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Isolated unilateral hypoglossal nerve palsy (IUHNP) is rare because of its complex course and close adjacent structures. Prostate cancer is a very rare cause of hypoglossal nerve palsy and reported scarcely. We herein report the first case of metastatic prostate cancer presented with IUHNP in Korea, which shows good clinico-radiological correlation.
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Background@#and Purpose It is challenging to detect Parkinson’s disease (PD) in its early stages, which has prompted researchers to develop techniques based on machine learning methods for detecting PD. However, previous studies did not fully incorporate the slow progression of PD over a long period of time nor consider that its symptoms occur in a time-sequential manner. Contributing to the literature on PD, which has relied heavily on cross-sectional data, this study aimed to develop a method for detecting PD early that can process time-series information using the long short-term memory (LSTM) algorithm. @*Methods@#We sampled 926 patients with PD and 9,260 subjects without PD using medicalclaims data. The LSTM algorithm was tested using diagnostic histories, which contained the diagnostic codes and their respective time information. We compared the prediction power of the 12-month diagnostic codes under two different settings over the 4 years prior to the first PD diagnosis. @*Results@#The model that was trained using the most-recent 12-month diagnostic codes had the best performance, with an accuracy of 94.25%, a sensitivity of 82.91%, and a specificity of 95.26%. The other three models (12-month codes from 2, 3, and 4 years prior) were found to have comparable performances, with accuracies of 92.27%, 91.86%, and 91.81%, respectively.The areas under the curve from our data settings ranged from 0.839 to 0.923. @*Conclusions@#We explored the possibility that PD specialists could benefit from our proposed machine learning method as an early detection method for PD.
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Background@#To evaluate workload in emergency rooms (ERs) among clinical specialties including neurology and investigate characteristics of neurological consultations in ER. @*Methods@#A nationwide survey was conducted to evaluate the number of specialists, resident physicians/surgeons, and emergency consultations of each clinical specialty in Korean tertiary referral hospitals from 2018 to 2019. Characteristics of neurological emergency consultations during the same period were investigated in one of the hospitals that participated. @*Results@#A total of 23 hospitals were included. Four irrelevant clinical specialties were excluded. The number of neurology specialists and resident physicians were 12.5/hospital (4.1% of all specialists) and 6.4/hospital (3.4% of all resident physicians/surgeons), respectively, while the mean numbers of specialists and resident physicians or surgeons per clinical specialty were 13.7/hospital and 8.6/hospital, respectively. Neurological consultations accounted for 11.0% of all ER consultations for severe patients (Korean Triage and Acuity Scale level 1-3). Annual ER consultations for severe patients per neurology specialist was 274.1, which was only second to pediatrics (290.0). Annual ER consultations for severe patients per resident physician was 406.6 which was 1.6 times higher than the second highest (internal medicine, 247.0). Frequent conditions for neurological ER consultation were dizziness (24.8%), motor weakness (23.9%), headache (10.6%), dysarthria (9.9%), and seizures (7.7%). Frequent primary diagnoses were cerebrovascular diseases (29.0%) and episodic and paroxysmal disorders (24.9%). @*Conclusions@#Workloads of neurology specialists for ER consultation were significantly heavy, and the workload of neurology resident physicians was the heaviest among all specialties. This should be considered in health care policies.
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Background@#Because Korea is the fastest aging country, the stroke incidence is increasing rapidly. We investigate the trend of the number of patients with acute stroke in recent years and estimate the burden of the neurologist to treat the acute stroke patient visited the emergency department. @*Methods@#We requested a questionnaire survey to all teaching hospitals on the number of hospital beds, the number of stroke patients who visited the emergency department, the number of stroke patients in charge of the neurologist, and the number of days on duty of residents from 2016 to 2019. @*Results@#Of 69 teaching hospitals, 41 hospitals answered the survey. The average hospital beds per hospital were increased annually from 909 to 916. The average patients who visited the emergency department with stroke and were in charged to neurologists were rapidly increased from 799 to 867 per hospital. In particular, the number of patients with hyperacute cerebral infarction requiring the thrombolytic administration or mechanical thrombectomy were rapidly increased from 105 to 131. On the other hand, the average number of residents per hospital was decreased from 5.1 to 4.8. Therefore, the days on duty per resident were increased from 74 to 77. @*Conclusions@#The number of acute stroke patients, especially, hyperacute stroke required the rapid cooperation and high labor were increasing rapidly in recent years. However, because the number of residents were decreased, the burden was increasing. To improve the quality of acute stroke treatment, it is necessary to increase the number of residents.
