Cerebral-perfusion-based single-photon emission computed tomography (SPECT) staging using NeuroGam® in patients with moyamoya disease

Background and purpose

Cerebral angiography (CA) is the gold standard for moyamoya disease (MMD) staging and diagnosis, but CA findings are not well correlated with clinical symptoms. The purpose of this study was to establish novel cerebral-perfusion-based staging for MMD that is well correlated with clinical symptoms.

Materials and methods

From 2010 to 2015, regional cerebrovascular reserve (rCVR) was examined by single-photon emission computed tomography (SPECT) using NeuroGam® (Segamicorp, Houston, TX, USA) in 30 patients (17 women, 13 men; 60 hemispheres; mean 42.0 years old [range 5–60 years old]) with MMD, which was diagnosed by CA and magnetic resonance angiography (MRA). Brain CT or brain magnetic resonance imaging (MRI) was used to evaluate neurological conditions such as transient ischemic attack (TIA), cerebral hemorrhage, and cerebral infarction. A novel staging system for MMD was developed by combining findings from CA, MRI, and SPECT with NeuroGam®.

Results

Our novel staging system was strongly associated with clinical symptoms. Twenty-two hemispheres out of 60 were categorized as stage I, 24 hemispheres were categorized as stage II, and 14 hemispheres were categorized as stage III. Hemispheres with higher scores exhibited a higher incidence of clinical symptoms. These findings indicate that cerebral-perfusion-based staging is predictive of MMD clinical symptoms.

Conclusion

Perfusion-based SPECT staging correlates well with clinical symptoms and may be a reliable alternative to the Suzuki staging by CA.

     

Cerebellar contribution to spatial event processing: do spatial procedures contribute to formation of spatial declarative knowledge?

Spatial knowledge of an environment involves two distinct competencies: declarative spatial knowledge, linked to where environmental cues are and where the subject is with respect to the cues, and, at the same time, procedural spatial knowledge, linked to how to move into the environment. It has been previously demonstrated that hemicerebellectomized (HCbed) rats are impaired in developing efficient exploration strategies, but not in building spatial maps or in utilizing localizing cues. The aim of the present study was to analyse the relationships between spatial procedural and declarative knowledge by using the open field test. HCbed rats have been tested in two different protocols of the open field task. The results indicate that HCbed animals succeeded in moving inside the arena, in contacting the objects and in habituating to the new environment. However, HCbed animals did not react to environmental changes, when their impaired explorative pattern was inappropriate to the environment, suggesting that they were not able to represent a new environment because they were not able to explore it appropriately. Nevertheless, when their altered procedures were favoured by object arrangement, they detected environmental changes as efficiently as did normal rats. This finding suggests that no declarative spatial learning is possible without appropriate procedural spatial learning.     

How could Theory of Mind contribute to the differentiation of social adjustment profiles of children with externalizing behavior disorders and children with intellectual disabilities?

This study compared Theory of Mind (ToM) emotion and belief abilities in 43 children with externalized behavior (EB) disorders presenting low intelligence, 40 children with intellectual disabilities (ID) and 33 typically developing (TD) preschoolers (as a control group), matched for developmental age. The links between their ToM abilities, their level in seven self-regulation strategies as displayed in social problem-solving tasks and their social adjustment profiles (assessed by the Social Competence and Behavior Evaluation, completed by their teachers) were examined. Children with EB presented lower comprehension of causes of emotions and lower self-regulation of joint attention and of attention than children with ID and TD children. In comparison with TD children, lower social adjustment was observed in nearly all dimensions of profiles in both atypical groups. Specifically, children with EB were significantly angrier than children with ID. Although variable patterns of positive correlations were obtained in atypical groups between self-regulation strategies and ToM abilities, the most numerous positive links were obtained in the group with EB. Regression analyses showed that developmental age predicted ToM abilities and certain dimensions of social adjustment profiles in atypical groups. In the ID group, ToM emotions predicted general adaptation, affective adaptation, interactions with peers and with adults and low internalizing problems. In the EB group, general adaptation was predicted by ToM emotions and self-regulation, interactions with peers by ToM beliefs, and a low level of externalizing problems by ToM emotions. Some implications for intervention and perspectives for research are suggested.     

