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https://www.readbyqxmd.com/read/29427344/directional-communication-during-movement-execution-interferes-with-tremor-in-parkinson-s-disease
#1
Georgios Naros, Florian Grimm, Daniel Weiss, Alireza Gharabaghi
BACKGROUND: Both the cerebello-thalamo-cortical circuit and the basal ganglia/cortical motor loop have been postulated to be generators of tremor in PD. The recent suggestion that the basal ganglia trigger tremor episodes and the cerebello-thalamo-cortical circuitry modulates tremor amplitude combines both competing hypotheses. However, the role of the STN in tremor generation and the impact of proprioceptive feedback on tremor suppression during voluntary movements have not been considered in this model yet...
February 2018: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29410313/motor-dysfunction-as-research-domain-in-the-period-preceding-manifest-schizophrenia-a-systematic-review
#2
REVIEW
Dusan Hirjak, Andreas Meyer-Lindenberg, Katharina M Kubera, Philipp A Thomann, Robert C Wolf
Schizophrenia is a severe behavioral syndrome of neurodevelopmental nature marked by primary or genuine motor abnormalities (GMA), which refer to spontaneous and medication-independent motor phenomena. Since motor dysfunction thus might be a consequence of events occurring during early childhood and adolescence, GMA can be detected in the period preceding manifest schizophrenia. However, the question whether motor system dysfunction might be a promising motor intermediate phenotype for schizophrenia remains unanswered...
February 2, 2018: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/29404647/-essential-tremor-state-of-the-art
#3
REVIEW
G Deuschl, D Berg
Essential tremor (ET) is currently classified as a syndrome rather than a unique disease, primarily involving monosymptomatic action tremor in both hands. Different etiologies are presumed to underlie this condition. Currently only a few monogenetic conditions are known to present with this syndrome. If accompanied by additional symptoms that do not in themselves constitute a new syndrome, such as abnormal tandem gait or postures, the syndrome should be diagnosed as "ET plus". ET is associated with abnormal rhythmic activation of the cerebello-thalamo-cortical tremor circuit...
February 5, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29396401/abnormal-cerebellar-processing-of-the-neck-proprioceptive-information-drives-dysfunctions-in-cervical-dystonia
#4
T Popa, C Hubsch, P James, A Richard, M Russo, S Pradeep, S Krishan, E Roze, S Meunier, A Kishore
The cerebellum can influence the responsiveness of the primary motor cortex (M1) to undergo spike timing-dependent plastic changes through a complex mechanism involving multiple relays in the cerebello-thalamo-cortical pathway. Previous TMS studies showed that cerebellar cortex excitation can block the increase in M1 excitability induced by a paired-associative stimulation (PAS), while cerebellar cortex inhibition would enhance it. Since cerebellum is known to be affected in many types of dystonia, this bidirectional modulation was assessed in 22 patients with cervical dystonia and 23 healthy controls...
February 2, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29390414/prenatal-diagnosis-of-joubert-syndrome-a-case-report-and-literature-review
#5
Lingling Zhu, Limei Xie
INTRODUCTION: Joubert syndrome (JS) is a rare autosomal recessive inherited disease belonging to ciliopathy with the causative mutation of genes. Except for X-linked inheritance, the high recurrence rate of a family is about 25%. After birth, it may cause a series of neurological symptoms, even with retina, kidney, liver, and other organ abnormalities, which is defined as Joubert syndrome and related disorders (JSRD). Molecular genetics research contributes to disease prediction and genetic counseling...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29387530/connectivity-derived-thalamic-segmentation-in-deep-brain-stimulation-for-tremor
#6
Harith Akram, Viswas Dayal, Philipp Mahlknecht, Dejan Georgiev, Jonathan Hyam, Thomas Foltynie, Patricia Limousin, Enrico De Vita, Marjan Jahanshahi, John Ashburner, Tim Behrens, Marwan Hariz, Ludvic Zrinzo
The ventral intermediate nucleus (VIM) of the thalamus is an established surgical target for stereotactic ablation and deep brain stimulation (DBS) in the treatment of tremor in Parkinson's disease (PD) and essential tremor (ET). It is centrally placed on a cerebello-thalamo-cortical network connecting the primary motor cortex, to the dentate nucleus of the contralateral cerebellum through the dentato-rubro-thalamic tract (DRT). The VIM is not readily visible on conventional MR imaging, so identifying the surgical target traditionally involved indirect targeting that relies on atlas-defined coordinates...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29376441/spontaneous-haemorrhage-into-a-large-abducens-nerve-schwannoma-a-case-report
#7
Daniel Brown, Nicolas McConnell, Richard List, Farouk Olubajo, Robin Highley, Mathew Storey, Gueorgui Kounin
PURPOSE: Abducens schwannomas are rare tumors that are not known to present with acute haemorrhage. We present a case of a 59 year-old female on warfarin who presented acutely with a sudden onset headache, nausea and photophobia. Neuroimaging revealed an acute haemorrhage into a lesion that entered Dorello's canal and was consistent with an abducens nerve schwannoma. MATERIALS AND METHODS: The patient's case notes, imaging, histology and operative recording were reviewed retrospectively to compile this case report...
