Assessment of Patients with Intracerebral Hemorrhage or Hemorrhagic Transformation in the VENOST Study.

INTRODUCTION: Cerebral venous and sinus thrombosis (CVST) may lead to cerebral edema and increased intracranial pressure; besides, ischemic or hemorrhagic lesions may develop. Intracerebral hemorrhages occur in approximately one-third of CVST patients. We assessed and compared the findings of the cerebral hemorrhage (CH) group and the CVST group. MATERIALS AND METHODS: In the VENOST study, medical records of 1,193 patients with CVST, aged over 18 years, were obtained from 35 national stroke centers. Demographic characteristics, clinical symptoms, signs at the admission, radiological findings, etiologic factors, acute and maintenance treatment, and outcome results were reported. The number of involved sinuses or veins, localizations of thrombus, and lesions on CT and MRI scans were recorded. RESULTS: CH was detected in the brain imaging of 241 (21.1%) patients, as hemorrhagic infarction in 198 patients and intracerebral hemorrhage in 43 patients. Gynecologic causes comprised the largest percentage (41.7%) of etiology and risk factors in the CVST group. In the CH group, headache associated with other neurological symptoms was more frequent. These neurological symptoms were epileptic seizures (46.9%), nausea and/or vomiting (36.5%), altered consciousness (36.5%), and focal neurological deficits (33.6%). mRS was ≥3 in 23.1% of the patients in the CH group. DISCUSSION AND CONCLUSION: CVST, an important cause of stroke in the young, should be monitored closely if the patients have additional symptoms of headache, multiple sinus involvement, and CH. Older age and parenchymal lesion, either hemorrhagic infarction or intracerebral hemorrhage, imply poor outcome.

Behçet's disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study.

OBJECTIVE: This study was performed to determine the rate of cerebral venous sinus thrombosis (CVST) among cases of Behçet's disease (BD) included in a multicentre study of cerebral venous sinus thrombosis (VENOST). METHODS: VENOST was a retrospective and prospective national multicentre observational study that included 1144 patients with CVST. The patients were classified according to aetiologic factors, time of CVST symptom onset, sinus involvement, treatment approach and prognosis. RESULTS: BD was shown to be a causative factor of CVST in 108 (9.4%) of 1144 patients. The mean age of patients in the BD group was 35.27 years and 68.5% were men, whereas in the non-BD CVST group, the mean age was 40.57 years and 28.3% were men (P < 0.001). Among the aetiologic factors for patients aged 18-36 years, BD was predominant for men, and puerperium was predominant for women. The onset of symptoms in the BD group was consistent with the subacute form. The transverse sinuses were the most common sites of thrombosis, followed by the superior sagittal sinuses. The most common symptom was headache (96.2%), followed by visual field defects (38%). CONCLUSIONS: BD was found in 9.4% of patients in our VENOST series. Patients with BD were younger and showed a male predominance. The functional outcome of CVST in patients with BD was good; only 12% of patients presenting with cranial nerve involvement and altered consciousness at the beginning had a poor outcome (modified Rankin Score ⩾2).

Cerebral Venous Sinus Thrombosis as a Rare Complication of Systemic Lupus Erythematosus: Subgroup Analysis of the VENOST Study.