Elementary school epilepsy survey (ESES): A new measure of elementary school students’ knowledge and attitudes about epilepsy

ObjectiveNo peer-reviewed, published, psychometrically tested scales are available to assess elementary school students’ general knowledge and attitudes about epilepsy. Such a scale is needed for evaluation of the effectiveness of classroom education programs. The purpose of this work was to develop and validate a brief, reliable scale for grades 4–6 to assess students’ knowledge and attitudes about epilepsy and persons diagnosed with epilepsy.MethodsDevelopment of the 22-item Elementary School Epilepsy Survey (ESES) followed standard protocol for scale development. It includes a 12-item Knowledge subscale and a 10-item Attitudes subscale. The ESES was administered during regular classroom time given and repeated 1 week later. No educational intervention took place.ResultsMean age of the 155 students was 11 years (range 9.8–13.9): grade 4, 56 students; grade 5, 36; and grade 6, 63 students. The ESES Total scale and Attitudes subscale had good internal consistency (Cronbach’s α = 0.72 and 0.81, respectively). As expected, the Knowledge subscale had low internal reliability (Cronbach’s α = 0.50). Test–retest scores indicated good reliability and strong discriminant validity, with significant increases noted in all ESES scores with increasing age and in those who knew someone with epilepsy.ConclusionThe ESES detects developmental and experiential trends in students’ knowledge and attitude about epilepsy. It has good internal consistency and test–retest reliability.     

“Atypical forms” of benign epilepsy with centrotemporal spikes (BECTS): How to diagnose and guide these children. A practical/scientific approach

Benign epilepsy with centrotemporal spikes (BECTS) epilepsy, also known as rolandic epilepsy, is the most common childhood type of epilepsy. There is debate on its “benign” definition given the numerous literature data on its correlation to cognitive morbidity. Although its prognosis is often favorable, BECTS can present or evolve however to an atypical form, characterized by a worse prognosis and negative impact on cognitive development. It is possible that abnormal electrical activity, marker of neurological dysfunction, has the potential to disrupt neural network function and development. Numerous studies tried to identify clinical or electroencephalographic criteria for atypical forms and atypical evolution of BECTS in order to guide follow-up and treatment of patients and to predict their outcome. This review provides a compact summery of literature data with a focus on predictive features of future cognitive decline.     

Episodic vertigo related to migraine (90 cases): vestibular migraine?

A retrospective study was conducted on 90 patients with episodic vertigo that could be related to migraine as the most probable pathomechanism. Since the majority of the patients did not fulfill the criteria of the International Headache Society (IHS) for basilar migraine, the diagnosis was substantiated by disease course, medical efficacy in treating (ergotamines) and preventing (metoprolol, flunarizine) attacks, ocular motor abnormalities in the symptom-free interval, and careful exclusion of the most relevant differential diagnoses, such as transient ischemic attacks, Menière’s disease, and vestibular paroxysmia. The following clinical features were elaborated. The initial manifestation could occur at any time throughout life, with a peak in the fourth decade in men and a “plateau” between the third and fifth decades in women. The duration of rotational (78%) and/or to-and-fro vertigo (38%) could last from a few seconds to several hours or, less frequently, even days; duration of a few minutes or of several hours was most frequent. Monosymptomatic audiovestibular attacks (78%) occurred as vertigo associated with auditory symptoms in only 16%. Vertigo was not associated with headache in 32% of the patients. In the symptom-free interval 66% of the patients showed mild central ocular motor signs such as vertical (48%) and/or horizontal (22%) saccadic pursuit, gaze-evoked nystagmus (27%), moderate positional nystagmus (11%), and spontaneous nystagmus (11%). Combinations with other forms of migraine were found in 52%. Thus, migraine is a relevant differential diagnosis for episodic vertigo. According to the criteria of the IHS, only 7.8% of these patients would be diagnosed as having basilar migraine. However, to ensure that at least those presenting with monosymptomatic episodic vertigo (78% in our study) receive effective treatment, we propose the use of the more appropriate term “vestibular migraine.” Received: 22 September 1998 Received in revised form: 3 March 1999 Accepted: 16 March 1999     

Which electroencephalography (EEG) for epilepsy? The relative usefulness of different EEG protocols in patients with possible epilepsy

Background and aim

Electroencephalography (EEG) is an essential investigative tool for use in young people with epilepsy. This study assesses the effects of different EEG protocols on the yield of EEG abnormalities in young people with possible new epilepsy.

Methods

85 patients presenting to the unit underwent three EEGs with differing protocols: routine EEG (r‐EEG), sleep‐deprived EEG (SD‐EEG), EEG carried out during drug‐induced sleep (DI‐EEG). The yield of EEG abnormalities was compared using each EEG protocol.

