PURPOSE: Transcranial sonography (TCS) magnetic resonance (MR) fusion imaging and digital image analysis are useful tools for the evaluation of various brain pathologies. This study aimed to compare the echogenicity of predefined brain structures in Huntington's disease (HD) patients and healthy controls by TCS-MR fusion imaging using Virtual Navigator and digitized image analysis. MATERIALS AND METHODS: The echogenicity of the caudate nucleus (CN), substantia nigra (SN), lentiform nucleus (LN), insula, and brainstem raphe (BR) evaluated by TCS-MR fusion imaging using digitized image analysis was compared between 21 HD patients and 23 healthy controls. The cutoff values of echogenicity indices for the CN, LN, insula, and BR with optimal sensitivity and specificity were calculated using receiver operating characteristic analysis. RESULTS: The mean echogenicity indices for the CN (67.0±22.6 vs. 37.9±7.6, p<0.0001), LN (110.7±23.6 vs. 59.7±11.1, p<0.0001), and insula (121.7±39.1 vs. 70.8±23.0, p<0.0001) were significantly higher in HD patients than in healthy controls. In contrast, BR echogenicity (24.8±5.3 vs. 30.1±5.3, p<0.001) was lower in HD patients than in healthy controls. The area under the curve was 90.9%, 95.5%, 84.1%, and 81.8% for the CN, LN, insula, and BR, respectively. The sensitivity and specificity were 86% and 96%, respectively, for the CN and 90% and 100%, respectively, for the LN. CONCLUSION: Increased CN, LN, and insula echogenicity and decreased BR echogenicity are typical findings in HD patients. The high sensitivity and specificity of the CN and LN hyperechogenicity in TCS-MR fusion imaging make them promising diagnostic markers for HD.
- MeSH
- Adult MeSH
- Huntington Disease * diagnostic imaging MeSH
- Image Interpretation, Computer-Assisted methods MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging * methods MeSH
- Brain * diagnostic imaging MeSH
- Cerebral Cortex diagnostic imaging MeSH
- Multimodal Imaging methods MeSH
- Caudate Nucleus diagnostic imaging MeSH
- Image Processing, Computer-Assisted methods MeSH
- Reference Values MeSH
- ROC Curve MeSH
- Aged MeSH
- Sensitivity and Specificity MeSH
- Ultrasonography, Doppler, Transcranial methods MeSH
- User-Computer Interface MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
This is an international multicentre study aimed at evaluating the combined value of dopaminergic neuroimaging and clinical features in predicting future phenoconversion of idiopathic REM sleep behaviour (iRBD) subjects to overt synucleinopathy. Nine centres sent 123I-FP-CIT-SPECT data of 344 iRBD patients and 256 controls for centralized analysis. 123I-FP-CIT-SPECT images were semiquantified using DaTQUANTTM, obtaining putamen and caudate specific to non-displaceable binding ratios (SBRs). The following clinical variables were also analysed: (i) Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale, motor section score; (ii) Mini-Mental State Examination score; (iii) constipation; and (iv) hyposmia. Kaplan-Meier survival analysis was performed to estimate conversion risk. Hazard ratios for each variable were calculated with Cox regression. A generalized logistic regression model was applied to identify the best combination of risk factors. Bayesian classifier was used to identify the baseline features predicting phenoconversion to parkinsonism or dementia. After quality check of the data, 263 iRBD patients (67.6 ± 7.3 years, 229 males) and 243 control subjects (67.2 ± 10.1 years, 110 males) were analysed. Fifty-two (20%) patients developed a synucleinopathy after average follow-up of 2 years. The best combination of risk factors was putamen dopaminergic dysfunction of the most affected hemisphere on imaging, defined as the lower value between either putamina (P < 0.000001), constipation, (P < 0.000001) and age over 70 years (P = 0.0002). Combined features obtained from the generalized logistic regression achieved a hazard ratio of 5.71 (95% confidence interval 2.85-11.43). Bayesian classifier suggested that patients with higher Mini-Mental State Examination score and lower caudate SBR asymmetry were more likely to develop parkinsonism, while patients with the opposite pattern were more likely to develop dementia. This study shows that iRBD patients older than 70 with constipation and reduced nigro-putaminal dopaminergic function are at high risk of short-term phenoconversion to an overt synucleinopathy, providing an effective stratification approach for future neuroprotective trials. Moreover, we provide cut-off values for the significant predictors of phenoconversion to be used in single subjects.
