Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 1987, a 69-year-old female suffering from epilepsy and right hemiparesis was admitted to the hospital and given conservative therapy. During the next three weeks she became a stuporous state. Steroids were then prescribed, and she made a complete recovery. Following hormonal and immunological investigations, a diagnosis of Hashimoto's disease with hypothyroidism was made. Following her discharge, thyroxin and anticonvulsant treatments continued. On May 30, 1989, at the age of seventy-one, the patient was found lying unconscious at home, and taken to our hospital. She had signs of a confused state, and seizures of her right arm on admission. Cranial computed tomography (CT) revealed a low density area in the left parieto-occipital lobe, which was heterogeneously enhanced by contrast medium. Magnetic resonance imaging (MRI) showed a lesion of low signal intensity in T1-weighted images and high signal intensity in T2-weighted images, which was homogeneously enhanced by Gd DTPA. Cerebral angiography disclosed no abnormality. Two weeks later, follow-up CT showed that the lesion had become larger and markedly enhanced. A brain tumor, probably malignant lymphoma, was suspected. On June 14, stereotaxic biopsy of the enhanced lesion was performed. Histopathological examination disclosed dense infiltration of the entire walls of many small parenchymal vessels, both arterioles and venules, by lymphocytes. Treatment for angiitis (betamethasone 16 mg daily) was started on June 20, then gradually tapered, and the lesion diminished on CT. Our case has some features of both isolated angiitis of the central nervous system and encephalopathy in compensated Hashimoto's disease. It is suggested that some common base of the autoimmune diseases exists in this case.
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PMID:[Isolated angiitis of the CNS associated with Hashimoto's disease]. 161 79

Tuberculosis (TB) is a rare cause of spinal arachnoiditis. It may occur primarily or secondary to intracranial or vertebral infection; unlike other types of arachnoiditis, it frequently involves the spinal cord as well as the meninges and the nerve roots. We retrospectively reviewed 13 conventional myelograms, eight CT myelograms, and five Gd-DTPA-enhanced MR images in 13 patients with spinal TB radiculomyelitis (arachnoiditis). Eleven patients had intracranial TB meningitis at the time of diagnosis or before. Ten patients were less than 30 years old. Conventional myelographic findings included a block of the CSF (11/13), most commonly at the level of the conus medullaris; irregular or indistinct thecal sac contour (9/13); multiple fine and/or coarse nodular defects (8/13); nerve-root thickening (7/13); and vertical bandlike adhesive defects (4/13). CT myelography showed intradural nodular masses suggesting tuberculomas at or just above the level of the block (4/8), irregularity of the spinal cord surface (4/8), irregular filling or obliteration of subarachnoidal space (6/8), and root thickening (5/8). Gd-DTPA-enhanced MR images revealed enhancing nodules suggesting tuberculomas (2/5); enhancement of the dura-arachnoid complex around the cord (3/5); and segmental enhancement of the thoracic cord, suggesting either infarction caused by vasculitis or TB myelitis in association with diffuse cord swelling (1/5). Plain MR findings were much less conspicuous, showing only an indistinct or irregular dura-arachnoid-cord complex (4/5). In conclusion, the conventional myelographic findings are considered to be virtually diagnostic of spinal TB radiculomyelitis in young patients with antecedent or coexisting TB meningitis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Tuberculous arachnoiditis of the spine: findings on myelography, CT, and MR imaging. 251 92

The objective of this work is to report the MRI findings in patients with radiation myelopathy due to accidental local over-irradiation syndrome. Eight patients (seven males and one female) were suffering from over-irradiation syndrome as a result of treatments from a malfunctioning linear electron accelerator. The mean accidental estimated dose was 136 Gy delivered to the "open-neck" (seven cases) and to the thoracic wall (one case), during a mean of 5.4 sessions (range 1-9 sessions). Paresthesia and weakness in the upper extremities were the earliest symptoms (87.5 %), with evolution to paralysis in all patients. No patient is alive (mean survival time 64 days). In all cases MRI was negative for neurologic lesions in the acute phase ( < 90 days from irradiation; Radiation Therapy Oncology Group scoring system). Late signs of radiation myelitis manifested as high-intensity signals on T2-weighted images in three patients, and as Gd-DTPA enhancement of T1-weighted images in one case. Autopsies performed on four patients who died in acute phase showed morphologic alterations in white matter: edema in 75 %, and necrosis and glial reaction as well as obliterative vasculitis in all cases. In cases of over-irradiation, MRI may be normal in acute phase even if the patients have severe neurologic deficit, as positive MRI findings appear only in delayed radiation myelitis.
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PMID:Radiation myelopathy in over-irradiated patients: MR imaging findings. 908 65

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

A 61-year-old woman presented with high fever, headache and left facial palsy with diplopia. Histopathological examination of the biopsied specimens taken from nasal mucosa and kidney revealed a granulomatous angiitis with giant cell infiltration. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed a thickening of dura mater in the middle cranial fossa and tentorium cerebelli. The observed left facial and occulomotor palsy was considered to be caused by pachymeningitis associated with Wegener's granulomatosis (WG). Cyclophosphamide combined with prednisolone effectively improved the symptoms. However, the patient died of acute interstitial pneumonitis, presumably caused by cyclophosphamide. The pathohistology obtained in the autopsy revealed a fibrous thickening of the dura mater in the left meningen with a segmental scarring of the arteries and a necrotizing arteritis in the kidney.
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PMID:An autopsy case of Wegener's granulomatosis with pachymeningitis. 1005 50

We reported a case of X-linked lymphoproliferative syndrome (XLP) with multiple nodular lesions in the brain and lungs. A 21-year-old man was admitted because of one month history of low grade fever, headache, nausea, and amnesia. He developed agammaglobulinemia following Epstein-Barr virus infection at 3-year-old, and thereafter was administered 7.5g of immunoglobulin every 3 weeks with a diagnosis of XLP. Physical examination was unremarkable on admission. Neurological examination revealed disorientation of time, and bilateral gaze-evoked nystagmus. Neuropsychological tests demonstrated impairment of recent memory and calculation. Pleocytosis (83/microl) and increase of protein (1269 mg/dl) and IgG (141 mg/dl) in the CSF were observed. Brain MRI showed multiple nodular lesions with high intense signal on T2-weighted images and Gd-DTPA enhancement on T1-weighted images. Chest CT showed multiple nodular lesions in the bilateral lungs. The needle lung biopsy was performed, which showed infiltration of lymphocytes around the vessels. An immunohistochemical study showed that the infiltrating cells were mainly CD8 positive T lymphocytes. B lymphocyte and plasma cells were not seen. The histological findings excluded intravascular malignant lymphoma and lymphomatoid granulomatosis. Therefore we diagnosed lymphoid vasculitis. The patient developed pancytopenia caused by hemophagocytic syndrome 48 days after admission and was treated with 1 g of methylprednisolone per day for 3 days and a tapered dose of steroid (500 mg to 125 mg of methylprednisolone and 60 mg to 10mg of predonisolone) for 21 days, which resulted in the improvement of clinical features (hemophagocytic syndrome and central nervous system symptoms) and the abnormal CSF findings. The multple nodular lesions in the brain and the lungs shrank 1 month after treatment and disappeared 11 months later. There are few reports concerning lymphoid vasculitis with XLP, and no effective treatment has been described. Our case suggests that steroid therapy may be useful for the treatment of lymphoid vasculitis in XLP.
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PMID:[Case of X-linked lymphoproliferative syndrome (XLP) with multiple nodular lesions in the brain]. 1676 91