Dynamic Internal Jugular Vein Compression by Hypertrophic Hyoid Bone: Management and Outcomes.

Extracranial osseous compression of the internal jugular vein (IJV) is exceedingly rare. The clinical manifestations of IJV obstruction are very heterogeneous and subtle, and arriving at a diagnosis can be challenging. We describe a case of dynamic IJV compression in a 40-year-old male with progressive, positional, ill-defined right periorbital and neck pain associated with photosensitivity. Imaging showed a hypertrophic right hyoid bone; computed tomography venogram (CTV) with challenging maneuvers demonstrated dynamic compression of the ipsilateral IJV by a hypertrophied hyoid bone and thyroid cartilage. The patient underwent decompression of the right jugular vein which resulted in the resolution of his symptoms. The clinical manifestations of extracranial IJV impingement are variable and diagnostically challenging. Disturbances in extracranial IJV outflow is a diagnosis of exclusion and could be responsible for atypical facial pain in a select group of patients. This entity should be considered in the differential of atypical facial, especially when symptoms tend to be positional.

Recurrent Stroke in Giant Cell Arteritis Despite Immunotherapy.

OBJECTIVE: To describe rare but important cerebrovascular complications of giant cell arteritis (GCA). CASE REPORT: We report a 59-year-old man who initially presented with vasculitis of the lower extremities. While on steroids, he developed strokes in multiple vascular territories. The conventional angiogram showed stenosis of bilateral carotid and vertebral vessels as they entered the dura. Temporal artery biopsy confirmed GCA. He began cyclophosphamide treatment, which stabilized his clinical course; however, this was switched to tocilizumab by an outside rheumatologist. Two months later, the patient had progression of vessel stenosis and suffered additional strokes. Despite interventions to augment cerebral perfusion, the infarctions continued to expand and the patient passed away. CONCLUSIONS: This case highlights several important features of strokes in GCA: the predilection for the dural entry point of cerebral blood vessels, the progression of disease despite steroids, and the need to quickly escalate treatment in these cases. As seen in our patient, however, this disease carries high morbidity and mortality and patients often have poor outcomes despite aggressive immunosuppression.

Ascending Spinal Cord Infarction Secondary to Recurrent Spinal Cord Cavernous Malformation Hemorrhage.

We report a case of a 58-year-old Hispanic man who developed ascending paraparesis over several weeks secondary to recurrent hemorrhages and resulting in spinal cord ischemia from a low thoracic spinal cord cavernous malformation. The patient's deterioration was attributed to recurrent hemorrhage of a thoracic intramedullary cavernous malformation at T11 resulting in vascular congestion and spinal cord ischemia. The patient was found to have a heterozygous mutation on exon 13 of gene KRIT1, which was consistent with autosomal dominant familial cerebral cavernous malformations. Expedited surgical intervention potentially could have prevented this patient's progressive paraplegia.

Minocycline-induced polyarteritis nodosa-like vasculitis presenting as brainstem stroke.

Minocycline use has been associated with the development of autoimmune disorders, including drug-induced vasculitis. Previously published reports suggest that clinical manifestations are limited to cutaneous, constitutional, or musculoskeletal symptoms. To our knowledge there has been only one reported patient with ischemic stroke in the setting of minocycline-induced vasculitis. We describe a 26-year-old woman, with no vascular risk factors, who had an ischemic pontine stroke in the setting of biopsy-proven minocycline-induced polyarteritis nodosa-like vasculitis. Discontinuation of minocycline resulted in resolution of the vasculitis, and she has not had any recurrent ischemic events. This report shows that ischemic strokes may occur as a result of minocycline-induced vasculitis. While this is likely a rare association, recognition is important given the widespread use of minocycline and the potential for devastating consequences in a young population. Consequently, drug-induced vasculitis should be considered in patients with an ischemic stroke taking minocycline.

Aortic fenestration for chronic aortic dissection type B complicated by transient ischemic attacks of spinal cord.

