Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare meningoencephalitis that presents with focal neurological deficits, cortical microbleeds and asymmetrical white matter lesions, usually steroid-responsive. It shares radiological features with amyloid related imaging abnormalities (ARIA), described in Alzheimer's disease (AD) patients that receive antiamyloid therapies, suggesting a common pathophysiological process. Clinical, neuropsychological and radiological description; APOE genotyping and cerebrospinal fluid (CSF) biomarkers (Aβ 42, Aβ 40, t-Tau, p-Tau and anti-A b autoantibodies) were determined in four patients with probable CAA-ri. PET imaging with Florbetapir was performed in two subjects, and one of them had a PET-PIB additionally. Patients received steroids and were clinically evaluated. T hree patients had imaging and CSF follow-up. Mean age of presentation was 73.5 years (range 69-78), 50% were men. All patients presented with neurological focal symptoms and cognitive impairment. MRI showed cortical microbleeds and asymmetrical hyperintense white matter lesions. Allelic frequency for APOE-e4 was 62.5%. Aß40 and Aß42 levels were decreased and anti-Ab autoantibodies were high in all cases. A PET with Florbetapir, performed in two patients, and a PET-PIB performed in one of them, showed cortical amyloid deposits. After corticosteroid administration, a complete (1/4) or partial (3/4) resolution of focal symptoms was observed. One patient suffered an intracerebral lobar hemorrhage six months after the resolution of focal symptoms. On the follow-up MRI, a significant reduction of white matter lesions was appreciated. CSF anti-Ab autoantibodies at follow-up were markedly reduced in all cases. The pattern in CSF and amyloid PET biomarkers and the presence of APOE-e4 in CAA-ri suggests an important role of vascular amyloidosis. Anti-Ab autoantibodies may be a specific biomarker for this process, and could be useful for CAA-ri diagnosis and monitoring the response to treatment.
Impulsivity is an aspect of personality and a major component of multiple neuropsychiatric conditions. In Parkinson's disease, it has been associated with the expression of impulse control disorders, a highly prevalent non-motor complication. Even though multiple tests of impulsivity have been used in this context, the impact of test choice has not been addressed. The aim was to evaluate whether different impulsivity measures in Parkinson's disease share substantial inter-scale and anatomical correlations or rather mirror different underlying phenomena.In a consecutive sample of 89 Parkinson's disease patients without impulse control disorders, four common tests were evaluated assessing different aspects of impulsivity: impulsiveness trait, decisions under implicit risk with and without losses, and delay discounting. Correlations among test scores were analysed and each score was used as a regressor in a set of grey matter volume (GMV) voxel-based morphometry analyses to explore their brain structural correlates.No significant correlations were found between the different impulsivity tests. Furthermore, their structural brain correlates were divergent. Impulsiveness trait appeared to be associated with lower GMV in dorsal-lateral prefrontal cortices, implicit risk (with losses) with higher GMV in the left nucleus accumbens and lower left insular GMV, implicit risk (without losses) with higher GMV in the left lingual gyrus and lower GMV in the gyri recti and delay discounting with higher GMV in the left nucleus accumbens.In Parkinson's disease, different impulsivity measures reflect very dissimilar behavioural and brain structural correlates. Our results suggest that parkinsonian impulsivity is not a unitary phenomenon but rather a heterogeneous entity.
ABSTRACT Multiple sclerosis (MS) is a tissue-specific autoimmune disease of the central nervous system in which the antigen(s) remains elusive. Antibodies targeting the flotillin-1/2 (FLOT–1/2) complex have been described in 1-2% of the patients in a recent study. Other candidate antigens as anoctamin-2 (ANO2) or neurofascin-155 (NF155) have been previously described in MS patients, although their clinical relevance remains uncertain. Our study aims to analyse the frequency and clinical relevance of antibodies against NF155, ANO2 and the FLOT-1/2 complex in MS. Serum (n=252) and CSF (n=50) samples from 282 MS patients were included in the study. The control group was composed of 260 serum samples (71 healthy donors and 189 with other neuroinflammatory disorders). Anti-FLOT1/2, anti-ANO2 and anti-NF155 antibodies were tested by cell-based assays using transfected-HEK293 cells. We identified 6 MS patients with antibodies against the FLOT-1/2 complex (2.1%) and 1 MS patient with antibodies against ANO2 (0.35%). All MS patients were negative for anti-NF155 antibodies. Three of the anti-FLOT1/2 positive patients showed anti-FLOT-1/2 positivity in other serum samples extracted at different moments of their disease. IgG subclasses of anti-FLOT-1/2 antibodies were predominantly IgG1 and IgG3. We confirm that antibodies targeting the Flotillin-1/2 complex are present in a subgroup of patients with MS. Further studies are needed to understand the clinical and pathological relevance of anti-FLOT-1/2 autoantibodies in MS.
In patients with ischemic stroke (IS) or transient ischemic attack (TIA) and cortical superficial siderosis (cSS), there are few data regarding the risk of future cerebrovascular events and also about the benefits and safety of antithrombotic drugs for secondary prevention. We investigated the associations of cSS and stroke risk in patients with recent IS or TIA.
The authors describe a patient with persistent headache initially diagnosed as idiopathic hypertrophic pachymeningitis after a comprehensive study that included meningeal biopsy. Despite early response to corticosteroids, the headache relapsed 3 years later and he further developed painful ophthalmoplegia and multiple cranial neuropathies (V1, VI, VIII, and X). A cervical lymph node biopsy revealed caseating granulomas and antituberculous therapy was started. Symptoms had resolved at 3-month follow-up. The authors discuss tuberculosis as a possible cause of pachymeningitis, painful ophthalmoplegia, and multiple cranial neuropathy syndromes, and describe the common features of these clinical conditions.