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Vasculitis, Cerebral; Surgery
Vasculitis, Cerebral; Surgery TD Uschold and SW Chang, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, AZ, USA r 2014 Elsevier Inc. All rights reserved. This article is a revision of the previous edition article by David Sinclair, Ge´rard Mohr, volume 4, pp 639–641, r 2003, Elsevier Inc.
Classification and Clinical Findings The term central nervous system (CNS) vasculitis refers to inflammation of blood vessel walls (typically small to medium-sized arterioles) found in the pachymeninges (dura), leptomeninges (pia and arachnoid), cranial nerves, and/or the brain and spinal cord parenchyma. Primary CNS vasculitis is distinguished from secondary forms by the absence of systemic involvement or a known inciting cause. Table 1 is a useful conceptual framework for classifying the vasculitic entities of the CNS. Histological findings are widely disparate and have been reported to include immune complex deposition with resultant fibrinoid necrosis, lymphocytic infiltration, and/or granulomatous inflammation. In clinical and surgical practice, however, significant overlap in terms of presentation, neurological course, and treatment strategies often obscures the diagnosis at presentation. Primary CNS vasculitis describes a heterogeneous patient population along a spectrum of consistent but rarely perfectly stereotyped clinical and histological findings. For example, histologically and angiographically defined cases of primary CNS vasculitis appear to differ in terms of clinical presentation. Male gender, older to middle age, headache at presentation, diffuse neurological dysfunction, chronic or indolent course, and abnormal cerebrospinal fluid (CSF) findings predominate in histologically defined cases compared to angiographically defined cases. These differences may reflect alternative pathophysiological mechanisms between the two subpopulations as well as a predilection for more distal vasculature within the histologically defined group. Numerous designations have been described to further subcategorize primary CNS vasculitis. In the authors’ opinion, however, they tend to be too broad or insufficiently inclusive to merit routine clinical application. Clinical symptoms of both primary and secondary forms are varied and may include generalized malaise and fever (especially secondary vasculitides), headache, focal neurological dysfunction (with or without evidence of ischemia), encephalopathy, seizure, myeloradiculopathy, or coma. Clinical progression may be subacute (weeks to months) or rapid. Unexplained or atypical ischemic infarction may result from lumen occlusion, thrombus formation, or both. Alternatively, intraparenchymal or subarachnoid hemorrhage may result from similar processes and/or fibrinoid necrosis of the arterial wall. Typically, middle-aged to older females are affected. Childhood forms of systemic and CNS vasculitis have also been described. The primary evaluation relies on detailed systemic physical examination, history, comprehensive serological testing, CSF findings, and magnetic resonance imaging (MRI). Serological testing includes, but is not limited to, a complete blood count, erythrocyte sedimentation rate, C-reactive protein, Venereal
616
Disease Research Laboratory test, rapid plasma reagin, perinuclear antineutrophilic cytoplasmic antibody (p-ANCA), cytoplasmic-ANCA (c-ANCA), complement fixation, antinuclear antibody, and rheumatoid factor. CSF and imaging findings tend to be nonspecific, and most are predictive only in conjunction with history and serum testing. These largely nonspecific clinical and diagnostic findings merit a broad differential diagnosis, including atherosclerotic disease, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, myoclonic epilepsy and ragged-red fiber disease, demyelinating disease, posterior reversible encephalopathy syndrome, cerebral amyloid angiopathy (often distinguished by characteristic predilection for cortical hemorrhages among the aged), radiation injury, hematologic/coagulopathic disorders, moyamoya disease, and reversible cerebral vasoconstrictive syndromes such as vasospasm (e.g., associated with subarachnoid hemorrhage, eclampsia, or cocaine use). Diagnostic tissue or characteristic angiographic findings are requisite for the formal diagnosis of primary CNS vasculitis. A neurosurgical or endovascular intervention is most commonly requested as a definitive (in the case of histology) or supportive (in the case of angiography) test in this context. A catheter-based digital subtraction angiography (DSA) may reveal thrombosis, segmental and irregular changes in vessel caliber, lack of a distal vascular blush related to smaller-caliber vessel disease, and arterial or venous stasis. The anterior circulation is most often affected. Extensive collateralization should raise the suspicion of a more chronic pathology such as moyamoya disease. DSA may be normal in 20–30% of otherwise confirmed cases of primary CNS vasculitis. Brain and leptomeningeal biopsy (both required) are commonly needed in cases of diffuse disease, when angiography is equivocal, and when no other site is deemed suitable for biopsy (e.g., lung, skin, and liver). CNS biopsy can be associated with a false-negative outcome or can be a nondiagnostic in as many as 25% of cases. Considerable controversy remains over the ideal site for parenchymal biopsy. Our preference remains to sample the most accessible region of noneloquent cortex likely affected based on preoperative MRI (when feasible). For cases of suspected temporal arteritis, temporal artery biopsy is reported to have a sensitivity of approximately 90%. Negative results may be avoided by harvesting long arterial segments (to avoid sampling error related to skipped lesions). A biopsy of the contralateral temporal artery may also improve predictive value. For both primary CNS vasculitis and temporal arteritis, prompt biopsy is important. Early biopsy before initiation of high-dose steroid treatment or immediately thereafter is likely to improve diagnostic yield. In either case, however, withholding treatment for urgent cases awaiting biopsy or for selected cases of biopsy-negative findings despite a high clinical suspicion is rarely advocated.
