Giulia Turri

123I-FP-CIT SPECT in the differential diagnosis between dementia with Lewy bodies and other dementias

AIM To systematically review the utility of dopamine system imaging using 123I-FP-CIT SPECT in the differential diagnosis between dementia with Lewy bodies (DLB) and other dementia syndromes. METHODS We searched MEDLINE, CENTRAL and ClinicalTrials.gov to identify studies reporting enough data to determine accuracy measure of 123I-FP-CIT SPECT in differentiating between DLB and other dementia syndromes. Studies including patients with Parkinsons disease or other parkinsonisms associated with abnormal DAT imaging were excluded. The methodological quality of studies was evaluated with QUADAS-2. RESULTS Eight studies were included. Studies adopting a clinical diagnosis as a reference standard showed sensitivity and specificity values of DaTSCAN in differentiating between DLB and non-DLB dementia syndromes (all subtypes, AD and FTD) consistently higher than 80%, both considering a visual and a semiquantitative analysis. The meta-analyses from the three studies using a neuropathological reference standard yielded sensitivity and specificity values higher than those adopting a clinical diagnostic reference. CONCLUSIONS 123I-FP-CIT SPECT can represent an accurate method to differentiate between DLB and other dementia syndromes. However, most data in the literature derive from studies adopting the clinical diagnosis as the reference diagnostic standard and which are therefore intrinsically unable to demonstrate an accuracy of DAT imaging above that of careful clinical diagnosis alone. The very few studies providing information on the neuropathologic correlation for the DaTSCAN findings show however high sensitivity and specificity values, suggesting that SPECT scan is more accurate than clinical diagnosis and may prove useful in supporting the clinical diagnosis of DLB.

Psychogenic nonepileptic seizures and movement disorders A comparative review

PURPOSE OF REVIEW Neurologic symptoms due to a psychogenic cause are frequently seen in clinical practice. Psychogenic nonepileptic seizures (PNES) and psychogenic movement disorders (PMD) are among the most common psychogenic neurologic disorders. PNES and PMD are usually investigated and managed separately by different neurology subspecialists. We review the main epidemiologic and clinical features of both PNES and PMD, aiming to highlight their similarities and differences and to see whether a common framework for these disorders exists. RECENT FINDINGS Data from the literature show that there is a profound overlap between PNES and PMD, which would argue for a larger unifying pathophysiology with variable phenotypic manifestations. SUMMARY Collaborative and integrated research among epileptologists, movement disorders experts, psychiatrists, psychologists, and physiotherapists may increase our collective knowledge about the pathophysiologic mechanisms of PNES and PMD and therefore improve outcomes for these patients.

Late-onset multiple sclerosis presenting with cognitive dysfunction and severe cortical/infratentorial atrophy

Objective: Although cognitive dysfunction is a relevant aspect of multiple sclerosis (MS) from the earliest disease phase, cognitive onset is unusual thus jeopardizing early and accurate diagnosis. Here we describe 12 patients presenting with cognitive dysfunction as primary manifestation of MS with either mild or no impairment in non-cognitive neurological domains. Methods: Twelve patients with cognitive onset who were subsequently diagnosed with MS (CI-MS) were included in this retrospective study. Twelve cognitively normal MS patients (CN-MS), 12 healthy controls and four patients having progressive supranuclear palsy (PSP) served as the reference population. Results: Ten CI-MS patients had progressive clinical course and all patients had late disease onset (median age = 49 years; range = 40–58 years). Among cognitive functions, frontal domains were the most involved. Compared to CN-MS and healthy controls, significant cortical and infratentorial atrophy characterized CI-MS patients. Selective atrophy of midbrain tegmentum with relative sparing of pons, known as “The Hummingbird sign,” was observed in eight CI-MS and in three PSP patients. Discussion: Our observation suggests that MS diagnosis should be taken into consideration in case of cognitive dysfunction, particularly when associated with slowly progressive disease course and severe cortical, cerebellar and brainstem atrophy even in the absence of other major neurological symptoms and signs.

Unusual Case of Solitary Intraparenchymal Brain Plasmacytoma

Case Report A 50-year-old, previously healthy woman was referred to the emergency unit of our institution for sudden motor aphasia resolving spontaneously in 15 minutes. Magnetic resonance imaging (MRI) of the brain showed infiltrative contrast-enhancing tissue in the left temporal-insular lobe and thalamus, with surrounding edema and a mild axial median shift (Figs 1A and 1B). The tissue also infiltrated the corpus callosum with initial diffusion to the white matter of the right hemisphere. No dura mater or skull involvement was documented. MRI spectroscopy confirmed the presence of infiltrating lymphomalike tissue. Electroencephalography showed nonspecific wave slowing in the left frontotemporal region.