- MeSH
- Tomography, Emission-Computed, Single-Photon MeSH
- Kaplan-Meier Estimate MeSH
- Middle Aged MeSH
- Humans MeSH
- Caudate Nucleus diagnostic imaging metabolism MeSH
- REM Sleep Behavior Disorder diagnostic imaging metabolism MeSH
- Dopamine Plasma Membrane Transport Proteins metabolism MeSH
- Putamen diagnostic imaging metabolism MeSH
- Retrospective Studies MeSH
- ROC Curve MeSH
- Aged MeSH
- Synucleinopathies diagnostic imaging metabolism MeSH
- Tropanes MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Research Support, Non-U.S. Gov't MeSH
Recently developed therapeutic approaches for the treatment of Huntington's disease (HD) require preclinical testing in large animal models. The minipig is a suitable experimental animal because of its large gyrencephalic brain, body weight of 70-100 kg, long lifespan, and anatomical, physiological and metabolic resemblance to humans. The Libechov transgenic minipig model for HD (TgHD) has proven useful for proof of concept of developing new therapies. However, to evaluate the efficacy of different therapies on disease progression, a broader phenotypic characterization of the TgHD minipig is needed. In this study, we analyzed the brain tissues of TgHD minipigs at the age of 48 and 60-70 months, and compared them to wild-type animals. We were able to demonstrate not only an accumulation of different forms of mutant huntingtin (mHTT) in TgHD brain, but also pathological changes associated with cellular damage caused by mHTT. At 48 months, we detected pathological changes that included the demyelination of brain white matter, loss of function of striatal neurons in the putamen and activation of microglia. At 60-70 months, we found a clear marker of neurodegeneration: significant cell loss detected in the caudate nucleus, putamen and cortex. This was accompanied by clusters of structures accumulating in the neurites of some neurons, a sign of their degeneration that is also seen in Alzheimer's disease, and a significant activation of astrocytes. In summary, our data demonstrate age-dependent neuropathology with later onset of neurodegeneration in TgHD minipigs.
- MeSH
- White Matter pathology ultrastructure MeSH
- Biomarkers metabolism MeSH
- Nerve Degeneration pathology MeSH
- Animals, Genetically Modified MeSH
- Genotype MeSH
- Weight Loss MeSH
- Huntington Disease pathology MeSH
- Body Mass Index MeSH
- Humans MeSH
- Swine, Miniature MeSH
- Disease Models, Animal MeSH
- Motor Cortex pathology ultrastructure MeSH
- Myelin Sheath metabolism MeSH
- Caudate Nucleus pathology ultrastructure MeSH
- Swine MeSH
- Huntingtin Protein metabolism MeSH
- Protein Aggregates MeSH
- Aging pathology MeSH
- Animals MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Female MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Déjà vu (DV) is an eerie phenomenon experienced frequently as an aura of temporal lobe epilepsy, but also reported commonly by healthy individuals. The former pathological manifestation appears to result from aberrant neural activity among brain structures within the medial temporal lobes. Recent studies also implicate medial temporal brain structures in the non-pathological experience of DV, but as one element of a diffuse neuroanatomical correlate; it remains to be seen if neural activity among the medial temporal lobes also underlies this benign manifestation. The present study set out to investigate this. Due to its unpredictable and infrequent occurrence, however, non-pathological DV does not lend itself easily to functional neuroimaging. Instead, we draw on research showing that brain structure covaries among regions that interact frequently as nodes of functional networks. Specifically, we assessed whether grey-matter covariance among structures implicated in non-pathological DV differs according to the frequency with which the phenomenon is experienced. This revealed two diverging patterns of structural covariation: Among the first, comprised primarily of medial temporal structures and the caudate, grey-matter volume becomes more positively correlated with higher frequency of DV experience. The second pattern encompasses medial and lateral temporal structures, among which greater DV frequency is associated with more negatively correlated grey matter. Using a meta-analytic method of co-activation mapping, we demonstrate a higher probability of functional interactions among brain structures constituting the former pattern, particularly during memory-related processes. Our findings suggest that altered neural signalling within memory-related medial temporal brain structures underlies both pathological and non-pathological DV.
