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Movement Disorders Clinical Practice Jul 2021
PubMed: 34307755
DOI: 10.1002/mdc3.13230 -
Turkish Neurosurgery Aug 2020Telovelar and transvermian approaches for medulloblastoma excision have high complication and recurrence rates. This study aims to describe a novel surgical approach to...
AIM
Telovelar and transvermian approaches for medulloblastoma excision have high complication and recurrence rates. This study aims to describe a novel surgical approach to decrease such rates.
MATERIAL AND METHODS
A retrospective review was performed. The novel technique was performed in similar conditions for all patients. It involves early intra-operative identification of the superior part of the floor of the fourth ventricle, so that the inferior part of the tumour can be viewed directly and excised thoroughly. Importance was given to the pattern of tumour growth and CSF flow dynamics.
RESULTS
A total of 58 patients underwent this surgery between February-2006 and May-2016. Mean age was 13.2 years (Range - 6 months to 55 years). Follow-up ranged from 1 to 11 years. 49 patients (84.4%) who were under the age of 3 years were administered craniospinal radiation as well as chemotherapy, while 9 patients (15.6%) over the age of 3 years were administered only chemotherapy. Total excision could be performed in 50 cases (86.2%) and subtotal excision ( 90% excision) in 8 cases (13.8%). The tumour recurred in only 1 patient (1.72%). The complications included akinetic mutism (8.6%), meningitis (8.6%), 6th and 7th nerve paresis/palsy (5.17%), and chest infection (3.44%). Death occurred in 2 patients (3.44%), causes of death were unrelated to surgery.
CONCLUSION
This study highlights the possible benefits of this novel approach to medulloblastoma excision by decreasing recurrence and complication rates and increasing rates of total excision. However, studies with large cohorts need to be performed to evaluate its efficacy.
PubMed: 34169985
DOI: 10.5137/1019-5149.JTN.30670-20.3 -
Brain and Behavior Aug 2021Transcranial magnetic stimulation is a noninvasive treatment used to modulate cortical excitability. Its use over the last two decades has expanded, ranging from...
BACKGROUND
Transcranial magnetic stimulation is a noninvasive treatment used to modulate cortical excitability. Its use over the last two decades has expanded, ranging from psychiatric disorders to traumatic brain injury and poststroke rehabilitation.
OBJECTIVES
We present the case of a 59-year-old male patient who presented in a decreased state of consciousness due to a right frontal glioblastoma, wherein his state was not improved by a successful surgery and could not be explained by any other condition. Due to his poor prognosis, we examine the benefits of receiving transcranial magnetic stimulation treatment to improve his akinetic mutism.
METHODS
We utilized independent component analysis with resting-state functional magnetic resonance imaging (rsfMRI) to better understand his cortical functionality. The imaging suggested absence of the default mode network (DMN). The patient underwent five sessions of navigated intermittent theta burst stimulation to the ipsilesional inferior parietal lobule and inferior frontal gyrus, with the aim of improving his default mode network functionality.
RESULTS
No other treatments resulted in an improvement of this patient's condition; however, 3 weeks following transcranial magnetic stimulation treatment, the patient was more alert and interactive, and his follow-up rsfMRI scan demonstrated a partially intact default mode network.
CONCLUSION
This case raises important questions regarding the clinical utility of transcranial magnetic stimulation to improve the connectivity of important cerebral networks and subsequent related functional recovery.
Topics: Akinetic Mutism; Brain; Default Mode Network; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Parietal Lobe; Transcranial Magnetic Stimulation
PubMed: 34145791
DOI: 10.1002/brb3.2180 -
Prion Dec 2021Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD...
Swallowing function in long-term survivors of Creutzfeldt-Jakob disease (CJD) has not been elucidated. Herein, we report a patient with MM2-cortical-type sporadic CJD (MM2C-type sCJD) with long-term preservation of pharyngeal swallowing function using videofluoroscopic (VF) examination of swallowing. A 55-year-old woman was admitted to hospital because of dyscalculia and memory disturbance 3 years after the onset of these symptoms. Neurological examination revealed dementia, extrapyramidal signs, and delusion. Diffusion-weighted MRI revealed bilateral hyperintensity in the basal ganglia and frontal, temporal, and parietal cortices. No mutation with the methionine homozygote at codon 129 was found on PRNP gene analysis. VF was performed 68 months after the onset. Although bolus transport from the oral cavity to the pharynx worsened, the pharyngeal swallowing function was preserved even 68 months after onset. Serial MRI examinations revealed no apparent atrophy of the brainstem. Single photon emission computed tomography revealed that the regional cerebral blood flow in the brainstem was preserved. These findings suggest that pseudobulbar palsy is the pathophysiology underlying dysphagia in long-term survivors of MM2C-type sCJD, probably owing to preserved brainstem function even in a state of akinetic mutism.
Topics: Creutzfeldt-Jakob Syndrome; Deglutition; Diffusion Magnetic Resonance Imaging; Female; Humans; Middle Aged; Pharynx; Tomography, Emission-Computed, Single-Photon
PubMed: 34078217
DOI: 10.1080/19336896.2021.1930851 -
Brain, Behavior, & Immunity - Health Aug 2021
Review
PubMed: 34002170
DOI: 10.1016/j.bbih.2021.100272 -
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 -
Brain and Behavior Apr 2021The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis...
BACKGROUND
The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis during the early stage. So far, there are limited data related to SSPE in preschool children.
METHODS
In order to summarize the clinical data and evolution of SSPE in preschool children and to expand the phenotypes of SSPE, the medical charts of preschool patients diagnosed with SSPE were retrospectively reviewed and analyzed; the clinical outcomes of the enrolled cases were evaluated and followed up.
