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Compared with those involving the central nervous system, lymphomas involving the peripheral nervous system, namely neurolymphomatosis, are extremely rare. Neurolymphomatosis is classified as primary or secondary; the former is much rarer than the latter. Herein, we present an autopsied case of primary cauda equina lymphoma (PCEL), a type of primary neurolymphomatosis, with a literature review of autopsied cases of PCEL as well as primary neurolymphomatosis other than PCEL (non-PCEL primary neurolymphomatosis). A 70-year-old woman presented with difficulty walking, followed by paraplegia and then bladder and bowel disturbance. On magnetic resonance imaging, the cauda equina was diffusely enlarged and enhanced with gadolinium. The brainstem and cerebellum were also enhanced with gadolinium along their surface. The differential diagnosis of the patient included meningeal tumors (other than lymphomas), lymphomas, or sarcoidosis. The biopsy of the cauda equina was planned for a definite diagnosis, but because the patient deteriorated so rapidly, it was not performed. Eventually, she was affected by cranial nerve palsies. With the definite diagnosis being undetermined, the patient died approximately 1.5 years after the onset of disesase. At autopsy, the cauda equina was replaced by a bulky mass composed of atypical B-lymphoid cells, consistent with diffuse large B-cell lymphoma (DLBCL). The spinal cord was heavily infiltrated, as were the spinal/cranial nerves and subarachnoid space. There was metastasis in the left adrenal. The patient was finally diagnosed postmortem as PCEL with a DLBCL phenotype. To date, there have been a limited number of autopsied cases of PCEL and non-PCEL primary neurolymphomatosis (nine cases in all, including ours). The diagnosis is, without exception, B-cell lymphoma including DLBCL, and the histology features central nervous system parenchymal infiltration, nerve root involvement, and subarachnoid dissemination (lymphomatous meningitis). Metastases are not uncommon. All clinicians and pathologists should be aware of lymphomas primarily involving the peripheral nervous system.
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Cauda Equina , Linfoma de Células B Grandes Difuso , Neurolinfomatosis , Femenino , Humanos , Anciano , Cauda Equina/patología , Neurolinfomatosis/complicaciones , Neurolinfomatosis/patología , Gadolinio , AutopsiaRESUMEN
Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disorder represented by eosinophilic intranuclear inclusions (EIIs) and GGC/CGG repeat expansion in the NOTCH2NLC gene. We report here two adult cases of NIID, genetically confirmed, with manifestation of encephalopathy-like symptoms and address the histopathologic findings obtained by brain biopsies, with a focus on "astrocytic" intranuclear inclusions (AIIs). Case 1 presented with paroxysmal restlessness, vertigo, or fever and was later involved in severe dementia and tetraparesis. Case 2 presented with forgetfulness and then with paroxysmal fever and headache. In both cases, delimited areas with gadolinium enhancement on magnetic resonance imaging and corresponding hyperperfusion were detected, leading to brain biopsies of the cortex. On histology, Case 1 showed an abnormal lamination, where the thickness of layers was different from usual. Both neurons and astrocytes showed some dysmorphologic features. Notably, astrocytes rather than neurons harbored EIIs. Case 2 showed a cortex, where neurons tended to be arrayed in a columnar fashion. Astrocytes showed some dysmorphologic features. Notably, much more astrocytes than neurons harbored EIIs. By a double-labeling immunofluorescence study for p62/NeuN and p62/glial fibrillary acidic protein, the predominance of AIIs was confirmed in both cases. Considering the physiological functions of astrocytes for the development and maintenance of the cortex, the encephalopathy-like symptoms, dynamic change of cerebral blood flow, and cortical dysmorphology can reasonably be explained by the dysfunction of EII-bearing astrocytes rather than EII-bearing neurons. This study suggests the presence of a subtype of NIID where AIIs rather than "neuronal" intranuclear inclusions are likely a key player in the pathogenesis of NIID, particularly in cases with encephalopathy-like symptoms. The importance of AIIs ("gliopathy") should be more appreciated in future studies of NIID.
