Kinetics of cerebral blood flow velocities during treatment for delayed cerebral ischemia in aneurysmal subarachnoid hemorrhage.

BACKGROUND: In aneurysmal subarachnoid hemorrhage (aSAH), one of the main determinants of prognosis is delayed cerebral ischemia (DCI). Transcranial Doppler (TCD) is used to monitor vasospasm and DCI. We aimed to better understand cerebral hemodynamics response to hypertension induction (HI) with norepinephrine (NE) and inotropic therapy with milrinone so that TCD can be a bedside tool in helping to guide DCI therapies. Our primary objective was to determine TCD blood flow velocity (BFV) kinetics during HI and inotropic therapy for DCI treatment. Secondly, we performed an analysis by treatment subgroups and evaluated clinical response to therapies. METHODS: We performed a prospective observational cohort study in a Brazilian high-volume center for aSAH. Patients with aSAH admitted between 2016 and 2018 who received NE or milrinone for DCI treatment were included. TCDs were performed before therapy initiation (t0) and 45 (t1) and 90 min (t2) from the onset of therapy. For each DCI event, we analyzed the highest mean flow velocity (MFV) and the mean MFV and compared their kinetics over time. The National Institutes of Health Stroke Scale was determined at t0, t1, and t2. RESULTS: Ninety-eight patients with aSAH were admitted during the study period. Twenty-one (21.4%) developed DCI, of whom six had DCI twice, leading to a total of 27 analyzed DCI events (12 treated with HI and 15 with milrinone). Patients treated with NE had their mean arterial pressure raised (85 mm Hg in t0, 112 mm Hg in t2 [p < 0.001]), whereas those treated with milrinone had a significant decrease in mean arterial pressure over treatment (94 mm Hg in t0, 88 mm Hg in t2 [p = 0.004]). Among all treated patients, there was a significant drop from t0 to t2 but not to t1 in the highest MFV and in the highest mean MFV. Among those treated with HI, there were no significant changes from t0 to t1 or t2 (highest MFV in t0 163.2 cm/s, in t1 172.9 cm/s [p = 0.27], and in t2 164 cm/s [p = 0.936]). Conversely, in those treated with milrinone, there was a significant decrease from t0 to t1 and to t2 (highest MFV in t0 197.1 cm/s, in t1 172.8 cm/s [p = 0.012], in t2 159 cm/s [p = 0.002]). Regarding clinical outcomes, we observed a significant improvement in mean National Institutes of Health Stroke Scale scores from 17 to 16 in t1 (p < 0.001) and to 15 in t2 (p = 0.002). CONCLUSIONS: BFV analyzed by TCD in patients with aSAH who developed DCI and were treated with milrinone or NE significantly decreased in a time-dependent way. Milrinone effectively decrease cerebral BFV, whereas NE do not. Clinical improvement was achieved with both treatment strategies.

Waveform Morphology as a Surrogate for ICP Monitoring: A Comparison Between an Invasive and a Noninvasive Method.

BACKGROUND: Although the placement of an intraventricular catheter remains the gold standard technique for measuring intracranial pressure (ICP), the method has several limitations. Therefore, noninvasive alternatives to ICP (ICPni) measurement are of great interest. The main objective of this study was to compare the correlation and agreement of wave morphology between ICP (standard intraventricular ICP monitoring) and a new ICPni monitor in patients admitted with stroke. The second objective was to estimate the discrimination of the noninvasive method to detect intracranial hypertension. METHODS: We prospectively collected data of adults admitted to an intensive care unit with subarachnoid hemorrhage, intracerebral hemorrhage, or ischemic stroke in whom an invasive ICP monitor was placed. Measurements were simultaneously collected from two parameters [time-to-peak (TTP) and the ratio regarding the second and first peak of the ICP wave (P2/P1 ratio)] of ICP and ICPni wave morphology monitors (Brain4care). Intracranial hypertension was defined as an invasively measured sustained ICP > 20 mm Hg for at least 5 min. RESULTS: We studied 18 patients (subarachnoid hemorrhage = 14; intracerebral hemorrhage = 3; ischemic stroke = 1) on 60 occasions with a median age of 52 ± 14.3 years. A total of 197,400 waves (2495 min) from both ICP (standard ICP monitoring) and the ICPni monitor were sliced into 1-min-long segments, and we determined TTP and the P2/P1 ratio from the mean pulse. The median invasively measured ICP was 13 (9.8-16.2) mm Hg, and intracranial hypertension was present on 18 occasions (30%). The correlation and agreement between invasive and noninvasive methods for wave morphology were strong for the P2/P1 ratio and moderate for TTP using categoric (κ agreement 88.1% and 71.3%, respectively) and continuous (intraclass correlation coefficient 0.831 and 0.584, respectively) measures. There was a moderate but significant correlation with the mean ICP value (P2/P1 ratio r = 0.427; TTP r = 0.353; p < 0.001 for all) between noninvasive and invasive techniques. The areas under the curve to estimate intracranial hypertension were 0.786 [95% confidence interval (CI) 0.72-0.93] for the P2/P1 ratio and 0.694 (95% CI 0.60-0.74) for TTP. CONCLUSIONS: The new ICPni wave morphology monitor showed a good agreement with the standard invasive method and an acceptable discriminatory power to detect intracranial hypertension. Clinical trial registration Trial registration: NCT05121155.

