Background and Aims:

Intracranial pressure (ICP) monitoring is still reserved for the critically ill neurosurgical patient, because reliable and practical noninvasive techniques are lacking. In the present study, the analysis of a new tool able to register ICP waveforms obtained from skull pulsations (B4C) was performed in a set of neurocritical patients, correlating its data with ICP waveforms obtained from invasive catheter monitoring.

Methods:

Neurocritical patients under ICP monitoring were consecutively included for the evaluation with B4C sensor concomitantly. An ultrasound guided internal jugular veins (IJVs) compression was performed for elevation of ICP from baseline. ICP values, amplitudes of the peaks found in ICP and B4C waveforms (P2/P1 ratio) were analyzed.

Results:

Among 32 patients, traumatic brain injury, subarachnoid hemorrhage, and stroke were among the main causes for ICP monitoring. Correlation between ICP mean values for either ICP or B4C parameters were moderate (r .44) and improved when groups were separated according to skull situation (opened/intact). Very strong correlation was found for ICP P2/P1 ratio >1 and B4C P2/P1 ratio >1.2 (AUC .84). The induced elevation of ICP was compatible with strong correlation tendency between both techniques ICP waveforms (AUC .86).

Conclusions:

The novel noninvasive technique reliably reproduces the ICP waveform. This new technology may expand applicability of ICP monitoring.

Background and Aims:

Successful magnetic resonance-guided focussed-ultrasound thalamotomy (MRgFUS) for medication-refractory essential tremor (ET) requires precise targeting of the thalamic ventrointermediate (VIM) nucleus. Diffusion-tensor imaging (DTI)-tractography localises VIM more accurately than standard indirect-targeting, although outcome-comparisons are limited. We prospectively evaluated deterministic tractography-based targeting and indirect-targeting MRgFUS outcomes in medication-refractory ET over 3-years.

Methods:

136 ET patients refractory to ≥3 medications with significant dominant-hand tremor were included (2 centres). 58 received tractography-based MRgFUS. 78 received indirect-targeting MRgFUS. All tractography-based patients underwent pre-treatment 3T-MRI, 3-tract deterministic DTI-tractography (targeting dentatorubrothalamic, pyramidal and somatosensory tracts) for contralateral VIM localisation (Brainlab iPlan), and indirect-targeting for VIM location comparison, before tractography-based direct-targeting MRgFUS. Indirect-targeting patient outcomes were correlated with overlap between MRgFUS-lesion and tractographic region-of-interest retrospectively-derived from pre-treatment DTI (Spearman’s rank). Total and hand tremor (Clinical Rating Scale for Tremor [CRST] total and A+B subscores), quality-of-life (QUEST score) and adverse events (AEs) were independently-compared 3-monthly for 3-years.

Results:

Mean CRST total, A+B, and QUEST scores improved post-MRgFUS from similar baselines in both groups (3-year: tractography -38.9,-12.8,-31.8; indirect-targeting -30.6,-10.0,-23.9,p<0.0001). Tractography-group had significantly-greater improvements at all timepoints (3-year mean change from baseline intergroup difference: CRST -6.2,p<0.0001; CRST-A+B -1.4,p=0.0015; QUEST -5.2,p=0.0046) (Table1–3). Anteroposterior coordinate in tractography-group differed significantly from indirect targeting-generated coordinate (7.7±0.8 vs 6.3±0.2mm,p<0.001). In indirect-targeting group, region-of-interest and lesion overlap correlated with greater CRST percentage-reduction (r=-0.732,-0.768,-0.814 at 1, 2, 3-years,p<0.03). Tractography-group had non-significantly reduced AE rate.

Conclusions:

In the largest prospective study with longest follow-up to-date, tractography-based MRgFUS demonstrates superior long-term efficacy, VIM-targeting accuracy and quality-of-life improvement over indirect-targeting MRgFUS for medication-refractory ET.

Background and Aims:

To apply mathematical modeling to unravel MRI connectomic signatures among the amyotrophic lateral sclerosis (ALS)-behavioral variant of frontotemporal dementia (bvFTD) spectrum.