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INTRODUCTION: The magnetic resonance imaging (MRI) findings of hippocampal sclerosis (HS) include decreased volume, increased signal intensity, and hippocampal gray-white matter boundary blurring (HGWBB). Given that the layered structure is obscure in HS, there have been no reports on the quantitative evaluation of HGWBB and its relationship with the clinical outcome. Thus, this study aims to correlate the extent of HGWBB to its clinical manifestation of HS. METHODS: Fifty-four patients with temporal lobe epilepsy who underwent hippocampal resection were enrolled. To evaluate HGWBB quantitatively, we defined an index by calculating the standard deviation of the intrahippocampal signal on short tau inversion recovery. In addition, we created a prognostic scoring system using four criteria, including hippocampal signal intensity, size of hippocampal cross-sectional area, presence of temporal lobe lesions, and the HGWBB index. RESULTS: The HGWBB index was significantly lower on the affected side than on the unaffected side (p < 0.001). This trend was more prominent in the poor prognosis group than that in the good prognosis group. The prognostic scoring system revealed that when three or more criteria were positive, the prognostic accuracy reached 87.5% sensitivity and 71.7% specificity. CONCLUSION: The HGWBB index is useful for the diagnosis of temporal lobe epilepsy with HS and for predicting seizure outcomes when used with another index of hippocampal volume loss and increased signal intensity.
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Epilepsia del Lóbulo Temporal , Esclerosis del Hipocampo , Sustancia Blanca , Humanos , Epilepsia del Lóbulo Temporal/complicaciones , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/cirugía , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/patología , Hipocampo/patología , Corteza Cerebral/patología , Imagen por Resonancia Magnética/métodos , Esclerosis/patologíaRESUMEN
Neuronal intranuclear inclusion disease (NIID) is a neurological disorder characterized by eosinophilic intranuclear inclusions (INI) in systemic organs and various cell types. High-intensity signals along the corticomedullary junction on diffusion-weighted imaging and presence of cellular p62-INI in skin biopsy are known indicators for NIID. Furthermore, GGC repeat expansion in NOTCH2NLC is a characteristic genetic alteration in patients with NIID. This report presents the clinical and detailed pathological features of a male older adult with NIID. We also confirmed the presence of fluid-attenuated inversion recovery high-intensity signals in the cerebellar paravermal area, showing similar pathological changes in high-intensity signals along the corticomedullary junction on diffusion-weighted imaging.
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Cuerpos de Inclusión Intranucleares , Enfermedades Neurodegenerativas , Humanos , Masculino , Anciano , Cuerpos de Inclusión Intranucleares/patología , Enfermedades Neurodegenerativas/patología , Imagen por Resonancia Magnética , Imagen de Difusión por Resonancia MagnéticaRESUMEN
BACKGROUND AND PURPOSE: Chorea-acanthocytosis, a rare neurodegenerative disease, affects both the striatum and the medial temporal lobe which may cause involuntary movements and epilepsy, respectively. We examined the imaging changes of the hippocampus/amygdala and the striatum as well as clinical symptoms. MATERIALS AND METHODS: We retrospectively reviewed 29 MRI and 13 SPECT studies and the clinical findings of seven genetically confirmed chorea-acanthocytosis patients. We evaluated the time-dependent imaging changes of the hippocampus/amygdala and striatum and examined the relationships among these images and symptoms. RESULTS: The initial symptom was epilepsy in four patients and involuntary movements in three patients. These symptoms were eventually noted in five and all seven patients, respectively. On MRI, most patients showed striatum atrophy before a hippocampus/amygdala abnormality emerged, but one patient showed a hippocampus/amygdala abnormality before striatum atrophy. Abnormal MRI findings of hippocampus/amygdala were noted in five patients and atrophy of striatum in all seven patients. SPECT demonstrated hypoperfusion of hippocampus/amygdala in three patients and that of striatum in all five available patients. Four patients demonstrated hypoperfusion of striatum earlier than that of hippocampus/amygdala and one patient showed hypoperfusion of both simultaneously. Many imaging abnormal lesions were accompanied by their corresponding symptoms, but not always so. CONCLUSION: Striatum abnormalities were the initial imaging findings in many chorea-acanthocytosis patients, but epilepsy or hippocampus/amygdala imaging abnormalities may be the only findings at the early stage. It is important to understand the detailed clinical and imaging time courses for the diagnosis of chorea-acanthocytosis.
