Medial medullary infarction: clinical, radiological findings and long-term prognosis.

BACKGROUND AND AIM: Medial medullary infarction (MMI) is a rare type of posterior circulation stroke. We aim to examine the clinical and radiological features, etiology, and prognosis of patients with MMI. METHOD: MMI patients registered consecutively in the stroke databank of the Istanbul Medical Faculty between January 1999 and April 2022 were included in the study. Medullary lesions were rostrocaudally classified as rostral, middle, and caudal, and ventrodorsally as ventral, middle, and dorsal. The etiological classification was performed, and functional outcome was assessed based on the modified Rankin Scale (mRS). Overall survival was estimated using the Kaplan-Meier technique. RESULTS: We examined 48 cases of MMI including 9 with bilateral MMI. There were 34 men (70%), and mean age was 62.9 (± 12.8) years. The median NIHSS score was 7 (IQR; 4.5-10.5). The most common symptom was motor dysfunction. The medullary lesions were located caudally in 4 patients, rostrally in 30, rostromedially in 10, and rostro-medio-caudally in 2 patients. On ventro-dorsal classification; unilateral lesions were found ventrally in 19, ventromedially in 11, and ventro-medio-dorsally in 4 patients. The median follow-up duration was 20 months (interquartile range (IQR); 1-60). According to the third-month mRS, 39% of the patients were considered to have a good prognosis. CONCLUSION: The most common etiology was distal vertebral artery atherosclerosis. More than 50% of the patients could walk unassisted in the long-term follow-up, and stroke recurrence was infrequent. Patients with bilateral MMI had poor outcomes.

Neuropsychiatric outcomes and caregiver distress in primary progressive aphasia.

BACKGROUND: In this study, we aimed to outline the neuropsychiatric consequences of primary progressive aphasia (PPA) and to understand how neuropsychiatric symptomatology affects distress in caregivers. METHODS: The Neuropsychiatric Inventory (NPI) including the distress index (NPI-Distress) was used. Additional information about the caregiver burden was obtained using Zarit Burden Interview (ZBI). NPI, NPI-Distress, and ZBI data from 17 patients with a clinical diagnosis of PPA were compared with 10 stroke aphasia patients. Neuropsychiatric symptomatology was investigated based on three clusters; Mood, Frontal/Comportmental, and Psychotic/Disruptive. Additionally, the Activities of Daily Living Questionnaire (ADLQ) was used to outline the functional impairment. Twelve healthy controls were included to compare the neurocognitive test scores with PPA and stroke aphasia groups. RESULTS: A greater number of neuropsychiatric symptoms were observed in the PPA group compared to the stroke aphasia group. The number of symptoms in Mood, and Frontal/Comportmental clusters were greater than the number of symptoms in Psychotic/Disruptive clusters in the PPA group, whereas no significant relationship between the number of symptoms and symptom clusters was found in the stroke aphasia group. In the PPA group, a strong correlation was found between the NPI-Frequency × Severity scores and the NPI-Distress scores. Moreover, the NPI-Distress scores in the PPA group strongly correlated with the ZBI scores. Scores for anxiety, irritability/lability, and apathy had a stronger correlation with the NPI-Distress scores compared to the other NPI symptoms. The Communication subscale was the most impaired domain in the PPA group. Travel, and Employment and Recreation subscales showed greater functional impairment in the stroke aphasia group compared to the PPA group. CONCLUSIONS: Neuropsychiatric symptoms in PPA in our study were more frequent than previously reported. Furthermore, the distress index of the NPI was not only correlated with the severity of the neuropsychiatric symptoms but also reflected the overall burden on the caregivers in the PPA group.

Lesion probability map in cerebral vein thrombosis due to Behçet's disease.

AIM: Behçet's disease (BD) is a multisystemic inflammatory disease. Cerebral venous sinus thrombosis (CVST) is the second most common form of neuro-BD after parenchymal central nervous system involvement. The purpose of this study was to construct flow-void probability maps of patients with CVST with and without BD to visually illustrate the impacted cerebral venous sinuses, to compare the subgroups of patients, and investigate the effect of thrombus localization on clinical findings. METHODS: Seventeen patients with a diagnosis of BD-related CVST (CVST-BD) and 23 patients with a diagnosis of CVST related to other etiologies (CVST-O) were included. We collected data including gender, age at onset of BD and CVST, presenting symptoms, neurological findings, and the etiology. High-resolution magnetic resonance venographies obtained during CVST were used to mark and digitalize thrombosed areas. Thrombus probability and subtraction maps were created to reveal the differences between the subgroups. RESULTS: Remarkably, all patients with CVST-BD had thrombosis in the transverse sinus (TS). However, TS was affected in 73.9% of the CVST-O patients (17/17 in CVST-BD vs 17/23 in CVST-O, P = .03). Thrombosis developed mostly in the superior sagittal sinus (SSS) and TS in the CVST-O group (11/23, 47.8% and 17/23, 73.9%, respectively). The frequency of SSS thrombosis tended to be higher in the CVST-O (47.8% vs 23.5%, P = .19). CONCLUSION: Venous infarction and hemorrhage were less common in patients with CVST-BD. The only clinical symptom in most of the CSVT patients with BD was headache due to elevated intracranial pressure. TS thrombosis was more common in patients with BD.

