Alien hand syndrome in ruptured aneurysms: case report and review of the literature.

Alien hand syndrome is a rare condition associated with lesions of the corpus callosum and of the supplementary motor area, which can be caused by ruptured cerebral aneurysms. We present a novel case of a patient with a subarachnoid haemorrhage from an aneurysm of the anterior communicating artery who developed alien hand syndrome and intermanual conflict. In addition, we performed a systematic literature search and evaluated data on clinical presentation, treatment and radiological findings from relevant papers. To date, 17 cases of alien hand syndrome in aneurysmatic subarachnoid haemorrhage have been reported. Aneurysms of the anterior communicating artery were the most common origin of subarachnoid haemorrhage (10/17), followed by pericallosal artery aneurysms (7/10). The prognosis appears to be favourable; however, damage to the supplementary motor area during treatment should be avoided to keep symptoms minimal.

[Double vision and a wrong track]. / Doppelbilder und eine falsche Fährte.

A 72-year-old woman presented to our emergency centre with acute horizontal diplopia. Neurological examination revealed an isolated abducens nerve paresis on the left. Hilar enlargement seen on the chest x-ray and an elevated serum ACE level led us to suspect sarcoidosis, but the patient declined further evaluation. In the following days, her visual acuity decreased steadily, and she developed cervicothoracic pain, left sided ptosis, weakness of the right arm, and general asthenia. When she was readmitted as an emergency case, neurological examination revealed decreased visual acuity, external ophthalmoplegia and ptosis on the left and a C8 radicular lesion on the right. Imaging studies showed multilocular lesions, e. g. in the left orbital space, spinal epidural manifestations and lymphoma nodular involvement, including retroperitoneally. Laboratory chemistry showed elevated serum levels of ACE, sIL2 receptor and an elevated CD4 / CD8 ratio while bronchoalveolar lavage indicated lymphocytic alveolitis. The biopsy performed under the left M. masseter with a presumptive diagnosis of sarcoidosis, showed a diffuse large-cell B-cell lymphoma. We initiated immuno-chemotherapy following the R-CHOP schema with a curative approach. The case shows the lack of specificity of clinical, imaging and laboratory findings and thus underlines the need for histology in the differential diagnosis of sarcoidosis.

Postoperative MRI localisation of electrodes and clinical efficacy of pallidal deep brain stimulation in cervical dystonia.

INTRODUCTION: Pallidal deep brain stimulation (DBS) has been shown to be effective in cervical dystonia (CD) with an improvement of about 50-60% in the Toronto Western Spasmodic Torticollis Rating (TWSTR) Scale. However, predictive factors for the efficacy of DBS in CD are missing with the anatomical location of the electrodes being one of the most important potential predictive factors. METHODS: In the present blinded observational study we correlated the anatomical localisation of DBS contacts with the relative clinical improvement (CI %) in the TWSTR as achieved by DBS at different pallidal contacts in 20 patients with CD. Localisations of DBS contacts were derived from postoperative MRI-data following anatomical normalisation into the standard Montreal Neurological Institute stereotactic space. The CIs following 76 bilateral test stimulations of 24 h were mapped to stereotactic coordinates of the corresponding bilateral 152 active contacts and were allocated to low CI (<30%; n=74), intermediate CI (≥30%; <60%; n=52) or high CI (≥60%; n=26). RESULTS: Euclidean distances between contacts and the centroid differed between the three clusters (p<0.001) indicating different anatomical variances between clusters. The Euclidean distances between contacts and the centroid of the cluster with high CIs correlated with the individual level of CIs (r=-0.61; p<0.0001). This relationship was best fitted with an exponential regression curve (r(2)=0.41). DISCUSSION: Our data show that the clinical effect of pallidal DBS on CD displays an exponential decay over anatomical distance from an optimised target localisation within a subregion of the internal pallidum. The results will allow a comparison of future DBS studies with postoperative MRI by verifying optimised (for instance pallidal) targeting in DBS-treated patients.

Spasticity treatment with onabotulinumtoxin A: data from a prospective German real-life patient registry.

