Follow-up Brain MRI Research Articles

Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up

PurposeTo understand the long-term neurological outcomes and chronological changes of brain MRIs in patients with febrile infection-related epilepsy syndrome (FIRES). MethodsFrom December 2000 to May 2016, 29 patients diagnosed with FIRES were collected retrospectively. The demographic distribution, clinical manifestations, neuroimaging findings, and treatment methodology were described. Follow-up clinical outcomes and chronological evolution of neuroimaging findings were analyzed. ResultsThe median age of disease onset was 8.9 years. The median duration of hospitalization was 87 days. During the period of hospitalization, more than 50% of patients exhibited complications such as skin rash, liver function impairment, and arrhythmia. Abnormal findings were found in 38% of neuroimaging studies in the initial study and in 87% of the follow-up brain MRI. Focal abnormal signal changes over the periventricular white matter suggested the more extensive lesions would be associated with a poorer clinical outcome. The median duration of follow-up was 5 years in 23 patients. In total, 87% of patients exhibited residual and/or refractory epilepsy. Regarding cognitive function, 26% of patients had normal intelligence quotient, 26% had learning disability and mild to moderate mental retardation, and 48% had severe mental retardation or vegetative status. The mortality rate at acute stage was 10%, and that at chronic stage was 13%. ConclusionsLiver function impairment, skin rash, and arrhythmia are frequently seen during hospitalization for FIRES at the acute stage of disease course. The higher grade of periventricular white matter lesions suggested poorer neurological outcomes. Studies on the pathomechanism of FIRES are crucially needed so that new treatment strategies for FIRES can be developed, which may improve long-term outcomes.

Autoimmune hypophysitis

A 82-year-old male patient with metastatic lung adenocarcinoma presented to the outpatient clinic at the University of Texas MD Anderson Cancer Center (Houston, TX, USA) in April, 2015 with worsening fatigue, headache, dizziness, blurry vision, and nausea. He had received treatment with an immune checkpoint inhibitor, ipilimumab (an anti CTLA-4 antibody) 4 weeks before presentation. A brain MRI showed a diffuse pituitary enlargement in the T1-weighted coronal section (figure, A) and sagittal section (figure, B), with increased thickening of the pituitary stalk and enhancement of adjacent dura, suggestive of hypophysitis. The endocrine workup was consistent with hypophysitis with a central adrenal insufficiency, central hypothyroidism and hypogonadism, but with intact posterior pituitary function. The patient's symptoms improved significantly with initial high-dose corticosteroids (prednisone 60 mg daily) and supplementation of the affected hormones, and the follow-up brain MRI after 1 month showed a normal-sized pituitary gland.

Norethisterone enanthate-induced cerebral venous sinus thrombosis (CVST)

A 23-year-old East Indian woman with no significant medical history, except a depot-norethisterone enanthate injection taken 3 weeks prior to admission, presented with a gradually worsening headache for the past...

Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI.

Patients with acute disseminated encephalomyelitis (ADEM) are presumed to have radiological monophasic disease, but this is uncertain since follow-up brain MRI is not routinely performed. We aimed to ascertain combined radiological and clinical monophasic disease in ADEM patients and to assess whether performing early (<6months) follow-up brain MRI has prognostic value for subsequent multiphasic disease. We retrospectively studied the medical records of patients initially diagnosed with ADEM (years 2000-2014) at the Massachusetts General Hospital, USA. A neuroimaging specialist, masked to clinical events, reviewed all available brain MRIs. We included 62 patients (25 male; 30 pediatric; median clinical follow-up 3years) and classified them into two subgroups: (1) clinically monophasic (no new, recurrent or worsening neurological symptoms >3months after onset) (n=45), and (2) clinically multiphasic (clinical relapse >3months after onset) (n=17). All clinically monophasic patients with brain MRI follow-up (n=30) also had radiological monophasic disease a median of 2years after ADEM onset. New lesions (58 vs. 14%) and persistent lesions (100 vs. 18%) on early brain MRI [available in 40 patients (65%)], as well as clinical flares (53 vs. 20%), were more common in clinically multiphasic versus monophasic patients. These early follow-up data allowed us to predict multiphasic disease with reasonable accuracy in a multivariable model (AUC=0.73). We conclude that performing early follow-up brain MRI routinely in ADEM patients would aid clinicians in predicting multiphasic disease and may stratify patients who would benefit from initiation of disease-modifying therapy for multiple sclerosis.

