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Medical Science Monitor : International... Feb 2022Akinetic mutism (AM) is characterized by the complete absence of spontaneous behavior (akinesia) and speech (mutism) with the preservation of executive functions for... (Review)
Review
A Review of Studies on the Role of Diffusion Tensor Magnetic Resonance Imaging Tractography in the Evaluation of the Fronto-Subcortical Circuit in Patients with Akinetic Mutism.
Akinetic mutism (AM) is characterized by the complete absence of spontaneous behavior (akinesia) and speech (mutism) with the preservation of executive functions for movements and speaking. Elucidation of the pathophysiological mechanisms or neural correlates for AM is clinically important because patients can recover from AM after medication and neuromodulation. The fronto-subcortical circuit is a critically important neural structure in the pathophysiology of AM. Using diffusion tensor tractography, a few neural tracts in the fronto-subcortical circuit can be reconstructed. This mini-review article evaluated 6 DTT-based studies on the fronto-subcortical circuit injury in patients with AM. According to these results, the neural tracts among the fronto-subcortical circuit, which are related to AM, were as follows (in decreasing order of importance): 1) the prefronto-caudate tract, 2) the prefronto-thalamic tract, and 3) the cingulum. In particular, the medial prefrontal cortex is an important brain area related to recovery from AM. However, only 6 studies on this topic have been published, and most were case reports. In addition, these studies analyzed only a few neural tracts in the fronto-subcortical circuit. Because AM is a rare disorder, studies involving a large number of subjects might be impossible. Nevertheless, an analysis of various neural tracts in the fronto-subcortical circuit is necessary. For this, reconstruction of the other neural tracts in the fronto-subcortical circuit should be performed first. This review aims to present the findings from recent studies on the role of DTT in evaluation of fronto-subcortical circuit injury in patients with AK.
Topics: Akinetic Mutism; Diffusion Magnetic Resonance Imaging; Diffusion Tensor Imaging; Humans; Neural Pathways; Prefrontal Cortex
PubMed: 35181647
DOI: 10.12659/MSM.936251 -
Frontiers in Neurology 2021Catatonia is a psychomotor syndrome common to several medical and neuropsychiatric disorders. Here, we report on the case of a 95-year-old woman who underwent a radical...
Catatonia is a psychomotor syndrome common to several medical and neuropsychiatric disorders. Here, we report on the case of a 95-year-old woman who underwent a radical change in personality characterized by sexual disinhibition, and physical and verbal aggressiveness. Over several months, she developed verbal stereotypies, gait deterioration, and double incontinence. She eventually developed mutism and an active opposition to all attempts to be fed or cared for. Benzodiazepines, olanzapine and electroconvulsive therapy were of no benefit. Magnetic resonance imaging revealed asymmetric (more severe on the right) frontotemporal, parietal, and upper brainstem atrophy. She died from sepsis without recovering from stupor seven years after the onset of symptoms. We believe that the initial behavioral disinhibition was related to the frontotemporal injury, whereas catatonic stupor reflected the progression of the degenerative process to the parietal cortices. Our case adds to the small number of cases of catatonia as a symptom of degenerative dementia. It also supports the idea that damage to the parietal cortex gives rise to pathological avoidance of which catatonic stupor represents an extreme form.
PubMed: 35115996
DOI: 10.3389/fneur.2021.798264 -
Cureus Dec 2021Delayed leukoencephalopathy in the aftermath of toxic exposure and cerebral hypoxia-ischemia is known as "delayed post-hypoxic leukoencephalopathy" (DPHL) but the name...
Delayed leukoencephalopathy in the aftermath of toxic exposure and cerebral hypoxia-ischemia is known as "delayed post-hypoxic leukoencephalopathy" (DPHL) but the name "delayed toxic-hypoxic leukoencephalopathy" (DTHL) may be more accurate if toxic and hypoxic mechanisms are both involved in the pathogenesis of delayed leukoencephalopathy. DTHL is characterized by initial recovery from toxic exposure and cerebral hypoxia-ischemia, clinical stability over a few weeks, and subsequent neurological deterioration with the sudden emergence of diffuse white matter disease. A 46-year-old man suffered respiratory failure and hypotension as a result of opioid overdose. Brain MRI showed watershed infarcts and EEG showed diffuse theta-delta slowing consistent with global cerebral hypoperfusion. He recovered fully and was discharged with intact cognitive function. Three weeks later, he presented with abulia and psychomotor retardation. MRI revealed extensive white matter hyperintensity and EEG showed diffuse polymorphic delta activity. DTHL was diagnosed based on classic MRI features, history of opioid overdose and hypoxic brain injury, and negative test results for etiology of white matter disease. He developed akinetic mutism prompting administration of methylprednisolone 1000-mg IV q24h for five days. He also received amantadine 100-mg PO q12h. His cognition, motivation, and psychomotor function slowly improved and returned to baseline about two months after the overdose. Clinic reassessment two and a half months after the overdose revealed normal cognitive function, slight residual MRI hyperintensity, and mild EEG slowing anteriorly. Toxic-metabolic myelinopathy causing diffuse demyelination in the deep white matter is a perfect explanation for the patient's neurological symptoms, MRI changes, EEG findings, and time course of recovery.
