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

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-ophthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.
 ...
PMID:Wall-eyed bilateral internuclear ophthalmoplegia (Webino syndrome) and myelopathy in pyoderma gangrenosum. 209 99

Two human retroviruses, HIV-1 and HTLV-I, have been associated with myelopathies in addition to other neurologic disorders. We report an American dually infected with HIV-1 and HTLV-I who developed steroid-responsive myeloneuropathy. This 28-year-old bisexual man developed interstitial pneumonitis and a transient midthoracic sensory level followed by the evolution of a slowly progressive spastic paraparesis and sensorimotor neuropathy. Serologic studies demonstrated coinfection with both HIV-1 and HTLV-I. Peripheral blood absolute CD4 count was persistently within the normal range. Cranial MRI was normal and spinal MRI showed T3-T10 atrophy. Serial CSF analyses demonstrated marked intrathecal synthesis of anti-HTLV-I IgG, lymphocytic pleocytosis, elevated protein and immunoglobulin G, and oligoclonal bands. HIV-1 was isolated from CSF but not from peripheral nerve. Lymphoproliferative studies confirmed spontaneous proliferation in both blood and CSF. Soluble interleukin 2 receptor and soluble CD8 were greatly elevated in blood and CSF when compared with patients with HIV-related vacuolar myelopathy and seronegative patients with other causes of myelopathy. Nerve biopsy showed epi- and endoneurial CD8+ lymphocytic infiltration without vasculitis; muscle biopsy showed features of acute and chronic denervation. A 6-week course of prednisone produced sustained improvement in leg strength and walking times. We speculate that the myeloneuropathy was caused by HTLV-I in the setting of coinfection with HIV-1.
 ...
PMID:Steroid-responsive myeloneuropathy in a man dually infected with HIV-1 and HTLV-I. 216 Oct 92

Granulomatous angiitis is a frequently fatal central nervous system vasculitis of unknown pathogenesis sporadically associated with Hodgkin's disease, mixed cellularity type. We describe a 58-year-old woman presenting with headaches, progressive spastic paraparesis, equivocal computed tomography findings, and magnetic resonance imaging findings of increased signal intensity biparietally on T2-weighted imaging in a relatively discrete pattern. Magnetic resonance imaging-directed serial stereotactic biopsies revealed granulomatous angiitis throughout the right parieto-occipital region and splenium. Simultaneous biopsy of an enlarged submandibular mass revealed Hodgkin's disease, lymphocyte-predominant type. This case shows that granulomatous angiitis may be associated with magnetic resonance imaging findings suggesting a mass lesion in lymphocyte-predominant as well as mixed cellularity Hodgkin's disease.
 ...
PMID:Granulomatous angiitis masquerading as a mass lesion. Magnetic resonance imaging and stereotactic biopsy findings in a patient with occult Hodgkin's disease. 264 73

A patient with CNS vasculitis limited to small vessels is described. Initial spinal cord involvement with paraparesis was followed by multifocal cerebral and cerebellar involvement, depicted by magnetic resonance imaging (MRI). She also had recurrent cerebral bleeding. A diagnosis of vasculitis was made by cerebral biopsy. Immunosuppressive therapy resulted in clinical improvement and resolution of the MRI abnormalities.
 ...
PMID:An unusual case of central nervous system vasculitis. 361 7

To define the pathophysiology of spinal cord dysfunction associated with spinal epidural abscess formation, we developed an experimental model. Spinal epidural abscesses were produced in rabbits by injecting Staphylococcus aureus into the posterior thoracolumbar epidural space under direct vision. Progressive neurological deficits were detected in 18 of 20 animals; severe paraparesis or paraplegia occurred in 75%, and sphincter dysfunction occurred in 55%. Clinical data, including the results of plain spine roentgenography, myelography, and biochemical and bacteriological examination of the cerebrospinal fluid, were recorded. Epidural abscesses with varying degrees of spinal cord compression were confirmed pathologically in 95% of the experimental group. Spinal cord white matter changes included vacuolization, loss of myelin, and axonal swelling. The gray matter of the spinal cords was relatively preserved. There was no microscopic evidence of thrombosis or vasculitis in the major blood vessels supplying the spinal cords. Histopathological changes detected in the spinal cords were more consistent with direct compression of neural tissue than with infarction. The progressive clinical course and the histopathological changes in the spinal cord after compression by abscess closely resembled those of experimental compression of the spinal cord by epidural neoplasm.
 ...
PMID:Experimental spinal epidural abscess: a pathophysiological model in the rabbit. 361 65

