Posterior reversible encephalopathy syndrome as a revealing manifestation of Guillain-Barré syndrome

We report a patient with a Guillain-Barré syndrome (GBS) revealed by a posterior reversible encephalopathy syndrome (PRES). The PRES is typically associated with bilateral parieto-occipital T2 and FLAIR hyperintense MRI lesions and observed in various etiologic conditions leading to acute arterial hypertension. PRES results from a breakdown of the circulatory autoregulation, many in the posterior cerebral territories. GBS can be considered as an independent risk factor of PRES, due to acute dysautonomia and pain with consecutive arterial hypertension, as well as to cytokine production changing capillary permability. Such patients with PRES-revealed GBS may be treated with intravenous immunoglobulin therapy only after exclusion of any ischemic or hemorrhagic cerebral complications, and after control of the blood pressure and of the encephalopathic signs and symptoms.     

Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction]

A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.     

Atypical clinical course in two patients with GNB1 variants who developed acute encephalopathy

IntroductionVariants in the GNB1 gene, which encodes the β1 subunit of a trimeric G protein, can cause moderate to severe psychomotor retardation. Acute encephalopathies have also been observed in patients with central nervous system abnormalities; however, severe neurological sequelae have not previously been reported.Case presentationsPatient 1 was a Japanese female with a de novo GNB1 variant (c.284 T > C). At 8 months old she contracted influenza A and developed generalized convulsions. In the acute phase, brain magnetic resonance imaging (MRI) findings indicated acute encephalopathy; diffuse cerebral atrophy was present 1 month later. Although multidisciplinary treatment was administered, she had severe neurological sequelae including spastic tetraplegia, severe intellectual disabilities, and refractory epilepsy. Patient 2 was a Japanese male with a de novo GNB1 variant (c.239 T > C). He experienced an unexplained respiratory arrest aged 17 years; refractory convulsions developed. Brain MRI at 1 month showed bilateral basal ganglia high intensities; at 3 months, diffuse cerebral cortex and white matter atrophy was observed. Despite multidisciplinary treatment, he developed severe spastic tetraplegia and mental regression.DiscussionWe report two patients with GNB1 variants who had acute lesions on brain MRI and unexpected disease courses. In such patients with acute neurological deterioration, multidisciplinary treatment is required; patients should also be carefully observed for progression to acute encephalopathy.     

Cerebral white matter lacerations in children caused by repetitive head trauma

It has been known that infants less than 1 year develop cerebral white matter (WM) lacerations associated with head trauma, however, there has been no report of similar WM lesions over 1 year. We report three teenage boys (11, 12, and 18 years at final MRI studies) with acquired WM lacerations associated with recurrent head trauma who developed neurologic symptoms such as spastic paralysis, afebrile convulsions, and cognitive impairment. Two of them (patients 1 and 2) were given a diagnosis of autism spectrum disorder and had a history of repeated severe self-inflicted head trauma from preschool age. Patient 3, who practiced karate and boxing from preschool age, showed gradual declining intellectual ability. Brain MRI of the three patients revealed severe lacerations in the bilateral cerebral WM. Previous neuroimaging showed no WM lacerations at 4 and 5 years in patients 1 and 2, or mild WM lacerations at 17 years in patient 3, indicating the WM lacerations could have been acquired in childhood. It is suggested that repetition of head trauma in children can cause cerebral WM lacerations and brain dysfunction.     

自身免疫性脑炎临床表现与MRI特征分析

目的对自身免疫性脑炎的临床症状与MRI特征进行分析,提高临床医师对自身免疫性脑炎的临床特征及MRI表现的认识。方法纳入自身免疫性脑炎33例,收集一般临床资料,采用MRI观察颅内病变情况,分析其主要症状及影像学特征。结果本组自身免疫性脑炎的主要症状为精神行为异常27例(81.8%),癫痫发作26例(78.8%),18例(54.5%),发热11例(33.3%),中枢性低通气9例(27.3%)等,其中不自主运动和发热发生率LE组较NMDA组低。MRI阳性者10例,脑实质受累8例,脑膜受累3例,脑实质依次累及枕叶、双侧海马、额叶、顶叶、颞叶及丘脑、小脑等部位。结论精神行为异常及癫痫发作为自身免疫性脑炎最常见的两个症状,MRI可发现边缘系统异常信号,其中FLAIR序列最为敏感。     

