In his brief report on the structure of the gray matter of the central nervous system (1873), in which he described the "black reaction", Golgi noted the ramifications of the axon. This discovery prompted the French hist...In his brief report on the structure of the gray matter of the central nervous system (1873), in which he described the "black reaction", Golgi noted the ramifications of the axon. This discovery prompted the French histologist Louis Antoine Ranvier, one of the first to try the black reaction outside Italy, to propose an ingenious theory of referred pain in his Traité technique d'histologie. Ranvier suggested that the nerve fibers originating from the irritated area and those coming from the region to which the sensation is referred converge on the same axon and thus the same cell body, causing the spatial dislocation of sensation. This theory of referred pain is a powerful example of the extraordinary clinical-physiological impact of the first of Golgi's neurocytological discoveries.
A 79-year-old woman with hypertension was evaluated 3 hours and 20 minutes after the sudden onset of left-sided weakness which lasted about 15 minutes and was followed by involuntary, coarse, flinging movements of the le...A 79-year-old woman with hypertension was evaluated 3 hours and 20 minutes after the sudden onset of left-sided weakness which lasted about 15 minutes and was followed by involuntary, coarse, flinging movements of the left extremities (hemiballistic), occurring every few minutes, and facial asymmetry. Brain computed tomography revealed no abnormalities. The patient received intravenous thrombolysis with 0.9 mg/kg of alteplase 4 hours after the symptom onset. Involuntary movements and central facial nerve paresis subsided within 48 hours of the thrombolysis. Magnetic resonance imaging at day 5 revealed restricted diffusion within the right globus pallidus, which was a new ischemic lesion. Thrombolysis should be considered in hemiballism as a presenting symptom of acute stroke.
Piccolo G, Borutti G, Franciotta D
… +5 more, Arbasino C, Dallocchio C, Camana C, Gola G, Egitto MG
Funct Neurol
· 2015 · PMID 26214029
Posterior reversible encephalopathy syndrome (PRES) is a rare occurrence in patients with Guillain-Barré syndrome (GBS). Two patients with neuroradiological evidence of PRES without central nervous system (CNS) symptoms...Posterior reversible encephalopathy syndrome (PRES) is a rare occurrence in patients with Guillain-Barré syndrome (GBS). Two patients with neuroradiological evidence of PRES without central nervous system (CNS) symptoms were recently reported. We present the case of a GBS patient with minimal CNS symptoms and magnetic resonance imaging findings consistent with PRES. PRES in GBS might be an underestimated condition. It should be suspected in GBS patients in the presence of even mild CNS symptoms. A timely PRES diagnosis along with early correction of autonomic system dysfunction in GBS patients is recommended to prevent possible dangerous CNS complications.
Nascimbeni A, Caruso S, Salatino A
… +4 more, Carenza M, Rigano M, Raviolo A, Ricci R
Funct Neurol
· 2015 · PMID 26214028
Mild cognitive impairment (MCI) entails a high risk of developing Alzheimer's dementia. In MCI patients gait impairment, which increases the risk of falls and institutionalization, is an early motor sign. A dualtask (DT)...Mild cognitive impairment (MCI) entails a high risk of developing Alzheimer's dementia. In MCI patients gait impairment, which increases the risk of falls and institutionalization, is an early motor sign. A dualtask (DT) paradigm might improve the observation of this phenomenon. The aim of this study was to investigate motor-cognitive interference in a sample of MCI patients and a group of matched healthy controls submitted to DT conditions. To this end, three different cognitive tasks were used: counting backwards, short story recall and a phonemic fluency task. Overall, the patients, compared with the healthy participants, performed worse on the cognitive tasks and showed some degree of gait impairment. In the DT conditions, both groups showed significant gait disruption independently of the concomitant cognitive task. As regards cognitive performance, counting backwards worsened during dual tasking, while short story recall improved in both groups. Overall, our results suggest that the use of a DT paradigm does not improve the early detection of MCI. Our findings of enhanced story recall during walking might have interesting implications for rehabilitation of memory function.