This study aimed at providing real-life baseline, injection and outcome data for the treatment of various forms of spasticity with onabotulinumtoxin A in Germany. Prospective data were collected in an open multicenter patient registry from 2005 until 2010, encompassing the experience of ten specialized German centers in the treatment of spasticity using onabotulinumtoxin A in 508 patients with a total of 2005 treatment sessions. Disease entities comprised spasticity following stroke (both ischemic and hemorrhagic), traumatic brain injury, multiple sclerosis, cerebral palsy, and anoxia. Sustained improvement was observed in a variety of outcome parameters including goal attainment and motor performance scores for up to five repeated injection sessions. No significant differences between disease entities or between upper and lower limb treatment were observed with regard to efficacy and safety following onabotulinumtoxin A treatment. Minor to moderate side effects were reported in <1 % of the study population. We conclude that repetitive treatment of focal and multifocal spasticity with onabotulinumtoxin A provides a safe and efficacious therapeutic strategy for patients with different disease entities of the central nervous system.

The pEGASUS-HPC stent system for intracranial arterial stenosis: a single-center case series.

BACKGROUND: Intracranial arterial stenting is a technique for the treatment of symptomatic stenosis. In this single-center retrospective case series we evaluated a novel low profile laser-cut stent with an antithrombogenic hydrophilic polymer coating (pEGASUS-HPC, Phenox GmbH, Bochum, Germany) for the treatment of intracranial stenosis in the setting of acute ischemic stroke and elective cases. METHODS: All patients treated with pEGASUS-HPC for one or more intracranial arterial stenoses at our institution were retrospectively included. Clinical, imaging and procedural parameters as well as clinical and imaging follow-up data were collected. RESULTS: We performed 43 interventions in 41 patients with 42 stenoses in our neurovascular center between August 2021 and February 2024. Twenty-one patients (51.2%) were female and the mean±SD age was 71±10.8 years. Thirty-seven (86.1%) procedures were performed in the setting of endovascular acute ischemic stroke treatment. Technical or procedural complications occurred in seven patients (16.3%), six in the thrombectomy group and one in the elective group. One stent-related hemorrhagic complication (subarachnoid hemorrhage) occurred in emergency cases and symptomatic intracerebral hemorrhage occurred in one patient treated in an elective setting. Overall stenosis reduction following pEGASUS-HPC stent implantation was 53.0±18.0%. On follow-up imaging, which was available for 16 patients (37.2%) after an average of 32±58.6 days, 62.5% of the stents were patent. CONCLUSION: Our single-center case series demonstrates the feasibility of using the pEGASUS-HPC stent system, especially in emergency situations when thrombectomy fails.

Thrombectomy With the pRESET vs Solitaire Stent Retrievers as First-Line Large Vessel Occlusion Stroke Treatment: A Randomized Clinical Trial.

Importance: Stent retriever-based thrombectomy is highly beneficial in large vessel occlusion (LVO) strokes. Many stent retriever designs are currently available, but comparison of these technologies in well-conducted studies is lacking. Objective: To determine whether thrombectomy for LVO stroke with the pRESET stent retriever is noninferior to treatment with the Solitaire stent retriever. Design, Setting, and Participants: This study was a multicenter, prospective, randomized, controlled, open-label, adaptive, noninferiority trial with blinded primary end point evaluation. Between October 2019 and February 2022, multicenter participation occurred across 19 research hospitals and/or universities in the US and 5 in Germany. Patients with LVO stroke were enrolled and included up to 8 hours after symptom onset. Interventions: Patients underwent 1:1 randomization to thrombectomy with the pRESET or Solitaire stent retriever. Main Outcomes and Measures: The primary outcome was the difference in the rate of 90-day functional independence across the 2 devices, using a -12.5% noninferiority margin for the lower bound of the 1-sided 95% CI of the difference between pRESET and Solitaire retrievers. Results: Of 340 randomized patients, 170 (50.0%) were female, and the median (IQR) age was 73.0 (64.0-82.0) years. The study procedure was completed in 322 of the 340 randomized patients. The primary end point of 90-day functional independence was achieved by 95 patients (54.9%; 95% CI, 48.7-61.1) in the pRESET group and in 96 (57.5%; 95% CI, 51.2-63.8) in the Solitaire group (absolute difference, -2.57%; 95% CI, -11.42 to 6.28). As the lower bound of the 95% CI was greater than -12.5%, the pRESET retriever was deemed noninferior to the Solitaire retriever. The noninferiority of pRESET over Solitaire was also observed in the secondary clinical end point (90-day shift in modified Rankin Scale score) and in both angiographic end points (Expanded Treatment in Cerebral Infarction [eTICI] score of 2b50 or greater within 3 passes: 146 of 173 [84.4%] vs 149 of 167 [89.2%]; absolute difference, -4.83%; 95% CI, -10.84 to 1.19; eTICI of 2c or greater following the first pass: 76 of 173 [43.7%] vs 74 of 167 [44.3%]; absolute difference, -0.63%; 95% CI, -9.48 to 8.21). Symptomatic intracranial hemorrhage occurred in 0 patients in the pRESET group and 2 (1.2%) in the Solitaire group. Mortality occurred in 25 (14.5%) in the pRESET group and in 24 (14.4%) in the Solitaire group at 90 days. Findings of the per-protocol and as-treated analyses were in concordance with findings of the intention-to-treat analysis. Conclusions and Relevance: In this study, among patients with LVO stroke, thrombectomy with the pRESET stent retriever was noninferior to thrombectomy with the Solitaire stent retriever. Findings suggest that pRESET offers a safe and effective option for flow restoration and disability reduction in patients with LVO stroke.