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Background@#and Purpose Nonconvulsive status epilepticus (NCSE) is challenging to diagnose. This study aimed to describe and classify the clinical features and electroencephalography (EEG) findings of patients with de novo NCSE and to correlate them with clinical outcomes. @*Methods@#We retrospectively reviewed the medical and EEG records of patients admitted to our institution with altered mentation and EEG abnormalities from January 1, 2013 to December 31, 2018. We evaluated premorbid modified Rankin Scale (mRS) scores, underlying disorders, precipitating factors, clinical manifestations, laboratory tests, and outcomes after a 3-month follow-up. Patients who met the Salzburg Consensus Criteria for NCSE were categorized into good-outcome and poor-outcome groups. A good outcome was defined as 1) clinical and electrographic seizures ceasing after treatment, and 2) an mRS score of ≤2 or remaining unchanged during the 3-month follow-up. A poor outcome was defined as 1) death, 2) seizures continuing despite treatment, or 3) a follow-up mRS score of ≥3 in a patient with a premorbid mRS score of ≤2, or a follow-up mRS score that increased in a patient with a premorbid mRS score of ≥3. @*Results@#The 48 included patients comprised 37 categorized into the good-outcome group and 11 into the poor-outcome group. The presence of acute metabolic disturbances was significantly correlated with poor outcome (p=0.036), while the other analyzed variables were not significantly correlated with outcomes. @*Conclusions@#Acute metabolic disturbances in NCSE are associated with poor outcomes. Adequate treatment of underlying reversible disorders alongside controlling seizures is critical for patients with NCSE.
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The term dopa-responsive dystonia (DRD) is used to describe a group of neurometabolic disorders, which are characterized by dystonia, and are typically associated with diurnal fluctuations and respond to levodopa treatment. Autosomal dominant DRD (DYT5a, MIM# 128230) is caused by a heterozygous mutation in the GTP cyclohydrolase 1 (GCH1) gene (MIM# 600225). GCH1 encodes an enzyme, which is involved in the biosynthesis of tetrahydrobiopterin, an essential co-factor for tyrosine hydroxylase. Herein, we report the case of a 16-year-old girl who was diagnosed with DYT5a. She exhibited additional unusual clinical features, including intellectual disability, depression, multiple skeletal anomalies, and short stature, which are not commonly observed in patients with DYT5a. The patient harbored a heterozygous missense variant, c.539A>C, p.Gln180Pro, in the GCH1 gene, which was identified by targeted gene panel analysis using next-generation sequencing.
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Metabolic encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is characterized by encephalopathy mimicking acute stroke, which is rarely observed in adulthood. We present a case of a female who presented with several neurologic deficits and diagnosed with MELAS syndrome. Brain magnetic resonance imaging (MRI) showed acute lesions mimicking stroke, which were not compatible with specific vascular territories. Magnetic resonance spectroscopy showed lactate peaks in both symptomatic and asymptomatic area. Single proton emission computed tomography (SPECT) shows hyperperfusion in the same area. On follow-up image, MRI lesion nearly disappeared and hyperperfusion on SPECT changed into hypoperfusion. We discuss the clinical characteristics and image findings of our patient.
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Background@#The median-to-ulnar comparison test (MUCT), and increasingly, ultrasonography (US) are considered as complementary to and more sensitive than median nerve conduction study (NCS) in diagnosing carpal tunnel syndrome (CTS). @*Methods@#In consecutive patients with hand paresthesia compatible with CTS but with normal median NCS, we additionally performed the MUCT and analyzed whether it yielded better diagnostic sensitivity. @*Results@#In total, 163 hands of clinically diagnosed CTS patients were examined with routine NCS. The MUCT and US were performed in 81 hands and 31 hands, respectively. While median NCS was diagnostic in 85 (52.1%) hands, MUCT failed to demonstrate superior sensitivity over median NCS in the other hands and US revealed related abnormalities better than both routine NCS (p=0.006) and MUCT (p=0.002). @*Conclusions@#The MUCT offered no additional diagnostic benefit. On the other hand, sonographic examination had higher sensitivity for the diagnosis of CTS when applying several diagnostic criteria. Thus, US could be the screening test for diagnosing CTS prior to NCS with higher sensitivity than MUCT. However, further studies are needed to define the appropriate diagnostic criteria for US.