Spinal cord injury is a rare complication in patients with aortic dissection. The extrinsic arterial supply to the spinal cord, diminishing caudally, often becomes critically dependent on the great radicular artery (GRA) of Adamkiewicz at the thoracolumbar spine. There are no prior reports of spinal injury or ischemia caused by chronic aortic dissection. We report on a 51-year-old patient with chronic type B dissection of the aorta from below the subclavian takeoff through the iliac arteries, presented with multiple episodes of transient (1 to 5 minutes) spinal ischemic attacks, entailing sudden loss of motor and sensory functions in both legs, with collapse of the patient on the ground. GRA imaging acquired with 64-channel computed tomography angiography enabled aortic fenestration from T11 to L1, performed with supraceliac aortic cross-clamping (T8 to L2) via thoracoabdominal access. We critically appraise the pertinent literature.

Prolonged coma from refractory status epilepticus.

OBJECTIVE: Status epilepticus is a life-threatening medical condition. In its most severe form, refractory status epilepticus (RSE) seizures may not respond to first and second-line anti-epileptic drugs. RSE is associated with a high mortality and significant medical complications in survivors with prolonged hospitalizations. METHODS: We describe the clinical course of RSE in the setting of new onset lupus in a 31-year-old male who required prolonged barbiturate coma. RESULTS: Seizure stopped on day 64 of treatment. Prior to the resolution of seizures, discussion around withdrawal of care took place between the physicians and patient's family. Medical care was continued because of the patient's age, normal serial MRI studies, and the patient's reversible medical condition. CONCLUSION: Few evidence-based data exist to guide management of RSE. Our case emphasizes the need for continuous aggressive therapy when neuroimaging remains normal.

Clinical importance of cardiac troponin release and cardiac abnormalities in patients with supratentorial cerebral hemorrhages.

OBJECTIVE: To determine the Incidence of cardiac troponin T (cTnT) elevation, electrocardiographic (ECG) changes, and arrhythmias in supratentorial intracerebral hemorrhage (ICH) and their association with early mortality. PATIENTS AND METHODS: Patients with supratentorial ICHs admitted to Mayo Clinic, Rochester, Minn, from March 1998 to October 2003 were studied. We excluded moribund patients with ICHs who died within 12 hours of hospital admission. Cardiac troponin T levels measured on admission and day 2 were determined by a third-generation enzyme-linked immunosorbent assay. Continuous ECG monitoring was performed In all patients. Computed tomographic scans were graded and correlated with abnormal cardiac variables. RESULTS: Peak levels of cTnT were elevated at 0.035 to 1.2 microg/L (mean +/- SD, 0.27 +/- 0.38 microg/L) in 10 (20%) of 49 patients and were not associated with changes in creatine kinase MB fraction or ECG results. The cTnT levels did not correlate with location or side of hemorrhage or mortality at 30 days. Seventy (64%) of 110 patients displayed ECG abnormalities. The ECG changes did not correlate with the location or side of ICH, hydrocephalus, midline shift, or extension to the ventricles. CONCLUSION: The cTnT elevations in survivors of acute ICH are frequent but without confirmatory ECG changes that suggest mild myocardial injury. One-month mortality is not influenced by such cTnT elevations. In addition, ECG abnormalities are common but likely benign in patients with supratentorial ICH who survive the initial insult.

Tako-tsubo cardiomyopathy in aneurysmal subarachnoid hemorrhage: an underappreciated ventricular dysfunction.

OBJECT: Neurogenic stunned myocardium in aneurysmal subarachnoid hemorrhage (SAH) is associated with a wide spectrum of reversible left ventricular wall motion abnormalities and includes a subset of patients with a pattern of apical akinesia and concomitant sparing of basal segments called "tako-tsubo cardiomyopathy". METHODS: After obtaining institutional review board approval, the authors retrospectively identified among all patients admitted to the Mayo Clinic's Neurological Intensive Care Unit between January 1990 and January 2005 those with aneurysmal SAH who had met the echocardiographic criteria for tako-tsubo cardiomyopathy. Among 24 patients with SAH-induced reversible cardiac dysfunction, the authors identified eight with SAH-induced tako-tsubo cardiomyopathy. All eight patients were women with a mean age of 55.5 years (range 38.6-71.1). Seven patients presented with a poor-grade SAH, reflected by a Hunt and Hess grade of III or IV. Four patients underwent aneurysm clip application, and four underwent endovascular coil occlusion. The initial mean ejection fraction (EF) was 38% (range 25-55%), and the mean EF at recovery was 55% (range 40-68%). Cerebral vasospasm developed in six patients, but cerebral infarction developed in only three patients. CONCLUSIONS: The authors describe the largest cohort with aneurysmal SAH-induced tako-tsubo cardiomyopathy. In the SAH population, tako-tsubo cardiomyopathy predominates in postmenopausal women and is often associated with pulmonary edema, prolonged intubation, and cerebral vasospasm. Additional studies are warranted to understand the complex mechanism involved in tako-tsubo cardiomyopathy and its intriguing relationship to neurogenic stunned myocardium.