Encyclopedia of the Neurological Sciences, Volume 4
doi:10.1016/B978-0-12-385157-4.00798-3
Vasculitis, Cerebral; Surgery
Table 1
Classification for cerebral vasculitidesa
Infectious cerebral vasculitis Bacterial Borrelia Haemophilus influenzae Neisseria meningitidis Streptococcus pneumoniae Group A b-hemolytic streptococcus (rheumatic fever) Tuberculosis Syphilis Viral Herpes simplex ophthalmicus Human immunodeficiency virus Cytomegalovirus Papovavirus Rickettsial Protozoan Toxoplasma Fungal Cryptococcosis Candidiasis Mucormycosis Aspergillosis Parasitic Cysticercosis Inflammatory cerebral vasculitis Necrotizing Polyarteritis nodosa Systemic lupus erythematosus Radiation arteritis Polyangiitis overlap syndrome Rheumatoid arthritis/juvenile rheumatoid arthritis Sjo¨gren’s vasculitis Granulomatous Neurosarcoidosis Giant cell arteritis Temporal arteritis Takayasu’s arteritis Lymphomatoid granulomatosis Wegner’s granulomatosis Primary central nervous system vasculitis Scleroderma Lymphocytic Allergic granulomatous angiitis Behcet’s syndrome Retinocochleocerebral vasculopathy Sneddon’s syndrome Degos disease Hypersensitivity/allergic vasculitis Drug-abuse arteritis Tumor-related vasculitis (dermatomyositis and polymyositis) Serum sickness Henoch–Schonlein purpura a
Important childhood vasculitides in bold. Source: Reproduced from Sinclair D and Mohr G Vasculitis, cerebral, surgery. In: Aminoff MJ and Daroff RB (eds.) Encyclopedia of Neurological Sciences, vol. 4, p. 640. Oxford: Elsevier.
617
See also: Cerebral Vasculitis. Endocannabinoids. Gilles de la Tourette’s Syndrome. Membrane Potential. Revascularization, Cerebral. Vasculitic Neuropathy
Further Reading Alhalabi M and Moore PM (1994) Serial angiography in isolated angiitis of the central nervous system. Neurology 44: 1221–1226. Alrawi A, Trobe JD, Blaivas M, and Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53: 858–860. Calabrese LH and Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 67: 20–39. Cellucci T and Benseler SM (2010) Central nervous system vasculitis in children. Current Opinion in Rheumatology 22: 590–597. Cohen BA and Biller J (1992) Hemorrhagic stroke due to cerebral vasculitis and the role of immunosuppressive therapy. Neurosurgery Clinics of North America 3: 611–624. Hellman DB and Stone JH (2002) Vasculitis of the central nervous system. In: Asbury AK, McKhann GM, McDonald WI, Goadbsy PJ, and McArthur JC (eds.) Diseases of the Nervous System: Clinical Neuroscience and Therapeutic Principles, 3rd edn., pp. 1547–1557. Cambridge, UK: Cambridge University Press. Hurst RW and Grossman RI (1994) Neuroradiology of central nervous system vasculitis. Seminars in Neurology 14: 320–340. McDonnell PJ, Moore GW, Miller NR, Hutchins GM, and Green WR (1986) Temporal arteritis. A clinicopathologic study. Ophthalmology 93: 518–530. Moore PM and Cupps TR (1983) Neurological complications of vasculitis. Annals of Neurology 14: 155–167. Sole-Llenas J and Pons-Tortella E (1978) Cerebral angiitis. Neuroradiology 15: 1–11. Woolfenden AR, Tong DC, Marks MP, Ali AO, and Albers GW (1998) Angiographically defined primary angiitis of the CNS: Is it really benign? Neurology 51: 183–188.
