Intermittent Rhythmic Delta Activity Research Articles

Electrographic Correlates of Plateau Waves in Patients With Leptomeningeal Metastases

We describe video electroencephalography (video-EEG) correlates of transient neurological attacks due to plateau waves-paroxysmal elevations in intracranial pressure-in patients with leptomeningeal metastases. We identified 3 patients with leptomeningeal metastases, intracranial hypertension, and transient neurological attacks captured on video-EEG without evidence of seizures or epileptiform activity. We identified all clinical events on video and reviewed the corresponding EEG data for evidence of abnormalities. All 3 patients had mild to moderate slowing and 2 had frontal intermittent rhythmic delta activity during background EEG recording. There were 33 clinical events recorded and stereotyped for each patient. All 33 events were associated with an increase in delta range slowing of ≥30% compared to the background. This abnormality started ≤2 minutes before the onset of clinical symptoms and persisted for minutes after clinical resolution. This study is the first to carefully describe the electrographic correlates of transient neurological attacks due to plateau waves in patients with leptomeningeal metastasis. Clinical attacks were consistently associated with a possible EEG signature of diffuse delta range slowing. Future studies can validate the sensitivity and specificity of these EEG changes as a prognostic and/or response biomarker in patients with leptomeningeal metastases with or without intracranial hypertension.

Electroencephalographic findings in schizophreniform and affective disorders

Objective: Pathological findings in electroencephalography (EEG) are discussed as a possible marker of organic mental disorders and a therapeutic response to anticonvulsive medication under these conditions.Methods: We compared the prevalence of EEG abnormalities in 100 patients with schizophrenia, 100 patients with schizoaffective disorder, 51 patients with acute polymorphic psychotic disorder, 100 patients with bipolar disorder, 100 patients with unipolar major depression and 76 healthy control subjects with the findings of a previous study using well-diagnosed, large control samples (13,658 pilots and aircrew personnel).Results: We detected an increased number of pathological EEG findings with intermittent rhythmic delta or theta activity in 7% of patients with schizophrenia, 7% of patients with schizoaffective disorder, 5.9% of patients with acute polymorphic psychosis, 6% of patients with bipolar disorder, 4% of unipolar depressed patients and 3.9% of the own control group, compared to 1% of strictly controlled healthy subjects. One-sided logistic regression revealed an association between pathological EEGs and the diagnosis of schizophrenia (Wald W = 3.466, p = 0.0315), schizoaffective disorder (W = 3.466, p = 0.0315) and bipolar disorder (W = 2.862, p = 0.0455).Conclusions: We suggest that the previously developed local area network inhibition model for a potential paraepileptic pathomechanism can explain the relevance of such findings in different psychiatric disorders.

Delta Activity at Sleep Onset and Cognitive Performance in Community-Dwelling Older Adults.

Frontal intermittent rhythmic delta activity (FIRDA) has long been considered to be an abnormal variant in the electroencephalogram (EEG) among older adults. Prior work also indicates a predominance of slow wave EEG activity among patients with dementia. However, instability of state control occurring with aging generally and among many neurodegenerative diseases raises the possibility that FIRDA might represent the intrusion of sleep related elements of the EEG into the waking state. We examined delta activity at sleep onset (DASO) in community-dwelling, older adults without dementia, and examined whether this activity is related to poorer cognitive performance. 153 community-dwelling, older adults without dementia underwent overnight polysomnography and measures of global cognition, delayed verbal memory, information processing speed, attention, inhibition, verbal naming, and visuospatial ability. Delta activity during sleep/wake transitions (scored either as Waking or N1) was analyzed visually. Participants were 83 women and 70 men, mean age 71.3 ± 0.6 y. DASO was present in 30 participants (19.6%). Age, years of education, sex, and body mass index did not differ between DASO (+) and (-) groups. Multiple regression analyses indicated faster reading of the Stroop color words in DASO (+) subjects (P = 0.007). None of the other cognitive domains differed between the two groups. DASO was relatively common in our sample of community-dwelling, older adults without dementia. DASO was not associated with poorer performance on any cognitive domain. Instead, individuals with DASO demonstrated better performance on a simple reading task. Although these findings suggest that an abnormal EEG activity may represent normal variation, our work underscores the importance of distinguishing DASO from FIRDA when examining sleep in older adults. A commentary on this article appears in this issue on page 725.

Increased Prevalence of Intermittent Rhythmic Delta or Theta Activity (IRDA/IRTA) in the Electroencephalograms (EEGs) of Patients with Borderline Personality Disorder.