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Background@#The median-to-ulnar comparison test (MUCT), and increasingly, ultrasonography (US) are considered as complementary to and more sensitive than median nerve conduction study (NCS) in diagnosing carpal tunnel syndrome (CTS). @*Methods@#In consecutive patients with hand paresthesia compatible with CTS but with normal median NCS, we additionally performed the MUCT and analyzed whether it yielded better diagnostic sensitivity. @*Results@#In total, 163 hands of clinically diagnosed CTS patients were examined with routine NCS. The MUCT and US were performed in 81 hands and 31 hands, respectively. While median NCS was diagnostic in 85 (52.1%) hands, MUCT failed to demonstrate superior sensitivity over median NCS in the other hands and US revealed related abnormalities better than both routine NCS (p=0.006) and MUCT (p=0.002). @*Conclusions@#The MUCT offered no additional diagnostic benefit. On the other hand, sonographic examination had higher sensitivity for the diagnosis of CTS when applying several diagnostic criteria. Thus, US could be the screening test for diagnosing CTS prior to NCS with higher sensitivity than MUCT. However, further studies are needed to define the appropriate diagnostic criteria for US.
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OBJECTIVE: Musculoskeletal problems are more common in patients with Parkinson's disease (PD) than in normal elderly, but the impact of musculoskeletal problems on health-related quality of life (HRQoL) in patients with PD is unknown. METHODS: Four hundred consecutive patients with PD were enrolled for the evaluation of musculoskeletal problems and HRQoL. HRQoL was assessed by the 36-Item Short Form Health Survey, which comprised physical health and mental health. RESULTS: Of the total patients, 265 patients had musculoskeletal problems, and 135 patients did not have musculoskeletal problems. Patients with musculoskeletal problems reported lower levels of HRQoL in terms of physical health than did patients without musculoskeletal problems (p < 0.05). In women, all components of physical health were lower in patients with musculoskeletal problems than in patients without musculoskeletal problems (p < 0.05). Meanwhile, in men, only the bodily pain score of physical health was lower in patients with musculoskeletal problems than in patients without musculoskeletal problems. Mental health and physical health were negatively correlated with depression, Unified Parkinson's Disease Rating Scale I & II scores, and pain severity from musculoskeletal problems, in that order (p < 0.01 for all). CONCLUSION: These results suggest that musculoskeletal problems in patients with PD affect HRQoL significantly, mainly in terms of physical health rather than mental health and especially in women rather than men. Musculoskeletal problems should not be overlooked in the care of patients with PD.
Subject(s)
Aged , Female , Humans , Male , Depression , Health Surveys , Mental Health , Parkinson Disease , Quality of LifeABSTRACT
OBJECTIVE: We examined whether amantadine can prevent the development of dyskinesia. METHODS: Patients with drug-naïve Parkinson's disease (PD), younger than 70 years of age and in the early stage of PD (Hoehn and Yahr scale < 3), were recruited from April 2011 to December 2014. The exclusion criteria included the previous use of antiparkinsonian medication, the presence of dyskinesia, significant psychological disorders, and previous history of a hypersensitivity reaction. Patients were consecutively assigned to one of 3 treatment groups in an open label fashion: Group A-1, amantadine first and then levodopa when needed; Group A-2, amantadine first, dopamine agonist when needed, and then levodopa; and Group B, dopamine agonist first and then levodopa when needed. The primary endpoint was the development of dyskinesia, which was analyzed by the Kaplan-Meier survival rate. RESULTS: A total of 80 patients were enrolled: Group A-1 (n = 27), Group A-2 (n = 27), and Group B (n = 26). Twenty-four patients were excluded from the analysis due to the following: withdrawal of amantadine or dopamine agonist (n = 9), alternative diagnosis (n = 2), withdrawal of consent (n = 1), and breach in the protocol (n = 12). After exclusion, 5 of the 56 (8.93%) patients developed dyskinesia. Patients in Group A-1 and A-2 tended to develop dyskinesia less often than those in Group B (cumulative survival rates of 0.933, 0.929, and 0.700 for A-1, A-2, and B, respectively; p = 0.453). CONCLUSION: Amantadine as an initial treatment may decrease the incidence of dyskinesia in patients with drug-naïve PD.
