Sensitivity of CT angiography,T2-weighted MRI,and magnetic resonance angiography in detecting cerebral arteriovenous malformations and associated aneurysms

An increasing number of patients with arteriovenous malformations (AVM) are being screened with expedient, safe, and non-invasive methods such as computed tomography angiography (CTA), MRI, and magnetic resonance angiography (MRA). Only a paucity of small series have examined the sensitivity of these modalities in detecting AVMs. In this study, we reviewed 125 patients with AVMs, demonstrating overall sensitivities of 90% for CTA, 89% for T2-weighted MRI, and 74% for MRA. The greater sensitivity of CTA in detecting AVMs compared to MRA did meet statistical significance (p_{CTA, MRA} = 0.036). For unruptured AVMs, the sensitivities were 96%, 97% and 71% (p_{CTA, MRA} = 0.043) and for ruptured AVMs, they were 87%, 83% and 77%, respectively. The sensitivity was 100% for both CTA and T2-weighted MRI for AVMs larger than 3 cm. Importantly, associated aneurysms were best detected via CTA, with a sensitivity of 88%, as compared to 29% for MRI and 27% for MRA (p_{CTA, MRA} = 1.2 × 10^?6), with the greatest distinction occurring within the subgroup of intranidal aneurysms with a sensitivity of 83% for CTA compared to 0% for both MRI and MRA (p_{CTA, MRA} = 0.005).     

Diagnostic accuracy of flair in detection of acute subarachnoid hemorrhage in patients presenting with severe headache

BackgroundFluid attenuation inversion recovery (FLAIR) magnetic resonance imaging (MRI) sequences are previously described for the evaluation of acute subarachnoid hemorrhage (SAH) and demonstrated good sensitivity. This study was designed to find the diagnostic accuracy of FLAIR in detection of acute SAH in patients presenting with severe headache considering the fact that controversy has been observed in previous studies.ObjectiveTo determine diagnostic accuracy of FLAIR in detection of acute subarachnoid hemorrhage in patients presenting with severe headache using lumber puncture as gold standard.MethodologyA total of 245 patients fulfilling selection criteria were enrolled in the study through the emergency department of Combined Military Hospital, Lahore. MRI was performed by Philips Intera Achieva 1.5 T super conducting MR unit (Philips Medical Systems, the Netherlands), with the use of a head coil. FLAIR examination was performed at 6700/150 (TR/TE) with an inversion time (TI) of 2200 ms, a field of view 230 mm, matrix 189 × 256, scan time of 3 min 50s and section thickness 5 mm in axial plane. Following MRI, patients underwent lumbar puncture for cerebrospinal fluid (CSF) examination after 8–12 h from the onset of event. MRI and CSF analysis results were then compared.ResultsOut of 245 cases, 49.39% (n = 121) were between 20–55 years of age while 50.61% (n = 124) were between 56–70 years of age, mean ± sd was calculated as 52.13 ± 10.45 years, 53.88% (n = 132) were male while 46.12% (n = 113) were females, frequency of acute subarachnoid hemorrhage in patients presenting with severe headache was recorded as 5.71%(n = 14), diagnostic accuracy of FLAIR in detection of acute subarachnoid hemorrhage in patients presenting with severe headache taking lumbar puncture as gold standard as 78.57% sensitivity, 96.53% specificity, 57.89% positive predictive value, 98.67% negative predictive value and accuracy rate was calculated as 95.29%.ConclusionDiagnostic accuracy of FLAIR in detection of acute subarachnoid hemorrhage in patients presenting with severe headache taking lumbar puncture as gold standard is higher and reliable.     