Verbal and Nonverbal Memory in Neurodegenerative and Stroke Aphasia: Evidence From the Turkish Version of the Three Words Three Shapes Test.

BACKGROUND: Although language impairment is the most salient feature of cognitive impairment in both primary progressive aphasia (PPA) and stroke aphasia (SA), memory can also be impaired in both patient populations. OBJECTIVE: To identify distinctive features of verbal and nonverbal memory processing in individuals with PPA and those with SA. METHOD: We gave individuals with PPA (n = 14), those with SA (n = 8), and healthy controls (HC; n = 13) a comprehensive neuropsychological test battery and the Turkish version of the Three Words Three Shapes Test (3W3S-Turkish). The 3W3S-Turkish Test includes five subtests: Copy, Incidental Recall, Acquisition, Delayed Recall, and Recognition. High-resolution brain scans were performed in a subset of individuals with PPA and those with SA. Lesion distribution was limited to the dorsal language areas in the SA group, whereas peak atrophy areas in the PPA group extended beyond the language network, including the medial temporal lobe, precuneus, and posterior/medial portions of the cingulate cortex. RESULTS: Both the PPA and SA groups showed impairment in incidental recall, and the PPA group showed additional impairment in delayed recall. Greater impairment for verbal stimuli suggestive of material-specific memory impairment was evident in the PPA group's scores on the Incidental Recall and Delayed Recall subtests. Both aphasia groups retained the acquired information regardless of material type. CONCLUSION: Although both aphasia groups shared similarities in the involvement of the dorsal prefrontal working memory/attention network, the PPA group showed greater impairment in delayed recall compared with the SA group.

Periodic electroclinical seizures following an ischemic stroke revealed by continuous-EEG.

BACKGROUND: Periodic EEG patterns are mostly associated with critical illnesses and acute disruptions of the central nervous system. Periodic or cyclic seizures are extremely rare phenomena, most of which are nonconvulsive, only reported in critically ill patients. Here we report a patient with periodic focal impaired awareness seizures following a minor stroke and address possible pathophysiological mechanisms. CASE: A 49 years old male patient presented with periodic seizures, associated with an acute stroke in the left occipital and parietal regions. These focal seizures, recorded during long-term video-EEG monitoring in the scalp EEG, appeared every 9-11 min, and responded to iv valproic acid treatment but not to iv treatments of diazepam, phenytoin, and levetiracetam. DISCUSSION: We believe that the blood-brain barrier disruption due to stroke, in conjunction with hyperglycemia and antiphospholipid antibodies have led to an imbalance of the surrounding tissue and sustained hyperexcitability to a point of pacemaker potentials. It is tempting to speculate that repetitive cycles of cortical spreading depression due to tissue injury have aided the periodicity of the seizures. CONCLUSION: Continuous EEG monitoring is crucial, not only to diagnose and appropriately treat accompanying subtle seizures but also to further understand the underlying intriguing pathophysiological processes like periodicity.

CHADS2 and CHA2DS2-VASc Scores Can Guide the Evaluation of Cryptogenic Ischemic Stroke.