Subarachnoid hemorrhage: neurointensive care and aneurysm repair.

Aneurysmal subarachnoid hemorrhage (SAH) is often a neurologic catastrophe. Diagnosing SAH can be challenging, and treatment is complex, sophisticated, multidisciplinary, and rarely routine. This review emphasizes treatment in the intensive care unit, surgical and endovascular therapeutic options, and the current state of treatment of major complications such as cerebral vasospasm, acute hydrocephalus, and rebleeding. Outcome assessment in survivors of SAH and controversies in screening of family members are discussed.

Management of acute ischemic stroke.

The treatment of acute ischemic stroke has evolved from observation and the passage of time dictating outcome to an approach that emphasizes time from ictus, rapid response, and a dedicated treatment team. We review the treatment of acute ischemic stroke from the prehospital setting, to the emergency department, to the inpatient hospital setting. We discuss the importance of prehospital assessment and treatment, including the use of elements of the neurologic examination, recognition of symptoms that can mimic those of acute ischemic stroke, and rapid transport of patients who are potential candidates for thrombolytic therapy to hospitals with that capability. Coordinated management of acute ischemic stroke in the emergency department is critical as well, beginning with non-contrast-enhanced computed tomography of the brain. The advantages of a multidisciplinary dedicated stroke team are discussed, as are thrombolytic therapy and other inpatient treatment options. Finally, we cover evolving management strategies, treatments, and tools that could improve patient outcomes.

Digital video-electroencephalographic monitoring in the neurological-neurosurgical intensive care unit: clinical features and outcome.

BACKGROUND: Prolonged electroencephalographic (EEG) recordings in the neurological-neurosurgical intensive care unit (NICU) may be performed in patients with status epilepticus, repetitive seizure activity, or an encephalopathy with or without seizures. The electroclinical correlation and neurological outcome of patients undergoing digital video-EEG monitoring (DVEEG) in the NICU has not been determined. OBJECTIVES: To evaluate the clinical utility and prognostic importance of the DVEEG in the NICU. METHODS: We retrospectively evaluated 105 patients who underwent DVEEG in the NICU at the Mayo Clinic, Rochester, Minn, between January 1, 1994, and July 31, 2001. All patients had a routine EEG recording performed prior to DVEEG. RESULTS: The mean age of the patients at the time of the DVEEG was 54 years (age range, 16-88 years). The mean duration of the DVEEG was 2.9 days (range, 1-17 days). Forty-four patients (42%) had a severe encephalopathy (Glasgow Coma Scale score, <8) at the time of the DVEEG. Forty-five patients (42.8%) had generalized convulsive status epilepticus, 19 patients (18.1%) had nonconvulsive status epilepticus, and 7 patients (6.7%) had epilepsia partialis continua. The mean duration of follow-up was 7 months (range, 1-54 months). The outcome in 84 patients included death in 38 patients, severe neurological deficits, that is, bed bound and needs support for activities of daily living, in 6 patients, and a vegetative state in 3 patients. Fifteen individuals had no neurological impairment during follow-up. Refractory status epilepticus (P<.003), hypoxic-ischemic encephalopathy (P<.004), and multiple cerebral infarcts (P<.003) were the factors associated with increased mortality in univariate analysis. With multivariate logistic regression analysis only the presence of multiple strokes (P<.03; odds ratio, 5.62) was predictive of mortality. CONCLUSIONS: Continuous EEG monitoring is essential in the diagnosis and treatment of refractory status epilepticus or an encephalopathy with seizures in the NICU. A minority of these patients, however, experienced a favorable neurological outcome.

Predictors of cerebral infarction in aneurysmal subarachnoid hemorrhage.