Symptomatic Cerebral Vasospasm and Delayed Cerebral Ischemia Following Transsphenoidal Resection of a Craniopharyngioma.

BACKGROUND: Vasospasm has been rarely described as a complication associated with craniopharyngioma surgery. Herein we describe a patient who developed symptomatic vasospasm and delayed cerebral ischemia after transsphenoidal surgery for a craniopharyngioma. CASE REPORT: A 67-year-old woman became drowsy 2 weeks after a transsphenoidal resection of a craniopharyngioma. A head computed tomography (CT) was unremarkable except for postoperative findings. Electroencephalogram and laboratory studies were within the normal limits. A repeated CT scan 48 hours after the initial symptoms showed bilateral infarcts in the territory of the anterior cerebral arteries (ACA). Transcranial Doppler (TCD) showed increased blood flow velocities in both anterior cerebral arteries (169 cm/second in the left ACA and 145 cm/second in the right ACA) and right middle cerebral artery (164 cm/second) compatible with vasospasm. A CT angiography confirmed the findings. She was treated with induced hypertension and her level of consciousness improved. TCD velocities normalized after 2 weeks. CONCLUSION: Cerebral vasospasm should be considered in the differential diagnosis of patients with altered neurologic status in the postoperative period following a craniopharyngioma resection.

Cerebral Vasospasm and Delayed Cerebral Ischemia after Warfarin-Induced Subarachnoid Hemorrhage.

BACKGROUND: Subarachnoid hemorrhage (SAH) associated with anticoagulation is a rare event. About 30% of in-hospital patients with aneurysmal SAH develop delayed cerebral ischemia (DCI); however, the occurrence of vasospasm and DCI in patients with nonaneurysmal SAH is still controversial. METHODS: This study is a case report of a patient experiencing an anticoagulation-induced SAH complicated by vasospasm and DCI. RESULTS: A 38-year-old woman presented to our hospital with a sudden onset of severe headache. Head computed tomography (CT) showed bleeding in the posterior fossa subarachnoid space. There was no aneurysm in the CT angiography. The International Normalized Ratio at hospital admission was 9. Anticoagulation was held, and she was treated with fresh frozen plasma (15 mg/kg) and parenteral vitamin K. The patient remained in the intensive care unit and had daily transcranial Doppler (TCD) monitorization. TCD examination detected increased blood flow velocities in the basilar and vertebral arteries, meeting criteria for definitive vasospasm. CT angiography confirmed the presence of posterior circulation vasospasm. Magnetic resonance (MR) imaging 2 weeks after the bleeding showed a small area of restricted diffusion in the left superior cerebellar artery territory. MR angiography showed resolution of the vasospasm at this time point and TCD velocities normalized. CONCLUSIONS: In conclusion, this case report suggests that vasospasm and consequent DCI is a possible mechanism of secondary lesion after anticoagulation-induced SAH. To our knowledge, this is the first report of vasospasm and DCI due to warfarin-associated SAH.

A Brazilian series utilizing the SMASH-U system for etiologic classification of intracerebral hemorrhage.