Association of prolonged QTc interval with Takotsubo cardiomyopathy: A neurocardiac syndrome inside the mystery of the insula of Reil

The Takotsubo cardiomyopathy is often considered autochthonous to the heart, although the primary problem may be not in the heart muscle itself. Instead, similar to several Takotsubo‐like cardiac pathologies seen in acute neurological diseases, it may reflect the capacity of the nervous system to injure the heart. Persuasive evidence exists that shocking emotional stress promotes direct heart injuries. Moreover, clinical and laboratory research shows that cardiac structural damage can occur in the presence of a normal heart, especially in the context of seizures, stroke, and traumatic brain injury or under conditions of psychological stress. The aim of this review is to summarize the clinical implications of these observations, several of which focus on the pivotal role of the insula of Reil in the brain‐heart connection, to unravel the mystery of Takotsubo cardiomyopathy pathogenesis.

QTc interval in patients with multiple sclerosis: an inference from the insula of Reil?

The aim of this study was to investigate the correlation between the duration of the QTc interval and the brain lesion load at the level of the structures involved in superior autonomic control (insula, cingulate cortex and amygdala‐hippocampus) in multiple sclerosis (MS) patients.

Unilateral fixed mydriasis: an uncommon presentation of temporal lobe epilepsy.

Unilateral mydriasis can represent a diagnostic challenge and it may carry ominous significance when associated with other signs or symptoms. Third cranial nerve compression should be excluded in cases of unilateral mydriasis with no light response [1, 2]. Very rarely, focal seizures may cause unilateral fixed mydriasis [3–6]. A 47-year-old woman had transient loss of consciousness. She denied previous medical problems. At admission, physical examination showed mild left hemiparesis. Brain computed tomography (CT) scan showed right fronto-basal hypodensity (Fig. 1a). The day after, she suddenly developed headache worsening, nausea and dizziness. Physical examination confirmed mild left hemiparesis and showed the appearance of right unilateral mydriasis which was unresponsive to light and waxed and waned, in that longlasting periods of right fixed mydriasis alternated with periods of symmetric pupils and normal bilateral response to light. Mydriasis was associated with some excessive sweating and facial flushing. Eye exposure to drugs and intraocular diseases were excluded by a neuro-ophthalmologist. Brain CT angiography, digital angiography and lumbar puncture ruled out intracranial aneurysm and subarachnoid haemorrhage. The following night, she developed seizures which started with fixed right mydriasis and left-sided clonic jerks followed by secondary generalization; seizures recurred on the following three days and sodium valproate was started. In this period, she presented some episodes of right unilateral mydriasis unresponsive to light. Mydriasis and left hemiparesis disappeared when seizures stopped. Brain magnetic resonance imaging (MRI) confirmed the right fronto-basal lesion with normal diffusion-weighted sequences, suggesting encephalomalacia (Fig. 1b). Ictal electroencephalography (EEG) was not recorded. Interictal EEG was normal on two occasions, during an episode of right fixed mydriasis, and after sleep deprivation. She underwent 256-channel densearray EEG (dEEG, Electrical Geodesics Inc., Eugene, Oregon, USA), which showed spikes on zygomatic-temporal electrodes and localized the spike focus in the right inferior temporal gyrus (Fig. 1c, d). At eight-month followup, seizures and mydriasis disappeared and the patient was well. The patient gave informed consent for case report. Unilateral fixed mydriasis without extraocular motility deficit is rare. The parasympathetic fibers, which cause pupil constriction, lie in the most peripheral part of the third cranial nerve and, because of their superficial location, are the most susceptible to compression [1]. Because of its close position to the superior cerebellar and the posterior cerebral arteries, the third cranial nerve may be compressed by intracranial aneurysms, especially those arising from the posterior communicating to internal carotid artery junction [2]. In our patient, right fixed mydriasis, hemiparesis and headache suggested subarachnoid haemorrhage due to ruptured intracranial aneurysm compressing the right third cranial nerve, but investigations excluded this hypothesis. Tonic Adie pupil [1, 2] was excluded because light-near dissociation and segmental sphincter palsy were absent and deep tendon reflexes were preserved. Opthalmoplegia associated with migraine or cluster headache [2] was excluded because of no previous S. Tamburin (&) G. Turri P. Kuhdari A. Fiaschi P. Manganotti Department of Neurological, Neuropsychological, Morphological and Movement Sciences, G.B. Rossi Hospital, University of Verona, Piazzale Scuro 10, 37134 Verona, Italy e-mail: stefano.tamburin@univr.it

