Acta neurologica Taiwanica最新文献

Although it is known that Fabry disease should be included in the differential diagnosis of young stroke and fever of unknown origin, it has not been reported in the literature of stroke with fever as the presentation of Fabry disease. This is relevant because stroke with fever may misguide the differential diagnosis. Here we present a young stroke patient with fever. DWI of brain MRI revealed acute multiple infarctions. Due to the fever, infectious and inflammatory origins such as infective endocarditis and autoimmune diseases were examined first. However, we could not identify the source of fever after fever workup. The fever did not respond to one week of acetaminophen and antibiotics, but responded promptly to steroid. Our patient is also a case of de novo mutation rather than being inherited that further complicates the diagnosis of this patient. Because of the rare combined presentation of stroke with fever, Fabry disease should also be considered in stroke with fever, even without family history of Fabry disease.

ation. No significant past medical history except for pharmacologically controlled mild hypertension. During the neurological examination the patient appeared alert, oriented and showed no deficit of strength, sensitivity and coordination. An expressive temporary aphasia was confirmed and Transient Ischemic Attacks (TIA) was suspected. Cranial Computed Tomography Angiography (CTA) showed hypoplasia of left Internal Carotid Artery (ICA) with a focal duplication in the intracavernous segment (Figure 1, arrowhead). Circle of Willis appears to be regular, with the left middle cerebral artery supported by the vertebrobasilar system through the left posterior communicating artery of increased caliber, and by the contralateral ICA via anterior communicating artery. Consequently, patient underwent Digital Subtraction Angiography (DSA) lateral view that confirmed the segmental duplication of the left intracavernous ICA (Figure 2, arrowhead). Vessels had a lightly winding course within the cavernous sinus and the intracranial branches downstream of their confluence presented a slight delay in visualization. After few hours her symptoms completely regressed and, in agreement with clinical and imaging data, diagnosis of TIA was made. The transient ischemic event was, in fact, most likely caused by low flow to the left cerebral hemisphere due to hypoplasia of the ICA and aforementioned abnormalities of its intracavernous tract that caused alteration and reduction of intracranial flow distribution. Medical treatment with anti-platelet drugs was started and patient was discharged with a clinical, laboratory and imaging follow-up program. Duplications of ICA in the intracranial tract are very rare(1,2). Most of the cases are localized in the supraclinoid segment. To our knowledge this is the first case described in literature of true duplication of ICA in the intracavernous tract. Patients with congenital variants or acquired pathology of ICA are mostly asymptomatic, but when symptoms appear, patients must be investigated(1-3). CTA is considered the first line non-invasive diagnostic method for intracranial vascular anatomy. At present, medical treatment remains the choice in patients with no-complicated duplications of ICA in the intracranial tract.

Purpose: Appropriate dosing of direct oral anticoagulants (DOACs) requires consideration of renal function. Discordance between commonly used estimated glomerular filtration rate (eGFR) and creatinine clearance (CrCl) might affect the dosing appropriateness in stroke patients with atrial fibrillation (AF). We aimed to explore the effect of renal function estimates on the dosing patterns in a real-world setting.

Methods: Using a hospital-based stroke registry, we identified consecutive patients between 2014 and 2017 who were hospitalized for acute stroke, had AF, and started DOACs within 90 days after stroke. We compared the difference between eGFR and CrCl in assessing appropriateness of dosage. Effectiveness and safety outcomes were verified by chart review, and event rates were presented as per 100 person-years.

Results: Of the156 patients with mean age 74±11 years, 72 (46%) were prescribed dabigatran and 84 (54%) rivaroxaban. Substituting eGFR for CrCl would have 55% (37/67) of patients with CrCl less than 50 mL/min and 89% (8/9) of patients with CrCl less than 30 mL/min not correctly classified, and potentially lead to overdosing. The misclassification would cause underdosing in 6% (5/89) of patients with CrCl ≥50 mL/min and 1% (1/147) of patients with CrCl ≥30 mL/min. In reality, the substitution resulted in reduction of overdosing from 10% to 4% for dabigatran and from 2% to 1% for rivaroxaban; underdosing increased from 17% to 26% for rivaroxaban. After median follow-up of 17 months, 33 patients developed outcomes including 21 major bleedings. The event rate was 6.9% per year (95% CI, 4.1%-11.4%) for effectiveness, and 9.6% per year (95% CI, 6.3%-14.8%) for safety.

