Isolated spinal cord granulomatous angiitis: a case report and review of the literature.

INTRODUCTION: Isolated spinal cord angiitis (ISCA) is very rare disease. But, it is frequently encountered in the differential diagnosis of atypical spinal cord syndromes. CASE PRESENTATION AND REVIEW OF THE LITERATURE: We present a 31-year-old male who presented with progressive paraparesis, and diagnosed with pathologically confirmed ISCA. Longitudinal cystic transverse myelitis was documented in spinal MRI. He responded well to cyclophosphamide and steroid combination, and no relapse was noted during the 4-year follow-up. A standard systematic analysis of the germane literature disclosed 15 more ISCA cases. In total 16 cases (mean age: 46.5, 10 males), ISCA was diagnosed with pathological evaluation in all (Biopsy in 11, Autopsy in 5). MRI lesion is characterized by usually multisegmental longitudinal and sometimes cystic expansile lesions. In seven cases, it was described as "(pseudo)tumoral" by the authors. Albeit absence of elevation of CSF protein/WBC or "compatible" spinal MRI lesion may aid to exclude ISCA to some extent, pathological confirmation is currently necessary for the diagnosis. In 11 cases, ISCA was treated similar to primary supratentorial vasculitis. Mortality rate is 31%. DISCUSSION: ISCA diagnosis, a typical example of which we have presented here, can only be established by tissue examination. However, noninvasive diagnostic criteria are critically needed. Our data suggest that this can only be possible with multinational multicenter prospective registry.

Clinical challenges associated with the endovascular treatment of acute stroke in a patient with infective endocarditis.

Although stroke is common in infective endocarditis (IE), only 26 cases of thrombectomy have been reported to date for IE-related acute stroke. We report a 40-year-old man who presented with left middle cerebral artery occlusion of unknown cause. Multiple attempts of mechanical aspiration thrombectomy and stentrievers failed to recanalize the artery. Effective revascularization was eventually achieved by placing a self-expanding intracranial stent. Post-procedurally the patient was diagnosed with IE with mitral valve insufficiency, mandating emergent valvular replacement while the patient was still on tirofiban infusion. On follow-up, the patient had a modified Rankin's score of 0, had no recurrent stroke, and the intracranial stent remained patent yet stenosed. Based on the use of a self-expanding intracranial stent in the setting of IE, we discuss the consequences of the fibrotic and inflammatory content of the embolus and the associated high risk of intracranial hemorrhage which complicates clinical decision making.

Neutrophil-to-Lymphocyte Ratio and Response to Intravenous Thrombolysis in Patients with Acute Ischemic Stroke.

BACKGROUND AND AIMS: The Neutrophil-to-Lymphocyte Ratio (NLR) is suggested as a readily available and inexpensive biomarker to predict prognosis of acute stroke. Experience with intravenous (IV) tissue plasminogen activator (tPA) treatment is limited. METHODS: Total 142 (80 female, age: 69 ± 13 yearr) consecutive acute stroke patients treated with IV tPA were evaluated. Admission and 24th hour lymphocyte, neutrophil, and monocyte counts were measured and the NLR was calculated. RESULTS: Average NLR elevated (by 3.47 ± 6.75) significantly from admission to 24th hour (P< .001). Total 52% of patients exerted good response to IV tPA (NIHSS ≤1 or decrease in NIHSS ≥4 at end of 24 hour), while 27% showed dramatic response (decrease in NIHSS ≥8 at end of 24 hour). The patients with "thrombolysis resistance" had significantly higher 24 hour Neutrophil-to-Lymphocyte Ratio (24h NLR) (P= .001). At the end of 3rd month, 46.5% of patients had favorable (modified Rankin's score, mRS 0-2) and 32.4% had excellent (mRS 0-1) outcome. Patients without favorable/excellent outcome had significantly higher 24h NLRs. Regression analysis indicated that post-tPA, but not admission NLR, was an independent negative predictor of excellent (ß =-.216, P= .006) and favorable (ß = -.179, P= .034) outcome after adjustment for age, hypertension, and admission NIHSS. Nine patients who developed symptomatic intracerebral hemorrhage had elevated pre-tPA (7.6 ± 7.39 versus 3.33 ± 3.07, P< .001) and 24h NLR (26.2 ± 18.6 versus 5.78 ± 4.47, P< .001). Of note, receiver operating characteristics analysis failed to detect any reliable NLR threshold for absence of tPA effectiveness/dramatic response, 3rd month good/excellent outcome or any type tPA-induced hemorrhage. CONCLUSIONS: As a marker of stroke-associated acute stress response, the NLR, which increases during the first 24 hours, is an epiphenomenon of poor prognosis. However, pretreatment NLR values have no importance in predicting IV tPA response.

RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome.

OBJECTIVE: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies. METHODS: We identified consecutive patients from our institutional databases admitted in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the CNS (PACNS). In addition, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies. RESULTS: The RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS2 score (range -2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3-4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage correctly diagnosed 25 of 37 patients with RCVS2 scores 3-4 across the derivation and validation cohorts. CONCLUSION: RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.

Frontal Intermittent Rhythmic Delta Activity (FIRDA) in the Neurological Intensive Care: Prevalence, Determinants, and Clinical Significance.

INTRODUCTION: Frontal intermittent rhythmic delta activity (FIRDA), a transient rhythmic slow wave pattern over the anterior EEG leads, has been reported in a wide variety of cerebral lesions and different metabolic disturbances. Few authors have analyzed the frequency and clinical significance of FIRDA in the critical care setting. We aimed to better understand these issues in our intensive care cohort and if possible, try to delineate its underlying mechanisms. METHODS: Video-EEG reports of consecutive adult patients in the neurological intensive care unit (NICU) since 2009 were retrospectively reviewed to identify cases with FIRDA. Demographic, clinical, and laboratory data were obtained from EEG reports and patient charts. Age- and sex-matched patients with acute stroke, hospitalized in NICU and no FIRDA on video-EEG monitoring served as the control group. RESULTS: Among 162 patients who underwent video-EEG monitoring, FIRDA was documented in 17%. Female prevalence was 50% and age ranged from 23 to 82 years. Twenty-three (82%) of patients with FIRDA had a diagnosis of stroke. Comparison of demographic characteristics, EEG findings, metabolic disturbances and prognoses revealed no differences between stroke cases with and without FIRDA, except for higher frequency of acute and chronic isolated posterior circulation infarcts in patients with FIRDA. CONCLUSION: FIRDA is more commonly encountered in the neurocritical care setting as compared with outpatient EEG clinics. Our findings in stroke patients indicate that involved vascular territories may be related to the generation of FIRDA.

Coexisting vascular lesions in reversible cerebral vasoconstriction syndrome.

Background The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) is not known. Published cases have documented coexisting cervical artery dissection and unruptured aneurysms, raising the possibility that ultrastructural vessel wall abnormalities underlie the development of vascular lesions as well as RCVS. Methods In this retrospective study we compared the frequency of neurovascular abnormalities in 158 consecutive RCVS patients, 44 patients with primary angiitis of the central nervous system (PACNS, positive controls), and 177 non-stroke patients with acute neurological symptoms (non-arteriopathy controls). Results Coexisting neurovascular abnormalities were significantly higher ( p < 0.001) in RCVS (23%) as compared to the PACNS (5%) or non-arteriopathy groups (8%). Cervical artery dissections were noted only in the RCVS group (8%, p < 0.001). The RCVS group had more unruptured aneurysms than PACNS (13% vs. 5%, p = 0.099) or non-arteriopathy controls (13% vs. 7%, p = 0.05). Seven RCVS patients also had other vascular malformations (venous anomaly, cavernous malformations, fibromuscular dysplasia). There was no significant association between coexisting vascular abnormalities and brain lesions or discharge clinical outcome in the RCVS group. Conclusion The high prevalence and heterogeneous anatomy of coexisting vascular lesions suggest that subtle ultrastructural arterial wall abnormalities may contribute to their development and also predispose to RCVS.

Acute Middle Cerebral Artery Occlusion Treated by Thrombectomy in a Patient with Myelodysplastic Syndrome and Severe Thrombocytopenia.

OBJECTIVE: Experience on thrombolysis and/or thrombectomy for acute major ischemic strokes in the setting of deep (less than 40,000/mm(3)) thrombocytopenia is limited. METHODS: Case report and review of the literature. RESULTS: A 63-year-old female with myelodysplastic syndrome presented with left middle cerebral artery stroke within 2 hours of symptom onset. Severe thrombocytopenia (10.000/mm(3)) precluded systemic thrombolysis. However, endovascular thrombectomy provided successful recanalization and dramatic clinical recovery with NIHSS score decreasing from 20 to 2 soon after the procedure. Her modified Rankin scale was 1 at the end of the third month. CONCLUSION: This exceptional case highlights that neurothrombectomy could be feasible and of justifiable merit even in the setting of critically low thrombocytopenia if a meticulous procedure is followed in subjects with severe acute stroke.

