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Clinical Medicine Insights. Case Reports 2024Cerebral infarct associated with varicella-zoster virus (VZV) has been reported in the literature, while isolated central dizziness due to lateral medullary infarct...
BACKGROUND
Cerebral infarct associated with varicella-zoster virus (VZV) has been reported in the literature, while isolated central dizziness due to lateral medullary infarct (LMI) following VZV infection is rarely reported.
CASE REPORT
We report the case of a 65-year-old man who presented to the neurology department because of herpes zoster on the right trigeminal nerve distribution. At 12 hours after admission, he developed transient vertigo along with nausea and unsteady walking and left-sided spontaneous horizontal nystagmus, gaze-evoked nystagmus, and upbeat nystagmus. The other usual signs of LMI including Horner syndrome, dysarthria, swallowing difficulty, and hemibody sensory change were absent. Video head impulse indicated decreased head impulse gain of the vestibulo-ocular reflex for the bilateral horizontal, anterior, and posterior semicircular canals with abnormal saccade waves. Suppression head impulse paradigm showed few downward saccades reflecting anti-compensatory saccades after the end of the head impulse back to the head-fixed target and decreased vestibulo-ocular reflex gain values of bilateral semicircular canals. Brain magnetic resonance imaging (MRI) showed a small infarct in the far dorsolateral portion of the left rostral medulla. The cerebrospinal fluid was positive for VZV DNA.
CONCLUSIONS
In patients with VZV infection who develop dizziness, the possibility of cerebral infarct should be considered. Patients with facial herpes zoster and neurological symptoms always be screened for stroke using MRI and lumbar puncture should be performed and acyclovir administered empirically.
PubMed: 38895742
DOI: 10.1177/11795476241262213 -
Journal of Voice : Official Journal of... Jun 2024This research aims to identify acoustic features which can distinguish patients with Parkinson's disease (PD patients) and healthy speakers.
PURPOSE
This research aims to identify acoustic features which can distinguish patients with Parkinson's disease (PD patients) and healthy speakers.
METHODS
Thirty PD patients and 30 healthy speakers were recruited in the experiment, and their speech was collected, including three vowels (/i/, /a/, and /u/) and nine consonants (/p/, /pʰ/, /t/, /tʰ/, /k/, /kʰ/, /l/, /m/, and /n/). Acoustic features like fundamental frequency (F0), Jitter, Shimmer, harmonics-to-noise ratio (HNR), first formant (F1), second formant (F2), third formant (F3), first bandwidth (B1), second bandwidth (B2), third bandwidth (B3), voice onset, voice onset time were analyzed in our experiment. Two-sample independent t test and the nonparametric Mann-Whitney U (MWU) test were carried out alternatively to compare the acoustic measures between the PD patients and healthy speakers. In addition, after figuring out the effective acoustic features for distinguishing PD patients and healthy speakers, we adopted two methods to detect PD patients: (1) Built classifiers based on the effective acoustic features and (2) Trained support vector machine classifiers via the effective acoustic features.
RESULTS
Significant differences were found between the male PD group and the male health control in vowel /i/ (Jitter and Shimmer) and /a/ (Shimmer and HNR). Among female subjects, significant differences were observed in F0 standard deviation (F0 SD) of /u/ between the two groups. Additionally, significant differences between PD group and health control were also found in the F3 of /i/ and /n/, whereas other acoustic features showed no significant differences between the two groups. The HNR of vowel /a/ performed the best classification accuracy compared with the other six acoustic features above found to distinguish PD patients and healthy speakers.
CONCLUSIONS
PD can cause changes in the articulation and phonation of PD patients, wherein increases or decreases occur in some acoustic features. Therefore, the use of acoustic features to detect PD is expected to be a low-cost and large-scale diagnostic method.
PubMed: 38890016
DOI: 10.1016/j.jvoice.2024.05.018 -
Neuropsychopharmacology Reports Jun 2024Anticholinergic toxicity is a common occurrence in the emergency room, making it crucial for emergency clinicians to have a good understanding of this toxidrome. The...