AIM: Systemic lupus erythematosus (SLE) is an unusual risk factor for cerebral venous sinus thrombosis (CVST). As few CVST patients with SLE have been reported, little is known regarding its frequency as an underlying etiology, clinical characteristics, or long-term outcome. We evaluated a large cohort of CVST patients with SLE in a multicenter study of cerebral venous thrombosis, the VENOST study, and their clinical characteristics. MATERIAL AND METHOD: Among the 1144 CVST patients in the VENOST cohort, patients diagnosed with SLE were studied. Their demographic and clinical characteristics, etiological risk factors, venous involvement status, and outcomes were recorded. RESULTS: In total, 15 (1.31%) of 1144 CVST patients had SLE. The mean age of these patients was 39.9 ± 12.1 years and 13 (86.7%) were female. Presenting symptoms included headache (73.3%), visual field defects (40.0%), and altered consciousness (26.7%). The main sinuses involved were the transverse (60.0%), sagittal (40.0%), and sigmoid (20.0%) sinuses. Parenchymal involvement was not seen in 73.3% of the patients. On the modified Rankin scale, 92.9% of the patients scored 0-1 at the 1-month follow-up and 90.9% scored 0-1 at the 1-year follow-up. CONCLUSIONS: SLE was found in 1.31% of the CVST patients, most frequently in young women. Headache was the most common symptom and the CVST onset was chronic in the majority of cases. The patient outcomes were favorable. CVST should be suspected in SLE patients, even in those with isolated chronic headache symptoms with or without other neurological findings.

Clinical and imaging features associated with intracranial internal carotid artery calcifications in patients with ischemic stroke.

INTRODUCTION: Intracranial internal carotid artery calcifications (ICAC), a frequent finding on imaging studies, are predictive of future stroke risk in population-based studies. The clinical significance of this observation among ischemic stroke patients is however less clear. In this study, we analyzed ICAC burden in relation to vascular risk factor profile, stroke etiology, and extent of craniocervical vascular calcifications in a consecutive series of ischemic stroke patients. METHODS: The burden of ICAC was determined both on non-contrast CT and CT-angiography source images by semiquantitative scoring algorithms. The distribution of vascular risk factors, etiologic stroke subtype, and calcification burden in other craniocervical arteries was assessed among patients with no ICAC, mild-moderate ICAC, and severe ICAC. RESULTS: Of 319 patients included into the study, 28 % had no ICAC, 35 % had mild-moderate ICAC, and 37 % had severe ICAC on CT angiography. Independent factors associated with ICAC burden in multivariate analysis included age (p < 0.001), diabetes mellitus (p = 0.006), and coronary artery disease (p < 0.001). Furthermore, a stroke etiology of large artery atherosclerosis or cardioaortic embolism was significantly related to higher ICAC burden (p = 0.006). Patients with severe ICAC were more likely to harbor calcifications in other vascular beds (p < 0.001). All of these findings persisted when analyses were repeated with CT-based ICAC burden assessments. CONCLUSION: ICAC burden reflects a continuum of atherosclerotic disease involving carotid arteries together with other craniocervical vascular beds. ICAC is significantly associated with stroke of large vessel or cardioembolic origin. This information might help the clinician in prioritizing etiologic work-up in the acute period.

Diabetic striatopathy in a patient with hemiballism.

Diabetic striatopathy is a rare and life-threatening manifestation of diabetes mellitus. The disease commonly affects individuals of Asian descent, females, and the elderly. Patients usually present with hemiballism-hemichorea caused by nonketotic hyperglycemia. Hemiballism-hemichorea is defined as involuntary continuous random appearing movement involving one side of the body. This movement disorder may develop secondary to stroke, diabetic striatopathy, neoplasm, infection, Wilson's disease, and thyrotoxicosis. Despite being rare, prompt recognition of a hyperglycemia-induced hemiballism-hemichorea is essential because the symptoms are reversible with correction of hyperglycemia. Diagnosis is possible based on blood analysis and neuroimaging findings. Laboratory tests reveal raised blood glucose and hemoglobin A1C levels which indicate poorly controlled diabetes. Neuroimaging provides suggestive findings of diabetic striatopathy which are hyperattenuation on computed tomography and hyperintensity on T1-weighted magnetic resonance imaging in the basal ganglia. In this case report, our aim is to present neuroimaging findings in an adult man with sudden onset of hemiballism associated with nonketotic hyperglycemia.

Can 3D radiological calculations predict operational difficulties for rectal cancer?: A single center retrospective analysis.