Classification and Clinical Findings The term central nervous system (CNS) vasculitis refers to inflammation of blood vessel walls (typically small to medium-sized arterioles) found in the pachymeninges (dura), leptomeninges (pia and arachnoid), cranial nerves, and/or the brain and spinal cord parenchyma. Primary CNS vasculitis is distinguished from secondary forms by the absence of systemic involvement or a known inciting cause. Table 1 is a useful conceptual framework for classifying the vasculitic entities of the CNS. Histological findings are widely disparate and have been reported to include immune complex deposition with resultant fibrinoid necrosis, lymphocytic infiltration, and/or granulomatous inflammation. In clinical and surgical practice, however, significant overlap in terms of presentation, neurological course, and treatment strategies often obscures the diagnosis at presentation. Primary CNS vasculitis describes a heterogeneous patient population along a spectrum of consistent but rarely perfectly stereotyped clinical and histological findings. For example, histologically and angiographically defined cases of primary CNS vasculitis appear to differ in terms of clinical presentation. Male gender, older to middle age, headache at presentation, diffuse neurological dysfunction, chronic or indolent course, and abnormal cerebrospinal fluid (CSF) findings predominate in histologically defined cases compared to angiographically defined cases. These differences may reflect alternative pathophysiological mechanisms between the two subpopulations as well as a predilection for more distal vasculature within the histologically defined group. Numerous designations have been described to further subcategorize primary CNS vasculitis. In the authors’ opinion, however, they tend to be too broad or insufficiently inclusive to merit routine clinical application. Clinical symptoms of both primary and secondary forms are varied and may include generalized malaise and fever (especially secondary vasculitides), headache, focal neurological dysfunction (with or without evidence of ischemia), encephalopathy, seizure, myeloradiculopathy, or coma. Clinical progression may be subacute (weeks to months) or rapid. Unexplained or atypical ischemic infarction may result from lumen occlusion, thrombus formation, or both. Alternatively, intraparenchymal or subarachnoid hemorrhage may result from similar processes and/or fibrinoid necrosis of the arterial wall. Typically, middle-aged to older females are affected. Childhood forms of systemic and CNS vasculitis have also been described. The primary evaluation relies on detailed systemic physical examination, history, comprehensive serological testing, CSF findings, and magnetic resonance imaging (MRI). Serological testing includes, but is not limited to, a complete blood count, erythrocyte sedimentation rate, C-reactive protein, Venereal
616
Disease Research Laboratory test, rapid plasma reagin, perinuclear antineutrophilic cytoplasmic antibody (p-ANCA), cytoplasmic-ANCA (c-ANCA), complement fixation, antinuclear antibody, and rheumatoid factor. CSF and imaging findings tend to be nonspecific, and most are predictive only in conjunction with history and serum testing. These largely nonspecific clinical and diagnostic findings merit a broad differential diagnosis, including atherosclerotic disease, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, myoclonic epilepsy and ragged-red fiber disease, demyelinating disease, posterior reversible encephalopathy syndrome, cerebral amyloid angiopathy (often distinguished by characteristic predilection for cortical hemorrhages among the aged), radiation injury, hematologic/coagulopathic disorders, moyamoya disease, and reversible cerebral vasoconstrictive syndromes such as vasospasm (e.g., associated with subarachnoid hemorrhage, eclampsia, or cocaine use). Diagnostic tissue or characteristic angiographic findings are requisite for the formal diagnosis of primary CNS vasculitis. A neurosurgical or endovascular intervention is most commonly requested as a definitive (in the case of histology) or supportive (in the case of angiography) test in this context. A catheter-based digital subtraction angiography (DSA) may reveal thrombosis, segmental and irregular changes in vessel caliber, lack of a distal vascular blush related to smaller-caliber vessel disease, and arterial or venous stasis. The anterior circulation is most often affected. Extensive collateralization should raise the suspicion of a more chronic pathology such as moyamoya disease. DSA may be normal in 20–30% of otherwise confirmed cases of primary CNS vasculitis. Brain and leptomeningeal biopsy (both required) are commonly needed in cases of diffuse disease, when angiography is equivocal, and when no other site is deemed suitable for biopsy (e.g., lung, skin, and liver). CNS biopsy can be associated with a false-negative outcome or can be a nondiagnostic in as many as 25% of cases. Considerable controversy remains over the ideal site for parenchymal biopsy. Our preference remains to sample the most accessible region of noneloquent cortex likely affected based on preoperative MRI (when feasible). For cases of suspected temporal arteritis, temporal artery biopsy is reported to have a sensitivity of approximately 90%. Negative results may be avoided by harvesting long arterial segments (to avoid sampling error related to skipped lesions). A biopsy of the contralateral temporal artery may also improve predictive value. For both primary CNS vasculitis and temporal arteritis, prompt biopsy is important. Early biopsy before initiation of high-dose steroid treatment or immediately thereafter is likely to improve diagnostic yield. In either case, however, withholding treatment for urgent cases awaiting biopsy or for selected cases of biopsy-negative findings despite a high clinical suspicion is rarely advocated.