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Epilepsia del Lóbulo Temporal , Neuroacantocitosis , Enfermedades Neurodegenerativas , Atrofia , Hipocampo , Humanos , Imagen por Resonancia Magnética , Neuroacantocitosis/diagnóstico por imagen , Estudios RetrospectivosRESUMEN
PURPOSE: Machado-Joseph disease (MJD/SCA3) is the most frequent spinocerebellar ataxia worldwide. Pathological studies revealed less melanin-containing dopaminergic neurons in the substantia nigra (SN) pars compacta in MJD/SCA3 patients. The purpose of this study was to quantify the damage in the SN reported for MJD/SCA3 patients in vivo using neuromelanin MR imaging. METHODS: We retrospectively reviewed the clinical information and MR imaging in sixteen MJD/SCA3 patients and fourteen healthy controls. High-resolution T1-WI of the SN were acquired using a 3-T MR system. The neuromelanin-related contrast (NRC), which was defined as the number of pixels of high-signal-intensity areas on T1-WI in the SN, was calculated semi-automatically. The NRC values were compared between MJD/SCA3 patients and normal controls. RESULTS: The NRC values were significantly lower in MJD/SCA3 patients than in healthy controls. In MJD/SCA3 patients, a significant negative correlation existed between disease durations and NRC values. No significant difference in the NRC values was revealed between the MJD/SCA3 patients with and without parkinsonism. CONCLUSION: Neuromelanin in the SN may decrease at the early stage of the disease and continue to decrease over time with the disease duration in MJD/SCA3 patients. The NRC may be a useful biological index for monitoring MJD/SCA3.
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Enfermedad de Machado-Joseph/diagnóstico por imagen , Enfermedad de Machado-Joseph/metabolismo , Imagen por Resonancia Magnética/métodos , Melaninas/metabolismo , Sustancia Negra/diagnóstico por imagen , Sustancia Negra/metabolismo , Adulto , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
OBJECTIVES: To evaluate the diagnostic performance of deep learning with the convolutional neural networks (CNN) to distinguish each representative parkinsonian disorder using MRI. METHODS: This clinical retrospective study was approved by the institutional review board, and the requirement for written informed consent was waived. Midsagittal T1-weighted MRI of a total of 419 subjects (125 Parkinson's disease (PD), 98 progressive supranuclear palsy (PSP), and 54 multiple system atrophy with predominant parkinsonian features (MSA-P) patients, and 142 normal subjects) between January 2012 and April 2016 was retrospectively assessed. To deal with the overfitting problem of deep learning, all subjects were randomly divided into training (85%) and validation (15%) data sets with the same proportions of each disease and normal subjects. We trained the CNN to distinguish each parkinsonian disorder using single midsagittal T1-weighted MRI with a training group to minimize the differences between predicted output probabilities and the clinical diagnoses; then, we adopted the trained CNN to the validation data set. Subjects were classified into each parkinsonian disorder or normal condition according to the final diagnosis of the trained CNN, and we assessed the diagnostic performance of the CNN. RESULTS: The accuracies of diagnostic performances regarding PD, PSP, MSA-P, and normal subjects were 96.8, 93.7, 95.2, and 98.4%, respectively. The areas under the receiver operating characteristic curves for distinguishing each condition from others (PD, PSP, MSA-P, and normal subjects) were 0.995, 0.982, 0.990, and 1.000, respectively. CONCLUSION: Deep learning with CNN enables highly accurate discrimination of parkinsonian disorders using MRI. KEY POINTS: ⢠Deep learning convolution neural network achieves differential diagnosis of PD, PSP, MSA-P, and normal controls with an accuracy of 96.8, 93.7, 95.2, and 98.4%, respectively. ⢠The areas under the curves for distinguishing between PD, PSP, MSA-P, and normality were 0.995, 0.982, 0.990, and 1.000, respectively. ⢠CNN may learn important features that humans not notice, and has a possibility to perform previously impossible diagnoses.
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Aprendizaje Profundo , Trastornos Parkinsonianos/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Atrofia de Múltiples Sistemas/diagnóstico , Redes Neurales de la Computación , Enfermedad de Parkinson/diagnóstico , Prueba de Estudio Conceptual , Curva ROC , Estudios Retrospectivos , Parálisis Supranuclear Progresiva/diagnósticoRESUMEN
Multinodular and vacuolating neuronal tumors (MVNT) have been referred to as distinctive neuronal tumors whose characteristic features include multiple nodules localized in the subcortical white matter. MVNT are composed of vacuolating dysplastic neurons reactive to HuC/HuD. A significant overexpression of alpha-internexin (INA) limited to the stroma of nodules was reported in one tumor. Since genetic analyses have failed to demonstrate any consistent alterations, the nosological position as well as the nature of MVNT, namely, neoplastic or dysplastic, remains unclear. We herein present another example of MVNT involving the amygdala and anterior hippocampus in a 41-year-old man. In addition to the nodular lesions described earlier, we found INA-positive ribbon-like lesions that replaced neuropil and extended along the hippocampal gray matter. We also identified dysplastic neurons infiltrating into the CA4 hilus of the hippocampus. Intense INA expression was present in the stroma as well as the cytoplasmic membrane of dysplastic neurons and their processes. While the invasiveness suggested a neoplasm, a relatively restrictive, either nodular or ribbon-like growth pattern with INA-positive abnormal neuropil suggested a hamartoma. Such quasi-tumors should be accommodated in the World Health Organization classification of tumors of the central nervous system, as are dysembryoplastic neuroepithelial tumor and Lhermitte-Duclos disease.