Results

98 patients were recruited to the study. Of the 85 patients who completed the study, 33 (39%) showed no discernible abnormality on any of their EEG recordings. 36 patients (43%) showed generalised spike and wave during at least one EEG recording, whereas 15 (18%) had a focal discharge evident at some stage. SD‐EEG had a sensitivity of 92% among these patients, whereas the sensitivity of DI‐EEG and r‐EEG was 58% and 44%, respectively. The difference between the yield from SD‐EEG was significantly higher than that from other protocols (p<0.001). Among the 15 patients showing focal discharges, SD‐EEG provoked abnormalities in 11 (73%). r‐EEG and DI‐EEG each produced abnormalities in 40% and 27%, respectively. 7 patients (47%) had changes seen only after sleep deprivation. In 2 (13%), the only abnormalities were seen on r‐EEG. In only 1 patient with focal discharges (7%) was the focal change noted solely after drug‐induced sleep. These differences did not reach significance.

Conclusion

EEG has an important role in the classification of epilepsies. SD‐EEG is an easy and inexpensive way of increasing the yield of EEG abnormalities. Using this as the preferred protocol may help reduce the numbers of EEGs carried out in young patients presenting with epilepsy.Electroencephalography (EEG) is an essential investigative tool for use in young people with epilepsy.^1,2,3,4,5 The clinical onset of the idiopathic generalised epilepsies (IGEs) is most common in adolescence and early adulthood, and this is when EEG is most valuable.⁵ Differentiation of IGE from partial epilepsy should be done as early as possible, because of the important implications it will have on treatment choice,⁶ planned duration of treatment⁶ and prognosis.⁷ Despite its utility, EEG is not always easily available in many parts of the UK. Clinical targeting is desirable to allow efficient use of this resource.⁸The effect of sleep deprivation on EEG has long been recognised.^2,3,9 For reasons that are unclear, the incidence of epileptiform abnormalities on EEG is increased by sleep deprivation. Some authors feel that this effect leads to an enhanced yield of epileptiform abnormalities even when compared with routine EEG (r‐EEG) that includes a period of sleep,³ although one study did not support the special effect of sleep deprivation.⁴Although the role of the EEG in early epilepsy is widely recognised in this age group, there are few data on the relative sensitivity of EEG protocols in detecting epileptiform changes. One study⁹ looked at the incidence and frequency of epileptiform abnormalities after EEG with drug‐induced sleep (DI‐EEG) and EEG after sleep deprivation (SD‐EEG). This cohort consisted largely of patients already on treatment for localisation‐related epilepsies.Carpay et al¹⁰ repeated SD‐EEG in a cohort of younger people with normal r‐EEG and found that 34% of them showed various epileptiform abnormalities. The age range and selection criteria limit the applicability of these data. An earlier study¹¹ had carried out SD‐EEG in 114 patients who had a previously normal r‐EEG, some of whom were receiving drugs for epilepsy. Despite this, 47 patients exhibited some clearly ictal activity, but this selection precluded direct comparison of elicited epileptiform changes in different protocols.In young adults with newly diagnosed epilepsies, the role of provocative testing (either SD‐EEG or DI‐EEG) remains unclear, as the relative yield of generalised discharges in each protocol has yet to be properly compared. In young adults with newly diagnosed epilepsy, which EEG protocol is best? Should SD‐EEG be the preferred protocol? Is DI‐EEG as sensitive or specific as SD‐EEG in detecting abnormalities?     

123I]N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)nortropane single-photon emission computed tomography brain imaging in the diagnosis of dementia with Lewy bodies

Early, accurate diagnosis of dementia with Lewy bodies (DLB), in particular its differentiation from Alzheimer's disease, is important for optimal management, providing patients/carers with information about the likely symptomatology and illness course, allowing initiation of effective pharmacotherapy, and avoiding the consequences of neuroleptic sensitivity. Clinical diagnosis of DLB has high specificity but low sensitivity. Clinical trials of [(123)I]N-ω-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl)nortropane single-photon emission computed tomography ([(123)I]FP-CIT SPECT) indicate high positive and negative percent agreement with reference to clinical diagnosis, and high sensitivity and specificity in patients with neuropathologically confirmed diagnoses of DLB. An abnormal [(123)I]FP-CIT SPECT image in patients fulfilling criteria for possible DLB advances the certainty of a diagnosis to probable DLB. [(123)I]FP-CIT SPECT, by identifying the striatal dopaminergic deficit, can be a valuable diagnostic aid and can provide support to a clinical diagnosis of DLB in patients with dementia. The technique is likely to be of particular utility in patients with dementia with an uncertain diagnosis.     