MRI的FLAIR和SET2WI序列对急性脑梗死诊断的对比研究

目的研究MRI诊断脑梗死FLAIR序列和常规SET2WI序列的临床应用价值。方法对51例经常规MRI诊断为脑梗死的患者进行FLAIR序列成像，并与SE序列T2加权像比较。结果SET2WI显示急性脑梗死病灶105个，FLAIR显示急性脑梗死病灶110个病灶，有2个脑干部位病灶仅为SET2WI显示，而SET2WI显示的腔隙性病灶经FLAIR序列显示其中有10个为低信号，考虑为血管周围间隙，7个为低信号件周围高信号，考虑为陈旧性脑梗死灶，未予计算在内。结论急性脑梗死诊断中FLAIR序列成像与SET2WI序列互为补充，应作为常规序列使用。     

可复性后部脑病综合征的影像学诊断

目的探讨可复性后部脑病综合征（PRES）的影像学表现.方法回顾性分析了12例PRES病人的临床和影像学资料,其中9例为子痫/先兆子痫,2例为高血压脑病,1例为环孢菌素A（CSA）的神经毒性.12例均行MRI检查,其中7例同时行钆喷替酸葡甲胺（Gd-DTPA）增强扫描,4例行磁共振血管造影（3D-TOF MRA）检查,1例行弥散加权成像（DWI）.7例行CT平扫检查,2例行脑血管造影（DSA）检查.结果MRI显示病灶基本上呈双侧对称性分布,多数病灶位于顶、枕叶脑实质内,T1WI呈等或略低信号,T2WI呈高信号,FLAIR像显示皮层和皮层下白质明显高信号影,较T1WI、T2WI更加清楚.注射Gd-DTPA后多无明显异常对比增强.1例DWI显示双侧顶、枕叶及额叶皮层内弥散受限呈高信号,ADC图显示邻近的皮层下白质呈高信号.4例CT显示双侧顶、枕叶及额叶对称性斑片状低密度影,3例CT未见异常.经对症处理后复查示所有病灶几乎完全吸收消失.结论PRES的影像学表现具有特征性.MRI应作为诊断本病的首选手段.     

Diffusion-weighted magnetic resonance imagings at the acute stage in two patients with spectacular shrinking deficit due to cardioembolic stroke]

We report diffusion-weighted magnetic resonance imagings (DWI) at the acute stage of two patients with spectacular shrinking deficit (SSD) due to cardioembolic stroke. Patient 1 was a 74-year-old woman with atrial fibrillation (Af) who had been admitted for acute cholecystitis. She abruptly developed consciousness disturbance, global aphasia and right hemiparesis. Her neurological symptoms rapidly improved 30 minutes after onset, and completely disappeared in four hours. Patient 2 was a 84-year-old woman with Af who had been on medication of warfarin potassium for three years. She abruptly developed consciousness disturbance and left hemiplegia. Her neurological symptoms rapidly improved 90 minutes after onset, and almost completely disappeared in ten hours. Their conditions were consistent with SSD in acute cardioembolic stroke. DWI of Patient 1 taken 27 hours after onset showed hyperintense signal areas in the insular and temporal cortices of the left middle cerebral artery territory, and in the parietal cortex corresponding to the border zone between the territories of the left middle cerebral artery and posterior cerebral artery. DWI of Patient 2 taken 39 hours after onset showed hyperintense signal areas in the insular and frontal cortices of the right middle cerebral artery territory, and in the parietal cortex corresponding to the border zone between the territories of the right middle cerebral artery and posterior cerebral artery. They indicated multifocal ischemic injuries at the acute stage. The T2-weighted MRI of Patient 2 showed a slight hyperintense signal area only in the right parietal cortex, but the fluid-attenuated inversion recovery (FLAIR) in both patients showed no abnormal signals in the corresponding areas. To our knowledge, ischemic lesions in DWI of SSD at the acute stage after rapid recovery have not been reported previously. DWI is useful in SSD for detecting ischemic injuries of cardioembolic origin at the early stage.     

