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A 21-channel EEG with the patient awake demonstrates periodic sharp-wave complex discharges, which are of greater amplitude over the left frontotemporal region and are superimposed on diffusely disorganized and slow background.
Source publication
We report the use of diffusion-weighted MR imaging in the early diagnosis and monitoring of the progression of a histopathologically proved case of sporadic Creutzfeldt-Jakob disease. Ribbon-like areas of hyperintensity in the cerebral cortex on diffusion-weighted images corresponded to the localization of periodic sharp-wave complexes on the elect...
Contexts in source publication
Context 1
... at 6 months from onset of symptoms showed diffusely slow and disorganized waking cerebral background activity and left frontotemporal periodic sharp-wave complex discharges (Fig 2), which corresponded to the hyperintense signals on DW images. Left frontal brain biopsy demonstrated gray matter spongiform degeneration with characteristic intracytoplasmic vacuoles, which are the primary pathologic features of sporadic CJD. ...
Context 2
... 2 months (Fig 1), coincident with the deteriora- tion of the patient's MMSE score and independence for performing activities of daily living. At 6 months of illness, EEG revealed periodic sharp-wave complex dis- charges over the left frontotemporal cortex that corre- lated with DW imaging changes (Fig 2). Notably, EEG did not demonstrate similar changes in the right tem- poral cortex where comparable DW imaging abnor- malities were also seen (Figs 1 and 2). ...
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Citations
... [10] The DWI of this patient exhibited a lace-like slight increase in signal intensity in bilateral parietal-occipital and frontal lobes, which is consistent with many other reports of sCJD. [11] The typical EEG appearances in sCJD are periodic, triphasic sharp wave complexes. [10] The EEG of this patient displayed triphasic sharp wave that continued to appear and could be observed bilaterally, which were typical EEG manifestations and supported the diagnosis of sCJD. ...
Rationale:
Creutzfeldt-Jakob disease (CJD) is an infrequent and rapidly fatal neurodegenerative disease without effective cure. Common presentations of CJD include rapidly progressive cognitive decline, behavioral changes, cerebellar dysfunction, and visual disturbances. Since clinicians may see only very few cases during their professional career, it is important to be familiar with the clinical presentation and progression, and allow for quick diagnosis.
Patient concerns:
We reported an elderly woman had recurrent attacks of dizziness in the preceding month. She began to suffer progression of memory disturbance half a month before admission and was admitted to our department in a coma.
Diagnosis:
The accessory examinations of magnetic resonance imaging (MRI), electroencephalography (EEG), 14-3-3 protein in cerebrospinal fluid and S100 protein in serum support the diagnosis of sporadic CJD (sCJD). Combined with this evidence and clinical symptom, we made a clinical diagnosis of sCJD.
Interventions:
Supportive treatment.
Outcomes:
After 2 months of active treatment, the patient's condition had not improved, and the patient died 82 days after admission.
Lessons:
Clinicians should attach importance to sCJD, which is significant for the prevention of transmission and treatment.
... Typically, diffusion-weighted hyperintensity is progressive and persistent over many weeks, affecting the striatum and cortex. The gyriform DWI-hyperintense areas in the cerebral cortex (Bcortical ribbon^sign) correlate to the location of periodic sharp-wave complexes on EEG [66]. DWI hyperintensity may resolve late in the disease. ...
Diffusion-weighted imaging (DWI) has revolutionised stroke imaging since its introduction in the mid-1980s, and it has also become a pillar of current neuroimaging. Diffusion abnormalities represent alterations in the random movement of water molecules in tissues, revealing their microarchitecture, and occur in many neurological conditions. DWI provides useful information, increasing the sensitivity of MRI as a diagnostic tool, narrowing the differential diagnosis, providing prognostic information, aiding in treatment planning and evaluating response to treatment. Recently, there have been several technical improvements in DWI, leading to reduced acquisition time and artefacts and enabling the development of diffusion tensor imaging (DTI) as a tool for assessing white matter. We aim to review the main clinical uses of DWI, focusing on the physiological mechanisms that lead to diffusion abnormalities. Common pitfalls will also be addressed.