White Matter Microstructure and Subcortical Gray Matter Structure Volumes in Aspartylglucosaminuria; a 5-Year Follow-up Brain MRI Study of an Adolescent with Aspartylglucosaminuria and His Healthy Twin Brother.

Aspartylglucosaminuria is an inherited, lysosomal storage disease causing progressive decline in cognitive and motor functions. The aim of this study was to evaluate volumes of subcortical gray matter structures and white matter microstructure in aspartylglucosaminuria in adolescence in a longitudinal study for the first time. A boy with aspartylglucosaminuria and his healthy twin brother were imaged twice with a 3.0T MRI scanner at the ages of 10 and 15years. Subcortical gray matter structure volumes were measured using an atlas-based automatic method, and diffusion tensor imaging was used to evaluate the white matter microstructure of the corpus callosum and the thalamocortical pulvinar tracts. The subcortical gray matter structures were smaller at onset and diminished at follow-up in the affected twin, with the exception of the amygdala which was larger and remained the size. The largest difference in volume between the twins was found in the thalami. The total gray and white matter volumes decreased in the affected twin. In diffusion tensor imaging analysis, the fractional anisotropy was decreased at onset in the affected twin compared to the healthy brother in the evaluated tracts. The axial, radial and mean diffusivity values were increased in the affected twin. The difference between the twins increased slightly at follow-up. The findings suggest that volumetric measurements and diffusion tensor imaging based microstructural analysis may be useful modalities for monitoring disease progression and response to emerging treatment in aspartylglucosaminuria, but further studies with more subjects are necessary to confirm the results.

P3.04-007 Detection of Brain Metastasis in Resected Lung Cancer with the New Postoperative Follow-Up Program: Topic: Miscellaneous I

In our previous study, follow-up brain MRI for the detection of early brain metastasis was examined every 2 months during the first 6 months after operation until 1995. In the study, asymptomatic brain metastases were found at the frequency of 2.3%. After that, the follow-up program was changed as follows; brain MRI performed on a postoperative patient at two points of 2-3 months (early check time) and 5-6 months (late check time) after operation. We reviewed the detection rate of brain metastasis in the new program. Between January 1996 and December 2009, 954 patients with primary lung cancer underwent complete surgical resection. Of 954 cases, 712 received brain MRI in accordance with the new follow-up program. The frequency, the point of brain metastases detection, treatment for brain metastases, and prognosis were reviewed. Of total 712 cases, 24 (3.4%) patients with brain metastases were detected as initial recurrence lesion with follow-up MRI. Seven of these 24 cases were detected at the point of early check time (early group). The remaining 17 cases were detected at the point of late check time (late group). In the early group, 3 patients had a single metastasis and 1 had three lesions. The remaining 3 had more than four lesions. On the other hand, 10 of 17 late group had a single metastasis and 5 had two or three lesions, and the remaining 2 had more than four lesions. In early group, the pathological stagings were 2 stage 1, 3 stage 2, 2 stage 3. All cases of solitary metastasis and 1case of three metastatic lesions were treated with stereotactic radiosurgery (SRS). Two cases with more than four lesions were treated with whole brain radiotherapy (WBRT). In late group, the pathological stagings were 5 stage 1, 5 stage 2, 7 stage 3. All but 2 cases were treated with SRS. The overall median survival time from thoracic surgery was 17 months in early group and 20 months in late group. Two cases from the late group were recurrence free for 104 and 81 months. In early group, they frequently had multiple brain metastases and were treated with WBRT. On the other hand, in late group, a single metastasis was discovered in many patients and was treated with SRS. Among them, some patients had long recurrence-free survival. From these results, we changed postoperative follow-up program for detection of the brain metastasis to check at only one point of 5-6 months after operation.