BACKGROUND AND AIMS: Cryptogenic ischemic strokes (CIS) are treated with antiplatelets for stroke prevention in routine clinical practice. The objective of this study was to investigate whether the CHADS2 and CHA2DS2-VASc scores may be used to identify the patients with CIS at higher risk of ischemic stroke despite antiplatelet therapy. MATERIAL AND METHODS: We calculated CHADS2 and CHA2DS2-VASc scores in patients with first ever CIS; those previously managed with antiplatelets (AP group) and in those without antiplatelets (non-AP group), using the prospectively recorded data of the Istanbul Medical School Stroke Registry from 1996-2014. RESULTS: Of the 4466 IS patients, 886 patients with first ever IS had complete data for score calculation. Seventy-five (39 women) of them were diagnosed with CIS. CHADS2 and CHA2DS2-VASc scores were significantly higher in the AP group of 19 patients in comparison to the non-AP group of 56 patients (P = 0.005 and P = 0.009, respectively). ROC curve analyses showed an area under curve (AUC) of 0.705 (CI: 0.57-0.84; P = 0.008) for CHADS2 score ≥3 and AUC of 0.699 (CI: 0.57-0.82; P = 0.01) for CHA2DS2-VASc score ≥4. Vascular diseases were more frequent in the AP group and these patients were older than the patients in the non-AP group (P = 0.025, P = 0.024; respectively). CONCLUSIONS: CHA2DS2-VASc score ≥ 4 and CHADS2 score ≥3 may be used as a predictor of the occurrence of IS despite regular antiplatelet use and suggest an embolic source which will respond better to anticogulation. Our results support that CHADS2 and CHA2DS2-VASc scores may be useful to identify subgroups among patients with CIS for individualizing diagnostic approach, planning future workup and preventive treatment.

Senile-Onset Subacute Sclerosing Panencephalitis, Presenting With Peculiar Findings.

Subacute sclerosing panencephalitis (SSPE) is a well-known childhood disease; however, the adult onset of SSPE cases are also widely recognized where the oldest case reported is 52 years old. We report a 61-year-old woman patient presenting with atypical clinical and EEG features, diagnosed with SSPE. Measles and SSPE have decreased dramatically owing to worldwide immunization programs; however, there are still reasons to consider SSPE in differential diagnosis even in patients presenting with atypical clinical findings and older ages. First, there is a generation who missed the immunization era, constituting a latent disease pool. Second, antivaccination movements have led to a decline in MMR (measles, mumps, rubella) vaccination worldwide, leading to measles outbreaks and potential future SSPE cases. Third, most of the vaccination programs start measles immunization at the age of 12 months, leading to a shift in the incidence below the age of 1 year, when the risk of developing SSPE in adult life is higher. Finally, disruption in vaccination programs, in which fast disease transmission due to close contact living, unhygienic conditions of refugee camps, and limited access to health care in displaced populations have also led to measles outbreaks. In conclusion, we believe that neurologists for adults should consider SSPE in differential diagnosis, even in older patients with atypical presentations.

Clinical Features of Cancer Associated Ischemic Stroke.

INTRODUCTION: Cerebrovascular disease is the second most common cause of central nervous system involvement in cancer patients after metastases. Procoagulant substances secreted by tumour cells, cancer therapy, and embolus from tumour have been implicated in ischemic stroke (IS) in cancer patients. METHODS: Four thousand twenty patients with IS were prospectively recorded to the Istanbul Medical School Stroke Registry from August 1997 to May 2016. Ninety-two patients with IS and cancer and 3928 IS patients without cancer constituted the two groups. RESULTS: Hypertension, hyperlipidemia, and congestive heart failure were less commonly encountered in cancer stroke patients. Lacunar and posterior circulation syndromes were less common and total anterior circulation syndrome was more common in cancer patients (p<0.001). Multiple territory infarcts were more frequently seen in patients having overt or non-overt disseminated intravascular coagulopathy (DIC) compared to those without DIC. Median survival was significantly shorter in patients with cancer-related stroke. CONCLUSION: Cancer associated stroke without other underlying etiology is a specific group of stroke patients presenting with multiple territorial infarcts, overt or non-overt disseminated intravascular coagulopathy, and poor prognosis.

Intracranial hypertension related to cerebral venous thrombosis; and acute ischemic stroke with micro-infarcts associated with IgG4-related disease.

IgG4-related systemic disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells, affecting multiple organs. This report describes a case who was diagnosed with IgG4-RD, having cerebral venous thrombosis and a subsequent acute ischemic stroke of undetermined cause. A 47-year-old woman presented with headache, visual disturbance and eyelid swelling and two years later she was admitted with acute attacks of mild left lower limb sensory-motor monoparesis. Indirect immunohistochemistry assay showed elevated level of IgG4, labeling neurons of the central nervous system, suggesting an immunological process possibly affecting vascular structures. Our experience suggests that IgG4-RD may be considered in patients with ischemic stroke and cerebral venous system involvement.

Late onset aura may herald cerebral amyloid angiopathy: A case report.

