Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 73-year-old woman was admitted to hospital with a one-month history of temporal headache, low-grade fever, fatigue, nocturnal sweats and pleural pain. On the fifth day after admission she developed chest pain at the left site of the thorax, productive cough and progressive dyspnea. A pleural effusion was revealed on physical examination, as well as a bilateral temporal artery thickening. An erythrocyte sedimentation rate of 135 mm in the 1st hour was found. Chest X-ray showed left pleural effusion. Thoracocentesis revealed serous fluid exudate. A percutaneous pleural biopsy showed only minimal inflammatory changes. Temporal artery biopsy showed giant cell arteritis. The patient received prednisone 60 mg/daily with a dramatic clinical response. Pleural effusion is a rare manifestation of temporal arteritis; only seven cases have been reported worldwide. We present a new case of temporal arteritis with pleurisy.
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PMID:Pleural effusion in temporal arteritis. 1279 76

Temporal arteritis was first described in the late nineteenth century. Despite considerable progress in understanding the disease, its rarity in the young and in those who are not of Scandinavian ethnicity remains unexplained. Microbiologic agents and immunologic mechanisms have been implicated as causative factors. Although steroids remain the drug of choice, the use of other immunologic therapies has been proposed. This paper reviews the disease's history, probable etiologies, clinical manifestations, and its diagnostic and treatment challenges.
Curr Pain Headache Rep 2003 Aug
PMID:Recent advances in temporal arteritis. 1282 79

New daily persistent headache (NDPH), which is the acute onset of headache within 3 days and is persistent for 15 days or more each month for at least 3 months, is a predominantly female heterogeneous subtype of chronic daily headache, typically with migraine features of unknown etiology. NDPH may be a presentation of other primary headaches such as new onset migraine, tension, or benign thunderclap headache. The headaches can be difficult to treat. The diagnosis is one of excluding the many secondary types or NDPH mimics, which is especially critical early in the course of the disease when a secondary etiology is more likely. NDPH mimics include postmeningitis headache, NDPH with medication rebound, neoplasms, temporal arteritis, chronic meningitis, chronic subdural hematoma, post-traumatic headaches, sphenoid sinusitis, hypertension, subarachnoid hemorrhage, low cerebrospinal fluid pressure syndrome, cervical artery dissections, pseudotumor cerebri without papilledema, and cerebral venous thrombosis.
Curr Pain Headache Rep 2003 Aug
PMID:New daily persistent headache. 1282 80

Primary erythermalgia is a rare autosomal dominant disease characterised by intermittent burning pain with redness and heat in the extremities. A previous study established the linkage of primary erythermalgia to a 7.94 cM interval on chromosome 2q, but the causative gene was not identified. We performed linkage analysis in a Chinese family with primary erythermalgia, and screened the mutations in the two candidate genes, SCN9A and GCA, in the family and a sporadic patient. Linkage analysis yielded a maximum lod score of 2.11 for both markers D2S2370 and D2S2330. Based on critical recombination events in two patients in the family, we further limited the genetic region to 5.98 cM between D2S2370 and D2S2345. We then identified two missense mutations in SCN9A in the family (T2573A) and the sporadic patient (T2543C). Our data suggest that mutations in SCN9A cause primary erythermalgia. SCN9A, encoding a voltage-gated sodium channel alpha subunit predominantly expressed in sensory and sympathetic neurones, may play an important role in nociception and vasomotor regulation.
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PMID:Mutations in SCN9A, encoding a sodium channel alpha subunit, in patients with primary erythermalgia. 1498 75

This article discusses various cranial neuralgias and headaches linked to vascular disorders, including trigeminal neuralgia, intracerebral hemorrhage, temporal arteritis, and central nervous system angiitis. Also discussed are diagnostic methods, treatment options, and pain management.
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PMID:Cranial neuralgias and headaches associated with cranial vascular disorders. 1502 8