Low anterior resection, performing total mesorectal excision with appropriate pelvic dissection to prevent local recurrence, is probably the most challenging type of surgery in colorectal surgery, especially in a narrow pelvis. In this study, we aimed to predict the operation difficulty of rectal cancer by comparing the operation time with 2D and 3D pelvimetry. Sixty-six patients who underwent total mesorectal excision after neoadjuvant chemoradiotherapy due to primary rectal cancer located in the middle and lower rectum (10 cm from the anus) were included in the study. Surgery notes were reviewed and data on demographic factors, tumor stage, duration of surgery, and types of surgery were collected, as well as pelvimetric parameters. All protocols had 2D T2-weighted sequences in 3 planes (axial, sagittal, and coronal). Pelvimetric measurements were made by measuring 8 pelvic lengths and 2 angles. Pelvis and tumor volume were measured by manual margin monitoring. In each slice, both pelvis and tumor boundaries were manually drawn individually in the sagittal plane. Pelvis and tumor volumes were calculated from the set of adjacent images by summing slice thickness and products of area measurements within the pelvis and tumor boundaries. In our results, no correlation was observed with operation time, including pelvic volume. Exception for this were interacetabular distance and tumor volume. In the regression test, the only parameter that correlated with the operation time was tumor volume. In conclusion, we believe that tumor volumetric calculations may be useful in predicting difficult distal rectal carcinoma surgeries.

The Effect of Antioxidant Astaxanthin on Intestinal Ischemia Reperfusion Damage in Rats.

BACKGROUND: Mesenteric ischemia is a frequently encountered disease in surgical clinics, difficult to diagnose, and very mortal if not treated. Our study investigated the effects of astaxanthin, which is known to have potent antioxidant properties and is also known to have anti-inflammatory effects on ischemia-reperfusion (I/R) injury. METHODS: A total of 32 healthy Wistar albino female rats were used in our study. Subjects were randomized and equally divided into 4 groups; control (laparotomy group only), I/R (transient mesenteric ischemia group only), astaxanthin 1 mg/kg and 10 mg/kg doses. The transient ischemia time was 60 minutes and the reperfusion time was 120 minutes. Tissue samples were taken from intracardiac blood and terminal ileum after reperfusion. Superoxide dismutase (SOD), catalase (CAT), malondialdehyde (MDA) from blood samples, interleukin-1 (IL-1), IL-6, tumor necrosis factor-α (TNFα), Caspase-3, P53 tests from terminal ileum were studied. Tissue samples were also taken for histopathological evaluation. RESULTS: At the end of the study, both doses of astaxanthin were found to significantly reduce MDA level, CAT, and SOD enzymatic activity, whereas higher doses of astaxanthin significantly reduced MDA level, CAT, and SOD enzyme activities. In addition, cytokines such as TNFα, IL-1 and IL-6 were found to be reduced at both doses of astaxanthin, but only significantly inhibited at higher doses. We observed that inhibition of apoptosis reduced caspase-3 activity and P53 and deoxyribonucleic acid (DNA) fragmentation. CONCLUSION: Astaxanthin, a potent antioxidant, and anti-inflammatory, significantly reduces ischemia and reperfusion injury, especially when used at a dose of 10 mg/kg. These data need to be confirmed by larger animal series and clinical studies.

Community-acquired hypernatremia in elderly and very elderly patients admitted to the hospital: clinical characteristics and outcomes.

