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BACKGROUND: Immune-mediated necrotizing myopathy (IMNM) is an idiopathic inflammatory myopathy (IIM). Though patients with IMNM were not considered to show skin rash, several reports have showed atypical skin conditions in patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibody-positive IMNM (HMGCR-IMNM). The incidence and phenotype of skin conditions in patients with HMGCR-IMNM are not fully known. RESULTS: Among the 100 IIM patients diagnosed from April 2015 through August 2022, 34 (34%) presented some form of skin condition, with 27 having typical skin rashes; this included 13 patients with dermatomyositis (DM), 8 with anti-synthetase syndrome (ASS), and 6 with IMNM. Meanwhile, 8 of 19 patients with HMGCR-IMNM (42%) presented atypical skin lesions, but no patients with other IIMs did (p < 0.001). Skin eruption with ash-like scales was observed in four HMGCR-IMNM patients, and non-scaly red patches and lumps in the other four patients; accordingly, their skin manifestations were considered as other dermal diseases except for IIM. However, skin and muscle biopsies revealed the atypical skin conditions of patients with HMGCR-IMNM to have the same pathological background, formed by Bcl-2-positive lymphocyte infiltrations. CONCLUSIONS: HMGCR-IMNM patients frequently have atypical skin conditions of the neck and back. Skin biopsy specimens from these lesions showed the same Bcl-2-positive lymphocytic infiltrations as muscle biopsy specimens regardless of the different gross dermal findings. Thus, such atypical skin conditions may be suggestive for HMGCR-IMNM.
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Autoanticuerpos , Hidroximetilglutaril-CoA Reductasas , Miositis , Piel , Femenino , Humanos , Masculino , Autoanticuerpos/inmunología , Autoanticuerpos/sangre , Biopsia , Hidroximetilglutaril-CoA Reductasas/inmunología , Enfermedades Musculares/inmunología , Enfermedades Musculares/diagnóstico , Miositis/inmunología , Miositis/diagnóstico , Piel/patología , Piel/inmunología , Enfermedades de la Piel/inmunología , Enfermedades de la Piel/etiologíaRESUMEN
OBJECTIVE: To elucidate the incidence and risk factors for paradoxical effects (i.e., increased seizure frequency, increased seizure severity, or onset of new seizure types) of levetiracetam (LEV) in people with epilepsy (PWE) and identify the usefulness of electroencephalography (EEG) in predicting these effects. METHODS: We examined data for consecutive PWE treated with LEV. All PWE underwent EEG and magnetic resonance imaging (MRI) before LEV administration. We also evaluated the incidence of paradoxical LEV effects and conducted multivariate logistic regression analyses to identify the associated factors. RESULTS: In total, 210 (66.2%) of 317 PWEs treated in our department had a history of LEV use. The incidence of paradoxical LEV effects was 5.2% (n = 11) and was significantly associated with a high LEV dose (p = 0.029), high seizure frequency (p = 0.005), temporal lobe epilepsy (p = 0.004), focal awareness seizure (p = 0.004), focal impaired awareness seizure (p = 0.007), spike (p = 0.015), rhythmic epileptiform discharges (REDs; p = 0.003), and MRI-identified focal cortical dysplasia (FCD; p < 0.0001). Multivariate analyses revealed that REDs (odds ratio [OR] = 5.35, p = 0.048, 95% confidence interval [CI]: 1.01-28.21) were independently associated with paradoxical LEV effects. CONCLUSIONS: Paradoxical LEV effects occurred in PWE, particularly in those with drug-resistant focal epilepsy. Furthermore, the occurrence of REDs in EEG was an independent factor associated with the paradoxical effects of LEV in PWE.
