Pearls & Oy-sters: A Case Report of Holmes Tremor Due to Nigrostriatal Dopamine Disruption That Responded to Dopamine Replacement Therapy.

Holmes tremor (HT), also known as midbrain, rubral, or cerebellar pathway outflow tremor, occurs because of disturbances of the cerebellothalamic pathway. This tremor is usually related to lesions in the midbrain peduncular region involving the superior cerebellar peduncle, the red nucleus, and possibly the nigrostriatal circuitry. Common etiologies resulting in HT include tumor, ischemia, and demyelination. We report a case of progressive left-sided HT in an otherwise healthy man with additional symptoms of parkinsonism, hypoesthesia, right oculomotor nerve palsy, cognitive dysfunction, and hypersomnolence. Imaging investigations revealed a right-sided thalamic and midbrain glioma. Dopamine transport imaging demonstrated significant dopaminergic denervation in the right caudate and putamen. The degree of striatal dopamine transporter deficiency was more severe than expected in a patient with Parkinson disease. A trial of dopaminergic agent resulted in significant improvement of the tremor and associated symptoms. Interruption of the nigrostriatal pathway can occur in cases of HT because of midbrain peduncular lesion. The striatal dopaminergic function imaging may have a role in assessing presynaptic dopamine dysfunction and guiding treatment.

Extracranial Venous abnormalities: A true pathological finding in patients with multiple sclerosis or an anatomical variant?

OBJECTIVE: To evaluate the extracranial venous anatomy with contrast-enhanced MR venogram (CE-MRV) in patients without multiple sclerosis (MS), and assess the prevalence of various venous anomalies such as asymmetry and stenosis in this population. MATERIALS AND METHODS: We prospectively recruited 100 patients without MS, aged 18-60 years, referred for contrast-enhanced MRI. They underwent additional CE-MRV from skull base to mediastinum on a 3T scanner. Exclusion criteria included prior neck radiation, neck surgery, neck/mediastinal masses or significant cardiac or pulmonary disease. Two neuroradiologists independently evaluated the studies to document asymmetry and stenosis in the jugular veins and prominence of collateral veins. RESULTS: Asymmetry of internal jugular veins (IJVs) was found in 75 % of subjects. Both observers found stenosis in the IJVs with fair agreement. Most stenoses were located in the upper IJV segments. Asymmetrical vertebral veins and prominence of extracranial collateral veins, in particular the external jugular veins, was not uncommon. CONCLUSION: It is common to have stenoses and asymmetry of the IJVs as well as prominence of the collateral veins of the neck in patients without MS. These findings are in contrast to prior reports suggesting collateral venous drainage is rare except in MS patients. KEY POINTS: • The venous anatomy of the neck in patients without MS demonstrates multiple variants • Asymmetry and stenoses of the internal jugular veins are common • Collateral neck veins are not uncommon in patients without MS • These findings do not support the theory of chronic cerebrospinal venous insufficiency • MR venography is a useful imaging modality for assessing venous anatomy.

Early Rivaroxaban Use After Cardioembolic Stroke May Not Result in Hemorrhagic Transformation: A Prospective Magnetic Resonance Imaging Study.

BACKGROUND AND PURPOSE: Early anticoagulation after cardioembolic stroke remains controversial because of the potential for hemorrhagic transformation (HT). We tested the safety and feasibility of initiating rivaroxaban ≤14 days after cardioembolic stroke/transient ischemic attack. METHODS: A prospective, open-label study of patients with atrial fibrillation treated with rivaroxaban ≤14 days of transient ischemic attack or ischemic stroke (National Institute of Health Stroke Scale <9). All patients underwent magnetic resonance imaging <24 hours of rivaroxaban initiation and day 7. The primary end point was symptomatic HT at day 7. RESULTS: Sixty patients (mean±SD age 71±19 years, 82% stroke/18% transient ischemic attack) were enrolled. Median (interquartile range) time from onset to rivaroxaban was 3 (5) days. At treatment initiation, median National Institute of Health Stroke Scale was 2 (4), and median diffusion-weighted imaging volume was 7.9 (13.7) mL. At baseline, HT was present in 25 (42%) patients (hemorrhagic infarct [HI]1=19, HI2=6). On follow-up magnetic resonance imaging, no patients developed symptomatic HT. New asymptomatic HI1 developed in 3 patients, and asymptomatic progression from HI1 to HI2 occurred in 5 patients; otherwise, HT remained unchanged at day 7. CONCLUSIONS: These data support the safety of rivaroxaban initiation ≤14 days of mild-moderate cardioembolic stroke/transient ischemic attack. Magnetic resonance imaging evidence of petechial HT, which is common, does not appear to increase the risk of symptomatic HT.