BACKGROUND: The clinical features, outcome and cost burden of community-acquired hypernatremia (CAH) in elderly and very elderly patients are not well known. Our aim was to investigate the etiologies, reasons for admission, clinical courses, outcomes, complications, and cost assessments of the elderly patients with CAH. MATERIAL/METHODS: We conducted a retrospective study in our tertiary hospital. Elderly and very elderly patients evaluated in the emergency department (ED) from January 1, 2010 to December 31, 2010 (n=4960) were included. Totally, 102 patients older than 65 years and diagnosed with CAH were evaluated. The patients were divided into 2 main groups according to their age: elderly (65-74 years old) (group 1) (n=38), and very elderly (>74 years) (group 2) (n=64). RESULTS: Our overall observed prevalence of CAH was 2.0% (n=102, 102/4960). In particular, the prevalences of CAH in group 1 and group 2 were 1.0% (38/3651) and 4.8% (64/1309), respectively (p<0.001). Totally, 62 patients had been treated by renin-angiotensin system (RAS) blockers (ie, ACE-inhibitors). Alzheimer's disease had been diagnosed in 46.1% of the subjects. The mean Katz scores at the time of admission were 2.4 ± 1.9 and 1.1 ± 1.0 in group 1 and 2, respectively (p<0.001). The mean cost was higher in group 2 than in group 1 (2407.13 ± 734.54 USD, and 2141.12 ± 1387.14 USD, respectively) (p<0.01). The need for intensive care was significantly greater in group 2 as compared to group 1. CONCLUSIONS: The important determinants of "CAH" in elderly subjects are accompanying Alzheimer's disease, oral intake impairment, and concomitant treatment with RAS blockers.

Are the conventional scoring systems efficient in predicting mortality of acute mesenteric ischemia?: Mortality estimation in patients with AMI.

Acute mesenteric ischemia is a surgical emergency with high morbidity and mortality rates. Therefore, it is important to determine the prognosis for this disease. In the present study, we aimed to compare the prediction accuracy of 3 scoring systems: Acute physiology and chronic health evaluation II, sequential organ failure assessment score and simplified acute physiology score II (SAPS II). The retrospective cohort study was conducted in a university hospital. Eighty-two acute mesenteric ischemia patients were evaluated retrospectively. The mortality prediction abilities of the scoring systems were evaluated by comparing the prediction rates of > 10%, 30% and 50% and the actual mortality among survivors and non-survivors in pairs. Predicted mortality rates among survivors and non-survivors differed among the 3 classification systems. The mortality estimates of the SAPS II were closer to the actual mortality rates. Analysis of the estimated mortality rates as mortality risk limits showed that acute physiology and chronic health evaluation II was superior to sequential organ failure assessment score and SAPS II in estimating mortality rates, whereas SAPS II was more successful in detecting survivors. The estimated mortality rates of the 3 rating systems, the estimated mortality rates were higher in the non-survivor group than in the survivor group. The accuracy of the SAPS II in determining prognosis was relatively better.

Rapid and reversible optogenetic silencing of synaptic transmission by clustering of synaptic vesicles.

Acutely silencing specific neurons informs about their functional roles in circuits and behavior. Existing optogenetic silencers include ion pumps, channels, metabotropic receptors, and tools that damage the neurotransmitter release machinery. While the former hyperpolarize the cell, alter ionic gradients or cellular biochemistry, the latter allow only slow recovery, requiring de novo synthesis. Thus, tools combining fast activation and reversibility are needed. Here, we use light-evoked homo-oligomerization of cryptochrome CRY2 to silence synaptic transmission, by clustering synaptic vesicles (SVs). We benchmark this tool, optoSynC, in Caenorhabditis elegans, zebrafish, and murine hippocampal neurons. optoSynC clusters SVs, observable by electron microscopy. Locomotion silencing occurs with tauon ~7.2 s and recovers with tauoff ~6.5 min after light-off. optoSynC can inhibit exocytosis for several hours, at very low light intensities, does not affect ion currents, biochemistry or synaptic proteins, and may further allow manipulating different SV pools and the transfer of SVs between them.

Headache as the sole presenting symptom of cerebral venous sinuses thrombosis: Subgroup analysis of data from the VENOST study.