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Epilepsia Refractaria , Epilepsias Parciales , Epilepsia , Humanos , Levetiracetam/efectos adversos , Epilepsia/complicaciones , Epilepsia/tratamiento farmacológico , Epilepsia/inducido químicamente , Convulsiones/tratamiento farmacológico , Convulsiones/inducido químicamente , Epilepsias Parciales/tratamiento farmacológico , Electroencefalografía , Epilepsia Refractaria/tratamiento farmacológico , Anticonvulsivantes/efectos adversos , Resultado del TratamientoRESUMEN
BACKGROUND: Both fragile X-associated tremor/ataxia syndrome (FXTAS) and late-onset neuronal intranuclear inclusion disease (NIID) show CGG/GGC trinucleotide repeat expansions. Differentiating these diseases are difficult because of the similarity in their clinical and radiological features. It is unclear that skin biopsy can distinguish NIID from FXTAS. We performed a skin biopsy in an FXTAS case with cognitive dysfunction and peripheral neuropathy without tremor, which was initially suspected to be NIID. CASE PRESENTATION: The patient underwent neurological assessment and examinations, including laboratory tests, electrophysiologic test, imaging, skin biopsy, and genetic test. A brain MRI showed hyperintensity lesions along the corticomedullary junction on diffusion-weighted imaging (DWI) in addition to middle cerebellar peduncle sign (MCP sign). We suspected NIID from the clinical picture and the radiological findings, and performed a skin biopsy. The skin biopsy specimen showed ubiquitin- and p62-positive intranuclear inclusions, suggesting NIID. However, a genetic analysis for NIID using repeat-primed polymerase chain reaction (RP-PCR) revealed no expansion detected in the Notch 2 N-terminal like C (NOTCH2NLC) gene. We then performed genetic analysis for FXTAS using RP-PCR, which revealed a repeat CGG/GGC expansion in the FMRP translational regulator 1 (FMR1) gene. The number of repeats was 83. We finally diagnosed the patient with FXTAS rather than NIID. CONCLUSIONS: For the differential diagnosis of FXTAS and NIID, a skin biopsy alone is insufficient; instead, genetic analysis, is essential. Further investigations in additional cases based on genetic analysis are needed to elucidate the clinical and pathological differences between FXTAS and NIID.
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Cuerpos de Inclusión Intranucleares , Temblor , Ataxia , Biopsia , Proteína de la Discapacidad Intelectual del Síndrome del Cromosoma X Frágil , Síndrome del Cromosoma X Frágil , Humanos , Enfermedades NeurodegenerativasRESUMEN
OBJECTIVES: The present study aimed to examine the effectiveness of proton magnetic resonance spectroscopy (1HMRS) in determining the progression of neurological symptoms resulting in acute ischemic stroke in patients with lenticulostriate artery (LSA) infarction. MATERIALS AND METHODS: 1HMRS was performed within 72 h after neurological symptom onset. Voxel of interest was placed in tissue that included the pyramidal tract and identified diffusion weighted echo planar spin-echo sequence (DWI) coronal images. Infarct volume in DWI was calculated using the ABC/2 method. 1HMRS data (tNAA, tCr, Glx, tCho, and Ins) were analyzed using LCModel. Progressive neurological symptoms were defined as an increase of 1 or more in the NIHSS score. Patients who underwent 1HMRS after progressive neurological symptoms were excluded. RESULTS: In total, 77 patients were enrolled. Of these, 19 patients had progressive neurological symptoms. The patients with progressive neurological symptoms were significantly more likely to be female and had higher tCho/tCr values, higher rates of axial slices ≥ 3 slices on DWI, higher infarct volume on DWI, higher maximum diameter of infarction of axial slice on DWI, and higher SBP on admission compared to those without. Multivariable logistic analysis revealed that higher tCho/tCr values were independently associated with progressive neurological symptoms after adjusting for age, sex, and initial DWI infarct volume (tCho/tCr per 0.01 increase, OR 1.26, 95% CI 1.03-1.52, P = 0.022). CONCLUSIONS: Increased tCho/tCr score were associated with progressive neurological symptoms in patients with LSA ischemic stroke. Quantitative evaluation of 1HMRS parameters may be useful for predicting the progression of neurological symptoms.
