Surgical and endovascular cerebral revascularization for cerebral vasculitis with inflammatory vessel stenosis: a case series.

Autoimmune vasculitides affect the cerebral vasculature significantly in a considerable number of cases. When immunosuppressive treatments fail to prevent stenosis in cerebral vessels, treatment options for affected patients become limited. In this case series, we present four cases of pharmacoresistant vasculitis with recurrent transient ischemic attacks (TIAs) or stroke successfully treated with either extracranial-intracranial (EC-IC) bypass surgery or endovascular stenting. Both rescue treatments were effective and safe in the selected cases. Our experience suggests that cases of pharmacoresistant cerebral vasculitis with recurrent stroke may benefit from rescue revascularization in combination with maximum medical management.

Rare primary angiitis of the central nervous system affecting the clivus: A case report.

Diseases involving the clivus are highly variable, and the incidence of each disease is rare. Primary central nervous system vasculitis (PACNS) is a rare disease with very heterogeneous clinical manifestations, its diagnosis is often challenging, and histopathology is the gold standard. We report a patient with PACNS of the clivus, with a 1-month history of headache and diplopia, who was misdiagnosed as having a tumor of the clivus during prior treatment, due to computed tomography findings of clivus occupation and bone destruction. Endoscopic resection of the nasal clivus lesions was performed. Pathological examination revealed a small abscess with hemorrhage, necrosis, extensive infiltration of lymphocytes and plasma cells, and granulation tissue scar formation. After histopathological examination, the diagnosis was confirmed, and oral glucocorticoid and cyclophosphamide were commenced. This study is the first to report a tumor-like PACNS, that occurs in the clivus, thereby enriching our understanding of PACNS.

Necrotizing sarcoid granulomatosis simulating pulmonary malignancy: A case report.

RATIONALE: Necrotizing sarcoid granulomatosis (NSG) has recently been termed "sarcoidosis with NSG pattern" for the disease entity representing nodular sarcoidosis with granulomatous pulmonary angiitis. It is characterized by sarcoid-like granulomas, vasculitis, and a variable degree of necrosis. Its rarity and nonspecific clinical symptoms can easily lead to misdiagnosis or delayed diagnosis. PATIENT CONCERNS: We report a 67-year-old female with a biopsy-confirmed sarcoidosis with NSG pattern mimicking pulmonary malignancy on initial chest computed tomography scan. DIAGNOSES: Sarcoidosis with NSG pattern. INTERVENTIONS: The patient underwent video-assisted thoracoscopic surgery with a lung biopsy. No further treatment was performed after the lung biopsy. OUTCOMES: Follow-up imaging studies revealed spontaneous regression of the disease after 2 months. LESSONS: Awareness of this rare benign disease entity and overlapping radiologic manifestations with pulmonary malignancy or other granulomatous diseases can be helpful for making a precise diagnosis with a better differential diagnosis.

Characteristics of mass lesion presentation of primary angiitis of the central nervous system: A single center 11-year retrospective case-series study.

OBJECTIVE: Mass lesion presentation of primary angiitis of the central nervous system (ML-PACNS) is a special subtype of primary central nervous system vasculitis, which is difficult to be differentiated from other space-occupying disorders. We aimed to summarize our experience and improve diagnostic techniques of ML-PACNS. PATIENTS AND METHODS: Case records for 16 patients treated in the Department of Neurology of Six Medical Center of Chinese PLA General Hospital from December 2008 to November 2019 were examined. All patients were diagnosed with ML-PACNS by pathology. Clinical manifestations, neuroimaging results and pathological features were retrospectively analyzed. RESULTS: The 16 patients with ML-PACNS in the cohort (8 males) had a median age of 32 (range 19-56 years). On T1WI and T2WI, 12 cases showed mixed signals and 4 cases showed T1WI hypointense and T2WI hyperintense. Fifteen patients showed DWI hyperintense, of which 9 cases showed hypointense in the middle of the lesion. Gadolinium enhancement of all cases was irregular. All patients had a brain biopsy (stereotactic procedure in 10 and open-wedge surgery in 6) which showed lymphocytic vasculitis in the majority of patients (15/16) and necrotizing vasculitis in one patient. CONCLUSIONS: ML-PACNS is an important differential candidate for tumefactive demyelinating lesions, CNS infections and brain tumors. Mixed T1WI and T2WI signals as well as central hypointense surrounded by hyperintense lesions on DWI may be useful imaging features for the diagnosis of ML-PACNS which has implications for those who have not yet had a biopsy. ML-PACNS may have different pathological type from PACNS.

Extracranial-Intracranial Bypass for Cerebral Vasculitis After Graft-Versus-Host Disease: Case Report and Review of the Literature.