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Amígdala del Cerebelo/patología , Neoplasias Encefálicas/patología , Proteínas de Filamentos Intermediarios/metabolismo , Malformaciones del Desarrollo Cortical/patología , Adulto , Amígdala del Cerebelo/metabolismo , Neoplasias Encefálicas/metabolismo , Hipocampo/metabolismo , Hipocampo/patología , Humanos , Masculino , Malformaciones del Desarrollo Cortical/metabolismoRESUMEN
OBJECTIVE: Amygdala enlargement (AE) has been suggested to be a subtype of mesial temporal lobe epilepsy (MTLE). However, most reports related to AE have referred to imaging studies, and there have been few reports regarding surgical and pathological findings. The present study was performed to clarify the surgical outcomes and pathology of AE. METHODS: Eighty patients with drug-resistant MTLE were treated surgically at the Tokyo Metropolitan Neurological Hospital between April 2010 and July 2013. Of these patients, 11 were diagnosed as AE based on presurgical MRI. Nine patients with AE underwent selective amygdalohippocampectomy, while the remaining two patients underwent selective amygdalotomy with hippocampal transection. Intraoperative EEG was routinely performed. The histopathology of the resected amygdala tissue was evaluated and compared with the amygdala tissue of patients with hippocampal sclerosis. RESULTS: Pathological findings indicated that 10 of 11 specimens had closely clustering hypertrophic neurons with vacuolisation of the background matrix. Slight gliosis was seen in nine specimens, while the remaining two showed no gliotic changes. Intraoperative EEG showed abnormal sharp waves that seemed to originate not from the amygdala but from the hippocampus in all cases. Ten patients became seizure-free during the postoperative follow-up period. CONCLUSIONS: Histopathologically, clustering hypertrophic neurons and vacuolation with slight gliosis or without gliosis were considered to be pathological characteristics of AE. Amygdalohippocampectomy or hippocampal transection with amygdalotomy is effective for seizure control in patients with AE.
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Amígdala del Cerebelo/cirugía , Epilepsia del Lóbulo Temporal/cirugía , Adolescente , Adulto , Amígdala del Cerebelo/patología , Electroencefalografía , Epilepsia del Lóbulo Temporal/patología , Femenino , Humanos , Hipertrofia , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neuroimagen , Resultado del Tratamiento , Adulto JovenRESUMEN
AIMS: The purpose of this study was to evaluate the clinical impact of addition of [(11)C]Pittsburgh compound-B positron emission tomography ((11)C-PiB PET) on routine clinical diagnosis of Alzheimer's disease (AD) dementia and mild cognitive impairment (MCI), and to assess diagnostic agreement between clinical criteria and research criteria of the National Institute on Aging-Alzheimer's Association. METHODS: The diagnosis in 85 patients was made according to clinical criteria. Imaging examinations, including both magnetic resonance imaging and single-photon emission computed tomography/computed tomography to identify neuronal injury (NI), and (11)C-PiB PET to identify amyloid were performed, and all subjects were re-categorized according to the research criteria. RESULTS: Among 40 patients with probable AD dementia (ProAD), 37 were NI-positive, 29 were (11)C-PiB-positive, and 27 who were both NI- and (11C-PiB-positive were categorized as having 'ProAD dementia with a high level of evidence of the AD pathophysiological process'. Among 20 patients with possible AD dementia (PosAD), 17 were NI-positive, and six who were both NI- and (11)C-PiB-positive were categorized as having 'PosAD with evidence of the AD pathophysiological process'. Among 25 patients with MCI, 18 were NI-positive, 13 were (11)C-PiB-positive, and 10 who were both NI- and (11)C-PiB-positive were categorized as having 'MCI due to AD-high likelihood'. CONCLUSIONS: Diagnostic concordance between clinical criteria and research criteria may not be high in this study. (11)C-PiB PET may be of value in making the diagnosis of dementia and MCI in cases with high diagnostic uncertainty.
