Progressive chorea and dystonia associated with a large arteriovenous malformation

Movement Disorder ◽

Progressive Movement ◽

A 9-year-old male with intellectual disability and epilepsy presenting with a progressive movement disorder characterized by chorea and dystonia primarily affecting the left face, arm, and leg and attributed to an arteriovenous malformation centered within the right thalamus with bilateral extension into the basal ganglia, midbrain and pons is reported.

BrainPath-Mediated Resection of a Ruptured Subcortical Arteriovenous Malformation

Operative Neurosurgery ◽

10.1093/ons/opx186 ◽

2017 ◽

Vol 15 (1) ◽

pp. 32-38 ◽

Cited By ~ 5

Author(s):

Alex M Witek ◽

Nina Z Moore ◽

M Adeeb Sebai ◽

Mark D Bain

Keyword(s):

Basal Ganglia ◽

Intracerebral Hemorrhage ◽

Frontal Lobe ◽

Tubular Retractor ◽

Postoperative Imaging ◽

Hematoma Evacuation ◽

AbstractBACKGROUNDAlthough tubular retractor systems have gained popularity for other indications, there have been few reports of their use for arteriovenous malformation (AVM) surgery. A patient was diagnosed with a ruptured 1.2-cm subcortical AVM after presenting with intracerebral hemorrhage in the right frontal lobe and anterior basal ganglia. The characteristics of this AVM made it amenable to resection using a tubular retractor.OBJECTIVETo demonstrate the feasibility and safety of AVM resection using a tubular retractor system.METHODSResection of the ruptured 1.2-cm subcortical AVM was performed utilizing the BrainPathTM (NICO corp, Indianapolis, Indiana) tubular retractor system.RESULTSThe BrainPathTM approach provided sufficient visualization and surgical freedom to permit successful AVM resection and hematoma evacuation. Postoperative imaging demonstrated near total hematoma removal and angiographic obliteration of the AVM. There were no complications, and the patient made an excellent recovery.CONCLUSIONTubular retractors warrant consideration for accessing small, deep, ruptured AVMs. The nuances of such systems and their role in AVM surgery are discussed.

Community Living, Intellectual Disability and Extensive Support Needs: A Rights-Based Approach to Assessment and Intervention

International Journal of Environmental Research and Public Health ◽

10.3390/ijerph18063175 ◽

2021 ◽

Vol 18 (6) ◽

pp. 3175

Author(s):

Laura Esteban ◽

Patricia Navas ◽

Miguel Ángel Verdugo ◽

Víctor B. Arias

Keyword(s):

Intellectual Disability ◽

Independent Living ◽

Structural Equation ◽

Equation Modeling ◽

Community Living ◽

Support Needs ◽

Related Services ◽

The Right To Privacy ◽

People with intellectual disability (ID) and extensive support needs experience poorer quality of life than their peers whose disability is not as severe. Many of them live in residential settings that limit community participation and prevent them from exercising control over their lives. This work analyzes the extent to which professional practices are aimed at promoting the right to community living for people with ID and extensive support needs, as well as the rights that are particularly linked to it, such as the right to habilitation and rehabilitation and the right to privacy. A specific questionnaire was designed and administered to 729 adults with intellectual disability (M = 37.05; DT = 12.79) living in different settings (family home, residential facilities and group homes). Measurement and structural models were estimated using exploratory structural equation modeling. Results obtained reveal that people with extensive support needs receive less support in terms of guaranteeing their right to independent living and privacy, especially when they live in disability-related services. This study highlights the need to implement and monitor, using valid and reliable indicators, mesosystem strategies that guarantee the right to live and participate in the community, especially for individuals with ID and extensive support needs.