Anticholinergic toxicity is a common occurrence in the emergency room, making it crucial for emergency clinicians to have a good understanding of this toxidrome. The neuropsychiatric effects of anticholinergic agents and anabolic steroids (ASs) can manifest as symptoms like anxiety, agitation, dysarthria, confusion, seizures, visual hallucinations, bizarre behavior, delirium, psychosis, and coma. When dealing with a conscious patient who has ingested an anticholinergic substance, a detailed history of ingestion can aid clinicians in making an accurate diagnosis. However, the lack of information about the substances consumed can complicate diagnosis. In cases where the exposure is unknown, clinicians should consider anticholinergic poisoning in patients showing signs of altered mental status and physical examination findings consistent with anticholinergic toxicity. We report four cases presenting a range of symptoms, including neuropsychiatric manifestations, following the ingestion of the same bodybuilding powders with anticholinergic properties. All four patients consumed yellow and white powders at the same time and in the same place. Laboratory analysis revealed that yellow powder and white powder contained ASs and cyproheptadine, respectively.
PubMed: 38889254
DOI: 10.1002/npr2.12460 -
Indian Journal of Otolaryngology and... Jun 2024Osmotic Demyelination Syndrome (ODS) is one of the primary cause of demyelination in alcoholics and malnourished resulting in various kind of clinical manifestations in...
Osmotic Demyelination Syndrome (ODS) is one of the primary cause of demyelination in alcoholics and malnourished resulting in various kind of clinical manifestations in pontine symptoms, neuro-behavioural symptoms movement disorders as well as speech and language and swallowing difficulties. The study was done to introspect the prognosis of swallowing therapy in a patient with ODS using MASA (Mann Assessment of Swallowing Ability). A 36 years old male with a history of regular alcohol intake and hypertensiom reported in our healthcare centre with hypoaklemia and speech, language and swallowing difficulty. Magnetic resonance imaging of the brain showed bilateral symmetrical T2 and T2 FLAIR (fluid attenuated inversion recovery) hyperintensity lesion in the bilateral basal ganglia involving the caudate nuclei, putaminal region and bilateral thalami with similar lesion along the mid brain. There were no areas of acute restriction in diffusion study. The findings suggested of Osmotic Demyelination Syndrome (ODS).When accessed with N-DAT, WAB and MASA; patient was diagnosed with Spastic Dysarthria, Transcortical Motor Aphasia and Moderate Dysphagia. Intervention was provided using speech and swallowing therapy and when introspected with MASA scores, improvement was seen within 5 days and statistically 97% of variance was seen inferring the progressing trend in the MASA scores. This study concludes that MASA can be an effective tool in introspecting the prognosis of Dysphagia in ODS and early intervention in the management of dysphasia shows positive results.
PubMed: 38883499
DOI: 10.1007/s12070-023-04457-y -
Cerebellum (London, England) Jun 2024Autoimmune cerebellar ataxia (ACA) is a condition characterized by progressive ataxia resulting from an immune-mediated attack on cerebellar structures. The presence of...
Autoimmune cerebellar ataxia (ACA) is a condition characterized by progressive ataxia resulting from an immune-mediated attack on cerebellar structures. The presence of anti-Tr/DNER antibodies, strongly associated with Hodgkin lymphoma, has been identified in ACA. However, cases with no underlying malignancy are rare. We report the case of a 49-year-old woman presenting with progressive ataxia, slurred speech, and dizziness over three months. The patient exhibited significant cerebellar symptoms, including dysarthria and limb ataxia, without signs of other systemic illnesses. Comprehensive investigations, including imaging, lumbar puncture, and autoantibody testing, were performed. The cerebrospinal fluid (CSF) sample revealed positivity for Tr/DNER antibodies, leading to a diagnosis of autoimmune cerebellar ataxia. The patient underwent nine sessions of plasmapheresis, followed by six doses of intravenous immunoglobulin (IVIG), resulting in significant clinical improvement. Despite extensive cancer screening, no underlying malignancy was detected, suggesting a non-tumor origin of anti-Tr/DNER antibodies. The patient's gait improved, ataxia resolved, and cerebellar tests normalized following treatment. The patient was further managed with rituximab treatment every six months. This case represents a presentation of anti-Tr/DNER-associated autoimmune cerebellar ataxia without malignancy. The successful treatment with plasmapheresis and IVIG suggests that these interventions may be effective in managing autoimmune cerebellar ataxia associated with anti-Tr/DNER antibodies. Further research is needed to understand the underlying mechanisms of this condition and to determine the optimal treatment strategies.