BACKGROUND: Understanding the causes of intracerebral hemorrhage (ICH) is crucial for effective treatment and preventing recurrences. The SMASH-U scale is a suggested method for classifying and predicting the outcomes of ICH. OBJECTIVE: To describe the SMASH-U classification and outcomes by etiology in patients admitted to a comprehensive stroke center in São Paulo, Brazil. METHODS: A retrospective analysis was conducted on patients admitted to the hospital or outpatient clinic between April 2015 and January 2018. Two stroke neurologists evaluated the SMASH-U classification, and patients with incomplete medical records were excluded. RESULTS: Out of the 2000 patients with a stroke diagnosis evaluated, 140 were included in the final analysis. The mean age was 57.9 (± 15.5) years, and 54.3% were male. Hypertension was the most frequent etiology, accounting for 41.4% of cases, followed by amyloid angiopathy (18.5%) and structural lesions (14.1%). Structural lesions were more common among women and patients under 45 years old. Favorable outcomes were observed in 61% of patients with structural lesions, compared to 10% of patients with medication-related etiologies. CONCLUSION: This study provides important evidence regarding the etiological classification of Brazilian patients with ICH. Hypertension and amyloid angiopathy were the most frequent causes, while structural lesions and systemic diseases were more common in younger patients.

ANTECEDENTES: Compreender as causas da hemorragia intracerebral (HIC) é crucial para o tratamento eficaz e prevenção de recorrências. A escala SMASH-U é um método sugerido para classificar e prever os resultados da HIC. OBJETIVO: Descrever a classificação SMASH-U e os resultados por etiologia em pacientes admitidos em um centro de acidente vascular cerebral (AVC) em São Paulo, Brasil. MéTODOS: Foi realizada uma análise retrospectiva de pacientes admitidos no hospital ou ambulatório entre abril de 2015 e janeiro de 2018. Dois neurologistas especializados em doenças cerebrovasculares avaliaram a classificação SMASH-U e pacientes com prontuários incompletos foram excluídos. RESULTADOS: Dos 2000 pacientes com diagnóstico de AVC avaliados, 140 foram incluídos na análise final. A idade média foi de 57,9 (±15,5) anos e 54,3% eram do sexo masculino. A hipertensão foi a etiologia mais frequente, correspondendo a 41,4% dos casos, seguida pela angiopatia amiloide (18,5%) e lesões estruturais (14,1%). As lesões estruturais foram mais comuns em mulheres e pacientes com menos de 45 anos. Resultados favoráveis foram observados em 61% dos pacientes com lesões estruturais, em comparação com 10% dos pacientes com etiologias relacionadas a medicamentos. CONCLUSãO: Este estudo fornece evidências importantes sobre a classificação etiológica de pacientes brasileiros com HIC. A hipertensão e a angiopatia amiloide foram as causas mais frequentes, enquanto lesões estruturais e doenças sistêmicas foram mais comuns em pacientes mais jovens.

The essential can be invisible to the eyes: the "fogging effect" phenomenon in the subacute stage of ischemic stroke.

Cerebral infarcts can be overlooked or grossly underestimated in the second and third week after an ischemic stroke. We report a patient who presented with a vanishing stroke on a follow-up brain computed tomography scan, a condition known as the "fogging effect" phenomenon.

Multimodal monitoring intracranial pressure by invasive and noninvasive means.

Although the placement of an intraventricular catheter remains the gold standard method for the diagnosis of intracranial hypertension (ICH), the technique has several limitations including but not limited to its invasiveness. Current noninvasive methods, however, still lack robust evidence to support their clinical use. We aimed to estimate, as an exploratory hypothesis generating analysis, the discriminative power of four noninvasive methods to diagnose ICH. We prospectively collected data from adult intensive care unit (ICU) patients with subarachnoid hemorrhage (SAH), intraparenchymal hemorrhage (IPH), and ischemic stroke (IS) in whom invasive intracranial pressure (ICP) monitoring had been placed. Measures were simultaneously collected from the following noninvasive methods: optic nerve sheath diameter (ONSD), pulsatility index (PI) using transcranial Doppler (TCD), a 5-point visual scale designed for brain Computed Tomography (CT), and two parameters (time-to-peak [TTP] and P2/P1 ratio) of a noninvasive ICP wave morphology monitor (Brain4Care[B4c]). ICH was defined as a sustained ICP > 20 mmHg for at least 5 min. We studied 18 patients (SAH = 14; ICH = 3; IS = 1) on 60 occasions with a mean age of 52 ± 14.3 years. All methods were recorded simultaneously, except for the CT, which was performed within 24 h of the other methods. The median ICP was 13 [9.8-16.2] mmHg, and intracranial hypertension was present on 18 occasions (30%). Median values from the noninvasive techniques were ONSD 4.9 [4.40-5.41] mm, PI 1.22 [1.04-1.43], CT scale 3 points [IQR: 3.0], P2/P1 ratio 1.16 [1.09-1.23], and TTP 0.215 [0.193-0.237]. There was a significant statistical correlation between all the noninvasive techniques and invasive ICP (ONSD, r = 0.29; PI, r = 0.62; CT, r = 0.21; P2/P1 ratio, r = 0.35; TTP, r = 0.35, p < 0.001 for all comparisons). The area under the curve (AUC) to estimate intracranial hypertension was 0.69 [CIs = 0.62-0.78] for the ONSD, 0.75 [95% CIs 0.69-0.83] for the PI, 0.64 [95%Cis 0.59-069] for CT, 0.79 [95% CIs 0.72-0.93] for P2/P1 ratio, and 0.69 [95% CIs 0.60-0.74] for TTP. When the various techniques were combined, an AUC of 0.86 [0.76-0.93]) was obtained. The best pair of methods was the TCD and B4cth an AUC of 0.80 (0.72-0.88). Noninvasive technique measurements correlate with ICP and have an acceptable discrimination ability in diagnosing ICH. The multimodal combination of PI (TCD) and wave morphology monitor may improve the ability of the noninvasive methods to diagnose ICH. The observed variability in non-invasive ICP estimations underscores the need for comprehensive investigations to elucidate the optimal method-application alignment across distinct clinical scenarios.