Conclusion: Although substituting eGFR for CrCl carries potential risks of DOAC overdosing in patients with AF, the effect might be offset by clinicians' predilection for lower dosage in this stroke cohort.

Purpose: Patients with end-stage renal disease (ESRD) have higher risks of subdural hemorrhage (SDH) and subsequent 30-day mortality. However, evidences regarding optimal mode of dialysis therapy during acute management are sparse. We aimed to compare the outcomes of ESRD patients who received continuous peritoneal dialysis (CPD) or extended hemodialysis (EHD) after SDH and determined factors associated with 30-day mortality.

Methods: We retrospectively reviewed consecutive patients with SDH and ESRD in a medical center. The clinical parameters and outcomes were compared between CPD and EHD groups. Factors associated with 30-day mortality were analyzed.

Results: We reviewed 32 patients, including 22 received EHD, 8 received CPD, and 2 received continuous veno-venous hemodialysis. Neurosurgery was done in 9 (28%) of them. There was no significant difference in baseline parameters and outcomes between EHD and CPD groups. The overall 30-day mortality rate was 19%. Lower Glasgow coma scale (GCS, median [interquartile range]: 10 [7-12] vs. 15 [11-15], p = 0.02) and larger changes in absolute mean arterial pressure (MAP: 26.5 [10.5-46.0] vs. 7.5 [2.0-17.8] mmHg, p = 0.01) during the first dialysis therapy were noted in patients with 30-day mortality. In multivariate analysis, consciousness disturbance at presentation was an independent risk factor for 30-day mortality.

Conclusion: Among ESRD patients with SDH, the 30-day mortality rates were similar between EHD and CPD groups. MAP change during dialysis might be an important modifiable risk factor for 30-day mortality, though the effect was not significant in multivariate analysis. Further prospective studies with larger sample size are warranted.

Objectives: Social Stigma is potentially a major problem in multiple sclerosis patients which strongly affects the quality of life. The aim of this study was to determine the prevalence of perceived stigma in patients with MS in a sample of Iranian population.

Methods: This cross-sectional study was performed on 305 MS patients who were referred to Iranian Multiple Sclerosis Society in 2014. The main variables were perceived stigma, age, sex, marital status, educational level, occupation, duration of the disease, type of symptoms, disability expanded status scale, family history and economic status. Social stigma was measured through a 20-item questionnaire which was developed by authors and the reliability was assessed in a pilot study.

Results: The frequency of perceived stigma was significantly associated with occupation, disease duration, and visibility of symptoms, level of disability and the economic condition. There were no significant relationships between perceived stigma and age, sex, marital status, level of education and family history. About 44 percent of patients preferred to hide their disease from others and 52.6 percent believed that this disease would stigmatize them in society. Nearly, half of patients preferred not to mention their disease in job interviews.

Conclusion: Recognition of the impacts of perceived stigma on various aspects of the patients' lives are necessary to find appropriate strategies to deal with stigma and its consequences. Training programs can improve the patients' skills for coping with stigma. Furthermore, programs aimed to upgrade public knowledge and reduce the negative attitudes toward the disease should be promoted.

Purpose: Significant improvement of stroke symptoms may occur in acute stroke patients due to large artery occlusion (LAO) treated with intravenous thrombolysis (IVT). Directly proceeding with endovascular thrombectomy (EVT) would be a clinical dilemma.