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Enfermedad Cerebrovascular de los Ganglios Basales/diagnóstico , Biomarcadores/metabolismo , Infarto Encefálico/diagnóstico , Colina/metabolismo , Creatina/metabolismo , Espectroscopía de Protones por Resonancia Magnética , Anciano , Anciano de 80 o más Años , Enfermedad Cerebrovascular de los Ganglios Basales/metabolismo , Enfermedad Cerebrovascular de los Ganglios Basales/fisiopatología , Infarto Encefálico/metabolismo , Infarto Encefálico/fisiopatología , Imagen de Difusión por Resonancia Magnética , Evaluación de la Discapacidad , Progresión de la Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Valor Predictivo de las PruebasRESUMEN
Recent findings indicate that the human cardiovascular system is regulated by a cortical network comprised of the insular cortex (Ic), anterior cingulate gyrus, and amygdala which is necessary for the regulation of the central autonomic network system. Alzheimer disease (AD) affects the Ic at a preclinical stage. The pathology of AD at the Ic is suggested to predispose the cardiovascular system to detrimental changes such as increased blood pressure variability (BPV). In this review article, we focus on the physiology of the Ic in the relationship between the central autonomic network and BPV. We provide a summary of the published evidence regarding the relationship between Ic damage and exaggerated BPV in the context of AD pathology.
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Enfermedad de Alzheimer/patología , Sistema Nervioso Autónomo , Presión Sanguínea/fisiología , Corteza Cerebral/fisiología , Sistema Nervioso Autónomo/fisiología , Sistema Nervioso Autónomo/fisiopatología , Humanos , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND AND PURPOSE: The influence of a weather front passage is rarely evaluated on stroke events. We hypothesized that a weather front passage on the stroke onset day or during the previous days may play an important role in the incidence of stroke. METHODS: A multicenter retrospective study was conducted to evaluate the frequency of stroke events and their interaction with weather front passages. Consecutive acute stroke patients (nâ¯=â¯3935, 73.5 ± 12.4 years, 1610 females) who were admitted to 7 stroke hospitals in 3 cities from January 2012 to December 2013 were enrolled in this study. Multivariate Poisson regression models involving time lag variables were used to compare the daily rates of stroke events with the day of a weather front passage and the previous 6 days, adjusting for considerable influences of ambient temperature and atmospheric pressure. RESULTS: There were a total of 33 cold fronts and 13 warm fronts that passed over the 3 cities during the study period. The frequency of ischemic stroke significantly increased when a warm front passed on the previous day (risk ratio 1.34, 95% confidence interval 1.07-1.69, P= .016). CONCLUSIONS: This study indicated that a weather front passage on the previous days may be associated with the occurrence of stroke.
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Isquemia Encefálica/epidemiología , Calor , Accidente Cerebrovascular/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Presión Atmosférica , Isquemia Encefálica/diagnóstico , Niño , Femenino , Humanos , Incidencia , Japón/epidemiología , Masculino , Persona de Mediana Edad , Admisión del Paciente , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Accidente Cerebrovascular/diagnóstico , Factores de Tiempo , Adulto JovenRESUMEN
The cardiovascular system is regulated by a central autonomic network (CAN) consisting of the insular cortex, anterior cingulate gyrus, and amygdala. Because the insular cortex often tends to be damaged in patients with herpes simplex virus (HSV) encephalitis, the autonomic instability observed in these patients was suggested to be moderated by an insular cortex lesion. Here, we report the case of a 51-year-old Japanese male who was hospitalized following a collapse 5 days earlier; he was diagnosed as herpes encephalitis. Diffusion-weighted MRI revealed asymmetric right greater hyperintensity throughout his insular cortex and anterior cingulate gyrus. At 1 week after admission, transthoracic echo showed diffuse hypokinesis in the left ventricle (LV). Cardiac 123I-meta-iodobenzylguanidine uptake (123I-MIBG) scintigraphy revealed reduced uptake in the inferior and posterior wall. Electrocardiograhy at rest showed that the coefficient variation of RR intervals (CVR-R) was reduced, and the corrected QT (QTc) interval length was prolonged. In this HSV encephalitis patient, signs of a right insular cortex lesion and autonomic instability were observed: LV hypokinesis, regional reduced 123I-MIBG uptake, decreased CVR-R, and QTc interval prolongation. Our patient's autonomic instability may thus be derived from disrupted autonomic balance due to the right insular cortex lesion.
