The Compound Muscle Action Potential as Neurophysiological Marker for Amyotrophic Lateral Sclerosis.

Objectives: To definite the peripheral nervous involvement in ALS through the repeated use of the compound motor action potential (CMAP) to test the progression of disease, to determine different change of phrenic CMAP and forced vital capacity (FVC) in spinal and bulbar onset, and to establish clinical and neurophysiological features of patients with poor prognosis. Material & Methods: CMAP from phrenic, ulnar, and medial plantar nerves, Medical Research Council (MRC) score, revised ALS functional rating scale (ALSFRS-R) and FVC were evaluated in 117 ALS patients every three months in one year-period. Results: Bulbar onset patients had lower FVC but similar amplitude of phrenic CMAP at baseline compared to spinal onset patients. The patients with poor prognosis had lower phrenic CMAP and FVC at baseline. CMAP values, when compared to the rate found in the previous visit, reduced significantly in both poor and good prognosis groups during the entire follow-up period, while the FVC reduced significantly only in the first three months. Conclusions: CMAP is a reproducible sensitive marker for motor neurons loss and collateral reinnervation in ALS also in a short period of time. The changes in CMAP, MRC, FVC and ALSFRS-R score resulted correlated, but CMAP is the only parameter with the advantage to demonstrate objectively the progression of disease in both patients with poor and good prognosis for the entire period of follow-up. It should be used as clinical outcome of ALS in clinical trials, taking advantage of its objectivity and selectivity for peripheral nervous system study

Diffusion Magnetic Resonance Imaging Microstructural Abnormalities in Multiple System Atrophy: A Comprehensive Review

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by autonomic failure, ataxia, and/or parkinsonism. Its prominent pathological alterations can be investigated using diffusion magnetic resonance imaging (dMRI), a technique that exploits the characteristics of water random motion inside brain tissue. The aim of this report was to review currently available literature on the application of dMRI in MSA and to describe microstructural abnormalities, diagnostic applications, and pathophysiological correlates. Sixty-four published studies involving microstructural investigation using dMRI in MSA were included. Widespread microstructural abnormalities of white matter were described, especially in the middle cerebellar peduncle, corticospinal tract, and hemispheric fibers. Gray matter degeneration was identified as well, with diffuse involvement of subcortical structures, especially in the putamina. Diagnostic applications of dMRI were mostly explored for the differential diagnosis between MSA parkinsonism and Parkinson's disease. Recently, machine learning algorithms for image processing and disease classification have demonstrated high diagnostic accuracy, showing potential for translation into clinical practice. To a lesser extent, clinical correlates of microstructural abnormalities have also been investigated, and abnormalities related to motor, ocular, and cognitive impairments were described. dMRI in MSA has contributed to in vivo identification of known pathological abnormalities. Translation into clinical practice of the latest advancements for the differential diagnosis between MSA and other forms of parkinsonism seems feasible. Current limitations involve the possibility of correctly diagnosing MSA in the very early stages, when the clinical diagnosis is most uncertain. Furthermore, pathophysiological correlates of microstructural abnormalities remain understudied. (c) 2022 International Parkinson and Movement Disorder Society

Aripiprazole in the treatment of Huntington’s disease: a case series

Objectives: The aim of the study was to describe the effects of aripiprazole, a new atypical antipsychotic drug that acts as a partial dopamine agonist on motor, behavioral and cognitive functions in patients with genetically confirmed Huntington's disease (HD). Methods and results: Three HD patients were evaluated for Unified Huntington Disease Rating Scale part I and II and Beck Depression Inventory at baseline, after two months and one-year treatment. Aripiprazole effectively controlled involuntary movements and psychiatric symptoms, with effects on cognitive functions. Conclusions: Our case reports suggest that aripiprazole is well tolerated, remarkably improving some of the motor and behavioral symptoms in patients affected by HD. Randomized, controlled, long-term studies are warranted

Are olfactory ensheathing cells a promising cell therapy tool?

Olfactory Ensheathing Cells (OECs) show a peculiar plasticity and represent a unique population in the olfactory system supporting the continuous neuronal turnover and sheathing olfactory axons. They exhibit antigenic and morphological characteristics both of astrocytes and of Schwann Cells. In vitro, OECs promote axonal growth, moreover in vivo they can form myelin, promoting remyelination of damaged axons. In the last two decades, OECs have emerged as possible supportive cells for regeneration and functional recovery of damaged Central Nervous System (CNS). A characterization was performed both by flow cytometry and immunocytochemistry for the following markers: Vimentin, S-100β, Nestin, Glial Fibrillary Acidic Protein, Myelin, Neural Cell Adhesion Molecule, Low-affinity Nerve Growth Factor Receptor p75, Microtubule Associated Protein-2 and Protein Gene Product 9.5. In order to study the modulation of these markers, OECs were also grown in different culture conditions: standard or serum-free media with/without Growth Factors (GFs), such as basic Fibroblast Growth Factor and Glial Derived Neurotrophic Factor. Basal apoptosis was evaluated by annexin and propidium iodide analysis as well as after exposition to 6-hydroxydopamine (6-OHDA). Neural stem cells and a neuroblastoma cell line (SH-SY5Y) were used as control, primary OECs were prepared from postnatal mouse (P1) olfactory bulbs. Moreover, neuroprotective properties of OECs on 6-OHDA-treated cells were evaluated by an in vitro co-culture system or addition of OEC conditioned medium. We observed: 1) change of OEC usual morphology, reduction of both cell viability and marker expression in serum-free medium; 2) positive influence of GFs on both viability and marker expression; 3) no increased apoptosis after a prolonged exposition to 6-OHDA; 4) OEC neuroprotective effect, albeit non statistically significant, on 6-OHDA treated SH-SY5Y cells. These peculiar properties of OECs might render them as useful potential clinical agents being able to support injured CNS

Do Women Have a Higher Risk of Adverse Events after Carotid Revascularization?

Carotid artery stenosis is thought to cause up to 10% of ischemic strokes. Till now, the optimal treatment between carotid endarterectomy (CEA) and carotid artery stenting (CAS) remains debated, in particular for specific subgroups of patients. Available data suggest that female have higher risk of perioperative adverse events, but conflicting results comparing CEA and CAS regarding the benefit for male or female are present in the literature. A systematic review of recent publications on gender-related differences in operative risks is reported. Moreover, a consecutive cohort of 912 symptomatic and asymptomatic patients undergoing CEA (407, 44.6%) or CAS (505, 55.4%) in a single institution has been evaluated to determine the influence of gender (59.7% male vs. 40.3% female) on the outcomes after both revascularization procedures at 30 days and during 3 years of follow-up. Our experience seems to confirm literature data as regarding female higher risk of restenosis. Female patients had higher periprocedural (2.7% female vs. 0.9% male; p < 0.05) and long-term (11.4% female vs. 4.6% male; p < 0.05) restenosis rate. In conclusion, female anatomic and pathologic parameters should be taken into account for an accurate diagnosis of carotid stenosis and guidelines should be adjusted consequently