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.

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.

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 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.

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).

Is there any relationship between quality of life and polysomnographically detected sleep parameters/disorders in stable myasthenia gravis?

It is known that quality of life in myasthenia gravis is positively correlated with subjective sleep quality, still no data is available regarding the relationship between QOL and polysomnographically detected sleep parameters and disorders. In this study, we tried to highlighten this relationship, by performing polysomnography. Sleep-related complaints were evaluated in face-to-face interviews with 19 clinically stable MG patients and 26 healthy controls. During the interviews questionnaires assessing sleep quality, excessive daytime sleepiness, fatigue, depression, anxiety, and Turkish version of the MG-QOL 15-item scale [(MG-QOL15(T)] were administered and then an overnight polysomnography was performed. Sleep disorders, especially obstructive sleep apnea and fatigue were higher, whereas subjective sleep duration was significantly lower, in patients than controls. Excessive daytime sleepiness and poor sleep quality were not different between patients and controls. Other than percentage of sleep stage III, which was negatively correlated with MG-QOL15(T) scores, neither other sleep parameters nor sleep disorders were correlated with MG-QOL15(T) scores. MG composite, subjective sleep duration, fatigue severity and Hamilton depression rating scale scores were found to be positively correlated with MG-QOL15(T) scores. It was shown that decreasing disease severity and enhancing psychological well-being will improve patients' quality of life. We recommend that our findings should be repeated in a large prospective cohort of MG patients.

Reliability and validity of the Turkish version of myastheniagravis-quality of life questionnaire-15 item.

BACKGROUND/AIM: The myasthenia gravis-quality of life questionnaire 15 item (MG-QOL15) is a validated, short, and easy to use disease-specific quality of life (QOL) tool in myasthenia gravis. Other than Turkish, a lot of versions of the MG-QOL15 have been used in different languages in different cultures. Therefore, the aim of this study was to translate and construct a validated and adapted Turkish version of the MG-QOL15 [MG-QOL15(T)]. MATERIALS AND METHODS: After translation, back-translation, and comparison of the 2 English versions of the MG-QOL15, it was tested by 22 monolingual healthy individuals and then 23 patients with clinically stable MG. Afterwards, 11 voluntary patients out of these 23 patients were interviewed for a second time. During the second interview, the MG-QOL15(T) and the 36-item short-form health survey (Turkish version) were administered simultaneously. RESULTS: The MG-QOL15(T) was found to have high internal consistency (1st and 2nd evaluation Cronbach's alphas were 0.958 and 0.928, respectively), test-retest reliability, and concurrent validity. The MG-QOL15(T) was negatively correlated with physical functioning, general health, vitality, and social functioning domains and with the physical and mental composite scores of the SF-36. CONCLUSION: The MG-QOL15(T) is accepted to be a valid, reliable, valuable tool for measuring disease-specific QOL in Turkish patients with MG.

Sleep Disturbances in Essential Tremor and Parkinson Disease: A Polysomnographic Study.

OBJECTIVE: Sleep problems are a common non-motor complication of Parkinson disease (PD), and patients with essential tremor (ET) share a number of motor and non-motor features of PD. To clarify the relationship between these disorders, we evaluated the sleep problems in patients with ET and PD using assessment scales and objective polysomnographic (PSG) testing. METHOD: Twenty-one consecutive patients with PD, 16 with ET, and 14 healthy subjects participated in this study and were compared in terms of sleep related complaints, final sleep related diagnosis, and polysomnographic features. RESULTS: The results of our study have shown that patients with PD were more likely than were those with ET to have a history of REM sleep behavior disorders (RBD) (p = 0.001) and excessive daytime sleepiness (p ≤ 0.05). Additionally, PSG data revealed that ET patients had lower mean SpO2 values (p ≤ 0.05) and REM without atonia (RWA) (p = 0.032) than did patients with PD. CONCLUSION: This is the first study to use PSG to evaluate sleep problems both in ET and PD patients. The results point out different sleep problems in these two common movement disorders which should be investigated in further studies.

Assessment of Relation Between Subjective Memory Complaints and Objective Cognitive Performance of Elderly Over 55 Years Old Age.

INTRODUCTION: This study investigated the frequency of forgetfulness in elderly individuals over 55 years of age and examined the association of subjective memory complaints (SMCs) with objective cognitive functions,, depression and other risk factors. METHODS: We recruited 405 patients over 55 years of age who were referred to Neurology, Cardiology, or Physical Therapy and Rehabilitation outpatient clinics. All subjects were questioned regarding forgetfulness and then were administered the Subjective Memory Complaint (SMC) Scale, Mini Mental Test (MMT), Verbal Fluency Test (VFT), Clock Drawing Test (CDT) and the Geriatric Depression Scale (GDS). Subjects with SMC were compared with those without SMC in terms of cognition, depression and some laboratory parameters. RESULTS: Of the patients, 42.5% complained of forgetfulness. None of these patients had been admitted to hospital for this complaint. Women and patients with low education had more forgetfulness as well as poorer results on the SMC Scale, MMT, VFT, and GDS. Patients with SMC had lower hemoglobin, ferritin and free T4 levels. Female gender and depression was found to be a risk factor for SMCs. CONCLUSION: SMCs are common in people over 55 years of age. Being a woman as well as depression was found to be a risk factor for SMC. Since depression is a treatable condition, these people should be assessed carefully in terms of depressive symptoms. Laboratory parameters, such as hemoglobin, ferritin and free T4 levels should be investigated in patients with SMC. Unlike the other cognitive tests, CDT performance is independent of subjective memory complaints. Elderly patients rarely visit hospital with complaint of SMC, therefore, clinicians should be watchful for this problem.