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Enfermedades del Sistema Nervioso Autónomo/etiología , Corteza Cerebral/patología , Encefalitis por Herpes Simple/patología , Corteza Cerebral/virología , Encefalitis por Herpes Simple/complicaciones , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: The aim of this study was to elucidate the influence of insular infarction on blood pressure (BP) variability and outcomes according to the region of the insular cortex affected. METHODS: A total of 90 patients diagnosed with acute unilateral ischemic stroke were registered. The BP variability was calculated over 24 h after admission (hyperacute) and for 2-3 days after admission (acute). Patients were classified into groups of right and left, and then right anterior, right posterior, left anterior, and left posterior insular infarction. RESULTS: Patients with insular infarction showed a significantly larger infarct volume, higher modified Rankin scale scores, and lower SD and coefficient of variation (CV) of -systolic BP in the hyperacute phase than shown by patients without insular infarction (p < 0.01, p < 0.01, p = 0.02, and p = 0.03, respectively). The SD and CV of systolic BP in the hyperacute phase showed significant differences among the 3 groups with right insular infarction, with left insular infarction, and without insular infarction (p < 0.05 and p < 0.05, respectively). There was a tendency for the systolic BP variability to be lower in patients with right anterior insular infarction than in patients with infarcts in other areas. CONCLUSION: The right insular cortex, especially the anterior part, might be a hub for autonomic nervous regulation.
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Presión Sanguínea/fisiología , Corteza Cerebral/patología , Corteza Cerebral/fisiopatología , Accidente Cerebrovascular/patología , Accidente Cerebrovascular/fisiopatología , Anciano , Anciano de 80 o más Años , Isquemia Encefálica/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Autoimmune forms of encephalitis have been associated with autoantibodies against synaptic cell surface antigens such as NMDA- and AMPA-type glutamate receptors, GABA(B) receptor, and LGI1. However, it remains unclear how many synaptic autoantigens are yet to be defined. Using immunoproteomics, we identified autoantibodies against the GABA(A) receptor in human sera from two patients diagnosed with encephalitis who presented with cognitive impairment and multifocal brain MRI abnormalities. Both patients had antibodies directed against the extracellular epitope of the ß3 subunit of the GABA(A) receptor. The ß3-subunit-containing GABA(A) receptor was a major target of the patients' serum antibodies in rat hippocampal neurons because the serum reactivity to the neuronal surface was greatly decreased by 80% when the ß3 subunit was knocked down. Our developed multiplex ELISA testing showed that both patients had similar levels of GABA(A) receptor antibodies, one patient also had a low level of LGI1 antibodies, and the other also had CASPR2 antibodies. Application of the patients' serum at the time of symptom presentation of encephalitis to rat hippocampal neuron cultures specifically decreased both synaptic and surface GABA(A) receptors. Furthermore, treatment of neurons with the patients' serum selectively reduced miniature IPSC amplitude and frequency without affecting miniature EPSCs. These results strongly suggest that the patients' GABA(A) receptor antibodies play a central role in the patients' symptoms. Therefore, this study establishes anti-GABA(A) receptor encephalitis and expands the pathogenic roles of GABA(A) receptor autoantibodies.
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Autoanticuerpos/sangre , Encefalopatías/sangre , Encefalopatías/inmunología , Encéfalo/patología , Enfermedad de Hashimoto/sangre , Enfermedad de Hashimoto/inmunología , Receptores de GABA-A/inmunología , Animales , Proteínas Reguladoras de la Apoptosis/inmunología , Encéfalo/metabolismo , Encefalopatías/complicaciones , Encefalopatías/patología , Células Cultivadas , Chlorocebus aethiops , Trastornos del Conocimiento/etiología , Encefalitis , Femenino , Enfermedad de Hashimoto/complicaciones , Enfermedad de Hashimoto/patología , Hipocampo/citología , Humanos , Péptidos y Proteínas de Señalización Intracelular , Masculino , Potenciales de la Membrana/efectos de los fármacos , Potenciales de la Membrana/genética , Persona de Mediana Edad , Neuronas/efectos de los fármacos , Neuronas/metabolismo , Neurotransmisores/farmacología , Unión Proteica/efectos de los fármacos , Unión Proteica/genética , Proteínas/inmunología , RatasRESUMEN
BACKGROUND: This study evaluated the rates of new lesions on diffusion-weighted images (DWIs) of magnetic resonance imaging (MRI) and hemorrhagic transformation (HT) during 2 weeks after acute ischemic stroke (AIS) in patients with atrial fibrillation (Af) who were given one of the non-vitamin K antagonist oral anticoagulants (NOACs); this was then compared with those who were given warfarin. METHODS AND RESULTS: Consecutive AIS patients with Af were enrolled between January 2008 and June 2013, and those selected were patients who had a MRI that included DWIs both on admission and after 2 weeks, and those given only wafrarin (warfarin group) or only one of the NOACs (NOAC group) within 2 weeks of admission. Of all 257 enrolled patients, 50 patients were selected for the NOAC group (median age of 80.0 years) and 125 patients for the warfarin group (median age of 80.0 years). Both NOAC and warfarin were started at a median of the second day after admission. There was no significant difference in the rates of new lesions on DWIs (26.0% vs. 28.0%, P=0.7888) and HT (30.0% vs. 39.2%, P=0.2536) between the NOAC and warfarin groups. The NOAC group had a lower rate of concomitant use of heparin (44.0% vs. 92.8%, P<0.0001) than the warfarin group. CONCLUSIONS: This study suggests that NOACs are suitable for AIS patients with Af, perhaps even better than warfarin, given their simplicity.
