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The purposes of this article are to report a case with temporal arteritis (TA) and to summarize and reanalyze the cases of temporal arteritis associated with fever in published articles for understanding better the clinical features of TA. A case with biopsy-proven TA is reported. The publications with TA and fever were searched by using MEDLINE in English from 1966 to 1999. Three hundred sixty cases of temporal arteritis associated with fever were reanalyzed. The results showed that a case of biopsy-proven TA with typically clinical manifestation was initially misdiagnosed and that the reanalysis of 360 cases revealed that the common clinical findings at presentation were abnormal temporal arteries, headache, low fever, loss of weight, polymyalgia rheumatica, jaw claudication, vision disorder, arthralgis or myalyias, and ear pain and that the uncommon clinical findings at presentation were high fever, malaise, anorexia, breast pain, transient ischemic attack/stroke, cough, mental disorder, diarrhea, and uterine prolapse, etc. Laboratory findings were the range of erythrocyte sedimentation rate (ESR) 14 to 149 with a mean of 97.0 mm/hr, white blood cells being normal or increased in the range of 10.9 to 22.9 x 10(9)/L, hemoglobin level 7 to 16 g/dL, the platelets count increased to 785 x 10(9)/L, and microscopic hematuria. The diagnosis was made by a combination of clinical features, an increased ESR, a response to steroids, and, most specifically, temporal artery biopsy. The initial diagnosis was misdiagnosed in 38.2% of patients. In conclusion, the features of TA associated with fever have not been widely appreciated yet. TA is a common cause of fever of unknown origin (FUO) in the elderly. TA should be considered when patients complain of common and uncommon manifestations. An elevated ESR will aid in the diagnosis of TA, and temporal artery biopsy will provide certainty.
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PMID:Temporal arteritis and fever: report of a case and a clinical reanalysis of 360 cases. 1110 64

This paper reports a case of spontaneous indirect carotid cavernous fistula that presented with pulsatile tinnitus, left-sided temporal headache and left-sided ptosis. The pulsatile tinnitus, its aetiology and investigation are discussed. The importance of pulsatile tinnitus is highlighted, with a discussion of carotid-cavernous fistulas. This case illustrates that clinically silent cavernous sinus thrombosis can give rise to spontaneous indirect carotid cavernous fistula. Magnetic resonance imaging angiography was used in diagnosis. Treatment ranges from observation, as in our case, to transvenous endovascular techniques.
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PMID:Indirect carotid cavernous fistula presenting as pulsatile tinnitus. 1112 53

We report facial palsy as the sole cranial neuropathy complicating an ipsilateral internal carotid artery dissection. A previously healthy 44-year-old man developed retro-orbital and temporal headache with associated nausea while engaged in modest physical exercise. On the following morning he noticed a left ptosis and miotic pupil. One week later he woke with a left facial weakness. On the same day he had a 90-minute episode of expressive dysphasia. Magnetic resonance imaging and angiography demonstrated left internal carotid artery dissection. The temporal association between our patient's facial nerve palsy and typical features of spontaneous internal carotid artery dissection suggests a common aetiology. We suggest that involvement of the VII cranial nerve in isolation followed disruption of an anomalous nutrient artery. The delay in clinical manifestation may imply extension of the dissection.
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PMID:Facial nerve palsy secondary to internal carotid artery dissection. 1113 63

Cluster headache is a primary headache with well-defined diagnostic criteria. Nevertheless, in 3-5% of patients this syndrome is secondary to diverse cranial structural abnormalities. Atypical features which suggest a secondary or symptomatic origin include absence of periodicity and regular hourly recurrence, persistence of background pain among attacks, unsatisfactory response to treatment, and presence of neurological signs other than ptosis or miosis. We present two patients with symptomatic cluster-like headache. In one, the syndrome was associated with a fistula of the right superficial temporal artery. After embolization the pain attacks ceased. The second patient presented a ventricular xanthoma, located in the occipital horn; after surgical excision, the pain attacks did not recur. The progressive increase in frequency and absence of regularly hourly recurrence of pain episodes was a common feature in both cases. We comment and revise the pathophysiology of this syndrome, with emphasis on the case of the xanthoma, since we did not find a similar case described. If a secondary cluster-like headache is suspected, neuroimaging studies should be done.
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PMID:[Symptomatic cluster headache: presentation of 2 cases]. 1119 50

On November, 1997, a 15-year-old boy visited our hospital because of headache, fever and arthralgia. He was treated with 5 mg/day of prednisolone thereafter. On October 21, 1998, he was admitted because of remittent fever and multiple arthralgia and diagnosis of juvenile rheumatoid arthritis (JRA) was made. He was also found to have hypertension of 210/110 mmHg, and soon developed ptosis of the eye, facial paresis and perceptive deafness of the right side. Cerebrospinal fluid showed protein of 98 mg/dl and mildly elevated IgG, IgA and IgM levels with normal cell count. Brain MRI examination revealed multiple cerebral lesions in the frontal, parietal and cerebellar areas on the right, whose cause was thought to be vasculitis. Renal angiography demonstrated a right renal artery stenosis, compatible with renovascular hypertension. He was treated with 60 mg of prednisolone per day, which brought about a satisfactory improvement of the above rheumatic and neurologic signs. On November 17, 1998, he received a follow-up study of MRI, which failed to show any cerebral lesions, supporting the effectiveness of prednisolone. An angiotensin converting enzyme inhibitor successfully normalized hypertension and renin activity in serum, although renal blood flow did not increase.
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PMID:[A case with juvenile rheumatoid arthritis who developed cerebral vasculitis and venovascular hypertension]. 1121 60