PubMed: 35004070
DOI: 10.7759/cureus.20271 -
Prion Dec 2022An 84-year-old woman who had been diagnosed as having dementia with Lewy body (DLB) upon initial examination exhibited cognitive impairments and person delusional...
An 84-year-old woman who had been diagnosed as having dementia with Lewy body (DLB) upon initial examination exhibited cognitive impairments and person delusional misidentification (DMS): she transiently claimed that her spouse was a stranger. She was re-examined at the age of 89 years; her frequency of speech and activities of daily living had both decreased, leading to verbal communication difficulties complicated by sensory aphasia, and brain diffusion-weighted (DW) magnetic resonance imaging (MRI) showed cortical hyperintensities in some areas of both hemispheres. About 4 months later, the DW high-intensity areas were observed to have expanded into diffuse cortical areas. While the clinical features of Creutzfeldt Jakob disease (CJD) (myoclonus; ataxia; parkinsonism; rapidly progressive cognitive impairments; periodic sharp discharges on electroencephalograms) were not observed, a genetic analysis of the prion protein () gene, which was performed because of a family history of dementia, revealed a V180I mutation (heterozygosis: valine/isoleucine) suggesting genetic CJD (g-CJD). Her activity progressively decreased, reaching akinetic mutism about 11 months after the re-examination. Finally, she suffered from severe bedsores and died from aspiration pneumonia at the age of 90 years. The present report describes the first case of person DMS as an initial neuropsychiatric symptom for V180I g-CJD; the typical long-term clinical symptoms of CJD were not observed in this patient. The inclusion of person DMS as an initial clinical symptom and the presence of expansive cortical hyperintensity areas may be useful for clinicians attempting to diagnosis V180I g-CJD in patients with elusive symptoms.
Topics: Activities of Daily Living; Aged, 80 and over; Creutzfeldt-Jakob Syndrome; Female; Humans; Mutation; Prion Proteins; Prions
PubMed: 34965177
DOI: 10.1080/19336896.2021.2017701 -
Neurologia (Barcelona, Spain) Oct 2022
PubMed: 34785833
DOI: 10.1016/j.nrl.2021.09.007 -
Case Reports in Psychiatry 2021. Catatonia is caused by a variety of psychiatric and organic conditions. The onset, clinical profile, and response to treatment may vary depending on the underlying...
. Catatonia is caused by a variety of psychiatric and organic conditions. The onset, clinical profile, and response to treatment may vary depending on the underlying cause. Catatonia is more likely to be associated with neurotic and psychotic disorders, but some psychiatric symptoms are key components in the clinical presentation of other medical conditions. . We report the case of a woman who started showing paroxysmal recurrent episodes since the age of 57 years, characterized by surrounding disconnection, disorientation, and muscle spasm (myoclonus), followed by a postictal state. In the following months, the symptoms evolved to akinetic mutism, catatonia, and rapidly progressive vision and audition loss. She underwent a battery of tests, most of them inconclusive, until a neoplastic meningoencephalitis was diagnosed after more than two years of symptoms. Numerous medical conditions can mimic psychiatric disorders. This uncommon presentation may lead to a late diagnosis and treatment initiation, increasing significantly morbidity and mortality. A differential diagnosis with infectious, autoimmune, and neoplastic etiologies should always be carried out.
PubMed: 34745679
DOI: 10.1155/2021/5936673 -
Wideochirurgia I Inne Techniki... Sep 2021Endoscopic methods have gained a well-established position in surgical treatment of colloid cysts of third ventricle. However, the possibility of total tumor removal...
INTRODUCTION
Endoscopic methods have gained a well-established position in surgical treatment of colloid cysts of third ventricle. However, the possibility of total tumor removal with this method and the long-term effectiveness of treatment are being questioned.
AIM
Personal twenty years' experience in treatment of third ventricle colloid cysts is presented on the basis of retrospective analysis.
MATERIAL AND METHODS
The study group included 58 patients diagnosed by neuroimaging (head CT/MRI) with third ventricle colloid cyst. Post-hospital follow-up ranged from 18 to 42 months. Long-term follow-up head CT/MRI was performed in 39 patients.
RESULTS
The colloid cyst was removed totally in 47 (81%) patients. In 11 cases, the colloid cyst's wall was tightly adherent to the roof of the third ventricle, which limited the radicality of the procedure. Sixteen patients demonstrated memory impairments, 4 patients epilepsy and another 2 akinetic mutism in the direct postoperative course. One patient died as a result of complications unrelated to the procedure. The average hospitalization was 5 days. In the late period after surgery, remission of the most, previously, reported ailments and symptoms has been reported. Surgical treatment for hydrocephalus was needed in 7 patients. In 3 cases cyst recurrence was diagnosed which required reoperation.
CONCLUSIONS
The endoscopic methods allow the total removal of a third ventricle colloid cyst in most patients. Leaving a small coagulated fragment of the cyst rarely results in its recurrence. This method results in effective treatment with a low complications rate, shortens hospitalization time and brings the patient a high level of satisfaction with a quick recovery.
PubMed: 34691312
DOI: 10.5114/wiitm.2021.103957 -
Folia Neuropathologica 2021Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy with the fatal outcome, caused by the accumulation of pathological prion protein in the central nervous... (Review)
Review
Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy with the fatal outcome, caused by the accumulation of pathological prion protein in the central nervous system (CNS). CJD is classified into four types: sporadic (sCJD), familial or genetic (fCJD), iatrogenic (iCJD) and variant form (vCJD). The recognition of CJD is based on the clinical presentation, neuroimaging, electroencephalography and biochemical tests. The hyperintense signals in basal ganglia on brain magnetic resonance imaging (MRI), periodic sharp and slow wave complexes (PSWCs) in the electroencephalogram as well as presence of neuronal proteins such as protein 14-3-3 in the cerebrospinal fluid (CSF) support the diagnosis. The definite diagnosis of CJD still demands neuropathological confirmation. We report the case of a 56-year-old woman with the rapidly progressive cognitive impairment, motor dysfunctions and the fulminant neurological deterioration to akinetic mutism during the five weeks' hospitalisation. The probable diagnosis of sCJD was based on medical history and characteristic findings in MRI. The positive result of the real-time quaking-induced conversion (RT-QuIC) test and presence of protein 14-3-3 were obtained post-mortem and definite diagnosis was confirmed by neuropathological examination. In this paper we would like to emphasize the difficulties in reaching the diagnosis and the need for a series of diagnostic examinations in different points of time to obtain the confirming results.
Topics: Brain; Creutzfeldt-Jakob Syndrome; Diagnosis, Differential; Electroencephalography; Female; Humans; Magnetic Resonance Imaging; Middle Aged
PubMed: 34628799
DOI: 10.5114/fn.2021.109429 -
Journal of the Academy of... 2021Coronavirus disease 2019 (COVID-19) has been associated with neuropsychiatric complications ranging from new-onset psychosis to delirium, dysexecutive syndromes,... (Review)
Review
BACKGROUND
Coronavirus disease 2019 (COVID-19) has been associated with neuropsychiatric complications ranging from new-onset psychosis to delirium, dysexecutive syndromes, catatonia, and akinetic mutism (AM). AM can be conceptualized as a disorder of motivation wherein patients exhibit a loss of speech and spontaneous movement, owing to disruption of underlying frontal-subcortical circuits.
OBJECTIVES
The objectives of this study were to review the concept and differential diagnosis of AM, as well as the clinical literature on AM in COVID-19 and discuss potential implications for underlying functional neuroanatomy and mechanistic pathways, as well as clinical management.
METHODS
A narrative literature review was performed using PubMed querying published articles for topics associated with AM and its occurrence in COVID-19.
RESULTS
AM has been described in case reports and a prospective cohort study of patients with COVID with neurological complaints. Three COVID-19 AM subgroups can be distinguished, including individuals with severe respiratory illness, those with meningoencephalitis, and those with delirium and pre-existing neuropsychiatric illness. Electrophysiology and functional imaging suggest COVID-19 AM may result from underlying frontal lobe dysfunction and disruption of associated distributed circuits subserving goal-directed behavior. Distinctive combinations of pathophysiological mechanisms may be at play in the different subgroups of COVID-19 AM cases.
CONCLUSION
AM has been described in association with COVID-19 and may manifest in clinically heterogenous subgroups with distinct underlying mechanisms. The diagnosis of AM and evaluation of potential etiologies can be complex. The occurrence of AM contributes evidence to the hypothesis of frontal lobe dysfunction in COVID-19.
Topics: Akinetic Mutism; COVID-19; Humans; Motivation; Prospective Studies; SARS-CoV-2
PubMed: 34461295
DOI: 10.1016/j.jaclp.2021.08.009 -
Movement Disorders Clinical Practice Jul 2021
PubMed: 34307755
DOI: 10.1002/mdc3.13230