Introduction: An increased prevalence of pathological electroencephalography (EEG) signals has been reported in patients with borderline personality disorder (BPD). In an elaborative case description of such a patient with intermittent rhythmic delta and theta activity (IRDA/IRTA), the BPD symptoms where linked to the frequency of the IRDAs/IRTAs and vanished with the IRDAs/IRTAs following anticonvulsive therapy. This observation raised a question regarding the prevalence of such EEG abnormalities in BPD patients. The aim of this retrospective study was to identify the frequency of EEG abnormalities in a carefully analyzed psychiatric collective. Following earlier reports, we hypothesized an increased prevalence of EEG abnormalities in BPD patients.Participants and Methods: We recruited 96 consecutive patients with BPD from the archive of a university clinic for psychiatry and psychotherapy, and compared the prevalence of EEG abnormalities to those of 76 healthy controls subjects. The EEGs were rated by three different blinded clinicians, including a consultant specializing in epilepsy from the local epilepsy center.Results: We found a significant increase in the prevalence of IRDAs and IRTAs in BPD patients (14.6%) compared to the control subjects (3.9%; p = 0.020).Discussion: In this blinded retrospective case-control study, we were able to confirm an increased prevalence of pathological EEG findings (IRDAs/IRTAs only) in BPD patients. The major limitation of this study is that the control group was not matched on age and gender. Therefore, the results should be regarded as preliminary findings of an open uncontrolled, retrospective study. Future research performing prospective, controlled studies is needed to verify our findings and answer the question of whether such EEG findings might predict a positive response to anticonvulsive pharmacological treatment.

Therapeutic Outcomes and Prognostic Factors in Childhood Absence Epilepsy.

Background and PurposeChildhood absence epilepsy (CAE) is one of the most common types of pediatric epilepsy. It is generally treated with ethosuximide (ESM), valproic acid (VPA), or lamotrigine (LTG), but the efficacy and adverse effects of these drugs remain controversial. This study compared initial therapy treatment outcomes, including VPA-LTG combination, and assessed clinical factors that may predict treatment response and prognosis.MethodsSixty-seven patients with typical CAE were retrospectively enrolled at the Korea University Medical Center. We reviewed patients' clinical characteristics, including age of seizure onset, seizure-free interval, duration of seizure-free period, freedom from treatment failure, breakthrough seizures frequency, and electroencephalogram (EEG) findings.ResultsThe age at seizure onset was 7.9±2.7 years (mean±SD), and follow-up duration was 4.4±3.7 years. Initially, 22 children were treated with ESM (32.8%), 23 with VPA (34.3%), 14 with LTG (20.9%), and 8 with VPA-LTG combination (11.9%). After 48 months of therapy, the rate of freedom from treatment failure was significantly higher for the VPA-LTG combination therapy than in the three monotherapy groups (p=0.012). The treatment dose administrated in the VPA-LTG combination group was less than that in the VPA and LTG monotherapy groups. The shorter interval to loss of 3-Hz spike-and-wave complexes and the presence of occipital intermittent rhythmic delta activity on EEG were significant factors predicting good treatment response.ConclusionsThis study showed that low-dose VPA-LTG combination therapy has a good efficacy and fewer side effects than other treatments, and it should thus be considered as a firstline therapy in absence epilepsy.

Consistent topography and amplitude symmetry are more typical than morphology of epileptiform discharges in genetic generalized epilepsy

ObjectiveTo provide a quantitative evaluation of typical electroencephalographic (EEG) abnormalities in genetic generalized epilepsy (GGE). MethodsWe prospectively performed 24-h ambulatory EEG recordings in a cohort of patients with GGE. The diagnosis was established according to the International League Against Epilepsy criteria. Details of all epileptiform discharges across the 24-h time scale were entered into an electronic database. We carried out descriptive statistics to provide a quantitative breakdown of typical EEG abnormalities. ResultsA total of 6923 epileptiform discharges from 105 abnormal 24-h ambulatory EEGs were analyzed. 96% of discharges were symmetric in amplitude with fronto-central maximum topographically. Only 24% of the paroxysms had typical morphology while 43% were regular. Photoparoxysmal response, eye-closure sensitivity and hyperventilation-induced generalized paroxysms were less common in around 10%, whereas occipital intermittent rhythmic delta activity was very rare (2%). ConclusionOur results indicate that generalized discharges with symmetric amplitude and fronto-central maxima are the most consistent findings in GGE, and other features are observed less frequently. SignificanceEpileptiform discharges displaying highly consistent amplitude symmetry coupled with fronto-central topography should provoke consideration of GGE. Recognition of variations from typical abnormalities is important to avoid the risk of misdiagnosis and delayed diagnosis.

