Nicolas Nicastro

Iron-Deficiency Anemia as a Rare Cause of Cerebral Venous Thrombosis and Pulmonary Embolism

Cerebral venous thrombosis (CVT) is a relatively rare cause of stroke and has a wide spectrum of unspecific symptoms, which may delay diagnosis. There are many etiologies, including hematological disorders, trauma, infection, and dehydration. Iron-deficiency anemia (IDA) has been reported as an extremely rare cause of CVT, especially in adults.

Added Value of Combined Semi-Quantitative and Visual [123I]FP-CIT SPECT Analyses for the Diagnosis of Dementia With Lewy Bodies

Purpose of the Report To assess the validity of a semi-quantitative 123I-FP-CIT SPECT method, compared to the commonly used visual analysis, in patients with probable dementia with Lewy bodies (DLB) and Alzheimer’s disease (AD). We also studied DLB specific uptake impairment pattern and correlation of uptake in the presence or absence of parkinsonism. Materials and Methods Among 1202 scans performed at our center from 2003 to 2015, we identified 93 subjects with probable DLB (mean age 76.9 ± 6.8 years, 37% women) and 18 with AD (mean age 76.9 ± 8.1 years, 50% women). Independent visual and semi-quantitative assessments based on previously established on-site reference values (including volumes-of-interest uptake, caudate-to-putamen ratio and striatal asymmetry index) were performed and compared between both groups. Results Visual staging was considered abnormal in 96.8% of DLB patients, whereas 97.8% of subjects had an abnormal semi-quantitative assessment. Combining both methods yielded a 100% sensitivity. Patients with DLB exhibited a more pronounced impairment of putaminal uptake when associated with parkinsonism, whereas a more diffuse pattern and significantly higher uptake values were observed in the subgroup of DLB patients without parkinsonism (resp. striatal uptake 1.61 ± 0.66 vs. 2.28 ± 0.52, P = 0.01). A minority of AD subjects show minimal alterations of presynaptic dopaminergic transport (striatal uptake 3.07 ± 0.41), values being always significantly higher than those from DLB patients, irrespective of the presence of parkinsonism (P < 0.0001) or not (P = 0.002). Conclusions Additional use of semi-quantitative analysis allows a higher discrimination of DLB from AD and demonstrates a specific pattern of degeneration in DLB patients according to their motor phenotype.

Discriminating among degenerative parkinsonisms using advanced 123I-ioflupane SPECT analyses

123I-ioflupane single photon emission computed tomography (SPECT) is a sensitive and well established imaging tool in Parkinsons disease (PD) and atypical parkinsonian syndromes (APS), yet a discrimination between PD and APS has been considered inconsistent at least based on visual inspection or simple region of interest analyses. We here reappraise this issue by applying advanced image analysis techniques to separate PD from the various APS. This study included 392 consecutive patients with degenerative parkinsonism undergoing 123I-ioflupane SPECT at our institution over the last decade: 306 PD, 24 multiple system atrophy (MSA), 32 progressive supranuclear palsy (PSP) and 30 corticobasal degeneration (CBD) patients. Data analysis included voxel-wise univariate statistical parametric mapping and multivariate pattern recognition using linear discriminant classifiers. MSA and PSP showed less ioflupane uptake in the head of caudate nucleus relative to PD and CBD, yet there was no difference between MSA and PSP. CBD had higher uptake in both putamen relative to PD, MSA and PSP. Classification was significant for PD versus APS (AUC 0.69, p < 0.05) and between APS subtypes (MSA vs CBD AUC 0.80, p < 0.05; MSA vs PSP AUC 0.69 p < 0.05; CBD vs PSP AUC 0.69 p < 0.05). Both striatal and extra-striatal regions contain classification information, yet the combination of both regions does not significantly improve classification accuracy. PD, MSA, PSP and CBD have distinct patterns of dopaminergic depletion on 123I-ioflupane SPECT. The high specificity of 84–90% for PD versus APS indicates that the classifier is particularly useful for confirming APS cases.