Acute treatment costs of stroke in Brazil.

BACKGROUND AND PURPOSE: Although stroke is the leading cause of death in Brazil, little information exist on the acute treatment provided for stroke and its associated costs. This study addresses this gap by both clinically and economically characterizing the acute treatment of first-ever intracerebral hemorrhage (ICH) and ischemic stroke (IS) in Brazil. METHODS: Retrospective medical chart review using data from two high-volume stroke centers in São Paulo, Brazil. Clinical and resource utilization data for all patients admitted to the stroke centers with a first-ever stroke between January 1, 2006 and May 31, 2007 were collected and the mean acute treatment costs per person were calculated by assigning appropriate unit cost data to all resource use. Cost estimates in Brazilian reals (BRL) were converted to US dollars (USD) using the 2005 purchasing power parity index. National costs of acute treatment for incident strokes were estimated by extrapolation of mean cost estimate per person to national incidence data for the two types of stroke. The mean costs of acute treatment on a national scale were examined in sensitivity analysis. RESULTS: A total of 316 stroke patients were identified and their demographic and clinical characteristics, patterns of care, and outcomes were examined. Mean length of hospital stay was 12.0 +/- 8.8 days for ICH and 13.3 +/-23.4 days for IS. Ninety-one percent of the ICH patients and 68% of the IS patients were admitted to an intensive care unit (ICU). Mean total costs of initial hospitalization were USD 4,101 (SD +/-4,254) for ICH and USD 1,902 (SD +/-1,426) for IS. In multivariate analysis, hemorrhagic stroke, development of pneumonia, neurosurgical intervention, stay in ICU, and physical therapy were all significant independent predictors of acute treatment costs. Aggregate national health care expenditures for acute treatment of incident ICH were USD 122.4 million (range 30.8-274.2) and USD 326.9 million for IS (range 82.4-732.2). CONCLUSION: Acute treatment costs of incident ICH and IS in Brazil are substantial and primarily driven by the intensity of hospital treatment and in-hospital complications. With the expected increase in the incidence of stroke in Brazil over the coming decades, these results emphasize the need for effective preventive and acute medical care.

A Brazilian series utilizing the SMASH-U system for etiologic classification of intracerebral hemorrhage / Classificação etiológica de pacientes admitidos com hemorragia intracerebral utilizando a escala SMASH-U: uma série brasileira

Abstract Background Understanding the causes of intracerebral hemorrhage (ICH) is crucial for effective treatment and preventing recurrences. The SMASH-U scale is a suggested method for classifying and predicting the outcomes of ICH. Objective To describe the SMASH-U classification and outcomes by etiology in patients admitted to a comprehensive stroke center in São Paulo, Brazil. Methods A retrospective analysis was conducted on patients admitted to the hospital or outpatient clinic between April 2015 and January 2018. Two stroke neurologists evaluated the SMASH-U classification, and patients with incomplete medical records were excluded. Results Out of the 2000 patients with a stroke diagnosis evaluated, 140 were included in the final analysis. The mean age was 57.9 (± 15.5) years, and 54.3% were male. Hypertension was the most frequent etiology, accounting for 41.4% of cases, followed by amyloid angiopathy (18.5%) and structural lesions (14.1%). Structural lesions were more common among women and patients under 45 years old. Favorable outcomes were observed in 61% of patients with structural lesions, compared to 10% of patients with medication-related etiologies. Conclusion This study provides important evidence regarding the etiological classification of Brazilian patients with ICH. Hypertension and amyloid angiopathy were the most frequent causes, while structural lesions and systemic diseases were more common in younger patients.