Methods:

83 ALS, 35 bvFTD and 61 controls underwent clinical, cognitive evaluations and MRI scan. ALS were divided in 54 ALS with only motor deficits (ALS-cn), 21 ALS cognitively/behaviorally impaired (ALS-ci/bi) and 8 ALS with bvFTD (ALS-FTD).

Mathematical models based on connectomic approach and ROC curve analysis identified the characteristic patterns of damage of ALS-cn and bvFTD. Single-subject analysis was performed on ALS-ci/bi and ALS-FTD to assess shared features with ALS-cn and bvFTD.

Results:

The structural/functional patterns of damage characterizing ALS-cn and bvFTD were identified. ALS-like pattern resulted in a focused structural damage within motor areas. bvFTD-like pattern resulted in widespread structural damage and decreased functional connectivity, mainly in frontotemporoparietal areas. ALS-ci/bi showed structural damage mostly overlapping with the ALS-like pattern, whereas they diverged from ALS-cn in functional connectivity showing enhanced functional connectivity within sensorimotor and decreased functional connectivity in frontotemporal areas. The latter mirrored the bvFTD-like pattern. ALS-FTD patients resembled the bvFTD-like pattern of damage both structurally and functionally, with a frank structural ALS-like damage in motor areas.

Conclusions:

Alterations of the frontotemporoparietal network characterized bvFTD-like pattern, while a focal damage within sensorimotor-basal ganglia areas defined the ALS-like pattern. Commonalities and differences relative to the two ends of ALS-FTD spectrum were found in ALS-ci/bi and ALS-FTD.

Funding: The Italian Ministry of Health (GR-2011-02351217; GR-2013-02357415; RF-2011-02351193), AriSLA (ConnectALS), European Research Council (StG2016_714388_NeuroTRACK).

Background and Aims:

Temporal lobe epilepsy (TLE) is frequently associated with major depressive disorder (MDD). Since the amygdala plays a role in both disorders and T2-signal changes might translate gliotic dysfunction, we measured the T2-signal in TLE regarding MDD and antiseizure drugs (ASD) response, as it might help understand the coexistence of these diseases.

Methods:

We included 119 individuals: MDD*responsive (n=10), MDD*ASD-resistant (n=19), no-MDD*responsive (n=12), no-MDD*ASD-resistant (n=17), MDD-only (n=26) and controls (n=35). Using Aftervoxel (http://www.bergo.eng.br/academic/aftervoxel/), we performed relaxometry in T2 coronal multi-echo images (two slices of 3-mm thick) acquired at 3T MRI-scanner. MDD diagnosis followed DSM-V criteria. We performed generalized linear model with log link function, including MDD and pharmacoresponse as main effects and MDD*pharmacoresponse interaction. We tested the relationship between ipsilateral T2-signal and Beck Depression Inventory (BDI) using partial correlations, controlling for scholarity, age and seizure frequency. We set p<0.05 as significant.

Results:

We found independent effects of MDD and pharmacoresponse in the ipsilateral T2-signal (both p<0.001), namely: increased ipsilateral T2-signal in MDD*responsive, MDD*ASD-resistant, no-MDD*responsive and no-MDD*ASD-resistant compared to controls (all p<0.004); in MDD*ASD-resistant (p<0.001) compared to no-MDD*responsive; in MDD*responsive, MDD*ASD-resistant and no-MDD*ASD-resistant compared to MDD-only (all p<0.001). Regarding contralateral amygdala, there was an effect of pharmacoresponse (p<0.001), but no MDD (p=0.53) nor pharmacoresponse*MDD effects, with increased T2-signal in responsive and ASD-resistant patients compared to controls (p<0.01), regardless MDD presence. Greater BDI correlated with increased T2-signal (r=0.31 p=0.03) in TLE group.

Conclusions:

Our data suggest a bilateral effect of pharmacoresponse and only ipsilateral of MDD in amygdalar T2-signal in TLE.

Acknowledgement: FAPESP 2019/08390-9 and 2013/07559-3

Background and Aims:

Migraine ranks as the second leading cause of disability worldwide. Current prophylactic drugs, aiming at reducing disease burden and acute medication intake, are not targeted to migraine, and limited by side effects. Novel antibodies anti-CGRP are preferentially intended for patients with severe phenotype due to their high cost.