PubMed: 38874737
DOI: 10.1007/s12311-024-01711-z -
Radiology Case Reports Aug 2024A 79-year-old woman with a history of resection of the ascending colon cancer presented with conscious disturbance, dysarthria, nausea, and dizziness. Computed...
A 79-year-old woman with a history of resection of the ascending colon cancer presented with conscious disturbance, dysarthria, nausea, and dizziness. Computed tomography (CT) revealed striking high-density lesions in the left cerebellum and left frontal lobe with slight perifocal edema. These lesions were suspected the coexistence of spontaneous cerebellar hemorrhage and frontal lobe metastasis, or multiple brain metastases with massive hematoma. Because of the mass effect of the cerebellar lesion and impaired consciousness, she underwent emergency resection of the cerebellar lesion which was found to be composed of grayish abnormal soft solid tissue and did not include an obvious hematoma mass. The pathological findings were consistent with brain metastasis from colon cancer. This is an impressive rare case of intraoperative solid brain metastasis with a clearly homogenous hyper-dense CT appearance mimicking intracerebral hematoma.
PubMed: 38872752
DOI: 10.1016/j.radcr.2024.05.011 -
Radiology Case Reports Aug 2024Situs inversus is a rare congenital abnormality characterized by mirror-image transposition of the major visceral organs and vessels. Few reports have discussed the use...
Situs inversus is a rare congenital abnormality characterized by mirror-image transposition of the major visceral organs and vessels. Few reports have discussed the use of mechanical thrombectomy in acute ischemic stroke with situs inversus. We present such a case, to raise awareness and deepen the knowledge on these cases. A 44-year-old man was admitted to our hospital with sudden-onset dysarthria and left-sided paresis. Computed tomography (CT) angiography revealed situs inversus and occlusion in the internal carotid artery. First, intravenous tissue plasminogen activator was administered, followed by immediate reperfusion with mechanical thrombectomy. We achieved thrombolysis in cerebral infarction grade 3. After the procedure, the patient fully recovered. Prompt diagnosis is crucial for rapid recanalization in patients with vascular anomalies such as situs inversus.
PubMed: 38872738
DOI: 10.1016/j.radcr.2024.05.012 -
Molecular Genetics and Metabolism Jun 2024The late-onset GM2 gangliosidoses, comprising late-onset Tay-Sachs and Sandhoff diseases, are rare, slowly progressive, neurogenetic disorders primarily characterized by...
The late-onset GM2 gangliosidoses, comprising late-onset Tay-Sachs and Sandhoff diseases, are rare, slowly progressive, neurogenetic disorders primarily characterized by neurogenic weakness, ataxia, and dysarthria. The aim of this longitudinal study was to characterize the natural history of late-onset GM2 gangliosidoses using a number of clinical outcome assessments to measure different aspects of disease burden and progression over time, including neurological, functional, and quality of life, to inform the design of future clinical interventional trials. Patients attending the United States National Tay-Sachs & Allied Diseases Family Conference between 2015 and 2019 underwent annual clinical outcome assessments. Currently, there are no clinical outcome assessments validated to assess late-onset GM2 gangliosidoses; therefore, instruments used or designed for diseases with similar features, or to address various aspects of the clinical presentations, were used. Clinical outcome assessments included the Friedreich's Ataxia Rating Scale, the 9-Hole Peg Test, and the Assessment of Intelligibility of Dysarthric Speech. Twenty-three patients participated in at least one meeting visit (late-onset Tay-Sachs, n = 19; late-onset Sandhoff, n = 4). Patients had high disease burden at baseline, and scores for the different clinical outcome assessments were generally lower than would be expected for the general population. Longitudinal analyses showed slow, but statistically significant, neurological progression as evidenced by worsening scores on the 9-Hole Peg Test (2.68%/year, 95% CI: 0.13-5.29; p = 0.04) and the Friedreich's Ataxia Rating Scale neurological examination (1.31 points/year, 95% CI: 0.26-2.35; p = 0.02). Time since diagnosis to study entry correlated with worsening scores on the 9-Hole Peg Test (r = 0.728; p < 0.001), Friedreich's Ataxia Rating Scale neurological examination (r = 0.727; p < 0.001), and Assessment of Intelligibility of Dysarthric Speech intelligibility (r = -0.654; p = 0.001). In summary, patients with late-onset GM2 gangliosidoses had high disease burden and slow disease progression. Several clinical outcome assessments suitable for clinical trials showed only small changes and standardized effect sizes (change/standard deviation of change) over 4 years. These longitudinal natural history study results illustrate the challenge of identifying responsive endpoints for clinical trials in rare, slowly progressive, neurogenerative disorders where arguably the treatment goal is to halt or decrease the rate of decline rather than improve clinical status. Furthermore, powering such a study would require a large sample size and/or a long study duration, neither of which is an attractive option for an ultra-rare disease with no available treatment. These findings support the development of potentially more sensitive late-onset GM2 gangliosidoses-specific rating instruments and/or surrogate endpoints for use in future clinical trials.