- MeSH
- Databases as Topic MeSH
- Deja Vu * MeSH
- Adult MeSH
- Epilepsy, Temporal Lobe diagnostic imaging physiopathology psychology MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Brain Mapping methods MeSH
- Meta-Analysis as Topic MeSH
- Least-Squares Analysis MeSH
- Young Adult MeSH
- Neural Pathways diagnostic imaging physiology physiopathology MeSH
- Caudate Nucleus diagnostic imaging physiology physiopathology MeSH
- Gray Matter diagnostic imaging physiology physiopathology MeSH
- Temporal Lobe diagnostic imaging physiology physiopathology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Stručný autorský přehled výsledků stimulační terapie epilepsie v nc. dentatus, nc. caudatus a v epileptickém ložisku, je historickým dokladem využítí v léčbě technické možnosti stereotaxe a externalizovaných elektrod poprvé standardním způsobem. Byla to současně příležitost uvažovat o významu paměti v genezi epilepsie a o možnosti nových postupů v jejím léčení.
The concise overview of the results of dentate nucleus, caudate nucleus and direct epileptogenic focus stimulation provides historical evidence about the first – time use of stereotactic techniques and externalised electrodes in epilepsy patiens in standard fashion. At the same time the technique have provided the possibility to think of the role of memory in epileptogenesis and also of the new treatment approaches for epilepsy patients
The evidence for the existence of neurogenesis in the adult mammalian brain, including humans is now widely accepted. Despite the fact that adult neural stem cells appear to be very promising, a wide range of their unrevealed properties, abilities but also limitations under physiological and especially pathological conditions still need to be investigated and explained. Huntington's disease (HD) is characterized by successive degeneration of relatively well-defined neuronal population. Moreover, the most affected region, the caudate nucleus, is adjacent to the subependymal zone (SEZ) neurogenic region. Therefore, the possibility to harness the endogenous neural stem cell capacity for repairing, or at least restricting, the fatal neurodegenerative process in HD patients using promoted neurogenesis in the adult SEZ represent the exciting new possibility in clinical management of this disorder. On the other hand, many questions have to be answered before neuronal replacement therapies using endogenous precursors become a reality, particularly in relation to neurodegenerative diseases. Fundamental for all experimental, functional and future clinical studies is detailed morphological description of structures involved in the process of neurogenesis. The objectives of this review are to describe neurogenesis in the adult murine and human brain (with particular emphasis to morphological aspects of this process) and to determine to what extent it is affected in animal models of HD and in the human HD brain. Due to very limited evidence referring to the impact of striatal pathology of HD phenotype on the adult neurogenesis in the SEZ, some results gained from our studies on two rat models of HD, i.e. the neurotoxic lesion and transgenic HD rats, and on human HD brains are discussed.
- MeSH
- Cell Differentiation physiology MeSH
- Nerve Degeneration pathology MeSH
- Phenotype MeSH
- Huntington Disease pathology physiopathology therapy MeSH
- Humans MeSH
- Neural Stem Cells physiology transplantation MeSH
- Neurogenesis physiology MeSH
- Neurons pathology physiology MeSH
- Caudate Nucleus pathology physiopathology MeSH
- Cell Proliferation MeSH
- Mammals MeSH
- Animals MeSH
- Check Tag
- Humans MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
- Review MeSH
Úvod: Technika susceptibilitně vážených MR obrazů (SWI) se stala vhodným nástrojem k detekci mikro krvácení (MK) a také sledování změn signálu V bazálních gangliích. Tyto změny signálu jsou přisuzovány zvýšené koncentraci železa, ale mohou být také způsobeny kalcifikacemi nebo starším krvácením. V této studii bylo SWI použito k detekci MK a signálových změn v nucleus caudatus (NC), putamen (PUT) a globus palidus (GP) u pacientů s Alzheimerovou chorobou (AD) a mild cognitive impairement (MCI). Material a metody: Celkem bylo zhodnoceno 47 subjektů, z toho bylo 19 pacientů s AD, 16 pacientů s MCI a 12 zdravých subjektů. Vyšetření byla realizována na MR systému Siemens Trio Tim 3T a měření SWI bylo provedeno s následujícími parametry sekvence: TR = 33 ms, TE = 20 ms, sklápěcí úhel = 15°, velikost voxelu 0,6 x 0,6 x 0,8 mm, čas měření 5:20 min. Byl hodnocen relativní signál v NC, PUT a GP a v celém zobrazeném objemu mozku zaznamenán počet jednotlivých MK. Změny signálu v BG a počet MK byly korelovány s údajem MMSE. Výsledky: Z vyšetřených subjektů se alespoň 1 leze MK nalezla u 29 subjektů (62 %), více než 4 leze u 8 subjektů (17 %). U pacientů s AD se MK vyskytovaly ve vyšší míře (v průměru 1,89 leze na pacienta) než u MCI (1,38 leze na pacienta) a kontrol (0,41 leze na pacienta). Tyto rozdíly však nebyly signifikantní. Nejvyšší pokles signálu ve strukturách BG s efektem susceptibility byl zaznamenán u některých subjektů v GP, menší pak v PUT, naopak změny V NC prakticky nebyly. I v tomto případě je častější výskyt poklesu signálu u pacientů, lze ho však najít i u řady zdravých kontrol. Závěr: Oba studované jevy pomocí SWI představují jiný aspekt možného příspěvku k progresu demence typu AD (vaskulární příspěvek a de¬ gradace tkáně v BG). Změny na SWI mohou přispět do celkového morfologického skóre pacienta a vysvětlit příčinu v některých případech.