January 29, 2018: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29376092/neuronal-activity-and-outcomes-from-thalamic-surgery-for-spinocerebellar-ataxia
#8
Takao Hashimoto, Abirami Muralidharan, Kunihiro Yoshida, Tetsuya Goto, Takehiro Yako, Kenneth B Baker, Jerrold L Vitek
Objectives: We investigated the effects of deep brain stimulation (DBS) or lesions of the ventral intermediate nucleus (Vim) of the thalamus for spinocerebellar ataxia (SCA) and examined the pathophysiological role of neuronal activity of the Vim underlying ataxia. Methods: Five patients with SCA with cortical atrophy (ages 60-69 years; 2 sporadic and three familial SCA) and five patients with essential tremor (ET) (ages 57-71 years) were treated with Vim surgery...
January 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29356974/structural-cerebellar-correlates-of-cognitive-functions-in-spinocerebellar-ataxia-type-2
#9
G Olivito, M Lupo, C Iacobacci, S Clausi, S Romano, M Masciullo, M Molinari, M Cercignani, M Bozzali, M Leggio
Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant neurodegenerative disease involving the cerebellum and characterized by a typical motor syndrome. In addition, the presence of cognitive impairment is now widely acknowledged as a feature of SCA2. Given the extensive connections between the cerebellum and associative cerebral areas, it is reasonable to hypothesize that cerebellar neurodegeneration associated with SCA2 may impact on the cerebellar modulation of the cerebral cortex, thus resulting in functional impairment...
January 22, 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29354585/neuroanatomical-mechanism-of-cerebellar-mutism-after-stroke
#10
Sekwang Lee, Yoon Hye Na, Hyun Im Moon, Woo Suk Tae, Sung-Bom Pyun
Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech due to cerebellar lesions. CM is often reported in children. We describe a rare case of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability, decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging revealed significant volume reduction of medial frontal projection fibers from the corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps major and minor and bilateral cingulum-angular bundles were not visualized...
December 2017: Annals of Rehabilitation Medicine
https://www.readbyqxmd.com/read/29325939/liponeurocytoma-of-the-cerebello-pontine-angle
#11
Carmela Chiaramonte, Sylvain Rabaste, Timothee Jacquesson, David Meyronet, François Cotton, Emmanuel Jouanneau, Moncef Berhouma
Liponeurocytoma is a very rare tumor classified as grade II (neuronal and mixed neuronal-glial tumors) according to 2016 WHO classification of Tumors of the Central Nervous System. The median age is 50-years-old and the most frequent location is the posterior cranial fossa especially within the cerebellar hemispheres; those arising in the cerebellopontine angle are exceptional. We herein report the clinical, radiological and pathological characteristics of a cerebellopontine angle liponeurocytoma in a 35-year-old woman...
January 8, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29278012/pilocytic-astrocytoma-of-the-cerebellopontine-angle-mimicking-vestibular-schwannoma-report-of-a-rare-entity
#12
Gautam Dutta, Daljit Singh, Hukum Singh, Deepashu Sachdeva, Vikas Kumar, Ashutosh Chaturvedi
We present a rare case of a 55-yr old patient of pilocytic astrocytoma of the cerebello-pontine angle mimicking a vestibular schwannoma. The tumor protruded into the porus acusticus causing enlargement of the internal auditory meatus, which is quite an unusual feature of glial tumours.
December 26, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/29204315/pathogenesis-of-primary-orthostatic-tremor-current-concepts-and-controversies
#13
REVIEW
Abhishek Lenka, Pramod Kumar Pal, Danish Ejaz Bhatti, Elan D Louis
Background: Orthostatic tremor (OT), a rare and complex movement disorder, is characterized by rapid tremor of both legs and the trunk while standing. These disappear while the patient is either lying down or walking. OT may be idiopathic/primary or it may coexist with several neurological conditions (secondary OT/OT plus). Primary OT remains an enigmatic movement disorder and its pathogenesis and neural correlates are not fully understood. Methods: A PubMed search was conducted in July 2017 to identify articles for this review...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/29123475/cerebello-cortical-differences-in-effective-connectivity-of-the-dominant-and-non-dominant-hand-during-a-visuomotor-paradigm-of-grip-force-control
#14
Eric Moulton, Cécile Galléa, Claire Kemlin, Romain Valabregue, Marc A Maier, Pavel Lindberg, Charlotte Rosso
Structural and functional differences are known to exist within the cortical sensorimotor networks with respect to the dominant vs. non-dominant hand. Similarly, the cerebellum, a key structure in the sensorimotor network with its cerebello-cortical connections, has been reported to respond differently when using the dominant vs. non-dominant hand. Several groups have already investigated causal interactions during diverse motor paradigms using effective connectivity but few have studied the larger visuomotor network, including key structures such as the parietal cortex and the cerebellum, with both hands...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/29119634/the-cerebral-basis-of-parkinsonian-tremor-a-network-perspective
#15
Rick C Helmich
Tremor in Parkinson's disease is a poorly understood sign. Although it is one of the clinical hallmarks of the disease, its pathophysiology remains unclear. It is clear that tremor involves different neural mechanisms than bradykinesia and rigidity, the other core motor signs of Parkinson's disease. In particular, the role of dopamine in tremor has been heavily debated given clinical observations that tremor has a variable response to dopaminergic medication. From a neuroscience perspective, tremor is also a special sign; unlike other motor signs, it has a clear electrophysiological signature (frequency, phase, and power)...