Anaemia on admission is associated with more severe intracerebral haemorrhage and worse outcomes.

BACKGROUND: Lower haemoglobin levels may impair cerebral oxygen delivery and threaten tissue viability in the setting of acute brain injury. Few studies have examined the association between haemoglobin levels and outcomes after spontaneous intracerebral haemorrhage. AIMS: We evaluated whether anaemia on admission was associated with greater intracerebral haemorrhage severity and worse outcome. METHODS: Consecutive patients with spontaneous intracerebral haemorrhage were analyzed from the Registry of the Canadian Stroke Network. Admission haemoglobin was related to stroke severity (using the Canadian Neurological Scale), modified Rankin score at discharge, and one-year mortality. Adjustment was made for potential confounders including age, gender, medical history, warfarin use, glucose, creatinine, blood pressure, and intraventricular haemorrhage. RESULTS: Two thousand four hundred six patients with intracerebral haemorrhage were studied of whom 23% had anaemia (haemoglobin <120 g/l) on admission, including 4% with haemoglobin <100 g/l. Patients with anaemia were more likely to have severe neurological deficits at presentation [haemoglobin ≤ 100 g/l, adjusted odds ratio 4.04 (95% confidence interval 2.39, 6.84); haemoglobin 101-120 g/l, adjusted odds ratio 1.93 (95% confidence interval 1.43, 2.59), both P < 0.0001]. In nonanticoagulated patients, severe anaemia was also associated with poor outcome (modified Rankin score 4-6) at discharge [haemoglobin ≤ 100 g/l, adjusted odds ratio 2.42 (95% confidence interval 1.07-5.47), P = 0.034] and increased mortality at one-year [haemoglobin ≤ 100 g/l, adjusted hazard ratio 1.73 (95% confidence interval 1.22-2.45), P = 0.002]. CONCLUSIONS: Anaemia on admission is associated with greater intracerebral haemorrhage severity and worse outcomes. The utility of transfusion remains unclear in this setting.

Auditory-evoked potentials during coma: do they improve our prediction of awakening in comatose patients?

OBJECTIVE: The mismatch negativity (MMN), an auditory event-related potential, has been identified as a good indicator of recovery of consciousness during coma. We explored the predictive value of the MMN and other auditory-evoked potentials including brainstem and middle-latency potentials for predicting awakening in comatose patients after cardiac arrest or cardiogenic shock. MATERIALS AND METHODS: Auditory brainstem, middle-latency (Pa wave), and event-related potentials (N100 and MMN waves) were recorded in 17 comatose patients and 9 surgical patients matched by age and coronary artery disease. Comatose patients were followed up daily to determine recovery of consciousness and classified as awakened and nonawakened. RESULTS: Among the auditory-evoked potentials, the presence or absence of MMN best discriminated between patients who awakened or those who did not. Mismatch negativity was present during coma in all patients who awakened (7/7) and in 2 of those (2/10) who did not awaken. In patients who awakened and in whom MMN was detected, 3 of those awakened between 2 and 3 days and 4 between 9 and 21 days after evoked potential examination. All awakened patients had intact N100 waves and identifiable brainstem and middle-latency waves. In nonawakened patients, N100 and Pa waves were detected in 5 cases (50%) and brainstem waves in 9 (90%). CONCLUSIONS: The MMN is a good predictor of awakening in comatose patients after cardiac arrest and cardiogenic shock and can be measured days before awakening encouraging ongoing life support.