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Enfermedad de Alzheimer/diagnóstico por imagen , Amiloide/metabolismo , Benzotiazoles , Encéfalo/diagnóstico por imagen , Radioisótopos de Carbono , Disfunción Cognitiva/diagnóstico por imagen , Anciano , Anciano de 80 o más Años , Enfermedad de Alzheimer/diagnóstico , Compuestos de Anilina , Encéfalo/metabolismo , Encéfalo/patología , Disfunción Cognitiva/diagnóstico , Demencia/diagnóstico , Demencia/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Imagen Multimodal , Tomografía de Emisión de Positrones , Tiazoles , Tomografía Computarizada de Emisión de Fotón Único , Tomografía Computarizada por Rayos XRESUMEN
Static encephalopathy of childhood with neurodegeneration in adulthood (SENDA) is an X-linked dominant neurodegenerative disorder, and is classified as a subtype of neurodegeneration with brain iron accumulation. Recently, de novo heterozygous mutations in WDR45 at Xp11.23 have been reported in patients with SENDA. We report the clinical and neuroradiological findings of a patient with SENDA with a novel c.322del mutation in WDR45. In this patient, characteristic MRI findings were useful for diagnosis.
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Encefalopatías/complicaciones , Encefalopatías/genética , Proteínas Portadoras/genética , Mutación/genética , Enfermedades Neurodegenerativas/complicaciones , Enfermedades Neurodegenerativas/genética , Adulto , Secuencia de Bases , Encefalopatías/patología , Niño , Preescolar , Análisis Mutacional de ADN , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética , Datos de Secuencia MolecularRESUMEN
OBJECTIVES: The purpose of this study was to clarify the concordance of diagnostic abilities and interobserver agreement between 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and brain perfusion single photon-emission computed tomography (SPECT) in patients with Alzheimer's disease (AD) who were diagnosed according to the research criteria of the National Institute of Aging-Alzheimer's Association Workshop. METHODS: Fifty-five patients with "AD and mild cognitive impairment (MCI)" (n = 40) and "non-AD" (n = 15) were evaluated with 18F-FDG PET and (99m)Tc-ethyl cysteinate dimer (ECD) SPECT during an 8-week period. Three radiologists independently graded the regional uptake in the frontal, temporal, parietal, and occipital lobes as well as the precuneus/posterior cingulate cortex in both images. Kappa values were used to determine the interobserver reliability regarding regional uptake. RESULTS: The regions with better interobserver reliability between 18F-FDG PET and (99m)Tc-ECD SPECT were the frontal, parietal, and temporal lobes. The (99m)Tc-ECD SPECT agreement in the occipital lobes was not significant. The frontal, temporal, and parietal lobes showed good correlations between 18F-FDG PET and (99m)Tc-ECD SPECT in the degree of uptake, but the occipital lobe and precuneus/posterior cingulate cortex did not show good correlations. The diagnostic accuracy rates of "AD and MCI" ranged from 60% to 70% in both of the techniques. CONCLUSIONS: The degree of uptake on 18F-FDG PET and (99m)Tc-ECD SPECT showed significant correlations in the frontal, temporal, and parietal lobes. The diagnostic abilities of 18F-FDG PET and (99m)Tc-ECD SPECT for "AD and MCI," when diagnosed according to the National Institute of Aging-Alzheimer's Association Workshop criteria, were nearly identical.