Introduction: Recently, the new technique of susceptibility weighted imaging (SWI) has become suitable tool to detect micro-bleeding (MB) an d also to follow MR signal changes in basal ganglia (BG). These signal changes are supposed to be closely connected to iron depos ition increase but they also could be caused by calcification or older hemorrhage. In this study, SWI was used to detect MB and to measure sig nal in nucleus caudatus (NC), putamen (PUT) and globus palidus in patients with Alzheimer disease (AD) and mild cognitive impairment (MCI). Material and methods: We examined 47 subjects: 19 AD patients, 16 MCI patients and 12 healthy age matched volunteers. All examinations were done on Siemens Trio 3T MR system using SWI sequence with following parameters: TR = 33 ms, TE = 20 ms, flip angle = 15°, voxel size of 0,6 × 0,6 × 0,8 mm, measurement time 5:20 min. Relative signal in NC, PUT and GP was evaluated and number of MB in entire brain vol ume was calculated. Signal changes in BG were correlated with MMSE. Results: At least one MB lesion was found in 29 subjects (62 %) and more then 4 lesions in 8 subjects (17 %). In AD patients group, MB lesions were found more frequently (1.89 lesions per subject in average) then in MCI group (1.38 lesions per subject) and control group (0.41 lesions per subject). However, these differences were not significant. The most significant signal decrease was found in GP in some subjects but almost no change of signal intensity in NC was detect ed. Also in this case, more often the low signal in GP was found in AD group but we could see this effect also in more healthy subjects. Conclusions: Both effects studied using SWI represent different aspects of possible contribution to the progression of the dementia of the AD type (vascular contribution and tissue degradation in BG). Evaluated changes in SWI can provide valuable information into the e ntire charac- terization of the clinical state of AD patients and also explain some cases.
- Keywords
- Alzheimerova choroba, MR zobrazování, susceptibilitně vážené zobrazování,
- MeSH
- Alzheimer Disease diagnosis MeSH
- Financing, Organized MeSH
- Cognition Disorders diagnosis MeSH
- Humans MeSH
- Magnetic Resonance Imaging methods instrumentation MeSH
- Caudate Nucleus pathology MeSH
- Putamen pathology MeSH
- Aged MeSH
- Check Tag
- Humans MeSH
- Aged MeSH
Akantocyty jsou atypické erytrocyty s ostrými výběžky membrány. Jako neuroakantocytózy (NA) jsou označována velmi různorodá onemocnění, u kterých neurologická symptomatika je doprovázena výskytem akantocytů. Záchyt akantocytů však není specifický a nemusí být obligátním příznakem NA. V textu jsou popsány svým výskytem nejčastější i raritní NA, jejich etiologie, klinický a laboratorní obraz. Autoři navrhují vlastní diferenciálně diagnostický postup, založený na výskytu atrofie caput nuclei caudati, který může stanovení diagnózy urychlit a zlevnit.
