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

Chronic paroxysmal hemicrania (CPH) is a relatively new condition first described in 1973. Since its first description, over 80 cases have been reported worldwide. Very few cases have been reported in children. We report the presentation of an 8-year-old boy to illustrate the problems in diagnosis and treatment of this rare disease in childhood.
Headache 1994 Oct
PMID:Chronic paroxysmal hemicrania in a child. 777 83

A 21-year-old man was hospitalized with complaints of headache, nausea, polyuria, reduced body hair and reduced libido. Plain CT scan and MRI revealed multiple tumors in the pineal and suprasellar regions, and in the dorsal aspect of the medulla oblongata. Endocrinological examination showed hypopituitarism due to hypothalamic dysfunction. Replacement therapy with hydrocortisone was started preoperatively. Suprasellar tumor was explored. Postoperative CT scan and MRI showed marked diminishment of not only suprasellar tumor, but also tumors in the two other regions. These tumors were supposed to be sensitive to corticosteroid hormone. Histopathological diagnosis was T cell type malignant lymphoma. The patient was followed up for 10 months post-operatively with no recurrence on CT scan. Primary intracranial malignant lymphoma is not a rare disease today. However, primary intracranial T cell type lymphoma is extremely rare. Hypopituitarism due to suprasellar malignant lymphoma is also rare, and only 3 such cases have been reported previously. In our case, the tumor was located in the pineal and suprasellar regions, and the dorsal aspect of the medulla oblongata. Such disseminated malignant lymphoma as ours shows radiological resemblance to germ cell tumor. No such diseminated malignant lymphoma has been reported previously. We think that, in its radiological and clinical features, our case is very suggestive of primary intracranial malignant lymphoma.
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PMID:[A case of primary intracranial T cell type malignant lymphoma, radiologically resembling germ cell tumor and presenting hypopituitarism]. 839 52

Benign intracranial hypertension-pseudotumor cerebri (PTC) is a rare disease in childhood. We report about our experience in five children with PTC, aged two to ten years. The main symptoms were headache, palsy of VI.th cranial nerve and papilledema. Neuroradiologic studies showed normal ventricles in four and a slit ventricle in one. Enlarged optic nerve sheaths were found in one child prior to therapy. The cerebro spinal (CSF) fluid pressure varied from 380 mm to 480 mm CSF. Four children received acetazolamide, one child dexamethasone. Permanent visual deficit did not occur in any child.
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PMID:[Benign intracranial hypertension in childhood--pseudotumor cerebri]. 841 99

Listerial brainstem encephalitis is a rare disease. Only 62 cases have been reported previously; all were in adults, only 8% of whom were immunosuppressed. The disease has a characteristic biphasic course: a nonspecific prodrome of headache, nausea or vomiting, and fever lasting for several days is followed by progressive asymmetrical cranial-nerve palsies, cerebellar signs, hemiparesis or hypesthesia, and impairment of consciousness. Neck stiffness was initially present in only 55% of the cases described thus far. Studies of cerebrospinal fluid often revealed only mild abnormalities. Cultures of cerebrospinal fluid and blood were positive in 41% and 61% of cases, respectively. Respiratory failure occurred in 41% of cases. Initial computed tomography of the brain often gave normal results; magnetic resonance imaging better demonstrated brainstem abnormalities. Overall mortality was 51%. All untreated patients died. When treatment with ampicillin or penicillin was initiated early, the rate of survival was > 70%; however, neurological sequelae developed in 61% of survivors.
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PMID:Brainstem encephalitis (rhombencephalitis) due to Listeria monocytogenes: case report and review. 850 61