Impaired cerebrovascular reactivity is associated with recurrent stroke in patients with severe intracranial arterial stenosis: A C02 BOLD fMRI study

Background and PurposeSevere intracranial atherosclerotic stenosis (SIAS) remains at risk of recurrent ischemic events despite intensive medical management. Exhausted cerebrovascular reserve seems to be associated with higher risk of recurrent stroke.Materials and MethodsWe used whole brain MRI to estimate basal perfusion using dynamic susceptibility contrast and cerebrovascular reactivity (CVR) to hypercapnic challenge (CO2 inhalation) using BOLD contrast, in 20 patients with symptomatic SIAS (> 70%) of the middle cerebral artery (MCA) or the distal internal carotid artery. We studied relationships between individual clinical, biological, radiological baseline characteristics, recurrent ischemic events, basal perfusion parameters (mean transit time, delay, time to peak, cerebral blood flow and volume), and CVR measured in MCA territories (CVRMCA), and reported using laterality indices (LI).ResultsTen patients had an impaired CVR with (|LI| CVRMCA ≥ 0.08). During a mean follow-up of 3.3 years, all recurrent ipsilateral ischemic events occurred within the first year. They were more frequent in impaired CVRMCA group (n = 7/10 patients) than in normal CVRMCA group (n = 1/10), with different survival curves (log rank, P = 0.007).ConclusionImpaired CVR is associated with an increased rate of recurrent stroke in patients with symptomatic SIAS. CVR mapping should be used as a well tolerated method to select higher-risk patients in further therapeutic trials such as endovascular procedures.     

Neurosonographical follow-up in patients with spontaneous cervical artery dissection

OBJECTIVE: Neurovascular ultrasound (nUS) is widely used as a screening and monitoring tool in patients with spontaneous cervical artery dissection (sCAD). The aim of the study was to describe the sonographical course of the affected arteries in patients with a MRI-proven sCAD by repetitive nUS. METHODS: Thirty-seven consecutive patients aged<60 years with 1.5 T MRI-proven sCAD were prospectively investigated by nUS, and within 48 hours after admission before MRI. The patients were re-investigated after 6 months and again after a period>12 months. RESULTS: Forty-nine sCAD were detected in 37 patients; 24 lesions (49%) were located in the internal carotid arteries (ICA), and 25 (51%) in the vertebral arteries (VA). An arterial occlusion was found in 13 arteries (27%). The recanalization rate of occluded arteries was 62%. Regression of stenosis/occlusion within the first 6 months was found in 34 (69%) of the affected arteries, while between 6 and >12 months, the improvement rate was lower (19%). A complete recanalization without residual stenosis after 6 months was found in 39%. In only one artery, initial high grade ICA stenosis progressed to complete persistent occlusion (2%). DISCUSSION: The course of arterial stenosis or occlusion caused by sCAD is highly dynamic during the first 6 month after the event. The vast majority of arteries show regression of stenosis or recanalization of initial occlusion. Only a minority of patients experience a persistent deterioration of the vessel status.     

Clinical and imaging features of central neurocytomas

To describe the clinical and imaging characteristics of patients with central neurocytoma (CN), we reviewed data on 27 patients who had histologically confirmed CN and were treated in our institution between 1999 and 2010. Neuro-imaging findings on CT scan (n = 18) and MRI (n = 25) were retrospectively evaluated. There were 15 males and 12 females with a mean age of 29 years (range, 11–46 years). The most frequent presentations included headache (n = 21) and vomiting (n = 6). Tumor sites included bilateral lateral ventricles (n = 10), right lateral ventricle (n = 7), left lateral ventricle (n = 7) and fourth ventricle (n = 3). On MRI, the T1-weighted signal was hypointense in 12 patients and isointense in 13, and the T2-weighted signal was isointense in 8 patients and hyperintense in 15. CT scans/MRI revealed a cystic component in 18 patients. Tumors showed a mild to marked enhancement in 26 patients. Flow voids from tumor vessels on MRI were present in 14 patients, and calcification was noted in six of 18 patients with CT scans. All lateral ventricle tumors were resected through a transcortical or transcallosal approach. Gross total resection was achieved in 19 patients, near total in two and subtotal in six. One patient died of cerebral infarction in the perioperative period. At the last follow up, there were three known clinical recurrences in this series. However, no recurrence was noted in 17 patients who underwent gross total resection with no adjuvant therapy.     