OBJECTIVES: Headache is the most common complaint in cerebral venous sinus thrombosis (CVST) and it may sometimes be the only symptom in these patients. This retrospective and prospective study was an investigation of any differences in terms of clinical risk factors, radiological findings, or prognosis in patients with CVST who presented with isolated headache (IH) and cases with other concomitant findings (non-isolated headache [NIH]). METHODS: A total of 1144 patients from a multicenter study of cerebral venous sinus thrombosis (VENOST study) were enrolled in this research. The demographic, biochemical, clinical, and radiological aspects of 287 IH cases and 857 NIH cases were compared. RESULTS: There were twice as many women as men in the study group. In the IH group, when gender distribution was evaluated by age group, no statistically significant difference was found. The onset of headache was frequently subacute and chronic in the IH group, but an acute onset was more common in the NIH group. Other neurological findings were observed in 29% of the IH group during follow-up. A previous history of deep, cerebral, or other venous thromboembolism was less common in the IH group than in the NIH group. Transverse sinus involvement was greater in the IH group, whereas sagittal sinus involvement was greater in the NIH group. The presence of a plasminogen activator inhibitor (PAI) mutation was significantly greater in the IH group. CONCLUSION: IH and CVST should be kept in mind if a patient has subacute or chronic headache. PAI, which has an important role in thrombolytic events, may be a risk factor in CVST. Detailed hematological investigations should be considered. Additional studies are needed.

Epileptic seizures in cerebral venous sinus thrombosis: Subgroup analysis of VENOST study.

PURPOSE: The aim of this study is to evaluate the presence and prognostic impact of early seizures in cerebral venous sinus thrombosis patients (CVST). METHOD: VENOST is a retrospective and prospective national multicenter observational study. CVST patients with or without epileptic seizures (ES) were analyzed and compared in terms of demographic and imaging data, causative factors, clinical variables, and prognosis in a total of 1126 patients. RESULTS: The mean age of the patients in the ES group was 39.73 ± 12.64 and 40.17 ± 14.02 years in the non-ES group (p > 0.05). Epileptic seizures were more common (76.6 %) in females (p < 0.001). Early ES occurred in 269 of 1126 patients (23.9 %). Epileptic seizures mainly presented in the acute phase (71.4 %) of the disease (p < 0.001). Majority of these (60.5 %) were in the first 24 h of the CVST. The most common neurological signs were focal neurologic deficits (29.9 %) and altered consciousness (31.4 %) in the ES group. Superior sagittal sinus (SSS) and cortical veins (CV) involvement were the most common sites of thrombosis and the mostly related etiology were found puerperium in seizure group (30.3 % vs 13.9 %). Patients with seizures had worse outcome in the first month of the disease (p < 0.001) but these did not have any influence thereafter. CONCLUSIONS: In this largest CVST cohort (VENOST) reported female sex, presence of focal neurological deficits and altered consciousness, thrombosis of the SSS and CVs, hemorrhagic infarction were risk factors for ES occurrence in patients with CVST.

Cerebral Venous Sinus Thrombosis in Women: Subgroup Analysis of the VENOST Study.

BACKGROUND: Early diagnosis of cerebral venous sinus thrombosis (CVST) associated with reproductive health-related risk factors (RHRF) including pregnancy, puerperium, and oral contraceptive (OC) use can prevent severe neurological sequelae; thus, the symptoms must be documented in detail for each group. METHODS: Out of 1144 patients with CVST, a total of 777 women were enrolled from a multicenter for the study of cerebral venous sinus thrombosis (VENOST). Demographic, biochemical, clinical, and radiological aspects were compared for 324 cases with RHRF and 453 cases without RHRF. RESULTS: The mean age of the RHRF (-) group (43.2 ± 13 years) was significantly higher than of the RHRF (+) group (34 ± 9 years). A previous history of deep venous thrombosis (3%), isolated cavernous sinus involvement (1%), cranial neuropathy (13%), comorbid malignancy (7%), and its disability scores after 12 months (9%) were significantly higher in the RHRF (-) group. The RHRF (+) group consisted of 44% cases of puerperium, 33% cases of OC users and 23% of pregnant women. The mean age was found to be higher in OC users (38 ± 9 years). A previous history of deep venous thrombosis was slightly higher in the pregnancy subgroup (4%). Epileptic seizures were more common in the puerperium group (44%). CONCLUSION: The results of our study indicate that the risk of CSVT increases parallel to age, OC use, and puerperium period. In addition, when considering the frequency of findings and symptoms, epileptic seizures in the puerperium subgroup of the RHRF (+) group and malignancies in the RHRF (-) group may accompany the CSVT. In daily practice, predicting these risks for the CSVT and early recognition of the symptoms will provide significant benefits to patients.

