Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a 44-year-old woman with progressive headache, ataxia, and seizures in association with multifocal cerebral and cerebellar leukoencephalopathy, intracranial calcifications, and cysts. The cause of death was intracerebellar hemorrhage while taking warfarin. Pathologic features on biopsy included angiomatous-like blood vessels, intense gliosis, and Rosenthal fiber formation in the white matter. Genetic analyses did not identify any significant mutations in two candidate genes.
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PMID:An adult case of leukoencephalopathy with intracranial calcifications and cysts. 1713 Apr 35

The case of a 72-year-old demented woman having episodes of strokes without any risk factors for cardiovascular disease is reported. Her elder brother and sister have also had stroke episodes since their middle age. She experienced hallucinations, delusions, and recurrent headaches since the age of 55. She has gradually developed gait disturbance and cognitive impairment. Brain MRI revealed extensive leukoaraiosis and multiple lacunar infarcts in the deep white matter and brainstem. Repeated MRI incidentally disclosed fresh hemorrhage in the dorsal subcortical temporal lobe, which appeared to be asymptomatic. Anti-platelet agents were not used during disease progression. We detected G975C mutation of the Notch3 gene and diagnosed our patient's disease as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). This report suggests that arteriopathy of CADASIL could cause a hemorrhagic process, indicating that, in such a case, routine administration of anti-platelet agent to prevent recurrent ischemic stroke is not recommended.
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PMID:[Case of CADASIL showing spontaneous subcortical hemorrhage with a novel mutation of Notch3 gene]. 1726 Aug 7

Neurologic complications (NCs) are a significant cause of morbidity and mortality in patients who undergo liver transplantation (LT). The aim of this study was to evaluate the incidence and type of NCs and associated factors in pediatric LT patients. We retrospectively reviewed NCs in the medical records of 40 consecutive infants, children, and adolescents who underwent LT at our institution. The subjects consisted of 23 boys and 17 girls (median age, 8.5 +/- 0.85 yr; range, 11 months to 17 yr). The indications for LT were Wilson's disease in 10 patients, fulminant hepatic failure (FHF) in nine, and other types of chronic liver disease in 21. NCs were found in 14 patients (35%). Those 14 individuals experienced a total of 16 episodes of NCs (two separate episodes in two of the patients). The most common NCs were seizure (seven episodes in six patients) and posterior leukoencephalopathy syndrome (PLES; five episodes in four patients). Seizure was the presenting symptom in three episodes of PLES. Two episodes of diffuse encephalopathy were observed in two patients, and two episodes of psychiatric symptoms occurred in two patients. We also noted one episode of tremor in one patient, one episode of acute dystonic reaction in one patient, and one episode of headache in one patient. Patients with Wilson's disease had a higher incidence of NCs (60%) than did patients without Wilson's disease (26.7%); however, this difference was not significant. The incidence of NCs was 44% in patients with FHF and 35% in those without FHF. That difference also was not significant. Immunosuppressive agents were the primary cause of 13 of the 16 episodes of NC. Uremia with hypertension, hypoxia, and hypomagnesemia caused one neurologic episode each. NCs, which are frequent in the first 30 days after pediatric LT, did not affect survival in this group. NCs were reversed by the discontinuation or reduction of immunosuppressive agents in 12 episodes, correction of hypomagnesemia and the reduction of immunosuppressive agents in one episode, and the correction of uremia and hypertension in one episode. Refractory epilepsy developed in one patient, and death unrelated to NCs occurred in one. The mortality rate was 7.1% (n = 1) in patients with NCs and 15.4% (n = 4) in those without NCs (p = 0.64). NCs are an important complication after LT. It is essential that each transplantation team collaborate with pediatric neurologists to ensure the rapid and accurate diagnosis of NCs in infants, children, and adolescents after LT and to prevent the delay of appropriate treatment.
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PMID:Neurological complications of liver transplantation in pediatric patients: a single center experience. 1730 Apr 94

Posterior leukoencephalopathy syndrome is a recently identified clinical and radiologic entity. The characteristic radiologic findings are bilateral gray and white matter edema in the posterior regions of the cerebral hemispheres. This article reports clinical and radiologic findings in 10 consecutive episodes of posterior leukoencephalopathy syndrome that were diagnosed in 9 children and adolescents. The causes were immunosuppressive therapy in 7 patients and a combination of renal failure and hypertension in 3. The most common presenting symptoms were seizure and altered consciousness; others included headache, sixth nerve palsy, and cortical blindness. Imaging demonstrated abnormalities in the parietal and occipital lobes in all 10 episodes. The signs and symptoms resolved after immunosuppressive agents were reduced or discontinued, or after uremia and hypertension were corrected. Four patients underwent follow-up cranial imaging, and the images showed nearly complete or complete resolution. The syndrome was clinically reversible in all patients.
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PMID:Posterior leukoencephalopathy syndrome in children and adolescents. 1762 19