胚胎发育不良性神经上皮肿瘤的MRI表现

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)的MRI影像学表现及临床特点,以期提高对DNT的诊断水平.方法 收集经手术病理证实的12例DNT患者临床资料,回顾性分析其影像学表现及临床特点.结果 12例患者肿瘤均位于幕上大脑半球皮层内,颞叶8例,额叶及顶叶各2例,其中6例累及邻近脑白质.MRI平扫中,T1WI呈低或等低混杂信号,T2WI及FLAIR呈高信号,肿瘤表现为囊性或以囊性部分为主,部分内可见网状分隔.5例病灶表现为"三角征",5例表现为"脑回征",另2例表现为圆形病灶.肿瘤均无明显占位效应,1例瘤周轻度水肿,增强扫描仅1例有轻度不均匀强化.结论 DNT符合一般良性肿瘤的生物学特征,MRI诊断价值很大.大脑半球皮层内囊性病变,无占位效应,瘤周无水肿,出现"三角征"和"脑回征"等征象可提示DNT的诊断.

Abstract：

Objective To study the MRI appearance and clinical features of dysembryoplastic neuroepithelial tumor (DNT) to improve accurate diagnosis of DNT. Methods The clinical data and MRI appearance of 12 patients with DNT confirmed by surgery and pathology were analyzed retrospectively. Routine MRI was performed in all of the 12 patients, and also dynamic contrast-enhanced MR imaging in 10 of them. Results Eight lesions resided in the temporal lobes, 2in the frontal lobe, and the other 2 in the parietal lobe. All of the 12 DNTs located in supratentorial hemisphere cortex, and 6 of them encroached the adjacent white matter. The lesions appeared as hypointense or iso-hypointense signal on T1WI of MRI, and hyperintense on T2WI and FLAIR of MRI.Cystic structure was shown in all the lesions or as its main part, and mesh-separated structure was also noted in some of the tumors. Five lesions appeared as a triangle in shape, 5 in gyrus-like shape and the remaining 2 in round shape. All the tumors had no significant mass effect with 1 having mild edema around the tumor. Enhanced MR imaging showed only 1 lesion having slight and heterogeneous enhancement. Conclusion DNT is accorded with general tumor's biological characteristics. MRI has great value on DNT's diagnosis. If a cystic lesion locates in the hemisphere cortex with a triangle in shape or gyrus-like shape, having no significant mass effect and peripheral edema, it has a great possibility of being DNT.     

MRI弥散加权成像(DWI)诊断急性、亚急性脑梗塞及相关定量参数的应用

目的:总结我院2003～2004年间急性、亚急性脑梗塞病16例患者的MRI弥散加权成像(DW1)的表现,与 T2WI及T2WI FLAIR序列进行比较,并对感兴趣区进行ADC(表观弥散系数)值测量,推测梗塞灶的面积。方法:急性、亚急性脑梗塞病例16例,其中4例为4小时内检查发现,行急诊溶栓;8例为24小时内检查;2例为3天后一周内检查发现。临床表现12例为脑卒中症状,另外,1例为术后甲状腺危象患者抢救成功后检杳发现脑梗塞病灶,2例颈椎病症状、1例病人以急性心梗入院,入院后检查有亚急性脑梗塞灶。结果:诊断急性、亚急性梗塞病灶24个,基底节区5个,放射冠区4 个,半卵圆中心区2个,小脑半球3个,脑干4个,同时或分别累及颞顶枕叶共6例。其中5名患者为多次发生梗塞,通过 DWI确定责任病灶7个。在放射冠区、小脑、及枕叶病灶明显,基底节区部分病灶显示欠佳,可能与病灶面积较小有关。 T2WI发现病灶5个,T2WI Flair发现病灶6个。TIWI显示病灶数为0个,结论:DWI对诊断急性脑梗塞以及鉴别新旧脑梗塞、确定责任病灶上有较高价值;ADC值的测量对诊断及推断缺血区面积有参考意义;而不同b值(弥散梯度因子)对诊断没有明显的影响。     

Reversible posterior leukoencephalopathy syndrome in Hashimoto's encephalopathy: a case report]

A 45-year-old woman with breast cancer was admitted to our hospital because of several episodes of disturbed consciousness and generalized convulsions. While these symptoms resolved quickly, dysphagia and bilateral blepharoptosis persisted. Neurological findings were improved by steroid therapy. MRI on the first hospital day showed T2/FLAIR high intensity lesions in both occipital lobes, but these lesions diminished on the 8th day, indicating reversible posterior leukoencephalopathy syndrome. A new lesion appeared in the left temporal lobe on the 8th day. A diagnosis of Hashimoto's encephalopathy (HE) was made due to the following features: 1) encephalitis not due to herpes simplex virus, 2) high titers of antithyroid antibodies in serum, 3) marked effectiveness of steroid therapy, and 4) antibodies against the amino terminal of alpha-enolase, a specific antigen for HE.     