Morphological differences between the aneurysmal and normal artery in patients with internal carotid–posterior communicating artery aneurysm

The aim of this study was to identify image-based morphological parameters that correlated with the formation of internal carotid artery–posterior communicating artery (ICA–PcomA) aneurysms. Morphological parameters obtained from 3-dimensional digital subtraction angiography (3D-DSA) were evaluated from nine patients with ICA–PcomA aneurysms, including the diameter of the ICA (Dica) and PcomA (Dpcom), the angle between the ICA and the origin of the PcomA (Apcom), and the angle between the ophthalmic and communicating segments of the ICA (Aica). Measurements were performed on both sides of each patient. Parameters were analyzed with a paired-samples t-test for significance. In additional, receiver operating characteristic (ROC) analysis was performed on the significant parameter. Statistically significant differences were found between the aneurysmal (45.28 ± 29.07°) and control sides (79.22 ± 17.83°) for Apcom (p = 0.020). In the ROC analysis, the area under the curve value of Apcom was 0.852, and the threshold for optimal sensitivity and specificity was 52.25°. Therefore, the Apcom parameter was correlated with the formation of an ICA–PcomA aneurysm, and seems to be a promising morphological parameter for risk assessment of aneurysm formation.     

Etiological,clinical, and radiological features of longitudinally extensive myelopathy in Chinese patients

Longitudinally extensive myelopathy (LEM) is a rare spinal syndrome, and was mostly assessed in western populations. In order to investigate the etiological, clinical, and radiological features of LEM in Chinese patients, we retrospectively analyzed eighty-nine (40 men and 49 women, median age 45.9 ± 15.7 years) patients with LEM hospitalized in China-Japan Friendship Hospital. LEM comprised autoimmune inflammatory myelitis (n = 53), metabolic and compressive disorders (n = 13), vascular diseases (n = 10), neoplastic diseases (n = 7), infectious diseases (n = 4), and syringomyelia (n = 2). Neuromyelitis optica spectrum disorders (NMOSD) was the most common cause of transverse myelopathy identified in LEM (38/89 [42.7%]) characterized by intractable vomiting and hiccups and painful tonic spasms. Subacute combined degeneration and anterior spinal artery syndrome accounted for the largest non-transverse LEM, which selectively affected the spinal dorsal and/or lateral columns and the spinal anterior region, respectively. Radicular pain was common in anterior spinal artery syndrome. Postrema (n = 15, 39.5%) and cervical (n = 31, 81.6%) lesions were significantly increased in NMOSD versus non-NMOSD (n = 7, 13.7% and n = 34, 66.7%, respectively, p < 0.05]. Axial T2-weighted MRI indicated that 46 (51.7%) patients exhibited complete lesions; 43 (48.3%) patients exhibited non-transverse lesions, mainly unilateral or symmetrical tract lesions. Twenty-four (51.1%) LEM patients exhibited distinct gadolinium contrast enhancement. In this Chinese cohort, LEM was primarily attributed to NMOSD. While the etiological distribution in the non-NMOSD group was different from western populations, clinical and imaging features may facilitate a differential diagnosis.     

Temporo-parietal theta activity correlates with misery perfusion in arterial occlusive disease

ObjectiveTemporo-parietal theta activity (TPTA), often detected in hemispheres with internal carotid (ICA) or middle cerebral artery (MCA) occlusive lesions, is more clearly separated from occipital alpha activity by magnetoencephalography (MEG) than electroencephalography. The present study investigated whether TPTA is correlated with misery perfusion, a surgically correctable type of hemodynamic impairment.MethodsAwake MEG was measured in 56 patients with ICA or MCA occlusive lesions. Regional cerebral blood flow (rCBF) and regional cerebrovascular reactivity (rCVR) to acetazolamide were measured in the MCA territory by xenon-133 single-photon emission computed tomography. MEG was repeated in 10 patients after vascular reconstruction surgery.ResultsFourteen patients showed TPTA in the lesion hemisphere (n = 13) or bilaterally (n = 1). The presence of TPTA was significantly correlated with both reduced rCBF and reduced rCVR (P = 0.0009). After surgery, TPTA disappeared in 7 of the 10 studied patients.ConclusionsThe presence of TPTA suggests misery perfusion, which is characterized by reduced rCBF and reduced rCVR.SignificanceMEG can detect the presence of reversible and surgically remediable cerebral ischemia before the development of complete infarction.     