Posterior reversible leukoencephalopathy syndrome (PRES) clinically presents with seizures, severe headaches, and mental and visual changes. Our goal was to describe the clinical features, triggering factors, neuro-imaging findings, and electroencephalogram (EEG) findings in a pediatric cohort with renal disease. We retrospectively analyzed the records of 18 children with the diagnosis of PRES between January 2001 and June 2006 at the University of Miami/Holtz Children's Hospital, USA. There were 22 PRES episodes. The most common clinical presentation was generalized tonic-clonic seizures in 59% (13/22). The most common identified trigger of PRES was hypertensive crisis in 59% (13/22). Almost half of the children had no evidence of on-going uncontrolled hypertension; 44% (8/18) had normal funduscopic examination findings, and 50% (9/18) had no or mild left ventricular hypertrophy. Two of the 18 patients had recurrent PRES episodes, three episodes each. Diffuse slowing was the most common finding on the EEGs. Atypical magnetic resonance imaging (MRI) findings were more prevalent in the imaged cases (62% vs 25%, P < 0.05). All the computerized tomography (CT) scans were normal, despite the positive MRI findings in four cases when both types of imaging was used. All the episodes had total clinical resolution. In conclusion, despite the diverse initial trigger, acute hypertension seems to be the common pathogenic pathway for pediatric PRES. MRI seems superior to CT, with better sensitivity due to its high resolution and diffusion-weighted imaging. The lesions do not necessarily have to be in the posterior white matter and may not be totally reversible.
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PMID:Posterior reversible encephalopathy syndrome in the pediatric renal population. 1769 37

Posterior reversible encepalopathy syndrome (PRES), or reversible posterior leukoencephalopathy, is a neurologic condition characterized by recognizable pattern of altered mental status, headache, visual changes and seizures in association with findings indicating a predominantly posterior leucoencephalopathy on imaging studies. It has rarely been described in children. We report two cases of pediatric systemic lupus erythematosus (SLE) complicated by PRES and review the literature.
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PMID:Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus. 1771 91

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a 'string and beads' appearance on cerebral arteries, which resolves spontaneously in 1-3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 +/- 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.
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PMID:The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. 2146 33

We report an adult end-stage renal disease patient with only cerebellum involvement of reversible posterior leukoencephalopathy syndrome (RPLS). A 37-year-old woman, diagnosed as chronic glomerulonephritis, was admitted to our hospital with progressive visual disturbance and severe headache. MRI revealed hyperintense signal intensity changes restricted in the cortex and subcortical white matter of the cerebellum. With appropriate control of blood pressure and intracranial pressure, her symptoms were improved and complete resolution of previous hyperinternse lesion was shown on MRI after the 19th day of admission. This is first case of isolated cerebellar involvement of RPLS without other cerebral involvement in an adult.
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PMID:Isolated cerebellar reversible leukoencephalopathy syndrome in a patient with end stage renal disease. 1817 4

A 32-year-old woman developed a headache, seizures, and stupor on postpartum day 8. An initial diagnosis of possible encephalitis was made considering the presence of fever, neck stiffness, and abnormal CSF findings. MRI demonstrated hyperintense signals consistent with bilateral borderzone areas. MRA showed severe proximal narrowing of anterior, middle, and posterior cerebral arteries bilaterally. The patient recovered completely over 2 weeks, and repeated MRI and MRA scans were normal. Reversible vasoconstrictions have been known to occur during puerperium, and the clinical symptoms of our patient resembled such cases. Prior reports attributed these cases of postpartum angiopathy to capillary leakage and edema resulting in leukoencephalopathy. Our case suggests reversible borderzone ischemia as an additional pathological process.
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PMID:Postpartum angiopathy associated with reversible borderzone ischemia. 1827 36

The article describes the case history of posterior leukoencephalopathy syndrome - a brain disorder that predominantly affects the cerebral white matter. Edematous lesions involve the posterior parietal and occipital lobes, and may spread to basal ganglia, brain stem and cerebellum. This rapidly evolving neurological condition is clinically characterized by headache, nausea and vomiting, seizures, visual disturbances and altered sensorial functions, and occasionally focal neurological deficit. Posterior leukoencephalopathy syndrome is often associated with an abrupt increase in blood pressure and is usually seen in patients with eclampsia, renal disease and hypertensive encephalopathy. It is also seen in the patients treated with cytotoxic and immunosuppressive drugs such as cyclosporine A, tacrolimus /FK-506, cisplatin, cytarabine, IVIg, erytrophoietin, and interferon alpha. The study demonstrated that lesions of posterior leukoencephalopathy syndrome are best visualized with magnetic resonance [MR] imaging. T2 weighted MR Images, at the height of symptoms, characteristically show diffuse hyper intensity selectively involving the parieto - occipital white matter. Occasionally the lesions also involve the grey matter. Computed tomography can also be used satisfactorily to detect hypodense lesions of posterior leukoencephalopathy. Early recognition of this condition is of paramount importance because prompt control of blood pressure, withdrawal of immunosuppressive agents will cause reversal of the syndrome. Delay in the diagnosis and treatment can result in permanent damage to affected brain tissues. The clinical data and radiological findings depicted in the study add to the investigation of the disorder.
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PMID:[Clinical report of posterior leukoencephalopathy syndrome]. 1832 87


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