Teaching points:
• DWI includes EPI, TSE, RESOLVE or EPI combined with reduced volume excitation. • DWI is the most sensitive sequence in stroke diagnosis and provides information about prognosis. • DWI helps in the detection of intramural haematomas (arterial dissection). • In diffusion imaging, ADC is inversely proportional to tumour cellularity. • DWI and DTI derived parameters can be used as biomarkers in different pathologies.
... Diffusion-weighted imaging abnormalities have been observed as early as 1 month after the onset of symptoms and can localize with periodic sharp wave complexes at EEG, which are observed in 67% of patients with sCJD (174). Antemortem reductions in ADC values are correlated with spongiform changes at postmortem autopsy, especially in the deep gray matter (R 2 = 0.78, P < .001) ...
Viral infections of the central nervous system (CNS) range in clinical severity, with the most severe proving fatal within a matter of days. Some of the more than 100 different viruses known to affect the brain and spinal cord are neurotropic with a predilection for producing CNS infection. The host response to viral infection of the CNS is responsible for the pathophysiology and imaging findings seen in affected patients. Viral CNS infections can take the form of meningitis, encephalitis, encephalomyelitis, or, when involving the spinal cord and nerve roots, encephal omyeloradiculitis. In 1982, an infectious particle termed a prion that lacked nucleic acid and therefore was not a virus was reported to produce the fatal neurodegenerative disease Creutzfeldt-Jakob disease and related disorders. These prion diseases produce characteristic neuroimaging findings that are distinct from those seen in most viral infections. The clinical and imaging findings associated with viral CNS infection are often nonspecific, with microbiologic analysis of cerebrospinal fluid the most useful single test allowing for diagnosis of a specific viral infection. This review details the spectrum of viral CNS infections and uses case material from the archives of the American Institute for Radiologic Pathology, with a focus on the specific clinical characteristics and magnetic resonance imaging features seen in these infections. Where possible, the imaging features that allow distinction of these infections from other CNS inflammatory conditions are highlighted.
... Limited research has been conducted towards understanding how the formation of amyloid plaques affects diffusion. Researchers utilizing diffusion-weighted magnetic resonance imaging (DW-MRI) have demonstrated a reduced interstitial fluid diffusion in the brain of APP23 transgenic mice [91] and reduced diffusivity in the white matter of patients suffering from sporadic Jakob-Creutzfeldt disease [92], another neuroencephalopathy associated with the formation of insoluble protein aggregates. Sykova and Collegues [93] analyzed the ECS and the volume fraction and diffusion of the cerebral cortex in APP23 mice using real-time tetramethylammonium (TMA) and DW-MRI methods. ...
Currently, AD has no cure and only treatments for the symptoms exist. Modern research still debates the toxic component of AD and the exact mechanism causing neurodegeneration. A hallmark of the disease is the production of the amyloid-beta (Aß) peptides and eventual self-assembly of these peptides into fibrils and extracellular Aß plaques. Both plaques and oligomers are proposed to be the direct cause of AD, but it remains unclear how the physical presence of these structures affect neuronal function and pathogenesis. Biomolecule aggregation is known to play a role in the pathogenesis of numerous diseases by restricting diffusion and bulk flow, and the same restriction could occur in the brain due to the dense amyloid plaques forming in the extracellular space. These plaques could prevent proper flow and diffusion of essential nutrients and prevent cellular waste removal by acting as extracellular channel blockades; however, limited models exist that address these issues. Alternative models and molecular tools need to be developed which focus on diffusion and bulk flow in relation to neural function and the physical presence of amyloid plaques. This review aims to evaluate the effect of the plaques on diffusion and bulk flow in relation to neural function in the brain.
... [3] ...