Arterial elongation (“redundancy”) is not a feature of spontaneous cervical artery dissection

The prevalence of elongation of the internal carotid arteries (ICA) is suggested to be significantly higher in patients with spontaneous cervical artery dissection (sCAD) than in other stroke patients. We reassessed this hypothesis in a case–control study by means of an improved semiautomated MR imaging technique. We compared the length of the cervical arteries in patients with and without sCAD. In 40 consecutive patients with MRI proven sCAD, we measured the arterial lengths of both ICA from the cervical bifurcation to the carotid-T, as well as both vertebral arteries (VA) from their origin to the vertebro-basilar junction. The measurements were performed on the basis of high-resolution, three-dimensional (3D) MR-angiographies with the use of specialized software calculating the length of the coaxial line of these arteries. These results were compared to the findings of 40 age-matched controls with an ischemic stroke due to other etiologies. The mean arterial lengths in patients with sCAD (left ICA 169.62 mm, right ICA 170.05 mm, left VA 233.56 mm, right VA 224.57 mm) compared to patients without sCAD (left ICA 171.07 mm, right ICA 171.88 mm, left VA 232.54 mm, right VA 222.08 mm) did not differ significantly. In our case–control study, cervical arteries are not elongated in patients with sCAD when compared to age-matched stroke patients due to other etiologies. The finding of an arterial elongation is not a distinct clinical marker in patients with suspected sCAD. The macroscopic appearance of the cervical arteries on MR-angiograms does not suggest an underlying elongative or dilatative arteriopathy.     

Diagnosing acute lacunar infarction using CT perfusion

The value of CT perfusion (CTP) in detecting acute lacunar infarcts (LACI) has not been well established. We tested the sensitivity of CTP for LACI. CTP maps of consecutive stroke patients from 2009–2013 were examined. MRI diffusion imaging was used to identify those with LACI. Two stroke neurologists independently evaluated the CTP maps for evidence of a perfusion lesion in a deep perforating artery territory. Cerebral blood volume (CBV), cerebral blood flow (CBF), mean transit time (MTT) and time to maximum (Tmax) maps were first examined in isolation and then in combination. Inter-observer agreement was measured using Cohen’s κ. The lesions identified were later confirmed against the diffusion MRI reference and the sensitivity and specificity of CTP maps calculated. A total of 63 patient scans were analysed. There were 32 patients with MRI-confirmed LACI within the coverage of CTP; 18 in the striatum, 10 thalamic, and four in the corona radiata. Another 31 patients had normal MRI. Inter-rater agreement was good (κ = 0.83). Sensitivity (blinded consensus) was highest for MTT (56.2%) compared to Tmax (25%, p < 0.001), CBV (9.3%, p = 0.021) and CBF (43.7%, p < 0.001). MTT maps enable detection of a significant proportion of LACI using CTP.     

Influence of vessel morphology and variations on technical and clinical success in mechanical thrombectomy: -In vivo and in vitro analyses-

PurposeTo determine the impact of vessel variation and anatomical features on technical and clinical success.Materials and methodsIn vitro blood clots (n = 100) were introduced into a silicon carotid-T flow model of 2, 3 or 4 mm. The ICA/M1 angle varied at 45°, 90°, 135° and 180°. Peripheral embolism was measured. In vivo 50 pat. (73.5 yrs., ± 15) with MCA occlusion were examined for siphon variation, ICA morphology, vessel diameter and angles. The patients were divided according to the clinical success (mRS): group A: mRS ≤ 2 after 90 day and group B: mRS ≥ 3. Furthermore the technical success (TICI) and number of retrieval (n) were analysed.ResultsIn vitro with larger vessel diameter the migrated thrombus load decreased (P = .001). The steeper the M1/ICA angles, the higher thrombus weighs (180°: 2.94 mg; 135°: 6.32 mg; 90°: 8.65 mg, 45°: 10.69 mg; P < .001). In vivo patients with mRS ≤ 2 had significantly lower NIHSS (16.5 vs 20, P = .009) and higher ASPECTS (9 vs 6, P < .05). TICI ≥ 2b was more often achieved (86.6 vs 40% P = .002). The procedure time was lower (45 vs. 80 min, P < .05) with smaller number of retrieval (1.5 vs 4, P < 05). Proximal ICA stenosis offers a trend to unfavourable outcome (P = .073). Siphon variation “D” is associated with less retrieval manoeuvre.ConclusionWhile in vitro there is a close correlation between embolism and vascular anatomy, in vivo carotid artery stenosis and siphon variation influence clinical and technical success.     