Transcranial Doppler ultrasound in neurovascular diseases: diagnostic and therapeutic aspects.

Albeit no direct anatomical information can be obtained, neurosonological methods provide real-time determination of velocity, and spectral waveform of blood flow in basal intracranial arteries adds significant benefit to the care of the patients with neurovascular diseases. Several features, such as relative simplicity in terms of interpretation and performance, significantly low cost, totally non-invasiveness, portability, and excellent temporal resolution, make neurosonology increasingly popular tool for evaluation, planning, and monitoring of treatment, and for determining prognosis in various neurovascular diseases. Usefulness of transcranial Doppler in diagnosing/monitoring subarachnoid hemorrhage related vasospasm and sickle cell vasculopathy is already well known. Utility in diagnosis of intracranial arterial stenosis, acute occlusion and recanalization, intracranial hemodynamic effect of the cervical arterial pathologies, intracranial pressure increase, and cerebral circulatory arrest are also well established. Neurosonological determination of vasomotor reactivity, cerebral autoregulation, neurovascular coupling, and micro-embolic signals detection are useful in the assessment of stroke risk, diagnosis of right-to-left shunting, and monitoring during surgery and interventional procedures. Transcranial Doppler is also an evolving ultrasound method with a therapeutic potential such as augmentation of clot lysis and cerebral delivery of thrombolytic or neuroprotective agent loaded nanobubbles in neurovascular diseases. The aim of this study is to give an overview of current usage of the different ultrasound modalities in different neurovascular diseases.

Transcranial sonography in Perry syndrome.

We report transcranial sonography findings in two siblings (one male and one female) of a proband with Perry syndrome. A moderate degree of parkinsonism and prominent apathy were present in both subjects. The female patient displayed dyspnea and weight loss, indicating more advanced stages of Perry syndrome and for whom recently discovered specific mutation of Perry syndrome had been identified. In both patients, transcranial sonography exhibited marked substantia nigra (SN) hyperechogenicity, which is characteristic of idiopathic Parkinson's disease. This observation, together with similar findings reported for several different types of monogenic forms of parkinsonism, supports the suggestions that SN hyperechogenicity may be a common sign of nigral degeneration irrespective of underlying molecular pathology.

Occipital cortex activation studied with simultaneous recordings of functional transcranial Doppler ultrasound (fTCD) and visual evoked potential (VEP) in cognitively normal human subjects: effect of healthy aging.

We evaluated effect of aging, gender and eye (sighting) dominance on relationship between visual evoked flow response (VEFR) and visual evoked potential (VEP), which refers to neurovascular coupling. The VEFR was defined as a percentage increase of the ratio of mean blood flow velocity in the contralateral (according to the side of dominant eye processing) posterior cerebral artery P2 segment to those in ipsilateral middle cerebral artery from the baseline during half-field stimulation. Vasoneural coupling index (CI) was defined as "100 x VEFR/VEP P100 amplitude". Compared to the healthy elderly subjects (n: 19; female/male: 6/13, mean age: 69.7 +/- 7), younger participants (n: 28; female/male: 16/12; mean age: 31.1 +/- 4.7) had significantly higher VEFR for both sides: 18.9 +/- 6.7% versus 11.2 +/- 6.7%, p < 0.001 and 17.3 +/- 7.7% versus 11.8 +/- 5.5%, p: 0.007, for the hemisphere contralateral to dominant and nondominant eye (D and ND side), respectively. Albeit absence of any correlation between their latencies, VEP and VEFR amplitudes were well correlated. However, this was significant only for younger subjects and more evident in D side. The CI was higher in young subjects compared to those in old ones (6.49 +/- 2.79 versus 4.75 +/- 2.35, respectively, p = 0.007). But, this age-related trend remained as borderline when sides were analyzed individually: In the young subjects CI was 5.99 +/- 2.21 and 6.96 +/- 3.22 for D and ND sides, while those were 4.27 +/- 2.60 and 5.19 +/- 2.07 in old ones. This study confirmed diminished visual evoked flow in relation with advancing age, and suggested that "weakened" neurovascular coupling (as evidenced by a decreased VEP and VEFR correlation along with decreased CI) as one of the underlying mechanisms.

