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

Sturge-Weber syndrome is associated with leptomeningeal angioma, trigeminal port-wine stain, epilepsy, and glaucoma. Clinically, many patients with Sturge-Weber syndrome are observed to have both seizures and headaches, but this has never been described in the literature. A questionnaire was mailed to 190 patients with reported comorbid epilepsy and headache as identified by the Sturge-Weber Foundation. Sixty-eight surveys were returned anonymously; 55 reported both seizures and headaches. The median age at headache onset was 8 years, with a median of three headaches per month. Fifty-eight percent felt that headaches were an equal or greater problem. Ibuprofen and acetaminophen were the most frequently tried abortive medications; 22% had tried sumatriptan. Only 22% reported a neurologist suggesting the use of an anticonvulsant as a preventive agent. Subjects with a family history of headaches had an earlier age at headache onset (7.5 vs 11 years; P = .02), and those with a family history of seizures were more likely to report behavior problems (69% vs 33%; P = .02). Subjects reporting learning problems or hemiparesis had an earlier age at seizure onset. Migraine-like headaches can be as significant a problem as epilepsy for patients with Sturge-Weber syndrome. Despite this, triptans and prophylactic medications (including anticonvulsants) were used in less than half of the patients. Correlations of family history with both age at symptom onset and behavior problems suggest that genetic substrate could be one factor determining the variable neurologic manifestations seen in Sturge-Weber syndrome.
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PMID:Comorbidity of epilepsy and headache in patients with Sturge-Weber syndrome. 1622 15

Neurocutaneous syndromes are heterogeneous group of disorders with abnormalities of central as well as peripheral nervous system. Neurofibromatosis type II (NF-II) is an autosomal dominant neurocutaneous syndrome rarely diagnosed in pediatric population. Diagnosis is based on clinical history and radioimaging. We present a 14 years old boy with headache and decreased hearing, who turned to be a case of neurofibromatosis type II.
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PMID:Neurofibromatosis type II: a rare neurocutaneous syndrome. 1762 95

Sturge-Weber syndrome is a rare, congenital, neurocutaneous disorder. It can be associated with a variety of symptoms including severe epilepsy. Patients often become symptomatic during childhood and the severity of the epilepsy correlates with the patient's neurological outcome. The patient reported here remained asymptomatic until age 24, when he started to experience migraine accompanied by visual scotoma and hemiparesis. Ten years later, he developed severe, refractory epilepsy, with prolonged postictal neurological deficits and ictal as well as post-ictal headaches. It became increasingly difficult to distinguish between migraines and seizures as both could last for several hours, as demonstrated in the two video EEGs. Both the epilepsy and the migraine may therefore have contributed to the patient's severe, neurological deterioration, probably by accelerating the progressive neuronal damage. Surgery improved the situation, but lesional areas were too extensive for complete removal. Late-onset symptoms in Sturge-Weber syndrome may thus result in a severe course for the disease. Early intervention and surgical treatment options are discussed.
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PMID:Late-onset epilepsy in a surgically-treated Sturge-Weber patient. 1901 74

Tuberous sclerosis complex is an autosomal dominant disorder affecting primarily the central nervous system, skin, and kidney caused by mutations in the TSC1 and TSC2 genes. Diagnosis is established with the identification of various neurocutaneous symptoms and multiple organ system hamartomas. The authors report on a 9-year-old patient with episodes of vertigo and headache followed by full spontaneous recovery. There was no history of seizures, mental retardation, or other neurologic sequelae, and psychomotor development was normal. Magnetic resonance imaging revealed pial angiomatosis of the left cerebellum and calcifications in the left parieto-occipital lobe consistent with the diagnosis of Sturge-Weber syndrome. At the age of 13, multiple renal angiomyolipomas and a single hypomelanotic macule were found, and subsequent imaging revealed several cortical tuberi. The diagnosis was confirmed by sequence analysis, which identified a novel missense mutation p.Ala460Thr in exon 13 of the TSC2 gene. Thus, mild tuberous sclerosis due to a hypomorphic mutation in TSC2 may present with isolated leptomeningeal angiomatosis.
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PMID:Unusual radiological presentation of tuberous sclerosis complex with leptomeningeal angiomatosis associated with a hypomorphic mutation in the TSC2 gene. 1925 84

Eszopiclone is the S-isomer of racemic zopiclone, a cyclopyrrolone with sedative-hypnotic activity that has been available in Europe, Canada, and Latin America since 1987. Eszopiclone acts by binding to the GABA(A) receptor. In contrast to the benzodiazepine (BZD) hypnotics, eszopiclone has more selectivity for certain subunits of the GABA(A) receptor. Oral eszopiclone is rapidly absorbed and extensively distributed to body tissues including the brain. Peak plasma concentrations are attained 1.0-1.6 hours after a 3 mg dose, while the mean elimination half-life is 6 hours. The half-life increases with age to about 9.0 hours in patients 65 years or older. Eszopiclone's pharmacokinetic (PK) profile is not substantially modified in patients suffering from renal failure or mild-to-moderate hepatic impairment, although patients with severe hepatic insufficiency should have a reduced dose. The subjective perception of improved sleep following eszopiclone 2 or 3 mg treatment has been demonstrated in randomized, double-blind, placebo-controlled studies of up to 6 months' duration. In these studies the drug significantly reduced sleep onset latency (SOL), the number of awakenings, and wake time after sleep onset (WASO) whereas total sleep time (TST) and quality of sleep were increased in non-elderly and elderly subjects. Sleep laboratory studies of the effects of eszopiclone have confirmed the drug's clinical efficacy in subjects with chronic primary insomnia. Eszopiclone, unlike BZD hypnotics, does not significantly alter values corresponding to slow wave sleep (SWS or stages 3 and 4) and rapid eye movement (REM) sleep. Rebound insomnia following withdrawal of eszopiclone has been examined in only one study. Discontinuation of the active treatment with 2 mg was followed by rebound insomnia in non-elderly subjects. Three-mg doses of eszopiclone administered for a period of up to 12 months was associated with a sustained beneficial effect on sleep induction and maintenance, with no occurrence of tolerance. The most common side-effects were unpleasant or bitter taste, headache, dyspepsia, pain, diarrhea, dry mouth, upper respiratory infection, urinary tract infection, dizziness, and accidental injury. New adverse events (withdrawal symptoms) including anxiety, abnormal dreams, hyperesthesia, nausea, and upset stomach were recorded in one study on the days following eszopiclone 2 or 3 mg discontinuation. Although dependence and abuse potential have not been formally assessed, unpublished data show that eszopiclone at doses of 6 and 12 mg produces euphoria effects similar to those of diazepam 20 mg in BZD drug addicts. In conclusion, available evidence tends to indicate that eszopiclone is effective and safe for the treatment of chronic primary insomnia in non-elderly and elderly subjects. Tolerance did not occur during active drug administration for a 12-month period. Thus eszopiclone can be efficacious not only during short- and intermediate-term administration but also in patients requiring prolonged regular drug usage.
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PMID:Eszopiclone: its use in the treatment of insomnia. 1930 May 73

