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Schizophrenia Research Jan 2024Catatonia occurs secondary to both primary psychiatric and neuromedical etiologies. Emerging evidence suggests possible linkages between causes of catatonia and...
Catatonia occurs secondary to both primary psychiatric and neuromedical etiologies. Emerging evidence suggests possible linkages between causes of catatonia and neuroinflammation. These include obvious infectious and inflammatory etiologies, common neuromedical illnesses such as delirium, and psychiatric entities such as depression and autism-spectrum disorders. Symptoms of sickness behavior, thought to be a downstream effect of the cytokine response, are common in many of these etiologies and overlap significantly with symptoms of catatonia. Furthermore, there are syndromes that overlap with catatonia that some would consider variants, including neuroleptic malignant syndrome (NMS) and akinetic mutism, which may also have neuroinflammatory underpinnings. Low serum iron, a common finding in NMS and malignant catatonia, may be caused by the acute phase response. Cellular hits involving either pathogen-associated molecular patterns (PAMP) danger signals or the damage-associated molecular patterns (DAMP) danger signals of severe psychosocial stress may set the stage for a common pathway immunoactivation state that could lower the threshold for a catatonic state in susceptible individuals. Immunoactivation leading to dysfunction in the anterior cingulate cortex (ACC)/mid-cingulate cortex (MCC)/medial prefrontal cortex (mPFC)/paralimbic cortico-striato-thalamo-cortical (CSTC) circuit, involved in motivation and movement, may be particularly important in generating the motor and behavioral symptoms of catatonia.
Topics: Humans; Catatonia; Neuroleptic Malignant Syndrome
PubMed: 36966063
DOI: 10.1016/j.schres.2023.03.013 -
Neuropsychological Rehabilitation Mar 2018Following a description about the characteristics of akinetic mutism (AM) and how it differs from locked-in syndrome (LIS) and a disorder of consciousness (DOC), we...
Following a description about the characteristics of akinetic mutism (AM) and how it differs from locked-in syndrome (LIS) and a disorder of consciousness (DOC), we present the case of David, a 71-year-old man with AM. David sustained a stroke following a middle cerebral artery (MCA) thrombus. A CT scan at the time detected old ischaemic infarcts affecting the right frontal lobe, the left occipital lobe and the left parietal lobe so David had bilateral brain damage. Initially thought to have a DOC, further neuropsychological assessments administered when David had improved a little, resulted in the diagnosis of AM. Although David spoke little, when he did speak, his words and phrases were well articulated, grammatical and with appropriate intonation. He was alert and visually aware and he was not paralysed. We discuss whether the diagnosis was correct and address the difficulties in assessing such patients.
Topics: Aged; Akinetic Mutism; Humans; Male; Neuropsychological Tests; Stroke
PubMed: 28150524
DOI: 10.1080/09602011.2017.1281147 -
Neurocase Feb 2024We report on a patient with delayed post-hypoxic leukoencephalopathy (DPHL) who showed akinetic mutism and gait disturbance, neural injuries that were demonstrated on...
We report on a patient with delayed post-hypoxic leukoencephalopathy (DPHL) who showed akinetic mutism and gait disturbance, neural injuries that were demonstrated on diffusion tensor tractography (DTT). A patient was exposed to carbon monoxide (CO) and rapidly recovered; however, two weeks after onset, he began to show cognitive impairment and gait disturbance. At six weeks after CO exposure, he showed akinetic mutism and gait inability. DTT at 6-weeks post-exposure showed discontinuations in neural connectivities of the caudate nucleus to the medial prefrontal and orbitofrontal cortex in both hemispheres. In addition, the corticoreticulospinal tract revealed severe thinning in both hemispheres.
Topics: Humans; Akinetic Mutism; Male; Leukoencephalopathies; Gait Disorders, Neurologic; Diffusion Tensor Imaging; Hypoxia, Brain; Middle Aged; Adult
PubMed: 38725351
DOI: 10.1080/13554794.2024.2353125 -
Neurological Sciences : Official... Aug 2023
Topics: Humans; Tacrolimus; Akinetic Mutism; Dysarthria; Liver Transplantation; Immunosuppressive Agents
PubMed: 36949300
DOI: 10.1007/s10072-023-06760-6 -
BMC Neurology Oct 2023Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and ultimately fatal neurodegenerative condition caused by prions. The clinical symptoms of CJD vary with its...
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and ultimately fatal neurodegenerative condition caused by prions. The clinical symptoms of CJD vary with its subtype, and may include dementia, visual hallucinations, myoclonus, ataxia, (extra)pyramidal signs and akinetic mutism. In the early course of disease however, several clinical symptoms of CJD may mimic those of co-existing morbidities.