Severe Hypomagnesaemia Causing Reversible Cerebellopathy

Hypomagnesaemia is common among hospitalised patients and is often under-recognised. Chronic alcohol abuse and alcohol withdrawal are known causes for severe hypomagnesaemia. Hypomagnesaemia can present with cardiac arrhythmias, seizures and other neurological symptoms, among which ataxia. We present a 57-year-old man with a history of chronic alcohol abuse who developed a subacute cerebellar syndrome with hypertension after alcohol withdrawal. A severe hypomagnesaemia of 0.19 mmol/L (normal values 0.70–1.10) was found. MRI showed diffuse, T2 hyperintense lesions in and swelling of the cerebellum. Symptoms, hypertension and MRI abnormalities significantly improved rapidly after intravenous magnesium supplementation. Hypomagnesaemia can cause a subacute, cerebellar syndrome and hypertension. Symptoms, hypertension and MRI abnormalities can be reversed with rapid magnesium supplementation. MRI abnormalities are similar to those caused by vascular endothelial dysregulation seen in posterior reversible encephalopathy syndrome (PRES). A similar case was recently described. We confirm that magnesium is likely to be involved in the pathophysiology of PRES.     

Deep cerebral venous thrombosis: a report of three cases and review of the literature]

Three cases of cerebral deep venous thrombosis (CDVT) were reported with review of the literature. A 47-year-old female had taken estrogen-derived drug. The other two patients had no specific past history. On MRI, T2-weighted and fluid-attenuated inversion recovery (FLAIR) images showed high signal intensity lesions at basal ganglia and thalamus. Diffusion-weighted image (DWI) detected only slightly high signal spots but apparent diffusion coefficient (ADC) images indicated mild increases of the ADC value. MR venogram and cerebral angiogram revealed obliteration of internal cerebral veins, great vein of Galen, and straight sinus. The two severely impaired patients received systemic heparinization, in which one patient preceded percutaneous transvenous angioplasty of straight sinus. One patient suffered cognitive disturbance and the other two patients fully recovered from their illness. The high signal intensity lesions on both T2-weighted image and FLAIR image disappeared and deep cerebral veins reappeared. The diagnosis of CDVT based on clinical symptoms is not simple but modern technology of MRI is very useful for diagnosis of CDVT. Once CDVT is detected, appropriate therapy should be started as soon as possible to avoid devastating outcome.     

Reversible posterior leukoencephalopathy syndrome: experience in 3 cases]

We report 3 cases with reversible posterior leukoencephalopathy syndrome (RPLS) accompanied by eclampsia or hypertensive encephalopathy. RPLS may develop in patients who have eclampsia or hypertensive encephalopathy or who are immunosuppressed. The findings on neuroimaging are characteristic of subcortical edema without infarction. A 27-year-old primigravida developed eclampsia at 37 weeks of gestation. MRI was performed 4 hours after the onset. The FLAIR sequence delineated extensive hyperintense lesions in the temporal and occipital lobe bilaterally. MR angiography(MRA) performed 6 days after the onset of symptoms clearly demonstrated intracranial vasospasm. Follow up MRI and MRA were performed 3 weeks after the onset. The MRI showed slight residual hyperintensity in the occipital lobe. The MRA showed the disappearance of the vasospasm. A 39-year-old woman on the 8th postpartum day presented with thunderclap headache, which led to a search for SAH. She visited our hospital, whose high arterial blood pressure (220/110 mmHg) was observed. Both CT and MRA were normal. MRI revealed abnormalities in the parieto-occipital regions bilaterally. Treatment of hypertension led to resolution of the posterior leukoencephalopathy. A 38-year-old woman on the 11th postpartum day suddenly developed vertigo, visual disturbance and generalized convulsion. MRI was performed 7 days after the onset. The FLAIR sequence delineated extensive hyperintense lesions in the occipital lobe bilaterally. MRA clearly demonstrated diffuse intracranial vasospasm. MRA performed 3 weeks after the onset showed the disappearance of the vasospasm. In conclusion, our experience suggests that the MRI and MRA noninvasively provide valuable findings which are complementary in the diagnosis and follow-up examination of a brain edema and vasospasm in RPLS.     