An unusual case of neuro-Behçet's disease presenting with co-occurence of cerebral venous sinus thrombosis with basilar artery occlusion.

Non-parenchymal neuro-Behçet disease generally affects cerebral venous sinuses, whereas intracranial intracerebral arterial involvement has been rarely reported. But co-involvement of both intracranial intracerebral artery and venous vascular systems in a patient at the same time has not been mentioned before. To the best of our knowledge, this case involving a 25-year-old male with a 7-year history of Behçet disease is the first reported of this type of involvement. He developed occlusion of the basilar artery together with thrombosis of the left sigmoid sinus, distal internal jugular vein, and straight sinus. He was successfully treated with a combination of high-dose steroid and cyclophosphamide. Cranial magnetic resonance angiography demonstrated the resolution of these abnormalities.

Sleep disorders in Behçet's disease, and their relationship with fatigue and quality of life.

Behçet's disease, a systemic vasculitis, can cause varying degrees of activity limitation, fatigue and quality of life impairment. To date, there have been no studies regarding sleep disturbance and its relationship with fatigue and life quality in Behçet's disease. We aimed to evaluate sleep disorders and polysomnographic parameters, and to determine their relationship with fatigue and quality of life in Behçet's disease. Fifty-one patients with Behçet's disease without any neurological involvement were interviewed regarding sleep disorders. Twenty-one subjects with no sleep complaints were included as the control group. Sleep-related complaints were evaluated in a face-to-face interview. Sleep quality, excessive daytime sleepiness, fatigue, depression, anxiety, disease activity/severity, and quality of life questionnaires and an overnight polysomnography were performed. Prevalences of restless legs syndrome (35.3%) and obstructive sleep apnea syndrome with/without other sleep disorders (32.5%) were higher than in the control group and the general population. Fatigue was higher in patients with restless legs syndrome and obstructive sleep apnea syndrome, and in those with lower minimum oxygen saturation; hence, only patients with restless legs syndrome had quality of life impairment. Sleep efficiency index and sleep continuity index were lower, and wake after sleep onset, respiratory disturbance index and apnea-hypopnea index were higher than in controls (P < 0.01). Neither sleep disorders nor polysomnographic parameters were related to disease activity and severity. In conclusion, it is important to question sleep disorder followed by a polysomnography, if necessary, in order to improve quality of life and fatigue in Behçet's disease.

Carotid cavernous fistula with bilateral thalamic infarct.

Carotid cavernous fistula (CCF) is an abnormal communication between cavernous sinus and carotid arterial system. Diagnosis depends on clinical manifestations and MRI findings and angiography. Clinical presentation of CCFs is characterized with chemosis, orbital bruit and pulsatile proptosis. Cranial nerve dysfunction and orbital pain might accompany these symptoms. Although spontaneous remission might occur, sometimes CCF might develop life threatening complications. Here in this case we presented a patient with CCF who afterwards developed bilateral thalamic infarct. Cerebral ischemia in CCF is a rare complication and CCF together with bilateral thalamic infarct has not been reported before.

Traumatic internal carotid artery dissection associated with playing soccer: a case report.

Soccer, one of the most popular sports worldwide among young men, can result in a wide range of orthopedic injuries. Although vascular injuries such as dissection occur rarely, they can cause significant mortality if left undiagnosed. We report herein a 31-year-old male who suffered a large middle cerebral artery infarction due to traumatic internal carotid artery dissection after a ball struck his head and neck. He recovered with mild neurologic deficit after decompressive surgery.

Bacterial translocation in experimental stroke: what happens to the gut barrier?

The reasons of post-stroke infections are still incompletely understood. Bacterial translocation (BT), the passage of viable microbes across an even anatomically intact intestinal barrier, has been described in many critical illnesses. To date, it has not been studied as a source of infection in an animal stroke model. To address this, a permanent left middle cerebral artery occlusion (MCAO) model in rats was used. After 24, 48, and 72 hours (h), sham and experimental groups were sacrificed and samples were taken for BT. Similarity between bacteria detected in tissues (blood, mesenteric lymph node, liver, spleen, and lung) and intestinal microflora was shown with phenotypic methods and antibiotyping. Possible ileum tissue injuries were shown by histopathologic examination (including morphometric analysis). Although there was no bacterial proliferation in the sham groups, 55.5%, 45.4%, and 30% bacterial proliferation was detected in MCAO groups at postoperative hour 24, 48, and 72, respectively. In MCAO groups the bacterial proliferation in tissues and ileum tissue injury scores were higher over time compared to sham groups (p < 0.05). Our findings support the view that stroke, itself leads to mucosal damage and bacterial translocation (Tab. 5, Fig. 2, Ref. 27).