Vaccination against Japanese Encephalitis (JE) has been carried out extensively in many Asian countries for the past 20 years. The vaccine was generally considered to be effective and of low reactogenity. However, since 1989 an unusual number of systemic reactions characterized mainly by generalized urticaria and/or angioedema following JE vaccination were reported from Australia, Canada and Denmark, 860 travellers were recruited during a period of 16 months for a prospective study with the aim to investigate the type and incidence of side effects following JE vaccination (JEV) in German travellers. 826 received a primary immunization (2 injections at days 0 and 7-14) and 34 received a single booster injection. A detailed standardized questionnaire was distributed to all vaccinees after the first injection. A total of 509 questionnaires could be evaluated, which represents a return rate of 59.2%. 46% of the vaccinees reported about no adverse events at all. 54% reported about one or more adverse effects. Local reactions at the injection site were observed by 209 vaccinees, while 65 reported about systemic side effects like headache, fever, dizziness and generalized rash. There was no significant difference following first or second injection of the primary immunization or the booster injection, respectively, regarding incidence, severity or type of side effects. 2.2% of the vaccinees reporting reactions sought medical advice and 1.8% were judged unfit for work for an average of 2.2 days. The amount of systemic reactions might indicate a potential hazard of serious anaphylactic reactions. Unlike hepatitis A. Japanese encephalitis is an extremely rare disease in travellers. Therefore, the risk of acquiring the disease when travelling to affected areas without prior immunization should be considered against the risk of developing serious side effects after vaccination. We conclude that JEV should remain restricted to travellers with an increased risk of acquiring JE.
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PMID:Adverse reactions to Japanese encephalitis vaccine in travellers. 870 68

Mastocytosis is a rare disease of mast-cell proliferation with involvement of the reticuloendothelial systems including skin, bone, gastrointestinal tract, liver, lungs, spleen, and lymph nodes. Systemic mastocytosis is characterized by a combination of symptoms that relate to the mast cells' release of vasoactive substances, such as histamine. These symptoms include urticaria pigmentosa, flushing, syncope with hypotension, headaches, nausea, vomiting, diarrhea, and occasional bronchospasm. The diagnosis of mastocytosis is typically based on the presence of the characteristic extraosseus manifestations. A well recognized roentgenographic feature seen in 70-75% of patients with mastocytosis is diffuse osteolysis and osteosclerosis, affecting primarily the axial skeleton and the ends of the long bones. Rarely, the bony involvement consists of generalized osteoporosis, which may lead to pathologic fracture, or solitary lesions (mastocytomas) which may cause symptoms of localized pain. Four patients with previously diagnosed systemic mastocytosis had unusual skeletal lesions. Clinical and laboratory evaluation of these patients eventually led to the correct diagnosis of systemic mastocytosis. We report these four cases to emphasize the need for thorough evaluation of unusual musculoskeletal findings in association with extraosseus symptoms that are characteristic of mastocytosis. Knowledge of a wide differential diagnosis of unusual skeletal lesions should include systemic mastosytosis.
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PMID:Mastocytosis presenting as a skeletal disorder. 912 84

Giant cell arteritis (GCA) is a spontaneous vasculitic syndrome specifically involving the walls of medium and large arteries. While involvement of other arterial beds is occasionally identified, this syndrome is most frequently recognized when symptomatic involvement of the temporal arteries occurs. Vascular lesions are characterized by patchy granulomatous infiltrates composed of T cells, macrophages, histiocytes, and giant cells. A better prognosis depends on early recognition of the clinical symptoms and prompt treatment. Diagnosis was based on the 5 clinical criteria previously used by the American College of Rheumatology (1990): 1) age 50 years or older; 2) new localized headache; 3) temporal artery tenderness or decrease in temporal artery pulse; 4) erythrocyte sedimentation over 50 mm/ hour; 5) abnormal result on artery biopsy. Giant cell arteritis was considered a rare disease under age 50; however, it is now known to be an important and significant cause of morbidity and mortality in elderly people. Therefore early recognition and treatment with corticosteroid are very important. There is no general agreement concerning the initial dosage, 40-65 mg/day are commonly recommended. After a few months the majority of patients can be treated with a low maintenance dosage of prednisolone (5 to 7.5 mg/day). The mean duration of treatment is about 5 years. The literature is reviewed and the clinical implications of this disease are discussed.
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PMID:[Horton's giant cell arteritis]. 928 Aug 67