Acute traumatic cervical cord injury in patients with os odontoideum

We retrospectively reviewed acute cervical cord injury after minor trauma in 10 patients with os odontoideum. Their clinical history, neurological symptoms, radiological investigations, follow-up period, American Spinal Injury Association impairment classification and motor score were reviewed. Before their traumatic injury, three patients were asymptomatic and seven reported myelopathic symptoms, including four patients with neck pain, two patients with unsteadiness and one patient with dizziness. Falls were the most common cause of injury (n = 6), followed by minor motor vehicle accidents (n = 3) and assault (n = 1). MRI and dynamic cervical lateral radiographs showed that all patients had atlantoaxial instability and cord compression. Most patients had spinal cord thinning and hyperintensity on T2-weighted MRI. Spinal cord compression was posterior (n = 5), or both anterior and posterior (n = 5). All patients underwent posterior rigid screw fixation and fusion, including atlantoaxial fusion (n = 8) and occipitocervical fusion (n = 2). We conclude that patients with asymptomatic or myelopathic atlantoaxial instability secondary to os odontoideum are at risk for acute spinal cord injury after minor traumatic injury. Fixation and fusion should be undertaken as prophylactic treatment for patients at risk of developing myelopathy and to avoid the neurological deterioration associated with acute traumatic cervical cord injury.     

Clinicoradiological presentation,management options and a review of sellar and suprasellar tuberculomas

Clinicoradiological presentation and management of patients with sellar and suprasellar tuberculomas (SST) were reviewed. The SSTs of eight patients were divided into five radiological subgroups: a sellar–suprasellar mass (n = 3); multiple coalescing ring enhancing granulomas (n = 2); an intrasellar abscess (n = 1); pachymeningitis with suprasellar extension (n = 1); and skull-base lesion involving the sella (n = 1). The predominant endocrinopathies were hypogonadism, hypothyroidism and diabetes insipidus. The management options included surgery utilizing the frontotemporal, transylvian approach (n = 4) or the transsphenoidal approach (n = 1), stereotactic biopsy and ventriculoperitoneal shunt (n = 1); endoscopic transsphenoidal biopsy (n = 1); and antituberculous therapy (ATT) without surgery (n = 1). All patients received ATT for 15–18 months. Patients with rapid visual deterioration (n = 2) or with associated intramedullary tuberculoma (n = 2) also received steroids for 2 weeks. At follow-up (range 10 months to 5.5 years; mean 3.2 years), the radiological response to ATT was evaluated. MRI after ATT showed resolution of SST in all except two patients with solid lesions. However, these lesions were smaller and had reduced contrast enhancement on imaging. Thus, SST may present with five radiological subtypes. Surgery is useful in obtaining histology. Short-term steroid therapy with ATT may reduce edema and adhesions around the optic nerve when rapid visual deterioration occurs and relieve symptoms of raised intracranial pressure. The often-associated hypopituitarism indicates the requirement for preoperative hormonal evaluation.     

Syndrome de Susac : étude de cinq cas

IntroductionSusac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss.MethodsWe report a series of five patients with Susac syndrome followed in our department from 1997 to 2007.ResultsThere were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n = 1), ophthalmological (n = 1), auditory (n = 1) and clinical triad (n = 2). Neurologic symptoms included encephalopathy (n = 2), headache (n = 5), transient ischemic attacks (n = 1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n = 4], cylophosphamid [n = 3], intravenous immunoglobulins [n = 5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n = 2), led to dramatic clinical improvement (n = 5).ConclusionDue to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.     