P14.08 Neurological complications of emerging targeted therapies

AbstractIntroduction:The development of new biological and immunotherapeutic agents to treat cancer associates unknown neurological toxicities. Crizotinib (Cri), an anaplastic lymphoma kinase and c-ros oncogene 1 hepatocyte growth factor receptor tyrosine-kinase inhibitor, has been approved for the treatment of advanced ALK-positive non-small cell lung cancer. Ipilimumab (Ipi), an anticytotoxic T-lymphocyte antigen-4 antibody, was the first agent to demonstrate improved survival in patients with metastatic melanoma. This report describes a patient treated with Ipi showing a not previously reported neurological adverse event; and a patient treated with Cri showing atypical brain metastases.Patients and methods:Patient 1(P1): 66-year-old male with a history of irresectable nasal melanoma, started on Ipi plus concomitant radiotherapy on September/2015, obtaining a radiological partial response. Two months later he developed progressive cognitive impairment, urinary incontinence and gait difficulties. A neuroaxis MRI revealed multiple acute and subacute subcentimeter ischemic lesions with contrast enhancement and restricted diffusion with diffuse FLAIR signal changes. CSF reported protein elevation, normal glucose and mild lymphocytic pleocytosis without neoplastic cells. Microbiological cultures and PCRs were negative. Echocardiography was normal. Patient 2(P2): 54-year-old non-smoker female with lung adenocarcinoma (EGFR wild-type and ALK translocation) and spinal metastatic disease at diagnosis, started on Cri after failure of first-line treatment with cisplatin plus pemetrexed. After 16 cycles, an increase in carcinoembryonic antigen was noted without progression of ganglio-pulmonary disease. Although asymptomatic, CNS progression was suspected and follow-up brain MRI reported multiple bilateral supra and infratentorial large cystic lesions with perilesional edema and no contrast enhancement.Results:P1: primary angiitis of the CNS was suspected, although arteriography was unremarkable. He received high-dose metilprednisolone followed by cyclophosphamide bolus. The inflammatory component of CSF and neurological symptoms presented partial improvement but brain MRI showed a dissociate response. P2: PET scan revealed irregular distribution of the radiotracer and hypometabolic lesions. Follow-up brain MRI revealed stabilization with a perfusion-weighted sequence reporting an increase in the relative cerebral blood volume of the bigger cystic lesion. These features led us to the diagnostic of indolent brain metastases.Conclusions:Early recognition of new neurological patterns of toxicity may lead to drug discontinuation or dose adjustment preventing further neurological deterioration. A better knowledge of these toxicities will help differentiate treatment-related complications from metastatic progression.

Subcortical grey matter volumes predict subsequent walking function in early multiple sclerosis

BackgroundAtrophy of subcortical grey matter structures has been reported to be associated with clinical measures of disability in multiple sclerosis (MS) patients. It is not clear if the degree of tissue loss in patients with very early MS is associated with changes in disability measures. ObjectiveTo study the association between subcortical grey matter structure volumes and clinical disability outcomes. MethodsRelapsing MS patients within 12months of clinical onset were enrolled in a neuroprotection trial of riluzole versus placebo with up to 36months of follow-up and serial brain MRI and clinical assessments. MRI metrics, including thalamic, putamen, caudate, pallidum and cerebellar cortical volume, were measured by an automated, custom-made FreeSurfer pipeline. Volumes were normalized for head size. Clinical measures included EDSS, MSFC scores and its components. Mixed model regression measured time trends and associations between imaging and clinical outcomes. Results42 patients with a mean follow-up of 30.6months were analyzed in this study. There was a statistically significant decrease in thalamus, caudate and putamen volumes, but not cerebellar cortical and pallidum volumes during the follow-up period. Baseline thalamus, caudate and putamen volumes predicted subsequent changes in the timed 25-ft walk test (p=0.036) and MSFC (p=0.024). There was a trend for an association between baseline caudate volume and subsequent change in the timed 25-ft walk test (p=0.084). No association between baseline imaging and subsequent EDSS changes were seen. ConclusionSubcortical grey matter volumes at early stages of MS are associated with subsequent changes in disability measures.

