Autoimmune Basal Ganglia Disorders

2012 ◽  
Vol 27 (11) ◽  
pp. 1470-1481 ◽  
Author(s):  
Russell C. Dale ◽  
Fabienne Brilot
Keyword(s):  
Basal Ganglia ◽  
Lupus Erythematosus ◽  
Autoimmune Disorder ◽  
Neuropsychiatric Disorders ◽  
Autoimmune Disorders ◽  
Obsessive Compulsive ◽  
Encephalitis Lethargica ◽  
Compulsive Disorder ◽  
Sydenham Chorea ◽  
Basal Ganglia Disorders

The basal ganglia are deep nuclei in the brain that include the caudate, putamen, globus pallidus, and substantia nigra. Pathological processes involving the basal ganglia often result in disorders of movement and behavior. A number of different autoimmune disorders predominantly involve the basal ganglia and can result in movement and psychiatric disorders. The classic basal ganglia autoimmune disorder is Sydenham chorea, a poststreptococcal neuropsychiatric disorder. Resurgence in the interest in Sydenham chorea is the result of the descriptions of other poststreptococcal neuropsychiatric disorders including tics and obsessive-compulsive disorder, broadly termed pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection. Encephalitic processes affecting the basal ganglia are also described including the syndromes basal ganglia encephalitis, encephalitis lethargica, and bilateral striatal necrosis. Last, systemic autoimmune disorders such as systemic lupus erythematosus and antiphospholipid syndrome can result in chorea or parkinsonism. Using paradigms learned from other autoantibody associated disorders, the authors discuss the autoantibody hypothesis and the role of systemic inflammation in autoimmune basal ganglia disorders. Identification of these entities is important as the clinician has an increasing therapeutic repertoire to modulate or suppress the aberrant immune system.

scholarly journals Anti-basal ganglia antibodies in primary obsessive–compulsive disorder: systematic review and meta-analysis

2014 ◽  
Vol 205 (1) ◽  
pp. 8-16 ◽  
Author(s):  
Daniel M. Pearlman ◽  
Haily S. Vora ◽  
Brian G. Marquis ◽  
Souhel Najjar ◽  
Lauren A. Dudley
Keyword(s):  
Basal Ganglia ◽  
Obsessive Compulsive Disorder ◽  
Meta Analysis ◽  
Experimental Studies ◽  
Neuropsychiatric Disorders ◽  
Control Group ◽  
Pool Data ◽  
Obsessive Compulsive ◽  
Eligibility Criteria ◽  
Compulsive Disorder

BackgroundAutoimmune-mediated basal ganglia dysfunction is implicated in the pathophysiology of neuropsychiatric disorders commonly manifesting with obsessive–compulsive features (e.g. Sydenham chorea). The relationship between autoimmunity and primary obsessive–compulsive disorder (OCD), however, is less clear.AimsTo pool data on serum and cerebrospinal fluid (CSF) anti-basal ganglia antibody (ABGA) positivity in primary OCD (without neurological or autoimmune comorbidity) relative to controls or neuropsychiatric disorders previously associated with increased odds of ABGA positivity.MethodWe performed electronic database and hand-searches for studies meeting pre-specified eligibility criteria from which we extracted data using a standardised form. We calculated pooled estimates of ABGA positivity using a random-effects model.ResultsSeven case–control studies totalling 844 participants met the eligibility criteria. Meta-analysis showed that a significantly greater proportion of those with primary OCD were ABGA seropositive compared with various controls (odds ratio (OR) = 4.97, 95% CI 2.88–8.55, P<0.00001). This effect was not associated with heterogeneity or publication bias, and remained significant after stratifying the analysis by age, gender, disease severity, illness duration, immunostaining methodology, study quality, publication type, kind of control group, and sample size. There were no significant differences in ABGA seropositivity for comparisons between primary OCD and Tourette syndrome, attention-deficit hyperactivity disorder or paediatric acute-onset neuropsychiatric syndrome. Results of one study testing CSF samples showed that a significantly greater proportion of participants with primary OCD were ABGA CSF-positive compared with healthy controls (OR = 5.60, 95% CI 1.04–30.20, P = 0.045).ConclusionsOdds of ABGA seropositivity are increased fivefold in primary OCD compared with controls, but are comparable to those associated with disorders previously associated with ABGA, providing circumstantial evidence of autoimmunity in a subset of those with primary OCD. Further experimental studies are needed to ascertain whether this relationship is causal.

scholarly journals Complex phonic tic and disinhibition in Tourette syndrome: case report

2001 ◽  
Vol 59 (3A) ◽  
pp. 587-589 ◽  
Author(s):  
Débora Palmini Maia ◽  
Francisco Cardoso
Keyword(s):  
Breast Cancer ◽  
Case Report ◽  
Basal Ganglia ◽  
Obsessive Compulsive Disorder ◽  
Tourette Syndrome ◽  
Limbic Cortex ◽  
Obsessive Compulsive ◽  
Compulsive Disorder ◽  
Behavioral Abnormalities ◽  
Motor Tics

Tourette syndrome (TS) is a neuropsychiatric disorder characterized by a combination of multiple motor tics and at least one phonic tic. TS patients often have associated behavioral abnormalities such as obsessive compulsive disorder, attention deficit and hyperactive disorder. Coprolalia, defined as emission of obscenities or swearing, is one type of complex vocal tic, present in 8% to 26% of patients. The pathophysiology of coprolalia and other complex phonic tics remains ill-defined. We report a patient whose complex phonic tic was characterized by repetitively saying "breast cancer" on seeing the son of aunt who suffered from this condition. The patient was unable to suppress the tic and did not meet criteria for obsessive compulsive disorder. The phenomenology herein described supports the theory that complex phonic tics result from disinhibition of the loop connecting the basal ganglia with the limbic cortex.