Enhanced cued recall has a high utility as a screening test in the diagnosis of Alzheimer's disease and mild cognitive impairment in Turkish people.

Enhanced cued recall (ECR) is highly sensitive and specific in discrimination of demented from non-demented elderly persons. The nature of the test promises that it can be applicable to subjects in different cultures and education level. We studied the utility of the test in a Turkish population. Eighty consecutive cases with dementia or mild cognitive impairment (MCI) and 33 elderly controls were studied. The utility of ECR was high in discriminating dementia from controls (area under curve (AUC)) of the ROC curve: 0.907 (95% confidence interval (CI): 0.830-0.953 for total recall), Alzheimer's disease from controls (AUC: 0.990 (95%CI: 0.934-0.998 for total recall)) and moderate (AUC: 0.625 (95%CI: 0.545-0.812 for third free recall)) in discriminating MCI from controls. Education did not affect the utility of the test. We conclude that ECR is a valuable test in assessment of elderly Turkish patients with a complaint of memory impairment.

Importance of jugular valve incompetence in contrast transcranial Doppler ultrasonography for the diagnosis of patent foramen ovale.

Transcranial Doppler (TCD) ultrasound with the intravenous injection of agitated saline as contrast (cTCD) is an effective method for detecting right-to-left intracardiac and extracardiac shunt (RLS); however, the sensitivity of cTCD in the diagnosis of RLS remains slightly less than that of transesophageal echocardiography, even in patients with adequate transtemporal ultrasonic bone windows. The authors present a case with cTCD underestimating RLS because of jugular valve incompetence in a 42-year-old man presenting with an episode of transient aphasia. Three weeks after transcatheter closure of a patent foramen ovale associated with an atrial septal aneurysm, he experienced 2 episodes of amaurosis fugax. Following a negative 45-minute embolus detection study with power M-mode TCD, the patient underwent a cTCD study with monitoring of the left middle cerebral artery (MCA), the anterior cerebral artery, and the submandibular extracranial internal carotid artery. A single microbubble (MB) was detected in the left MCA in only 1 of 5 studies; the remaining runs all failed to detect an RLS. Significant MB reflux was noted in the left internal jugular vein because of jugular valve incompetence. The authors conclude that incompetence of the jugular vein valve can result in a false negative cTCD study for RLS detection.

Cerebral Vasospasm Following Subarachnoid Hemorrhage.

Cerebral vasospasm and related ischemic stroke continue to be significant complicating factors in the course of many patients with subarachnoid hemorrhage from berry aneurysm rupture. The risk of this well-recognized but poorly understood complication can be estimated on the basis of patient medical history, neurologic examination, and head CT findings. Every patient with possible risk needs specialized neurologic intensive care unit care after aneurysm obliteration. Surgical and pharmacologic wash-out of subarachnoid blood around the basal arteries, proper management of intracranial pressure and fluid status, hyponatremia, hypomagnesemia, and fever, as well as use of calcium channel blockers, have been considered helpful in patient management prior to and with the symptomatic vasospasm development. Transcranial Doppler (TCD) ultrasound is important in detecting vasospasm before the patient suffers ischemic neurologic deficit or infarct. Elevated TCD velocities often initiate the use of triple-H (HHH: hypertension, hemodilution, and hypervolemia) therapy and subsequently guide it. Up to the end of the first 3 weeks after subarachnoid hemorrhage and aneurysm obliteration, development of any focal neurologic deficit or mental deterioration, unless convincingly proven otherwise, is assumed to be from cerebral vasospasm. When a hemodynamically significant vasospasm in the arterial segments of clinical concern is suggested, emergency cerebral angiography with balloon dilatation angioplasty or intra-arterial infusion of vasodilating agents may be helpful in relieving ischemic symptoms.

Diffusion MRI in three types of anoxic encephalopathy.

We report the clinical and diffusion-weighted MRI (DWI) features of three patients with different types of anoxic brain injury: attempted hanging (hypoxic hypoxia), carbon monoxide poisoning (histotoxic hypoxia), and hanging with cardiac arrest (hypoxic-ischemic encephalopathy, HIE). The first two patients, but not the third, recovered substantial neurological function. The distribution of DWI abnormalities was different and correlated well with the distinct neuropathological features of these entities. The prognosis after anoxic encephalopathy depends on the underlying mechanism and its severity, which may be reflected by DWI abnormalities.