Association of arterial stiffness with progression of subclinical brain and cognitive disease.

We tested whether abnormal arterial stiffness and blood pressure would be associated with progression of brain aging measured by brain MRI and neurocognitive testing. Framingham Offspring Cohort participants (n = 1,223, 61 ± 9 years, 56% women) without previous stroke or dementia underwent applanation tonometry, brain MRI, and neurocognitive testing at examination 7 (1998-2001). Follow-up brain MRI and neurocognitive testing was performed at examination 8 (2005-2008, mean interval 6.4 ± 1.3 years). We related examination 7 inverse-transformed carotid-femoral pulse wave velocity (iCFPWV), central pulse pressure (CPP), and mean arterial pressure to changes in the following variables between examinations 7 and 8: total cerebral brain volume, white matter hyperintensity volume, and performance on executive function and abstraction tasks, the Trail Making Test, Parts B and A (ΔTrails B-A), and Similarities tests. Higher baseline iCFPWV and CPP were associated with greater progression of neurocognitive decline (iCFPWV and ΔTrails B-A association: SD unit change in outcome variable per SD change in tonometry variable [β] ± SE = 0.10 ± 0.04, p = 0.019; CPP and ΔSimilarities association: -0.08 ± 0.03, p = 0.013). Higher mean arterial pressure, but not iCFPWV or CPP, was associated with increase in white matter hyperintensity volume ([β ± SE] 0.07 ± 0.03, p = 0.017). No tonometry measures were associated with change in cerebral brain volume. In middle-aged and older adults without evidence of clinical stroke or dementia, elevated arterial stiffness and pressure pulsatility are associated with longitudinal progression of subclinical vascular brain injury and greater neurocognitive decline. Treatments to reduce arterial stiffness may potentially reduce the progression of neurovascular disease and cognitive decline.

Association between Perivascular Spaces and Progression of White Matter Hyperintensities in Lacunar Stroke Patients.

ObjectivesPerivascular spaces are associated with MRI markers of cerebral small vessel disease, including white matter hyperintensities. Although perivascular spaces are considered to be an early MRI marker of cerebral small vessel disease, it is unknown whether they are associated with further progression of MRI markers, especially white matter hyperintensities. We determined the association between perivascular spaces and progression of white matter hyperintensities after 2-year follow-up in lacunar stroke patients.MethodsIn 118 lacunar stroke patients we obtained brain MRI and 24-hour ambulatory blood pressure measurements at baseline, and a follow-up brain MRI 2 years later. We visually graded perivascular spaces and white matter hyperintensities at baseline. Progression of white matter hyperintensities was assessed using a visual white matter hyperintensity change scale. Associations with white matter hyperintensity progression were tested with binary logistic regression analysis.ResultsExtensive basal ganglia perivascular spaces were associated with progression of white matter hyperintensities (OR 4.29; 95% CI: 1.28–14.32; p<0.05), after adjustment for age, gender, 24-hour blood pressure and vascular risk factors. This association lost significance after additional adjustment for baseline white matter hyperintensities. Centrum semiovale perivascular spaces were not associated with progression of white matter hyperintensities.ConclusionsOur study shows that extensive basal ganglia perivascular spaces are associated with progression of white matter hyperintensities in cerebral small vessel disease. However, this association was not independent of baseline white matter hyperintensities. Therefore, presence of white matter hyperintensities at baseline remains an important determinant of further progression of white matter hyperintensities in cerebral small vessel disease.