CASE PRESENTATION
We report a male in his 60s with a history of situs inversus totalis and Churg Strauss syndrome, who presented with speech fluency disturbances, neuropsychiatric symptoms and allodynia, a few months after becoming a widower. Initially presumed a bereavement disorder along with a flare-up of Churg Strauss, his symptoms gradually worsened with apraxia, myoclonic jerks and eventually, akinetic mutism. MRI revealed hyperintensities at the caudate nucleus and thalami, while the cerebrospinal fluid was positive for the 14-3-3 protein and the real-time quick test, making the diagnosis of CJD highly probable. This case illustrates the complexities that may arise in diagnosing CJD when pre-existing multimorbidity may cloud the clinical presentation. We also discuss the potential mechanisms underlying the co-occurrence of three rare conditions (situs inversus totalis, Churg Strauss syndrome, CJD) in one patient, taking into consideration the possibility of coincidence as well as common underlying factors.
CONCLUSIONS
The diagnosis of CJD may be easily missed when its clinical symptoms are obscured by those of pre-existing (rare) multimorbidity. This case highlights that when the multimorbidity has neurological manifestations, an extensive evaluation remains crucial to establish the diagnosis, minimize the risk of prion-transmission and provide appropriate guidance to patients and their caregivers.
Topics: Humans; Male; Creutzfeldt-Jakob Syndrome; Akinetic Mutism; Churg-Strauss Syndrome; Multimorbidity; Myoclonus; Situs Inversus
PubMed: 37784069
DOI: 10.1186/s12883-023-03401-5 -
MEDICC Review Oct 2016The study of consciousness disorders is a scientific challenge, and clinical differentiation among the various sorts of alterations in consciousness is difficult.... (Review)
Review
The study of consciousness disorders is a scientific challenge, and clinical differentiation among the various sorts of alterations in consciousness is difficult. Persistent vegetative state was defined in 1972, but years later cases appeared in which diagnosed patients showed signs of cognitive activity, and therefore could not be considered vegetative. Minimally conscious state was defined in 2002. This article discusses minimally conscious state based on a literature review and the author's clinical experience. A brief historical outline is given, starting from 1886 when Horsley analyzed level of consciousness. The article reviews criteria for defining minimally conscious state, as well as the differential diagnosis from persistent vegetative state, brain death, coma, locked-in syndrome and akinetic mutism. Modern discoveries of residual cognitive functioning and new neural correlates have contributed to increased knowledge of this condition. Regardless, minimally conscious state continues to be a challenge for neuroscientists around the world, with issues still to be resolved. KEYWORDS Consciousness, consciousness disorders, minimally conscious state, persistent vegetative state, coma, akinetic mutism, brain death, neurosciences, locked-in syndrome, coma, Cuba.
Topics: Brain Death; Coma; Diagnosis, Differential; Humans; Persistent Vegetative State; Quadriplegia
PubMed: 27829654
DOI: 10.37757/MR2016.V18.N4.9 -
Annals of Clinical and Translational... Apr 2021Many neurological manifestations are associated with COVID-19, including a distinct form of encephalopathy related to cytokine storm, the acute systemic inflammatory... (Review)
Review
OBJECTIVE
Many neurological manifestations are associated with COVID-19, including a distinct form of encephalopathy related to cytokine storm, the acute systemic inflammatory syndrome present in a subgroup of COVID-19 patients. Cytokine storm is also associated with immune effector cell-associated neurotoxicity syndrome (ICANS), a complication of chimeric antigen receptor T-cell (CAR-T) therapy, a highly effective treatment for refractory hematological malignancies. We investigated whether COVID-19-related encephalopathy, ICANS, and other encephalopathies associated with cytokine storm, share clinical and investigative findings.
METHODS
Narrative literature review.
RESULTS
Comparisons between COVID-19-related encephalopathy and ICANS revealed several overlapping features. Clinically, these included dysexecutive syndrome, language disturbances, akinetic mutism and delirium. EEG showed a prevalence of frontal abnormalities. Brain MRI was often unrevealing. CSF elevated cytokine levels have been reported. A direct correlation between cytokine storm intensity and severity of neurological manifestations has been shown for both conditions. Clinical recovery occurred spontaneously or following immunotherapies in most of the patients. Similar clinical and investigative features were also reported in other encephalopathies associated with cytokine storm, such as hemophagocytic lymphohistiocytosis, sepsis, and febrile infection-associated encephalopathies.
