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)

Malignant pheochromocytoma is a rare disease with a high mortality. Surgical removal is usually curative while chemotherapy and radiotherapy are palliative treatments. A case of metastatic malignant pheochromocytoma of the right adrenal gland is presented who had fluctuating blood pressure with episodic headache and raised urinary VMA levels. Thoraco-abdominal resection of the tumour resulted in control of blood pressure and patient was asymptomatic at 4 months follow-up.
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PMID:Metastatic malignant pheochromocytoma of adrenal gland. 1854 Oct 88

Superficial siderosis of the central nervous system is a very rare disease related to hemosiderin deposits in the brain, brainstem, cerebellum and spinal cord due to chronic subarachnoid hemorrhage. Chronic increased intracranial pressure develops in about one-third of affected cases. We report a patient with superficial siderosis and sudden intracranial pressure crisis. A 29-year-old man experienced a subacute episode of headache, tinnitus and blurred vision. Magnetic resonance imaging of the brain revealed hemosiderin deposits characteristic of superficial siderosis. Extensive diagnostic work-up excluded causative pathologies of bleeding. Lumbar puncture and continuous intra-ventricular cerebrospinal fluid (CSF) pressure monitoring revealed continuous CSF pressure increase. Implantation of a ventriculo-peritoneal shunt led to complete clinical recovery. Our case emphasizes that patients with superficial siderosis may present with sudden elevation of intracranial pressure due to chronic intracranial hypertension. In this situation permanent CSF drainage provides a useful therapeutic option.
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PMID:Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis. 1865 43

Dural sinus thrombosis (DST) has an annual incidence of 3-4 per million and can result from many aetiologies. Presentation of the disease can vary considerably, as can the aetiology and delay of symptoms to clinical detection. Symptoms on presentation include headache, seizures, focal neural deficits and altered mental status. There are many aetiological risk factors associated with DST, which include hypercoagulable states, oral contraceptive use, infection and mechanical causes such as cranial trauma. DST as a result of trauma is rare and aetiologies range from mechanical falls with or without skull fracture, firework explosions, gunshots to the head, blunt trauma to the head and closed head injury. Internal jugular vein thrombosis is also a rare disease and as with DST, traumatic aetiologies are uncommon. More common aetiologies include iatrogenic causes related to catheterisation as well as infectious causes (eg, Lemierre's syndrome). A case of thrombosis of the transverse sinus, sigmoid sinus and internal jugular vein associated with a closed head injury as the result of a motorcycle accident is presented.
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PMID:Closed traumatic head injury: dural sinus and internal jugular vein thrombosis. 1895 26

Primary central nervous system vasculitis (PCNSV) is a rare disease with an annual incidence rate of 2.4 cases per 1,000,000 person-years. PCNSV causes various neurological symptoms dominated by headache as well as consciousness and mental disturbances. The disease sometimes imitates a brain tumour on CT and rarely presents as stroke. The diagnosis of PCNSV is difficult and frequently requires a brain biopsy in which transmural vascular inflammation involving leptomeningeal or parenchymal vessels is typically found. Angiographic changes indicating an irregular course of vessels with characteristic segmental narrowing can be observed but sometimes the angiogram is normal. The authors present a 57-year-old man in whom PCNSV was diagnosed in brain biopsy. The patient was treated with corticosteroid pulses for 18 months with good effect lasting for 2.5 years. After one year of ending glucocorticoid therapy the symptoms had occurred again and in spite of combined therapy of glucocorticoids with cyclophosphamide the patient died.
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PMID:[Primary central nervous system vasculitis imitating a brain tumour--a case report]. 1897 42

Cystic glioblastoma multiforme (GBM) is a rare disease. Its exact prevalence has not yet been reported. Also, the mechanism of cyst formation remains to be elucidated. We report a case of GBM with a large peripheral cyst. A 43-year-old woman visited our clinic with a 3-month history of severe headache, memory impairment and general weakness. T1-weighted gadolinium-enhanced magnetic resonance (MR) image revealed a midline enhanced solid mass and bilateral symmetric banana-shaped peripheral cysts. A centrally enhanced mass was measured 2x4 cm in size and both mass and cysts as 7x7 cm. Both the frontal lobe and the frontal horn were severely compressed inferiorly and posteriorly. We resected a midline solid tumor and cysts via the bilateral interhemispheric transcortical approach. Histopathologic examination revealed GBM. The patient was subsequently treated with fractionated conventional brain radiation therapy, followed by temozolomide chemotherapy. Eighteen months later, there was no tumor recurrence and no neurological deficits were noted. Our patient showed no tumor recurrence and a long survival at a long follow-up.
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PMID:Midline glioblastoma multiforme with bilateral symmetric cysts. 1909 13

