Abnormal T2 Signal Research Articles

Transient awakening from vegetative state: Is high‐dose zolpidem more effective?

Vegetative state (VS) and the minimally conscious state are chronic disorders of consciousness (DOC), following severe brain injury. Zolpidem (Zp) is a non-benzodiazepine sedative/hypnotic drug, acting as a full agonist at the α1-containing gamma-aminobutyric acid (GABAA) receptors. Previously reported data have shown few cases of transient arousal from DOC after Zp administration at the maximum dosage of 20 mg/day,1 although the Zp ‘awakening mechanism’ is still not well known. A 52-year-old woman was in a persistent VS for 3 years following cardiac arrest, with a Coma Recovery Scale-Revised (CRS-R) score of 7. Brain magnetic resonance imaging showed widespread T2 signal abnormalities, mainly involving the basal ganglia, thalamus and frontal cortices, whereas a diffuse low amplitude theta delta-like rhythm was recorded during electroencephalography (EEG). As neither drugs nor behavioral rehabilitation were successful in improving the patient's condition, Zp was introduced and the dose was increased by 5 mg/week. The patient was evaluated through CRS-R and prolonged EEG, before and after the different dosage administrations. An evident improvement in awareness and wakefulness after Zp was detected by the rehabilitation team and her relatives only when a dose of 20 mg was reached. Indeed, EEG showed an increase in amplitude and voltage, with a clear theta rhythm over the right temporo-occipital region, either in resting-state or after acoustic and visual stimulation, and a CRS-Score of 11. Thus, we decided to increase Zp dosage up to 30 mg (administered for around 3 weeks), with a further improvement of CRS-R score (i.e. 13) and EEG (presence of theta-beta rhythm over both the temporal areas), and without relevant side-effects. Our case further supports the hypothesis of a positive effect of Zp, although at higher dosage, in improving consciousness in VS. After brain injury or cerebral anoxic damage there is a change either in the levels of glutamate (resulting in neurotoxicity) or GABA neurotransmission, with reduced cell metabolism and blood flow in brain areas adjacent to the damaged area. By binding to modified GABAA receptors of the ‘neurodormant cells’, Zp may cause reversal of the abnormal state and associated metabolic inhibition, with a consequent arousal.2 For the first time, we used a higher dose of Zp (30 mg) to evaluate whether the drug's response could be dose-dependent. Indeed, our patient's improvement was strictly related to the increase in Zp dosage, with a relatively good response at 30 mg. We are not able to explain this interesting finding, although a more effective action of high-dose Zp on the centrothalamic activity (by potentiating the ‘mesocircuit’),3 could be taken into account. Zp may be a valuable treatment in improving consciousness, although further studies should be fostered to confirm its efficacy, the proper dosage, and long-term safety.

Phase white matter signal abnormalities in patients with clinically isolated syndrome and other neurologic disorders.

Identifying MRI biomarkers that can differentiate multiple sclerosis patients from other neurological disorders is a subject of intense research. Our aim was to investigate phase WM signal abnormalities for their presence, prevalence, location, and diagnostic value among patients with clinically isolated syndrome and other neurologic disorders and age-, sex-, and group-matched healthy controls. Forty-eight patients with clinically isolated syndrome and 30 patients with other neurologic diseases and a healthy control group (n = 47) were included in the study. Subjects were scanned at 3T by using SWI-filtered phase and T2WI, with WM signal abnormalities ≥3 mm being classified. Patients with clinically isolated syndrome had significantly more phase and T2 WM signal abnormalities than healthy controls (P < .001). Phase WM signal abnormalities were more prevalent among patients with clinically isolated syndrome compared with patients with other neurologic disorders (4:1 ratio), whereas T2 WM signal abnormalities were more ubiquitous with a 2:1 ratio. The presence of phase WM signal abnormalities was sensitive for clinically isolated syndrome (70.8%) and achieved a moderate-to-high specificity for differentiating patients with clinically isolated syndrome and healthy controls, patients with other neurologic disorders, and patients with other neurologic disorders of other autoimmune origin (specificity, 70%-76.7%). Combining the presence of ≥2 phase lesions with the McDonald 2005 and 2010 criteria for dissemination in space improved the specificity (90%), but not the accuracy, in differentiating patients with clinically isolated syndrome from those with other neurologic disorders. In subanalyses among patients with clinically isolated syndrome who converted to clinically definite multiple sclerosis versus those who did not within a 3-year follow-up period, converters had significantly more phase (P = .008) but not T2 or T1 WM signal abnormalities. Phase WM signal abnormalities are prevalent among patients with clinically isolated syndrome. The presence of (multiple) phase WM signal abnormalities tended to be more predictive of conversion to clinically definite multiple sclerosis and was specific in differentiating patients with clinically isolated syndrome and other neurologic disorders, compared with T2 WM signal abnormalities; however, the accuracy remains similar to that of the current McDonald criteria.