Solid Cerebellar Hemangioblastoma with Peritumoral Edema: 5-Years Follow up

【Hemangioblastomas are angioblastic tumors of the central nervous system. Cerebellar hemangioblastomas are traditionally classified into two morphologic types-cystic and solid. Cystic hemangioblastomas are associated with peritumoral edema, but solid hemangioblastomas are not. We report a case of solid cerebellar hemangioblastoma with massive peritumoral edema. An 83-year-old female visited our hospital due to a sudden headache. Five years ago, she had been admitted to our hospital with similar headache and diagnosed with cerebellar hemangioblastoma. Follow-up brain MRI 5 years later showed an increased size of a homogeneous enhancing mass with aggravated peritumoral edema in the left lower cerebellar hemisphere. Cerebral angiography showed a highly vascularized mass in the cerebellum, which was compatible with a solid-type hemangioblastoma.】

Outcome of gamma knife thalamotomy in patients with an intractable tremor.

ObjectiveTremor is a common movement disorder that interferes with daily living. Since the medication for tremor has some limitations, surgical intervention is needed in many patients. In certain patients who cannot undergo aggressive surgical intervention, Gamma Knife thalamotomy (GKT) is a safe and effective alternative.MethodsFrom June 2012 to August 2013, 7 patients with an intractable tremor underwent GKT. Four of these 7 patients had medical comorbidities, and 3 patients refused to undergo traditional surgery. Each patient was evaluated with the modified Fahn-Tolosa-Marin tremor rating scale (TRS) along with analysis of handwriting samples. All of the patients underwent GKT with a maximal dose of 130 Gy to the left ventralis intermedius (VIM) nucleus of the thalamus. Follow-up brain MRI was performed after 3 to 8 months of GKT, and evaluation with the TRS was also performed.ResultsSix patients showed objective improvement in the TRS score. Excluding one patient who demonstrated tremor progression, there was 28.9% improvement in the TRS score. However, five patients showed subjective improvement in their symptoms. On comparing the TRS scores between follow-up periods of more and less than 4 months, the follow-up TRS score at more than 4 months of GKT was significantly improved compared to that at less than 4 months of GKT. Follow-up MRI showed radiosurgical changes in 5 patients.ConclusionGKT with a maximal dose of 130 Gy to the VIM is a safe procedure that can replace other surgical procedures.

MS-17 * PRIMARY LEPTOMENINGEAL PRIMITIVE NEUROECTODERMAL TUMOR

BACKGROUND: Isolated primary leptomeningeal primitive neuroectodermal tumor (PNET) without intraparenchymal lesion is very rare and to-date few case reports have been published. We report a rare case of leptomeningeal PNET and highlight its neuroimaging features, outcome and literature review. DESIGN AND METHODS: A medline search was conducted for queries including “Children”, “PNET”, leptomeningeal involvement”. Relevant papers were selected for review. CASE REPORT: A 5-year-old female presented with 4-month history of continuous vomiting and repeated headache. CT scan demonstrated communicating hydrocephalus. External ventricular drain was place to relieve the intracranial pressure. An MRI of the brain and the spine showed diffuse leptomeningeal infiltration of the brain and the spinal cord with contrast enhancement. Lumbar cerebrospinal fluid analysis revealed elevated protein but no malignant cells. Pathology of the leptomeningeal biopsywas consistent with neuroepithelial neoplasm. Immunohistochemical stains revealed tumor cells strongly positive for synaptophysin and CD56, retained INI-1, but negative for CD99. Cytogenetical analysis revealed absence of EWS translocation and a diagnosis of primary leptomeningeal PNET was established. Patient received radiation therapy with 36 Gy to the craniospinal axis, concomitantly with vincristine and carboplatin . Follow-up brain and spine MRI showed near complete resolution of the leptomeningeal disease. Family opted not to pursue any therapy and she remains alive with residual disease 8 months since diagnosis. DISCUSSION: Leptomeningeal PNET carries poor prognosis with most patients succumbing to their disease. There are seven case reports in the literature with dismal outcome with survival range from 2 weeks to thirteen months post diagnosis .Rarity of this clinical entity had hindered profiling therapeutic guidelines. Prospective studies are needed to understand if the elusive molecular biology of this rare disease differs from other central PNET. This could help in strategizing optimal therapy and provide a meaningful improvement in the survival of these children.