Resumo Antecedentes Compreender as causas da hemorragia intracerebral (HIC) é crucial para o tratamento eficaz e prevenção de recorrências. A escala SMASH-U é um método sugerido para classificar e prever os resultados da HIC. Objetivo Descrever a classificação SMASH-U e os resultados por etiologia em pacientes admitidos em um centro de acidente vascular cerebral (AVC) em São Paulo, Brasil. Métodos Foi realizada uma análise retrospectiva de pacientes admitidos no hospital ou ambulatório entre abril de 2015 e janeiro de 2018. Dois neurologistas especializados em doenças cerebrovasculares avaliaram a classificação SMASH-U e pacientes com prontuários incompletos foram excluídos. Resultados Dos 2000 pacientes com diagnóstico de AVC avaliados, 140 foram incluídos na análise final. A idade média foi de 57,9 (±15,5) anos e 54,3% eram do sexo masculino. A hipertensão foi a etiologia mais frequente, correspondendo a 41,4% dos casos, seguida pela angiopatia amiloide (18,5%) e lesões estruturais (14,1%). As lesões estruturais foram mais comuns em mulheres e pacientes com menos de 45 anos. Resultados favoráveis foram observados em 61% dos pacientes com lesões estruturais, em comparação com 10% dos pacientes com etiologias relacionadas a medicamentos. Conclusão Este estudo fornece evidências importantes sobre a classificação etiológica de pacientes brasileiros com HIC. A hipertensão e a angiopatia amiloide foram as causas mais frequentes, enquanto lesões estruturais e doenças sistêmicas foram mais comuns em pacientes mais jovens.

Clinical features associated with early hospital arrival after acute intracerebral hemorrhage: challenges for new trials.

BACKGROUND: Early hospital admission followed by correct diagnosis with minimum delay is a prerequisite for successful new interventions in acute intracerebral hemorrhage (ICH). The aim of this study was to evaluate clinical features associated with early hospital arrival in ICH patients and their influence on the outcome. METHODS: Data from all patients arriving within 24 h of the ICH onset were prospectively collected at 2 stroke centers in São Paulo, Brazil. The cutoff of 3 h was chosen to select 2 groups: 0-3 h (early) and >3-24 h (late). RESULTS: We identified 91 ICH patients (mean age 57.9 years, 62% men, 63% white) admitted within the first 24 h of symptom onset between March 2004 and April 2005. Systolic blood pressure, mean arterial pressure and pulse pressure were significantly higher in patients arriving within 3 h. Patients that arrived early also had a higher NIHSS score (p = 0.003), a lower Glasgow Coma Score (p = 0.001) and presence of intraventricular hemorrhage (p = 0.02). Lower ICH scores were more frequent in those that arrived late. Fourteen patients showed hematoma enlargement and the majority of them (n = 13) were admitted within the first 3 h from symptom onset (p = 0.01). Patients who arrived within the 3-hour window had a higher 30-day mortality (p = 0.0008) and a worse Rankin score after 6 months (p = 0.001). CONCLUSIONS: Treatment decisions in acute ICH may need to establish new combined approaches to maximize the number of eligible patients for early therapy considering the interactions between independent outcome predictors presented at early onset.

[Spontaneous carotid and vertebral arteries dissection in a multiethnic population]. / Dissecção espontânea das artérias carótidas e vertebrais em uma população multiétnica.

Spontaneous dissection of the carotid and vertebral arteries (SDCVA) is considered a rare cause of stroke, particularly in countries with multiethnic population. The objective was to evaluate the clinical and neuroimaging features of patients with SDCVA from a multiethnic population. Sixty-six patients diagnosed with SDCVA were studied at two tertiary hospitals at São Paulo. An initial questionnaire was completed and patients were followed prospectively. Among the patients studied, 82% were caucasian, 53% were male and the average age was 41.7 years old. The most frequent cardiovascular risk factors found were systemic hypertension and tobacco use. Other aspects evaluated were history of previous migraine, initial treatment and prognosis. In conclusion, although the population studied was multiethnic, there was a marked predominance of caucasians. The analysis of clinical and neuroimaging data from patients with SDCVA allows a better understanding of the disease, leading to an earlier diagnosis and more appropriate treatment.

Statin and fibrate associated myopathy: study of eight patients.