Neuroimaging in status epilepticus secondary to paraneoplastic autoimmune encephalitis

To describe the characteristic magnetic resonance imaging (MRI) findings of paraneoplastic autoimmune encephalitis in patients with new-onset status epilepticus. The neuroimaging and clinical data of five patients with paraneoplastic autoimmune encephalitis debuting as status epilepticus were retrospectively reviewed. All patients met the criteria for definite paraneoplastic syndrome and all underwent brain MRI during the status epilepticus episode or immediately after recovery. All patients showed hyperintense lesions on T2-weighted imaging (WI) involving the limbic structures, specifically the hippocampus. Three of them showed additional extra-limbic areas of signal abnormalities. The areas of T2 hyperintensity were related to the electroclinical onset of the seizures. In three patients, various techniques were used to study cerebral perfusion, such as arterial spin labelling MRI, single photon-emission computed tomography (SPECT) and 2-[(18)F]-fluoro-2-deoxy-d-glucose (FDG)-positron-emission tomography (PET). Arterial spin labelling showed hyperperfusion overlapping the inflammatory lesions, whereas PET and SPECT disclosed increased perfusion and increased metabolism. The subtraction SPECT co-registered to MRI (SISCOM) demonstrated hypermetabolism outside the areas of encephalitis. After clinical recovery, follow-up MRI revealed the development of atrophy in the initially affected hippocampus. Two patients who had recurrent paraneoplastic autoimmune encephalitis manifesting as status epilepticus showed new T2 lesions involving different structures. The presence of limbic and extra-limbic T2 signal abnormalities in new-onset status epilepticus should suggest the diagnosis of a paraneoplastic syndrome, especially when status epilepticus is refractory to treatment. The lesions are consistently seen as hyperintense on T2WI.

Spinal Cord Injury from Fluoroscopically Guided Intercostal Blocks with Phenol

Background: Image guided intercostal blocks are commonly performed and considered relatively safe. Chemical denervation is commonly used in clinical practice for treatment of chronic noncancer associated pain. Objective: To report a case of spinal cord injury resulting from fluoroscopically guided intercostal blocks with phenol. Study Design: Case report. Setting: Inpatient hospital service. Results/Case Report: A 53 year-old women was transferred from her local facility for acute onset of lower extremity paresis beginning shortly after right intercostal nerve injections of 2 mL of preservative-free phenol at the T7, 8, 9 levels. She had previous intercostal blocks for chronic rightsided mid thoracic/abdominal pain every 3 months for at least one year without sequelae. Within 20 minutes of the injection, she developed a sensation of right leg weakness and heaviness. Over several hours she developed worsening right leg weakness, and then left leg weakness, followed by urinary retention. Admission examination revealed severe right greater than left leg weakness, right lower extremity hyperesthesia to T10, absent lower extremity reflexes, and bilateral extensor plantar responses. Magnetic resonance imaging (MRI) of the entire spine demonstrated extensive T2/DWI hyperintensity in the central spinal cord from T1 to L1 with mild cord enlargement and enhancement at T7-9 (sites of injection). Extensive serum and cerebrospinal fluid (CSF) evaluation did not show any evidence of an infectious, inflammatory, or metabolic cause to her myelopathy. Repeat MRI of the entire spine demonstrated near complete resolution of the T2 signal abnormality. One month after presentation, despite radiographic improvement, the patient showed some clinical improvement, but remained walker dependent and with neurogenic bowel and bladder. Limitations: This report describes a single case report. Conclusion: This case offers several lessons for a pain specialist including 1) the potential for a neurologic catastrophe (spinal cord injury) from aqueous neurolytic intercostal blocks despite “safe” contrast spread; 2) potential mechanisms of neurogenic injury with intercostal blocks; 3) review of modifiable factors to decrease the risk of neurogenic injury; and 4) review of potential interventions (steroids, lumbar drain) to improve outcome in the setting of iatrogenic procedural related spinal cord injury. Key words: Phenol, myelopathy, paraparesis, chemical denervation, neurolysis, intercostal block, epidural spread, chronic pain, complication, transverse myelitis

