Cognitive deficits in vasculitis of the nervous system: a cross-sectional study.

Objectives: To identify the cognitive and functional deficits in a well-characterized group of patients with vasculitis of the nervous system. Methods: Sixty-seven patients diagnosed with Central Nervous System (CNS) or Peripheral nervous System (PNS) vasculitis over a 14-year period were retrospectively identified. Data on clinical presentation, laboratory, radiographic and tissue biopsy investigations, and treatment were collated. Cognitive, functional and quality of life evaluation assessments were performed in 31 patients who agreed to participate and included Addenbrooke's Cognitive Examination-revised (ACE-R), Nottingham Extended Activities of Daily Living (NEADL) and EQ-5D-3L quality of life questionnaires. Results: CNS vasculitis patients exhibited cognitive impairment, with a mean ACE-R score of 74/100 (standard deviation (SD) 16). NEADL and EQ-5D-3L scores were in the impaired range at 41/66 (SD 21) and 57/81 (SD 22), respectively. Patients with just PNS vasculitis exhibited fewer cognitive deficits with ACE-R and NEADL scores of 87 (SD 8) and 46 (SD 16) respectively. EQ-5D-3L score was in the impaired range of 65 (SD 22). Conclusions: Vasculitis of the nervous system and, in particular, CNS vasculitis causes cognitive impairment and deficits in functional ability. Such patients should be targeted for cognitive rehabilitation.

Cognitive outcomes of childhood primary CNS vasculitis.

OBJECTIVE: To characterize the clinical cognitive phenotypes and severity of cognitive burden according to disease subtype in children with primary central nervous system vasculitis (cPACNS). METHOD: This retrospective multicenter inflammatory brain disease database study examined the neuropsychological outcomes of 80 children (44 male; mean age = 7.89 years, SD = 4.17) consecutively diagnosed with primary CNS vasculitis between 1992 and 2016. Twenty-one children had small-vessel disease (AN_cPACNS), and 59 had large-vessel disease (including 49 nonprogressive [APNP_cPACNS] and 10 progressive [APP_cPACNS]). Neuroimaging revealed MRI abnormalities in 100% and 90% of children with large- and small-vessel vasculitis, respectively. The primary outcomes were Full Scale IQ (FSIQ) and the index scores from the Wechsler Intelligence Scale for Children-III (WISC-III, WISC-IV, and WISC-V). Analyses explored the effect of disease subtype. RESULTS: Intellectual functioning was assessed on average 2.82 years after symptom onset. Children with small-vessel CNS vasculitis had significantly lower FSIQ scores than did those with large-vessel CNS vasculitis (Ms = 81.90 vs. 94.82; p = .04). Intellectual disability (FSIQ < 70) was more frequent in children with small-vessel disease (24% vs. 5%). All groups displayed lower Working Memory and Processing Speed index scores relative to Verbal Comprehension and Perceptual Reasoning index scores. Group differences in FSIQ remained significant after controlling for the presence of seizures. CONCLUSION: Children with small-vessel CNS vasculitis are more likely to demonstrate deficits in intellectual functioning than are those with large-vessel disease, and children with both types of CNS vasculitis demonstrate relatively poor working memory and processing speed. (PsycINFO Database Record (c) 2019 APA, all rights reserved).

99mTc-HMPAO brain SPECT in the monitoring of cerebral vasculitis therapy. / 99mTc-HMPAO SPECT cerebral en la monitorización de la terapia de vasculitis.

OBJECTIVE: The central nervous system (CNS) may be involved in a variety of inflammatory diseases of the blood vessels, generally known as vasculitis. The clinical diagnosis of such involvement in early stages is difficult, since a mild cognitive impairment can be the only symptom. It was hypothesized that brain-perfusion SPECT would be able to reveal CNS involvement and to monitor the course of the disease. The purpose of this study was assess if and when an improvement of cerebral perfusion can be registered by SPECT during the follow-up of these diseases. MATERIAL AND METHODS: Eighteen patients affected by Systemic Lupus Erythematosus (SLE), 22 by undifferentiated vasculitis (UV), 5 by Behcet's disease (BD) and 5 by primary Sjogren's Syndrome (pSS) were enrolled in this prospective study. A 99mTc-HMPAO brain perfusion SPECT was performed before the treatment and was repeated during the follow-up at different time intervals. Image analysis was performed on 10 cerebral areas using a specific software. RESULTS: In the SLE patients, no significant improvement of brain perfusion was found. On the contrary, in the UV the cerebral uptake of the tracer significantly improved from the twenty-fourth month (18/22 patients). Patients with BD showed an improvement of scintigraphic findings (5/5 patients), while a similar result was obtained only in 2 of the patients with pSS. CONCLUSIONS: In conclusion, brain SPECT seems to be able to monitor the disease in UV, indicating the moment when an improvement of the cerebral perfusion is achieved. In SLE patients this scintigraphic technique did not show a significant improvement in CNS perfusion.

Longitudinal neuropsychological findings in amyloid beta-related angiitis (AßRA): a case report.