Anaplastic Medullary Ependymoma Presenting as Subarachnoid Hemorrhage

A-41-year old man presented with violent thunderclap headache and a bilateral proprioceptive sensibility deficit of the upper limbs. Cerebral CT scan and MRI were negative. Lumbar puncture confirmed subarachnoid hemorrhage (SAH), but cerebral angiography was negative. Three months later, the patient presented with paraparesis, and a thorough work-up revealed a diffuse, anaplastic extramedullary C7-D10 ependymoma with meningeal carcinomatosis considered the source of hemorrhage. The patient went through a D5-D8 laminectomy, temozolomide chemotherapy, and radiotherapy. The situation remained stable for a few months. In this paper, we would like to emphasize that spinal masses should be considered in cases of SAH with negative diagnostic findings for aneurysms or arteriovenous malformation.

Neurologic complications of sickle cell disease in Africa A systematic review and meta-analysis

Objective: To summarize prevalence data on the neurologic complications of sickle cell disease (SCD) in Africa. Methods: We searched EMBASE, PubMed, and African Index Medicus to identify all relevant articles published from inception to May 31, 2016. Each study was reviewed for methodologic quality. A random-effects model was used to estimate the prevalence of neurologic complications of SCD across studies. Results: Thirty-one studies were included. Methodologic quality was high or moderate in 90% of studies. Stroke, conditional and abnormal cerebral blood flow, seizures, and headache were the complications most frequently reported, with overall prevalence rates of 4.2%, 10.6%, 6.1%, 4.4%, and 18.9%, respectively. Some complications, like silent brain infarcts, peripheral neuropathies, neurocognitive deficits, or moyamoya disease, have been rarely or not studied at all in the African setting. Incidence data were scarce and of poor quality. Conclusions: The burden of neurologic complications of SCD is important in Africa and most likely underestimated. A better evaluation of this burden requires larger prospective studies using standard up-to-date screening methods. Accessibility to diagnostic tools such as neuroimaging, transcranial Doppler, EEG, and neuropsychological evaluation, as well as to preventive and therapeutic interventions and trained health care providers, should be improved in routine clinical practice.

Scan without evidence of dopaminergic deficit (SWEDD) in degenerative parkinsonism and dementia with Lewy bodies: A prospective study

BACKGROUND 123I-FP-CIT SPECT imaging is a reliable method to assess presynaptic dopaminergic pathways in degenerative parkinsonisms and dementia with Lewy bodies (DLB). METHODS We aimed at examining sensitivity of combined visual and semi-quantitative 123I-FP-CIT SPECT analyses in a prospective cohort of subjects with DLB and degenerative parkinsonisms - Parkinsons disease (PD), multiple system atrophy (MSA), corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP) to determine prevalence and clinical significance of scans without evidence of dopaminergic deficit (SWEDD). 372 scans performed from 2013 to 2016 with the same SPECT acquisition and processing protocol were analyzed. We identified 155 patients with degenerative parkinsonism and 53 with DLB. Diagnoses relied on validated clinical criteria for each condition. Semi-quantitative assessment was based on previously established local reference limits (including striatal volumes of interest uptake, caudate-to-putamen ratio and striatal asymmetry index). RESULTS 3/155 (2.1%) subjects with degenerative parkinsonism (1 CBS, 1 MSA-C and 1 PD) and 1/53 (1.9%) with DLB had a normal visual SPECT. Subsequent semi-quantitative analysis showed mild striatal uptake impairment for the DLB and the PD subject. Therefore, only two patients (1 CBS and 1 MSA) had a strictly normal combined assessment. CONCLUSIONS The present study shows that SWEDD cases represent a negligible proportion of patients with degenerative conditions (1.3%), when stringent diagnostic criteria are applied, a thorough follow-up is performed and visual SPECT analysis is combined with precise semi-quantitative assessment.

Transient global amnesia mimics: Transient epileptic amnesia

We describe the case of a 79-year-old patient referred for suspected transient global amnesia, after an episode of anterograde amnesia which lasted 90 min. An EEG, performed after the episode, showed bilateral temporal electrographic seizures, orienting the diagnosis toward a transient epileptic amnesia. Transient epileptic amnesia is defined by temporal lobe epilepsy characterized by recurrent transient amnestic episodes of 30–90 min in duration, sometimes associated with olfactory hallucinations or oral automatisms. Response to antiepileptic drugs is excellent. We would like to raise awareness toward this epileptic amnesia when facing atypical or recurrent transient amnestic episodes.