Feasibility of Combined Intracavitary-Interstitial High-Dose-Rate Brachytherapy With a Novel MR-Compatible Split-Ring Applicator

The purpose of this study is to demonstrate clinical feasibility and preliminary dosimetric evaluation of combined intracavitary-interstitial HDR brachytherapy for cervical cancer, using a novel split-ring applicator and three-dimensional (3D) volume based treatment planning. A 43-year old female with Stage IIB cervical cancer underwent external beam radiation therapy (EBRT) with a 4-field technique to 45 Gy followed by a left-sided parametrial boost to a total dose of 50.4 Gy. The EBRT was combined with high-dose-rate (HDR) brachytherapy to deliver an additional 30 Gy in six fractions. The HDR brachytherapy was delivered using a novel MR compatible split-ring applicator with needle guide-holes in the split-ring caps allowing placement of interstitial needles (Figure, panel A). MRI and CT were used for delineating high risk clinical target volume (HR-CTV) and intermediate risk clinical target volume (IR-CTV) and 3D volume based treatment planning was performed. The patient had persistent T2 signal abnormality on MRI within the left corpus immediately adjacent to the bladder. Due to the unfavorable organ-at-risk (OAR) topography and extent of disease, a single interstitial needle was placed at the site of the MRI T2 weighted signal abnormality and two treatment plans were generated one with intracavitary applicators alone and the other with combined intracavitary applicators and interstitial needle. The DVHs for HR-CTV and IR-CTV and OARs (rectum, bladder, and bowel) were compared between the intracavitary and the combined intracavitary-interstitial applications for a single HDR application. The placement of the split-ring applicator and interstitial needle was well tolerated by the patient and completed under conscious sedation in a dedicated HDR brachytherapy suite. The MR compatible split ring applicator allowed clear delineation of the target volumes, OARs and their spatial relationships with the applicator when using conventional MRI pulse sequences. Using the intracavitary split-ring applicator alone, the D90 to the HR-CTV was 5.7 Gy. When a combined interstitial and intracavitary approach was employed, the D90 of HR-CTV increased to 7.2 Gy, an increase of 26.3%, which allowed the total EQD2 dose of HR-CTV increasing to about 89Gy (ABS guideline is EQD2 80-90Gy). The dose to the IR-CTV improved marginally (1.5%). The dose to the bladder was reduced from 6.3Gy (EQD2= 99 Gy) to 4.6Gy (Eqd2=86Gy) achieving ABS recommended dose constraints of 90 Gy (EQD2). The Figure demonstrates in panel A the MR compatbile split-ring applicator. In panel B is the axial CT slice showing the titanium needle placed in the target volume. Panel C presents the prescribed isodose distribution and panel D depicts the 3D view of the applicators with respect to patient anatomy. This preliminary study demonstrates the dosimetric advantages and clinical feasibility of using a combined intracavitary and interstitial split-ring applicator in patients with significant residual disease after completing external beam radiation therapy. In this study, the addition of a solitary interstitial needle provided substantial reduction of bladder dose and improved the dose coverage to the high-risk target volume. Further clinical investigation in a larger patient cohort is warranted to explore the dosimetric advantages of this approach and evaluate if these advantages translate into improved clinical outcomes.