Ciancarelli I, De Amicis D, Di Massimo C
… +4 more, Sandrini G, Pistarini C, Carolei A, Tozzi Ciancarelli MG
Funct Neurol
· 2015 · PMID 26214026
The influence of intensive multifunctional neurorehabilitation on serum levels of Cu/Zn-superoxide dismutase (Cu/Zn-SOD), neuron-specific enolase (NSE), and 8-hydroxy-2-deoxyguanosine (8-OHdG), as markers of oxidative da...The influence of intensive multifunctional neurorehabilitation on serum levels of Cu/Zn-superoxide dismutase (Cu/Zn-SOD), neuron-specific enolase (NSE), and 8-hydroxy-2-deoxyguanosine (8-OHdG), as markers of oxidative damage, was evaluated in symptomatic patients with Huntington's disease (HD). Improved clinical outcome measures were observed after neurorehabilitation. Baseline levels of Cu/Zn-SOD, NSE and 8-OHdG were higher than those observed in controls. Cu/Zn-SOD and NSE values decreased after neurorehabilitation, but were still higher than those measured in controls. Cu/Zn-SOD and NSE correlated positively before (r=0.659; p=0.003) and after rehabilitation (r=0.553, p=0.017). 8-OHdG values decreased after neurorehabilitation without reaching significance when compared with baseline values (p=0.145). No correlation was observed between the measured oxidative markers and the assessed clinical outcome measures, either before or after neurorehabilitation. The findings reported in the present paper provide evidence of the effectiveness of neurorehabilitation in reducing oxidative damage in HD patients and underline the limit of serum oxidative markers for the evaluation of clinical features of HD.
Zarrouk A, Nury T, Dauphin A
… +8 more, Frère P, Riedinger JM, Bachelet CM, Frouin F, Moreau T, Hammami M, Kahn E, Lizard G
Funct Neurol
· 2015 · PMID 26214025
Disorganization of the cytoskeleton of neurons has major consequences on the transport of neurotransmitters via the microtubule network. The interaction of cytoskeleton proteins (actin and tubulin) was studied in neurona...Disorganization of the cytoskeleton of neurons has major consequences on the transport of neurotransmitters via the microtubule network. The interaction of cytoskeleton proteins (actin and tubulin) was studied in neuronal SK-N-BE cells treated with tetracosanoic acid (C24:0), which is cytotoxic and increased in Alzheimer's disease patients. When SK-N-BE cells were treated with C24:0, mitochondrial dysfunctions and a non-apoptotic mode of cell death were observed. Fluorescence microscopy revealed shrunken cells with perinuclear condensation of actin and tubulin. Impact of C24:0 on actin-microtubule interaction in human neuronal SK-N-BE cells: evaluation by FRET confocal spectral imaging microscopy after dual staining with rhodamine-phalloidin and tubulin tracker green After staining with rhodamine-phalloidin and with an antibody raised against α-/β-tubulin, modifications of F-actin and α-/β-tubulin levels were detected by flow cytometry. Lower levels of α-tubulin were found by Western blotting. In C24:0-treated cells, spectral analysis and fluorescence recovery after photobleaching (FRAP) measured by confocal microscopy proved the existence of fluorescence resonance energy transfer (FRET) when actin and tubulin were stained with tubulin tracker and rhodamine-phalloidin demonstrating actin and tubulin co-localization/interaction. In control cells, no FRET was observed. Our data demonstrate quantitative changes in actin and tubulin, and modified interactions between actin and tubulin in SK-N-BE cells treated with C24:0. They also show that FRET confocal imaging microscopy is an interesting method for specifying the impact of cytotoxic compounds on cytoskeleton proteins.