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Enfermedad de Alzheimer/diagnóstico por imagen , Trastornos del Conocimiento/diagnóstico por imagen , Cisteína/análogos & derivados , Fluorodesoxiglucosa F18 , Compuestos de Organotecnecio , Tomografía de Emisión de Positrones/métodos , Radiofármacos , Tomografía Computarizada de Emisión de Fotón Único/métodos , Anciano , Anciano de 80 o más Años , Enfermedad de Alzheimer/metabolismo , Encéfalo/metabolismo , Trastornos del Conocimiento/metabolismo , Femenino , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Tomografía de Emisión de Positrones/normas , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Tomografía Computarizada de Emisión de Fotón Único/normasRESUMEN
BACKGROUND AND PURPOSE: In infants with hemimegalencephaly, asymmetrical white-matter intensities suggestive of advanced myelination are observed as well as aberrant midsagittal fibers (AMFs) specific to hemimegalencephaly. Also noted are otherwise unreported abnormally enlarged periventricular fibers (APVFs) running anteroposteriorly along the caudate nucleus. This study investigated the degree of myelination and presence of aberrant fibers in hemimegalencephaly through a retrospective review of MRI scans in relation to histopathological findings. MATERIALS AND METHODS: MRI scans of 24 infants with hemimegalencephaly (13 boys and 11 girls, 1-9 months old) were evaluated, focusing on the presence and signal intensities of AMFs and APVFs. White-matter signal intensities on T1- and T2-weighted imaging of the cerebral hemisphere were also evaluated and compared with the timetable for normal myelination. Surgical specimens were pathologically examined with Klüver-Barrera staining in four patients. RESULTS: AMFs and APVFs were observed in 18 and nine patients, respectively, while 22 patients had accelerated myelination of the megalencephalic hemisphere that tended to extend along fiber pathways including AMFs and APVFs. In six cases, accelerated myelination even extended into the contralateral hemisphere via the corpus callosum or AMFs. Histopathological analysis identified hypermyelination with disarrayed myelinated fibers corresponding to MRI findings. CONCLUSION: Accelerated myelination is frequently observed in patients with hemimegalencephaly and tends to extend along fiber pathways, including aberrant or abnormal fibers, as seen in 75% of hemimegalencephaly patients. Accelerated myelination may reflect propagation pathways of abnormal brain activity in such patients.
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Encéfalo/patología , Imagen de Difusión Tensora/métodos , Hemimegalencefalia/patología , Vaina de Mielina/patología , Fibras Nerviosas Mielínicas/patología , Vías Nerviosas/patología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Reproducibilidad de los Resultados , Sensibilidad y EspecificidadRESUMEN
Background: Subdural electrode (SDE) implantation is an important method of diagnosing epileptogenic lesions and mapping brain function, even with the current preference for stereoelectroencephalography. We developed a novel method to assess SDEs and the brain surface during the electrode implantation procedure using brain images printed onto permeable films and intraoperative fluoroscopy. This method can help verify the location of the electrode during surgery and improve the accuracy of SDE implantation. Methods: We performed preoperative imaging by magnetic resonance imaging and computed tomography. Subsequently, the images were edited and fused to visualize the gyrus and sulcus better. We printed the images on permeable films and superimposed them on the intraoperative fluoroscopy display. The intraoperative and postoperative coordinates of the electrodes were obtained after the implantation surgery, and the differences in the locations were calculated. Results: Permeable films were created for a total of eight patients with intractable epilepsy. The median difference of the electrodes between the intraoperative and postoperative images was 4.6 mm (Interquartile range 2.9-7.1). The locations of electrodes implanted outside the operation field were not significantly different from those implanted inside. Conclusion: Our new method may guide the implantation of SDEs into their planned location.
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This study aimed to determine the prevalence of lumbar ligamentum flavum lesions and identify correlations between radiological and pathological findings. We conducted an observational cross-sectional study of 349 patients (lumbar: n = 296, thoracic: n = 39, lumbar and thoracic: n = 14, mean age: 69 ± 12 years, male: 74%) who underwent posterior surgery for thoracolumbar spinal canal stenosis between January 2008 and April 2023 at our hospital.Computed tomography (CT) revealed that the prevalence of ligamentum flavum lesions defined as a high-density area with a CT value of 200 Hounsfield Unit or higher in the lumbar and thoracic spine was 47% (147/310) and 85% (45/53), respectively. CT showed that most patients had radiologically suspected ossification in the lumbar (90%) and thoracic spine (98%) than radiologically suspected calcification. Lumbar lesions were thinner than the thoracic lesions (2.5 vs 3.7 mm, p < 0.01). Pathological examinations were performed in specimens collected from 34 cases (lumbar: n = 13, thoracic: n = 21), and ossification was found in 62% (8/13) and 95% (20/21) of lumbar and thoracic lesions (p = 0.02), respectively. Lastly, ossification was confirmed pathologically in 72% (8/11) and 95% (19/20) of lumbar and thoracic lesions that showed ossification on imaging (p = 0.13), respectively. The literature review revealed that the prevalence of the lumbar ligamentum flavum lesions varied from 1.5 to 35% and the patient population was mostly asymptomatic.Collectively, we found that the prevalence of lumbar ligamentum flavum lesions in symptomatic patients was greater than previously reported. Histologically confirmed ossification was less common in lumbar lesions than in thoracic lesions.