PubMed: 38870773
DOI: 10.1016/j.ymgme.2024.108512 -
NPJ Parkinson's Disease Jun 2024Approximately 90% of Parkinson's patients (PD) suffer from dysarthria. However, there is currently a lack of research on acoustic measurements and speech impairment... (Review)
Review
Approximately 90% of Parkinson's patients (PD) suffer from dysarthria. However, there is currently a lack of research on acoustic measurements and speech impairment patterns among Mandarin-speaking individuals with PD. This study aims to assess the diagnosis and disease monitoring possibility in Mandarin-speaking PD patients through the recommended speech paradigm for non-tonal languages, and to explore the anatomical and functional substrates. We examined total of 160 native Mandarin-speaking Chinese participants consisting of 80 PD patients, 40 healthy controls (HC), and 40 MRI controls. We screened the optimal acoustic metric combination for PD diagnosis. Finally, we used the objective metrics to predict the patient's motor status using the Naïve Bayes model and analyzed the correlations between cortical thickness, subcortical volumes, functional connectivity, and network properties. Comprehensive acoustic screening based on prosodic, articulation, and phonation abnormalities allows differentiation between HC and PD with an area under the curve of 0.931. Patients with slowed reading exhibited atrophy of the fusiform gyrus (FDR p = 0.010, R = 0.391), reduced functional connectivity between the fusiform gyrus and motor cortex, and increased nodal local efficiency (NLE) and nodal efficiency (NE) in bilateral pallidum. Patients with prolonged pauses demonstrated atrophy in the left hippocampus, along with decreased NLE and NE. The acoustic assessment in Mandarin proves effective in diagnosis and disease monitoring for Mandarin-speaking PD patients, generalizing standardized acoustic guidelines beyond non-tonal languages. The speech impairment in Mandarin-speaking PD patients not only involves motor aspects of speech but also encompasses the cognitive processes underlying language generation.
PubMed: 38866758
DOI: 10.1038/s41531-024-00720-3 -
MedRxiv : the Preprint Server For... Jun 2024Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor neuron disease that causes progressive muscle weakness. Progressive bulbar dysfunction causes dysarthria...
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor neuron disease that causes progressive muscle weakness. Progressive bulbar dysfunction causes dysarthria and thus social isolation, reducing quality of life. The Everything ALS Speech Study obtained longitudinal clinical information and speech recordings from 292 participants. In a subset of 120 participants, we measured speaking rate (SR) and listener effort (LE), a measure of dysarthria severity rated by speech pathologists from recordings. LE intra- and inter-rater reliability was very high (ICC 0.88 to 0.92). LE correlated with other measures of dysarthria at baseline. LE changed over time in participants with ALS (slope 0.77 pts/month; p<0.001) but not controls (slope 0.005 pts/month; p=0.807). The slope of LE progression was similar in all participants with ALS who had bulbar dysfunction at baseline, regardless of ALS site of onset. LE could be a remotely collected clinically meaningful clinical outcome assessment for ALS clinical trials.
PubMed: 38853969
DOI: 10.1101/2024.05.31.24308140