Serra L, Petrucci A, Spanò B
… +10 more, Torso M, Olivito G, Lispi L, Costanzi-Porrini S, Giulietti G, Koch G, Giacanelli M, Caltagirone C, Cercignani M, Bozzali M
Funct Neurol
· 2015 · PMID 26214024
Myotonic dystrophy type 1 (DM1) is a multisystemic disorder dominated by muscular impairment and brain dysfunctions. Although brain damage has previously been demonstrated in DM1, its associations with the genetics and c...Myotonic dystrophy type 1 (DM1) is a multisystemic disorder dominated by muscular impairment and brain dysfunctions. Although brain damage has previously been demonstrated in DM1, its associations with the genetics and clinical/neuropsychological features of the disease are controversial. This study assessed the differential role of gray matter (GM) and white matter (WM) damage in determining higher-level dysfunctions in DM1. Ten patients with genetically confirmed DM1 and 16 healthy How genetics affects the brain to produce higher-level dysfunctions in myotonic dystrophy type 1 matched controls entered the study. The patients underwent a neuropsychological assessment and quantification of CTG triplet expansion. All the subjects underwent MR scanning at 3T, with studies including T1-weighted volumes and diffusion-weighted images. Voxel-based morphometry and tractbased spatial statistics were used for unbiased quantification of regional GM atrophy and WM integrity. The DM1 patients showed widespread involvement of both tissues. The extent of the damage correlated with CTG triplet expansion and cognition. This study supports the idea that genetic abnormalities in DM1mainly target the WM, but GM involvement is also crucial in determining the clinical characteristics of DM1.
Vitali P, Di Perri C, Vaudano AE
… +2 more, Meletti S, Villani F
Funct Neurol
· 2015 · PMID 26214023
Functional magnetic resonance imaging (fMRI), which has high spatial resolution, is increasingly used to evaluate cerebral functions in neurological and psychiatric diseases. The main limitation of fMRI is that it detect...Functional magnetic resonance imaging (fMRI), which has high spatial resolution, is increasingly used to evaluate cerebral functions in neurological and psychiatric diseases. The main limitation of fMRI is that it detects neural activity indirectly, through the associated slow hemodynamic variations. Because neurovascular coupling can be regionally altered by pathological conditions or drugs, fMRI responses may not truly reflect neural activity. Electroencephalography (EEG) recordings, which directly detect neural activity with optimal temporal resolution, can now be obtained during fMRI data acquisition. Therefore, there is a growing interest in combining the techniques to obtain simultaneous EEG-fMRI recordings. The EEG-fMRI approach has several promising clinical applications. The first is the detection of cortical areas involved in interictal and ictal epileptic activity. Second, combining evoked potentials with fMRI could be an accurate way to study eloquent cortical areas for the planning of neurosurgery or rehabilitation, circumventing the above-mentioned limitation of fMRI. Finally, the use of this approach to evaluate the functional connectivity of resting-state networks would extend the applications of EEG-fMRI to uncooperative or unconscious patients. Integration of multimodal neuroimaging methods: a rationale for clinical applications of simultaneous EEG-fMRI.
Fancellu R, Corsini E, Bernardi G
… +6 more, Buzzo P, Ferrari ML, Lamantea E, Garaventa A, Truini M, Salvarani S
Funct Neurol
· 2014 · PMID 25764259
We describe a case of cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. A 28-year-old woman developed progressive ataxia with hyporeflexia at the age of 19. Brain MRI showed progressive cere...We describe a case of cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. A 28-year-old woman developed progressive ataxia with hyporeflexia at the age of 19. Brain MRI showed progressive cerebellar atrophy. Neurophysiological studies, screening of immune-mediated ataxias, oncological markers, vitamin E and genetic tests for spinocerebellar ataxia types 1,2,3, Friedreich ataxia and POLG1 were negative. Anti-Hu antibodies were positive in Western blot and indirect immunofluorescence (1:640). Total-body computed tomography revealed a mediastinum mass; the histological diagnosis was maturing ganglioneuroma. Immunohistochemistry showed a mild reaction between the tumor and the patient's serum, and no reaction between the tumor and control serum. After surgery, serum anti-Hu titer decreased, while ataxic symptoms initially worsened and then stabilized. Ganglioneuroma is a benign tumor, usually derived from the maturation of a neuroblastoma. The benign histology and the presence of anti-Hu antibodies could be related to the positive oncological prognosis and to the slow clinical course mimicking a degenerative ataxia.