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Anticoagulantes/administración & dosificación , Fibrilación Atrial/tratamiento farmacológico , Isquemia Encefálica/tratamiento farmacológico , Heparina/administración & dosificación , Accidente Cerebrovascular/tratamiento farmacológico , Warfarina/administración & dosificación , Enfermedad Aguda , Administración Oral , Anciano , Anciano de 80 o más Años , Anticoagulantes/efectos adversos , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico por imagen , Isquemia Encefálica/complicaciones , Isquemia Encefálica/diagnóstico por imagen , Heparina/efectos adversos , Humanos , Imagen por Resonancia Magnética , Masculino , Radiografía , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/diagnóstico por imagen , Factores de Tiempo , Vitamina K , Warfarina/efectos adversosRESUMEN
BACKGROUND: The timing of warfarin administration for acute ischemic stroke (AIS) patients with atrial fibrillation (Af) has not been established. We hypothesized that achieving targeted prothrombin time and international normalized ratio (PT-INR) at 2 weeks could prevent AIS patients with Af from developing a new lesion on diffusion-weighted magnetic resonance imaging (DW-MRI). METHODS: Of consecutively enrolled AIS patients with Af between 2008 and 2011, we selected the patients who were given warfarin within 2 weeks of admission and had DW-MRI and blood test for PT-INR both on admission and at 2 weeks. Warfarin was started as early as possible and heparin was administered until the targeted PT-INR (2.0-3.0 for patients aged <70 years or 1.6-2.6 for those aged ≥70 years) was achieved. RESULTS: One hundred and twenty-three patients were selected, consisting of 88 patients without a new lesion and 35 patients with a new lesion. Patients with a new lesion had a significantly higher median score on National Institutes of Health Stroke Scale (11.0 vs. 5.5, P = .0053), a lower rate of achieving targeted PT-INR at 2 weeks (25.7% vs. 48.9%, P = .0190), and a lower median dosage of warfarin at 2 weeks (2.0 mg vs. 2.5 mg, P = .0209) than patients without a new lesion. Multivariate logistic regression analysis showed that failure to achieve targeted PT-INR (P = .0298) was significantly associated with the occurrence of a new lesion. CONCLUSIONS: Our findings suggest that achieving targeted PT-INR at 2 weeks by using warfarin prevents new lesions in AIS patients with Af.
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Anticoagulantes/uso terapéutico , Imagen de Difusión por Resonancia Magnética/métodos , Heparina/uso terapéutico , Relación Normalizada Internacional/normas , Accidente Cerebrovascular/prevención & control , Warfarina/uso terapéutico , Anciano , Anciano de 80 o más Años , Anticoagulantes/administración & dosificación , Fibrilación Atrial , Femenino , Heparina/administración & dosificación , Hospitalización , Humanos , Masculino , Persona de Mediana Edad , Protrombina/metabolismo , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/patología , Factores de Tiempo , Resultado del Tratamiento , Warfarina/administración & dosificaciónRESUMEN
BACKGROUND: The hyperintense vessel sign (HVS) on fluid-attenuated inversion recovery images can frequently be detected in patients with acute cerebral infarction attributable to large artery stenosis or occlusion. The prognostic values and clinical characteristics of HVS remain to be elucidated. The aim of this study was to evaluate the association of HVS with ischemic lesions and severity of neurologic deficit. METHODS: A total of 96 consecutive acute ischemic stroke patients (54 women, median age 76.5 [range 39-97] years), who had symptomatic severe stenosis or occlusion in the proximal middle cerebral artery that was detected with magnetic resonance angiography within 24 hours of onset, were enrolled. The extent of HVS was graded by a systematic quantitative scoring system (the HVS distribution score) based on Alberta Stroke Program Early Computed Tomographic Score. RESULTS: An HVS was detected in 89 patients (93%) at admission, and the patients who displayed wider HVS distribution scores exhibited more severe neurologic deficits at admission (P<.05). The follow-up magnetic resonance imaging, which was obtained in 79 patients (82%), was performed an average of 13 days. The association between HVS distribution score and final ischemic lesions was strongly observed (n=67, P<.05) but not in the patients with intravenous thrombolysis (n=12, P=.06). CONCLUSIONS: Although the distribution of HVS reflected final ischemic lesion, this association might not apply to the patients with the thrombolysis treatment. The interpretation of HVS distribution score with acute ischemic stroke patients should be discussed dependent on thrombolysis.