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

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 ◽  
The Right

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.

Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Mohammad O AL fattani ◽  
Asmaa M Al Refaie ◽  
Shuruq Hassan Alsulami ◽  
Najia Al Hojaili
Keyword(s):  
Febrile Illness ◽  
Neuropsychiatric Disorders ◽  
Age Group ◽  
Pediatric Age ◽  
Streptococcal Infections ◽  
Obsessive Compulsive ◽  
Tic Disorder ◽  
Compulsive Disorder ◽  
Pediatric Age Group ◽  
Suicidal Attempts

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a rear disease in pediatric age group which constitute a unique manifestation associated with obsessive-compulsive disorder (OCD) and tic disorder in children. However, this diagnosis has carried a lot of controversies, related mainly to its management. Here we are presenting one case of PANDAS syndrome who was treated successfully with full coarse of antibiotic for 10 days, where all his symptoms disappeared completely in subsequent days including suicidal attempts. PANDAS should be considered in children with neuropsychiatric disorders (tics, obsessive behavior etc.) especially if symptoms associated within a period of infection such as febrile illness or sore throats.

scholarly journals The Subthalamic Nucleus: Unravelling New Roles and Mechanisms in the Control of Action

2018 ◽  
Vol 25 (1) ◽  
pp. 48-64 ◽  
Author(s):  
Tora Bonnevie ◽  
Kareem A. Zaghloul
Keyword(s):  
Deep Brain Stimulation ◽  
Basal Ganglia ◽  
Subthalamic Nucleus ◽  
Action Control ◽  
Brain Disorders ◽  
Obsessive Compulsive ◽  
Compulsive Disorder ◽  
High Level ◽  
Deep Brain

How do we decide what we do? This is the essence of action control, the process of selecting the most appropriate response among multiple possible choices. Suboptimal action control can involve a failure to initiate or adapt actions, or conversely it can involve making actions impulsively. There has been an increasing focus on the specific role of the subthalamic nucleus (STN) in action control. This has been fueled by the clinical relevance of this basal ganglia nucleus as a target for deep brain stimulation (DBS), primarily in Parkinson’s disease but also in obsessive-compulsive disorder. The context of DBS has opened windows to study STN function in ways that link neuroscientific and clinical fields closely together, contributing to an exceptionally high level of two-way translation. In this review, we first outline the role of the STN in both motor and nonmotor action control, and then discuss how these functions might be implemented by neuronal activity in the STN. Gaining a better understanding of these topics will not only provide important insights into the neurophysiology of action control but also the pathophysiological mechanisms relevant for several brain disorders and their therapies.

scholarly journals Neuropsychological And Psychopathological Profile Of Anti-Nmdar Encephalitis: A Possible Pathophysiological Model For Pediatric Neuropsychiatric Disorders

2018 ◽  
Vol 34 (8) ◽  
pp. 1309-1319 ◽  
Author(s):  
Elisa Cainelli ◽  
Margherita Nosadini ◽  
Stefano Sartori ◽  
Agnese Suppiej
Keyword(s):  
Executive Functions ◽  
Psychiatric Symptoms ◽  
Autoimmune Disorder ◽  
Neuropsychiatric Disorders ◽  
Cognitive Outcome ◽  
Obsessive Compulsive ◽  
Nmdar Encephalitis ◽  
Visual Motor ◽  
Comprehensive Framework

Abstract Objective Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a severe, but treatable, autoimmune disorder, characterized by autoantibodies causing hypofunction of blocking NMDA receptors leading to a unique constellation of cognitive, motor, and psychiatric symptoms. Neuropsychological and psychopathological outcome has not been fully explored, particularly in children. Aim of this study was to investigate pediatric anti-NMDAR encephalitis as a model of impairment of the complex frontal-subcortical circuits who are implicated in several of the childhood neuropsychiatric disorders. Method Seven children diagnosed with anti-NMDAR encephalitis at our department underwent an evaluation of the global mental functioning before discharge, a neuropsychological and psychological/behavioral standardized examination within one month after discharge and subsequently were followed up longitudinally for mean 35 months (range 24–48 months). Collected neuropsychological data were evaluated retrospectively. Results Deficits in attention, executive functions and/or visual motor functions involving executive functions were seen in all children within one month after discharge. These deficits were long lasting in about a half of the patients. In addition, four patients developed persistent psychopathological dysfunctions: difficulties to regulate their own behavior, impulsivity, hyperactivity, irritability, apathy, and obsessive-compulsive symptoms. Conclusions Our data are in line with research suggesting a crucial role of the executive functions impairments in cognitive outcome disturbance of anti-NMDAR encephalitis. We found also behavioral and psychological deficits pointing to a more comprehensive framework of frontal-subcortical dysfunction, in which the NMDA mediated transmission appear to have a role, as suggested by neurobiological, pharmacological, and neuroimaging studies.

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