Longitudinal Changes After Amygdala Surgery for Intractable Aggressive Behavior: Clinical, Imaging Genetics, and Deformation-Based Morphometry Study—A Case Series

Neurosurgery ◽  
2021 ◽  
Vol 89 (Supplement_2) ◽  
pp. S97-S97
Author(s):  
Flavia Venetucci Gouveia ◽  
Jürgen Germann ◽  
Rosa de Morais ◽  
Erich Talamoni Fonoff ◽  
Clement Hamani ◽  
...  
Neurosurgery ◽  
2020 ◽  
Author(s):  
Flavia Venetucci Gouveia ◽  
Jürgen Germann ◽  
Rosa de Morais ◽  
Erich Talamoni Fonoff ◽  
Clement Hamani ◽  
...  

Abstract BACKGROUND Intractable aggressive behavior (iAB) is a devastating behavioral disorder that may affect psychiatric patients. These patients have reduced quality of life, are more challenging to treat as they impose a high caregiver burden and require specialized care. Neuromodulatory interventions targeting the amygdala, a key hub in the circuitry of aggressive behavior (AB), may provide symptom alleviation. OBJECTIVE To Report clinical and imaging findings from a case series of iAB patients treated with bilateral amygdala ablation. METHODS This series included 4 cases (3 males, 19-32 years old) who underwent bilateral amygdala radiofrequency ablation for iAB hallmarked by life-threatening self-injury and social aggression. Pre- and postassessments involved full clinical, psychiatric, and neurosurgical evaluations, including scales quantifying AB, general agitation, quality of life, and magnetic resonance imaging (MRI). RESULTS Postsurgery assessments revealed decreased aggression and agitation and improved quality of life. AB was correlated with testosterone levels and testosterone/cortisol ratio in males. No clinically significant side effects were observed. Imaging analyses showed preoperative amygdala volumes within normal populational range and confirmed lesion locations. The reductions in aggressive symptoms were accompanied by significant postsurgical volumetric reductions in brain areas classically associated with AB and increases in regions related to somatosensation. The local volumetric reductions are found in areas that in a normal brain show high expression levels of genes related to AB (eg, aminergic transmission) using gene expression data provided by the Allen brain atlas. CONCLUSION These findings provide new insight into the whole brain neurocircuitry of aggression and suggest a role of altered somatosensation and possible novel neuromodulation targets.


2017 ◽  
Vol 19 (2) ◽  
pp. 182-195 ◽  
Author(s):  
Friederike Knerlich-Lukoschus ◽  
Mary B. Connolly ◽  
Glenda Hendson ◽  
Paul Steinbok ◽  
Christopher Dunham

OBJECTIVE Focal cortical dysplasia (FCD) Type II is divided into 2 subgroups based on the absence (IIA) or presence (IIB) of balloon cells. In particular, extratemporal FCD Type IIA and IIB is not completely understood in terms of clinical, imaging, biological, and neuropathological differences. The aim of the authors was to analyze distinctions between these 2 formal entities and address clinical, MRI, and immunohistochemical features of extratemporal epilepsies in children. METHODS Cases formerly classified as Palmini FCD Type II nontemporal epilepsies were identified through the prospectively maintained epilepsy database at the British Columbia Children's Hospital in Vancouver, Canada. Clinical data, including age of seizure onset, age at surgery, seizure type(s) and frequency, affected brain region(s), intraoperative electrocorticographic findings, and outcome defined by Engel's classification were obtained for each patient. Preoperative and postoperative MRI results were reevaluated. H & E–stained tissue sections were reevaluated by using the 2011 International League Against Epilepsy classification system and additional immunostaining for standard cellular markers (neuronal nuclei, neurofilament, glial fibrillary acidic protein, CD68). Two additional established markers of pathology in epilepsy resection, namely, CD34 and α-B crystallin, were applied. RESULTS Seven nontemporal FCD Type IIA and 7 Type B cases were included. Patients with FCD Type IIA presented with an earlier age of epilepsy onset and slightly better Engel outcome. Radiology distinguished FCD Types IIA and IIB, in that Type IIB presented more frequently with characteristic cortical alterations. Nonphosphorylated neurofilament protein staining confirmed dysplastic cells in dyslaminated areas. The white-gray matter junction was focally blurred in patients with FCD Type IIB. α-B crystallin highlighted glial cells in the white matter and subpial layer with either of the 2 FCD Type II subtypes and balloon cells in patients with FCD Type IIB. α-B crystallin positivity proved to be a valuable tool for confirming the histological diagnosis of FCD Type IIB in specimens with rare balloon cells or difficult section orientation. Distinct nonendothelial cellular CD34 staining was found exclusively in tissue from patients with MRI-positive FCD Type IIB. CONCLUSIONS Extratemporal FCD Types IIA and IIB in the pediatric age group exhibited imaging and immunohistochemical characteristics; cellular immunoreactivity to CD34 emerged as an especially potential surrogate marker for lesional FCD Type IIB, providing additional evidence that FCD Types IIA and IIB might differ in their etiology and biology. Although the sample number in this study was small, the results further support the theory that postoperative outcome—defined by Engel's classification—is multifactorial and determined by not only histology but also the extent of the initial lesion, its location in eloquent areas, intraoperative electrocorticographic findings, and achieved resection grade.