Long-lasting functional disabilities in patients who recover from coma after cardiac operations.

BACKGROUND: Uncertainty regarding the long-term functional outcome of patients who awaken from coma after cardiac operations is difficult for families and physicians and may delay rehabilitation. We studied the long-term functional status of these patients to determine if duration of coma predicted outcome. METHODS: We followed 71 patients who underwent cardiac operations; recovered their ability to respond to verbal commands after coma associated with postoperative stroke, encephalopathy, and/or seizures; and were discharged from the hospital. The Glasgow Outcome Scale Extended (GOSE) was used to assess functional disability 2 to 4 years after discharge. Outcomes were classified as favorable (GOSE scores 7 and 8) and unfavorable (GOSE scores 1-6). RESULTS: Of 71 patients identified, 39 were interviewed, 15 died, 1 refused to be interviewed, and 16 were lost to follow-up. Of the 54 patients with completed GOSE evaluations, only 15 (28%) had favorable outcomes. Among patients with unfavorable outcomes, 15 (28%) died, 14 (26%) survived with moderate disabilities, and 10 (18%) had severe disabilities. Factors associated with unfavorable outcomes were increases in duration of coma (p = 0.007), time in intensive care (p = 0.006), length of hospitalization (p = 0.004), and postoperative serum creatine kinase levels (p = 0.006). Only duration of coma was an independent predictor of unfavorable outcome (odds ratio [OR], 1.25; 95% confidence interval [CI], 1.008-1.537; p = 0.042). Patients with durations of coma greater than 4 days were more likely to have unfavorable outcomes (OR, 5.1; 95% CI, 1.3-21.3; p = 0.02). CONCLUSIONS: Two thirds of comatose patients who survived to discharge after cardiac operations had unfavorable long-term functional outcomes. A longer duration of unconsciousness is a predictor of unfavorable outcome.

Heparin dosing is associated with diffusion weighted imaging lesion load following aneurysm coiling.

BACKGROUND AND PURPOSE: Diffusion weighted imaging (DWI) may be used to evaluate post-coiling ischemia. Heparinization protocols for cerebral aneurysm coiling procedures differ among operators and centers, with little literature surrounding its effect on DWI lesions. The goal of this study was to determine which factors, including heparinization protocols, may affect DWI lesion load post-coiling. MATERIALS AND METHODS: A review of 135 coiling procedures over 5 years at our centre was performed. Procedural data including length of procedure, number of coils used, stent or balloon assistance and operators were collected. Procedures were either assigned as using a bolus dose (>2000 U at any one time) or small aliquots of heparin (≤2000 U). Postprocedure DWI was reviewed and lesions were classified as small (< 5mm), medium (5-10 mm) or large (>10 mm). The cases were then classified into group 1 (≤5 small lesions) or group 2 (>5 small lesions or ≥1 medium or large lesion). Multivariate regression of the procedural variables for the two groups was calculated. A p value of <0.05 was considered significant. RESULTS: There were 78 procedures in group 1 and 57 procedures in group 2. Patients who received small aliquots (n=37) versus boluses of heparin (n=98) intraprocedurally had significantly greater frequency and size of DWI lesions (p=0.03). None of the other procedural variables was found to impact on lesion load. CONCLUSIONS: More substantial DWI lesions were associated with small aliquots of heparin dosage compared with bolus doses. Heparin boluses should be preferentially administered during aneurysm coiling.

Outcome in small aneurysms (<4 mm) treated by endovascular coiling.