No correlation of substantia nigra echogenicity and nigrostriatal degradation in Parkinson's disease.

BACKGROUND: Substantia nigra hyperechogenicity assessed by transcranial sonography is a typical finding in up to 90% of patients with idiopathic Parkinson's disease, although its value as a surrogate marker for disease progression in Parkinson's disease is controversial. (123) I-FP-CIT-single photon emission computed tomography (SPECT) represents an established paraclinical surrogate marker to quantify the nigrostriatal dopaminergic deficit in Parkinson's disease. Whereas most studies found no correlation between extent of substantia nigra echogenicity and the putaminal FP-CIT binding ratio, a more recent analysis reported opposite results. METHODS: In 92 patients with Parkinson's disease the substantia nigra echogenicity was compared with the putaminal FP-CIT binding ratio using an investigator-independent SPECT analysis protocol and with several clinical parameters. RESULTS: No correlation was found between the substantia nigra hyperechogenicity and the FP-CIT binding ratio or the disease severity. CONCLUSIONS: Substantia nigra hyperechogenicity does not reflect the degree of the nigrostriatal degeneration or the clinical state of the disease progression.

Functional Aplasia of the Contralateral A1 Segment Influences Clinical Outcome in Patients with Occlusion of the Distal Internal Carotid Artery.

Background: The importance of an A1 aplasia remains unclear in stroke patients. In this work, we analyze the impact of an A1 aplasia contralateral to an acute occlusion of the distal internal carotid artery (ICA) on clinical outcomes. Methods: We conducted a retrospective study of consecutive stroke patients treated with mechanical thrombectomy at 12 tertiary care centers between January 2015 and February 2021 due to an occlusion of the distal ICA. Functional A1 aplasia was defined as the absence of A1 or hypoplastic A1 (>50% reduction to the contralateral site). Functional independence was measured by the modified Rankin Scale (mRS ≤ 2). Results: In total, 81 out of 1068 (8%) patients had functional A1 aplasia contralateral to distal ICA occlusion. Patients with functional contralateral A1 aplasia were more severely affected on admission (median NIHSS 18, IQR 15−23 vs. 17, IQR 13−21; aOR: 0.672, 95% CI: 0.448−1.007, p = 0.054) and post-interventional ischemic damage was larger (median ASPECTS 5, IQR 1−7, vs. 6, IQR 3−8; aOR: 1.817, 95% CI: 1.184−2.789, p = 0.006). Infarction occurred more often within the ipsilateral ACA territory (20/76, 26% vs. 110/961, 11%; aOR: 2.482, 95% CI: 1.389−4.437, p = 0.002) and both ACA territories (8/76, 11% vs. 5/961, 1%; aOR: 17.968, 95% CI: 4.979−64.847, p ≤ 0.001). Functional contralateral A1 aplasia was associated with a lower rate of functional independence at discharge (6/81, 8% vs. 194/965, 20%; aOR: 2.579, 95% CI: 1.086−6.122, p = 0.032) and after 90 days (5/55, 9% vs. 170/723, 24%; aOR: 2.664, 95% CI: 1.031−6.883, p = 0.043). Conclusions: A functional A1 aplasia contralateral to a distal ICA occlusion is associated with a poorer clinical outcome.