RESULTS
Overall, we included three cases in the study. Their onset age was 5 years and 2 months, 4 years and 3 months, and 4 years and 2 months, respectively. All patients presented drop attacks or jerks as the onset symptom, and one patient had concurrent gait disturbance. Atypical periodic complexes on electroencephalography (EEG) were recorded in all patients. The brain magnetic resonance imaging (MRI) findings of two cases showed demyelinating lesions predominantly on the white matter. The neurological conditions of all cases deteriorated rapidly. Two children died at 21 months and 6 months after onset, respectively. The other case progressively developed vegetative status and akinetic mutism within 4 months.
CONCLUSIONS
In younger children, the characteristic features of SSPE may be seizures and gait instability as onset manifestations, atypical periodic complexes on EEG, and rapid worsening of neurological conditions.
Topics: Child, Preschool; Electroencephalography; Humans; Magnetic Resonance Imaging; Retrospective Studies; Seizures; Subacute Sclerosing Panencephalitis
PubMed: 33543580
DOI: 10.1002/brb3.2051 -
Cureus Jan 2021Pediatric akinetic mutism syndrome is a clinical disease resulting from cerebellar injury and characterized by the absence of speech or reduced speech, emotional... (Review)
Review
Pediatric akinetic mutism syndrome is a clinical disease resulting from cerebellar injury and characterized by the absence of speech or reduced speech, emotional lability, there may also be hypotonia, oropharyngeal dysfunction/dysphagia, bladder and intestinal incontinence, or other behavioral disorders and neurological signals. It is described as the most recurrent complication in children, after posterior fossa tumor surgery, mainly related to cerebellar midline injuries. An increasing number of research and prospective reviews have provided valuable information on cerebellar mutism syndrome in recent years. The purpose of this review was to elucidate the pathophysiological basis and the predictive factors for this syndrome. Most cases of mutism are due to injury cerebellar tracts and cerebellar-cerebral circuits, involving particularly distinct points of the dentate-thalamus-cortical and dentato-rubro-thalamus-cortical. Advanced neuroimaging techniques, such as tractography and perfusion studies, have contributed to demonstrating changes in these pathways in patients with pediatric cerebellar mutism.
PubMed: 33542880
DOI: 10.7759/cureus.12593 -
Prion Dec 2021Methionine/methionine type 1 (MM1-type) sporadic Creutzfeldt-Jakob disease (sCJD), known as the 'classic type,' shows typical clinicopathological sCJD findings. In...
Methionine/methionine type 1 (MM1-type) sporadic Creutzfeldt-Jakob disease (sCJD), known as the 'classic type,' shows typical clinicopathological sCJD findings. In general, patients reach an akinetic mutism state within a few months of disease onset and die soon after if supportive therapies are not administered. Here, we describe remarkable neuropathologic observations of MM1-type sCJD in a 48-year-old, Japanese man with an unusually prolonged akinetic mutism state. In the early disease stages, the patient exhibited abnormal behaviour with gait disturbance and rapidly progressive cognitive dysfunction. Diffusion-weighted magnetic resonance imaging revealed extensive cerebral cortical hyperintensity. Prion protein (PrP) gene analysis revealed no mutations, and the polymorphic codon 129 exhibited methionine homozygosity. Although the patient remained stable with tube feeding for more than 2 years after reaching the akinetic mutism state, he died because of central respiratory failure 30 months after disease onset. Neuropathologic investigation showed extensive devastating lesions, such as status spongiosus, and typical spongiform changes could no longer be observed in the cerebral neocortex. Conspicuous pyramidal tract degeneration was observed. However, the regions commonly preserved in MM1-type sCJD pathology were still relatively preserved. Immunostaining revealed extensive diffuse synaptic-type PrP deposition in the grey matter. The pathological findings suggested that sCJD is a neurodegenerative disease that shows system degeneration; there are primary and secondary degenerative regions and distinct preserved regions, even in cases with prolonged disease duration. In addition, it is considered that there is a limited survival period for MM1-type sCJD, even if active symptomatic treatment is provided.
Topics: Akinetic Mutism; Creutzfeldt-Jakob Syndrome; Humans; Male; Methionine; Middle Aged; Neurodegenerative Diseases; Prion Proteins
PubMed: 33472525
DOI: 10.1080/19336896.2020.1868931 -
Neurosciences (Riyadh, Saudi Arabia) Oct 2020The authors report a previously healthy 23-year-old male patient who presented with subarachnoid hemorrhage and was found to have a ruptured right distal anterior...
The authors report a previously healthy 23-year-old male patient who presented with subarachnoid hemorrhage and was found to have a ruptured right distal anterior cerebral artery aneurysm. He was treated by endovascular coiling technique, which was uneventful perioperatively. After a few days of mechanical ventilation and upon extubation, he expressed symptoms of apathy, drowsiness, lack of motivation, and lack of spontaneous motor function consistent with akinetic mutism. The magnetic resonance imaging of the brain revealed infarction of the whole body of corpus callosum. He remained in akinetic mutism status for twenty-one days before he started to show improvement until he fully recovered in 3 months. The authors report a unique finding where akinetic mutism resulted from infarction of the corpus callosum rather than medial frontal lobe (cingulate gyrus).
Topics: Akinetic Mutism; Aneurysm, Ruptured; Cerebral Arterial Diseases; Cerebral Infarction; Corpus Callosum; Endovascular Procedures; Humans; Intracranial Aneurysm; Male; Recovery of Function; Subarachnoid Hemorrhage; Young Adult
PubMed: 33459293
DOI: 10.17712/nsj.2020.5.20200071