Multiple cranial nerve involvement caused by Brucella melitensis.

A case of neurobrucellosis complicated by optic, abducens and vestibulocochlear nerve palsies is reported. Brucella melitensis was isolated in the cerebrospinal fluid, and the patient was diagnosed with retrobulbar neuritis. Despite medical treatment, the patient developed optic atrophy. Multiple cranial nerve involvement should be kept in mind in patients presenting with blurred vision and double vision, especially in regions where brucellosis is endemic.

A case of headache, double vision and ptosis in emergency department: Tolosa-Hunt syndrome.

Headache and double vision symptoms can be seen especially neurological and optical problems. We present Tolosa-Hunt syndrome in a 34-year-old female patient who presented to the emergency department (ED) with the complaints of unilateral severe headache, double vision and ptosis, presented asymmetric contrast enhancement in the right superior cavernous sinus on MR. This pain was reduced with steroid therapy. Pulse steroid therapy of 1 mg/kg/day was planned based on the patient's clinical manifestation, MRI findings and presumed diagnosis of THS. It was observed at patient's follow-up that there were occasional headache, ptosis and double vision were disappeared and MRI findings were regressed. In the differential diagnosis of patients presenting to the ED with the complaints of headache and double vision, rare pathologies such as THS should be considered in addition of common pathologies such as neurological and ocular diseases.

Elevated blood lead concentrations in essential tremor: a case-control study in Mersin, Turkey.

BACKGROUND: Essential tremor (ET) is one of the most common neurologic disorders. Aside from underlying susceptibility genes, recent studies have also begun to focus on environmental toxic factors. Yet there remains a paucity of information on such factors, making studies of environmental factors important. A recent study in New York City found blood lead concentrations to be elevated in ET cases compared with matched controls. Chronic exposure to lead produces cerebellar damage, and this could predispose individuals to develop ET. OBJECTIVE: The aim of this study was to determine whether the elevation in blood lead concentrations observed in a single study in New York was similarly present in ET cases sampled from a completely different geographic region. METHODS: Blood lead concentrations were measured in 105 ET cases and 105 controls at Mersin University, Mersin, Turkey. RESULTS: The median blood lead concentration was 2.7 microg/dL in ET cases compared with 1.5 microg/dL in controls (p < 0.001). In an unadjusted logistic regression model, blood lead concentration was associated with diagnosis: odds ratio (OR) = 4.01; 95% confidence interval (CI), 2.53-6.37; p < 0.001 (i.e., each 1-microg/dL increase in blood lead concentration was associated with a 4-fold increased odds of ET). This association was more robust when cases were compared with a subsample of controls who did not share the same home environment (OR = 8.13; 95% CI, 3.05-21.65; p < 0.001). In adjusted models, results were similar. CONCLUSIONS: These data replicate those of a previous study in New York and demonstrate an association between the environmental toxicant lead and a common neurologic disorder.

Bilateral thalamic lesions: A pictorial review.

Bithalamic lesions are uncommon, however, both focal and systemic disorders may present bilateral abnormalities in the thalamus in different acute and chronic clinical situations. Neuroimaging, in particular magnetic resonance imaging, plays an essential role in diagnostic approach. Imaging features such as signal alterations, diffusion restriction or contrast enhancement are helpful in characterization of these abnormalities. The location of the lesions may provide key information because some pathologies typically involve a certain part of the thalamus. In addition to thalamic findings, neuroimaging findings in other parts of the brain associated with the clinical and laboratory information should be taken into account to make a correct diagnosis.

Therapeutic Plasma Exchange in Patients with Neurologic Disorders: Review of 63 Cases.

Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1). TPE was frontline therapy in 57 % of the patients (n = 36). Total number of TPE sessions was 517; median number of sessions per patient was 8 (range 1-66). TPE was done through a central venous access in 97 % and through a peripheral venous access in 3 % of the patients. Human albumin was used as replacement fluid in 49 %, hydroxyethyl starch (HES) in 49 % and fresh frozen plasma in 2 % of the cases. Adverse reactions were recorded in 60 % of the patients. Total ratio of complications in 517 TPE procedures was 10.8 % and these were mild and manageable such as allergic reactions and hypotension. Overall response rate was 81 %. Interestingly, complication and response rates were similar in both HES and human albumin groups. We conclude that TPE is an effective treatment in neurologic diseases in which autoimmunity plays an important role in the pathogenesis and HES can be used instead of albumin as replacement fluid in these disorders, since it is cost-effective, has similar efficacy and complication rates.

Holter monitoring for 24 hours in patients with thromboembolic stroke and sinus rhythm diagnosed in the emergency department.

It is well known that patients with ischemic stroke show ST-T abnormalities and various rhythm abnormalities on an electrocardiogram (ECG). The most commonly encountered rhythm abnormality is atrial fibrillation. It was recently shown that paroxysmal atrial fibrillation (PAF) is an important causative factor in patients with stroke. Detection of PAF is important in identifying the cause, prognosis, and treatment in patients with thromboembolic stroke. Investigators in the present study followed patients with thromboembolic stroke who had been admitted to the emergency department in sinus rhythm; 24-h Holter monitoring was used, and patients were assessed at referral and every 6 h for 24 h with ECG, which was used to detect rhythm disturbances, especially PAF. In 26 patients with stroke who came to the emergency department, acute thromboembolic stroke was diagnosed on the basis of magnetic resonance imaging; no rhythm abnormalities were noted on Holter monitoring. Eighteen patients were male and 8 were female (mean age: 66+/-13 y). Arrhythmia was identified on ECG in 3 patients (11%) and on 24-h Holter monitoring in 24 patients (92%). PAF was diagnosed in 3 patients (11%) on ECG and in 11 patients (42%) on Holter monitoring. In 2 patients, nonsustained ventricular tachycardia was detected only on Holter monitoring, which was found to be significantly superior to ECG for the detection of arrhythmias (P<.001). Investigators found no significant relationship between PAF and variables such as hypertension, diabetes, coronary artery disease, history of myocardial infarction, ST-T changes, and elevations in cardiac markers. However, a significant relationship (P<.01) was seen between nonsustained ventricular tachycardia and a history of myocardial infarction. No relationship was discerned between arrhythmia and stroke localization. Study results suggested that (1) PAF is a commonly diagnosed rhythm abnormality, and (2) Holter monitoring is superior to routine ECG for the detection of arrhythmias such as PAF in patients anticipated to have thromboembolic stroke with sinus rhythm.

Three Turkish families with different transthyretin mutations.

Transthyretin (TTR)-related hereditary amyloidosis, also called familial amyloid polyneuropathy (FAP), is a rare autosomal dominant systemic disorder that presents with progressive axonal sensory, autonomic and/or motor neuropathies. The present report describes three families with three different TTR mutations who were followed from 1995 to 2014. Only one of these families expressed the Val30Met mutation, which is the most common mutation in endemic regions; all members of this family had late disease onset but varied severities and clinical presentations of the disease. The second family expressed the Thr49Ser mutation, which has not been well documented previously. Our limited experience obtained from these patients indicates that this mutation presents with autonomic neuropathy but a greater degree of cardiac involvement, especially fatal heart failure. The third mutation, Glu54Lys, has been identified as a cause of severe familial amyloid polyneuropathy; the family members with this mutation exhibited severe motor and autonomic neuropathy, early vitreous opacity, and fatal heart failure. Three of the patients with the Val30Met mutation were treated with tafamidis for longer than one year and cessation of the polyneuropathy resulted. However, a short trial of tafamidis in two patients with the Glu54Lys mutation, who showed severe systemic and neurological involvement, did not gain any clinical benefits.