Hippocampal sclerosis after febrile status epilepticus: The FEBSTAT study

Whether febrile status epilepticus (FSE) produces hippocampal sclerosis (HS) and temporal lobe epilepsy (TLE) has long been debated. Our objective is to determine whether FSE produces acute hippocampal injury that evolves to HS. FEBSTAT and 2 affiliated studies prospectively recruited 226 children aged 1 month to 6 years with FSE and controls with simple febrile seizures. All had acute magnetic resonance imaging (MRI), and follow-up MRI was obtained approximately 1 year later in the majority. Visual interpretation by 2 neuroradiologists informed only of subject age was augmented by hippocampal volumetrics, analysis of the intrahippocampal distribution of T2 signal, and apparent diffusion coefficients. Hippocampal T2 hyperintensity, maximum in Sommer's sector, occurred acutely after FSE in 22 of 226 children in association with increased volume. Follow-up MRI obtained on 14 of the 22 with acute T2 hyperintensity showed HS in 10 and reduced hippocampal volume in 12. In contrast, follow-up of 116 children without acute hyperintensity showed abnormal T2 signal in only 1 (following another episode of FSE). Furthermore, compared to controls with simple febrile seizures, FSE subjects with normal acute MRI had abnormally low right to left hippocampal volume ratios, smaller hippocampi initially, and reduced hippocampal growth. Hippocampal T2 hyperintensity after FSE represents acute injury often evolving to a radiological appearance of HS after 1 year. Furthermore, impaired growth of normal-appearing hippocampi after FSE suggests subtle injury even in the absence of T2 hyperintensity. Longer follow-up is needed to determine the relationship of these findings to TLE.

Myocardial T2 signal enhancement in hypertrophic cardiomyopathy: prevalence, clinical profile and pathologic correlation

Background Abnormal myocardial T2 signal has been described in a minority of patients with hypertrophic cardiomyopathy (HCM). This phenomenon has been associated with arrhythmic outcomes and, given its more restrictive prevalence versus late gadolinium enhancement (LGE), offers a potentially useful biomarker for risk stratification. To date, postulated mechanisms include active tissue disease or unique collagen species, however, no histopathologic correlation has been reported. In this study, we examine the prevalence of T2 signal abnormalities among patients with HCM and examine for associations with other imaging-based markers of HCM phenotypic expression. Incrementally, we provide comprehensive histopathologic correlation of a patient undergoing cardiac transplantation receiving detailed in-vivo and ex-vivo (explanted heart) imaging. Methods Consecutive patients with HCM underwent a standardized imaging protocol using a 3-Tesla MR system. This was inclusive of T2-weighted imaging using a short-Tau inversion-recovery (STIR) fast spin echo sequence in serial short axis imaging planes both with and without body surface coils. Cine and LGE imaging was performed in identical slice orientations. Blinded analysis of left ventricular (LV) wall thickness, LV volumes, T2 signal enhancement and LGE signal abnormalities were performed using commercial software. In a patient planned for transplantation, a detailed in-vivo and ex-vivo imaging protocol was performed, the latter performing high-resolution isotropic T2-weighted imaging. Tissue was sampled from regions with and without abnormal T2 signal and exposed to comprehensive histologic examination. Results

Improved Detection of Cardiac Sarcoidosis Using Magnetic Resonance with Myocardial T2 Mapping

Improved Detection of Cardiac Sarcoidosis Using Magnetic Resonance with Myocardial T2 Mapping

Fulminant cerebellitis with radiological recurrence in an adult patient with Crohn's disease