The potential for person-centred planning to support the community participation of adults with an intellectual disability

Journal of Intellectual Disabilities ◽

10.1177/17446295211022125 ◽

2021 ◽

pp. 174462952110221

Author(s):

Darren McCausland ◽

Esther Murphy ◽

Mary McCarron ◽

Philip McCallion

Keyword(s):

Intellectual Disability ◽

Mixed Methods ◽

Community Participation ◽

Social Inclusion ◽

Mixed Methods Approach ◽

Complex Needs ◽

Qualitative Case Studies ◽

Quantitative Analyses ◽

The Right ◽

The Impact

Person-centred planning (PCP) puts individuals with an intellectual disability at the centre of service and support planning, identifying how individuals wish to live their lives and what is needed to make that possible. PCP has been identified as having the potential to facilitate improved social inclusion and community participation. A mixed-methods approach combined quantitative analyses with qualitative case studies of individuals with severe-profound intellectual disability to assess the impact of PCP on community participation for adults with an intellectual disability at a disability service in Dublin. We conclude that PCP may provide a good basis to plan community participation and, with the right supports in place, may provide opportunities for people with complex needs to improve their community participation. Supports including familiar staff and family are critical to the success of PCP for people with complex needs, and their absence may undermine the best intentions of PCP for this population.

Spontaneous Thrombosis of an Orbital Arteriovenous Malformation Revealing Hereditary Haemorrhagic Telangiectasia (Rendu-Osler-Weber Disease)

Interventional Neuroradiology ◽

10.1177/159101991101700411 ◽

2011 ◽

Vol 17 (4) ◽

pp. 466-471 ◽

Cited By ~ 5

Author(s):

C. Van Went ◽

A. Ozanne ◽

G. Saliou ◽

G. Dethorey ◽

I. De Monchy ◽

...

Keyword(s):

Visual Acuity ◽

Intraocular Pressure ◽

Genetic Disorder ◽

Hereditary Haemorrhagic Telangiectasia ◽

Spontaneous Thrombosis ◽

Vein Thrombosis ◽

Recurrent Epistaxis ◽

The Right ◽

Central Retinal Vein

Hereditary Haemorrhagic Telangiectasia (HHT) is a genetic disorder responsible for cutaneous or mucosal telangiectasia and arteriovenous malformations (AVMs). The most frequent locations are lung and brain. In contrast, orbital AVMs are very rare. We describe a case of symptomatic orbital arteriovenous malformation due to spontaneous thrombosis. A 65-year-old woman was referred for chronic right eye proptosis associated with dilation of conjunctival vessels with a jellyfish pattern. Right visual acuity was 20/40 and intraocular pressure was 40 mmHg. Personal and familial history of recurrent epistaxis, associated with multiple telangiectasia within lips and palate, led to the diagnosis of HHT. Magnetic resonance imaging (MRI) completed with cerebral angiography found a giant and occluded AVM within the right orbit. Other AVMs were also found in brain and chest, confirming the diagnosis. Antiglaucomatous eyedrops were added to reduce intraocular pressure and a steroid therapy was begun. Two months later, visual acuity decreased in the right eye, due to a central retinal vein thrombosis. In conclusion, Most brain or pulmonary AVM can be treated by embolization. By contrast, this treatment in case of orbital location can lead to central retinal artery and/or central retinal vein occlusion, which may also appear as a spontaneous complication of the orbital AVM. Therapeutic management of orbital AVM is thus not standardized, and the balance between spontaneous and iatrogenic risk of visual loss has to be taken into account.

BOLD Coherence Reveals Segregated Functional Neural Interactions When Adapting to Distinct Torque Perturbations

Journal of Neurophysiology ◽

10.1152/jn.00405.2006 ◽

2007 ◽

Vol 97 (3) ◽

pp. 2107-2120 ◽

Cited By ~ 16

Author(s):

Eugene Tunik ◽

Paul J. Schmitt ◽

Scott T. Grafton

Keyword(s):