We conducted a monocentric, longitudinal, retrospective, cohort study, to evaluate efficacy and safety at 3 months (T1) of different first and second-line prophylactic treatments in a “real life” population, afferent to our tertiary headache centre.

Methods:

We enrolled 294 patients diagnosed with episodic and chronic migraine, with and without aura, according to ICHD-3beta criteria, regardless of age and comorbidity. The primary outcome was reduction in monthly migraine headache days at T1 compared to baseline, defined as ≥ 30% (R30) and ≥50% (R50) reduction. We also collected side effects at T1.

Results:

At T1, 162 patients (55%) achieved R30, while 138 patients (47%) R50. Among the 9 treatments considered, responders were more frequently treated with flunarizine than non-responders (25.9% vs 12.9%, p＜0.006, and 25.4% vs 15.4%, p＜0.04, for R30 and R50 respectively), and with monotherapy (93.8% vs 83.3%, p=0.005, and 93.5% vs 85.2%, p=0.025, for R30 and R50 respectively). Flunarizine had a number needed to treat for R50 of 2.9 and to harm of 8.9.

Conclusions:

Flunarizine showed the best profile regarding efficacy and safety in our population. This result could suggest its use as first line treatment in migraine prophylaxis and preliminary to anti-CGRP antibodies.

Background and Aims:

Postherpetic neuralgia (PHN) is the most common and bearable complication of herpes zoster (HZ). This pain may have negative impact on the patient's all aspect of daily life and health-related quality of life (HRQOL). Despite numerous advances in treatment, many patients remain resistant to the current therapy options. It is the first time subcutaneous injection of methylprednisolone acetate and lidocaine has been used to treat refractory PHN. We report the results of this treatment evaluating pain relief and HRQOL improvement in this disorder.

Methods:

: A total of 43 patients with refractory PHN was enrolled in the observational study. All patients received daily subcutaneous injection of methylprednisolone acetate and lidocaine for 10 consecutive days. The severity of pain was assessed by using Visual Analog Scale (VAS) and 36-Item Short Form Survey (SF-36) were applied to evaluate HRQOL. Assessment of the pain and HRQOL was carried out at baseline and posttreatment at 4 weeks as well as 6 and 12 months.

Results:

At baseline, all patients experienced severe PHN with average VAS scores of 8.44 ± 0.85 (minimum 7; maximum 10). At 4 weeks, 6 months and 12 months after treatment, the pain had significantly decreased (p < 0.001), and all subjects showed significant improvement in all eight domains of HRQOL. No major adverse events associated with the subcutaneous injection were observed.

Conclusions:

Our results indicate that subcutaneous injection of methylprednisolone acetate and lidocaine can be an effective and safe treatment for PHN.

Background and Aims:

Background: Wilson disease is genetic disorder of copper metabolism which causes hepatic & neurologic manifestations mainly. Chelation therapy specially penicillamine is given as first line treatment in children with symptomatic wilson disease.

Objective: To assess the safety & the clinical outcome of treatment with low dose penicillamine in Neurologic wilson disease

Methods:

Methods: A longitudinal observational study was conducted at Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Thirty-nine (39) patients of Neurologic wilson disease who fulfill the inclusion and exclusion criteria were evaluated at Department of Paediatric Neurology, during January 2015 to December 2019. All study children were treated with low dose penicillamine (Cap Artamin 250 mg) rather with conventional dose penicillamine .Subsequent follow up was performed at 2 weeks, 1, 3 and 6 months.

Results:

Results: Total number of studied cases were 39. Mean age was 10.2 ± 3.1 year and male to female ratio was 2:1. Common presenting features were progressive deterioration of school performance (89.74%), gait disturbance (92.31%), dysarthria (92.31%) and dystonia (82.06%) of our studied children. Majority of the children (74.36%) were improved with low pencillamine therapy. Commonest side effects were worsening of neurological symptoms in (25.64%) and rash in (5.1%) cases after penicillamine therapy.

Conclusions:

Conclusion: In our study about three forth cases of Neurologic Wilson diseases were improved with low dose penicillamine therapy. One fourth cases were deteriorated with this therapy that is lower compared to previously reported high dose therapy.