There are few reports of cerebellitis in adults and reports of recurrent cerebellitis are extremely rare. This report highlights both the fulminant course that may follow cerebellitis and a potentially important association with Crohn's disease. A 41-year-old man presented with headache and cerebellar dysfunction a month after ileo-colonic resection for Crohn's disease. MRI demonstrated T2-signal abnormalities, pial enhancement and cerebellar enlargement. His course was complicated by obstructive hydrocephalus, intraventricular hemorrhage associated with external ventricular drain placement and left transverse sinus venous thrombosis. Treatment with high-dose intravenous steroids and anti-coagulation resulted in improvement. Asymptomatic radiological worsening 13months after initial presentation occurred during a relapse of Crohn's disease. He was treated with intravenous steroids and maintenance azathioprine for his Crohn's and suspected immune mediated cerebellitis. At last follow-up 4years after initial presentation his clinical status remained unchanged and radiological changes had stabilized. Adult onset cerebellitis may be fulminant and recurrent. Early immunotherapy in acute relapsing cerebellitis may speed recovery and prevent recurrence. Onset and radiological relapse of cerebellitis during Crohn's disease flare-ups as in our case possibly suggests a shared autoimmune pathogenesis.

Intramedullary Spinal Cord Metastases: Visibility on PET and Correlation with MRI Features

Studies systematically evaluating the detection of intramedullary spinal cord metastasis with PET are lacking. Our purpose was to evaluate the visibility of intramedullary spinal cord metastasis on PET in a single institutional series and to correlate PET and MR imaging features. Patients were included if pretreatment MR imaging identifying an intramedullary spinal cord metastasis and an [(18)F] FDG-PET examination near the time of MR imaging were available. PET examinations were retrospectively reviewed, with reviewers blinded and then unblinded to the PET report and MR imaging findings. PET intramedullary spinal cord metastasis features were compared with and correlated with previously analyzed MR imaging lesion characteristics. Original clinical PET reports were reviewed. The final study sample was 10 PET examinations in 10 patients with 13 intramedullary spinal cord metastases. In 7 (70%) patients, retrospective blinded review demonstrated convincing evidence of 10 (77%) intramedullary spinal cord metastases. Three MR imaging features correlated with intramedullary spinal cord metastases being visible on PET compared with those nonvisible, respectively: larger lesion enhancement size: mean size: 32.1 mm versus 6.0 mm (P = .038); larger longitudinal extent of T2 signal abnormality: mean 5.6 versus 1.0 segments (P = .0081); and larger ratio of extent of T2 signal abnormality to contrast enhancement: 3.8 versus 1.0 (P = .0069). Intramedullary spinal cord metastasis was confidently reported clinically in 2 (20%) patients, accounting for 5 (38%) intramedullary spinal cord metastases. Most intramedullary spinal cord metastases can be detected on PET when performed near the time of pretreatment MR imaging. However, intramedullary spinal cord metastases may not be clinically reported on PET. Larger lesions with more edema are more likely to be visible. The spinal cord should be specifically and carefully assessed on PET for evidence of intramedullary spinal cord metastases to provide timely diagnosis.

Structural Abnormalities in Patients with Insular/Peri-insular Epilepsy: Spectrum, Frequency, and Pharmacoresistance

Between 2002 and 2010, a total of 48 patients were seen at our epilepsy clinic with insular/peri-insular cortex epilepsy. Review of their MR imaging scans revealed a neoplastic lesion in 27% of patients, a malformation of cortical development in 21%, a vascular malformation in 19%, and atrophy/gliosis from an acquired insult in 17%. MR imaging results were normal in 4 patients. Other miscellaneous findings included a case of Rasmussen encephalitis, a nonspecific insular millimetric T2 signal abnormality, a neuroepithelial cyst, and hippocampal sclerosis without MR imaging evidence of dual insular pathologic features (despite depth electrode-proven insular seizures). Refractoriness to antiepileptic drug treatment was present in 56% of patients: 100% for patients with malformations of cortical development (1.0; 95% CI, 0.72-1.0), 50.0% (0.5; 95% CI, 0.21-0.78) in the presence of atrophy/gliosis from acquired insults, 39% (0.39; 95% CI, 0.14-0.68) for neoplastic lesions, and 22.2% (0.22; 95% CI, 0.06-0.55) for vascular malformations.