A woman, aged 59 years, developed a constant, left, occipital headache associated with episodes of discrete exacerbations occurring three to five times daily for 3 days, each lasting 15 to 20 minutes, and associated with left ptosis, conjunctival injection, and redness of the left ear. Pain-free remissions, which usually lasted 2 weeks, ceased after a mild neck injury, but the headaches responded promptly to indomethacin. This case, illustrating a transition from an occipital episodic to chronic paroxysmal hemicrania, is discussed as a variation of the trigeminal-autonomic cephalalgias.
Headache
PMID:Extratrigeminal episodic paroxysmal hemicrania. Further clinical evidence of functionally relevant brain stem connections. 1127 7

A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.
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PMID:Classical malignant rhabdoid tumor of central nervous system in 9-year-old Korean. 1129 95

We report a man in whom a 15 cm. renal tumor was excised at the age of 49. The pathological examination showed a clear cell carcinoma. Five years later, he presented with headache, vomiting and unilateral palpebral ptosis. Imaging studies showed a sellar tumor with pituitary apoplexy. The tumor was excised and the pathological study disclosed a clear cell tumor, positive for vimentin, cytokeratins AE1 and AE3 and immunohistochemically negative for LH, TSH, ACTH and GH. Considering the similar histopathological features, it was considered as a metastasis of the renal tumor. The patient was supplemented with thyroid, adrenal and gonadal hormones. Seven years later, he presented a new tumor in the remaining kidney, that corresponded to a cystic papillary renal cell carcinoma. Afterwards, he presented a transitional urinary bladder tumor. Mortality associated to renal cell tumors is 90% at 5 years, and pituitary metastases are extraordinarily uncommon.
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PMID:[Apoplexy in pituitary metastasis of renal cell carcinoma. Clinical case followed for 7 years]. 1134 89

A 34-year-old man developed fever, headache, nausea, double vision, exophthalmus, ptosis, disturbance of vision and oculomotor nerve palsy. Magnetic resonance imaging and cerebral angiography led to the clinical diagnoses of cavernous sinus thrombophlebitis and suspicion of bacterial aneurysm of the left internal carotid artery, respectively. Peptostreptococcus was detected in blood culture analysis. He died 15 days after admission. Systemic organs showed several septic changes. In particular, the bilateral cavernous sinuses were enlarged and showed severe neutrophilic leukocyte infiltration of the walls and organization, recanalization and abscesses in thrombi. In anterior to the middle cranial fossa, abscess-forming, necrotic, hemorrhagic meningitis, purulent sphenoid sinusitis, pyogenic osteomyelitis of the sphenoid bone, suppurative encephalitis, and inflammatory necrosis of the hypophysis were seen. Based on these findings, we diagnosed the patient with cavernous sinus thrombophlebitis caused by sphenoid sinusitis.
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PMID:Cavernous sinus thrombophlebitis caused by sphenoid sinusitis--report of autopsy case. 1143 Apr 92

Congenital disorders of glycosylation (CDG) and mitochondrial diseases are multisystem disorders with clinical characteristics that may overlap. We present four patients with CDG whose phenotypes suggested the diagnosis of a mitochondrial disease. Patients 1 and 2 are siblings with hemiplegic headache, stroke-like episodes, lactic acidaemia and history of maternal migraine; their initial clinical diagnosis was MELAS syndrome (mitochondrial encephalopathy, lactic acidosis and stroke-like episodes). Patient 3 suffers from ataxia, neuropathy, ophtalmoplegia and retinitis pigmentosa suggestive of NARP (neuropathy, ataxia, and retinitis pigmentosa) syndrome. Patient 4 presented with neurological regression mimicking Leigh disease, with ptosis, myoclonus, ataxia and brainstem and cerebellar atrophy. Screening for mitochondrial disease including enzyme and mtDNA investigations on muscle biopsy were performed on Patients 1, 2 and 4 with normal results. However, evidence for a glycosylation disorder was substantiated by an increased carbohydrate deficient transferrin (CDT). The isoelectric focussing pattern of serum sialotransferrin was typical of CDG type I in Patients 1, 2 and 3 and was shifted towards the less sialylated bands in case 4. A deficiency of phosphomanomutase (PMM) confirmed the diagnosis of CDG-Ia in Patients 1, 2 and 3, who are compound heterozygous for mutations R141H/T237M (Patients 1 and 2) and R141H/P113L (Patient 3). In Patient 4, PMM activity was normal, and further enzymatic and molecular studies are underway. As the search for the primary defect in mitochondrial diseases is often unsuccessful, the pool of mitochondrial patients that remain without definite diagnosis might include CDG cases. Routine screening for CDG may avoid precocious invasive investigations.
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PMID:Congenital disorders of glycosylation (CDG) may be underdiagnosed when mimicking mitochondrial disease. 1158 67


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