Endogenous dopamine (DA) competes with the binding of a radiolabeled D₃ receptor partial agonist in vivo: a positron emission tomography study

A series of microPET imaging studies were conducted in anesthetized rhesus monkeys using the dopamine D₃‐selective partial agonist, [¹⁸F] 5 . There was variable uptake in regions of brain known to express a high density of D₃ receptors under baseline conditions. Pretreatment with lorazepam (1 mg/kg, i.v. 30 min) to reduce endogenous dopamine activity before tracer injection resulted in a dramatic increase in uptake in the caudate, putamen, and thalamus, and an increase in the binding potential (BP) values, a measure of D₃ receptor binding in vivo. These data indicate that there is a high level of competition between [¹⁸F] 5 and endogenous dopamine for D₃ receptors in vivo. Synapse 2011. © 2011 Wiley‐Liss, Inc.     

Galloway–Mowat syndrome: An early-onset progressive encephalopathy with intractable epilepsy associated to renal impairment. Two novel cases and review of literature

Galloway–Mowat Syndrome (GMS) is an autosomal recessively inherited condition which manifests with severe encephalopathy, featuring microcephaly, developmental delay, and early-onset intractable epilepsy. Patients typically show also renal involvement from the onset. We report two siblings with GMS presenting with early-onset, intractable epilepsy and neurological deterioration, later followed by renal impairment. In both patients intractable epilepsy started during the first months of life and included a combination of spasms, focal and myoclonic/atonic seizures, along with psychomotor retardation and dysmorphic features. One of the patient died from fulminating renal failure at age 6 years. The other patient developed only isolated proteinuria from the age 3 years. Our cases differ from ‘classic’ GMS, as manifested the clinical and laboratory features of renal involvement only some years later the onset of epilepsy and neurological symptoms. Therefore, the diagnosis of GMS should be considered in infants with intractable epilepsy, encephalopathy, and multiple neurological deficits, also in absence of renal manifestations. The literature data about the electroclinical features of epilepsy in GMS are also reviewed.     

Acquisition of human immunodeficiency virus infection in a patient with multiple sclerosis: could these conditions positively influence each other’s course?

Journal of NeuroVirology - Patients with human immunodeficiency virus (HIV) infection have a decreased risk of developing multiple sclerosis (MS) and MS patients very rarely contract HIV infection....     

Perspectives of an innovative ophthalmological technology: Optical coherence tomography (OCT) – What should be of interest to the neurologist?

Ocular coherence tomography has revolutionised the prospects of measuring the loss of retinal ganglion cells secondary to degenerative diseases and monitoring time-dependent changes of optic disc morphology, since the resolution has been improved considerably and the time required has been reduced. Although the non-invasive technique promises a high inter-session reproducibility, the limitations of retinal imaging and the problems of segmenting of the retinal layers have to be taken into account. While the first studies were limited to single sessions in small groups, further trials will elucidate how the retinal nerve fibre layer (RNFL) is altered in the course of different episodic forms of multiple sclerosis. This review points out that the examination technique already provides comprehensive information, valuable in the daily care of neurological patients.     

Can neurological soft signs (NSS) contribute to the prediction of dangerousness in mentally disordered offenders?

The prediction of dangerousness in mentally disordered offenders is still as well complex as insufficiently empirically proven. So far more or less valid criteria for predicting recidivism have been determined concerning historical and clinical aspects. Whereas biological, neurological and neuropsychological variables that could be of importance for the development of chronic delinquent behavior have mostly been neglected. This article deals with the relevance of neurological soft signs (NSS) for the prediction of dangerousness in mentally ill offenders as one possible factor in a multidimensional model. 155 patients were examined after a minimum time at risk of 2 years (mean 4 years). The results showed that on the one hand there is no relevant and valid connection between neurological soft signs and recidivism for the general heterogeneous group of forensic patients. On the other hand similar results arose for the subgroup of patients with a personality disorder without intellectual deficits as for the homogeneous group of offenders in prisons. Therefore a significant meaning of NSS for certain subgroups can be presumed.