Stroke in systemic lupus erythematosus: epidemiology, mechanism, and long-term outcome.

OBJECTIVE: To determine stroke prevalence, mechanisms, and long-term outcome in a cohort of Hispanic patients with systemic lupus erythematosus (SLE). METHODS: We analyzed demographical data, the timing between SLE diagnosis and stroke onset, stroke type, recurrence, and outcomes from an institutional database of 4451 patients with SLE followed from 1993 to 2018. RESULTS: We observed 139 strokes (3.1%), for an incidence rate of 1.25 per 1000 person-years: 81 (58.3%) acute ischemic stroke (AIS), 19 (13.7%) subarachnoid hemorrhage (SAH), 17 (12.2%) cerebral venous thrombosis, 13 (9.4%) intracerebral hemorrhage (ICH), and 9 (6.5%) transient ischemic attack. Median time from SLE diagnosis to acute stroke was 60 months (interquartile range 12-132 months). AIS had a bimodal presentation with 26% occurring within the first year and 30% >10 years after SLE diagnosis. In contrast, 75% of ICH cases occurred >3 years (and 34% >10 years) after SLE diagnosis. The most important cause of AIS was secondary antiphospholipid syndrome (48%). Hypertension was associated with 69% of ICH cases, while aneurysmal rupture was observed in 78% of SAH cases. Excellent recovery at hospital discharge was observed in 65%. Stroke recurrence was observed in 7%. The long-term all-cause fatality rate was 8%. CONCLUSIONS: The prevalence of stroke in this cohort was 3.1%. Ischemic strokes had a bimodal presentation, occurring either early after SLE diagnosis or after a several-year delay. Half of the hemorrhagic strokes occurred >10 years after the diagnosis of SLE. Clinical outcome was usually good with a relatively low recurrence rate.

Natural history of longitudinally extensive transverse myelitis in 35 Hispanic patients with systemic lupus erythematosus: good short-term functional outcome and paradoxical increase in long-term mortality.

Background and objective Acute transverse myelitis (TM) is an infrequent neurological complication of systemic lupus erythematosus (SLE). Short-term outcome varies widely between cohorts. Little is known about the epidemiology and long-term functional outcome of TM associated to SLE. Methods Patients with SLE and acute TM were identified during hospital admission, visits to the Emergency Room or the Neurology Outpatient Clinic. We evaluated ambispectively those patients with SLE presenting with clinical myelopathy and corroborated with spinal MRI. Cases were divided as partial (non-paralyzing) or complete (paralyzing). We determined long-term functional outcome as well as mortality in those patients with follow-up periods of at least five years. Results We identified 35 patients (partial, n = 15; complete, n = 20) in which complete clinical and imaging data were available (26 with follow-up ≥ 5 years). Patients with complete TM were significantly older than those with partial forms. Positive antiphospholipid antibodies were observed in 80% of patients, suggesting a possible mechanistical role. Surprisingly, functional recovery at one year was in general good; however, we observed a five-year mortality of 31% because of sepsis (in 10 cases) or pulmonary embolism (in one case). Conclusions Short-term outcome of SLE-related TM is generally good, and recurrence rate is low. However, we observed a long-term fatality rate of 31% for reasons unrelated to TM, suggesting that TM is a manifestation of severe immune dysregulation and a predictor of severity and mortality in patients with SLE.

Development of measures of polyneuropathy impairment in hATTR amyloidosis: From NIS to mNIS + 7.

Hereditary transthyretin-mediated amyloidosis (hATTR amyloidosis) is a rare, life-threatening disease, caused by point mutations in the transthyretin gene. It is a heterogeneous, multisystem disease with rapidly progressing polyneuropathy (including sensory, motor, and autonomic impairments) and cardiac dysfunction. Measures used to assess polyneuropathy in other diseases have been tested as endpoints in hATTR amyloidosis clinical trials (i.e. Neuropathy Impairment Score [NIS], NIS-lower limb, and NIS + 7), yet the unique nature of the polyneuropathy in this disease has necessitated modifications to these scales. In particular, the heterogeneous impairment and the aggressive disease course have been key drivers in developing scales that better capture the disease burden and progression of polyneuropathy in hATTR amyloidosis. The modified NIS + 7 (mNIS + 7) scale was specifically designed to assess polyneuropathy impairment in patients with hATTR amyloidosis, and has been the primary endpoint in two recent, phase III studies in this disease. The mNIS + 7 uses highly standardized, quantitative, and referenced assessments to quantify decreased muscle weakness, muscle stretch reflexes, sensory loss, and autonomic impairment. Physicians using this scale in clinical trials should be specifically trained and monitored to minimize variability. This article discusses the different scales that have been/are being used to assess polyneuropathy in patients with hATTR amyloidosis, their correlation with other disease assessments, and reflects on how and why scales have evolved to the latest iteration of mNIS + 7.

[Prothrombotic states and cerebral ischemia]. / Estados protrombóticos e isquemia cerebral.