Distinguishing ischemic stroke from the stroke-like lesions of MELAS using apparent diffusion coefficient mapping

We report two patients with migraine, acute visual field defects and other neurological symptoms who were found to have high T(2) signal and FLAIR abnormalities on brain MRI in temporal and parieto-occipital regions. In these patients, the apparent diffusion coefficient (ADC) of their lesions was increased, distinguishing these lesions from those of ischemic stroke. Both were ultimately diagnosed with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). We conclude that conventional MRI when used with diffusion-weighted MR imaging may be invaluable in detecting mitochondrial-related CNS dysfunction.     

Formes cavitaires de sclérose en plaques : étude multicentrique sur vingt patients

IntroductionCavitary white matter changes are mainly described in leukodystrophies and especially in vanishing white matter disease. Large cavitary lesions are not typical for multiple sclerosis (MS).MethodsWe studied MS patients with large cavitary brain lesions. Patient characteristics, disease onset/duration/subtype, expanded disability status scale (EDSS), mini mental state (MMS), vanishing white matter disease genetic analysis, and MRI characteristics of the cavitary lesions were analyzed.ResultsTwenty patients were analyzed (6 men and 14 women). Mean age at disease onset was 37.6 (range 17–58). Mean disease duration was 10 years (range 2–20). Five patients had initial relapsing-remitting MS and nine patients had primary-progressive MS. Mean EDSS was 5.5 (range 2–8). Mean MMS was 20/30. Vanishing white matter disease genetic analysis was performed and negative in seven patients. Inferior corpus callosum lesions were seen in all patients with available sagittal FLAIR sequences. Cavitary lesions were strictly supratentorial, and located inside the diffuse leukoencephalopathy, with often a posterior predominance.ConclusionMS patients with large cavitary lesions seem to represent a MS subgroup, predominantly women, with relatively late disease onset, predominantly primary-progressive type, relatively high EDSS scores, and severe cognitive dysfunction.     

Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome?

Posterior reversible encephalopathy syndrome (PRES) has a distinctive clinical presentation and typical neuroimaging findings. However, data on its clinical course and recurrence are scarce. This study aims to investigate its clinical profile and factors that predict recurrence. We included patients diagnosed with PRES between 2005 and 2010 and collected data on demographics, presenting symptoms, co-morbidities, risk factors, clinical parameters, MRI findings, complications and recurrence. Patients were categorized into two groups: PRES due to primary hypertension and PRES due to secondary causes. Correlation with presenting symptoms, radiological features, and recurrence were analyzed. PRES was identified in 28 patients. Fourteen (50%) had primary hypertension. Secondary causes included immunosuppression-related (39%), preeclampsia/eclampsia (7%), and marijuana-intake-related (4%) causes. Patients presented with altered mental status (79%), headache (75%), seizure (68%), visual disturbance (39%) and hemiparesis (21%). On MRI 93% had the typical parietal–occipital involvement. The frontal lobe was affected in 64%, cerebellum in 29%, brainstem in 21%, and basal ganglia in 11%. About 36% had cortical involvement; 21% had diffusion-restricted lesions. Non-aneurysmal subarachnoid haemorrhage was found in 18% of patients and intracerebral hemorrhage in 14% of patients. No significant difference existed in presenting symptoms and the MRI distribution of vasogenic edema between the primary hypertension group and the secondary causes group. Recurrence occurred in four patients (14.3%, 95% confidence interval 4.2–33.7) and was significantly associated (p = 0.05) with primary hypertension as the etiology. Intensive monitoring and treatment of hypertension is recommended for reducing morbidity.     