Clinical utility of electroencephalography in encephalopathy

Encephalopathy is an entity characterized by temporary or permanent disorder of brain functions: memory, memorising, perception, personality disorders, psychomotoral hyperactivity, agitation, hallucination, illusion, delirium, disorientation, seizures, asterixis, myoclonismus, coma and death. Encephalopathy causes are various and electroencephalography (EEG) represents an important encephalopathy diagnostic procedures, despite low specificity. Typically, EEG manifestations of encephalopathy are loss of normal rhythms and slow diffuse brain activity. More specific patterns are: triphasic waves in hepatic or uremic encephalopathy and in Jakob–Creutzfeldt disease or tricyclic waves discharge with frontal or fronto-occipital gradient after aggressive hemodialysis. Spikes, triphasic waves, intermittent rhythmic delta activity (IRDA), localized frontally (FIRDA) may be observed in Hashimoto encephalopathy. FIRDA may be observed during episodes of hypoglucemia, hypernatremia, hepatic encephalopathy. Hyperglycemia may provoke periodic lateralized epyleptic discharges (PLEDs). PLEDs and generalized periodic epileptic discharges (GPEDs) may be observed in patients with viral encephalitis and hypoxic encephalopathy. Burst suppression pattern (BSP) in anoxic patients and electro-cortical inactivity in hypothermia are the usual signs of fatal prognosis. Specificity of EEG in encephalopathy, despite limitations, may offer clinical utility in estimation of diseases’ severity and prognosis.

Are There Any Specific EEG Findings in Autoimmune Epilepsies?

This study evaluated the EEG findings of patients whose seizures were associated with a possible autoimmune etiology. Our aim was to find clues to distinguish patients with antineuronal antibodies (Ab) through EEG studies. We reviewed our database and identified antineuronal Ab positive epilepsy patients with or without autoimmune encephalitis. These patients had Abs to N-methyl-d-aspartate receptor (NMDAR) (n = 5), glycine receptor (GLY-R) (n = 5), contactin-associated protein-like 2 (CASPR-2) (n = 4), uncharacterized voltage-gated potassium channel complex (VGKC) antigens (n = 2), glutamic acid decarboxylase (GAD) (n = 2), Hu (n = 1), and amphiphysin (n = 1). The control group consisted of 21 seronegative epilepsy or encephalopathy patients with similar clinical features. EEG findings were compared between the groups in a blindfolded design. We did not find any significant difference in EEG findings between antineuronal Ab positive epilepsy patients and seronegative control group. It was remarkable that four seropositive but none of the seronegative patients presented with nonconvulsive status epilepticus (NCSE) or focal motor status epilepticus. Continuous theta and delta rhythms were observed in 5 (71%) seropositive patients with autoimmune encephalitis and 2 (25%) seronegative patients. Eight (40 %) seropositive patients showed a frontal intermittent rhythmic delta activity (FIRDA) pattern as opposed to 5 (24%) seronegative patients. Two patients with NMDAR Ab positivity showed rhythmic delta waves superimposed with beta frequency activity resembling "delta brush" pattern. EEG seems as a limited diagnostic tool in differentiating epilepsy and/or encephalopathy patients with a possible autoimmune etiology from those without. However, antineuronal Abs associated with encephalitis should be considered in the etiology of status epilepticus forms. A possible autoimmune etiology for seizures may be considered in the presence of continuous slow waves, FIRDA, and delta brush pattern in the EEG.

Occipital intermittent rhythmical delta activity in a schoolgirl with microcephaly

The clinical significance of occipital intermittent rhythmic delta activity (OIRDA) on the electroencephalogram (EEG) has not been fully established. Over the years, it has been suggested that the interpretation of the OIRDA electroencephalographic pattern may be related to metabolic problems, structural lesions, infections and epilepsy. Recent studies suggested that this pattern occurs almost exclusively in children and is probably of epileptic origin in most case. It has been associated with primary generalized epilepsy syndromes, such as childhood absence. An 8-year-old schoolgirl, attending the 3rd grade, with easy distractibility, inattention, low school performance,and microcephaly is described. EEG was performed awake during hyperventilation revealed occipital intermittent rhythmic delta with a frequency of 3 Hz, bilateral and synchronous, with 2nd and 4th sec of duration. Antiepileptic therapy with valproic acid was begun. After being treated for 8 months, no OIRDA was detected on follow up EEG. The presence of OIRDA activity on EEG in a child should alert us to the presence of absence epilepsy.