Case report: A 55-year-old male suddenly suffered from global aphasia and right side hemiplegia at work. Left proximal middle cerebral artery (MCA) occlusion was shown on CT angiography. After IVT, NIHSS significantly improved from 11 to 4 points. EVT was withheld due to rapidly improving symptoms and low NIHSS scores. However, stroke in progression occurred 7 hours after onset with NIHSS scores increasing from 4 to 13 points. A follow-up brain CT scan showed an Alberta Stroke Program Early CT score of 9 and no hemorrhage. Successful recanalization of the left MCA by EVT was performed at 10.5 hours after onset. The patient was discharged with a NIHSS score of 3 and his modified Rankin Scale score was 0 at 3 months.

Conclusion: In LAO patients, directly proceeding EVT following IVT may not be hesitated. In addition, while LAO patients with incomplete IV-tPA treatment responses result in transient improvement of neurological symptoms but later reversed, EVT may be a potential rescue therapy in carefully selected patients.

Purpose: Hemiballism caused by hypoglycemia is rare. We presented a case who suffered from episodic hemiballism induced by hypoglycemia with spontaneously recovery after sleep. The possible mechanism of these self-limited episodes was also discussed.

Case report: An 82-year-old female diabetic patient took oral anti-diabetic drugs (OADs) regularly. The doctor changed OADs doses and her appetite became poor before admission. She suffered from episodic left side involuntary movements with consciousness disturbance, and recovered spontaneously after a six-to-eight hour sleep in every attack at home. Very low finger sugar (20 mg/ dl) was noted while attack at admission. Brain computed tomography (CT), magnetic resonance imaging (MRI) and electroencephalography (EEG) were non-remarkable. Brain technetium-99mlabeled ethyl cysteinate dimer single-photon emission computed tomography (Tc-99m-ECD SPECT) showed relative hyperperfusion over right side basal ganglion and thalamus. No further involuntary movement was observed after better sugar control.

Conclusion: We suppose that sleep modify the glucose counterregulatory responses with increased growth hormone, which salvage hypoglycemic status in our presented case. With this report, we would like to draw clinicians' attention to including the treatable hypoglycemia state in the differential diagnosis of episodic involuntary movements.

Purpose: To investigate the clinical characteristics, laboratory features and prognostic factors of elderly patients with postneurosurgical bacterial meningitis.

Methods: Five hundred and forty patients with adult bacterial meningitis (ABM) were collected from 1986-2015, of whom 167 were ≥ 65 years. Of these 167 elderly patients, 82 had postneurosurgical infections and 85 had spontaneous infections. Clinical, laboratory and therapeutic data of these two groups were compared.

Results: The 82 elderly ABM patients with postneurosurgical infections included 48 men and 34 women with a median age of 71 years (range: 65-84 years). In addition to the postneurosurgical condition, the other most common underlying conditions included diabetes mellitus (29.3%) and hydrocephalus (29.3%). The major clinical presentations were fever (80.5%), altered consciousness (50.0%), hydrocephalus (43.9%), seizure (24.4%) and septic shock (15.9%). Of the implicated pathogens, staphylococcal species (spp.) were the most common (31.7%), followed by Acinetobacter spp. (12.2%), Enterobacter spp. (7.3%), Pseudomonas spp. (7.3%), Enterococcus faecalis (7.3%) and Escherichia coli (6.1%). The implicated staphylococcal spp. had a high rate of non-susceptibility to methicillin (84.6%), and the implicated Acinetobacter spp. and Enterobacter spp. had non-susceptible rates to ceftazidime of 60% and 50%, respectively. The mortality rate was 28.1%, and septic shock was the most significant prognostic factor. Compared with the clinical characteristics of the other 85 elderly patients with spontaneous ABM, there were significant differences in underlying condition, clinical and laboratory features and therapeutic outcomes.

Conclusions: Elderly patients accounted for 30.9% of all cases of ABM, of whom 49.1% had postneurosurgical ABM. The clinical characteristics of the elderly patients with postneurosurgical ABM were non-specific, and cerebrospinal fluid studies were needed to confirm the diagnosis. The mortality rate of this group of patients was high, and septic shock was an important prognostic factor. The clinical and laboratory features and therapeutic outcomes were different between the elderly patients with postneurosurgical and spontaneous ABM.