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Frontiers in Neurology 2018Akinetic mutism has often been used as the predictor of sporadic Creutzfeldt-Jacob disease (sCJD) endpoints, but it may be difficult for general physcians to assess....
Akinetic mutism has often been used as the predictor of sporadic Creutzfeldt-Jacob disease (sCJD) endpoints, but it may be difficult for general physcians to assess. Nasogastric (NG) tube insertion is indicated for many neurodegenerative diseases with a clinical course of swallowing failure, and can be more easily identified than akinetic mutism by general physicians. Therefore, the aim of this study was to identify whether there are predictive factors for early initiation of artificial feeding in patients with sCJD who require enteral nutrition due to swallowing failure. We retrospectively reviewed the medical records of all patients diagnosed with probable sCJD who were admitted to the neurology ward at a medical center in Taiwan from January 2002 to July 2017. We used Pearson's chi-squared test to detect the correlation of initial symptoms, neurological signs, brain magnetic resonance imaging (MRI), electroencephalography (EEG), and increased levels of 14-3-3 protein in cerebrospinal fluid (CSF) analysis. The Cox proportional hazards model was used to detect prognostic factors for early initiation of NG tube insertion in sCJD patients. The onset age ranged from 51 to 83 years, and mostly ranged from 60 to 79 years. Akinetic mutism was correlated with pyramidal tract signs, myoclonus, and extrapyramidal signs. Furthermore, myoclonus was revealed to be associated with pyramidal tract signs. Multivariate Cox regression analysis showed that myoclonus and elevated CSF levels of 14-3-3 protein are predictive of early NG insertion. Increased levels of 14-3-3 protein in CSF and the presence of myoclonus at diagnosis are predictive of early swallowing difficulty and indicate rapid deterioration in probable sCJD. In addition to akinetic mutism, early initiation of artificial feeding can be used to predict early deterioration in sCJD.
PubMed: 30018588
DOI: 10.3389/fneur.2018.00496 -
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 -
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 -
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 -
Frontiers in Neurology 2016Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms....
BACKGROUND
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive neurodegenerative disease that almost always results in death in under a year from onset of symptoms. Here, we report four cases of CJD with different clinical presentations diagnosed at our institution over a 2-year period.
CASES
The first patient is an 82-year-old woman who presented with depression, cognitive decline, and word-finding difficulty over 4 weeks. The patient deteriorated neurologically to akinetic mutism and death within 6 weeks of presentation. The second patient is a 54-year-old woman with liver cirrhosis who presented with confusion, ataxia, and multiple falls over 4 weeks. She was treated initially for hepatic encephalopathy but continued to progress to mutism, startle myoclonus, and obtundation. Death occurred within 4 weeks of presentation. The third patient is a 58-year-old woman who presented with an 8-week history of confusion, urinary incontinence, Parkinsonism, ataxia, and myoclonus. Death occurred within 2 months from presentation. The fourth patient is a 67-year-old man who presented with a 6-week history of headache, blurred vision, ataxia, and personality change and progressed to confusion, myoclonus, akinetic mutism, and obtundation. Death occurred within 3 weeks from presentation.
CONCLUSION
These four cases highlight the varied possible clinical presentations of CJD and demonstrate the importance of considering CJD in patients with atypical presentations of rapidly progressive cognitive decline. To diagnose CJD, brain biopsy remains the gold standard. However, the presence of CSF protein 14-3-3, typical MRI findings and suggestive EEG abnormalities, all support the diagnosis.
PubMed: 27621721
DOI: 10.3389/fneur.2016.00138 -
Therapeutic Advances in Neurological... 2022Ustekinumab, a monoclonal antibody against interleukin (IL)-12 and IL-23 approved for the treatment of Crohn's disease, has shown to be an effective therapy with a...
Ustekinumab, a monoclonal antibody against interleukin (IL)-12 and IL-23 approved for the treatment of Crohn's disease, has shown to be an effective therapy with a favourable safety profile. Clinical trials and real-world studies have reported very few neurological adverse events, including posterior reversible encephalopathy syndrome, idiopathic intracranial hypertension and headache. We describe the case of a 48-year-old man with Crohn's disease who initiated treatment with ustekinumab on top of ongoing treatment with methotrexate 25 mg/week who presented with an acute-onset encephalopathy that rapidly evolved to severe tetraparesis and akinetic mutism, associated with extensive leukoencephalopathy and restricted diffusion on brain magnetic resonance imaging (MRI), 1 month after the second dose of ustekinumab. Comprehensive in-patient diagnostic testing ruled out vascular, demyelinating, metabolic, tumoral and infectious etiologies. Brain biopsy showed patchy infiltrates of foamy histiocytes with perivascular distribution, associated with edema, diffuse astrocytic gliosis and focal perivascular axonal destruction without demyelination, and ustekinumab-induced neurotoxicity was suspected. After drug discontinuation, the patient presented a complete clinical recovery despite the persistence of leukoencephalopathy. In conclusion, in an era in which biological therapies are continually evolving and expanding, knowledge about the potential neurotoxicity of these new therapies and their management becomes crucial. Although ustekinumab-induced encephalopathy is uncommon, the recognition of this potentially serious side effect is important because prompt withdrawal is associated with a favourable outcome. Whether methotrexate played an additional contributing role is currently unknown, but it is a factor that should be considered.