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Isquemia Encefálica/diagnóstico , Encéfalo/patología , Accidente Cerebrovascular/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Isquemia Encefálica/tratamiento farmacológico , Isquemia Encefálica/patología , Femenino , Fibrinolíticos/uso terapéutico , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Pronóstico , Índice de Severidad de la Enfermedad , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/patología , Terapia TrombolíticaRESUMEN
INTRODUCTION: Accumulating evidence indicating that inflammatory responses play crucial roles in Parkinson's disease (PD) development provided a hypothesis that physiological alpha-synuclein may contribute to inflammatory responses against infections during non-advanced stages of PD. Thus, we examined the risk of catching a common cold in patients with PD as compared to other common brain diseases. METHODS: We extracted PD (non-advanced; without dementia) and control (AD: Alzheimer's disease, migraine, epilepsy, and ischemic stroke) patient data from insurance claim data available between 2010 and 2021. After confirming the clinical PD diagnosis, we investigated factors associated with cold diagnoses and used propensity score matching to identify differences in the incidence of colds between PD and control patients. RESULTS: Diagnosis of colds in PD patients (n = 726) and controls (AD = 377, migraine = 1019, epilepsy = 3414, ischemic stroke = 6943) was found in 1186 (9.5%) patients, which was independently associated with being female (odds ratio: OR 1.59; 95%CI 1.41-1.79; P < 0.0001), follow-up by neurologists (OR 1.30; 95%CI 1.15-1.48; P < 0.0001), diagnosis of PD (OR 0.30; 95%CI 0.20-0.45; P < 0.0001) and COVID-19 pandemic period (OR 0.58; 95%CI 0.47-0.72; P < 0.0001). After propensity score matching, the incidence of colds was significantly lower in PD (3.4%) versus in controls; AD (9.8%; P < 0.0001), migraine (13.3%; P < 0.0001), epilepsy (11.0%; P < 0.0001), ischemic stroke (8.8%; P < 0.0001). CONCLUSIONS: Patients with PD were less likely to be diagnosed with colds. However, several confounding factors will need to be examined. Moreover, alpha-synuclein may provide protective resistance to viral infections by activating the immune system due to chronic inflammation in non-advanced PD patients.
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Enfermedad de Alzheimer , COVID-19 , Resfriado Común , Accidente Cerebrovascular Isquémico , Trastornos Migrañosos , Enfermedad de Parkinson , Humanos , Femenino , Masculino , Enfermedad de Parkinson/epidemiología , Enfermedad de Parkinson/diagnóstico , alfa-Sinucleína , Resfriado Común/epidemiología , Puntaje de Propensión , Pandemias , COVID-19/epidemiología , Enfermedad de Alzheimer/diagnóstico , Accidente Cerebrovascular Isquémico/epidemiologíaRESUMEN
Neuralgic amyotrophy (NA) is a peripheral nervous system disorder involving multifocal distribution. Although nerve ultrasonography has shown potential for detecting NA lesions, no established detection method exists for distal forearm NA. A 59-year-old man presented with weakness of the muscles innervated by the left posterior interosseous nerve (PIN), median nerve (MN), anterior interosseous nerve (AIN), and ulnar nerve (UN), following severe left shoulder pain. This case suggests that nerve ultrasonography can help accurately diagnose distal forearm NA.
