Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.
-Right-to-left shunt (RLS) can be identified by contrast-enhanced transcranial Doppler (cTCD) in patent foramen ovale (PFO) patients. Aim: To evaluate cTCD for PFO screening comparing it to cTEE. Method: 45 previous cTCD performed for PFO diagnosis and correlated its findings with cTEE. Patients were submitted to a cTCD standardized technique and were divided in two groups according to RLS: Group 1, patients with a positive RLS and Group 2 when RLS was negative. Results: 29 (65%) patients were included in group 1 and 16 (35%) in group 2. PFO confirmation by cTEE was performed in 28 (62%) patients. cTCD had a 92.85% sensitivity, 82.35% specificity, 89.65% positive predictive value and 87.5% negative predictive value when compared to cTEE for PFO diagnosis. Conclusion: Standardized technique cTCD allows for RLS visualization in PFO patients with a good correlation with cTEE and can be used as a screening test before cTEE.KEy wORDS: patent foramen ovale, transesophageal echocardiography, transcranial Doppler, ultrasonography.
Doppler transcraniano contrastado para triagem de forame oval patente: existe boa correlação com ecocardiograma transesofágico?Resumo -A comunicação direita-esquerda (CDE) pode ser identificada por Doppler transcraniano contrastado (DTCc) em pacientes com forame oval patente (FOP Patent foramen ovale (PFO) is a congenital heart disease characterized by an opening between the right and left atria resulting from incomplete closure of the ostium secundum by the septum secundum 1 . Recent studies have found an increase prevalence of PFO in women with migraine with aura and young adults (less than 55 years old) with so-called "cryptogenic" ischemic stroke [2][3][4][5][6] . Emboli from the venous system can cross the PFO reaching the arterial circulation through a right-to-left shunt (RLS) and thus leading to a stroke. PFO diagnosis is done by using a contrast-enhanced technique (by injecting saline solution in a peripheral vein) while performing a transesophageal echocardiography (cTEE) and, when positive, it shows a high correlation with necropsy studies 7 . In spite of both high sensibility and specificity, cTEE have some limitations, such as expensiveness, patient's low tolerability and imperfect intra and inter-observer correlation, due to the fact that it is an operator-dependent method 8 . A great ad-
Epilepsy causes restrictions in the performance of various daily activities. The aiming of this study was to investigate whether these restrictions affect the perceived quality of life. The assessments Quality of Life in Epilepsy-31 (QOLIE-31) and Canadian Occupational Performance Measure (COPM) were applied in a sample that consisted of a single group of 34 subjects with at least two years of uncontrolled seizures. The results indicated that the most affected domains of QOLIE-31 were seizure worry, 29.77 (±21.72), and effects of drugs, 49.75 (±28.58), and for the COPM, the average of performance and satisfaction were respectively 3.10 (±3.07) and 4.45 (±3.29), and performance limitations most frequently cited were maintain employment (18), left home alone (15) and courses (15). The application of the Spearman correlation coefficient showed that the three main performance limitations posed by the COPM, especially regarding the level of satisfaction, influence the perception of quality of life. Thus, occupational performance proves to be an important area of intervention with subjects with epilepsy.
A young female patient with chronic renal failure due to a systemic vasculitis and a parkinsonian syndrome secondary to brain vasculitis, most likely systemic lupus erythematosus, is described. The patient had a dramatic response to a pulse of methylprednisolone, with remission of her parkinsonian symptoms.
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