November 9, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/29098451/tremor-in-multiple-sclerosis-is-associated-with-cerebello-thalamic-pathology
#16
Frederique Boonstra, Grace Florescu, Andrew Evans, Chris Steward, Peter Mitchell, Patricia Desmond, Brad Moffat, Helmut Butzkueven, Scott Kolbe, Anneke van der Walt
Tremor in people with multiple sclerosis (MS) is a frequent and debilitating symptom with a relatively poorly understood pathophysiology. To determine the relationship between clinical tremor severity and structural magnetic resonance imaging parameters. Eleven patients with clinically definite MS and right-sided upper limb tremor were studied. Tremor severity was assessed using the Bain score (overall severity, writing, and Archimedes spiral drawing). Cerebellar dysfunction was assessed using the Scale for the Assessment and Rating of Ataxia...
November 2, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/29088993/an-update-on-the-diagnosis-and-treatment-of-vestibular-schwannoma
#17
Jane Halliday, Scott A Rutherford, Martin G McCabe, Dafydd G Evans
Vestibular schwannomas (VS) account for approximately 85% of tumors in the cerebello-pontine angle, with a lifetime incidence of approximately 1 in 1000. Most are sporadic, with approximately 5% related to the tumor predisposition syndrome Neurofibromatosis Type 2 (NF2). The mainstays of management strategies are: observation, surgery, radiosurgery/radiotherapy and, for patients with NF2 and rapidly growing tumors or deteriorating neurologic function the targeted therapy bevacizumab. While morbidity and mortality rates related to treatment of VS have improved dramatically over the last decades, there are still significant improvements that could be made, in particular with regards to long-term facial nerve and hearing outcomes...
January 2018: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29074317/alteration-of-cortical-excitability-and-its-modulation-by-miglustat-in-niemann-pick-disease-type-c
#18
Shady Safwat Hassan, Carlos Trenado, Saskia Elben, Alfons Schnitzler, Stefan Jun Groiss
Niemann-Pick type C (NP-C) is a rare, neurodegenerative, lysosomal storage disease. Cortical excitability using different transcranial magnetic stimulation (TMS) protocols together with clinical and neuropsychological testing was longitudinally assessed in a patient with NP-C. Cerebellar inhibition, a measure for the integrity of the cerebello-thalamo-cortical network, was impaired. Short-latency afferent inhibition, a measure for cholinergic transmission, and cognitive functions were also impaired and improved under Miglustat treatment...
October 23, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29066004/microstructural-white-matter-abnormalities-in-patients-with-col6a3-mutations-dyt27-dystonia
#19
Angela Jochim, Yong Li, Michael Zech, Daniel Lam, Nadine Gross, Kathrin Koch, Claus Zimmer, Juliane Winkelmann, Bernhard Haslinger
INTRODUCTION: Recently, mutations in the collagen gene COL6A3 have been reported in patients with autosomal-recessive, isolated dystonia (DYT27). Zebrafish models of COL6A3 mutations showed deficits in axonal targeting mechanisms. Therefore, COL6A3 mutations have been considered to contribute to irregular sensorimotor circuit formation. To test this hypothesis, we examined structural abnormalities in cerebral fiber tracts of dystonia patients with COL6A3 mutations using diffusion tensor imaging...
October 14, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29062685/loss-of-inhibition-in-sensorimotor-networks-in-focal-hand-dystonia
#20
Cecile Gallea, Priyantha Herath, Valerie Voon, Alicja Lerner, John Ostuni, Ziad Saad, Shantalaxmi Thada, Jeffrey Solomon, Silvina G Horovitz, Mark Hallett
OBJECTIVE: To investigate GABA-ergic receptor density and associated brain functional and grey matter changes in focal hand dystonia (FHD). METHODS: 18 patients with FHD of the right hand and 18 age and gender matched healthy volunteers (HV) participated in this study. We measured the density of GABA-A receptors using [ 11 C] Flumazenil and perfusion using [ 15 O] H 2 O. Anatomical images were also used to measure grey matter volume with voxel-based morphometry (VBM)...
2018: NeuroImage: Clinical
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