Basal Ganglia ◽

Contextual Information ◽

Natural World ◽

Adaptive Responses ◽

Response Preparation ◽

Target Capture ◽

Neural Processes ◽

Neural Interactions ◽

The Right ◽

Level Analysis

In the natural world, we experience and adapt to multiple extrinsic perturbations. This poses a challenge to neural circuits in discriminating between different context-appropriate responses. Using event-related fMRI, we characterized the neural dynamics involved in this process by randomly delivering a position- or velocity-dependent torque perturbation to subjects’ arms during a target-capture task. Each perturbation was color-cued during movement preparation to provide contextual information. Although trajectories differed between perturbations, subjects significantly reduced error under both conditions. This was paralleled by reduced BOLD signal in the right dentate nucleus, the left sensorimotor cortex, and the left intraparietal sulcus. Trials included “NoGo” conditions to dissociate activity related to preparation from execution and adaptation. Subsequent analysis identified perturbation-specific neural processes underlying preparation (“NoGo”) and adaptation (“Go”) early and late into learning. Between-perturbation comparisons of BOLD magnitude revealed negligible differences for both preparation and adaptation trials. However, a network-level analysis of BOLD coherence revealed that by late learning, response preparation (“NoGo”) was attributed to a relative focusing of coherence within cortical and basal ganglia networks in both perturbation conditions, demonstrating a common network interaction for establishing arbitrary visuomotor associations. Conversely, late-learning adaptation (“Go”) was attributed to a focusing of BOLD coherence between a cortical–basal ganglia network in the viscous condition and between a cortical–cerebellar network in the positional condition. Our findings demonstrate that trial-to-trial acquisition of two distinct adaptive responses is attributed not to anatomically segregated regions, but to differential functional interactions within common sensorimotor circuits.

Motor Subcircuits Mediating the Control of Movement Velocity: A PET Study

Journal of Neurophysiology ◽

10.1152/jn.1998.80.4.2162 ◽

1998 ◽

Vol 80 (4) ◽

pp. 2162-2176 ◽

Cited By ~ 126

Author(s):

Robert S. Turner ◽

Scott T. Grafton ◽

John R. Votaw ◽

Mahlon R. Delong ◽

John M. Hoffman

Keyword(s):

Motor Control ◽

Basal Ganglia ◽

Right Hemisphere ◽

Movement Velocity ◽

Rate Effects ◽

Regression Slopes ◽

The Right ◽

Parietal Cortices ◽

Linear Contrasts ◽

Rate Of Movement

Turner, Robert S., Scott T. Grafton, John R. Votaw, Mahlon R. DeLong, and John M. Hoffman. Motor subcircuits mediating the control of movement velocity: a PET study. J. Neurophysiol. 80: 2162–2176, 1998. The influence of changes in the mean velocity of movement on regional cerebral blood flow (rCBF) was studied using positron emission tomography (PET) in nine healthy right-handed adults while they performed a smooth pursuit visuomanual tracking task. Images of relative rCBF were obtained while subjects moved a hand-held joystick to track the movement of a target at three different rates of a sinusoidal displacement (0.1, 0.4, and 0.7 Hz). Significant changes in rCBF between task conditions were detected using analysis of variance and weighted linear contrasts. The kinematics of arm and eye movements indicated that subjects performed tasks in a similar manner, particularly during the faster two tracking conditions. Significant increases in rCBF during arm movement (relative to an eye tracking only control condition) were detected in a widespread network of areas known for their involvement in motor control. The activated areas included primary sensorimotor (M1S1), dorsal and mesial premotor, and dorsal parietal cortices in the left hemisphere and to a lesser extent the sensorimotor and superior parietal cortices in the right hemisphere. Subcortically, activations were found in the left putamen, globus pallidus (GP), and thalamus, in the right basal ganglia, and in the right anterior cerebellum. Within the cerebral volume activated with movement, three areas had changes in rCBF that correlated positively with the rate of movement: left M1S1, left GP, and right anterior cerebellum. No movement-related sites had rCBF that correlated negatively with the rate of movement. Regressions of mean percent change (MPC) in rCBF onto mean hand velocity yielded two nonoverlapping subpopulations of movement-related loci, the three sites with significant rate effects and regression slopes steeper than 0.17 MPC⋅cm−1⋅s−1 and all other sites with nonsignificant rate effects and regression slopes below 0.1 MPC⋅cm−1⋅s−1. Moreover, the effects of movement per se and of movement velocity varied in magnitude independently. These results confirm previous reports that movement-related activations of M1S1 and cerebellum are sensitive to movement frequency or some covarying parameter of movement. The activation of GP with increasing movement velocity, not described in previous functional-imaging studies, supports the hypothesis that the basal ganglia motor circuit may be involved preferentially in controlling or monitoring the scale and/or dynamics of arm movements. The remaining areas that were activated equally for all movement rates may be involved in controlling higher level aspects of motor control that are independent of movement dynamics.