BACKGROUND: Coiling of small aneurysms can be technically challenging. These aspects of coiling tend to be less problematic in medium to large aneurysms as they are more accommodating of microcatheters and coils. When physicians are asked their opinion regarding aneurysm coilability in small aneurysms, the decision often lies in the operator's feeling that they could reasonably exclude the aneurysm with a complication rate similar to larger aneurysms. The purpose of our study was to investigate the feasibility, intraprocedural rupture rates and long term durability of endovascular coiling for small (≤4 mm) aneurysms compared with non-small (>4 mm) aneurysms. To control for factors such as vessel tortuosity and aneurysm location, a control group was chosen matched to the study group both in age and aneurysm location. MATERIALS AND METHODS: A retrospective review of 360 intracranial aneurysms coiled at our institution between 2003 and 2008 was performed. For the control group, intracranial aneurysms coiled in the same period matched to location and age were chosen. RESULTS: The frequency of intraprocedural perforations was 4/34 (0.12) and 3/68 (0.04) for the small and non-small cohort, respectively (p=0.22). All patients who had a perforation in the small aneurysm groups had a good clinical outcome compared with 1/3 in the non-small group (two mortalities). The frequency of recanalization for the small and non-small groups was 3/34 (0.09) and 23/68 (0.33), respectively (p=0.006). There was no retreatments in the small aneurysm group and five (0.07) in the non-small group (p=0.116). CONCLUSION: Coiling of small (≤4 mm) aneurysms is feasible with a reasonable complication rate. There is a non-significant increase in frequency of intraprocedural rupture with coiling of small aneurysms compared with controls matched to aneurysm location and age but this is not associated with increased morbidity. Coiling of small aneurysms leads to durable results at long term follow-up.

Transcortical venous approach for direct embolization of a cavernous sinus dural arteriovenous fistula: technical case report.

BACKGROUND AND IMPORTANCE: Dural arteriovenous fistulas (dAVFs) represent 10% to 15% of all intracranial arteriovenous malformations. Most often, embolization is accomplished with transfemoral catheter techniques. We present a case in which embolization of a cavernous sinus dAVF was made possible through transcranial cannulation of a cortical draining vein. CLINICAL PRESENTATION: An 82-year-old woman presented with diplopia, left sixth cranial nerve palsy, intraocular hypertension, and bilateral chemosis. Angiography revealed a complex cavernous dAVF with cortical venous reflux, supplied by both external carotid arteries and the left meningohypophyseal trunk. Percutaneous transvenous access failed, and only partial occlusion was achieved by transarterial embolization. A frontotemporal craniotomy was performed to access the superficial middle cerebral vein in the left sylvian fissure. Under fluoroscopic guidance, a microcatheter was advanced through this vein to the floor of the middle cranial fossa and into the dAVF, permitting coil occlusion. CONCLUSION: This transcranial vein technique may be a useful adjunct in dAVF therapy when percutaneous transarterial or transvenous approaches fail or are not possible.

Anoxic-ischemic encephalopathy and strokes causing impaired consciousness.

Coma due to global or focal ischemia or hemorrhage is reviewed. Impaired consciousness due to anoxic-ischemic encephalopathy after cardiac arrest is common but prognostically problematic. Recent guidelines need to be refined for those patients who have received therapeutic hypothermia. Strokes, both ischemic and hemorrhagic, can affect the level of consciousness by damaging specific brain structures involved in alertness because of widespread cerebral injury or secondary cerebral or systemic complications.

Angioplasty as an adjuvant therapy for the treatment of acute ischemic stroke.

BACKGROUND: Different endovascular techniques can be employed to achieve vessel recanalization in acute stroke. We assessed whether an endovascular strategy that included angioplasty was safe and effectively recanalized acutely occluded intracranial vessels. METHODS: We retrospectively reviewed 70 patients that received intra-arterial therapy for acute stroke. Patients were divided into two groups depending on whether they had received angioplasty as part of their endovascular treatment. RESULTS: Angioplasty was used in the treatment of 35/70 patients (50%). Median baseline NIHSS was 15. The site of occlusion was at the M1 in 11 patients, M1/M2 in 3, ICA/M1 in 13 and vertebrobasilar in 8 patients. Intravenous thrombolysis was administered to 16/35 patients (46%). Angioplasty was used alone in 4 patients, in combination with intra-arterial thrombolysis in 27 and with a mechanical retrieval device or stent in 13 patients. Recanalization (TICI 2-3) was achieved in 23/35 patients (66%). Median time from symptom onset to recanalization was six hours. In patients where angioplasty was employed, symptomatic intracranial hemorrhage occurred in 2/35 (6%), which was similar to patients that were not treated with angioplasty. A favorable functional outcome (mRS=2) was achieved in 20% (7/35) at 24 hour and 34% (12/35) at one month. All patients that had a favorable outcome had recanalized. CONCLUSION: In this small cohort, an endovascular treatment strategy that employed angioplasty was safe and effectively recanalized acutely occluded intracranial vessels. Angioplasty should be considered as a potential treatment option in interventional acute stroke trials.