Encyclopedia of the Neurological Sciences, Volume 4
doi:10.1016/B978-0-12-385157-4.00798-3
Vasculitis, Cerebral; Surgery
Table 1
Classification for cerebral vasculitidesa
Infectious cerebral vasculitis Bacterial Borrelia Haemophilus influenzae Neisseria meningitidis Streptococcus pneumoniae Group A b-hemolytic streptococcus (rheumatic fever) Tuberculosis Syphilis Viral Herpes simplex ophthalmicus Human immunodeficiency virus Cytomegalovirus Papovavirus Rickettsial Protozoan Toxoplasma Fungal Cryptococcosis Candidiasis Mucormycosis Aspergillosis Parasitic Cysticercosis Inflammatory cerebral vasculitis Necrotizing Polyarteritis nodosa Systemic lupus erythematosus Radiation arteritis Polyangiitis overlap syndrome Rheumatoid arthritis/juvenile rheumatoid arthritis Sjo¨gren’s vasculitis Granulomatous Neurosarcoidosis Giant cell arteritis Temporal arteritis Takayasu’s arteritis Lymphomatoid granulomatosis Wegner’s granulomatosis Primary central nervous system vasculitis Scleroderma Lymphocytic Allergic granulomatous angiitis Behcet’s syndrome Retinocochleocerebral vasculopathy Sneddon’s syndrome Degos disease Hypersensitivity/allergic vasculitis Drug-abuse arteritis Tumor-related vasculitis (dermatomyositis and polymyositis) Serum sickness Henoch–Schonlein purpura a
Important childhood vasculitides in bold. Source: Reproduced from Sinclair D and Mohr G Vasculitis, cerebral, surgery. In: Aminoff MJ and Daroff RB (eds.) Encyclopedia of Neurological Sciences, vol. 4, p. 640. Oxford: Elsevier.
617
See also: Cerebral Vasculitis. Endocannabinoids. Gilles de la Tourette’s Syndrome. Membrane Potential. Revascularization, Cerebral. Vasculitic Neuropathy
Further Reading Alhalabi M and Moore PM (1994) Serial angiography in isolated angiitis of the central nervous system. Neurology 44: 1221–1226. Alrawi A, Trobe JD, Blaivas M, and Musch DC (1999) Brain biopsy in primary angiitis of the central nervous system. Neurology 53: 858–860. Calabrese LH and Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 67: 20–39. Cellucci T and Benseler SM (2010) Central nervous system vasculitis in children. Current Opinion in Rheumatology 22: 590–597. Cohen BA and Biller J (1992) Hemorrhagic stroke due to cerebral vasculitis and the role of immunosuppressive therapy. Neurosurgery Clinics of North America 3: 611–624. Hellman DB and Stone JH (2002) Vasculitis of the central nervous system. In: Asbury AK, McKhann GM, McDonald WI, Goadbsy PJ, and McArthur JC (eds.) Diseases of the Nervous System: Clinical Neuroscience and Therapeutic Principles, 3rd edn., pp. 1547–1557. Cambridge, UK: Cambridge University Press. Hurst RW and Grossman RI (1994) Neuroradiology of central nervous system vasculitis. Seminars in Neurology 14: 320–340. McDonnell PJ, Moore GW, Miller NR, Hutchins GM, and Green WR (1986) Temporal arteritis. A clinicopathologic study. Ophthalmology 93: 518–530. Moore PM and Cupps TR (1983) Neurological complications of vasculitis. Annals of Neurology 14: 155–167. Sole-Llenas J and Pons-Tortella E (1978) Cerebral angiitis. Neuroradiology 15: 1–11. Woolfenden AR, Tong DC, Marks MP, Ali AO, and Albers GW (1998) Angiographically defined primary angiitis of the CNS: Is it really benign? Neurology 51: 183–188.