Evaluation of common mutations in the Mediterranean fever gene in Multiple Sclerosis patients: is it a susceptibility gene?

PURPOSE: Multiple Sclerosis (MS) is a disease of the central nervous system characterized by multiple areas of inflammation and demyelination in the white matter of the brain and spinal cord. MEFV gene, which is the main factor in familial Mediterranean fever, is an intracellular regulator of inflammation. This study was designed to determine if known mutations in pyrin domain of MEFV gene are involved in MS and associated with MS morbidity. METHODS: Fifty-three patients with MS and 66 healthy subjects, who were all Turkish, were included in this study. Five pyrin gene mutations (E148Q, M680I, M694V, M694I and V726A) were detected in the patients and controls by using the PRONTO FMF Basic Kit according to the manufacturer's instructions. RESULTS: Pyrin gene mutations were found in 20 of the 53 MS patients (38%) and in seven of the 66 healthy subjects (11%). The frequency of total pyrin domain mutations was significantly higher in the MS patients than in the healthy subjects (p<0.0001). The frequencies of M694V, E148Q and V726A mutations were significantly higher in the patients than in the healthy subjects (p=0.02, p=0.013, p=0.004 respectively). The mean time to reach EDSS score 3.0 was earlier in the patients with MEFV gene mutation (p=0.02) and the relapse rate was slightly higher among the MS patients carrying MEFV gene mutation (p=0.04). CONCLUSION: The results of this study supported the hypothesis that MS patients with MEFV mutation seem to have the susceptibility to develop a more progressive disease. Moreover, these data suggest that MEFV mutations may increase the risk of MS development.

Hyperhomocysteinemia as an independent risk factor for cardioembolic stroke in the Turkish population.

Homocysteine, a sulfur-containing amino acid, is an intermediate during the conversion of methionine to cysteine. Homocysteine can cause vascular injury and atherosclerotic plaque instability. In addition, homocysteine may be directly correlated with hyperlipidemia and lipoprotein(a) and inversely with high-density lipoprotein cholesterol. However, the results regarding the association of homocysteine level with subtypes of stroke and traditional risk factors for stroke have been inconsistent, perhaps due to ethnic differences. The aim of this study was to evaluate the role of serum homocysteine levels in Turkish patients diagnosed with atherosclerotic stroke and those with cardioembolic stroke. We measured homocysteine levels, traditional risk factors for stroke (hypertension, diabetes mellitus, and smoking) and lipoprotein(a) levels in 103 patients with large-vessel atherosclerotic stroke, 37 patients with cardioembolic stroke, and 37 controls with normal cranial magnetic resonance imaging. Only hypertension was found to be a risk factor in all patient groups (p = 0.001). Hyperhomocysteinemia (homocysteine level > or = 15.90 micromol/L) was more common in patients with large-vessel atherosclerotic stroke and cardioembolic stroke (p = 0.0435 and p = 0.007, respectively); nevertheless, it was found to be a risk factor only in patients with cardioembolic stroke (p = 0.023; odds ratio (OR): 5.745). Furthermore, in the patients with large-vessel atherosclerotic stroke, hyperhomocysteinemia was positively correlated with the lipoprotein(a) level (r = 0.227, p = 0.035). In conclusion, hyperhomocysteinemia is common in patients with large-vessel atherosclerotic stroke and cardioembolic stroke. More importantly, hyperhomocysteinemia is an independent risk factor only for cardioembolic stroke in the Turkish population.

Unnoticed dysautonomic syndrome of the face: Harlequin syndrome.

Harlequin sign and harlequin syndrome, which are used interchangeably in the literature, are characterized by sudden onset of hemifacial sweating and flushing, induced by exercise and heat. Hemifacial sweating and flushing with normal ocular sympathetic innervation, known as harlequin syndrome, is rarely associated with tonic pupils, parasympathetic oculomotor lesion and pre- or postganglionic sudomotor sympathetic deficit. In the literature, hemifacial sweating and flushing in patients with apparently abnormal ocular sympathetic innervation has been defined as harlequin sign. To date, a few reports of excessive hemifacial sweating and flushing in structural lesion have been documented. Herein, we report five patients with excessive hemifacial sweating and flushing, two of whom had a syrinx. In presenting the patients, we have attempted to distinguish harlequin syndrome from harlequin sign. With this in mind, Case 1 can be described as harlequin syndrome resembling Ross syndrome, Case 2 as harlequin syndrome with normal ocular sympathetic innervation, Case 3 as harlequin sign with congenital Horner syndrome, Case 4 as harlequin sign with sympathetic and parasympathetic denervation sensitivity, and Case 5 as harlequin syndrome associated with occult sympathetic denervation sensitivity. These cases are discussed together with a review of the literature.