Combined surgical treatment of arteriovenous malformation of the lower jaw

Endovascular Neuroradiology ◽

10.26683/2304-9359-2020-4(34)-95-104 ◽

2020 ◽

Vol 34 (4) ◽

pp. 95-104

Author(s):

D.V. Shchehlov ◽

V.M. Zahorodnii ◽

I.V. Altman ◽

N.V. Kiselyova ◽

I.I. Kashkish

Keyword(s):

Cerebral Angiography ◽

Combined Treatment ◽

The Body ◽

Facial Artery ◽

External Carotid ◽

Maxillary Artery ◽

Terminal Branch ◽

Lower Jaw ◽

The objective – to presents the observation of combined treatment of a patient with arteriovenous malformation of the lower jaw.A man, 21 years old, was hospitalized in the Scientific-Practical Center of Endovascular Neuroradiology NAMS of Ukraine with complaints of bleeding from a tooth socket after an attempt to remove the 6th tooth (first painter) of the lower jaw on the left. According to the performed survey radiography of the lower jaw, an aneurysmal bone cyst was revealed in the body of the lower jaw on the left, corresponding to the localization of bleeding. According to cerebral angiography, an arteriovenous malformation of the lower jaw was revealed on the left, the afferent arteries of which were: the right facial artery (a branch of the right external carotid artery (ECA)), the left facial artery (a branch of the left ECA), the lower alveolar artery, the superior-posterior alveolar artery (branches of the maxillary artery ‒ the terminal branch of the left ECA) with drainage into a vein, which was located in the body of the lower jaw. In order to exclude the malformation from the bloodstream and prevent bleeding, a controlled embolization of the malformation was performed using non-spherical emboli – polyvinyl alcohol (PVA) particles from Cook, USA. Using a transfemoral approach, a guide catheter was inserted into the orifice of the ECA, then a Headway 27 microcatheter (Microvention, USA) was passed through it along a Traxes 14 guide wire (Microvention, USA), the afferent arteries of the malformation were selectively cathete-rized in turn, and embolization was performed after superselective angiography. The patient was discharged in a satisfactory condition. Two weeks after the operation, the bleeding resumed. The performed control cerebral angiography revealed a relapse of the malformation with a change in its angioarchitectonics ‒ the filling of the malformation in the late arterial and venous phases of cerebral blood flow was noted. Re-embolization was performed using PVA emboli (Cook), which was supplemented by transcutaneous puncture of the drainage vein in the mandible and its embolization with histoacryl (B. Braun, Germany) and lipiodol (Guerbet, France) in a 1 : 1 ratio. Results. As a result of using this technique, it was possible to turn off the malformation completely. For 6 months from the moment of surgery, no bleeding was noted, and subsequently the patient had a tooth removed without complications.Conclusions. The proposed method for treating arteriovenous malformation of the lower jaw, proposed in this case, showed the effectiveness of a combination of endovascular embolization in combination with transcutaneous embolization of the draining vein and can be successfully used to treat this pathology.

The right to be Freepeople: Relational voluntary-assisted-advocacy as a psychological and ethical resource for decolonizing intellectual disability

Journal of Social and Political Psychology ◽

10.5964/jspp.v6i2.946 ◽

2018 ◽

Vol 6 (2) ◽

pp. 556-574 ◽

Cited By ~ 1

Author(s):

Charlotte Capri ◽

Leslie Swartz

Keyword(s):

Social Justice ◽

Intellectual Disability ◽

South African ◽

Daily Life ◽

Social Activism ◽

Relational Ethics ◽

Thematic Content Analysis ◽

Political Conditions ◽

The Right ◽

Empirical Material

Participating in social activism implies responsibility for its exchange and creation. We focus on Intellectual Disability (ID) as an advocacy site for individuals who are dependent on assistance with activities of daily life, and attend to the process of taking care during social justice projects. Our paper responds to current South African social justice controversies perpetrated against people who may be unable to independently mobilize against increasingly othering – even deadly – socio-political conditions. Underpinned by relational Ethics of Care, voluntary-assisted-advocacy can be a psychologically relational, intersubjective, and societal project that strives for ID citizenship-making and social justice. This paper draws on numerous interviews and a number of ethnographic observations in exploration of ID care. Empirical material was subjected to thematic content analysis, and participant quotes bring our argument to life. Relationships among people with Intellectual Disability (PWID) and non-ID assistant-advocates are asymmetrical. We can either uphold dominant non-ID voices, or transform socio-political ruling relations that maintain dependence on conditions of power and inequality. Our contributions to the advocacy we co-create today will shape the activism we will depend on in the future. We consider relational voluntary-assisted-advocacy as a psychological and ethical resource for sustainable, mutually satisfying social change.