Predictors of duration of unconsciousness in patients with coma after cardiac surgery.

OBJECTIVES: To describe clinical and brain imaging characteristics of patients who recovered and did not recover consciousness from a coma after cardiac surgery and to investigate predictors of the duration of unconsciousness in those patients who ultimately recovered consciousness. DESIGN: A retrospective analysis from a cohort of patients who developed coma after cardiac surgery. SETTING: A single university hospital. PARTICIPANTS: One hundred twelve patients with postoperative stroke, encephalopathy, and/or seizures who remained in coma longer than 24 hours after cardiac surgery. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The authors analyzed the patients' perioperative and intraoperative characteristics, laboratory values, noncontrast head computed tomography (CT) scans, and outcomes. Patients who did not recover consciousness (n = 16) were more likely to have been classified preoperatively as New York Heart Association class III/IV (p = 0.037). In patients who recovered consciousness (n = 96), only increased preoperative serum creatinine was an independent predictor of a longer duration of unconsciousness (p = 0.011). In patients who eventually recovered consciousness and had no acute findings on brain imaging, preoperative creatinine (p = 0.014), the lowest postoperative hemoglobin (p = 0.039), and surgical emergency (p = 0.045) were independent predictors of the duration of unconsciousness (p = 0.002). In patients who regained consciousness but had acute findings on brain imaging, cardiogenic shock (p = 0.012) and the insertion of an intra-aortic balloon pump before or during surgery (p = 0.025) predicted longer durations of unconsciousness (p < 0.001). CONCLUSIONS: In patients who ultimately recovered consciousness after being in a coma for at least 24 hours after cardiac surgery and have no abnormality on a brain CT scan, elevated preoperative serum creatinine, urgent cardiac surgery, and lower postoperative hemoglobin were correlated with an increased duration of unconsciousness.

Primary stenting for recurrent stenosis following carotid endarterectomy.

BACKGROUND: Carotid angioplasty and stenting is an accepted alternative treatment for severe restenosis following carotid endarterectomy. Balloons may not be required to effectively treat these lesions, given their altered histopathology compared to primary atherosclerotic plaque and tendency to be less calcified. Primary stenting using self-expanding stents alone may, therefore, be a safe and effective treatment for restenosis post-carotid endarterectomy. METHODS: We review our experience in the treatment of 12 patients with symptomatic severe restenosis following carotid endarterectomy with primary stent placement alone. RESULTS: Self-expanding stent placement alone reduced the mean internal carotid artery stenosis from 85% to 29%. Average peak systolic velocity determined at the time of ultrasonography decreased from 480 cm/s at initial presentation to 154 cm/s post-stent deployment and further decreased to 104 cm/s at one year follow-up. The stented arteries remained widely patent with no evidence of restenosis. A single peri-procedural ipsilateral transient ischemic event occurred. There were no cerebral or cardiac ischemic events recorded at one year of follow-up. CONCLUSIONS: In this series, primary stent placement without use of angioplasty balloons was a safe and effective treatment for symptomatic restenosis following carotid endarterectomy.

Direct carotid-cavernous fistula causing brainstem venous congestion.

A 39-year-old man who presented with unilateral proptosis and periocular pain rapidly developed reduced consciousness, facial numbness, dysarthria, and gait ataxia from a direct carotid-cavernous fistula (CCF) with drainage into posterior fossa veins. Brain MRI revealed abnormal signal throughout the brainstem, indicative of venous hypertension and edema. Closure of the fistula by detachable balloon eliminated the clinical and imaging abnormalities. This is the fifth reported case of brainstem complications of a direct CCF. It highlights potentially serious complications of this condition and their reversibility with prompt treatment.

