24岁男性头痛---病例示范
24岁男性头痛---病例示范
CLINICAL HISTORY-----临床病史
A 24-year-old Caucasian man was admitted following a 10-day history of severe headache leading to collapse on the street. On presentation, he was confused and agitated with left-sided weakness and a positive left Babinski sign. His medical history was significant for asthma and non-Hodgkin's lymphoma 10 years previously treated with chemotherapy and radiation therapy with no recurrence.
Routine bloodwork, hypercoagulability and autoimmune workups including anti-neutrophil cytoplasmic antibodies were all normal. Serology was negative for Arbovirus, Bartonella, Cryptococcus and the hepatitides. Consent for HIV testing was not obtained. Blood, cerebrospinal fluid and urine cultures were all negative. Blood and urine toxicological screening was unremarkable.
CT perfusion suggested ischemia in both middle cerebral artery (MCA) and left anterior cerebral artery (ACA) territories and infarction in the left posterior cerebral artery (PCA) territory. CT angiography showed filling defects in these territories as well (Figures 1, 2, 3). Treatment with intravenous tissue plasminogen activator (TPA) and intra-arterial thrombolysis was attempted. Initial anteroposterior angiogram confirmed occlusion of the right distal M1 MCA and left A2 ACA (Figure 4). Mechanical thrombolysis and 4mg of intra-arterial TPA were unsuccessful in reopening the right MCA. The patient deteriorated and was declared brain dead 31 hours after arrival to hospital.
PATHOLOGY-------病理
On post-mortem examination, the fresh brain weighed 1560g and showed oedema with bilateral transtentorial herniation and cerebellar protrusion. Coronal sections showed thromboembolic occlusion of both ACAs beyond their A2 segments and the left MCA from its origin to its bifurcation (Figure 5). All posterior fossa vessels were normal. Both common carotid and internal carotid arteries were patent, but the right external carotid artery appeared thickened with a reduced lumen. Gross examination of the remainder of the body was unremarkable.
HPS-stained permanent sections of the M1 and M2 segments of both MCAs, both ACAs beyond the anterior communicating artery and the left P2 PCA demonstrated a circumferential arteritic process composed of multi-nucleated giant cells, lymphocytes and histiocytes concentrated in the intima with fragmentation of the elastica interna (representative section; Figure 6). Elastica interna fragmentation by invading giant cells was highlighted by Elastic Van Gieson staining (Figure 7). CD68 staining revealed a sandwich-like pattern of immunoreactive cells in the intima and adventitia with relative sparing of the media (Figure 8). A CD4/CD8-positive lymphocytic and mononuclear cell infiltrate was present in the adventitia (CD4, Figure 9; CD8, Figure 10). Recent thrombosis in affected vessels caused varying degrees of corresponding neuronal ischemic change. There was no evidence of fibrinoid necrosis or previous healing lesions, but a small focus of non-fibrinoid necrosis was present. Sizes of vessels affected ranged from 2.0-3.5mm in diameter. Smaller vessels on the brain surface and the vertebrobasilar arteries were unaffected. Congo red, β-amyloid, Ziehl-Neelsen and PAS-D stains and in-situ hybridization for varicella-zoster virus were negative. Extracranially, patchy, non-thrombotic areas of a similar arteritic process involving giant cells were noted in both carotid siphons (HPS; Figure 11) and the initial segment of one renal artery (H&E; Figure 12). No other arteries or organs examined were involved.
DISCUSSION--------讨论
GCA is extremely rare in young patients, such that onset above age 49 is one of its American College of Rheumatology (ACR) diagnostic criteria. Due to the patient's atypical presentation, GCA was not suspected and an erythrocyte sedimentation rate and temporal artery sampling were not performed. Although GCA classically involves the temporal artery, it can involve other intracranial and extracranial arteries. The intracranial arteries most commonly affected are those of the vertebrobasilar system, but no histologically-confirmed cases of GCA involving the cerebral arteries have been reported. A rare form of GCA known as disseminated visceral giant cell arteritis (DVGCA) has been reported to involve exclusively the extracranial organs (4), but concurrent intracranial and extracranial involvement in GCA has never been reported in patients under age 50. The few reports describing concurrent involvement were all in older patients with intracranial involvement limited to the vertebrobasilar system (7, 9) or the anterior inferior cerebellar artery (6). In fact, intracranial GCA in patients under age 50 is a rare occurrence in itself; to our knowledge, only two such cases with histological features similar to ours have been described, but both were associated with infectious aetiologies (1, 2). Most of the cases of intracranial GCA, DVGCA and GCA with concurrent involvement (including our case) share the textbook histology of classic GCA. Takayasu arteritis (TA), which can resemble GCA histologically, was also considered since one of its ACR diagnostic criteria is onset below age 40. However, the patient did not demonstrate history or physical examination findings fulfilling other TA criteria and the aorta was uninvolved. Moreover, direct involvement of intracranial arteries by TA is extremely rare, with only a few reports to date (5, 10).
Granulomatous vasculitis of the CNS can be classified as primary angiitis of the CNS (PACNS) or secondary to systemic causes. By definition, PACNS is confined to the CNS and can only be established after ruling out secondary causes. It has been described to occur at any age, is male-predominant and lacks systemic symptoms (3). Histologically, small and medium leptomeningeal and cortical arteries are involved by a necrotizing granulomatous vasculitis. PACNS can also be indistinguishable from amyloid peptide-related angiitis without the appropriate immunohistochemical workup (8). The dissimilar microscopic picture, presence of extracranial arteritis and negative -amyloid staining rule out both diagnoses in our case. A secondary CNS vasculitis was also considered unlikely in our case as extensive workup and past medical history did not reveal any infection, systemic vasculitides, sarcoidosis or drugs.
We report the first occurrence of disseminated GCA with extracranial and anterior, middle and posterior cerebral arterial involvement causing fatal cerebral infarction in an atypical age group.
REFERENCES---------文献引用
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Sendino A, Barbado FJ, Gonzalez-Anglada I, Anton E, Lopez-Barea F, Vazquez JJ (1992) Temporal arteritis: a form of systemic panarteritis. Ann Rheum Dis.51(9):1082-4.
Sikaroodi H, Motamedi M, Kahnooji H, Gholamrezanezhad A, Yousefi N (2007) Stroke as the first manifestation of Takayasu arteritis. Acta Neurol Belg.107(1):18-21.
Contributed by Zhongchuan Will Chen, MDCM, Sean P Symons, MD, Beverley Young, RTC, Juan M. Bilbao, MD
3月-11-10 04:03:38
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