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Diagnosing neuralgic amyotrophy can be challenging in clinical practice. Here, we report the case of a 37-years old Japanese woman who suddenly developed neuropathic pain in the right upper limb after influenza vaccination. The pain, especially at night, was severe and unrelenting, which disturbed her sleep. However, X-ray and MRI did not reveal any fractures or muscle injuries, and brain MRI did not reveal any abnormalities. During neurological consultation, she was in a posture of flexion at the elbow and adduction at the shoulder. Manual muscle testing suggested weakness of the flexor pollicis longus, pronator quadratus, flexor carpi radialis (FCR), and pronator teres (PT), while the flexor digitorum profundus was intact. Medical history and neurological examination suggested neuralgic amyotrophy, particularly anterior interosseous nerve syndrome (AINS) with PT/FCR involvement. Innervation patterns on muscle MRI were compatible with the clinical findings. Conservative treatment with pain medication and oral corticosteroids relieved the pain to minimum discomfort, whereas weakness remained for approximately 3 months. For surgical exploration, lesions above the elbow and fascicles of the median nerve before branching to the PT/FCR were indicated on neurological examinations; thus, we performed high-resolution imaging to detect possible pathognomonic fascicular constrictions. While fascicular constrictions were not evident on ultrasonography, MR neurography indicated fascicular constriction proximal to the elbow joint line, of which the medial topographical regions of the median nerve were abnormally enlarged and showed marked hyperintensity on short-tau inversion recovery. In patients with AINS, when spontaneous regeneration cannot be expected, timely surgical exploration should be considered for a good outcome. In our case, MR neurography was a useful modality for assessing fascicular constrictions when the imaging protocols were appropriately optimized based on clinical assessment.
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Neuritis del Plexo Braquial , Nervio Mediano , Humanos , Femenino , Adulto , Nervio Mediano/diagnóstico por imagen , Neuritis del Plexo Braquial/diagnóstico por imagen , Neuritis del Plexo Braquial/etiología , Neuritis del Plexo Braquial/patología , Constricción , Imagen por Resonancia Magnética , Constricción Patológica/patología , DolorRESUMEN
Introduction: The present study characterized the degeneration of nigrostriatal dopaminergic neurons in the early stages of parkinsonian disorders using integrative neuroimaging analysis with neuromelanin-sensitive MRI and 123I-FP-CIT dopamine transporter (DAT) SPECT. Methods: Thirty-one, 30, and 29 patients with progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) with abnormal specific binding ratio (SBR) in either hemisphere (mean ± 2SD), and parkinsonism-predominant multiple system atrophy (MSA-P), respectively, were enrolled. Neuromelanin-related contrast (NRC) in the substantia nigra (NRCSN) and locus coeruleus (NRCLC) and the SBR of DAT SPECT were measured. All the patients underwent both examinations simultaneously within five years after symptom onset. After adjusting for interhemispheric asymmetry on neuromelanin-related MRI contrast using the Z-score, linear regression analysis of the NRCSN and SBR was performed for the most- and least-affected hemispheres, as defined by the interhemispheric differences per variable (SBR, NRCSN, standardized [SBR + NRCSN]) in each patient. Results: Although the variables did not differ significantly between PSP and CBS, a significant correlation was found for CBS in the most-affected hemisphere for all the definitions, including the clinically defined, most-affected hemisphere. No significant correlation was found between the NRCSN and SBR for any of the definitions in either PSP or MSA-P. Conclusion: Together with the findings of our previous study of dementia with Lewy bodies (DLB) and Parkinson's disease (PD), the present findings indicated that neural degeneration in the disorders examined may be categorized by the significance of the NRCSN-SBR correlation in PD and CBS and its non-significance in DLB, PSP, and MSA-P.
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Cerebral small vessel disease (cSVD) refers to a group of pathological processes with various etiologies affecting the small vessels of the brain. Most cases are sporadic, with age-related and hypertension-related sSVD and cerebral amyloid angiopathy being the most prevalent forms. Monogenic cSVD accounts for up to 5% of causes of stroke. Several causative genes have been identified. Sporadic cSVD has been widely studied whereas monogenic cSVD is still poorly characterized and understood. The majority of cases of both the sporadic and monogenic types, including cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), typically have their onset in adulthood. Types of cSVD with infantile and childhood onset are rare, and their diagnosis is often challenging. The present review discusses the clinical and neuroimaging findings of monogenic cSVD from the prenatal to adolescent period of development. Early diagnosis is crucial to enabling timely interventions and family counseling.