Subject(s)
Humans , Amantadine , Diagnosis , Dopamine Agonists , Dyskinesias , Hypersensitivity , Incidence , Levodopa , Parkinson Disease , Survival RateABSTRACT
Previously, we defined DRD as a syndrome of selective nigrostriatal dopamine deficiency caused by genetic defects in the dopamine synthetic pathway without nigral cell loss. DRD-plus also has the same etiologic background with DRD, but DRD-plus patients have more severe features that are not seen in DRD because of the severity of the genetic defect. However, there have been many reports of dystonia responsive to dopaminergic drugs that do not fit into DRD or DRD-plus (genetic defects in the dopamine synthetic pathway without nigral cell loss). We reframed the concept of DRD/DRD-plus and proposed the concept of DRD look-alike to include the additional cases described above. Examples of dystonia that is responsive to dopaminergic drugs include the following: transportopathies (dopamine transporter deficiency; vesicular monoamine transporter 2 deficiency); SOX6 mutation resulting in a developmentally decreased number of nigral cells; degenerative disorders with progressive loss of nigral cells (juvenile Parkinson's disease; pallidopyramidal syndrome; spinocerebellar ataxia type 3), and disorders that are not known to affect the nigrostriatal dopaminergic system (DYT1; GLUT1 deficiency; myoclonus-dystonia; ataxia telangiectasia). This classification will help with an etiologic diagnosis as well as planning the work up and guiding the therapy.
Subject(s)
Humans , Ataxia , Classification , Diagnosis , Dopamine , Dopamine Agents , Dystonia , Parkinson Disease , Spinocerebellar Ataxias , Vesicular Monoamine Transport ProteinsABSTRACT
Hemimasticatory spasm is a rare neurologic disorder characterized by unilateral, paroxysmal involuntary contraction of the masticatory muscles. It can be confused with other unilateral facial spasms. In this report, we present two patients with hemimasticatory spasm who were initially misdiagnosed with other disorders. The first patient was a 54-year-old man with a 9-year history of right facial spasm. He underwent microvascular decompression, without benefit, at another hospital with the diagnosis of hemifacial spasm two years prior to presenting at our hospital.On examination, right masseter and temporalis muscles had irregular contractions with twitches and prolonged spasms. His brain magnetic resonance imaging was normal.He showed a good response to carbamazepine. The second case was a 60-year-old man presented with a 10-year history of jaw-closing movement. Previous treatment for temporomandibular joint disorder was ineffective. He was previously diagnosed as oromandibular dystonia at another hospital, and biperiden and diazepam treatment resulted in slight improvement. There was tonic contraction and hypertrophy of the left masseter. He improved with carbamazepine. These cases illustrate the importance of hemimasticatory spasm as differential diagnosis, and the good response to carbamazepine.
Subject(s)
Nervous System DiseasesABSTRACT
Hashimoto's encephalopathy (HE) is a rare autoimmune disorder characterized by a nonspecific encephalopathy with high titers of serum anti-thyroid antibody in the absence of other defined causes. A 54-year-old woman was admitted due to recurrent seizures and confusion. Her serum anti-thyroid antibody level was elevated, and brain MRI showed multiple instances of vasogenic edema. Her symptoms disappeared after treatment with high-dose steroids and antiepileptic drugs. We propose that HE should be considered in the differential diagnosis of multiple vasogenic edema on brain imaging.