PubMed: 35237349
DOI: 10.1177/17562864221079682 -
Journal of Clinical Neurology (Seoul,... Dec 2011Oriental medicines have been associated with severe psychiatric, neurological, and other adverse medical events. These medicines occasionally cause a typical reversible...
BACKGROUND
Oriental medicines have been associated with severe psychiatric, neurological, and other adverse medical events. These medicines occasionally cause a typical reversible toxic encephalopathy, but most such cases are not recognized because these adverse events are complex and are associated with other systemic signs and symptoms.
CASE REPORT
We describe a married couple with rapid progressive cognitive impairment and akinetic mutism after taking the same oriental medicines on the same day. Brain magnetic resonance images of the couple showed typical leukoencephalopathy in the periventricular white matter and basal ganglia regions, bilaterally.
CONCLUSIONS
The development of neurobehavioral symptoms and toxic leukoencephalopathy in both patients following the ingestion of oriental medicines is suggestive of a cause-and-effect association, although such a relationship needs to be verified.
PubMed: 22259619
DOI: 10.3988/jcn.2011.7.4.223 -
Movement Disorders Clinical Practice Jul 2021
PubMed: 34307755
DOI: 10.1002/mdc3.13230 -
Journal of the Academy of... 2023Delirium is common in the setting of infection with severe acute respiratory syndrome coronavirus 2. Anecdotal evidence and case reports suggest that patients with...
BACKGROUND
Delirium is common in the setting of infection with severe acute respiratory syndrome coronavirus 2. Anecdotal evidence and case reports suggest that patients with delirium in the setting of Coronavirus 2019 (COVID-19) may exhibit specific features, including increased tone, abulia, and alogia.
OBJECTIVE
To determine whether differences exist in sociodemographic and medical characteristics, physical examination findings, and medication use in delirious patients with and without COVID-19 infection referred for psychiatric consultation.
METHODS
We undertook an exploratory, retrospective chart review of 486 patients seen by the psychiatry consultation service at a tertiary care hospital from March 10 to May 15, 2020. Delirious patients were diagnosed via clinical examination by a psychiatric consultant, and these patients were stratified by COVID-19 infection status. The strata were described and compared using bivariate analyses across sociodemographic, historical, objective, and treatment-related variables.
RESULTS
A total of 109 patients were diagnosed with delirium during the study period. Thirty-six were COVID-19+. Median age was 63 years and did not differ between groups. COVID-19+ patients with delirium were more likely to present from nursing facilities (39% vs 11%; Fisher's exact test; P = 0.001) and have a history of schizophrenia (11% vs 0%; Fisher's exact test; P = 0.011). Myoclonus (28% vs 4%; P = 0.002), hypertonia (36% vs 10%; P = 0.003), withdrawal (36% vs 15%; P = 0.011), akinesia (19% vs 6%; P = 0.034), abulia (19% vs 3%; P = 0.004), and alogia (25% vs 8%; P = 0.012) were more common in COVID-19+ patients. COVID-19+ delirious patients were significantly more likely to have received ketamine (28% vs 7%; P = 0.006), alpha-adrenergic agents besides dexmedetomidine (36% vs 14%; P = 0.014), and enteral antipsychotics (92% vs 66%; P = 0.007) at some point.
CONCLUSIONS
Patients with COVID-19 delirium referred for psychiatric consultation are more likely to reside in nursing facilities and have a history of schizophrenia than delirious patients without COVID-19. Patients with delirium in the setting of COVID-19 may exhibit features consistent with akinetic mutism. Psychiatrists must assess for such features, as they may influence management choices and the risk of side effects with agents commonly used in the setting of delirium.
Topics: Humans; Middle Aged; Retrospective Studies; COVID-19; Delirium; SARS-CoV-2; Demography
PubMed: 35948255
DOI: 10.1016/j.jaclp.2022.07.010 -
The Tohoku Journal of Experimental... Aug 1990In order to detect the functional subsets of the mesial frontal lobe, 26 patients with lesions involving mesial frontal lobe were investigated. The results were... (Review)
Review
In order to detect the functional subsets of the mesial frontal lobe, 26 patients with lesions involving mesial frontal lobe were investigated. The results were summarized as follows; (1) Hemiparesis predominantly in lower extremity was common motor deficit in patients with mesial frontal motor cortices. Four patients with supplementary motor damage sparing motor cortex showed long lasting hemiparesis. (2) Verbal adynamia was common symptom due to left supplementary motor and superior prefrontal damage. Transient mutism was found in two patients with superior prefrontal damage without the involvement of supplementary motor cortex. (3) Emotional disturbance characterized by depressed motivation was also common. No hemispheric laterality could be detected in these patients. (4) Dementia and memory disturbance related to the pole and anterior area of superior prefrontal damage. (5) Transient urinary incontinence was presented only by right superior prefrontal damage, and permanent incontinence related with bilateral damage. (6) Akinetic mutism was related with bilateral large damage including cingulate. (7) Motor neglect and dressing apraxia possibly due to programming disturbance related to lesions in right hemisphere.
Topics: Adult; Aged; Brain Injuries; Cognition Disorders; Dominance, Cerebral; Emotions; Female; Frontal Lobe; Humans; Male; Middle Aged; Movement Disorders; Nervous System Diseases; Neurocognitive Disorders; Radiography; Sensation; Speech Disorders; Urinary Incontinence
PubMed: 2082501
DOI: No ID Found