Granulomatose mit Polyangiitis zerebrale Manifestationen
Severe
ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review. Magnetic resonance imaging (MRI) scans from the first episode: a Enhanced sagittal T1 sequence: enlarged pituitary fossa with a lesion convex to the suprasellar area. The pituitary stalk was compressed. The size of the lesion was 43*15*17 mm, with a slightly long T1 and long T2 signal mixed with a short T1 signal and a long T2 signal with inhomogeneous enhancement. No obvious bone destruction was detected. b Enhanced coronal T1 sequence: optic chiasm compression with the bilateral cavernous sinus surrounded by the lesion. c Enhanced sagittal T1 sequence: the volume of the lesion was reduced significantly after IV glucocorticoid treatment. d Enhanced coronal T1 sequence: the morphology of the optic chiasm and cavernous sinus returned to normal after IV glucocorticoid administration
Severe
ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review. Magnetic resonance imaging (MRI) scans at recurrence and after biopsy. a Enhanced sagittal T1 sequence: scans after the recurrence of headache in Sept. 2014 showed an enlarged sella with a slightly sunken bottom. The pituitary was enlarged with a height of 1.15 cm and showed a heterogeneous signal, with a patchy, short T1 signal and an abnormal, long T2 signal. The pituitary stalk was shortened and thickened. b Enhanced coronal T1 sequence: a new lesion was detected after recurrence. There was nodular enhancement with abnormal T1 and T2 signals on the right side of the suprasellar region; the lesion was invading the pituitary stalk, infundibulum, right optic nerve, posterior right basal gyrus rectus of the frontal lobe, anterior perforated substance and extending to the internal carotid artery. c and d Enhanced T2 sequence: cerebral parenchymal edema was detected around the lesion. e Enhanced sagittal T1 sequence: changes were observed in the pituitary after the biopsy and i.v. administration of glucocorticoids. f Enhanced coronal T1 sequence: after the biopsy and i.v. administration of glucocorticoids, the parenchymal edema was significantly reduced
Severe
localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. Coronal contrast-enhanced CT image of the skull base and neck (pre-cyclophosphamide treatment). There is marked abnormal chronic inflammatory infiltration of the cavernous sinuses bilaterally (bold white arrow) and hypophyseal involvement (black arrow). Multiple lower cranial nerve palsies (Collet-Sicard syndrome) as a result of the skull base inflammatory involvement were evident on imaging, including right vocal cord palsy indicated by adducted right true vocal cord and enlarged right laryngeal ventricle (long white arrow)
Severe
localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. Coronal T2-weighted MRI image of the skull base and neck (pre-cyclophosphamide treatment). There is severe chronic inflammatory soft tissue abnormality affecting the cavernous sinuses and central and lateral skull base, including the left cavernous sinus and right sphenoid bone and foramen rotundum (large black arrows). Secondary cranial nerve palsies are present. Palsy of the mandibular division of the right trigeminal nerve has resulted in atrophy of the right masticator space muscle, in comparison to the more normal muscle volume on the left (short white arrows). Lower cranial nerve palsies are demonstrated by atrophy of the right superior pharyngeal constrictor muscle (short black arrow) and sternocleidomastoid muscles (long white arrows)
Severe
localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. MR images of the brainstem, skull base and pituitary gland. (a) Coronal contrast-enhanced fat-saturated T1-weighted image of the central skull base demonstrates pathological enhancement of the cavernous sinus, pituitary gland and pituitary stalk (arrow). The abnormalities are non-discrete and radiologically inconsistent with a microadenoma or invasive macro adenoma of the pituitary gland. (b) Dedicated thin-section coronal contrast-enhanced T1-weighted image of the pituitary demonstrates the abnormal enhancement and thickening of the pituitary stalk as well as the cavernous sinus and pituitary parenchyma in more detail. (c) Dedicated thin-section sagittal contrast-enhanced T1-weighted image of the pituitary demonstrates the abnormal generalised thickening and enhancement of the pituitary stalk (arrow) consistent with granulomatous infundibulitis
Severe
localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. Axial intracranial contrast-enhanced T1-weighted MR image after six pulses of cyclophosphamide. There is reduction in the volume of abnormal cavernous sinus enhancing chronic inflammatory tissue (arrows) compared with pre-treatment imaging
Dural masses:
meningiomas and their mimics. Granulomatosis with polyangiitis of the skull base. a, b Post-contrast T1-weighted axial and coronal MR images showing enhancing inflammatory soft tissue involving the skull base and cavernous sinuses. There is also inflammatory change within the adjacent sphenoid sinus. c T2-weighted axial image of the same lesion at the level of the cavernous sinuses. d Post-contrast axial CT image again showing avid enhancement within durally based abnormal soft tissue
Granulomatosis
with polyangiitis • Granulomatosis with polyangitis - Ganzer Fall bei Radiopaedia
CNS manifestations of granulomatosis with polyangiitis (GPA) are rare.
For a general discussion of the condition, please refer to the main article on granulomatosis with polyangiitis (GPA). For other organ-specific radiographic features, please refer to individual articles:
- granulomatosis with polyangiitis: pulmonary manifestations
- granulomatosis with polyangiitis: renal manifestations
- granulomatosis with polyangiitis: upper respiratory tract manifestations
- granulomatosis with polyangiitis: orbital manifestations
Granulomatosis with polyangiitis (previously known as Wegener granulomatosis), is a multi-system systemic necrotizing non-caseating granulomatous vasculitis affecting small to medium-sized arteries, capillaries and veins.
Epidemiology
CNS manifestations of GPA occur only in ~5% (range 2-9%) of the patients .
Pathology
GPA manifests in the CNS as :
- cerebral or meningeal granulomatous lesions (hypertrophic pachymeningitis )
- small vessel CNS vasculitis causing infarcts and arterial occlusion
- intracranial hemorrhage
- continuous invasion of an extracranial granuloma
Siehe auch:
Assoziationen und Differentialdiagnosen zu Granulomatose mit Polyangiitis zerebrale Manifestationen: