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Proceedings of the National Academy of... Oct 2018Our perception of free will is composed of a desire to act (volition) and a sense of responsibility for our actions (agency). Brain damage can disrupt these processes,...
Our perception of free will is composed of a desire to act (volition) and a sense of responsibility for our actions (agency). Brain damage can disrupt these processes, but which regions are most important for free will perception remains unclear. Here, we study focal brain lesions that disrupt volition, causing akinetic mutism ( = 28), or disrupt agency, causing alien limb syndrome ( = 50), to better localize these processes in the human brain. Lesion locations causing either syndrome were highly heterogeneous, occurring in a variety of different brain locations. We next used a recently validated technique termed lesion network mapping to determine whether these heterogeneous lesion locations localized to specific brain networks. Lesion locations causing akinetic mutism all fell within one network, defined by connectivity to the anterior cingulate cortex. Lesion locations causing alien limb fell within a separate network, defined by connectivity to the precuneus. Both findings were specific for these syndromes compared with brain lesions causing similar physical impairments but without disordered free will. Finally, our lesion-based localization matched network localization for brain stimulation locations that disrupt free will and neuroimaging abnormalities in patients with psychiatric disorders of free will without overt brain lesions. Collectively, our results demonstrate that lesions in different locations causing disordered volition and agency localize to unique brain networks, lending insight into the neuroanatomical substrate of free will perception.
Topics: Brain; Brain Diseases; Brain Mapping; Gyrus Cinguli; Humans; Neural Pathways; Neuroimaging; Personal Autonomy; Volition
PubMed: 30275309
DOI: 10.1073/pnas.1814117115 -
Frontiers in Human Neuroscience 2022This paper presents a case in whom a differential diagnosis of akinetic mutism with a disorder of consciousness was made using diffusion tensor tractography (DTT). A...
This paper presents a case in whom a differential diagnosis of akinetic mutism with a disorder of consciousness was made using diffusion tensor tractography (DTT). A 69-year-old female patient was diagnosed with subarachnoid hemorrhage, intraventricular hemorrhage, and intracerebral hemorrhage produced by the subarachnoid hemorrhage. She exhibited impaired consciousness with a Coma Recovery Scale-Revised score of 13 until 1 month after onset. Her impaired consciousness recovered slowly to a normal state according to the Coma Recovery Scale-Revised (23 points: full score) at 7 weeks after onset. On the other hand, she exhibited the typical clinical features of akinetic mutism (no spontaneous movement [akinesia] or speech [mutism]). On the DTT performed at 1-month, the upper, and lower dorsal ascending reticular activating systems, which are related to a disorder of consciousness, showed an almost normal state. In contrast, the prefronto-caudate and prefronto-thalamic tracts, which are related to akinetic mutism, showed severe injuries. These DTT results suggested that the patient's main clinical features were not a disorder of consciousness but akinetic mutism. Therefore, DTT for the ascending reticular activating system, and the prefronto-caudate and prefronto-thalamic tracts could provide additional evidence for a differential diagnosis of DOC and AM at the early stages of stroke.
PubMed: 35280207
DOI: 10.3389/fnhum.2022.778347 -
Brain, Behavior, & Immunity - Health Aug 2021
Review
PubMed: 34002170
DOI: 10.1016/j.bbih.2021.100272 -
Annals of Rehabilitation Medicine Dec 2017Cerebellar 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...
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. Cerebello-frontal pathway reconstructed from the FMRIB Software Library showed continuity of fibers, with decreased number of fibers on qualitative analysis. These results suggest that cerebello-frontal disconnection may be a neuroanatomical mechanism of CM. Damage of brain network between occipital lobe, cingulate and cerebellum caused by hemorrhage may also have role in the mechanism of CM in our case.
PubMed: 29354585
DOI: 10.5535/arm.2017.41.6.1076 -
Surgical Neurology International 2024Resection of bilateral parasagittal meningiomas of the dominant cortex is challenging. Some postoperative consequences are difficult to predict due to their low...
BACKGROUND
Resection of bilateral parasagittal meningiomas of the dominant cortex is challenging. Some postoperative consequences are difficult to predict due to their low incidence. However, it is essential to recognize reversible symptoms. Akinetic mutism is a devastating but reversible symptom that occurs after supplementary motor area (SMA) injury. This report aims to provide more information to support the clinical progression of this syndrome.
CASE DESCRIPTION
A 47-year-old woman presented with psychomotor retardation and subtle weakness, particularly on the left side. A palpable mass was identified at the head vertex. Magnetic resonance imaging revealed bilateral parasagittal meningiomas with bone and sinus invasion of the SMA. A craniotomy was performed to remove the intracapsular tumor. Two days after the operation, the patient developed gradual deterioration in her motor function until it became a lock-in-like syndrome. Then, 1.5 months after treatment in the hospital and rehabilitation unit, she gradually improved her motor, cognitive, and psychomotor skills. Total recovery was achieved after 1 year.
CONCLUSION
Surgery for lesions involving bilateral SMA can cause akinetic mutism. The typical manifestation of this syndrome may be devastating. However, it is reversible, and patients can regain full motor and cognitive functions over time without specific treatments. It is crucial to persevere and continue to provide the best care to the patient until recovery.
PubMed: 38742016
DOI: 10.25259/SNI_130_2024 -
Frontiers in Neurology 2023During her first year of junior high school, a 12-year-old Japanese girl with Down syndrome experienced dizziness, gait disruption, paroxysmal weakness in her hands, and...
Case report: Evolution of catatonic mutism and psychotic symptoms in an adolescent with Down syndrome: transition from Down syndrome disintegrative disorder to anti-N-methyl-D-aspartate receptor encephalitis.
During her first year of junior high school, a 12-year-old Japanese girl with Down syndrome experienced dizziness, gait disruption, paroxysmal weakness in her hands, and sluggish speaking. Regular blood tests and a brain MRI revealed no abnormalities, and she was tentatively diagnosed with adjustment disorder. Nine months later, the patient experienced a subacute sickness of chest pain, nausea, sleep problem with night terrors, and delusion of observation. Rapid deterioration then developed with simultaneous fever, akinetic mutism, loss of facial expression, and urine incontinence. These catatonic symptoms improved after a few weeks after admission and treatment with lorazepam, escitalopram, and aripiprazole. After discharge, nonetheless, daytime slumber, empty eyes, paradoxical laughter, and declined verbal communication persisted. Upon confirmation of the cerebrospinal N-methyl-D-aspartate (NMDA) receptor autoantibody, methylprednisolone pulse therapy was tried, but it had little effect. Visual hallucinations and cenesthopathy, as well as suicidal thoughts and delusions of death, have predominated in the following years. Cerebrospinal IL-1ra, IL-5, IL-15, CCL5, G-CSF, PDGFbb, and VFGF were raised in the early stage of initial medical attention with nonspecific complaints, but were less prominent in the later stages of catatonic mutism and psychotic symptoms. We suggest a disease concept of progression from Down syndrome disintegrative disorder to NMDA receptor encephalitis, based on this experience.
PubMed: 37360353
DOI: 10.3389/fneur.2023.1200541 -
Biological Psychiatry Sep 2020
Topics: Akinetic Mutism; Basal Ganglia; Benzodiazepines; Betacoronavirus; COVID-19; Catatonia; Coronavirus Infections; Delirium; Diagnosis, Differential; Electroconvulsive Therapy; History; Humans; Neurobiology; Neuropsychiatry; Pandemics; Patient Care Management; Pneumonia, Viral; SARS-CoV-2; gamma-Aminobutyric Acid
PubMed: 32792053
DOI: 10.1016/j.biopsych.2020.07.001 -
Tremor and Other Hyperkinetic Movements... 2023Creutzfeldt-Jakob disease (CJD) is a rare neuro degenerative disease that is mainly characterized by rapidly progressive dementia along with a varying combination of...
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a rare neuro degenerative disease that is mainly characterized by rapidly progressive dementia along with a varying combination of myoclonus, visual, cerebellar, pyramidal/extrapyramidal and akinetic mutism. Several movement disorders phenomenologies can occurs either at onset, as presenting symptom or during the course of illness. Present study aims to characterize the clinical, radiological features and the outcome of patients with CJD with movement disorders as the forthcoming manifestation.
METHODS
Chart review of patients with CJD with movement disorders. Demographic, clinical and radiological details of the patients were reviewed.
RESULTS
25 patients (13 males) of sCJD with median age at presentation of 58 years and median duration of illness of 5 months were included in the study. According to revised CDC diagnostic criteria 1 patient was classified as definite sCJD, 20 as probable and 2 as possible CJD. Myoclonus, ataxia and parkinsonism were the most common movement disorder and chorea was the least common. Magnetic resonance imaging of brain was performed in all and basal ganglia abnormality and cortical ribboning was seen in more than two-third of cases. Electroencephalographic abnormality was noted in 21 patients with triphasic waves and periodic sharp waves seen in 7 and 6 patients respectively. Cerebrospinal fluid 14-3-3 assay was abnormal in 2 out of 4 patients. Atypical presentations were noted in the form of ataxic presentation, CBS like presentation and choreiform presentation.
CONCLUSION
Myoclonus, ataxia and parkinsonism are the most frequent movement disorders phenomenology observed in patients with sCJD.
Topics: Male; Humans; Creutzfeldt-Jakob Syndrome; Myoclonus; Ataxia; Parkinsonian Disorders
PubMed: 37152622
DOI: 10.5334/tohm.753 -
Journal of Clinical and Experimental... 2022Central pontine myelinolysis (CPM) is a rare neurological complication reported in liver transplant recipients. A 16-year-old boy with Wilson disease underwent a living...
Central pontine myelinolysis (CPM) is a rare neurological complication reported in liver transplant recipients. A 16-year-old boy with Wilson disease underwent a living donor liver transplant for acute-on-chronic liver failure. On postoperative day 7, he was noted to have diplopia, dysphagia, and bilateral lower limb weakness with wide base gait with gradual progression to akinetic mutism. Magnetic resonance imaging (MRI) brain was performed which was suggestive of CPM, and it was attributed to tacrolimus. We stopped tacrolimus, and he was started on ciclosporin. His neurological symptoms started improving after 72 hours and he had a complete clinical recovery by 6 weeks. Repeat MRI brain at 16 weeks after liver transplantation showed complete radiological resolution of the pontine lesion.
PubMed: 35535097
DOI: 10.1016/j.jceh.2021.07.011 -
WMJ : Official Publication of the State... Oct 2022COVID-19 has been associated with neurological complications, including encephalopathy and akinetic mutism.
INTRODUCTION
COVID-19 has been associated with neurological complications, including encephalopathy and akinetic mutism.
CASE PRESENTATION
A 7-year-old unvaccinated boy presented with visual hallucinations, urinary incontinence, and akinetic mutism 13 days after he was exposed to COVID-19. He had minimal respiratory symptoms, including just 1 day of fever and cough. Evaluations showed slowing on electroencephalogram, normal cerebrospinal fluid, normal brain magnetic resonance imaging, and mild sinus bradycardia. He recovered rapidly to baseline after 5 days of intravenous methylprednisolone.
DISCUSSION
COVID-19-related encephalopathy including akinetic mutism is usually found in older adult patients with more severe COVID-19 illness. Our case demonstrates that akinetic mutism can present in children with mild COVID-19 illness and that it can respond rapidly and completely to intravenous methylprednisolone.
CONCLUSIONS
COVID-19-related encephalopathy may be immune mediated. A heightened awareness of its association with COVID-19 illness should lead to earlier diagnosis and consideration of immunomodulatory therapy.
Topics: Male; Child; Humans; Aged; Akinetic Mutism; COVID-19; Magnetic Resonance Imaging; Methylprednisolone
PubMed: 36301657
DOI: No ID Found