Corallo F, Marra A, Bramanti P
… +1 more, Calabrò RS
Funct Neurol
· 2014 · PMID 25764258
Cranioplasty is performed, using autograft materials, to treat the "trephined syndrome" in patients previously submitted to craniectomy. Indeed, considerable improvements in neuropsychological deficits, control of convul...Cranioplasty is performed, using autograft materials, to treat the "trephined syndrome" in patients previously submitted to craniectomy. Indeed, considerable improvements in neuropsychological deficits, control of convulsions and partial prevention of cerebral atrophy are achieved after this surgical procedure. We describe the pre- and post-operative neuropsychological and functional evaluation of a 30-year-old male patient who underwent cranioplasty following previous craniectomy for a subarachnoid hemorrhage due to a spontaneously ruptured aneurysm of the right middle cerebral artery.
Multiple sclerosis (MS) is a common demyelinating disease of the central nervous system in which an abnormal immune system response results in damage to the myelin sheaths surrounding nerves. The etiology of MS remains e...Multiple sclerosis (MS) is a common demyelinating disease of the central nervous system in which an abnormal immune system response results in damage to the myelin sheaths surrounding nerves. The etiology of MS remains elusive, although it has been suggested to be influenced by both genetic and environmental factors. Here, we report two exceptional cases of patients affected by both MS and Thomsen's myotonia. Electromyography and MRI scans confirmed the presence of both diseases in the referred cases. Although the mechanisms underlying the co-occurrence of MS and Thomsen's disease are unclear, we have endeavored to offer plausible explanations for it.
Suzuki T, Bunno Y, Onigata C
… +3 more, Tani M, Uragami S, Yoshida S
Funct Neurol
· 2014 · PMID 25764256
the median nerve at the wrist in subjects during two motor imagery conditions: holding and not holding the sensor of a pinch meter between the thumb and index finger. Our aim was to determine whether mental simulation wi...the median nerve at the wrist in subjects during two motor imagery conditions: holding and not holding the sensor of a pinch meter between the thumb and index finger. Our aim was to determine whether mental simulation without the muscle contraction associated with motion can increase the excitability of spinal neural function in patients with Parkinson's disease (PD). F-waves of the left thenar muscles were examined in 10 patients with PD under resting, holding and motor imagery conditions. For the holding condition, the subjects held the sensor of the pinch meter between their thumb and index finger. For the motor imagery conditions, the subjects were asked to imagine a 50% maximal voluntary isometric contraction holding and not holding the sensor of the pinch meter between their thumb and index finger (motor imagery "with"/"without sensor"). Persistence during motor imagery under the "with sensor" condition increased significantly compared with persistence during resting (n=10, z=2.2509, p=0.0244, Wilcoxon test). The F/M amplitude ratio during motor imagery under the "with sensor" condition increased significantly compared with that during resting (n=10, z=2.1915, p=0.0284, Wilcoxon test). Excitability of spinal neural function during motor imagery in Parkinson's disease Motor imagery under the "with the sensor" condition increased excitability of the spinal neural output to the thenar muscles. Because excitability of the spinal neural output to the thenar muscles during motor imagery "with the sensor" was significantly higher than that during resting, we suggest that movement preparation for a motor imagery task is important in patients with PD.