Neurological complications of sickle cell disease in Africa: protocol for a systematic review

Introduction Sickle cell disease (SCD) is highly prevalent in Africa. Considered as a public health problem, it is associated with high morbidity and mortality. Neurological complications of SCD can cause significant disability with important socioeconomic and psychological impact on the patients and their families, and can even lead to death if not properly managed. There are important knowledge gaps regarding the burden of neurological complications of SCD in African populations. We propose to conduct the first systematic review to summarise the epidemiological data available on neurological complications of SCD in Africa. Methods and analysis We will search PubMed, MEDLINE, EMBASE and the African Index Medicus from 1 January 1950 to 31 May 2016 for studies of neurological complications of SCD in Africa. After study selection, full-text paper acquisition, data extraction and synthesis, we will assess all studies for quality, risk of bias and heterogeneity. Appropriate methods of meta-analysis will be used to pool prevalence estimates from studies with similar features, globally and in major subgroups. This protocol complies with the 2015 Preferred Reporting Items for Systematic reviews and Meta-Analysis protocols (PRISMA-P) guidelines. Ethics and dissemination The proposed study will use published data. Therefore, there is no requirement for ethical approval. This review is expected to provide relevant data to help quantify the burden of neurological complications of SCD in African populations, inform policymakers and identify further research topics. The final report of the systematic review will be published in a peer-reviewed journal and presented at conferences. Review registration number CRD42016039574.

Pseudobulbar palsy due to deep-brain stimulation of the thalamic ventral intermediate nuclei

Essential tremor is the most common tremor disorder and s characterized by a symmetric postural and kinetic tremor of he upper extremities, with possible implication of head, lower imbs and voice. This condition can be associated with marked mpairment and medications (mainly beta-blockers and primione) achieve moderate improvement. Since the late 1990s, ventral ntermediate nucleus of the thalamus (Vim) deep-brain stimulation as largely replaced thalamotomy as the intervention of choice for rug-resistant disabling essential tremor.

The role of molecular imaging in assessing degenerative parkinsonism – an updated review

Diagnosing degenerative forms of parkinsonism still relies on a thorough clinical assessment, which in Parkinsons disease involves the presence of an asymmetric bradykinesia with rest tremor and/or rigidity that respond substantially to levodopa. Conversely, atypical forms, including multiple system atrophy, progressive supranuclear palsy and corticobasal degeneration, exhibit additional features (cerebellar or pyramidal signs, early postural instability), a poor response to dopamine replacement therapy and a bad prognosis. Consensus diagnostic criteria have excellent specificity, but lack sensitivity, and a clear diagnosis solely based on clinical evaluation is not always accurate, hence the need for diagnostic biomarkers. Nuclear medicine imaging is definitely one of them, allowing a qualitative and quantitative evaluation of in vivo functional integrity of monoaminergic (e.g., dopaminergic) pathways, brain metabolism and protein deposition and representing a unique window into these complex diseases. It has proved useful for early and accurate diagnosis, and possibly represents a valid biomarker of disease pathogenesis, progression and response to neuroprotective therapies. This review focuses on the nigrostriatal pathway dysfunctions (demonstrated with presynaptic dopamine positron emission tomography [PET] and single photon emission computed tomography [SPECT] ligands) that confirm a degenerative form of parkinsonism. In addition, 123I-metaiodobenzylguanidine cardiac scintigraphy can unveil postganglionic autonomic failure specifically encountered in Parkinsons disease. Brain 18F-fluorodeoxyglucose PET may also show a distinct hypometabolism for each degenerative form of parkinsonism. Since a few years ago, the proteins that aggregate in the brain of subjects with neurodegenerative diseases (tau and alpha-synuclein) can be evaluated in vivo by novel radioligands. These developments open new perspectives both as diagnostic tools and to understand the regional topography and burden of protein deposition on motor impairment and cognitive decline. The last part of the review proposes a strategic workup in the practical evaluation of a patient with parkinsonism.