Use of coumarin anticoagulants and cerebral microbleeds in the general population.

It remains undetermined whether the use of coumarin anticoagulants associates with cerebral microbleeds in the general population. We investigated whether (1) coumarin use relates to higher prevalence and incidence of microbleeds, (2) microbleeds are more frequent in people with higher maximum international normalized ratios (INRs), and (3) among coumarin users, variability in INR associates with microbleed presence. From the population-based Rotterdam Study, 4945 participants aged ≥45 years were included in the cross-sectional analysis, and 3069 participants had follow-up brain MRI. Information on coumarin use was obtained from automated pharmacy records. Coumarin users were monitored, and INR values were measured in consecutive visits. Presence and location of microbleeds were rated on brain MRI. We investigated the association of coumarin use with microbleeds using multivariable logistic regression. Overall, 8.6% had used coumarin anticoagulants before the first MRI and 5.9% before follow-up MRI. The prevalence of microbleeds was 19.4%, and the incidence was 6.9% during a mean follow-up of 3.9 years (SD, 0.5). Compared with never users, coumarin users had a higher prevalence of deep or infratentorial microbleeds and a higher incidence of any microbleeds, although statistical significance was not reached in the latter. A higher maximum INR was associated with deep or infratentorial microbleeds. Among coumarin users, a greater variability in INR was associated with a higher prevalence of microbleeds. Coumarin use is associated with microbleeds. Associations were strongest for people with greater variability in INR.

Acute unilateral cerebellitis, Epstein-Barr virus, and HIV

A 31-year-old man presented to the emergency depart ment with a 2-day history of severe headache, nausea, and vomiting. He reported intermittent tremors in his left hand and ataxic gait for 2 weeks. Neuro logical examinations showed dysarthria and left-sided dys metria on fi nger-tonose and heel-knee-shin tests. Brain MRI showed a poorly defi ned hetero geneous hyper intensive lesion of the left cerebellar hemisphere (fi gure, A [axial T2-weighted] and B [axial T1-weighted]) and obstructive hydrocephalus caused by a cerebellar mass-eff ect (fi gure, C [sagittal T1-weighted]). Serology investi gations for bacteria, fungi, and viruses indicated un remark able results; tests for HIV and EpsteinBarr virus (EBV) were positive. HIV viral load was 2554 copies per mL and CD4 count was 235 cells per μL. CSF analysis showed pleocytosis and increased protein concentration; bacterial and fungal cultures and examinations for syphilis, cryptococcus, toxo plasmosis, and acid-fast bacilli were negative. EBV PCR of CSF was positive. Stereotactic biopsy of the cerebellum was done, and histopathological examination indicated infl ammatory infi ltration with abundant lymphocytes and histiocytes without evidence of malignant disease. Furthermore, EBVencoded RNA was expressed in infi ltrating cells. Highly active antiretroviral therapy (tenofovir, lamivudine, efavirenz), antiviral therapy (ganciclovir), and steroids (beta methasone) were prescribed for EBV-related acute cerebellitis. Follow-up brain MRI showed regression of the cerebellar infl ammatory lesion (fi gure D–F), indicating a substantial improvement in the clinical condition. He recovered well without substantial neurological sequelae. CNS infection in patients with HIV can be associated with various pathogens including viruses, toxoplasmosis, syphilis, and cryptococcus. The clinical manifestations, specifi c area involved, and immune status of patients can assist with the diff erential diagnosis. In our patient, cerebellitis was a characteristic sign distinguishable from other causes. The possibility of an EBV infection should be considered in the diff erential diagnosis of CNS lesion in patients with HIV, and EBV-related malignant diseases should be diff erentiated, to ensure appropriate treatment.

