Encephalopathy with upper body hypertonia and myoclonus in patient with systemic lupus erythematosus and anti-CASPR2.

Systemic lupus erythematosus (SLE) may involve the nervous system but there are no specific biomarkers of neuroSLE. Limbic encephalitis has been rarely associated with SLE. We present a case of a 22-year-old black woman where typical SLE psychosis evolved to an encephalopathy with atypical features, normal MRI, electroencephalogram slowing and frontal and occipito-temporal hypometabolism on fluorodeoxyglucose positron emission tomography (FDG PET).Memory deficits, bizarre behaviour, psychosis, neuromyotonia and movement disorders have been described in autoimmune central nervous system disorders and associated with specific antibodies. Brain MRI may be normal and cortical brain hypometabolism on FDG PET scans has been reported. We have not found any report of limbic encephalitis or other SLE neurological manifestation associated to positive titres of anti-CASPR2 antibodies and this may warrant systematic investigation. In the rare cases of limbic encephalitis associated with SLE no specific antibodies were documented. Anti-CASPR2 antibodies have been associated not only with limbic encephalitis but also with neuromyotonia and Morvan syndrome. Although our patient had a specific pattern of tone abnormalities with an impressive cervical and upper limb hypertonicity and flaccid lower limbs, no myotonic discharges were found. We did not find any association between myoclonus and anti-CASPR2 antibodies. We cannot exclude that a non determined autoantibody could have played a role; however, clinical and FDG PET improvement supports an antibody-mediated injury, in this case of neuroSLE.

Epileptic manifestations in stroke patients treated with intravenous alteplase.

BACKGROUND AND PURPOSE: Intravenous alteplase (rtPA) may be associated with seizures and epileptic activity in the electroencephalogram (EEG). The aim of this work was to compare the frequency of seizures and EEG abnormalities between stroke patients treated and not treated with rtPA. METHODS: This was a prospective study of consecutive acute anterior circulation ischaemic stroke patients, with 1-year follow-up. Patients were previously independent, had an admission National Institute of Health Stroke Scale score ≥4, an acute ischaemic lesion and no previous seizures. They received standardized diagnostic and medical care. A video-EEG was performed in 72 h (first EEG); during admission (daily until day 7 and after that if neurological worsening); at discharge and 1 year after stroke. RESULTS: In all, 151 patients (101 treated with rtPA) were included. The frequency of acute and remote symptomatic seizures was not significantly different between rtPA treated and non-treated patients (P = 0.726 and P = 0.748, respectively). Clinical paroxysmal phenomena during rtPA perfusion were observed in five (5%) patients. In the first EEG, rtPA treated patients more often had background diffuse slowing (43.6% vs. 26.0%, P = 0.036). This difference was no longer observed at discharge (24.0% vs. 19.1%, P = 0.517) nor 1 year after (11.8% vs. 10.0%, P = 0.765). No differences were found in the frequency of epileptiform (P = 0.867) or periodic discharges (P = 0.381). CONCLUSIONS: Intravenous alteplase is not associated with an increased risk of clinical or electroencephalographic epileptic phenomena.

Epilepsia partialis continua after an anterior circulation ischaemic stroke.

BACKGROUND AND PURPOSE: Although cerebrovascular disorders are the main cause of epilepsia partialis continua (EPC) in adulthood, the frequency of EPC after stroke is unknown. The aim was to prospectively ascertain its frequency 1 year after an ischaemic stroke. METHODS: This was a prospective study of consecutive acute anterior circulation ischaemic stroke patients, previously independent, with an admission National Institutes of Health Stroke Scale score ≥4, an acute ischaemic lesion on imaging and no previous epileptic seizures. During admission patients received standardized diagnostic and medical care and were submitted to a neurophysiological evaluation protocol. One year after stroke, patients were re-evaluated by an epilepsy expert neurologist and performed a video-electroencephalogram with electromyography co-registration whenever myoclonus was observed during neurological examination for jerk-locked back averaging analysis (JLBA). EPC was defined as continuously repeated fragments of epileptic seizures, with preserved consciousness, lasting at least 1 h, and representing locally restricted epileptic activity. RESULTS: In all, 151 acute anterior circulation stroke patients were consecutively included and prospectively evaluated, but 23 died in the first year. One year after stroke, from 127 patients alive, 117 (92.1%) underwent clinical and neurophysiological evaluation. In two (1.7%) patients, EPC diagnosis was made both by clinical and electroencephalographic criteria, namely JLBA. Both patients had a history of remote symptomatic seizures and one of them acute symptomatic seizures and non-convulsive status epilepticus criteria during the first 7 days after stroke. CONCLUSIONS: Despite its low frequency, the high stroke incidence makes post-stroke EPC relevant. This study draws attention to this recognizable condition with therapeutic and eventually prognostic implications.