BACKGROUND: Clinical and radiologic predictors of cerebral infarction occurrence and location after aneurysmal subarachnoid hemorrhage have been seldom studied. METHODS: We evaluated all patients admitted to our hospital with aneurysmal subarachnoid hemorrhage between 1998 and 2000. Cerebral infarction was defined as a new hypodensity located in a vascular distribution on computed tomography (CT) scan. RESULTS: Fifty-seven of 143 patients (40%) developed a cerebral infarction. On univariate analysis, occurrence of cerebral infarction was associated with a worse World Federation of Neurological Surgeons grade (P=0.01), use of ventriculostomy catheter (P=0.01), preoperative vasospasm (P=0.03), surgical clipping (P=0.02), symptomatic vasospasm (P<0.01), and vasospasm on transcranial Doppler ultrasonography (TCD) or repeat angiogram (P<0.01). On multivariable analysis, only presence of symptoms ascribed to vasospasm (P<0.01) and evidence of vasospasm on TCD or angiogram predicted cerebral infarction (P<0.01). TCD and angiogram agreed on the diagnosis of vasospasm in 73% of cases (95% CI, 63% to 81%), but the diagnostic accuracy of this combination of tests was suboptimal for the prediction of cerebral infarction occurrence (sensitivity, 0.72; specificity, 0.68; positive predictive value, 0.67; negative predictive value, 0.72). Location of the cerebral infarction on delayed CT was predicted by neurological symptoms in 74%, by aneurysm location in 77%, and by angiographic vasospasm in 67%. CONCLUSIONS: Evidence of vasospasm on TCD and angiogram is predictive of cerebral infarction on CT scan but sensitivity and specificity are suboptimal. Cerebral infarction location cannot be predicted in one quarter to one third of patients by any of the studied clinical or radiological variables.

Progressive neurological decline after partial spontaneous thrombosis of a Spetzler-Martin Grade 5 arteriovenous malformation in a patient with Leiden factor V mutation: management and outcome.

The coexistence of a large intracranial arteriovenous malformation (AVM) and a hypercoagulation disorder is rare. The AVM puts the patient at risk for progressive neurological deficit, seizures, and, most importantly, intracranial hemorrhage The hypercoagulation disorder may result in an increased risk of stroke. The authors describe a 42-year-old man with a Spetzler-Martin Grade 5 AVM who experienced progressive neurological decline. He was subsequently discovered to have partial thrombosis of the AVM, deep cerebral and cortical venous thrombosis, and a hypercoagulation disorder. Hypercoagulation disorders causing neurological deficits are usually treated with anticoagulant medications; however, this approach was not thought to be safe in the presence of a large AVM. Therefore, the AVM nidus was surgically extirpated and a ventriculoperitoneal shunt was placed to treat the increased intracranial pressure caused by the cortical and deep cerebral venous thrombosis. Subsequently, lifelong oral anticoagulation was prescribed. The patient had a progressive neurological recovery and is now living independently at home. The occurrence of partial or complete spontaneous thrombosis of an AVM nidus should raise the possibility of an underlying hypercoagulation disorder.

Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm.

Seven patients, after seemingly uncomplicated surgery for ascending aorta aneurysm or dissection, with or without aortic valve replacement, developed an unusual and fairly stereotyped biphasic neurologic disorder without imaging evidence of related cerebral ischemia or infarct. The initial phase was mild, nonprogressive, or receding. The latent and progressive phase closely resembled a progressive supranuclear palsy phenotype. The disorder may prove to be self-limiting but leaves the patients with considerable neurologic deficits.

Cerebral angiitis mimicking posterior leukoencephalopathy.

Posterior leukoencephalopathy typically denotes neurotoxicity from immunosuppressive agents, malignant hypertension or eclampsia. It has not been documented in central nervous system angiitis. We present three cases associated with isolated cerebral angiitis after review of all cases of isolated CNS angiitis from 1998 to 2000. Additional lesions beyond the posterior occipital lesions became evident in two cases. A cerebral angiogram and possibly brain biopsy to detect isolated angiitis should be considered in patients with posterior leukoencephalopathy of unknown etiology.

Value of diffusion-weighted imaging in patients with a nonlocalizing examination and vasospasm from subarachnoid hemorrhage.