Cerebrospinal fluid ferritin — Unspecific and unsuitable for disease monitoring

Background and purposeSubarachnoid hemorrhage is sometimes difficult to diagnose radiologically. Cerebrospinal fluid (CSF) ferritin has been proposed to be highly specific and sensitive to detect hemorrhagic central nervous system (CNS) disease. We analyzed here the specificity of CSF ferritin in a large series of various CNS diseases and the influence of serum ferritin.Materials and methodsCSF ferritin, lactate, protein and total cell count were analyzed in 141 samples: neoplastic meningitis (n = 62), subarachnoid hemorrhage (n = 20), pyogenic infection (n = 10), viral infection (n = 10), multiple sclerosis (n = 10), borreliosis (n = 5) and normal controls (n = 24). Cerebrospinal fluid ferritin was measured with a microparticle immunoassay. In addition, serum and CSF ferritin were compared in 18 samples of bacterial and neoplastic meningitis.ResultsIn CNS hemorrhage, median ferritin was 51.55 μg/L (sensitivity: 90%) after the second lumbar puncture. In neoplastic meningitis, the median CSF ferritin was 16.3 μg/L (sensitivity: 45%). Interestingly, ferritin was higher in solid tumors than that in hematological neoplasms. In 90% of pyogenic inflammation, ferritin was elevated with a median of 53.35 μg/L, while only 50% of patients with viral infection had elevated CSF ferritin. In ventricular CSF, median ferritin was 163 μg/L, but only 20.6 μg/L in lumbar CSF. Ferritin was normal in multiple sclerosis and borreliosis.ConclusionsFerritin was elevated not only in hemorrhagic disease, but also in neoplastic and infectious meningitis. Ferritin was not a reliable marker of the course of disease. The influence of serum ferritin on CSF ferritin is negligible. We conclude that elevated CSF ferritin reliably, but unspecifically indicates severe CNS disease.     

Large artery intracranial stenosis in young adults with ischaemic stroke

Large artery intracranial stenosis (ICS) is a common finding in stroke patients, but is much less prevalent in Western countries than in Asia and in young adults than in the elderly. We investigated the prevalence and causes of ICS among French young adults with ischaemic stroke. Clinical and radiological data of patients aged 18–54 years treated consecutively for acute ischaemic stroke in the anterior circulation at a tertiary stroke centre were analysed retrospectively. Patients with > 50% ICS were identified. ICS was evaluated using TOF-MRA, vessel wall-MRI, digital subtraction angiography and CT-angiography. A total of 316 patients were included. ICS was diagnosed in 29 patients, resulting in a prevalence of 9.2% (95% CI, 6.2 to 13.3). The leading cause of ICS was atherosclerosis (n = 13), ahead of moyamoya disease (n = 4), dissection (n = 2), vasculitis (n = 2), and reversible cerebral vasoconstriction syndrome (n = 1). The cause of ICAS could not be determined in 7 patients. ICS was found in nearly one in 10 ischaemic strokes among French young adults. Atherosclerosis was the leading cause of ICS. The cause of ICS could not be determined in almost a quarter of the patients.     

Clinicopathologic correlations in 172 cases of rapid eye movement sleep behavior disorder with or without a coexisting neurologic disorder