A-67 Measuring Regional Cerebral Blood Flow with Single-Photon Emission Computed Tomography (SPECT) in Obsessive–Compulsive Disorder

Archives of Clinical Neuropsychology ◽

10.1093/arclin/acab062.85 ◽

2021 ◽

Vol 36 (6) ◽

pp. 1109-1109

Author(s):

Sophia G Perez ◽

Bailey McDonald ◽

Samantha Spagna ◽

Charles J Golden ◽

Kristen Willeumier ◽

...

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Basal Ganglia ◽

Limbic System ◽

Regional Cerebral Blood Flow ◽

Healthy Controls ◽

Regional Cerebral Blood ◽

Obsessive Compulsive ◽

Compulsive Disorder ◽

Abstract Objective To identify regional cerebral blood flow (rCBF) differences between individuals with Obsessive–Compulsive Disorder (OCD) and healthy controls. Mehtods: Healthy controls (n = 81, Mage = 41.9, 53.0% female, 42.0% Caucasian) and persons diagnosed by psychiatric examination with OCD (n = 1020, Mage = 34.8, 33.6% female, 66.3% Caucasian) were selected from a deidentified adult clinical outpatient database. Those with comorbid diagnoses were included. Significant differences (alpha = 0.001) were found for age [t(1099) = −4.4], gender [χ2(2) = 25.7], and race [χ2(12) = 30.1] between groups and therefore were controlled for. Significant rCBF differences were noted in the cerebellum [left:F(1,1096) = 21.6; right:F(1,1096) = 18.3], limbic system [left:F(1,1096) = 12.2; right:F(1,1096) = 10.4], and basal ganglia [left:F(1,1096) = 18.6; right:F(1,1096) = 18.3]. Results Group means comparisons indicated higher perfusion in the cerebellum for the OCD group. Lower perfusion was found in the limbic system and basal ganglia in the OCD group. This study found higher perfusion in the cerebellum among the OCD group. Previous research found increased rCBF in the left cerebellum in OCD before pharmacotherapy. In the right cerebellum, increased rCBF was found among participants with early-onset OCD. Conclusion Overall, there is limited research on the cerebellum because of its use as a reference point. No research was found regarding the limbic system in OCD using SPECT; however, other neuroimaging found increased amygdala reactivity to emotional face stimuli. This study found lower perfusion in the basal ganglia among the OCD group. Previous research found hypoperfusion in the right; however, hypoperfusion in the left was not significant. Updated OCD and rCBF research with SPECT are needed. Limitations included the inclusion of comorbidities and use of DSM-IV-TR rather than DSM-5 diagnosis criteria.

Brain Imaging and Treatment Response in Spasmodic Torticollis

The British Journal of Psychiatry ◽

10.1192/bjp.153.3.399 ◽

1988 ◽

Vol 153 (3) ◽

pp. 399-402 ◽

Cited By ~ 2

Author(s):

J. A. O. Besson ◽

K. P. Ebmeier ◽

H. G. Gemmell ◽

P. F. Sharp ◽

M. McFadyen ◽

...

Keyword(s):

Blood Flow ◽

Cerebral Blood Flow ◽

Basal Ganglia ◽

Drug Treatment ◽

Corpus Striatum ◽

Spasmodic Torticollis ◽

Flow Imaging ◽

Blood Flow Imaging ◽

The Right ◽

Selection Of

A patient with spasms of the neck, occurring when he turned his head to the left, responded to treatment with benzhexol. Cerebral blood flow imaging demonstrated reduced uptake in the right corpus striatum compared with the left. The study demonstrates the presence of an abnormality in the basal ganglia; it also illustrates response to drug treatment. Cerebral blood flow imaging may be useful in the detection of basal ganglia abnormalities in spasmodic torticollis and assist in the selection of cases which should be targeted for treatment with drugs.