Neuronavigation-guided biopsy for differential diagnosis of pseudotumoral demyelinating brain lesions

Abstract Marburg's disease (MD) is an extremely rare and aggressive form of multiple sclerosis (MS). In some cases, MD presents with tumefactive demyelinating lesions with a “tumor-like” appearance in MRI images, for which it may be difficult to achieve a form of differential diagnosis between definitive tumors or abscesses. Here we report a case of MD histopathologically confirmed after neuronavigationguided biopsy. Postoperative course was uneventful and following discharge, the patient attended outpatient follow-up appointments and received i.v. cyclophopsphamide, achieving progressive clinical remission. A nine-month follow-up brain MRI scan with gadolinium showed no signs of progressing demyelinating disease with an evident reduction of the biopsied lesion, and almost complete retrogression of the other two lesions. In our opinion, and through the analysis of currently available literature, early neuronavigation-guided biopsy is a highly recommend, valuable, and safe diagnostic tool; it is also preferable to stereotactic biopsy, since its benefits include a very low bleeding rate and brain damage risk, with minimum mortality and morbidity rates. It also allows the identification of the specific histological pattern, helping to select the best medical treatment approach and contributing to increase patient outcome and life expectancy.

Natural course of LGI1 encephalitis: 3–5 years of follow-up without immunotherapy

Antibodies against LGI1 (leucin-rich glioma-inactivated 1 protein) are associated with limbic encephalitis (LE), which is characterized by a favorable outcome following immunotherapy.Here, we present two cases, where antibodies against LGI1 were detected in the sera 36 and 53months after acute LE, respectively, and none of the patients received immunotherapy. LE showed characteristics of LGI1 encephalitis in both cases, including low sodium content in the sera; disorientation, hallucination, short-term memory loss; and epileptic seizures. One patient had faciobrachial tonic seizures. MRI indicated bilateral inflammation of the hippocampus in one case.We reviewed longitudinal clinical and MRI data covering 53 and 36months after LE without immunotherapy, respectively. Both patients became seizure-free and spontaneously recovered with mild/moderate cognitive impairment. No relapses have been observed. Follow-up brain MRI indicated early hippocampal sclerosis and global brain atrophy in one case characterized by more pronounced cognitive deficit. Memory and verbal fluency were affected most during the natural course of LGI1 encephalitis.LGI1 encephalitis had a monophasic course and spontaneously improved, suggesting that a relatively benign natural course may contribute to the favorable outcome observed after immunotherapy. Our data also indicate that LGI1 antibodies can be present in the sera without clinical disease activity.

A pathophysiologic approach for subacute encephalopathy with seizures in alcoholics (SESA) syndrome

Subacute encephalopathy with seizures in alcoholics (SESA) syndrome is a unique disease entity characterized by typical clinical and electroencephalographic (EEG) features in the setting of chronic alcoholism. We present two patients with distinctive serial MRI and EEG findings which suggest a clue to the underlying pathophysiologic mechanisms of SESA syndrome. Two patients with chronic alcoholism and alcoholic liver cirrhosis presented with generalized seizures and confused mental status. Brain MRI demonstrated restricted diffusion, increased T2-weighted signal intensity, and hyperperfusion in the presumed seizure focus and nearby posterior regions of the cerebral hemispheres. EEG showed periodic lateralized epileptiform discharges which were prominent in the posterior regions of the cerebral hemispheres ipsilateral to the side of brain MRI abnormalities. Even after patients clinically improved, these brain abnormalities persisted with progressive atrophic changes on follow-up brain MRI. These patients had not only the distinguishing clinical and EEG features of SESA syndrome, but also showed novel brain MRI abnormalities. These changes on MRI displayed characteristics of seizure-related changes. The posterior dominance of abnormalities on MRI and EEG suggests that the pathophysiologic mechanisms of SESA syndrome may share those of posterior reversible encephalopathy syndrome.