ObjectiveTo determine the pathologic substrates in patients with rapid eye movement (REM) sleep behavior disorder (RBD) with or without a coexisting neurologic disorder.MethodsThe clinical and neuropathologic findings were analyzed on all autopsied cases from one of the collaborating sites in North America and Europe, were evaluated from January 1990 to March 2012, and were diagnosed with polysomnogram (PSG)-proven or probable RBD with or without a coexisting neurologic disorder. The clinical and neuropathologic diagnoses were based on published criteria.Results172 cases were identified, of whom 143 (83%) were men. The mean ± SD age of onset in years for the core features were as follows – RBD, 62 ± 14 (range, 20–93), cognitive impairment (n = 147); 69 ± 10 (range, 22–90), parkinsonism (n = 151); 68 ± 9 (range, 20–92), and autonomic dysfunction (n = 42); 62 ± 12 (range, 23–81). Death age was 75 ± 9 years (range, 24–96). Eighty-two (48%) had RBD confirmed by PSG, 64 (37%) had a classic history of recurrent dream enactment behavior, and 26 (15%) screened positive for RBD by questionnaire. RBD preceded the onset of cognitive impairment, parkinsonism, or autonomic dysfunction in 87 (51%) patients by 10 ± 12 (range, 1–61) years. The primary clinical diagnoses among those with a coexisting neurologic disorder were dementia with Lewy bodies (n = 97), Parkinson’s disease with or without mild cognitive impairment or dementia (n = 32), multiple system atrophy (MSA) (n = 19), Alzheimer’s disease (AD)(n = 9) and other various disorders including secondary narcolepsy (n = 2) and neurodegeneration with brain iron accumulation-type 1 (NBAI-1) (n = 1). The neuropathologic diagnoses were Lewy body disease (LBD)(n = 77, including 1 case with a duplication in the gene encoding α-synuclein), combined LBD and AD (n = 59), MSA (n = 19), AD (n = 6), progressive supranulear palsy (PSP) (n = 2), other mixed neurodegenerative pathologies (n = 6), NBIA-1/LBD/tauopathy (n = 1), and hypothalamic structural lesions (n = 2). Among the neurodegenerative disorders associated with RBD (n = 170), 160 (94%) were synucleinopathies. The RBD-synucleinopathy association was particularly high when RBD preceded the onset of other neurodegenerative syndrome features.ConclusionsIn this large series of PSG-confirmed and probable RBD cases that underwent autopsy, the strong association of RBD with the synucleinopathies was further substantiated and a wider spectrum of disorders which can underlie RBD now are more apparent.     

Diagnostic efficacy and safety of gadoteridol compared to gadobutrol and gadoteric acid in a large sample of CNS MRI studies at 1.5 T

PurposeTo evaluate safety and diagnostic accuracy of gadoteridol vs. other macrocyclic gadolinium-based contrast agents (GBCAs) in a large cohort of consecutive and non-selected patients referred for CE-MRI of the CNS.Material and methodsBetween November 2017 and March 2018, we prospectively enrolled a consecutive cohort of patients referred for neuroradiological CE-MRI (1.5 T MRI). Image quality and adverse events were assessed. Diagnostic performance was determined for a subgroup of patients with truth standard findings available. Comparison was made between patients receiving gadoteridol and patients receiving other macrocyclic GBCAs. Inter-reader agreement (kappa) between two expert neuroradiologists was calculated for the diagnosis of malignancy.ResultsOverall, 460 patients (220 M/240F; mean age 54 ± 16 years) were enrolled of which 230 received gadoteridol (Group 1) and 230 either gadoteric acid or gadobutrol [n = 83 (36.1%) and n = 147 (63.9%), respectively; Group 2]. Image quality was rated as good or excellent in both groups. The sensitivity, specificity and diagnostic accuracy for determination of malignancy was 88.2%, 96.5% and 95.4%, respectively, for Group 1 and 93.7%, 97.4% and 96.9%, respectively, for Group 2, with no significant differences between groups (P > 0.75) for any determination. Inter-reader agreement for the identification of malignancy was excellent [K = 0.877 (95%CI: 0.758–0.995) and K = 0.818 (95%CI: 0.663–0.972) for groups 1 and 2, respectively; P = 0.0913]. Adverse events occurred in 5 of 460 (1.09%) patients overall, with no significant difference (P = 0.972) between groups.ConclusionGadoteridol was safe and guaranteed good image quality without significant differences when compared to gadobutrol and gadoteric acid in a wide range of CNS pathologies.     