We evaluated the value of diffusion-weighted imaging (DWI) in patients with suspected vasospasm from subarachnoid hemorrhage (SAH). Magnetic resonance imaging (MRI), including DWI, was performed in 5 patients with vasospasm resulting from SAH. Restricted diffusion (ischemia) was present in 3 patients with symptomatic vasospasm. A diffusion abnormality was not demonstrated in 2 patients with asymptomatic vasospasm despite persistently elevated velocities by transcranial Doppler ultrasonography in 1. In this patient, serial MRI studies showed no ischemia. Our results suggest that DWI may provide a method to assess the presence and severity of ischemia in patients with SAH and vasospasm and assist in patient management.

Computed tomographic determinants of neurologic deterioration in patients with large middle cerebral artery infarctions.

OBJECTIVE: To identify specific radiographic features on computed tomographic (CT) imaging that can predict neurologic deterioration in patients with large middle cerebral artery (MCA) infarctions. PATIENTS AND METHODS: We performed a 10-year retrospective review from January 1, 1991, through December 31, 2001, of medical records and CT scans of patients with large MCA infarctions. Neurologic deterioration was defied as progressive drowsiness or signs of herniation. The CT scans were grouped into 3 periods according to time after ictus. Radiographic features reviewed included hyperdense middle cerebral artery sign (HMCAS), more than a 50% loss of MCA territory, sulcal effacement, loss of lentiform nucleus or insular ribbon, and septal and pineal shift. Demographic and radiographic variables were compared by using t tests and the Fisher exact test. Prognostic values were calculated for all significant radiographic variables. RESULTS: Thirty-four CT scans in 22 patients before neurologic deterioration were compared with 47 scans obtained in 14 patients without neurologic worsening. There were no demographic differences between groups. Initial analysis revealed that early (<12 hours) involvement of more than 50% of the MCA territory (P=.047; odds ratio [OR], 14.02; 95% confidence interval [CI], 1.04-189.42) and the HMCAS at any time (P<.001; OR, 21.6; 95% CI, 3.54-130.04) were independent predictors of neurologic deterioration. The positive predictive power for early involvement of more than 50% of the MCA and the HMCAS was 0.75 and 0.91, respectively. CONCLUSION: The HMCAS and early CT evidence of more than 50% MCA involvement are predictive of neurologic deterioration in patients with large MCA infarcts.

Symptomatic vasospasm and outcomes following aneurysmal subarachnoid hemorrhage: a comparison between surgical repair and endovascular coil occlusion.

OBJECT: The authors studied patients with aneurysmal subarachnoid hemorrhage (SAH) to determine whether the incidence of symptomatic vasospasm or overall clinical outcomes differed between patients treated with craniotomy and clip application and those treated by endovascular coil occlusion. METHODS: The authors reviewed 415 consecutive patients with aneurysmal SAH who had been treated with either craniotomy and clip application or endovascular coil occlusion at a single institution between 1990 and 2000. Three hundred thirty-nine patients underwent surgical clip application procedures, whereas 76 patients underwent endovascular coil occlusion. Symptomatic vasospasm occurred in 39% of patients treated with clip application, 30% of patients treated with endovascular coil occlusion, and 37% of patients overall. Compared with patients treated with clip application, patients treated with endovascular coil occlusion were more likely to suffer acute hydrocephalus (50 compared with 34%, p = 0.008) and were more likely to harbor aneurysms in the posterior circulation (53 compared with 20%, p < 0.001). Logistic regression models controlling for patient age, admission World Federation of Neurosurgical Societies (WFNS) grade, acute hydrocephalus, aneurysm location, and day of treatment revealed that, among patients with an admission WFNS grade of I to III, endovascular coil occlusion carried a lower risk of symptomatic vasospasm (odds ratio [OR] 0.34, 95% confidence interval [CI] 0.14-0.8) and death or permanent neurological deficit due to vasospasm (OR 0.28, 95% CI 0.08-1) compared with craniotomy and clip application. Similar models revealed no difference in the likelihood of a Glasgow Outcome Scale score of 3 or less at the longest follow-up review (median 6 months) between treatment groups (OR 0.58, 95% CI 0.28-1.21). CONCLUSIONS: Patients with better clinical grades (WFNS Grades I-III) at hospital admission were less likely to suffer symptomatic vasospasm when treated by endovascular coil occlusion, compared with craniotomy and clip application. Nevertheless, there was no significant difference in overall outcome at the longest follow-up examination between the two treatment groups.