The EEG as a diagnostic tool in distinguishing between dementia with Lewy bodies and Alzheimer’s disease

ObjectiveCurrent diagnostic criteria for dementia with Lewy bodies (DLB) regard electroencephalogram (EEG) abnormalities as a supportive feature. It has also been suggested that EEG abnormalities in DLB are more extensive than in Alzheimer’s disease (AD). Still, the use of qualitative EEG analysis as a diagnostic tool to distinguish between DLB and AD remains rare in daily clinical practice because of conflicting studies and absence of a reliable scoring method. The Grand Total EEG (GTE) score has been used in one study to differentiate DLB from AD with good sensitivity and specificity (Roks et al., 2008). MethodsEEGs from 29 patients with DLB and 54 with AD were visually rated according to the GTE score. ResultsPatients with DLB had significantly higher median scores than patients with AD: 9 vs. 4. Patients with DLB could be distinguished from those with AD at a GTE cut-off score of 6.5 with a sensitivity of 79% and a specificity of 76%. The association between GTE and DLB was independent of age, gender, Mini Mental State Examination and use of medication. Frontal intermittent rhythmic delta activity (FIRDA) was found in 17.2% of patients with DLB compared to 1.8% with AD. Except for the lower cut-off score our results are comparable to the previous study on the GTE score. ConclusionThe GTE score has proven to be a reliable and simple scoring method applicable to daily clinical practice. Qualitative EEG analysis can help to differentiate DLB from AD with good sensitivity and specificity. SignificanceEEG should play a more prominent role in daily clinical practice as a diagnostic tool in differentiating DLB from AD. Future revisions of the diagnostic criteria for DLB should consider the other EEG abnormalities as mentioned in the GTE score, especially FIRDA.

Electroencephalographic changes in sporadic Creutzfeldt-Jakob disease and correlation with clinical stages: a retrospective analysis.

To analyze clinical features and EEG changes in patients with sporadic Creutzfeldt-Jakob disease and study the association between different EEG patterns and clinical stages. We retrospectively analyzed the clinical features and EEG patterns of 3 patients with clinical stage II and 10 patients with stage III sporadic Creutzfeldt-Jakob disease (EEG n = 17). Chi-square test was used to study the association between EEG features and disease stages. Diffuse slowing of the background was the dominant EEG pattern in stage II disease. All stage III patients showed additional abnormalities, such as frontal intermittent rhythmic delta activity, periodic discharges, and nonperiodic triphasic waves. Periodic discharges had significant association with stage III disease, present in 64% of EEGs in this group. Sixty-seven percentage of periodic discharges had a peculiar pattern labeled by us as posterior-anterior-posterior lag. The EEG of stage II sporadic Creutzfeldt-Jakob disease is characterized by background slowing while the presence of periodic discharge has a significant association with stage III of the disease. The posterior-anterior-posterior lag of periodic discharge is a new observation.

Rett-like onset in late-infantile neuronal ceroid lipofuscinosis (CLN7) caused by compound heterozygous mutation in the MFSD8 gene and review of the literature data on clinical onset signs

BackgroundWe present clinical and molecular findings of a patient with ceroid-lipofuscinosis CLN7, with a compound heterozygous mutation of the MFSD8 gene, with Rett syndrome clinical signs onset and a later development of full picture of vLINCL. Case presentationA 7 years-old female patient with normal development until the age 12 months, developed Rett like clinical picture (psychomotor regression, microcephaly, stereotypic hands movements in the midline, hyperventilation episodes) present at the onset of her condition (age 18 months), features still present at the initial evaluation in our clinic at age 5 years. ResultsMECP2 (methyl CpG binding protein 2) gene mutation was negative. At age 6 years she was readmitted for severe ataxia and blindness, seizures, and severe developmental regression leading to NCL (neuronal ceroid lipofuscinosis) suspicion. EEG showed slow background with IRDA (intermittent rhythmic delta activity). A conjunctive biopsy showed abnormal curvilinear and fingerprint lysosomal deposits, and genetic analysis revealed two heterozygous mutations of MFSD8 gene (c.881C > A p.Thr294Lys and c.754 + 2T > A) each inherited from carrier parents and a heterozygous variant (c.470A>C p.Asp157Ala) of CLN5 gene. ConclusionNCL should be suspected and MFSD8 genetic testing should also be considered in patients with Rett like phenotype at onset and negative MECP2 mutation. Such cases should be carefully and frequently re-evaluated in order to avoid delayed diagnosis and offer proper genetic advice to the family. In our knowledge, this might be the first case of CLN7 disease with Rett like onset described in the literature, which developed typical vLINCL clinical phenotype after age 5.5 years. A short review of the literature showing NCL onset modalities is presented.