Acanthocytes are atypical erythrocytes with sharp membrane protrusions. The range of diseases classified as neuroacanthocytosis (NA) includes a variety of disorders characterised by the occurrence of acanthocytes together with neurological symptoms. The detection of acanthocytes is, however, not specific and may not necessarily be a symptom of NA. The text describes both the most frequent and rare forms of NA, their etiology, and clinical and laboratory picture. The authors propose their own differential diagnostic approach based on the incidence of atrophy of the caput nuclei caudati which can make diagnosis faster and cheaper.
- MeSH
- Abetalipoproteinemia diagnosis etiology MeSH
- Acanthocytes pathology MeSH
- Chorea diagnosis etiology MeSH
- Neuroacanthocytosis diagnosis etiology genetics MeSH
- Diagnostic Techniques, Neurological utilization MeSH
- Diagnosis, Differential MeSH
- Financing, Organized MeSH
- Hematologic Tests methods utilization MeSH
- Huntington Disease diagnosis etiology MeSH
- Lesch-Nyhan Syndrome diagnosis etiology MeSH
- Humans MeSH
- Magnetic Resonance Imaging methods utilization MeSH
- Neurobehavioral Manifestations MeSH
- Caudate Nucleus physiology pathology MeSH
- Blood Specimen Collection methods utilization MeSH
- Tomography, X-Ray Computed methods utilization MeSH
- Check Tag
- Humans MeSH
- Publication type
- Review MeSH
Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disease. It has been hypothesized that changes of iron content in the brain may be involved in the pathogenesis of HD. To ascertain the hypothesis, we investigated the relationship between T2 relaxation time (T2), the number of cytosine-adenine-guanine triplet repeats (CAG) and clinical status in patients suffering from HD. 34 HD patients (mean age 50.1+/-11.8 standard deviation (SD) years) and 34 control subjects (49.6+/-13.3) were scanned using a 1.5 tesla magnetic resonance (MR) scanner and the patients underwent clinical and genetic testing. A multiple echo sequence was employed for T2 measurements. T2 from healthy volunteers matched previous studies. A T2 shortening was found in the pallidum of HD patients compared to controls (65.4+/-6.4 ms vs. 71.8+/-3.6 ms, P<0.00001). A correlation between the number of CAG and T2 was found for the left pallidum (decrease in T2, P<0.05) and an inverse correlation for the left caudate (increase in T2, P<0.05). In HD patients, alterations in iron levels may be caused by an alteration in its axonal transport. The observed T2/CAG covariations may reflect changes in levels and forms of iron: this suggests that HD patients with a higher genetic load have more ferritin-bound ("safe form") iron in the pallidum and/or more low-molecular ("toxic") iron in the caudate. An increase in "toxic" iron in the caudate may enable oxidative stress and thus underlie progression of the disease.
- MeSH
- Adult MeSH
- Trinucleotide Repeat Expansion MeSH
- Financing, Organized MeSH
- Huntington Disease genetics parasitology physiopathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Caudate Nucleus metabolism pathology MeSH
- Relaxation MeSH
- Aged MeSH
- Statistics as Topic MeSH
- Case-Control Studies MeSH
- Severity of Illness Index MeSH
- Iron metabolism MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
Attention deficit hyperactivity disorder (ADHD) is a common neuropsychiatry disorder with several key symptoms, such as inattentiveness, impulsivity and hyperactivity. Neuropsychiatry studies have implicated the frontostriatal circuit in the pathological physiology of the disorder. Using magnetic resonance imaging (MRI), we examined the basal ganglia in 13 ADHD patients and eight unaffected comparison children. The volume of caudate, putamen and globus pallidus was measured. In the ADHD patients, we detected an increased left > right asymmetry of the basal ganglia. This reversal of asymmetry in the globus pallidus and caudate nucleus were statistically significant. These finding provide further evidence of morphological brain abnormalities in ADHD.
- MeSH
- Basal Ganglia abnormalities anatomy & histology pathology MeSH
- Child MeSH
- Financing, Organized MeSH
- Functional Laterality MeSH
- Attention Deficit Disorder with Hyperactivity drug therapy pathology MeSH
- Humans MeSH
- Methylphenidate therapeutic use MeSH
- Statistics, Nonparametric MeSH
- Caudate Nucleus abnormalities anatomy & histology pathology MeSH
- Reference Values MeSH
- Central Nervous System Stimulants therapeutic use MeSH
- Case-Control Studies MeSH
- Organ Size MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