Acute behavioral crises in psychiatric inpatients with autism spectrum disorder (ASD): Recognition of concomitant medical or non-ASD psychiatric conditions predicts enhanced improvement

During adolescence, some individuals with autism spectrum disorder (ASD) engage in severe challenging behaviors, such as aggression, self-injury, disruption, agitation and tantrums. We aimed to assess risk factors associated with very acute behavioral crises in adolescents with ASD admitted to a dedicated neurobehavioral unit. We included retrospectively in 2008 and 2009 29 adolescents and young adults with ASD hospitalized for severe challenging behaviors and proposed a guideline (Perisse et al., 2010) that we applied prospectively for 29 patients recruited for the same indications between 2010 and 2012. In total, 58 patients were admitted (n = 70 hospitalizations, mean age = 15.66 (±4.07) years, 76% male). We systematically collected data describing socio-demographic characteristics, clinical variables (severity, presence of language, cognitive level), comorbid organic conditions, etiologic diagnosis of the episode, and treatments. We explored predictors of Global Assessment Functioning Scale (GAFS) score and duration of hospitalization at discharge. All but 2 patients exhibited severe autistic symptoms and intellectual disability (ID), and two-thirds had no functional verbal language. During the inpatient stay (mean = 84.3 (±94.9) days), patients doubled on average their GAFS scores (mean = 17.66 (±9.05) at admission vs. mean = 31.4 (±9.48) at discharge). Most common etiologies for acute behavioral crises were organic causes [n = 20 (28%), including epilepsy: n = 10 (14%) and painful medical conditions: n = 10 (14%)], environmental causes [n = 17 (25%) including lack of treatment: n = 11 (16%) and adjustment disorder: n = 6 (9%)], and non-ASD psychiatric condition [n = 33 (48%) including catatonia: n = 5 (7%), major depressive episode: n = 6 (9%), bipolar disorder: n = 4 (6%), schizophrenia: n = 6 (9%), other/unknown diagnosis: n = 12 (17%)]. We found no influence of age, gender, socio-economic status, migration, level of ID, or history of seizure on improvement of GAFS score at discharge. Severity of autism at admission was the only negative predictor (p < .001). Painful medical conditions (p = .04), non-ASD psychiatric diagnoses (p = .001), prior usage of specialized ASD care programs (p = .004), functional language (p = .007), as well as a higher number of challenging behaviors upon admission (p = .001) were associated with higher GAFS scores at discharge. Clinical severity at admission, based on the number of challenging behaviors (r = .35, p = .003) and GAFS score (r = −.32, p = .008) was correlated with a longer inpatient stay. Longer hospitalization was however correlated (r = .27, p = .03) with higher GAFS score at discharge even after adjustment for confounding factors. Challenging behaviors among adolescents with ASD may stem from diverse risk factors, including environmental problems, comorbid acute psychiatric conditions, or somatic illness such as epilepsy or acute pain. The management of these behavioral challenges requires a unified, multidisciplinary approach.     

The effect of war stress on multiple sclerosis exacerbations and radiological disease activity

ObjectiveThe relationship between stressful life events and multiple sclerosis (MS) exacerbations or radiological disease activity is at best controversial. The aim of this study is to examine the relationship between exposure to war-related events incurred during the July 2006 Israeli–Lebanese war and clinical relapses and MRI disease activity among Lebanese MS patients.MethodsWe studied a group of 216 patients with clinically definite relapsing remitting MS (RRMS), on whom clinical data was available for the war period and for the preceding and following year(s). The number of relapses was determined during the war period and during similar periods over a 3-year span. All patients with brain MRI during the war period had their scans reviewed for evidence of disease activity as defined by the presence of gadolinium enhancing (Gd+) lesions. A group of patients with brain MRI performed outside the war period was used for comparison.ResultsThe total number of relapses during the war period (n = 23) was significantly higher than during non-war periods (mean = 8.4, SD = 0.86) (p = 0.006). Of the 18 patients with brain MRI during the war, 5/7 with relapses and 1/11 without relapses had Gd+ lesions (p = 0.013). More patients had Gd+ lesions during the war period (33%) compared to controls (13%) (p = 0.075).InterpretationOur study shows that exposure to war-related events is likely to lead to an increase in both clinical relapses and MRI disease activity in patients with MS.