Prognostic Value of Clinical and Radiological Signs in the Postoperative Outcome of Spinal Dural Arteriovenous Fistula

Retrospective consecutive case series. To determine predictors of outcome in patients undergoing surgical treatment of spinal dural arteriovenous fistula (SDAVF). Most previous studies assessing postoperative outcome in patients with SDAVF have been limited due to small population size, lack of sufficient information on presurgical variables, or short time of postoperative follow-up. Consequently, the most reliable predictors of functional outcome after treatment of SDAVF are not yet well established. Retrospective analysis of consecutive patients with SDAVF treated surgically between June 1985 and March 2008 in our institution. The Aminoff-Logue gait (G) and micturition (M) scores were used to stratify the degree of disability and the G + M score was used as the primary outcome measure. Demographics, clinical presentation, time to diagnosis, fistula level, presurgical motor and sphincter impairment, and magnetic resonance imaging findings were assessed as prognosticators for postoperative outcomes. One hundred fifty-three patients were analyzed. Mean follow-up was 31 ± 36.2 months. Most patients were improved (44%) or stable (34%) upon the last follow-up. Among preoperative variables, worsening weakness with exertion was associated with a better G + M score at the last follow-up (P < 0.001) and presence of pinprick level was associated with a worse G + M score at the last follow-up (P = 0.020). On multivariable analysis, worsening weakness with exertion was associated with better outcome at the last follow-up, and higher G score at presentation and higher G + M score at discharge were associated with worse outcome at the last follow-up. Magnetic resonance images obtained postoperatively for 104 patients (mean, 19.1 ± 22.5 mo) showed complete resolution or improvement of the presurgical T2 signal abnormalities in 83.6% of cases. Changes in postoperative magnetic resonance image and fistula level did not correlate with functional outcomes. The degree of preoperative disability from SDAFV does not determine who will benefit most from surgery and even patients with severe deficits can improve after treatment. Patients with preoperative exertional claudication and without pinprick level on examination have greater chances of postsurgical improvement. 4.

Teaching Neuro Images : MRI guides genetics

A 12-year-old German girl presented with progressive spastic-ataxic gait and impaired fine motor skills starting from early childhood. MRI revealed striking T2-signal abnormalities in the cerebral and cerebellar white matter, pyramidal tracts and dorsal columns of the spinal cord, as well as lactate elevation in magnetic resonance spectroscopy, indicating leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) (figure). Sequencing of DARS2 1 revealed compound heterozygous frame shift and premature stop mutations (established c.228-11C>G, novel c.617_663del).

Acute EEG findings in children with febrile status epilepticus

The FEBSTAT (Consequences of Prolonged Febrile Seizures) study is prospectively addressing the relationships among serial EEG, MRI, and clinical follow-up in a cohort of children followed from the time of presentation with febrile status epilepticus (FSE). We recruited 199 children with FSE within 72 hours of presentation. Children underwent a detailed history, physical examination, MRI, and EEG within 72 hours. All EEGs were read by 2 teams and then conferenced. Associations with abnormal EEG were determined using logistic regression. Interrater reliability was assessed using the κ statistic. Of the 199 EEGs, 90 (45.2%) were abnormal with the most common abnormality being focal slowing (n = 47) or attenuation (n = 25); these were maximal over the temporal areas in almost all cases. Epileptiform abnormalities were present in 13 EEGs (6.5%). In adjusted analysis, the odds of focal slowing were significantly increased by focal FSE (odds ratio [OR] = 5.08) and hippocampal T2 signal abnormality (OR = 3.50) and significantly decreased with high peak temperature (OR = 0.18). Focal EEG attenuation was also associated with hippocampal T2 signal abnormality (OR = 3.3). Focal EEG slowing or attenuation are present in EEGs obtained within 72 hours of FSE in a substantial proportion of children and are highly associated with MRI evidence of acute hippocampal injury. These findings may be a sensitive and readily obtainable marker of acute injury associated with FSE.