Chorea-ballismus in diabetic patients with uncontrolled hyperglycaemia is often associated with a hyperintense signal on T1-weighted MRI in the basal ganglia. We report two patients: a 62-year old woman with type II diabetes mellitus and the nonketotic hyperosmolar state who presented with acute generalised chorea, and a 72-year old man with right hemichorea beginning one week after correction of severe hyperglycaemia and hyperosmolality as the presenting manifestation of type II diabetes. In both patients, chorea did not improve after correction of dehydration and hyperglycaemia, but responded within one week to treatment with low-dose haloperidol and tetrabenazine. Chorea in this setting is thought to be due to the effect of hyperglycaemia, hyperosmolarity and reduced perfusion on striatal metabolism, possibly in association with underlying focal cerebrovascular disease which leads to striatal dysfunction, astrocytosis, and hyperkinesia. We review 21 patients with this syndrome published from 2001 onwards, and provide a detailed discussion of pathogenesis, clinical features, imaging and prognosis. As demonstrated by our patients, the disorder can be the presenting symptom of DM; it may be unilateral or bilateral; and MRI findings correlate well with the clinical pattern. HCHB improves with correction of the metabolic abnormality in many patients, but some may need neuroleptic therapy, and a few have a poor response. The generally favourable outcome underlines the need for correct diagnosis and adequate management of the metabolic problem underlying the often dramatic onset of chorea.
... However, non-invasive tests (EEG, cerebrospinal fluid 14-3-3 protein or neuron-specific enolase levels) are also useful for ante mortem diagnosis, and lead to a diagnosis of ''probable sCJD'' with over 95% accuracy [2] when compared with autopsy. Recently a number of reports have focused attention on the typical imaging features of sCJD using diffusion-weighted imaging (DWI) which is more sensitive than these methods, particularly in early disease [3] [4] [5] [6] [7]. We report MRI findings in two cases of ''probable sCJD'' with typical clinical course and EEG, and discuss the role of DWI in diagnosis. ...
... Unilateral cortical with ipsilateral striatal or thalamic involvement is considered pathognomonic [5]. Hyperintensity on DWI in these locations with restricted diffusion correlates with vacuolation, spongiosis and prion protein deposition altering molecular motion of water [2] [3] [4] [5] [6] [7] [8] and is associated with focally reduced metabolism and perfusion on functional imaging [3]. Low ADC values correlate significantly with the degree of spongiosis and prion deposits, but not with gliosis or neuronal loss [8]. ...
... Unilateral cortical with ipsilateral striatal or thalamic involvement is considered pathognomonic [5]. Hyperintensity on DWI in these locations with restricted diffusion correlates with vacuolation, spongiosis and prion protein deposition altering molecular motion of water [2] [3] [4] [5] [6] [7] [8] and is associated with focally reduced metabolism and perfusion on functional imaging [3]. Low ADC values correlate significantly with the degree of spongiosis and prion deposits, but not with gliosis or neuronal loss [8]. ...
... De nombreux travaux ont suggéré que les hypersignaux en pondération de diffusion du cortex, des noyaux caudés, putamens et thalami étaient caractéristiques de la maladie, et apportaient un argument diagnostique majeur avant l'apparition de l'atrophie cérébrale ou l'observation d'anomalie de signal en pondération T2[18][19][20][21][22][23][24][25][26][27][28]. Pas d'atteinte corticale visible en pondération T2. ...
Creutzfeldt-Jakob disease is a fatal neurodegenerative disease characterized by an abnormal accumulation of human prion protein PrPSc. It causes a rapidly progressive dementia syndrome. The imaging work-up is based on MRI which often discloses the typical disease anomaly seen as high signal intensity in the basal ganglia and cerebral cortex on Flair sequences and/or more readily on diffusion weighted sequences. This work recalls our fundamental knowledge concerning this disease and presents MRI data from 7 cases and a review of the literature.
... The risk of fCJD is transmitted in an autosomal dominant inheritance pattern, with nearly 100% penetrance. 2 The imaging findings in sCJD typically consist of cortical atrophy and hyperintensities in the basal ganglia, thalamus, and cortex on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI). [3][4][5][6][7][8][9][10][11][12][13] fCJD has imaging findings and neuropathology that are, in general, similar to the most common forms of sCJD; however, most imaging studies on fCJD consist primarily of case reports or studies focused on sCJD that combine a few fCJD patients into a single CJD patient sample. 8,[14][15][16][17][18][19][20][21][22] fCJD studies are limited not only by small sample sizes but also by nonstandardized imaging protocols and clinical and pathophysiologic heterogeneity. ...
The E200K mutation of the PRNP (prion protein) gene is the most common cause of familial Creutzfeldt-Jakob disease (fCJD), which has imaging and clinical features that are similar to the sporadic form. The purpose of this study was to conduct a controlled and blinded evaluation of the sensitivity and specificity of MR imaging in this unique population.