BACKGROUND: Cerebral vasculitis owing to chronic graft-versus-host disease (GVHD) is very rare. To our knowledge, only 2 cases have been reported. We describe the first case of superficial temporal artery-middle cerebral artery (STA-MCA) bypass for cerebral vasculitis owing to GVHD. CASE DESCRIPTION: A 59-year-old woman presented with right upper extremity weakness and dysarthria 33 months after undergoing matching allogenic bone marrow transplantation. The patient had STA-MCA bypass for MCA occlusion and resting cerebral blood flow in the left MCA area improved. CONCLUSIONS: Although the mechanism of cerebral vasculitis after chronic GVHD is not known, cerebral vasculitis that causes cerebrovascular disease long after bone marrow transplantation should be considered. In this case, an STA-MCA bypass was efficient for the repeated ischemic attacks owing to cerebral vasculitis. Moreover, it is important to select the optimal recipient vessels to area originated neurologic symptoms.

Vessel Wall MRI for Targeting Biopsies of Intracranial Vasculitis.

Central nervous system vasculitides are elusive diseases that are challenging to diagnose because brain biopsies have high false-negative rates. We sought to test the ability of contrast-enhanced, high-resolution 3D vessel wall MR imaging to identify vascular inflammation and direct open biopsies of intracranial target vessels and adjacent brain parenchyma. Eight of 9 specimens revealed vascular inflammation. We conclude that vessel wall MR imaging can identify inflamed intracranial vessels, enabling precise localization of biopsy targets.

Diagnostic Utility of Contrast-enhanced 3D T1-weighted Imaging in Acute Cerebral Infarction Associated with Graves Disease.

Graves disease is rarely complicated with cerebrovascular steno-occlusive diseases. Previous studies have suggested several hypotheses for this occurrence, including excess thyroid hormone, which stimulates the sympathetic nervous system, which in turn causes an abnormal hemodynamic response with consequent atherosclerotic changes, and antithyroid antibodies cause local vascular inflammation in patients with Graves disease. However, radiological findings of vasculitis in patients with Graves disease and cerebral infarction remain less known. We report the case of a 30-year-old Japanese woman with acute cerebral infarction due to vasculitis associated with Graves disease. She was admitted to our hospital with a 4-day history of intermittent transient dysarthria and limb shaking of the left leg when standing. Three weeks before admission, she went to a local hospital because of general malaise and was diagnosed with Graves disease. Neurological examination revealed paralytic dysarthria, left central facial nerve palsy, and left hemiparesis (manual muscle testing, 4 of 5). Blood examinations showed hyperthyroidism (thyroid-stimulating hormone ≤.010 µU/mL; free T3 ≥25.0 pg/mL; free T4 ≥8.0 ng/dL) and elevation of antithyroid antibody levels (thyroid peroxidase antibody, 87 IU/mL). The vessel wall of the right internal carotid artery was markedly enhanced on contrast-enhanced three-dimensional T1-weighted magnetic resonance imaging, suggesting vasculitis. Magnetic resonance angiography revealed right internal carotid artery occlusion after the branching ophthalmic artery. Arterial stenosis due to vasculitis was considered the cause of hemodynamic ischemic stroke. Vessel wall imaging such as high-resolution contrast-enhanced T1-weighted imaging seems useful for assessing the underlying mechanism of stroke in patients with Graves disease.

A Singular Case of Neurosyphilis Manifesting Through a Meningovascular Chronic Inflammatory Process in Association with the Occurrence of Two Aneurysms Involving the Distal A2 Segment of Both Anterior Cerebral Arteries: A Case Report and Review of the Literature.

BACKGROUND: Although syphilis has become a rare disease in the Western world since the Second World War, it is believed to have infected 12 million people in 1999, with greater than 90% of cases occurring in the developing world. Moreover, since the year 2000, the rates of syphilis have been increasing in the United States, the United Kingdom, Australia, and Europe. Because of the mimic nature of the disease and the overall low rate of occurrence of its manifestations in advanced stages, a proper diagnosis may prove difficult. CASE REPORT: We report the case of a 41-year-old African man affected by neurosyphilis that manifested itself through a meningovascular chronic inflammatory process, with the peculiar feature of a bilateral aneurysm of probable mycotic origin involving the distal tract of A2 segment of both anterior cerebral arteries. CONCLUSIONS: Because of the mostly nonspecific nature of clinical manifestations of syphilis (particularly advanced syphilis) and its consequent tendency to masquerade as many other diseases, even a skilled physician may find its diagnosis quite challenging; thus, thorough clinical and radiologic investigations should be supported by serologic testing for syphilis in all cases of cognitive impairment. Mycotic intracranial aneurysms in association with neurosyphilis rarely are reported; however, they require early diagnosis and meticulous, individualized treatment. Because syphilis appears to be on the raise, further studies on the topic are warranted.

Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

Childhood primary angiitis of the central nervous system with metachronous hemorrhagic infarcts: a postmortem study with clinicopathologic correlation.