False-Positive FDG PET/CT Due to Liver Parenchymal Injury Caused By a Surgical Retractor

A 70-year-old man underwent partial gastrectomy with pathology demonstrating gastric follicular lymphoma. After surgery, a staging FDG PET/CT study demonstrated an FDG-avid low-attenuation band in the liver. Corresponding MRI demonstrated a high T2 signal abnormality. This was believed to represent liver parenchymal injury due to liver retraction during surgery. The patient was managed conservatively. MRI at 1 month of follow-up demonstrated resolution of the T2 signal abnormality. FDG PET/CT at 6 months of follow-up demonstrated resolution of FDG uptake. Tissue injury from surgical retraction can produce FDG-avid lesions that need to be distinguished from malignancy on PET/CT.

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In Hypertrophic Cardiomyopathy Reduction of Relative Resting Myocardial Blood Flow Is Related to Late Enhancement, T2-Signal and LV Wall Thickness

ObjectivesTo quantify resting myocardial blood flow (MBF) in the left ventricular (LV) wall of HCM patients and to determine the relationship to important parameters of disease: LV wall thickness, late gadolinium enhancement (LGE), T2-signal abnormalities (dark and bright signal), LV outflow tract obstruction and age.Materials and MethodsSeventy patients with proven HCM underwent cardiac MRI. Absolute and relative resting MBF were calculated from cardiac perfusion MRI by using the Fermi function model. The relationship between relative MBF and LV wall thickness, T2-signal abnormalities (T2 dark and T2 bright signal), LGE, age and LV outflow gradient as determined by echocardiography was determined using simple and multiple linear regression analysis. Categories of reduced and elevated perfusion in relation to non- or mildly affected reference segments were defined, and T2-signal characteristics and extent as well as pattern of LGE were examined. Statistical testing included linear and logistic regression analysis, unpaired t-test, odds ratios, and Fisher’s exact test.Results804 segments in 70 patients were included in the analysis. In a simple linear regression model LV wall thickness (p<0.001), extent of LGE (p<0.001), presence of edema, defined as focal T2 bright signal (p<0.001), T2 dark signal (p<0.001) and age (p = 0.032) correlated inversely with relative resting MBF. The LV outflow gradient did not show any effect on resting perfusion (p = 0.901). Multiple linear regression analysis revealed that LGE (p<0.001), edema (p = 0.026) and T2 dark signal (p = 0.019) were independent predictors of relative resting MBF. Segments with reduced resting perfusion demonstrated different LGE patterns compared to segments with elevated resting perfusion.ConclusionIn HCM resting MBF is significantly reduced depending on LV wall thickness, extent of LGE, focal T2 signal abnormalities and age. Furthermore, different patterns of perfusion in HCM patients have been defined, which may represent different stages of disease.

Clinical presentation and prognostic factors of spinal dural arteriovenous fistulas: an overview

Spinal dural arteriovenous fistulas (AVFs), the most common type of spinal cord vascular malformation, can be a challenge to diagnose and treat promptly. The disorder is rare, and the presenting clinical symptoms and signs are nonspecific and insidious at onset. Spinal dural AVFs preferentially affect middle-aged men, and patients most commonly present with gait abnormality or lower-extremity weakness and sensory disturbances. Symptoms gradually progress or decline in a stepwise manner and are commonly associated with pain and sphincter disturbances. Surgical or endovascular disconnection of the fistula has a high success rate with a low rate of morbidity. Motor symptoms are most likely to improve after treatment, followed by sensory disturbances, and lastly sphincter disturbances. Patients with severe neurological deficits at presentation tend to have worse posttreatment functional outcomes than those with mild or moderate pretreatment disability. However, improvement or stabilization of symptoms is seen in the vast majority of treated patients, and thus treatment is justified even in patients with substantial neurological deficits. The extent of intramedullary spinal cord T2 signal abnormality does not correlate with outcomes and should not be used as a prognostic factor.