INTERPRETATION
COVID-19-related encephalopathy and ICANS are characterized by a predominant electro-clinical frontal lobe dysfunction and share several features with other encephalopathies associated with cytokine storm, which may represent the common denominator of a clinical spectrum of neurological disorders. Therefore, we propose a unifying definition of cytokine storm-associated encephalopathy (CySE), and its diagnostic criteria.
Topics: Brain; Brain Diseases; COVID-19; Cytokine Release Syndrome; Humans; Immunotherapy, Adoptive; Receptors, Chimeric Antigen
PubMed: 33780166
DOI: 10.1002/acn3.51348 -
Clinical Neurology and Neurosurgery Jul 2024Cerebellar mutism syndrome (CMS) is a serious complication of posterior fossa surgeries affecting mainly pediatric age group. The pathophysiology is still not fully... (Review)
Review
INTRODUCTION
Cerebellar mutism syndrome (CMS) is a serious complication of posterior fossa surgeries affecting mainly pediatric age group. The pathophysiology is still not fully understood. It adversely affects the recovery of patients. There is no definitive and standardized management for CMS. However pharmacological therapy has been used in reported cases with variable effectiveness. We aim through this review to summarize the available evidence on pharmacological agents used to treat CMS.
METHOD
A thorough systematic review until December 2022, was conducted using PubMed Central, Embase, and Web of Science, databases to identify case reports and case series of CMS patients who underwent posterior fossa surgery and received pharmacological treatment. Patients with pathologies other than posterior fossa lesions were excluded from the study.
RESULTS
Of 592 initial studies, 8 studies met our eligibility criteria for inclusion, with 3 more studies were added through manual search; reporting on 13 patients. The median age of 13 years (Standard deviation SD=10.60). The most frequent agent used was Bromocriptine. Other agents were fluoxetine, midazolam, zolpidem, and arpiprazole. Most patients recovered within 48 hours of initiating medical therapy. The median follow-up period was 4 months (SD=13.8). All patients showed complete recovery at the end of follow-up period.
CONCLUSION
Cerebellar mutism syndrome is reported after posterior fossa surgeries, despite attempts to identify risk factors, pathophysiology, and management of CMS, it remains a challenging condition with significant morbidity. Different Pharmacological treatments have been proposed with promising results. Further studies and formalized clinical trials are needed to evaluate available options and their effectiveness.
Topics: Humans; Mutism; Neurosurgical Procedures; Postoperative Complications; Cranial Fossa, Posterior; Cerebellar Diseases; Child; Adolescent
PubMed: 38823197
DOI: 10.1016/j.clineuro.2024.108352 -
Handbook of Clinical Neurology 2019The catatonic syndrome is an example of a multifactorial neurobehavioral disorder that causes much morbidity and mortality but also has the potential to unlock the... (Review)
Review
The catatonic syndrome is an example of a multifactorial neurobehavioral disorder that causes much morbidity and mortality but also has the potential to unlock the mystery of how motivation and movement interact to produce behavior. In this chapter, an attempt is made to understand better the catatonic syndrome through the lens of neurobiology and neuropathophysiology updated by recent studies in molecular biology, genomics, inflammasomics, neuroimaging, neural network theory, and neuropsychopathology. This will result in a neurostructural model for the catatonic syndrome that centers on paralimbic regions including the anterior and midcingulate cortices, as they interface with striatal and thalamic nodes in the salience decision-making network. Examination of neurologic disorders like the abulic syndrome, which includes in its extreme catatonic form, akinetic mutism, will identify the cingulate cortex and paralimbic neighbors as regions of interest. This exploration has the potential to unlock mysteries of the brain cascade from motivation to movement and to clarify catatonia therapeutics. Such a synthesis may also help us discern meaning inherent in this complex neurobehavioral syndrome.
Topics: Basal Ganglia; Catatonia; Gyrus Cinguli; Humans; Thalamus
PubMed: 31731912
DOI: 10.1016/B978-0-444-64196-0.00012-1 -
The Journal of the Royal College of... Mar 2023Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of...
Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, visual disturbances, extrapyramidal signs and eventually akinetic mutism. Most patients present with varied clinical presentation, hence making it difficult to diagnose at an early stage. We report five cases of sporadic Creutzfeldt-Jakob disease presenting to a Tasmanian hospital in Australia over a period of 52 months. We highlight significant clinical features in all our patients including few atypical presentations, emphasise on relevant clinical biomarkers and illustrate characteristic abnormalities on electroencephalogram and neuroimaging.
Topics: Humans; Biomarkers; Creutzfeldt-Jakob Syndrome; Neurodegenerative Diseases; Tasmania
PubMed: 36680361
DOI: 10.1177/14782715221150580