Gas forming brain abscess is a rare disease caused by Klebsiella pneumoniae occurring in patients with impaired host defense mechanism such as diabetes mellitus or liver cirrhosis. A 59-year-old man with 2-year history of diabetes mellitus and 20-year history of liver cirrhosis presented to the hospital with headache. On the day after admission, severe headache was developed and he deteriorated rapidly. Brain CT showed a non-enhanced mass including multiple air density as well as surrounding edema seen in the right occipital lobe, and isodensity air-fluid level seen in the right lateral ventricle. Despite emergent ventricular drainage and intraventricular and intravenous administration of antibiotics, his condition progressively worsened to sepsis and to death after 5 days. Bacterial culture of blood and ventricular fluids disclosed a Gram (-) rod, Klebsiella pneumoniae. In this report we review the pathogenic mechanism and its management.
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PMID:Gas-forming brain abscess caused by Klebsiella pneumoniae. 1913 83

Sometimes, a clinical course that initially appears to be 'classic' turns out to be a manifestation of a rare disease. We report on a 62-year-old woman who presented initially with episodic headache, followed by cardiogenic shock. What was first thought to be an ST-segment elevation acute myocardial infarction, later to be a takostubo cardiomyopathy, finally appeared to be a catecholamine-induced cardiomyopathy due to a pheochromocytoma. This case illustrates that in a patient with presumed takotsubo cardiomyopathy and episodic headache, sweating, hypertension or tachycardia, pheochromocytoma needs to be excluded.
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PMID:From trivial headache to life-threatening disease. 1917 13

(1) Human insulin-like growth factor type 1 (IGF-1) is the main effector of growth hormone action. Primary IGF-1 deficiency is a rare disease, mainly resulting in very short stature; (2) Mecasermin is a recombinant IGF-1 marketed for this indication as a twice daily subcutaneous injection; (3) Clinical evaluation is mainly based on a non-comparative follow-up study of 76 children with an average age of 7 years, some of whom were treated for 8 years. The mean height at treatment initiation was 6.7 standard deviations below normal. Eight years later, it was 5.2 standard deviations below normal, i.e. their growth failure remained very severe; (4) The main short-term adverse effects of mecasermin are hypoglycaemia, headache and intracranial hypertension. Nearly one in 5 children developed tonsillar hypertrophy, resulting in otitis and hypoacusis; (5) Animal studies showed hypertrophy of other organs (kidneys, spleen and heart) as well as carcinogenic effects. The risk in humans is unknown; (6) The mecasermin packaging is not well-adapted (a multidose vial designed to be punctured several times), and is a potential source of contamination and errors. Prefilled pens or syringes would be easier to use; (7) In practice, the limited clinical benefits of mecasermin do not justify exposure to its potential risks.
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PMID:Mecasermin: new drug. Insufficient improvement in statural growth. 1963 20

A 29 year old male was admitted at the emergency room suffering from gradually worsening headache followed by nausea. In the hospital patient presented with lethargy, reduction of consciousness level and bilateral hypoacusis. Ophthalmic examination and fluorescein angiography showed retinal vasculitis. This finding was crucial to the diagnosis of Susac syndrome, a rare disease characterized by vasculopathy of retina, encephalic tissues and cochlea. Patient was treated with immunosuppressors to prevent him from developing severe sequelae of this disease.
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PMID:Retinal vasculitis in Susac syndrome: case report. 1966 76

Pulmonary arterial hypertension (PAH) is a rare disease characterized by vascular proliferation and remodeling, resulting in a progressive increase in pulmonary arterial resistance, right heart failure, and death. The pathogenesis of PAH is multifactorial, with endothelial cell dysfunction playing an integral role. This endothelial dysfunction is characterized by an overproduction of vasoconstrictors and proliferative factors, such as endothelin-1, and a reduction of vasodilators and antiproliferative factors, such prostacyclin and nitric oxide. Phosphodiesterase type 5 (PDE-5) is implicated in this process by inactivating cyclic guanosine monophosphate, the nitric oxide pathway second messenger. PDE-5 is abundantly expressed in lung tissue, and appears to be upregulated in PAH. Three oral PDE-5 inhibitors are available (sildenafil, tadalafil, and vardenafil) and are the recommended first-line treatment for erectile dysfunction. Experimental studies have shown the beneficial effects of PDE-5 inhibitors on pulmonary vascular remodeling and vasodilatation, justifying their investigation in PAH. Randomized clinical trials in monotherapy or combination therapy have been conducted in PAH with sildenafil and tadalafil, which are therefore currently the approved PDE-5 inhibitors in PAH treatment. Sildenafil and tadalafil significantly improve clinical status, exercise capacity, and hemodynamics of PAH patients. Combination therapy of PDE-5 inhibitors with prostacyclin analogs and endothelin receptor antagonists may be helpful in the management of PAH although further studies are needed in this area. The third PDE-5 inhibitor, vardenafil, is currently being investigated in PAH. Side effects are usually mild and transient and include headache, flushing, nasal congestion, digestive disorders, and myalgia. Mild and moderate renal or hepatic failure does not significantly affect the metabolism of PDE-5 inhibitors, whereas coadministration of bosentan decreases sildenafil and tadalafil plasma levels. Due to their clinical effectiveness, tolerance profile, and their oral administration, sildenafil and tadalafil are two of the recommended first-line therapies for PAH patients in World Health Organization functional classes II or III.
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PMID:Phosphodiesterase type 5 inhibitors in pulmonary arterial hypertension. 1976 39


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