Posterior Reversible Encephalopathy Syndrome in Cancer Patients (P06.015)

Objective: To explore the clinical manifestations, radiologic features and clinical outcome of posterior reversible encephalopathy syndrome (PRES) in patients with cancer. Background The PRES is a clinical-radiologic entity characterized by headache (HA), altered mental function (AMS), seizures and visual-disturbances in association with imaging findings. Previous literature about the management and outcome of the patients with PRES is limited. Design/Methods: We identified 64 patients with cancer who developed PRES at M.D.Anderson-Cancer-Center between 01/2006 and 06/2011. Clinical and radiographic data were abstracted from their records. Results: The average age at the onset was 52±17.7 years. 44 patients (69%,p=0.003) were women with mean age at onset of 49.6±17.8 years compared to men (mean age at onset 58.8±16 years, p=0.055). Most common cancer diagnoses were leukemia (36%), Lymphoma (9%), gastrointestinal tumors (8%), central-nervous-system tumors (8%). 16 patients (25%) and 35 patients (55%) were treated with tacrolimus and chemotherapy respectively. 22 patients (35%) received bone-marrow transplant (BMT). Clinical presentation included AMS (72%), seizures (69%), HA (33%) and visual-disturbances (19%). PRES was associated with hypertension (HTN) in 57 patients (89%). The brain MRI demonstrated T2 signal abnormalities in all patients and 29 patients (59%) had some evidence of hemorrhage. Seventeen of these 29 patients (59%) were thrombocytopenic. Clinical symptoms fully recovered in 36 patients (57%). Of the 27 (43%) patients with neurological deficits, nine patients (33%) had intracranial hemorrhage and thrombocytopenia, sixteen patients (59%) had a significant delay in diagnostic imaging (11.4±2.5 days versus 1.1±0.3 days, p=0.025) and half of them did not receive a prompt intervention. Conclusions: PRES is an increasingly recognized occurrence in cancer patients undergoing therapy. HTN seems to be the most important co-existent risk factor for development of PRES. Delay in diagnosis can result in permanent residual deficits. Underlying thrombocytopenia in cancer patients with PRES is associated with hemorrhagic complications and increases the risk of incomplete recovery. Disclosure: Dr. Paker has nothing to disclose. Dr. Kamiya Matsuoka has nothing to disclose. Dr. Chi has nothing to disclose. Dr. Loghin has nothing to disclose.

Acute Brain MRI Findings in 120 Malawian Children with Cerebral Malaria: New Insights into an Ancient Disease

There have been few neuroimaging studies of pediatric CM, a common often fatal tropical condition. We undertook a prospective study of pediatric CM to better characterize the MRI features of this syndrome, comparing findings in children meeting a stringent definition of CM with those in a control group who were infected with malaria but who were likely to have a nonmalarial cause of coma. Consecutive children admitted with traditionally defined CM (parasitemia, coma, and no other coma etiology evident) were eligible for this study. The presence or absence of malaria retinopathy was determined. MRI findings in children with ret+ CM (patients) were compared with those with ret- CM (controls). Two radiologists blinded to retinopathy status jointly developed a scoring procedure for image interpretation and provided independent reviews. MRI findings were compared between patients with and without retinopathy, to assess the specificity of changes for patients with very strictly defined CM. Of 152 children with clinically defined CM, 120 were ret+, and 32 were ret-. Abnormalities much more common in the patients with ret+ CM were markedly increased brain volume; abnormal T2 signal intensity; and DWI abnormalities in the cortical, deep gray, and white matter structures. Focal abnormalities rarely respected arterial vascular distributions. Most of the findings in the more clinically heterogeneous ret- group were normal, and none of the abnormalities noted were more prevalent in controls. Distinctive MRI findings present in patients meeting a stringent definition of CM may offer insights into disease pathogenesis and treatment.