Zucchella C, Capone A, Codella V
… +5 more, Vecchione C, Buccino G, Sandrini G, Pierelli F, Bartolo M
Funct Neurol
· 2014 · PMID 25764255
Cognitive impairment is a frequent complication of stroke. The aim of this study was to evaluate the effectiveness of cognitive training performed early after stroke. Ninety-two patients were randomly assigned to either...Cognitive impairment is a frequent complication of stroke. The aim of this study was to evaluate the effectiveness of cognitive training performed early after stroke. Ninety-two patients were randomly assigned to either the study group (SG) or the control group (CG). Cognitive rehabilitation consisted of 16 individual one-hour sessions in which patients performed therapist- guided computer exercises. The patients in the CG performed a sham intervention. After four weeks all the patients were re-evaluated. In the SG, significant improvements (p<0.05) were detected in all neuropsychological measures at the post-training evaluation, while the CG showed mild (not statistically significant) improvements on cognitive tests. Between-group analysis revealed statistically significant differences in the domains of memory and visual attention. Cognitive training performed early after stroke seems to be a viable option for improving cognitive outcome in stroke survivors. Further studies should assess whether this may favor their reintegration into everyday life.
Pizzella V, Marzetti L, Della Penna S
… +3 more, de Pasquale F, Zappasodi F, Romani GL
Funct Neurol
· 2014 · PMID 25764254
To progress toward understanding of the mechanisms underlying the functional organization of the human brain, either a bottom-up or a top-down approach may be adopted. The former starts from the study of the detailed fun...To progress toward understanding of the mechanisms underlying the functional organization of the human brain, either a bottom-up or a top-down approach may be adopted. The former starts from the study of the detailed functioning of a small number of neuronal assemblies, while the latter tries to decode brain functioning by considering the brain as a whole. This review discusses the top-down approach and the use of magnetoencephalography (MEG) to describe global brain properties. The main idea behind this approach is that the concurrence of several areas is required for the brain to instantiate a specific behavior/functioning. A central issue is therefore the study of brain functional connectivity and the concept of brain networks as ensembles of distant brain areas that preferentially exchange information. Importantly, the human brain is a dynamic device, and MEG is ideally suited to investigate phenomena on behaviorally relevant timescales, also offering the possibility of capturing behaviorally-related brain connectivity dynamics.
Retico A, Tosetti M, Muratori F
… +1 more, Calderoni S
Funct Neurol
· 2014 · PMID 25764253
Classification methods based on machine learning (ML) techniques are becoming widespread analysis tools in neuroimaging studies. They have the potential to enhance the diagnostic power of brain data, by assigning a predi...Classification methods based on machine learning (ML) techniques are becoming widespread analysis tools in neuroimaging studies. They have the potential to enhance the diagnostic power of brain data, by assigning a predictive index, either of pathology or of treatment response, to the single subject's acquisition. ML techniques are currently finding numerous applications in psychiatric illness, in addition to the widely studied neurodegenerative diseases. In this review we give a comprehensive account of the use of classification techniques applied to structural magnetic resonance images in autism spectrum disorders (ASDs). Understanding of these highly heterogeneous neurodevelopmental diseases could greatly benefit from additional descriptors of pathology and predictive indices extracted directly from brain data. A perspective is also provided on the future developments necessary to translate ML methods from the field of ASD research into the clinic.
Torricelli A, Contini D, Dalla Mora A
… +6 more, Pifferi A, Re R, Zucchelli L, Caffini M, Farina A, Spinelli L
Funct Neurol
· 2014 · PMID 25764252
The aim of this review is to present the state of the art of neurophotonics, a recently founded discipline lying at the interface between optics and neuroscience. While neurophotonics also includes invasive techniques fo...The aim of this review is to present the state of the art of neurophotonics, a recently founded discipline lying at the interface between optics and neuroscience. While neurophotonics also includes invasive techniques for animal studies, in this review we focus only on the non-invasive methods that use near infrared light to probe functional activity in the brain, namely the fast optical signal, diffuse correlation spectroscopy, and functional near infrared spectroscopy methods. We also present an overview of the physical principles of light propagation in biological tissues, and of the main physiological sources of signal. Finally, we discuss the open issues in models, instrumentation, data analysis and clinical approaches.