|
| Author | Age | Clinical presentation | Cerebral biopsy | MRI and vascular studies | Serology | Treatment | Outcome |
|
|
Nomoto et al. [3] | 18/F | Diagnosed with UC at age 15, presented with headache, transient confusion | No | MRA: diffuse narrowing
cerebral arteries with multiple segmental stenoses; common carotid and subclavian artery narrowing with irregularities | MPO-ANCA, PR3-ANCA within normal range | Prednisone | Resolution of neurological deficits |
|
| Pandian et al. [4] | 35/F | Unknown duration UC, presented with right side weakness, unsteady gait | No | Restricted diffusion in left ACA
territory; angiogram shows intracranial vessels with multiple areas stenosis and dilatation | Not reported | None | Not available |
|
| Nelson et al. [5] | 18/M | One month after diagnosis UC presented with generalized tonic clonic seizures and became comatose | Yes; acute
necrotizing vasculitis involving meningeal and cortical blood vessels with affected vessels showing fibrinoid necrosis with acute inflammatory cell infiltration | CT with multiple bilateral cerebral low density areas enhancing with contrast | ANA
negative | Prednisone, cyclophosphamide | Resolution of neurological deficits |
|
| Panani et al. [6] | 51/M | Eight-year diagnosis of UC presented with febrile illness, rash, acute deterioration | No; skin punch biopsy-lesions on the small vessels suggesting a possible systemic disease | CT head-ischemic lesions in white matter | p-ANCA MPO positive, anticardiolipin elevated | Prednisone, cyclophosphamide | Resolution of neurological deficits |
|
| Nemoto et al. [7] | 69/F | Sensorineural deafness, ptosis, peripheral facial palsies, hyperreflexia all ext. and later diagnosed with UC | No | T2 hyperintensities in midbrain, pons, bilateral cerebral white matter; no vascular study | ANA
negative | Corticosteroids | Improved but still had deafness and
worsening cerebral deep white matter lesions without any new clinical signs |
|
| Druschky et al. [8] | 37/M | Eight-year history of UC, weakness right arm, slurred speech, rapidly developing confusion | Not brain (upper arm skin biopsy showed perivascular infiltration with inflammation) | T2 hyperintensities periventricular and cerebellar, spinal cord; no vascular study | ANA, c-ANCA, p-ANCA negative | Corticosteroids, azathioprine, and cyclosporine A | Complete resolution |
|
| Dejaco et al. [9] | 58/M | Diagnosed with UC at age of 29, hemiparesthesia of face and left and right side body intermittently | No | T2 hyperintensities of centrum semiovale (reported as typical of microangiopathy associated with vasculitis) | c-ANCA, p-ANCA negative | Prednisolone, ASA | Complete recovery |
|
| Masaki et al. [10] | 19/F | Within 2 weeks presentation of bloody diarrhea developed generalized convulsive seizures and AMS; dysarthria, numbness of tongue and extremities | No | T2 and FLAIR multiple
hyperintensities in the corticomedullary; enhancing
lesions; cerebral angiogram: faint staining in parietooccipital area | c-ANCA, p-ANCA negative | Prednisolone, dextran, and colon resection | Complete
recovery |
|
| Bonrath et al. [11] | 72/M
61/F | Number 1 acute UC flare with AMS;
number 2 active UC with acute left hemiparesis | One brain biopsy showed postischemic changes, inconclusive | Number 1 MRI brain showed multiple perivascular signal changes and infarcts; number 2 MRI also consistent with vasculitis | p-ANCA, c-ANCA, MPO, and Pr3 negative in both cases | | |
|
| Carmona et al. [12] | 47/M | Developed UC 7 years prior, presented with right motor hemiparesis and aphasia | Yes (autopsy), small and medium size vessel showed necrotizing angiitis | CT head showed low attenuation in left parietal and occipital regions | Not reported | Decadron, mannitol | Death |
|
| Glotzer et al. [13] | 18/M | Diagnosed with UC 10 months prior, presented with left hemiparesis, hemianopia, AMS | Yes; necrotic mostly white
matter with polymorphonuclear lymphocytes perivascularly | Carotid angiogram showing displacement of ACA, MCA with parietooccipital mass | Not reported | Erythromycin,
clarithromycin, and amphotericin B | Complete neurological
recovery |
|
| Edwards [14] | 28/M | 2 months after diagnosis UC presented with right arm weakness, right facial paresis, GTCS | No | Bilateral carotid angiogram showing mulivessel segmental narrowing in small and medium arteries | ANA
negative | Dexamethasone | Residual left hemiparesis |
|
| Friol-Verceletto et al. [15] | 45/F | 14-year diagnosis of UC with spastic hemiparesis | No | Abnormal angiography | ESR
elevated | Not described | Not described |
|
| Karacostas et al. [16] | 32/F | At time of diagnosis UC developed left hemiparesis, AMS, generalized seizures | No | CT head number 1 right frontal pole
hypodensity; number 2 showed hemorrhagic transformation of the ischemic infarct and white matter edema; right side carotid angiogram reported as patent vessels | ANA and lupus anticoagulant negative | Prednisone | Significant neurological |
|