A young woman, known to have Sturge-Weber syndrome, was admitted with headache and left hemiparesis. Neuroimaging showed chronic occlusion of the venous sinuses without evidence of acute thrombus formation, or a recent vascular event.
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PMID:Acute hemiparesis in Sturge-Weber syndrome. 1944 61

We report a patient with Sturge-Weber Syndrome (SWS) who developed migraine-like headaches followed by cerebral infarction. SWS without facial nevus was diagnosed based on calcification detected by CT and pial angioma detected by enhanced MRI. His migraine-like headaches were preceded by left homonymous hemianopsia, which persisted for more than 60 min. Although homonymous hemianopsia disappeared with cessation of the headache until 13 years of age, from age 14 years onward, this homonymous hemianopsia persisted after the headaches ended. Moreover, reduced cerebral blood flow was seen in the right occipital area on SPECT. At first, his left homonymous hemianopsia persisted for several months after the headache disappeared, but it had recovered completely. However, the durations of episodes of left homonymous hemianopsia, which persisted after headache disappearance, gradually became longer. At last one year after his first admission, the visual defect had become permanent. SWS is well known to be associated with migraine attacks and hemianopsia. However, the course of our present patient, i.e. recurrent homonymous hemianopsia, associated with migraine-like headaches becoming permanent, is rare. The pathophysiological mechanism underlying this clinical course is uncertain. The efficacy of valproate and propranolol as preventive therapy has been inadequate, to date.
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PMID:Prolonged left homonymous hemianopsia associated with migraine-like attacks in a child with Sturge-Weber syndrome. 1980 Jul 49

Headache is relatively common in patients with cerebrovascular disorders. The reported frequency of stroke-related headache ranges from 7% to 65% and different types of headache, such as onset headache, sentinel headache, or delayed headache, may be observed in association with stroke. Headache can be attributed to ischemic stroke, transient ischemic attack, or non-traumatic intracranial hemorrhage, including intracerebral and subarachnoid hemorrhage. Headache at stroke onset is more common in subarachnoid hemorrhage, most prominently associated with severe headache, and in intracerebral hemorrhage than in ischemic stroke or transient ischemic attack. The typical presentation of subarachnoid hemorrhage includes the sudden onset of severe headache with nausea, vomiting, neck pain, photophobia, and loss of consciousness. Headache is the only symptom in about a third of patients with subarachnoid hemorrhage. The suddenness of onset and not its severity is the characteristic feature of the headache in subarachnoid hemorrhage. Referring to unruptured vascular malformations, the headache can be attributed to saccular aneurysm, arteriovenous malformation, dural arteriovenous fistula, dural cavernous angioma, and encephalotrigeminal or leptomeningeal angiomatosis (Sturge-Weber syndrome). It is very important to recognize that in the latter forms the onset of headache may indicate an upcoming bleeding complication.
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PMID:Headache attributed to stroke, TIA, intracerebral haemorrhage, or vascular malformation. 2081 53

Sturge-Weber syndrome is a rare congenital disorder. Seizures, stroke-like episodes, glaucoma, headache, and developmental delay are frequently associated features. An Internet-based questionnaire was designed to assess the frequency of use, effectiveness, and safety of aspirin treatment in Sturge-Weber syndrome. Thirty-four of 98 subjects who completed the survey reported having used aspirin. The mean number of reported stroke-like episodes was reduced from 1.1 to 0.3 per month in the year after starting aspirin (n = 26, p = .014). The median number of seizures was significantly reduced from 3 to 1 episodes per month (n = 21, p = .002). Thirty-nine percent of subjects reported a history of complications (predominantly increased bruising or gum/nose bleeding) while on aspirin; however, none reported discontinuing aspirin because of side effects. Our study showed a significant relative reduction in both self-reported seizure frequency and stroke-like episodes after starting aspirin. It also suggests that low-dose aspirin can be safely used in these patients.
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PMID:Survey of aspirin use in Sturge-Weber syndrome. 2142 42

A 24-year-old man with a history of Klippel-Trenaunay-Weber syndrome presented with severe headache and neck pain. Work-up revealed subarachnoid hemorrhage and evidence of multiple intracranial aneurysms. The patient was treated with open surgical clipping of his ruptured aneurysm and is currently doing well.
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PMID:Open surgical management of a ruptured intracranial aneurysm in Klippel-Trenaunay-Weber (KTW) syndrome. 2331 73


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