Amyloid ß-related angiitis (AßRA) is a clinicopathological diagnosis of primary central nervous system angiitis theoretically triggered by vascular deposition of amyloid ß peptide. Deposits of Aß are associated with degeneration of the vasculature, thereby increasing risks of a stroke and/or cognitive impairment. Despite this, no prior studies have presented a detailed neuropsychological profile associated with AßRA. We present longitudinal neuropsychological findings for the case of a 58-year-old man with biopsy-diagnosed AßRA. Neuropsychological test results and clinical presentation demonstrated a mild to moderate dysexecutive syndrome implicating dorsolateral frontal and orbitofrontal-subcortical systems involvement. Despite prior reports of cognitive decline following a diagnosis of AßRA, cognitive functioning remained relatively stable over a 15-month period after immunosuppressive treatment. For the most part, objective measures did not demonstrate a measurable change in cognitive functioning, except for a mild decline in memory. There were subjective reports of improvement in cognitive and adaptive functioning from the patient and his spouse over this time period. The clinical significance of these results is discussed in the context of theories of executive dysfunction, and with reference to previously-published cases of AßRA.

Isolated angiitis of the central nervous system: a case presented with atypical psychiatric symptoms.

Isolated angiitis of the central nervous system (IACNS) is a rare form of angiitis limited to the central nervous system. The clinical finding of the combined series revealed that headache was the most common symptom within a combination of focal and diffuse neurological deficits. The case, a 28-year-old man, is presented; the clinical presentation and diagnostic difficulties are discussed. The patient's symptoms began with an obvious atypical depression. In spite of an antidepressive treatment, his symptoms continued to worsen with personality, mood changes and euphoria added to the clinical picture. Meanwhile after several transient ischemic attacks, 6 months later, he was admitted with neurological symptoms including headache, diplopia, and cerebellar ataxia. The radiological investigation was mimicked by primary brain lymphoma. The brain biopsy excluding of lymphoma revealed parenchymal hemorrhage with nonspecific degenerative changes. In systemic investigation, no underlying cause for vasculitis could be found. Neurological but not psychological deficits and radiological lesions of the patient improved with steroid therapy. Since we could not find features of systemic vasculitis, the patient's lesions responded to corticosteroid treatment and neuropathological investigation revealed no lymphoma, We concluded that the most probable diagnosis would be IACNS.

[A new patient education program: vasculitis]. / Ein neues Schulungsprogramm. Ein Statusbericht: Vaskulitis.

Referring to the literature it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Programs base on cognitive behavioral interventions. Within the last years an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center Medical University of Luebeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. To evaluate the new program a documentation system consisting of patient and physician- administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters has been designed. Patients completed the questionnaires at baseline, 4 weeks, 6 and 12 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

[Cerebral angiitis and Goodpasture's syndrome]. / Angéite cérébrale et syndrome de Goodpasture.

Goodpasture's syndrome is a form of rapidly progressive glomerulonephritis with pulmonary hemorrhage in the presence of antiglomerular basement membrane antibodies. Concomittant central nervous system manifestations are exceptionally reported. We report such a case of an 55-year-old woman who developed fluctuant neurobehavioral manifestations over a 9 months period. Angiography showed cerebral arterial distal lesions compatible with the diagnosis of angiitis. The antiglomerular basement membrane antibody titer was elevated. Search for anti-neutrophil cytoplasmic antibody was negative. After the reintroduction of cyclophosphamide agent, clinical evolution was favourable. Clinical cerebral manifestations in our case are probably du to a angiitis. Few cases are reported in the literature. The role of antiglomerular basement membrane antibody in the development of the cerebral angiitis is possible.

Frequency and patterns of subclinical cognitive impairment in patients with ANCA-associated small vessel vasculitides.

We investigated the prevalence of disease-related cognitive impairment in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated small vessel vasculitides (SVV). We studied 43 patients with ANCA-associated SVV (Wegener's granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangiitis (MP)), with no evidence of focal neurological deficits and dementia and in whom other potential causes of cognitive decline were carefully excluded. All patients underwent a detailed neuropsychological evaluation and their performances were compared with those of matched healthy controls. Patients were considered to be affected by subclinical cognitive impairment when they had abnormal results in at least two neuropsychological tests. Magnetic resonance imaging (MRI) scans of the brain were also obtained in 11 patients.The average neuropsychological test scores were not significantly different between the SVV patients and the control subjects. Thirteen patients had abnormal results in two tests (seven patients) or three or more tests (six patients). Most frequently, abnormal tests were the Rey Figure Recall (six cases), the Wisconsin Card Sorting Test (six cases), and the reaction times (eight cases). The frequency and extent of brain MRI abnormalities were higher in impaired than in unimpaired patients. This study demonstrates that 30% of clinically nondemented SVV patients can have a subclinical neuropsychological impairment, characterized by mild abstract reasoning loss, mental speed reduction and nonverbal memory impairment. MRI findings in impaired patients are consistent with the presence of an SVV-mediated subcortical damage of the brain.