doppler and magnetic resonance imaging (MRI) and angiography, we evaluated 76 patients with sickle cell disease, 29 asymptomatic and 47 with neurological complications (seizures, stroke, transient ischemic attack, learning difficulty, headaches, or abnormal transcranial Doppler), who also underwent bolus-tracking perfusion MRI. The six patients with recent seizures also had electroencephalography. Group comparisons (seizure, nonseizure, and asymptomatic) indicated that abnormal transcranial Doppler was more common in the seizure (4/6; 67%) and nonseizure (26/41; 63%) groups than in the asymptomatic (10/29; 34%) group (chi2; p = 0.045), but abnormal structural MRI (chi2; p = 0.7) or magnetic resonance angiography (chi2; p = 0.2) were not. Relative decreased cerebral perfusion was found in all seizure patients and in 16 of 32 of the remaining patients with successful perfusion MRI (p = 0.03). In the seizure patients, the perfusion abnormalities in five were ipsilateral to electroencephalographic abnormalities; one had normal electroencephalogram results. These findings suggest that vasculopathy and focal hypoperfusion may be factors in the development of sickle cell disease-associated seizures.
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PMID:Sickle cell disease: ischemia and seizures. 1631 82

The purposes of this study were to determine the characteristics of headaches in children with sickle cell disease (SCD) and to assess the relationship between headache symptoms and children's physical and emotional status. A detailed headache questionnaire using International Classification of Headache Disorders (ICHD-2) criteria was mailed to a cohort (n = 50) of children with SCD, ages 9 to 17 years. Respondents also completed measures of functional disability and psychological distress. Headaches had occurred over the previous 3-month period in 76.2% of the patients. Frequent headaches were common, occurring greater than once a week in 31.2% of children. Average pain severity was reported as moderate on a 0-to-10 scale (mean = 5.8). Duration of headaches ranged from 30 minutes to several days, with a mean of 5 hours. Based on ICHD-2 criteria, 43.8% of children had headache symptoms consistent with migraines, 6.2% with migraine with aura, and 50.0% with tension-type headaches. Children with symptoms of migraine had significantly greater functional disability compared with children with symptoms of tension-type headaches (P < 0.01). Further studies to determine the characteristics and determinants of headaches experienced in SCD patients will help maximize treatment of headaches and enhance daily functioning in these patients.
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PMID:Headache symptoms in pediatric sickle cell patients. 1609 23

Headache occurs in sickle cell disease (SCD), but its characteristics and frequency have not previously been studied. Our aim was to study patterns of headache in adults with SCD and to correlate its presence with blood flow velocities measured by transcranial Doppler (TCD) and with brain magnetic resonance imaging (MRI) abnormalities. We studied 56 adults with SCD. Twenty-eight patients (50%) had severe and frequent headaches. In 20 patients (35.7%) the headache met the International Headache Society criteria for migraine without aura. Patients with frequent and severe headache presented TCD velocities significantly higher than those without headache, or with milder headache. No correlation was found between headache and abnormalities in brain MRI. A migraine-mimicking headache occurs in SCD but we should not understand it as a primary headache because the blood flow abnormalities secondary to SCD detected by TCD seem to play an important role in these patients.
Cephalalgia 2006 Jun
PMID:Migraine-mimicking headache and sickle cell disease: a transcranial Doppler study. 1668 6

Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.
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PMID:Skull bone infarctive crisis and deep vein thrombosis in homozygous sickle cell disease- case report and review of the literature. 1745

Children with sickle cell disease, a chronic hemolytic anemia, present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, transient ischemic attacks, 'soft neurological signs', seizures, headache, coma, visual loss, altered mental status, cognitive difficulties, and covert or 'silent' infarction. Those with ischemic stroke usually have stenosis or occlusion of the distal internal carotid and proximal middle cerebral arteries. Indefinite transfusion prevents recurrence in most patients who have had a stroke, and can prevent first stroke in those with high transcranial Doppler velocities. High white cell count, low hemoglobin and oxyhemoglobin desaturation predict neurological complications. Other risk factors for overt ischemic stroke include hypertension, previous transient ischemic attack, covert infarction and chest crisis. For hemorrhagic stroke, aneurysms are common in adults but not children, who often present with hypertension after transfusion or corticosteroids. Seizures are particularly common in patients with cerebrovascular disease and covert infarction; the latter is also associated with hyposplenism and infrequent pain. Factors associated with cognitive difficulties include thrombocytosis, infarction, large-vessel disease, and perfusion abnormality on neuroimaging. As well as investigating the role of genes and the possibility that hydroxyurea or blood pressure control reduce neurological complications, we should explore the modifiable effects of poor nutrition, chronic infection, hemolysis and oxyhemoglobin desaturation on stroke risk.
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PMID:Therapy insight: stroke risk and its management in patients with sickle cell disease. 1747 74

Several hereditary disorders induce angiopathy in the intracranial cerebrovasculature and thus cause ischemic strokes. MELAS is a maternally inherited mitochondrial disorder that produces stroke-like events. Sickle cell disease, which is the result of a single base pair substitution, is a major cause of strokes in children. Homocystinuria, an autosomal recessive syndrome, produces premature atherosclerosis. Hereditary cerebroretinal vasculopathy is an autosomal dominant disorder that causes retinal and brain infarctions. Fabry disease is an x-linked disorder that can cause stroke in adults. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy is an autosomal dominant syndrome that is associated with ischemic stroke and migraine-like headaches. The clinical presentation, stroke pathophysiology, and gene defects associated with these heritable disorders are reviewed.
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PMID:Mendelian and mitochondrial disorders associated with stroke. 1790 83

Erythema infectiosum (fifth disease) is the most common clinical presentation of acute parvovirus B19 infection in infancy. In healthy adults, most cases of infection are asymptomatic or accompanied by a flu-like syndrome like headaches and myalgia. Haematological manifestations are dominated by transient aplasia of erythroid progenitor cells which remains asymptomatic in most of non immunocompromised patients. Patients with sickle cell disease, thalassemia or other disorders associated with shortened red blood cell survival are at particular risk for marked anemia or red blood cell aplasia. In immunosuppressed patients, anemia may be chronic because of persistent viral load. Neutropenia, lymphopenia or thrombocytopenia have also been reported in acute parvovirus B19 infection. Mechanisms of these cytopenias are not yet elucidated. We present two patients with thrombopenia and/or neutropenia but without anemia due to acute parvovirus B19 infection.
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PMID:[Neutropenia and/or thrombocytopenia due to acute parvovirus B19 infection]. 1941 Dec 38

Treatment of pain in the setting of sickle cell disease remains unsatisfactory. The approach remains to treat the pain symptomatically with escalating doses of non-opioid and opioid medications while any underlying inciting process is investigated. For the majority of patients with sickle cell disease, pain will always be part of their lives. Advances in the treatment of sickle cell pain will depend on multiple approaches, including both pharmacologic and nonpharmacologic.
Curr Pain Headache Rep 2009 Aug
PMID:The management of sickle cell pain. 1958 93


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