Vitamin B12 and Progression of White Matter Lesions. A 2-Year Follow-Up Study in First-Ever Lacunar Stroke Patients

In cross-sectional studies periventricular white matter lesions (WML) were related to low plasma levels of vitamin B12. Whether low vitamin B12 levels are also related to progression of WML is still unknown. We studied baseline vitamin B12 levels and its association with progression of WML over 2 years of follow-up in first-ever lacunar stroke patients. In 107 first-ever lacunar stroke patients in whom baseline brain MRI and vitamin B12 status were available, we obtained a follow-up brain MRI after 2 years. We assessed progression of periventricular WML (pWML) and deep WML (dWML) using a visual WML change scale. We studied the relationship between baseline levels of plasma vitamin B12 and progression of WML after 2 years of follow-up by binary logistic regression analyses. Vitamin B12 deficiency was more frequent in patients with progression of pWML compared to those without progression (41.9% and 19.7% respectively, p = 0.02). Corrected for sex and age, progression of pWML was associated with lower baseline levels of vitamin B12 (OR 1.42 per 50 unit decrease, 95% CI 1.00-1.92). Vitamin B12 levels were not associated with progression of dWML. In conclusion progression of pWML after 2 years of follow-up relates to low levels of vitamin B12 at baseline in first-ever lacunar stroke patients. Whether this population could benefit from vitamin B12 supplementation is unknown and requires further investigation.

Posterior reversible encephalopathy syndrome in a patient with systemic lupus erythematosus after cessation of oral prednisone

Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder that is first described by Hinchey and colleagues in 1996 [1]. It is associated with various clinical features such as hypertension, seizures, headache, visual changes and conscious disturbance [2]. In previous reports, PRES have been reported in patients with SLE. The use of immunosuppressive drugs was an established cause of PRES in SLE [3]. Here we describe a case of PRES due to cessation of oral prednisone. To the best of our knowledge, this is the first report of PRES due to cessation of oral prednisone. A 34-year-old man presented to our department with headache and blurred vision for 1 day. He had a history of SLE and lupus nephritis for 6 years. Three months before admission, the patient presented to the department of rheumatology with a flare of SLE including malar rash, arthritis, and pancytopenia. The patient was free of ophthalmologic and neurologic symptoms then. He was hospitalized and received intravenous pulse therapy of methylprednisone at 250 mg/day given intravenously for 3 days. The patient was discharged symptom free and was taking a maintenance dose of oral prednisone at 60 mg/day. A week before admission, the patient had suddenly stopped taking oral prednisone. He gradually developed bilateral headache and blurred vision 6 days after cessation of oral prednisone. His headaches were described as constant, bilateral temporoparietal and were associated with nausea and vomiting. The patient was only able to distinguish between light and dark. The visual acuity was severely impaired and he was unable to count fingers before his eyes. On examination, the pupils were round, equal and accommodate to light. Optical fundus examination was unremarkable. The anti-nuclear antibodies and anti-dsDNA antibodies were positive. Laboratory examinations revealed urea nitrogen 13.7 mmol/l, serum creatinine 197 lmol/l, uric acid 885 lmol/l, C3 of 0.46 g/l (reference range 0.79–1.52 g/l), C4 of 0.16 (reference range 0.16–0.38 g/l). The admission head MRI was performed and fluid attenuated inversion recovery (FLAIR) sequence showed bilateral hyperintensities in the frontal, parietal, temporal, occipital lobes and the cerebellum, which is consistent with vasogenic edema (Fig. 1a). The patient continued oral prednisone at a dose of 50 mg on a daily basis after admission. The patient was free of symptoms 1 week after restoration of the oral prednisone treatment. Follow-up brain MRI was performed 6 days after admission and the vasogenic edema on FLAIR images was obviously resolved (Fig. 1b). The patient was discharged with complete clinical resolution. Posterior reversible encephalopathy syndrome is a clinical and radiological entity that may present with a constellation of symptoms including headache, seizures, visual disturbances and altered mental status. Although the precise mechanism remains incompletely understood, blood–brainbarrier leakage and endothelial dysfunction seem to play key role in the development of PRES. Radiological examination is important to rule out alternative causes and aid Q. Li Y. Wei P. Xie (&) Department of Neurology, The First Affiliated Hospital, Chongqing Medical University, Chongqing, China e-mail: peng_xie@yahoo.com