To date, 271 cases of HTLV1-associated paraplegia have been observed in Martinique (French West Indies). The clinical picture consisted mostly in a spastic paraparesis or paraplegia with sphincter disturbances (80%) and lower limbs pains (60%). The severity of the disease appeared variable: after a mean disease duration of 6.5 years, 40% of the patients could walk without help, 35% used a single crutch, and 25% used a couple of crutches or were confined to a wheelchair. A variable neuromuscular component was observed in 70 cases (25.4%). In 38 cases, the peripheral signs (SIGNS) or the myositis were only mild. In contrast, 25 patients presented with severe amyotrophy evoking amyotrophic lateral sclerosis, and 7 other had features of dermatopolymyositis. Lastly, an extra-neural spreading of the disease was extremely frequent, including lymphocytic alveolitis (76%), sicca syndrome (69%) and more rarely uveitis, arthritis or vasculitis.
 ...
PMID:[Paraplegia associated with HTLV 1 in Martinique. Study of 271 cases including 70 with neuromuscular involvement]. 781 96

Over a period of 25 years, a 67-year-old man again and again developed purpura over the lower legs. When hospitalized because of splenomegaly with thrombocytopenia the diagnosis of idiopathic thrombocytopenic purpura was made. Four years later, after an infection of the upper respiratory tract, purpura again developed, together with a symmetrical sensory-motor polyneuropathy with flaccid paraparesis. Biopsy of the N. suralis revealed a florid leukocytoclastic vasculitis. Morphologically there was severe nerve damage with Wallerian degeneration and subtotal nerve fibre loss. Immunological tests demonstrated essential mixed cryoglobulinaemia of IgG/IgM type. Cryoprecipitates must be considered as the cause of the vasculitis, which in turn produced the nerve lesions and purpura. This case underlines the importance of biopsy diagnosis of peripheral nerve and skeletal muscle, because it may demonstrate a treatable cause of a severe acute neuromyopathy.
 ...
PMID:[Mixed cryoglobulinemia and vasculitic neuromyopathy]. 851 23

We describe a 66-year-old woman with long-standing HTLV-I-associated myelopathy who developed multiple parenchymal hemorrhages and whose angiogram suggested cerebral vasculitis. After cyclophosphamide and glucocorticoid therapy, both her acute deficits and chronic paraparesis improved. HTLV-I may be an etiologic agent in isolated angiitis of CNS.
 ...
PMID:Multiple cerebral hemorrhages in HTLV-I-associated myelopathy. 843 10

A 36-year-old female was admitted with leptomeningeal melanoma associated with straight sinus thrombosis manifesting as headache and vomiting. Computed tomography and magnetic resonance imaging showed the subarachnoid space was diffusely enhanced. Her consciousness rapidly deteriorated to a coma. Angiography demonstrated straight sinus thrombosis. Thrombolysis by superselective catheterization and infusion of urokinase was successfully performed. She recovered consciousness, but developed paraparesis 2 weeks later. Malignant melanoma with meningeal dissemination was diagnosed by an open biopsy of the lumbar lesion. Angiitis induced by the infiltration of tumor cells and activation of the blood coagulation cascade was probably the causative mechanism of the sinus thrombosis.
 ...
PMID:Leptomeningeal melanoma associated with straight sinus thrombosis--case report. 936 36

A 48-year-old man presented with progressive spastic paraparesis and diffuse white matter involvement on neuroimaging that suggested a primary demyelinating disease. Brain biopsy 3 years after onset of symptoms demonstrated idiopathic granulomatous angiitis. In patients with MRI features of diffuse white matter disease of uncertain etiology, open brain biopsy of leptomeninges and parenchyma should be considered to exclude idiopathic granulomatous angiitis of the CNS.
 ...
PMID:Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease. 985 57


1 2 Next >>