This neuropathologic case study illustrates the discovery of metachronous hemorrhagic infarcts insinuating round mass-like lesions by magnetic resonance imaging in the setting of childhood primary angiitis of the central nervous system (cPACNS) raising diagnostic awareness of this unusual presentation in a clinical and neuroimaging context. The report underscores the importance of recurrent vasculitis-induced ischemic brain damage as a pathologic correlate of relapsing cPACNS and offers a critical reappraisal of common imitators as well as a clinicopathologic approach to differential diagnosis. Attention is drawn to the caveat that although magnetic resonance imaging findings at initial presentation may not be typical for stroke, they later exhibit attributes of cerebral infarction at both the subacute and chronic stages. A pattern of cPACNS characterized predominantly by multiple petechial-like cortical hemorrhages with pathologic features of hemorrhagic infarcts is recognized. The present study lends credence to the practice of a rigorous autopsy-based approach aimed at a better understanding of the anatomic pathology and biology of cPACNS and at facilitating prospective neuroimaging and biopsy-based surgical pathology correlations, ultimately enhancing diagnostic accuracy in clinical settings. Although PACNS is, by definition, a diagnosis of exclusion, it should be considered from the outset in the differential diagnosis of ischemic stroke or hemorrhagic stroke or of unusual and relapsing intra-axial mass-like CNS lesions in children, necessitating appropriate pathologic evaluation of brain biopsy specimens.

Cerebral vasculitis in severe Kawasaki disease: early detection by magnetic resonance imaging and good outcome after intensive treatment.

Kawasaki disease is an acute vasculitis, that has a classic complication of acquired coronary artery aneurysm. Severe forms with multi-organ involvement or neurological dysfunction are rare. Cerebral vascular involvement has been related to large-vessel injury or cardioembolism, leading to focal brain infarction. A 4-year-old female presented with unusual, rapidly catastrophic Kawasaki disease with refractory shock, acute renal failure, and coma, requiring intensive haemodynamic management. The observation of diffuse micro-haemorrhages (T2*-weighted sequence) associated with white matter injury on brain magnetic resonance imaging (MRI) pointed towards lesions of the medium/small blood vessels. Cerebral vasculitis was suspected and the immunosuppressive treatment was increased Subsequently, the patient's recovery was rapid. On follow-up severe, bilateral vitritis was evident and surgery improved visual outcome. Early recognition of severe or unusual forms of Kawasaki disease could lead to more favourable outcome using appropriate treatment strategies. Diffuse cerebral micro-haemorrhages on T2* brain MRI sequences might be a key sign for the diagnosis of medium or small cerebral vessel involvement.

Primary angiitis of the central nervous system: 2 atypical cases.

The presence of an angiitis process in the central nervous system (CNS) characterizes different groups of conditions: from idiopathic pachymeningitis to lymphoproliferative disorders. In absence of specific infections, inflammatory and neoplastic diseases, the term "PACNS" (Primary Angiitis of the CNS) was proposed to indicate a peculiar vascular inflammation of unknown origin of meningeal vessels extending to the brain or spinal cord parenchyma. We report two cases of PACNS with peculiar and atypical features: the first one with a possible Epstein Barr Virus (EBV) relationship, the second one with spinal cord involvement only, treated surgically. We also hypothesize a correlation between EBV chronic infection and possible subtypes of PACNS stressing the importance of EBER (EBV-encoded RNA) test in the routine examination of brain biopsies suspicious for PACNS.

Refractory status epilepticus secondary to CNS vasculitis, a role for epilepsy surgery.

Central nervous system (CNS) vasculitis is a rare group of disorders that affect vessels of the brain parenchyma and meninges. It presents with headache, cognitive changes, or seizures, yet without aggressive management, it carries a high degree of morbidity and mortality. Refractory status epilepticus (SE) has been reported with CNS vasculitis. Patients are treated with immunosuppression, antiepileptic drugs (AED), and anesthetic agents. Outcomes are usually poor. Epilepsy surgery for refractory partial SE has succeeded in patients. We present a comparison of two patients with refractory partial SE due to CNS vasculitis. One patient was treated medically and died, while the other underwent epilepsy surgery to remove the epileptic focus along with medical therapy and the patient had substantial recovery. We describe clinical, electrophysiological, pathological, and treatment features of both patients and discuss rationale for surgical intervention. This is the first case report of the use of epilepsy surgery for the treatment of refractory SE associated with CNS vasculitis.

Primary central nervous system vasculitis presenting as spinal subdural hematoma.