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Neuritis del Plexo Braquial , Enfermedades del Sistema Nervioso Periférico , Masculino , Humanos , Persona de Mediana Edad , Neuritis del Plexo Braquial/diagnóstico por imagen , Extremidad Superior , UltrasonografíaRESUMEN
Pontine autosomal dominant microangiopathy and leukoencephalopathy is one of hereditary cerebral small vessel diseases caused by pathogenic variants in COL4A1 3'UTR and characterized by multiple small infarctions in the pons. We attempted to establish radiological features of this disease. We performed whole exome sequencing and Sanger sequencing in one family with undetermined familial small vessel disease, followed by clinicoradiological assessment and a postmortem examination. We subsequently investigated clinicoradiological features of patients in a juvenile cerebral vessel disease cohort and searched for radiological features similar to those found in the aforementioned family. Sanger sequencing was performed in selected cohort patients in order to detect variants in the same gene. An identical variant in the COL4A1 3'UTR was observed in two patients with familial small vessel disease and the two selected patients, thereby confirming the pontine autosomal dominant microangiopathy and leukoencephalopathy diagnosis. Furthermore, postmortem examination showed that the distribution of thickened media tunica and hyalinized vessels was different from that in lacunar infarctions. The appearance of characteristic multiple oval small infarctions in the pons, which resemble raisin bread, enable us to make a diagnosis of pontine autosomal dominant microangiopathy and leukoencephalopathy. This feature, for which we coined the name 'raisin bread sign', was also correlated to the pathological changes.
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Factors that influence the decision of voluntary driving cessation in patients living with Parkinson's disease (PD) are still unclear. We aimed to reveal the factors affecting the decision of voluntary driving cessation in patients with PD. This hospital-based cross-sectional study recruited consecutive outpatients with PD. Data on sociodemographic and clinical characteristics and medication use were collected from the patients using semistructured interviews. Cognitive function was evaluated using the Japanese version of the Montreal Cognitive Assessment (MoCA-J). We excluded patients with dementia or motor impairment (Hoehn - Yahr stage > 3). We divided the patients into two groups, with and without voluntary driving cessation (D: driver; RD: retired driver), and conducted investigations using multivariate logistic regression analyses. Of the 40 patients, 8 (20.0%) voluntarily retired from driving. Patients who decided on driving cessation had a higher prevalence of freezing of gait (FOG) (D vs. RD, 25.0% vs. 87.5%; P = 0.001) and tended to have lower scores for attention in the MoCA-J (D vs. RD, 5.0 ± 1.2 vs. 4.1 ± 1.4; P = 0.086). Multivariable analysis showed that FOG was independently associated with driving cessation (odds ratio: 14.46, 95% confidence interval: 1.91-303.74). FOG was associated with voluntary driving cessation in patients with PD without dementia or severe motor impairment. Physicians should consider providing extensive social support to maintain patients' mobility and independence, especially if the patients have these clinical factors.
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Conducción de Automóvil , Trastornos Neurológicos de la Marcha , Enfermedad de Parkinson , Estudios Transversales , Trastornos Neurológicos de la Marcha/complicaciones , Trastornos Neurológicos de la Marcha/psicología , Humanos , Pruebas de Estado Mental y Demencia , Enfermedad de Parkinson/psicologíaRESUMEN
Cancer-associated ischemic stroke (CAS) refers to a hypercoagulation disorder related to malignant tumors, especially adenocarcinoma. Carbohydrate antigen (CA) 125 is a mucinous serum marker that might reflect hypercoagulation status, but the association between CA 125 and CAS is unclear across various types of cancer. The aim of this study was to investigate the associations among tumor markers, coagulation markers, and clinical factors in acute ischemic stroke (AIS) patients with active cancer. Consecutive AIS patients with active cancer (a diagnosis or ongoing active therapy for cancer within 6 months) were prospectively enrolled at four hospitals. D-dimer, C-reactive protein (CRP), carcinoembryonic antigen (CEA), CA19-9, and CA 125 levels were measured. Of 120 AIS patients with active cancer, 47 were diagnosed with CAS. CA 125 had the strongest correlations with D-dimer and CRP (ρ = 0.543, p < 0.001 and ρ = 0.452, p < 0.001, respectively). The areas under the receiver-operating characteristic curves for the diagnosis of CAS were 0.812 (95% CI 0.718-0.878) for CA 125, 0.714 (95% CI 0.602-0.801) for CEA, and 0.663 (95% CI 0.552-0.759) for CA 19-9. Multivariable analysis revealed that CA 125 levels in the highest quartile (OR 2.91, 95% CI 1.68-5.53), multiple lesions in multiple vascular territories observed on diffusion-weighted imaging, the absence of dyslipidemia, and the absence of atrial fibrillation were independently associated with CAS. Increased CA 125 levels, which indicate hypercoagulability, were useful for diagnosing CAS in AIS patients with active cancer.