Time course of NT-proBNP levels after acute ischemic stroke.

BACKGROUND: Studies suggest that N-terminal-pro-brain natriuretic peptide (NT-proBNP) can be a biomarker of cardioembolic stroke. However, the best time to measure it after stroke is unknown. We studied the time course of NT-proBNP in patients with ischemic stroke. METHODS: Consecutive acute ischemic stroke patients were admitted over 10 months to a Stroke Unit. Stroke type was classified according to TOAST. Blood samples were drawn within 24, 48, and 72 hours after stroke. Friedman test was used to compare NT-proBNP values across the 3 times in all, cardioembolic and non-cardioembolic stroke patients. Post hoc analysis with Wilcoxon signed-rank tests was conducted with a Bonferroni correction. Mann-Whitney test was used to compare median values of NT-proBNP between cardioembolic and non-cardioembolic stroke patients. ROC curves were drawn to determine NT-proBNP accuracy to diagnose cardioembolic stroke at 24, 48, and 72 hours after stroke onset. RESULTS: One hundred and one patients were included (29 cardioembolic) with a mean age of 64.5±12.3 years. NT-proBNP values for cardioembolic stroke were significantly higher (P < 0.001) than for non-cardioembolic stroke in the 3 time points. NT-proBNP was highest in the first 24-48 h after ischemic stroke and decreased significantly 72 h after stroke onset. The area under the curve for the three time points was similar. CONCLUSION: NT-proBNP levels were highest in the first 2 days after ischemic stroke and declined significantly thereafter. However, the area under the curve for the three time points was similar. The first 72 hours after ischemic stroke have a similar diagnostic accuracy to diagnose cardioembolic stroke.

Influence of climatic factors in the incidence of multiple sclerosis relapses in a Portuguese population.

BACKGROUND AND PURPOSE: Environmental factors are thought to be important in multiple sclerosis (MS) pathophysiology. We aimed to evaluate if there was an association between MS relapses and some climatic factors in a Portuguese population. METHODS: Four year retrospective study analyzing 414 MS relapses in 249 consecutive relapsing-remitting patients. Non-parametric statistics were used to compare the distribution of relapses across months and seasons. Spearman's coefficient was determined to evaluate the correlation between relapses frequency and maximum and minimum atmospheric temperatures, humidity and atmospheric pressure. RESULTS: The mean number of relapses was not significantly different between months or seasons. No correlation was found between relapse frequency and any climatic factor. CONCLUSION: Our series is one of the largest addressing the influence of specific climatic factors on MS relapses. The number of clinical MS relapses seems to be unrelated to climatic factors.

Central nervous system vasculitis associated with hepatitis C virus infection: a brain MRI-supported diagnosis.

BACKGROUND: The Hepatitis C virus (HCV) infection is associated with various extrahepatic manifestations, being the Central Nervous System (CNS) rarely involved. CASE REPORT: We report a case of a 54 year-old black man with arterial hypertension who presented with progressively worsening headaches, apathy, somnolence and left hemiparesis. Brain MRI showed an acute ischemic lesion in the left anterior cerebral artery (ACA) and an old ischemic infarct in the right ACA territory. Brain MRI with gadolinium revealed mural thickening and contrast enhancement of the A1 and A2 segments of the ACAs, of the middle and distal basilar artery and of the P1 segment of the left posterior cerebral artery, suggesting active vasculitis. Digital angiography confirmed those irregularities and stenosis. Laboratory evaluation revealed ESR (73 mm/h), transaminase elevation, elevated HCV viral load genotype 2, positive IGRA, negative cryoglobulins, CSF protein elevation with oligoclonal bands (mirror pattern) and no pleocytosis; investigation excluded other infectious causes. Pegylated interferon alpha-2a and ribavirin, corticotherapy and tuberculosis prophylaxis were started with clinical and imagiological improvement. CONCLUSION: The typical inflammation signs of the vascular wall demonstrated by the gadolinium-enhanced MRI strengthened the hypothesis of CNS vasculitis. The association with HCV infection is rare but should be investigated once specific therapeutic is required.