BACKGROUND: Although migraine usually begins in the early decades of life, late onset of migraine with aura is occasionally observed and can occur without headache, causing confusion in the differential diagnosis. CASE REPORT: A 72-year-old man presented with recurrent episodes of visual aura lasting for 20 minutes. These episodes had started at 57 years of age and were only once accompanied by a severe headache. Magnetic resonance imaging revealed changes in the periventricular white matter, left occipital haemorrhage and subcortical haemosiderin deposits, compatible with cerebral amyloid angiopathy. Previous treatment with antiplatelet drugs was discontinued. His episodes of visual aura stopped on treatment with lamotrigine and add-on treatment with verapamil. CONCLUSION: In patients with a late onset of migraine aura, doctors must consider other under-recognized causes of transient neurological symptoms, such as cerebral amyloid angiopathy. Blood-sensitive magnetic resonance imaging sequences are the best tool for the early detection of an underlying pathology and therefore treatment with antiplatelet/anticoagulant drugs should be avoided as this may increase the risk of haemorrhage.

Demographic and geographic vascular risk factor differences in European young adults with ischemic stroke: the 15 cities young stroke study.

BACKGROUND AND PURPOSE: We compared among young patients with ischemic stroke the distribution of vascular risk factors among sex, age groups, and 3 distinct geographic regions in Europe. METHODS: We included patients with first-ever ischemic stroke aged 15 to 49 years from existing hospital- or population-based prospective or consecutive young stroke registries involving 15 cities in 12 countries. Geographic regions were defined as northern (Finland, Norway), central (Austria, Belgium, France, Germany, Hungary, The Netherlands, Switzerland), and southern (Greece, Italy, Turkey) Europe. Hierarchical regression models were used for comparisons. RESULTS: In the study cohort (n=3944), the 3 most frequent risk factors were current smoking (48.7%), dyslipidemia (45.8%), and hypertension (35.9%). Compared with central (n=1868; median age, 43 years) and northern (n=1330; median age, 44 years) European patients, southern Europeans (n=746; median age, 41 years) were younger. No sex difference emerged between the regions, male:female ratio being 0.7 in those aged <34 years and reaching 1.7 in those aged 45 to 49 years. After accounting for confounders, no risk-factor differences emerged at the region level. Compared with females, males were older and they more frequently had dyslipidemia or coronary heart disease, or were smokers, irrespective of region. In both sexes, prevalence of family history of stroke, dyslipidemia, smoking, hypertension, diabetes mellitus, coronary heart disease, peripheral arterial disease, and atrial fibrillation positively correlated with age across all regions. CONCLUSIONS: Primary preventive strategies for ischemic stroke in young adults-having high rate of modifiable risk factors-should be targeted according to sex and age at continental level.

Cognitive impairment in neuro-Behcet's disease and multiple sclerosis: a comparative study.

Both multiple sclerosis (MS) and neuro-Behcet's disease (NBD) can cause a cognitive dysfunction mainly involving the executive functions. We conducted this study to clarify the probable differential cognitive/behavioral profiles of MS and NBD. Twenty consecutive cases with parenchymal NBD (13 male, seven female), and 20 cases with MS (five male, 15 female) were evaluated. Both groups had a thorough neurological examination; an evaluation for Expanded Disability Status Scale (EDSS), Multiple Sclerosis Functional Composite (MSFC), and Beck's Depression Scale; and a detailed neuropsychological evaluation masked to the diagnosis. Among the two groups, male/female ratio differed significantly while other demographic and clinical features were not different. In California Verbal Learning Test, both short- and long-term delayed recall and cued recognition were worse in neuro-Behcet's cases. They had impaired semantic clustering and increased false positives. Stroop Test was also more impaired in neuro-Behcet's cases. They needed significantly more trials to complete the first category of the Wisconsin Card Sorting Test and had a poorer total Frontal Behavioral Inventory Score. Our results suggest that neuro-Behcet's patients have a more severe "frontal"-executive dysfunction than MS patients.

Glycoprotein Ib-alpha Kozak polymorphism in ischemic stroke.