We compared the MR imaging characteristics of 15 early stage familial CJD patients (age, 60 +/- 7 years) with a group of 22 healthy subjects from the same families (age, 61 +/- 8 years). MR imaging included diffusion-weighted imaging (DWI), T2-weighted fast spin-echo imaging, and a fluid-attenuated inversion recovery (FLAIR) sequence. The scans were rated for abnormalities by an experienced neuroradiologist blind to diagnosis, group assignment, age, and sex.
Thirteen of 15 fCJD subjects had abnormal MR imaging. FLAIR signal intensity abnormality in the caudate or putamen nuclei demonstrated a sensitivity of 87% and specificity of 91%. DWI abnormality in the caudate nucleus showed a sensitivity of 73% and a specificity of 100%. Abnormalities in the thalamus (6 patients), cingulate gyrus (6 patients), frontal lobes (4 patients), and occipital lobes (3 patients) were best detected with DWI. No signal intensity abnormalities were demonstrated in the cerebellum. T2-weighted and T1-weighted sequences were uninformative.
FLAIR and DWI abnormalities in the caudate nucleus and putamen offer the best sensitivity and specificity for diagnosing fCJD. Our findings support recent recommendations that MR imaging should be added to the diagnostic evaluation of CJD.
... 57 Increased FLAIR signal corresponded to increased DWI signal intensity in the basal ganglia 58 and cortex. [58][59][60] The mechanism of signal change in FLAIR images continues to be debated. Astrogliosis was reported to be the cause of high FLAIR signal in one paper 61 and another demonstrated a strong correlation between FLAIR signal and accumulation of prion protein (PrP sc ) but did not find any correlation between gliosis or spongiform change on FLAIR. ...
... 24 65 High intensity areas on DWI have been correlated with low perfusion on SPECT, 72 hypometabolism on positron emission tomography (PET), 65 72 clinical findings, 72 disease progression, 60 71 neuropathology showing spongiform change, 64 neuronal loss and gliosis, 71 and periodic sharp wave activity on EEG. 60 72 76 Magnetic resonance spectroscopy Proton magnetic resonance spectroscopy (MRS) assesses the spectra of specific brain metabolites such as N-acetylaspartate (NAA), a decrease in which can serve as a marker of neuronal loss. Decreases in absolute levels or ratios of NAA have been seen in variant, 21 22 sporadic, 21 iatrogenic 74 and inherited CJD. ...
Imaging occupies an important role in the investigation of dementia and neurodegenerative disease. The role of imaging in prion disease used to be one of exclusion of other conditions. Over the past decade, the non-invasive nature of MRI, the improved range of magnetic resonance sequences and the availability of clinical and neuropathological correlation have led to a more prominent position of MRI and its inclusion in the diagnostic criteria for variant Creutzfeldt-Jakob disease. As experience of imaging in human prion disease increases, patterns of change related to strain and genotype may improve the diagnostic potential of imaging in the future, may reduce the need for more invasive testing and prove useful in future therapeutic trials. This paper reviews the current knowledge of imaging appearances in human prion disease.
... Involvement of the cerebral cortex in addition to the basal ganglia has been demonstrated in CJD [5], especially on fluid-attenuated inversion recovery (FLAIR) sequences and DW MR imaging [6 -9]. Some authors have found high sensitivity and better lesion conspicuity for early diagnosis and serial follow-up [7], whereas others have found that the DW MR hyperintensity corresponded to localized EEG changes in the cortex [9], as in our case. ...
A 49-year-old woman presented with progressive dementia. Diffusion-weighted MR images showed abnormalities in the cerebral cortex as well as the putamen and caudate head bilaterally. MR spectroscopic imaging revealed marked asymmetric decrease of normal metabolites in the right basal ganglia, with concordant hypoperfusion on single photon emission computed tomography. Diffusion-weighted MR imaging is more sensitive to cortical involvement in sporadic Creutzfeldt-Jakob Disease, and MR spectroscopy may yield evidence of asymmetric involvement. Combined functional and metabolic MR imaging may be useful in studying CJD.