Subject(s)
Female , Humans , Middle Aged , Anticonvulsants , Brain , Diagnosis, Differential , Edema , Magnetic Resonance Imaging , Neuroimaging , Seizures , SteroidsABSTRACT
Hashimoto's encephalopathy (HE) is a rare autoimmune disorder characterized by a nonspecific encephalopathy with high titers of serum anti-thyroid antibody in the absence of other defined causes. A 54-year-old woman was admitted due to recurrent seizures and confusion. Her serum anti-thyroid antibody level was elevated, and brain MRI showed multiple instances of vasogenic edema. Her symptoms disappeared after treatment with high-dose steroids and antiepileptic drugs. We propose that HE should be considered in the differential diagnosis of multiple vasogenic edema on brain imaging.
Subject(s)
Female , Humans , Middle Aged , Anticonvulsants , Brain , Diagnosis, Differential , Edema , Magnetic Resonance Imaging , Neuroimaging , Seizures , SteroidsABSTRACT
We investigated the effect of propofol and fentanyl on microelectrode recording (MER) and its clinical applicability during subthalamic nucleus (STN) deep brain stimulation (DBS) surgery. We analyzed 8 patients with Parkinson's disease, underwent bilateral STN DBS with MER. Their left sides were done under awake and then their right sides were done with a continuous infusion of propofol and fentanyl under local anesthesia. The electrode position was evaluated by preoperative MRI and postoperative CT. The clinical outcomes were assessed at six months after surgery. We isolated single unit activities from the left and the right side MERs. There was no significant difference in the mean firing rate between the left side MERs (38.7+/-16.8 spikes/sec, n=78) and the right side MERs (35.5+/-17.2 spikes/sec, n=66). The bursting pattern of spikes was more frequently observed in the right STN than in the left STN. All the electrode positions were within the STNs on both sides and the off-time Unified Parkinson's Disease Rating Scale part III scores at six months after surgery decreased by 67% of the preoperative level. In this study, a continuous infusion of propofol and fentanyl did not significantly interfere with the MER signals from the STN. The results of this study suggest that propofol and fentanyl can be used for STN DBS in patients with advanced Parkinson's disease improving the overall experience of the patients.
Subject(s)
Aged , Female , Humans , Male , Middle Aged , Anesthetics, Intravenous/pharmacology , Deep Brain Stimulation , Electrodes, Implanted , Fentanyl/pharmacology , Magnetic Resonance Imaging , Microelectrodes , Parkinson Disease/prevention & control , Propofol/pharmacology , Severity of Illness Index , Subthalamic Nucleus/drug effects , Tomography, X-Ray ComputedABSTRACT
BACKGROUND: Acute intracranial large-artery occlusions (AILAOs) are detected frequently and cause severe neurological disabilities. Most studies in this field do not focus on the natural history of AILAOs, but on the individual prognosis for each intervention. The aim of this study was to elucidate the clinical profiles and outcomes of AILAOs. METHODS: A consecutive series of patients hospitalized between January 2004 and October 2007 due to AILAO within 24 hours from onset were recruited. Based on a prospective stroke registry, their clinical profiles were collected. AILAO was defined as an intracranial internal carotid artery (ICA), middle cerebral artery (MCA), or basilar artery (BA) occlusion that could be confirmed by angiography, with relevant lesions on diffusion-weighted imaging (DWI). The modified Rankin Scale (MRS) score at 3 months and the recanalization rate within 14 days were recorded as outcomes. RESULTS: Among 1,047 patients with acute ischemic stroke who were examined within 24 hours of onset, 189 [18.1%; 101 men, 88 women; age 68.6+/-13.0 years (mean+/-SD); median National Institutes of Health Stroke Scale (NIHSS) score=11]. Occlusion sites were MCA M1, MCA M2, distal ICA, and BA in 99 (52.4%), 50 (26.5%), 20 (10.6%), and 20 (10.6%) of cases,respectively. Embolic sources were found in 103 (54.5%) cases. MRS scores were available for 184 (97.4%) of the patients, of which 78 had a favorable outcome (MRS: 0.2). Follow-up angiography was performed in 122 (64.6%) cases, with recanalization observed in 88 (72.1%) of these. Thrombolysis, occlusion site, presence of an embolic source, and initial glucose level were predictors of early recanalization (p<0.001). Recanalization status and initial NIHSS score were strongly correlated with a favorable outcome (p<0.005). CONCLUSIONS: This is the first report of the clinical profiles, outcomes, and their predictors in a cohort of Korean patients with AILAO.