Creutzfeldt-Jakob–Like Syndrome due to Hypercalcemic Encephalopathy

Hypercalcemia can cause a subacute syndrome of progressive dementia and marked changes in the electroencephalogram (EEG). We report a case of iatrogenic hypercalcemia with a close correlation between the clinical course and the EEG changes. A 73-year-old woman presented with a subacute syndrome of progressive dementia and bursts of 1.5 to 2 Hz intermittent rhythmic delta activity superimposed on a low-voltage background activity in the EEG. Clinical and EEG abnormalities rapidly resolved after normalization of serum calcium levels. As part of the diagnostic workup of a subacute progressive dementia, a serum calcium level and an EEG should be obtained to detect a Creutzfeldt-Jakob like syndrome in hypercalcemia. Unlike in Creutzfeldt-Jakob disease, and Creutzfeldt-Jakob-like syndrome induced by lithium intoxication, there are rarely myoclonic jerks and periodic discharges in hypercalcemic encephalopathy.

Temporal intermittent rhythmic delta activity and abdominal migraine

Temporal intermittent rhythmic delta activity and abdominal migraine

The diagnostic utility of EEG in early-onset dementia: a systematic review of the literature with narrative analysis

Early-onset dementia (EOD) is characterized by functionally impairing deterioration in memory, language, personality or visuospatial skills emerging under the age of 65. Cerebral functioning can be assessed by visual electroencephalography (EEG) interpretation. The aim of this systematic review is to evaluate the diagnostic utility of visual EEG in EOD focusing on Alzheimer's disease (AD), vascular dementia (VAD), dementia with Lewy bodies (DLB), and frontotemporal dementia (FTD). Medline, Embase, Scopus, Web of Knowledge, and Google Scholar were systematically searched for studies where EEGs were included in the diagnostic evaluation of patients with dementia under the age of 65. Each paper was quality assessed and the results grouped according to dementia cause with a narrative summary. 4,157 papers were screened, 12 studies met the eligibility criteria with a total of 965 patients. An abnormal EEG was common to all causes of EOD. EEG abnormalities are more severe in early-onset AD patients. EEG severity grade is independent of disease duration. Slow wave activity is common to all dementias, but is most prominent in DLB. Frontal intermittent rhythmic delta activity could be considered as supportive for the diagnosis of DLB as can a Grand Total EEG score of over 9.5. EEG is usually normal in FTD. Focal changes can be seen in advanced VAD. Studies employed small patient groups, varying diagnostic criteria, and only a minority of patient diagnoses was pathologically confirmed. EEG may be useful as an adjunct in the diagnosis of DLB and AD. Further prospective well-powered studies are required to investigate diagnostic utility more robustly.

Case of syndrome of headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) with focal slowing on electroencephalogram

We describe a case of headache and neurological deficits with cerebrospinal fluid (CSF) lymphocytosis in a patient presenting with a 3-week history of recurrent severe headaches associated with negative sensory symptoms and dysphasia. The patient had no cardiovascular risk factors and no family history of migraines. Neurological examination was unremarkable. Cerebral magnetic resonance imaging was unremarkable. CSF analysis revealed lymphocytosis (leucocytes 84 × 10(6)/L, 100% lymphocytes). Extensive laboratory investigations of CSF and serum did not reveal an infectious, autoimmune or metabolic cause. Visual evoked potentials were normal. Awake electroencephalogram revealed intermittent 3-5 Hz generalised slowing and frontal intermittent rhythmic delta activity, without epileptiform discharges. Repeat CSF analysis showed marked reduction of the total leucocyte count and remained negative for infectious aetiology. Propranolol was commenced, and no recurrence of headache or neurological symptoms was observed at follow-up. An extensive literature review on the topic is discussed.