2019 ◽  
Author(s):  
Flavia V. Gouveia ◽  
Jürgen Germann ◽  
Rosa M. C. B. de Morais ◽  
Erich T. Fonoff ◽  
Clement Hamani ◽  
...  

ABSTRACTIntractable aggressive behaviour is a devastating behavioural disorder that reach 30% of psychiatric aggressive patients. Neuromodulatory surgeries may be treatment alternatives to reduce suffering. We investigated the outcomes of bilateral amygdala radiofrequency ablation in four patients with intractable aggressive behaviour (life-threatening-self-injury and social aggression) by studying whole brain magnetic resonance imaging and clinical data. Post-surgery assessments revealed decreases in aggression and agitation and improvements in quality of life. Aggressive behaviour was positively correlated with serum testosterone levels and the testosterone/cortisol ratio in males. No clinically significant side effects were observed. Imaging analyses revealed preoperative amygdala volumes within normal range and confirmed appropriate lesion locations. Reduction in aggressiveness were accompanied by volumetric reduction in brain areas associated with aggressive behaviour (express genes related to aggressive behaviour), and increases in regions related to somatosensation. These findings further elucidates the neurocircuitry of aggression and suggests novel neuromodulation targets.


2021 ◽  
Vol 6 ◽  
pp. 247275122110337
Author(s):  
Wilber E. Bernaola-Paredes ◽  
Estefani Albuja-Rivadeneira ◽  
Franco Novelli ◽  
José Francisco Dalcin ◽  
José Guilherme Vartanian ◽  
...  

Study Design: Ameloblastoma is a benign odontogenic tumor of epithelial origin, with locally aggressive behavior; and due to its invasiveness has increased recurrence rates. It is more frequent in the mandible than the maxilla. Surgical treatment is performed with approaches ranging from conservative to radical surgery, either followed by microvascular reconstruction, or not. Adjuvant treatment has shown better local control in refractory cases. Multiple relapses are associated with BRAF gene mutation at codon 600. Objective: This case series aimed to describe the clinical, imaging, histopathological, therapeutic and mutational characterization of 3 patients with refractory ameloblastoma in the maxilla. Methods: Data of 3 patients were collected, and descriptions were provided of procedures such as clinical, imaging, surgical technique, histopathological subtype and molecular analysis for detection of BRAFV600E mutation. Results It was obtained a locoregional control after RT of two cases described. After BRAF mutation molecular analysis, no patient presented it. Conclusion: Surgery remains the gold standard for the treatment of ameloblastomas, even in refractory cases; however, an expanded approach to obtain free surgical margins, and reconstruction of the maxilla itself may be challenging. Adjuvant radiotherapy is still a controversial topic, but could favor reduction of the local recurrence rate in cases where the surgical margins are compromised after surgical resection. Further studies will be necessaries for analysis of the BRAFV600E mutation, for therapeutic purposes.


2020 ◽  
Vol 10 (6) ◽  
pp. 340 ◽  
Author(s):  
Jannis Achenbach ◽  
Charlotte Thiels ◽  
Thomas Lücke ◽  
Carsten Saft

Background: Studies on the clinical manifestation and course of disease in children suffering from Huntington’s disease (HD) are rare. Case reports of juvenile HD (onset ≤ 20 years) describe heterogeneous motoric and non-motoric symptoms, often accompanied with a delay in diagnosis. We aimed to describe this rare group of patients, especially with regard to socio-medical aspects and individual or common treatment strategies. In addition, we differentiated between juvenile and the recently defined pediatric HD population (onset < 18 years). Methods: Out of 2593 individual HD patients treated within the last 25 years in the Huntington Centre, North Rhine-Westphalia (NRW), 32 subjects were analyzed with an early onset younger than 21 years (1.23%, juvenile) and 18 of them younger than 18 years of age (0.69%, pediatric). Results: Beside a high degree of school problems, irritability or aggressive behavior (62.5% of pediatric and 31.2% of juvenile cases), serious problems concerning the social and family background were reported in 25% of the pediatric cohort. This includes an attempted rape and robbery at the age of 12, as problems caused by the affected children, but also alcohol-dependency in a two-year-old induced by a non-HD affected stepfather. A high degree of suicidal attempts and ideations (31.2% in pediatric and 33.3% in juvenile group) was reported, including drinking of solvents, swallowing razor blades or jumping from the fifth floor with following incomplete paraparesis. Beside dopaminergic drugs for treatment of bradykinesia, benzodiazepines and tetrabenazine for treatment of dystonia, cannabinoids, botulinum toxin injection and deep brain stimulation were used for the improvement of movement disorders, clozapine for the treatment of tremor, and dopa-induced hallucinations and zuclopenthixole for the treatment of severe aggressive behavior. Conclusions: Beside abnormalities in behavior from an early age due to HD pathology, children seem to have higher socio-medical problems related to additional burden caused by early affected parents, instable family backgrounds including drug abuse of a parent or multiple changes of partners. Treatment required individualized strategies in many cases.