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CADASIL , Enfermedades de los Pequeños Vasos Cerebrales , Accidente Cerebrovascular , Adolescente , Humanos , Niño , Enfermedades de los Pequeños Vasos Cerebrales/diagnóstico por imagen , Enfermedades de los Pequeños Vasos Cerebrales/genética , CADASIL/complicaciones , CADASIL/genética , Accidente Cerebrovascular/complicaciones , Infarto Cerebral/complicaciones , NeuroimagenRESUMEN
OBJECTIVE: Dysphagia in multiple system atrophy (MSA) is life-threatening and is caused by parkinsonism with cerebellar ataxia as a contributing factor. The present study investigated the relationship between dysphagia severity in MSA and the specific binding ratio (SBR) on dopamine transporter (DaT) SPECT using the Hyodo score, a qualitative scale for use with fiberoptic endoscopic evaluation of swallowing (FEES). METHODS: Hyodo score's ability to predict aspiration during a FEES examination of 88 patients with MSA was first tested. Then the clinical characteristics, Hyodo score, and SBR of patients with either predominant parkinsonism (MSA-P; n = 11) or cerebellar ataxia (MSA-C; n = 25) who underwent FEES and DaT SPECT simultaneously were compared. RESULTS: Logistic regression demonstrated that the Hyodo score was a significant predictive factor of aspiration (p = 0.003). The MSA-P group had a significantly higher Hyodo score (p = 0.026) and lower SBR (p = 0.011) than the MSA-C group while neither group demonstrated any significant difference in disease duration at the FEES examination. Linear regression demonstrated a significant, inverse correlation between the Hyodo score and SBR in the MSA-P (p = 0.044; r = -0.616) and MSA-C (p = 0.044; r = -0.406) groups. When the effect of SBR was removed by analysis of covariance, no significant difference in the Hyodo score remained between the groups. CONCLUSIONS: Our results suggested an association between presynaptic changes in nigrostriatal dopaminergic neurons and dysphagia severity in MSA which largely contributes to the difference in dysphagia severity between MSA-P and MSA-C.
Asunto(s)
Trastornos de Deglución , Proteínas de Transporte de Dopamina a través de la Membrana Plasmática , Atrofia de Múltiples Sistemas , Índice de Severidad de la Enfermedad , Tomografía Computarizada de Emisión de Fotón Único , Humanos , Atrofia de Múltiples Sistemas/diagnóstico por imagen , Atrofia de Múltiples Sistemas/complicaciones , Atrofia de Múltiples Sistemas/metabolismo , Masculino , Femenino , Trastornos de Deglución/diagnóstico por imagen , Trastornos de Deglución/etiología , Anciano , Proteínas de Transporte de Dopamina a través de la Membrana Plasmática/metabolismo , Persona de Mediana EdadRESUMEN
BACKGROUND: The prognostic impact of dysphagia in multiple system atrophy (MSA) remains controversial. This study aimed to investigate the relationship between dysphagia severity and survival in MSA and to elucidate whether this impact differs between MSA-cerebellar ataxia (MSA-C) and MSA-parkinsonism (MSA-P). METHODS: This retrospective study included 297 patients with MSA: 251 met criteria for clinically established MSA and 46 for clinically probable MSA. Among them, 171 had MSA-C and 126 had MSA-P. We evaluated symptomatic dysphagia within 3 years of onset and quantified dysphagia severity using the Hyodo score (0 to 12) through fibreoptic endoscopic evaluation of swallowing (FEES) and clinical features, including autonomic dysfunction and vocal cord paralysis. Patients were followed up until death or tracheostomy, and survival factors were analysed using the log-rank test and multivariate Cox proportional hazards model. RESULTS: Ninety patients developed symptomatic dysphagia within 3 years of onset, and 75 were evaluated for dysphagia severity using FEES. Survival from onset was shorter in patients with dysphagia within 3 years compared to those without (median: 4.2 years vs. 7.3 years; p < 0.001). Symptomatic dysphagia within 3 years of onset was an independent predictor of shorter survival in the multivariate Cox analysis. While the Hyodo score was higher in MSA-P than in MSA-C patients (p = 0.048), the Hyodo score was associated with survival in both MSA-C and MSA-P patients (log-rank p < 0.001 and p = 0.046, respectively). CONCLUSION: Symptomatic dysphagia within 3 years of onset predicts shorter survival in MSA-C and MSA-P patients.