INTRODUCTION: Hematological disorders per se represent unusual causes of cerebral ischemia, explaining in young people 4% of strokes. Hematological disorders that induce a thrombotic tendency contribute to overall ischemic stroke risk and may directly cause cerebral ischemia in patients without other risk factors. The frequency of cerebral infarctions caused by prothrombotic states is not known. DEVELOPMENT: This review will focus on disorders such as prothrombotic coagulopaties, including resistance to activated protein C and antiphospholipid syndrome as cause of cerebral infarction. Cerebral venous thrombosis and cerebral infarction from arterial origin are the most common form of neurological involvement. Pathophysiological mechanism of stroke in these patients are multiple and can include as in antiphospholipid syndrome embolism from valves abnormalities related to hematological disturbance, as well as thrombosis of extracranial or intracranial vessels. CONCLUSIONS: Is clear, however, that prothrombotic states could explains a high percentage of cases of those so called cryptogenic cerebral infarction in young people.

47 year-old man with HIV infection and hemiplegia.

CNS aspergillosis is often missed in the setting of advanced HIV infection, especially in the absence of presumed risk factors such as neutropenia or prior steroid treatment. We describe the postmortem evaluation of the brain of a patient with AIDS that developed progressive neurologic deterioration. Sequence brain MRIs, CSF analysis, and multiple presumed treatments failed to reveal the possible causes or improve his ongoing condition. His brain autopsy showed numerous abscesses with septated hyphae consistent with CNS angioinvasive aspergillosis.

Cerebral infarction in young women: analysis of 130 cases.

UNLABELLED: The aim of this study was to determine the risk factors and mechanism of cerebral infarction in young women. METHODS: We evaluated 130 consecutive women younger than 41 years of age with cerebral infarction and compared the risk factors with a control group of 122 healthy, age-matched women. RESULTS: The leading risk factors in patients with cerebral infarction were migraine (15%), tobacco use (15%), and oral contraceptive (OC) use (12%). Cerebral arteriograms were abnormal in 59% of patients (57 of 96). The causes of cerebral infarction were cardiac embolism in 36%, nonatherosclerotic vasculopathy in 25%, hematologic disorders in 8%, and migraine in 8%. The etiology could not be determined in 23% of patients. CONCLUSION: Migraine and OCs are independent risk factors for cerebral infarction in young women. The leading etiologies were rheumatic valve disease and nonatherosclerotic vasculopathy, hematologic disturbances, and migraine were responsible for a few cases.

PIP: This study examines the risk factors and mechanism of cerebral infarction in 130 women younger than 41 years of age with cerebral infarction. A control group of 122 healthy, age-matched women were used for comparison. Each patient underwent the following: complete blood count, biochemical profile, lipid profile, venereal disease laboratory test, erythrocyte sedimentation rate, and rheumatologic profile (rheumatoid factor, antinuclear antibodies, anti-DNA, C-reactive protein). All patients underwent computed tomography or magnetic resonance imaging, transthoracic or transesophageal echocardiography; while transcranial Doppler or sonography of vessels of the neck and cerebral angiography were performed electively. The results of evaluation revealed that the leading factors among patients with cerebral infarction were migraine (15%), tobacco use (15%), and oral contraceptive (OC) use (12%). Cerebral arteriograms were abnormal in 59% of patients. The causes of cerebral infarction were cardiac embolism (36%), nonatherosclerotic vasculopathy (25%), hematologic disorders (8%), and migraine (8%). The etiology could not be determined in 23% of patients. Migraine and OCs were considered as independent risk factors for cerebral infarction in young women.

Recurrent primary cerebral hemorrhage: frequency, mechanisms, and prognosis.

BACKGROUND AND PURPOSE: The frequency of recurrent primary cerebral hemorrhage (RPCH), mainly in cases related to hypertension, has been considered low. This study investigated the frequency, mechanisms, and prognosis of RPCH. METHODS: We evaluated 359 patients with neuroimaging evidence of cerebral hemorrhage and selected 22 with RPCH. RESULTS: Five patients (23%) were older than 70 years at the first cerebral hemorrhage. Mean ages at the first and second hemorrhages were 60 and 63 years, respectively. Risk factors included hypertension (86%), diabetes (27%), and tobacco and alcohol use (each 14%). Hypocholesterolemia was demonstrated in 35% of the patients. The most common pattern of recurrent bleeding was ganglionic-ganglionic, mainly related to hypertension. Overall mortality was 32%. Forty-one percent and 27% of patients, respectively, had incapacitating and nonincapacitating sequelae; 2 of the latter had RPCH with a lobar location. Ganglionic-ganglionic hemorrhage was associated with a poor prognosis; otherwise, this pattern was uncommon in patients with nonincapacitating sequelae. Analysis of the control of risk factors, primarily hypertension after the first cerebral hemorrhage, disclosed that 56% of patients did not gain subsequent control. CONCLUSIONS: Rebleeding after a first primary intracerebral hemorrhage is not uncommon. The main topographic pattern of bleeding, ganglionic-ganglionic, is likely the result of hypertension; the less common lobar-lobar pattern probably results from amyloid angiopathy.