Here we present a 53-year-old woman with rheumatoid pachymeningitis. The subject had rheumatoid arthritis (RA) for 15 years. In April, 1996 she began to experience intermittent headaches. In September, her headaches became severe and continuous. In October, she suddenly developed ptosis of the left eye and diplopia. She also started to have dysphagia and she found it increasingly difficult to eat. She was admitted to our hospital on November 1, 1996. Neurological examinations revealed palsies of the left IIIrd, IVth, and VIth, and bilateral IXth, and Xth cranial nerves. Laboratory findings showed leukocytosis, elevated blood sedimentation rate, and positive CRP. Serum RA titer was positive (30x). The cerebrospinal fluid was normal and bacteriological examination was negative. T1-weighted MRI demonstrated hypertrophic cranial dura extending from the falx cerebri to tentorium cerebelli, which was enhanced markedly by Gd-DTPA. The dura adjacent to the cavernous sinus and the clivus were also thickened, which probably caused her cranial polyneuropathies. The dural biopsy showed massive infiltration of the inflammatory cells throughout the dura, proliferation of collagen fibers, and necrotic granuloma with neutrophilic infiltrations. Neither rheumatoid nodules, nor vasculitis were found. Despite the absence of rheumatoid nodules in the dural biopsy, the clinical features, pathologic specimens, and MRI findings of the thickened dura were most consistent with rheumatoid pachymeningitis. Administration of dexamethason ameliorated her headache on the 4th hospital day, and the cranial polyneuropathies completely disappeared on the 35th hospital day. The dural enhancement previously seen on the contrast T1-weighted MRI was diminished. Serum RA titer was also normalized (10x). Rheumatoid pachymeningitis is an extremely rare disease, and only 16 cases were reported in the literatures. Hypertrophic pachymeningitis should be considered as a diagnostic possibility in RA patients who have prolonged headache, and Gd-DTPA MRI is recommended to demonstrate the dural involvement.
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PMID:[A case of rheumatoid pachymeningitis]. 943 Oct

Vasculitis confined to the central nervous system (CNS) is a rare disease usually characterized by headache and focal neurologic symptoms. Patients with primary vasculitis of the CNS may have symptoms and laboratory findings of systemic disease such as fatigue and elevated erythrocyte sedimentation rate, but by definition, focal inflammation should not be present outside the CNS. We describe 3 patients with uveitis in association with this diagnosis. The recognition of this association adds to the complex differential diagnosis of uveitis in association with CNS disease, and indicates that "isolated" angiitis of the CNS may display clinical features outside the brain and spinal cord.
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PMID:Uveitis and central nervous system vasculitis. 951 87

Progressive multifocal leukoencephalopathy (PML), a formerly rare disease, is estimated to occur in up to 5% of all patients with AIDS. The high prevalence of PML in AIDS patients currently enables a comprehensive evaluation of this disorder. We evaluated the clinical and radiographic features of PML in a large cohort of AIDS patients identified by retrospective chart review from 1981 to 1994. Two hundred and five patients were diagnosed with PML of which 154 met the inclusion criteria. Seventy-two (47%) were pathologically confirmed and the remaining 82 (53%) met clinical and radiographic criteria. There was a 12-fold increase in the frequency of PML between 1981-1984 and 1991-1994. PML affected 136 men and 18 women with AIDS. Eighty-four percent of cases were 20-50 years old (range 5 to 68 years). The most common AIDS risk factors were homosexuality (57%) among men and heterosexual transmission (28%) and intravenous drug abuse (28%) among women. In 27% of patients, PML heralded AIDS. Common manifestations included weakness, gait abnormalities, speech disturbance, cognitive disorders, headache, and visual impairment. The CD4 lymphocyte counts exceeded 200 cells in 11% at the time of presentation. Involvement of posterior fossa structures was evident in 48% of cranial magnetic resonance imaging (MRI) studies, but in only 11% of computed tomographies (CT) of the brain. Contrast enhancement, typically faint and peripheral, was seen in 10% of CT scans and 15% of MRIs. The median survival was 6 months and survival exceeded 1 year in 9%. PML is no longer a rare disease. It often heralds AIDS and may occur in the absence of significant decline in CD4 lymphocytes. Survival is generally poor, although prolonged survival beyond 1 year is not unusual.
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PMID:Progressive multifocal leukoencephalopathy in patients with HIV infection. 953 Oct 12


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