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Accidente Cerebrovascular Isquémico , Neoplasias , Accidente Cerebrovascular , Trombofilia , Biomarcadores de Tumor , Proteína C-Reactiva , Antígeno Ca-125 , Antígeno CA-19-9 , Antígeno Carcinoembrionario , Humanos , Neoplasias/complicaciones , Accidente Cerebrovascular/complicaciones , Trombofilia/complicacionesRESUMEN
BACKGROUND: Although hypercoagulability using D-dimer levels may be a useful marker for predicting outcomes in ischemic stroke patients with cancer, other biological markers for predicting outcomes are unclear. We aimed to investigate the associations between several biological markers and short-term or long-term outcomes among ischemic stroke patients with cancer. METHODS: Consecutive acute ischemic stroke patients with cancer (n = 309) were registered. Biological markers such as hemoglobin, albumin, C-reactive protein and D-dimer levels were assessed. Stroke outcomes, namely, a 3-month modified Rankin Scale score indicating poor functional outcome (mRS score of 3-6) and 1-year survival, were assessed. RESULTS: Of the 277 patients who could be assessed for 3 months outcome, 131 patients (47.3%) had a poor outcome at 3 months. Multivariable analysis revealed that increased D-dimer levels and decreased albumin levels were independently associated with poor stroke outcomes (adjusted odds ratio [aOR]: 1.04, 95% confidence interval [CI]: 1.00-1.08, and aOR: 0.50, 95% CI: 0.31-0.80, respectively). Of 309 patients, 70 patients (22.7%) died during the follow-up period (median, 241 days). Multivariate Cox proportional hazard analyses showed that high D-dimer levels and hypoalbuminemia were independently associated with mortality (adjusted hazard ratio [aHR]: 2.65, 95% CI: 1.37-5.12, and aHR: 2.29, 95% CI: 1.21-4.49, respectively). The effect of each biological marker on mortality was notably observed among patients with active cancer but not among those with nonactive cancer. CONCLUSION: Low albumin levels were independently associated with short- and long-term outcomes, as were D-dimer levels, in acute ischemic stroke patients with cancer.
Asunto(s)
Isquemia Encefálica , Accidente Cerebrovascular Isquémico , Neoplasias , Accidente Cerebrovascular , Biomarcadores , Isquemia Encefálica/complicaciones , Proteína C-Reactiva , Humanos , Neoplasias/complicaciones , Pronóstico , Factores de Riesgo , Accidente Cerebrovascular/complicacionesRESUMEN
INTRODUCTION: The purpose of this study was to assess the clinical feasibility of diffusion tensor imaging (DTI) for the evaluation of peripheral nerves in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: Using a 3-T magnetic resonance imaging scanner, we obtained DTI scans of the tibial nerves of 10 CIDP patients and 10 sex- and age-matched healthy volunteers. We prepared fractional anisotropy (FA) maps, measured the FA values of tibial nerves, and compared these values in the two study groups. In nine patients, we also performed tibial nerve conduction studies and analyzed the correlation between the FA values and parameters of the nerve conduction study. RESULTS: The tibial nerve FA values in CIDP patients (median 0.401, range 0.312-0.510) were significantly lower than those in healthy volunteers (median 0.530, range 0.469-0.647) (Mann-Whitney test, p < 0.01). They were significantly correlated with the amplitude of action potential (Spearman correlation coefficient, p = 0.04, r = 0.86) but not with nerve conduction velocity (p = 0.79, r = 0.11). CONCLUSION: Our preliminary data suggest that the noninvasive DTI assessment of peripheral nerves may provide useful information in patients with CIDP.