Postpartum headache: A prospective study

AIMS To prospectively assess the incidence and etiology (ie, primary vs symptomatic) of headache in women during the first month postdelivery, with particular emphasis on the type of presentation as a clue for identifying potentially harmful etiologies. A secondary aim was to evaluate the relative frequency of migraine- vs tension-type headache in cases of primary headache. METHODS A total of 900 consecutive women were enrolled in the study and examined within 3 days of delivery, both clinically and with transcranial color-coded sonography (TCCS). During the course of follow-up, all subjects presenting with headache suspected of being secondary to intracranial pathology underwent a complete clinical and instrumental assessment with TCCS and magnetic resonance imaging (MRI) and angiography. A telephone interview was administered to all subjects 1 month after delivery. Two-tailed t test, Mann-Whitney test, Pearson chi-square test, and multiple logistic regression were used to analyze the data. RESULTS At the end of the follow-up period, 241 women (26.8% of the sample) reported at least one headache attack. In 88 of these 241 cases (9.8%), the headache attack occurred soon after delivery and was already recorded at the first visit. Thunderclap headache occurred in 34 (3.8%) of the subjects. In all but one of these subjects, the course was spontaneously benign. None of the recorded variables allowed discrimination of the subjects with thunderclap headache from those without headache. Three subjects had thunderclap headache following dural anesthesia, and one subject was found to have reversible cerebral vasoconstriction syndrome. Headache with gradual onset was recorded in 207 subjects (23%). Three of these subjects fulfilled the criteria for pre-eclampsia, and 13 had postural headache after dural anesthesia. Migraine history and urinary protein were independent predictors of gradual onset headache, and migraine history and parity were significant independent predictors of pulsating pain with gradual onset headache. CONCLUSION Headache appeared early in the first days postdelivery, and its incidence increased in the first month thereafter. Predictors were different according to whether the headache had a gradual onset or a thunderclap presentation. Primary headache accounted for the overwhelming majority of the recorded cases.

Clinical outcomes of secondary prevention strategies for young patients with cryptogenic stroke and patent foramen ovale

Abstract Background The aim of this study was to compare the immediate and long-term clinical outcomes of medical therapy and percutaneous patent foramen ovale (PFO) closure as secondary prevention strategies in patients younger than 55 years of age presenting with cryptogenic stroke and PFO. Methods Between January 2006 and April 2015, all patients with the diagnosis of cryptogenic stroke and PFO were analysed and prospectively followed. Stroke was confirmed in 159 out of 309 patients (51%). In the remaining cases, other neurological conditions were found and therefore excluded from further analysis. Patients received PFO closure or medical therapy on the basis of a pre-specified algorithm. Primary outcome was the assessment of recurrent ischaemic events at follow-up. Results Percutaneous PFO closure was performed in 77 patients (48%) and 82 (52%) were treated medically. Mean follow-up was 51.6 ± 34.8 months. Two ischaemic strokes occurred in the medical group only (2.4% vs 0%; P = 0.16) and no complications related to the invasive procedure were observed. Conclusions The diagnosis of stroke in patients with PFO could be confirmed in 50% of cases only, underlining the importance of a multidisciplinary evaluation of these patients. A very low ischaemic recurrence rate was observed in the medical therapy group, suggesting that a personalized treatment based on a prespecified diagnostic algorithm yields good clinical results irrespective of the treatment modality. Given the low number of recurrences, larger cohorts may be needed to prove significant differences.

The dispersion of myocardial repolarization in ischemic stroke and intracranial hemorrhage

BACKGROUND Markers of dispersion of myocardial repolarization have been proposed to identify the patients at higher risk of malignant arrhythmic events. The aim of the present study is to assess a possible association of the electrocardiografic (ECG) markers of the dispersion of repolarization with the type of stroke, involvement of insula, neurological severity (National Institutes of Health Stroke Scale, NIHSS score), and disability (modified Rankin Scale, mRS score) in patients with a cerebrovascular event. METHODS We conducted a retrospective analysis based on data prospectively collected from consecutive patients with a cerebrovascular event who underwent 12‑lead ECG at admission to the Verona Stroke Unit. RESULTS Of the 63 patients included in the study, 55 had ischemic stroke and 8 intracranial hemorrhage. TpTe (time between the peak and the end of the T wave) and TpTe/QTc (TpTe/corrected time between the start of the Q wave and the end of the T wave) in lead V5 were higher in intracranial hemorrhage than in ischemic stroke (p = 0.03 and p = 0.04, respectively) and QT max (the longest QT calculated in the 12 leads) was higher in patients with involvement of insula (p ≤ 0.01). A correlation was found between QTc max and NIHSS score at admission (p = 0.02), QT max and NIHSS score at discharge (p = 0.05), and QT max and mRS score at discharge (p = 0.02). CONCLUSIONS TpTe and TpTe/QTc in V5 lead were associated with intracranial hemorrhage and QT max was associated with involvement of insula. The prolongation of QT was correlated with neurological severity and disability.