A 79-year-old man with myelodysplastic syndrome developed a right optic neuropathy with optic disc edema and intractable periocular pain, one month after undergoing removal of a gangrenous gallbladder. Although results of a temporal artery biopsy were negative, he was treated with prednisone for presumed temporal arteritis. Attempts at tapering the prednisone dose led to recurrence of periocular pain. On neuro-ophthalmologic evaluation six months after the prednisone treatment was begun, he had developed right fourth and sixth cranial nerve palsies, and magnetic resonance imaging demonstrated a right orbital apex mass. Trans-sphenoidal biopsy revealed Aspergillus fumigatus. During treatment of aspergillosis, the patient developed a left hemiparesis. Magnetic resonance imaging disclosed multiple ring-enhancing cerebral masses. Biopsy revealed Nocardia asteroides. The patient was successfully treated for both infections with recovery of neurologic function except for the right optic neuropathy. Although immunocompromised patients are known to be subject to multiple infections, this may be the first reported case of concurrent sino-orbital aspergillosis and cerebral nocardiosis.
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PMID:Concurrent sino-orbital aspergillosis and cerebral nocardiosis. 1517 67

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.
Curr Pain Headache Rep 2004 Aug
PMID:Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome. 1522 94

Medial arterial calcification, which has been increasingly recognized in end-stage renal disease (ESRD) patients, has been associated with acutely symptomatic vascular complications including calcific uremic arteriolopathy (calciphylaxis) and ischemic changes in the extremities. This report describes a 50-year-old ESRD patient on maintenance hemodialysis in whom medial arterial calcification developed with features mimicking the findings of temporal arteritis. He complained of persistent bilateral temporal area headaches with associated symptoms of blurred vision; pain in his shoulders, hips, and knees; and intermittent symptoms consistent with jaw claudication. He was not receiving calcium or vitamin D supplements. Superficial temporal arteries were dilated, tortuous, nodular, and tender to palpation. Ophthalmologic examination was unremarkable, except for the presence of peripapillary atrophy. Temporal artery biopsy results showed medial arterial calcification with mild inflammatory changes. No giant cells were identified. Additional long-term complications of medial arterial calcification have included the development of painful ischemic ulceration of the glans penis and extensive mitral annulus calcification detected by echocardiography. The findings in this patient show that clinical manifestations of medial artery calcification associated with ESRD can mimic those seen with other vascular diseases.
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PMID:Medial arterial calcification mimicking temporal arteritis. 1538 38

The headache is a very frequent symptom and represents the 0.36%-2.5% of all reasons of claim to Emergency Department. Even if it is rarely related to high risk diseases, it is mandatory to promptly differentiate life-threatening conditions. In order to establish a correct diagnostic and therapeutic pathway and ask for aimed specialistic consultation, the emergency physician must be familiar with the various categories of headache. It is important to distinguish between essential headache and secondary headache. All patients presenting to the emergency department with the complaint of headache should be interviewed carefully regarding their history. The quality of pain associated with the intensity, location, rate, duration, modality of onset, relieving or worsening conditions, response to drugs, symptoms or signs associated must be investigated as well. Careful neurological examination including the vision of fundus oculi and the evaluation of rigor nucalis can provide further important diagnostic information. Laboratory exams do not usually give significant issues in the majority of patients with headache. However, dosage of inflammation index can be useful when an infective or inflammatory disease is suspected. CT scan can rule-out the suspicion of organic intracranial causes. When the physician suspects meningitis or subarachnoid hemorrhage (SAH) not showed by CT scanning, rachicentesis can turn out diagnostic. The modality of onset, clinical characteristics and differential diagnosis of subarachnoid hemorrhage, intracranial hypertension, colloidal cyst of the third ventricle, trigeminal neuralgia, temporal arteritis and pituitary adenomas and apoplexy will be discussed. These diseases are not only of neurological and neurosurgical interest, but involve also the physician in the Emergency Department.
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PMID:The headache in the Emergency Department. 1554 36

Although the disease known as temporal arteritis, giant cell arteritis, and Horton's disease has been known since at least the 10th century, Hutchinson (1890) and Horton (1932) characterized the condition in the more recent medical literature. The diagnosis of this potentially serious illness can be surprisingly elusive, and treatment is fraught with some frustrating pitfalls. For the most part, careful evaluation of patients (usually elderly) with the typical presentation of head pain and constitutional signs yields prompt diagnosis. Treatment with corticosteroids, the standard since the mid-1950s, is usually very successful in relieving pain and tenderness and in preventing visual and other sequelae. This article discusses the evolution of medical understanding of the disease, pathophysiology, diagnosis, and modern treatment options.
Curr Pain Headache Rep 2005 Aug
PMID:Horton's disease: past and present. 1600 41


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