Late‐onset temporal lobe epilepsy in a patient with juvenile myoclonic epilepsy

We report a patient with longstanding, severe juvenile myoclonic epilepsy who subsequently developed features of temporal lobe epilepsy, which gradually became clinically dominant. Over the years, there was an electrographic evolution from the typical generalised epileptiform patterns, characteristic of juvenile myoclonic epilepsy, to the novel appearance of interictal temporal spikes immediately preceding bisynchronous discharges, and subsequently to temporal intermittent rhythmic delta activity and temporal lobe-onset seizures. In this rare case of coexistent primary generalised epilepsy and focal epilepsy, the epileptic networks of the two forms of epilepsy appear to overlap.

EEG Leading to the Diagnosis of Limbic Encephalitis

Limbic encephalitis is characterized by subacute onset of short-term memory loss, seizures, sleep disturbances, as well as psychiatric and behavioral symptoms. A subgroup is associated with voltage-gated potassium channel antibodies (VGKC-Abs). In many cases, brain magnetic resonance imaging (MRI) demonstrates hyperintense areas in the medial part of the temporal lobe. Also, pleiocytosis is frequently found. In this study, we describe a 69-year-old man with VGKC-Abs limbic encephalitis with generalized tonic-clonic seizures, increasing memory deficits, visual hallucinations, depression, and severe insomnia. Brain MRI and cerebrospinal fluid (CSF) were normal, while the electroencephalogram (EEG) showed bilateral frontal and temporal intermittent rhythmic delta activity with disorganization and slowing of background activity, ultimately leading to the diagnosis of limbic encephalitis. The patient improved markedly after starting immunosuppressive therapy, both clinically and electrophysiologically. In addition to temporal lobe involvement on the brain MRI and CSF inflammation, we propose EEG abnormalities as an additional diagnostic criterion for limbic encephalitis.

Electroencephalographic Dipole Source Modeling of Frontal Intermittent Rhythmic Delta Activity

Background: Frontal intermittent rhythmic delta activity (FIRDA) on electroencephalography (EEG) consists of a run of rhythmic delta waves with frontal predominance. Although FIRDA is a relatively common abnormal EEG finding, the underlying mechanisms that produce FIRDA remain unclear. The aim of this study was to investigate the cortical source of FIRDA using dipole source modeling. Methods: We selected EEG epochs, including typical FIRDAs, from EEG recordings obtained using 25 scalp electrodes on 5 subjects. We averaged these epochs by arranging the negative peaks of the delta waves at the Fp electrodes and estimated dipoles for nine averaged waveforms. Results: Averaged waveforms were explained by a single-dipole model in seven FIRDAs and by a two-dipole model in the remaining two FIRDAs with high reliability. Estimated dipoles had a radial orientation with respect to the frontal pole and were located in the medial frontal region. The anterior cingulate cortex was the most common dipole location. Conclusions: This is the first study to approach the fundamental FIRDA mechanism by dipole source modeling and to clarify that FIRDA may be generated from the medial frontal region, particularly from the anterior cingulate cortex.

Wartość badania MR we wczesnym rozpoznaniu choroby Creutzfeldta-Jakoba – doświadczenie własne

Creutzfeldt-Jakob disease (CJD) is a rare progressive neurodegenerative disorder, caused by the deposition of the pathological isoform of prion protein PrPsc in the central nervous system. The classic triad of symptoms consists of: rapidly progressive dementia, myoclonus and typical electroencephalographic findings (intermittent rhythmic delta activity and periodic sharp wave complexes). Detection of 14-3-3 protein in the cerebrospinal fluid plays an important diagnostic role as well. Magnetic resonance (MR) images of the brain have been recently incorporated into the diagnostic criteria of sporadic Creutzfeldt-Jakob disease. MR examinations were performed in a 65-year-old man and a 54-year-old woman with delusional disorder and cognitive dysfunction, respectively. Diffusion restriction (hyperintense signal in DWI) was shown in the cortex of the left parietal and occipital lobe in the first patient and symmetrically in the cortex of both cerebral hemispheres except for precentral gyri in the second one. In both cases, the first examinations were misread, with the suspicion of ischemic infarcts as the first differential diagnosis. Consultations and subsequent MR examinations in which lesions in subcortical nuclei appeared allowed for a diagnosis of probable CJD. In the first case it was confirmed by clinical picture, EEG and finally - autopsy. In the second case, EEG was not typical for CJD but the clinical course of the disease confirmed that diagnosis. The authors present the cases of two patients with characteristic MR images that allowed early diagnosis of probable Creutzfeldt-Jakob disease before the characteristic clinical picture appeared. Early diagnosis is nowadays important for the prevention of disease transmission and in the future - hopefully - for early treatment.