BACKGROUND: Recently, a T/C polymorphism in the Kozak sequence of glycoprotein Ib-alpha (GPIb-alpha) gene at position -5 from the initiator ATG codons, has been identified. The presence of -5C allele increases the surface expression of GPIb-IX-V complex in a gene dosage-dependent manner. It has been suggested that higher receptor levels might increase the adhesiveness of the platelets and confer risk for thrombosis. In this study, we aimed to investigate the association between GPIb-alpha Kozak polymorphism and ischemic stroke. METHODS: We prospectively and consecutively recruited 231 patients (118 women and 113 men; mean age: 65 ± 14.2 years) with first ever ischemic stroke admitted to Istanbul Faculty of Medicine Edip Aktin Stroke Unit between April 2007 and June 2009. Demographic features, risk factors, clinical, and etiological subtypes were analyzed. As the control group, 220 unrelated healthy subjects were included. RESULTS: We found that 156 patients had TT, 70 patients had TC, and 5 patients had CC genotype. At least one copy of C allele carriers were overrepresented in the ischemic stroke group (32.5%) compared with controls (23%) [odds ratio (OR): 0.61; 95% confidence interval (CI): 0.40-0.93; P = 0.03]. Among etiologic subtypes, the distribution of C allele carriers was the highest in patients with undetermined etiology (45%) and it was significantly higher than controls (OR: 0.36; 95% CI: 0.20-0.65; P = 0.0008). In other subtypes, there was no association with Kozak -5C allele. CONCLUSION: In conclusion, these encouraging preliminary results show that GPIb-alpha T/C polymorphism might increase the risk of ischemic stroke, especially in those with undetermined etiology.

Gender differences in acute stroke: Istanbul medical school stroke registry.

BACKGROUND: We aimed to investigate gender differences in Turkish stroke patients. MATERIAL AND METHODS: Demographics, risk factors, clinical and etiologic subtypes, laboratory findings, clinical course, and in-hospital prognosis of 1 522 patients with ischemic stroke (IS) and 320 patients with intracerebral hemorrhage prospectively registered in the Istanbul Medical School Stroke Registry (1994-2004) were analyzed separately. RESULTS: The mean age of IS patients was higher in females (n : 751) (P<0.0001). In males, smoking, ischemic heart disease, peripheral arterial disease, posterior circulation syndromes, and strokes due to large-artery atherosclerosis were more common (P<0.0001 for each). Prestroke disability, atrial fibrillation (P<0.0001), hypertension (P=0.041), modified Rankin Scale (mRS) 3-5 at admission (P<0.0001), total anterior circulation syndrome (P<0.0001), and cardioembolic stroke (P<0.0001) were more frequent in females. Female gender was an independent predictor of poor outcome (mRS 3-6). CONCLUSION: Gender differences were observed exclusively in patients with IS. Although our patients were younger than those reported, gender differences were similar.

A Case Report of Cerebral Venous Thrombosis in Polycythemia Vera Presenting with Intracranial and Spinal Subdural Hematoma.

Spinal subdural hematoma (SDH) is a rare condition and can be caused by several factors. Concomitant cranial and spinal SDH is even much less common. We present a 77-year-old male patient with lower back pain, paraparesis, and urinary retention following a sudden onset headache. Imaging revealed concomitant cranial and spinal SDH related to cerebral venous thrombosis (CVT) associated with hemorrhagic venous infarct. Laboratory examinations were consistent with polycythemia vera. There was no history of trauma and previous cranial surgery. Brain angiography did not reveal any evidence of arteriovenous fistula or vascular malformation. Since lower back pain occurred shortly after the headache and there was no other reasonable explanation for spinal hemorrhage, we suppose that the mechanism of spinal SDH is the migration of blood from the intracranial compartment. Therefore, this is the first report of concomitant spinal SDH and cerebral hemorrhage associated with CVT in a patient with myeloproliferative disease.

Clinical and magnetic resonance imaging findings of HIV-negative patients with neurosyphilis.

There have been no serial studies about neuroradiological findings of neurosyphilis in the literature. There have been only case reports concerning HIV negative patients with neurosyphilis. We present 8 HIV negative neurosyphilis patients two of whom are women. The mean age of the patients was 48 years+/-12.37. Five of the 8 patients had general paresis, two optic atrophy and one multiple cranial neuropathies. The CSF findings were quite similar in 6 of 8 patients. In half of the patients cranial MRI showed mild cerebral atrophy. Nonspecific hyperintense small foci in 3 patients are thought to be related to syphilis. Hyperintensity involving bilateral medial and anterior temporal regions more prominent on the left side was seen in one of the patients with general paresis. This finding may be due to cytotoxic edema associated with status epilepticus and may mimic herpes simplex and other limbic encephalitides. Though not typical, certain MRI findings guides for the diagnosis of neurosyphilis.

Stuttering due to ischemic stroke.

Acquired stuttering is a disorder of the fluency of speech. The mechanism underlying stuttering is unknown. It may occur after bilateral and unilateral cortical or subcortical brain damage. We report two cases who had stuttering resulting from left parietal infarction.