Lipid-lowering drugs have been occasionally associated with neuromuscular symptoms and muscle biopsy changes. We reported the clinical course and the muscle biopsy in eight patients with hyperlipoproteinemia, treated with lipid -lowering drugs (statins/fibrates). Five patients had myalgias while; in two cases there was proximal muscle weakness. All patients became asymptomatic after the withdrawal of the drug, although creatine kinase remained elevated. We performed muscle biopsy in six cases from three months to two years after suspension of the drug. We found variation in fibers diameters in all cases, with necrosis of fibers in five cases, inflammatory infiltration in one case, the presence of vacuolated fiber in one patient and ragged-red fibers in three subjects. We concluded that although the muscle biopsy findings were not specific, the prolonged use of statins and or fibrates might induce a chronic myopathy even in the absence of symptoms.

[Brazilian guidelines for the manegement of intracerebral hemorrhage]. / Diretrizes para o manejo de pacientes com hemorragia intraparenquimatosa cerebral espontânea.

Among the stroke subtypes, intracerebral hemorrhage (ICH) has the worst prognosis and still lacks a specific treatment. The present manuscript contains the Brazilian guidelines for the management of ICH. It was elaborated by the executive committee of the Brazilian Cerebrovascular Diseases Society and was based on a broad review of articles about the theme. The text aims to provide a rational for the management of patients with an acute ICH, with the diagnostic and therapeutic resources that are available in Brazil.

Dissecção espontânea das artérias carótidas e vertebrais em uma população multiétnica / Spontaneous carotid and vertebral arteries dissection in a multiethnic population

A dissecção espontânea das artérias carótidas e vertebrais (DEACV) é considerada uma causa rara de acidente vascular cerebral, particularmente em países com população multiétnica. O objetivo desse estudo foi avaliar características clínicas e de neuroimagem dos pacientes com DEACV em uma população multiétnica. Foram estudados 66 pacientes com diagnóstico de DEACV em dois hospitais terciários de São Paulo. Aplicou-se um questionário inicial e os pacientes foram seguidos prospectivamente. Dos pacientes estudados, 82 por cento eram brancos, 53 por cento eram homens e a média de idade foi 41,7 anos. Os fatores de risco cardiovasculares mais freqüentes foram hipertensão arterial e tabagismo. Outros aspectos avaliados foram história prévia de enxaqueca, tratamento inicial e prognóstico. Concluiu-se que apesar da população estudada ser multiétnica, houve um marcante predomínio de brancos. A análise das características clínicas e de neuroimagem dos pacientes com DEACV possibilita um melhor conhecimento da doença, levando a um diagnóstico precoce e tratamento mais adequado.

Spontaneous dissection of the carotid and vertebral arteries (SDCVA) is considered a rare cause of stroke, particularly in countries with multiethnic population. The objective was to evaluate the clinical and neuroimaging features of patients with SDCVA from a multiethnic population. Sixty-six patients diagnosed with SDCVA were studied at two tertiary hospitals at São Paulo. An initial questionnaire was completed and patients were followed prospectively. Among the patients studied, 82 percent were caucasian, 53 percent were male and the average age was 41.7 years old. The most frequent cardiovascular risk factors found were systemic hypertension and tobacco use. Other aspects evaluated were history of previous migraine, initial treatment and prognosis. In conclusion, although the population studied was multhiethnic, there was a marked predominance of caucasians. The analysis of clinical and neuroimaging data from patients with SDCVA allows a better understanding of the disease, leading to an earlier diagnosis and more appropriate treatment.

Statin and fibrate associed myopathy: study of eight patients

As drogas redutoras de colesterol são ocasionalmente associadas a sintomas neuromusculares e alterações morfológicas observadas na biopsia muscular. Relatamos o curso clínico e achado da biopsia muscular em oito pacientes com hiperlipoproteinemia tratados com drogas redutoras de colesterol (estatinas/fibratos). Cinco pacientes tiveram mialgia e em dois havia fraqueza muscular proximal. Todos os pacientes ficaram assintomáticos após retirada da medicação embora a creatinoquinase permanecesse elevada. Analisamos a biopsia muscular em seis casos realizados entre três meses e dois anos após a suspensão da droga. Encontramos variação no calibre das fibras em todos os casos com necrose de fibras em cinco, infiltrado inflamatório em um caso, presença de vacúolos em um e "ragged red fiber" em três deles. Concluímos que, embora os achados da biopsia muscular não fossem específicos, o uso prolongado de estatinas e/ou fibratos pode induzir a uma miopatia crônica até mesmo na ausência de sintomas.