Hemodynamic instability during carotid artery stenting: the relative contribution of stent deployment versus balloon dilation.

OBJECT: Hemodynamic instability may complicate carotid angioplasty and stenting in up to 40% of patients. The authors have previously demonstrated that primary self-expanding stent placement alone can gradually dilate severely stenosed carotid arteries without the use of balloons. The authors hypothesized that eliminating the balloon would reduce carotid baroreceptor stimulation, thereby decreasing the incidence of hemodynamic instability. METHODS: Ninety-seven high surgical risk patients with symptomatic, severely stenosed carotid arteries were treated with the intention of using a self-expanding stent alone. Seventy-seven arteries (79%) were treated with stenting alone, and 20 required angioplasty (21%). RESULTS: Intraprocedural bradycardia (heart rate < 60 bpm) developed in 29 patients (38%) and hypotension (systolic blood pressure < 90 mm Hg) occurred in 1 patient (1%) treated with stenting alone. Fourteen patients (70%) who underwent angioplasty and stenting had bradycardia, and hypotension developed in 4 (20%). Atropine, glycopyrrolate, or vasopressors were required in 8% of patients who received stenting alone, compared to 30% of patients who underwent angioplasty. In the first 24 hours after treatment, hypotension or bradycardia developed in 25 patients (32%) who had undergone stent placement alone, and in 15 patients (75%) after stent placement and balloon angioplasty. There was no difference in the occurrence of intra- or postprocedural hypertension (systolic blood pressure > 160 mm Hg) between patients treated with stenting alone or stenting and balloons. Factors independently associated with hemodynamic depression included baseline heart rate and balloon use. CONCLUSIONS: Hemodynamic instability during and after carotid artery stenting was observed more frequently when balloon angioplasty was required than when stent placement was performed without concurrent balloon angioplasty.

Results using a self-expanding stent alone in the treatment of severe symptomatic carotid bifurcation stenosis.

OBJECT: Conventional endovascular therapy for carotid stenosis involves placement of an embolic protection device followed by stent insertion and angioplasty. A simpler approach may be placement of a stent alone. The authors determined how often this approach could be used to treat patients with carotid stenosis, and assessed which factors would preclude this approach. METHODS: Over a period of 6 years, 97 patients with symptomatic carotid stenosis were treated with the intention of using a "stent-only" approach. Arteries in 77 patients (79%) were treated with stents alone, 13 required preinsertion balloon dilation, 6 postinsertion dilation, and 1 both pre- and postinsertion dilation. RESULTS: The mean stenosis according to North American Symptomatic Carotid Endarterectomy Trial criteria was reduced from 82 to 40% in the stent-only group and from 89 to 37% in the stent and balloon angioplasty group. The 30-day stroke and death rate was 7.2%. Patients were followed for a mean of 15 months. In the stent-alone group, the mean preoperative Doppler peak systolic velocity (PSV) was 409 cm/second, with an internal carotid artery/common carotid artery (ICA/CCA) ratio of 7.2. At follow-up review, the PSV decreased to 153 cm/second and the ICA/CCA ratio to 2.1. In the angioplasty group the mean preoperative PSV was 496 cm/second and the ICA/CCA ratio was 9.2, decreasing to 163 cm/second and 2, respectfully, at follow-up evaluation. Restenosis occurred in 12.8% of patients at 6 months and in 15.9% at 1 year. One stroke occurred during the follow-up period in each group. Using multivariable analysis, factors precluding the "stent-only" approach were as follows: severity of stenosis, circumferential calcification, and no history of hyperlipidemia. CONCLUSIONS: Balloons may not be required to treat all patients with carotid stenosis. A stent alone was feasible in 79% of patients, and 79% of patients were alive and free from ipsilateral stroke or restenosis at 1 year. Restenosis rates with this approach are higher than with conventional angioplasty and stent insertion. Carotid arteries with very severe stenoses (> 90%) and circumferential calcification may be more successfully treated with angioplasty combined with stent placement.