GLOS and HARM in patients with transient neurovascular symptoms with and without ischemic infarction

Background and purposeGadolinium leakage in ocular structures (GLOS) on fluid attenuated inversion recovery images (FLAIR) is a novel imaging marker in acute ischemic stroke and other neurological disorders.MethodsIn patients with transient neurovascular symptoms who underwent repeated MRI with intravenous contrast agent administration, the presence of acute ischemic lesions on diffusion-weighted images (DWI) as well as the frequency and pattern of blood-brain barrier and blood-retina barrier impairment as demonstrated by the hyperintense acute reperfusion marker (HARM) and GLOS respectively on postcontrast FLAIR were evaluated.ResultsOverall 28 patients with transient neurovascular symptoms (median age 70.5 years; 18 (64.3%) male) were included. Follow-up MRI was performed within 35 (IQR 21–47) hours after the initial MRI. On DWI, acute ischemic lesions were observed in 22 (78.6%). On contrast-enhanced FLAIR, GLOS was observed in 12 (42.9%) patients: in 1 (3.6%) only in the anterior chamber, and in 11 (39.3%) in the anterior chamber and vitreous body. HARM was observed in 3 (10.7%) patients. In one patient without ischemic lesion on DWI or HARM on FLAIR, GLOS was observed in the anterior chamber and vitreous body. Presence of GLOS was associated with higher age (p = 0.04) and detection of HARM (p = 0.03).ConclusionsIn patients with transient neurovascular symptoms, GLOS is a frequent finding and associated with HARM on contrast-enhanced FLAIR. As GLOS was observed in one patient without an ischemic lesion or HARM, it might be useful as an additional imaging marker.     

Posterior reversible encephalopathy syndrome in a child with bronchial asthma

Although posterior reversible encephalopathy syndrome (PRES) is caused by various conditions, there have been no reports on PRES associated with bronchial asthma. We report a case with PRES during the treatment for severe asthmatic attack. A 4-year-old girl was treated for asthmatic attack with steroids. From the 10th hospital day, hypertension, pulmonary edema, and cardiomegaly were observed. In spite of treatment with furosemide, she became lethargic and had a generalized convulsion on the 23rd hospital day. CT showed low density areas in the bilateral occipital white matter and MRI on the 28th hospital day demonstrated high intensity areas in the same regions on T2-weighted and FLAIR images. After discontinuation of corticosteroid and further antihypertensive therapy, her consciousness improved. MRI on the 67th hospital day had no abnormalities and no neurological sequelae were seen at 2 years after the event. We should be aware that PRES is a rare but important adverse event related to steroid therapy, because hypertension and water retention are major adverse effects of steroids.     

MRI sequence findings in sporadic Creutzfeldt–Jakob disease

MRI has had an important role in the diagnosis of Creutzfeldt–Jakob disease (CJD). The aim of our study was to compare the efficacy of different MRI sequences among six biopsy-proven patients with sporadic CJD (sCJD) and seven patients with probable sCJD. These 13 patients with CJD aged from 36 years to 75 years (mean age: 55.5 years) were evaluated with T1-weighted, T2-weighted, and fluid-attenuated inversion recovery (FLAIR) MRI and diffusion-weighted imaging (DWI). The characteristic MRI lesion pattern was found to be bilateral, symmetric and hyperintense signal changes in the basal ganglia and cortical regions. Two major lesion patterns were identified in all patients involving the cortex and basal ganglia. No signal abnormality was found in the thalamus. We found lesions in the cortex and basal ganglia in 7/13 patients (54%), isolated cortical involvement in 2/13 patients (15%), and isolated basal ganglia lesions in 4/13 patients (31%). The cortical involvement was widespread (in at least two regions) and usually included the frontal or occipital lobes (9/13, 69%) on DWI. Only one patient showed moderate high-signal intensity in the basal ganglia on T2-weighted MRI. T1-weighted MRI revealed no signal intensity abnormalities. We conclude that high signal changes in the basal ganglia and cerebral cortex on FLAIR and DWI are useful in the diagnosis of sCJD. Isolated cortical involvement on DWI and FLAIR should lead to a suspicion of CJD. DWI is the most sensitive MRI technique in the diagnosis of CJD, which supports an amendment to the clinical diagnostic criteria for sCJD to include findings from MRI.