Tic Reduction in Adult Onset Gilles De La Tourette Syndrome Using as Required Nabiximols Spray.

Background: Tourette syndrome (TS) manifests with motor and vocal tics that can reach disabling intensity. Established therapies may show insufficient relief or side effects. Cannabinoids have demonstrated therapeutic potential in small studies. This report presents buccal Nabiximols "as required" in the treatment of tics in TS. Case report: A 25-year-old man presented with stigmatizing motor and phonic tics after cessation of daily Cannabis use. After Tiaprid 300 mg per day had shown no sufficient effect a trial of Nabiximols reduced tics by >90%. Discussion: Nabiximols could be an adjunct treatment in TS for situations were tics are severly disabling.

Brain parenchyma sonography and 123I-FP-CIT SPECT in Parkinson's disease and essential tremor.

We aimed to investigate the accuracy of transcranial brain parenchyma sonography (TCS) for differentiation between idiopathic Parkinson's disease (PD) and essential tremor (ET) in comparison to (123)I-FP-CIT SPECT (FP-CIT SPECT). Seventy-four patients, in whom PD or ET was suspected on the basis of clinical criteria, were analyzed. The echogenicity of the substantia nigra (SN) and the striatal binding of dopamine transporters (DAT) were evaluated by TCS and FP-CIT SPECT, respectively. Three patients were excluded due to an insufficient transtemporal bone window using TCS. Forty-six and 25 patients were clinically classified as PD and ET. SPECT revealed a reduced DAT binding in 42 of all 71 included patients. Thirty-six of the 42 patients with abnormal FP-CIT SPECT findings had a pathological SN hyperechogenicity, whereas TCS findings in the remaining 6 patients were normal. In 27 of 29 patients with normal SPECT findings the SN echogenicity was regular. Referring to FP-CIT SPECT, the sensitivity and specificity of TCS for detection of PD were 86 and 93%; the positive and negative predictive values were 95 and 82%, respectively. Sensitivity and specificity in detection of clinically diagnosed PD patients were 78 and 92% for TCS and 91 and 100% for FP-CIT SPECT, respectively. In patients with pathological FP-CIT SPECT and pathological TCS, the extent of SN hyperechogenicity did not correlate with the degree of reduction in dopamine transporter binding on the side opposite of the more affected limb. TCS allows a reliable differentiation of PD and ET. The TCS SN hyperechogenicity does not correlate with the extent of dopaminergic neuron degeneration.

Influences of levodopa on adipose tissue and skeletal muscle metabolism in patients with idiopathic Parkinson's disease.

OBJECTIVE: The substantial weight loss in Parkinson's patients may be related to direct influences of levodopa treatment on fat mobilization/oxidation. We assessed systemic and local metabolic responses to levodopa/benserazide in patients with idiopathic Parkinson's disease. METHODS: We studied 10 Parkinson's disease patients and examined adipose tissue and skeletal muscle metabolism directly with microdialysis. We monitored dialysate concentrations of ethanol, glucose, lactate, pyruvate, and glycerol to assess tissue blood flow and metabolism before and after levodopa/benserazide intake. We also conducted in vitro studies on adipocytes from healthy women. RESULTS: Levodopa/benserazide increased serum levodopa, 3,4-dihydroxyphenylacetic acid (DOPAC), and norepinephrine (P < 0.01). Serum adipose tissue and skeletal muscle glycerol did not change or decreased. Adipose tissue glycerol was inversely correlated with serum levodopa concentrations (P < 0.05). In isolated adipocytes, levodopa attenuated isoproterenol-induced glycerol release (P < 0.05). CONCLUSION: Levodopa/benserazide elicits pronounced metabolic changes in both adipose tissue and skeletal muscle with a switch from lipid to carbohydrate metabolism. In adipose tissue, levodopa/benserazide failed to activate lipolysis. Therefore, we suggest that levodopa/benserazide does not induce fat wasting through direct and acute influences on adipose tissue metabolism.