Children ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. 121
Author(s):  
Marco Colizzi ◽  
Riccardo Bortoletto ◽  
Leonardo Zoccante

Children and adolescents with Tourette syndrome may suffer from comorbid psychological and behavioral difficulties, primarily Attention-Deficit Hyperactivity Disorder-related manifestations including impulsive, aggressive, and disruptive behavior, and Obsessive-Compulsive Disorder-related disturbances. Often, such additional problems represent the major cause of disability, requiring their prioritization above the tic symptomatology. Here, we present six cases of children and adolescents with treatment-resistant Tourette syndrome aged 11–17 years, whose symptoms, especially the non-tic symptoms such as aggressive behavior and obsessive symptoms, failed to respond adequately to at least two different antipsychotics and, where deemed appropriate, to a combination with a medication with a different therapeutic indication or chemical class (e.g., antidepressant or anticonvulsant). Such symptomatic manifestations were significantly reduced by the time of the subsequent control visit planned 30 days later, by using lurasidone as an add-on therapy to risperidone or aripiprazole (all p ≤ 0.009). No significant neuromotor or metabolic side effects were reported in all cases in a follow-up period ranging from 4 months to 6 months, supporting the stability of the observed clinical improvement. While still investigational, the preliminary evidence presented here gives reason to hope that lurasidone could possibly be an effective option in Tourette syndrome, warranting further investigation of its potential benefits in neurodevelopmental conditions.


2020 ◽  
Vol 28 ◽  
pp. 1-23 ◽  
Author(s):  
Paulo Victor Sgobbi de Souza ◽  
Bruno de Mattos Lombardi Badia ◽  
Eduardo Augusto Gonçalves ◽  
Igor Braga Farias ◽  
Wladimir Bocca Vieira de Rezende Pinto ◽  
...  

Introduction. Inclusion body myositis represents the most common acquired myopathy in clinical practice in patients over 50 years old. Despite classical approach to this myopathy as an inflammatory disorder, a muscle degenerative disorder is now considered the main mechanism linked to these vacuolar myopathies. Hereditary presentations, although quite rare, represent an expanding and underrecognized group in clinical practice. Objective. perform a structured review of the current literature regarding hereditary inclusion body myopathies. Method. review of U.S. NLM PubMed and MEDLINE database of original articles, case reports, case series and review articles including the terms “inclusion body myositis” OR “inclusion body myopathy” AND “genetics” OR “hereditary”. Results. We present in this article a wide review regarding the main clinical, imaging, pathophysiological, genetic and therapeutic aspects related to hereditary myopathies linked to seven different clinical and genetic presentations (GNE, MATR3, VCP, SQSTM1, MYH2, HNRNPA2B1 and HNRNPA1). Conclusion. Hereditary inclusion body myopathy is associated with at least 7 distinct clinic and genetic monogenic forms.


2017 ◽  
Vol 90 (1) ◽  
pp. 13-17 ◽  
Author(s):  
Liviu Ghervan ◽  
Andreea Zaharie ◽  
Bogdan Ene ◽  
Florin I. Elec

Small-cell carcinoma of the urinary bladder is a very rare pathology, but with a very aggressive behavior and disappointing prognosis. The literature concerning this type of cancer is scarce and physicians may encounter difficulty trying to manage it. Most articles involve the study of case series, without definite results due to the small number of patients. The present article aims at gathering the most significant articles and results in order to offer a broad perspective on the existing literature concerning this pathology.


2020 ◽  
Vol 10 (1) ◽  
pp. 11-21
Author(s):  
Andi Buanasari ◽  
Budi Anna Keliat ◽  
Herni Susanti

Background: Aggressive behavior frequently occurs in clients with schizophrenia and causes injuries to the clients themselves, others, and the environment. It becomes one of the most common factors causing rehospitalization in schizophrenic clients. Aggressive behavior can be managed by the intervention administered by nurses in the usual way (treatment as usual; TAU) as well as psychotherapy (acceptance and commitment therapy; ACT and family psychoeducation; FPE).Purpose: This study aimed to investigate the effectiveness of acceptance and commitment therapy (ACT) and family psychoeducation (FPE) on schizophrenic clients with aggressive behavior.Methods: This study used a case series method to report four selected cases of schizophrenic clients with aggressive behavior. Acceptance and commitment therapy (ACT), family psychoeducation (FPE), and treatment as usual (TAU) were delivered to four clients with aggressive behavior for six weeks using the Stuart Stress-Adaptation Model.Results: The finding showed decreased symptoms of aggressive behavior in cognitive, affective, physiological, behavioral, and social aspects and increased ability to control anger, to accept their problems, and to commit to the therapy after ACT, FPE, and TAU interventions.Conclusion: This report showed that TAU, ACT, and FPE effectively decreased the symptoms of aggressive behavior and increased the clients’ ability to control anger.


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