BACKGROUND: Primary central nervous system vasculitis (PCNSV) is a rare and poorly described vascular inflammatory condition confined within the central nervous system (CNS). Typical presentations of PCNSV include headache, hemiparesis, cerebral edema, and altered cognition. Vasculitic processes in the spinal cord are exceedingly rare. Although intracranial vasculitis has been associated with certain systemic inflammatory disorders and lymphomas, the etiology of spinal cord vasculitis is unclear. We report the first case of a patient in whom spinal subdural hematomas were the first presenting signs of PCNSV. CASE DESCRIPTION: A 69-year-old woman presented with sudden-onset severe back pain and paraplegia following an acute hypertensive event. Multiple spinal subdural hematomas were noted on magnetic resonance imaging, and emergent laminectomies were performed for evacuation. Vasculitic abnormalities confined to the CNS were noted intraoperatively and on imaging, leading to the unique diagnosis. CONCLUSIONS: This previously unreported etiology of nontraumatic acute spinal subdural hematomas suggests that spinal cord vasculitis as part of PCNSV should be included in the differential diagnosis.

Primary angiitis of the central nervous system mimicking tumor-like lesion--case report.

A 60-year-old man presented with a rare case of primary angiitis of the central nervous system mimicking a tumor-like lesion and manifesting as slight disorientation, left hemiparesis, and motor aphasia. Computed tomography showed multiple low density lesions in the left frontal lobe, brain stem, and right parietal lobe. Magnetic resonance images revealed a slightly enhanced mass lesion in the right parietal lobe with surrounding brain edema. Serum, cerebrospinal fluid, and other image examinations did not show any abnormal findings, so surgical removal of the right parietal mass was performed. Histological examination revealed that the mass consisted of hemorrhagic infarction without cellular atypia. Proliferations of endothelial cells in small and medium arteries, and infiltration of macrophages in the perivascular space were detected in the infarction tissues. The histological diagnosis was primary angiitis of the central nervous system.

Reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndromes (RCVS) are a group of disorders that have in common an acute presentation with headache, reversible vasoconstriction of cerebral arteries, with or without neurological signs and symptoms. In contrast to primary central nervous system vasculitis, they have a relatively benign course. We describe here a patient who was diagnosed with RCVS.

Granulomatosis with CNS involvement: a neuroimaging clinicopathologic correlation.

BACKGROUND AND PURPOSE: To present a rare case of a rheumatologic disorder with a central nervous system manifestation. METHODS: A 48-year-old female with history of nasal and auricular pain and inflammation developed bilateral intermittent scleritis that was refractory to several immunosuppressive medications. She later presented with headaches and right upper extremity parasthesias. Brain magnetic resonance imaging (MRI) was obtained and she was referred to a neurosurgeon. RESULTS: Neurological examination did not reveal any focal deficits. Laboratory analysis demonstrated an elevated erythrocyte sedimentation rate (ESR). Brain MRI with and without gadolinium contrast revealed an extra-axial, enhancing, left frontoparietal convexity lesion. The patient was taken to the operating room for biopsy and resection of the mass. Final pathology demonstrated dural granulomatosis with vasculitis, most consistent with intracranial Wegener's granulomatosis. The patient was discharged to follow-up with her rheumatologist for further medical management. CONCLUSIONS: Enhancing, intracranial dural lesions are associated with a variety of disease entities. Wegener's granulomatosis is one such entity and should be considered in the correct context.

Primary granulomatous angiitis of the central nervous system: findings of magnetic resonance spectroscopy and fractional anisotropy in diffusion tensor imaging prior to surgery. Case report.

Primary granulomatous angiitis of the central nervous system (CNS) is extremely rare. Its preoperative diagnosis is difficult as the condition displays nonspecific features on routine neuroimaging investigations. In this paper, the authors report findings of magnetic resonance (MR) spectroscopy and fractional anisotropy (FA) with diffusion tensor MR imaging in a case of granulomatous angiitis of the CNS. A 30-year-old man presented with morning headaches and grand mal seizures. An MR image revealed a mass resembling glioblastoma in the right temporal lobe. Magnetic resonance spectroscopy showed a high choline/creatine (Cho/Cr) ratio indicative of a malignant neoplasm, accompanied by a slight elevation of glutamate and glutamine. The FA value was very low, which is inconsistent with malignant glioma. The mass was totally removed surgically. Histologically, the peripheral lesion of the mass consisted of a rough accumulation of fat granule cells, infiltration of inflammatory cells, and distribution of capillary vessels. Some vessels within the lesion were replaced by granulomas. The histological diagnosis was granulomatous angiitis of the CNS. The MIB-1-positive rate of the granuloma was approximately 5%. Both MR spectroscopy and FA were unable to accurately diagnose granulomatous angiitis of the CNS prior to surgery; however, elevated Cho/Cr and glutamate and glutamine shown by MR spectroscopy may indicate the moderate proliferation potential of the granuloma and the inflammatory process, respectively, in this condition. Although the low FA value in the present case enabled the authors to rule out a diagnosis of glioblastoma, FA values in inflammatory lesions require careful interpretation.