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1.
Radiology ; 271(1): 172-82, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24475792

RESUMEN

PURPOSE: To provide normal values of the cervical spinal canal and spinal cord dimensions in several planes with respect to spinal level, age, sex, and body height. MATERIALS AND METHODS: This study was approved by the institutional review board; all individuals provided signed informed consent. In a prospective multicenter study, two blinded raters independently examined cervical spine magnetic resonance (MR) images of 140 healthy volunteers who were white. The midsagittal diameters and areas of spinal canal and spinal cord, respectively, were measured at the midvertebral levels of C1, C3, and C6. A multivariate general linear model described the influence of sex, body height, age, and spinal level on the measured values. RESULTS: There were differences for sex, spinal level, interaction between sex and level, and body height, while age had significant yet limited influence. Normative ranges for the sagittal diameters and areas of spinal canal and spinal cord were defined at C1, C3, and C6 levels for men and women. In addition to a calculation of normative ranges for a specific sex, spinal level, age, and body height data, data for three different height subgroups at 45 years of age were extracted. These results show a range of the spinal canal dimensions at C1 (from 10.7 to 19.7 mm), C3 (from 9.4 to 17.2 mm), and C6 (from 9.2 to 16.8 mm) levels. CONCLUSION: The dimensions of the cervical spinal canal and cord in healthy individuals are associated with spinal level, sex, age, and height. Online supplemental material is available for this article.


Asunto(s)
Vértebras Cervicales/anatomía & histología , Imagen por Resonancia Magnética/métodos , Canal Medular/anatomía & histología , Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Valores de Referencia , Encuestas y Cuestionarios , Suiza
2.
Semin Musculoskelet Radiol ; 17(4): 380-8, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-24101178

RESUMEN

The current knowledge and evidence around the merits of different imaging modalities for the evaluation of cervical spine injuries are reviewed. The National Emergency X-Radiography Use Study, Canadian Cervical Spine rule, and American College of Radiology appropriateness criteria are reviewed and summarized. The advantages and disadvantages of available imaging modalities for selected cervical spine injury patterns are also illuminated to simplify the decision making on when to use which modality.


Asunto(s)
Vértebras Cervicales/lesiones , Diagnóstico por Imagen , Traumatismos Vertebrales/diagnóstico , Enfermedad Aguda , Humanos , Traumatismos Vertebrales/fisiopatología
3.
Radiology ; 262(2): 567-75, 2012 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-22187629

RESUMEN

PURPOSE: To compare the magnetic resonance (MR) imaging findings in patients with acute whiplash injury with those in matched control subjects. MATERIALS AND METHODS: In a prospective multicenter controlled study, from 2005 to 2008, 100 consecutive patients underwent 1.5-T MR imaging examinations of the cervical spine within 48 hours after a motor vehicle accident. Findings in these patients were compared in a blinded fashion with those in 100 age- and sex-matched healthy control subjects. Four blinded independent readers assessed the presence of occult vertebral body and facet fractures, vertebral body and facet contusions, intervertebral disk herniations, ligamentum nuchae strains, ligamentum nuchae tears, muscle strains or tears, and perimuscular fluid. Accuracy (as compared with clinical findings) and interobserver reliability were calculated. RESULTS: Accuracy of MR imaging and interreader reliability were generally poor (sensitivity, 0.328; specificity, 0.728; positive and negative likelihood ratios, 1.283 and 1.084, respectively). MR imaging findings significantly associated with whiplash injuries were occult fracture (P<.01), bone marrow contusion of the vertebral body (P=.01), muscle strain (P<.01) or tear (P<.01), and the presence of perimuscular fluid (P<.01). While 10 findings thought to be specific for whiplash trauma were significantly (P<.01) more frequent in patients (507 observations), they were also regularly found in healthy control subjects (237 observations). There were no serious occult injuries that required immediate therapy. CONCLUSION: MR imaging at 1.5 T reveals only limited evidence of specific changes to the cervical spine and the surrounding tissues in patients with acute symptomatic whiplash injury compared with healthy control subjects.


Asunto(s)
Vértebras Cervicales/lesiones , Vértebras Cervicales/patología , Imagen por Resonancia Magnética/métodos , Lesiones por Latigazo Cervical/patología , Accidentes de Tránsito , Enfermedad Aguda , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Método Simple Ciego , Adulto Joven
4.
J Magn Reson Imaging ; 36(6): 1413-20, 2012 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-22865713

RESUMEN

PURPOSE: To investigate the role of the cervical spine muscles in whiplash injury. We hypothesized that (i) cervical muscle hypotrophy would be evident after a 6-month follow-up and, (ii) cervical muscle hypotrophy would correlate with symptom persistence probably related to pain or inactivity. MATERIALS AND METHODS: Ninety symptomatic patients (48 females) were recruited from our emergency department and examined within 48 h, and at 3, and 6 months after a motor vehicle accident. MRI cross-sectional muscle area (CSA) measurements were performed bilaterally of the cervical extensor and sternocleidomastoid muscles using transverse STIR (Short Tau inversion Recovery) sequences at the C2 (deep and total dorsal cervical extensor muscles), C4 (sternocleidomastoid muscles) and C5 (deep and total dorsal cervical extensor muscles) levels. Two blinded raters independently performed the measurements at each time point. First, CSA changes over time were analyzed and, second, CSAs were correlated with clinical outcomes (EuroQuol, Whiplash Disability Score, neck pain intensity [VAS], cervical spine mobility). RESULTS: There was a high agreement of CSA measurements between the two raters. Women consistently had smaller CSAs than men. There were no significant changes of CSAs over time at any of the three levels. There were no consistent significant correlations of CSA values with the clinical scores at all time points except with the body mass index. CONCLUSION: Our results do not support a major role of cervical muscle volume in the genesis of symptoms after whiplash injury.


Asunto(s)
Interpretación de Imagen Asistida por Computador/métodos , Imagen por Resonancia Magnética/métodos , Atrofia Muscular/etiología , Atrofia Muscular/patología , Músculos del Cuello/patología , Lesiones por Latigazo Cervical/complicaciones , Lesiones por Latigazo Cervical/patología , Adulto , Anatomía Transversal/métodos , Vértebras Cervicales/patología , Femenino , Estudios de Seguimiento , Humanos , Aumento de la Imagen/métodos , Masculino , Reproducibilidad de los Resultados , Sensibilidad y Especificidad
5.
J Magn Reson Imaging ; 33(3): 668-75, 2011 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-21563251

RESUMEN

PURPOSE: To quantitatively compare the muscle cross-sectional areas (CSAs) of the cervical muscles in symptomatic acute whiplash patients versus healthy controls. We hypothesized, that symptomatic whiplash patients have smaller cervical muscle CSAs than matched controls and that smaller cervical muscle CSAs in women might explain that women more frequently are symptomatic after whiplash injury than men. MATERIALS AND METHODS: Prospective controlled study. Thirty-eight consecutive acute whiplash patients were examined within 48 h after a motor vehicle accident and 38 healthy age- and sex-matched controls, each half female, half male, were examined with the same protocol. MRI CSA measurements were performed of the deep and total cervical extensor muscles as well as the sternocleidomastoid muscles using transversal STIR (Short T1 Inversion Recovery) sequences on level C2, C4, and C5 by two blinded raters. Clinical symptoms were assessed with patient questionnaires (EuroQuol 5D, Specific Whiplash Questionnaire, head- and neck pain intensity [VAS]). RESULTS: Agreement of measurements between the two raters was high (intraclass correlation 0.52 to 0.85 for the different levels). No significant difference in age and body mass index were seen between patients and controls and the distribution of genders across groups was identical. There were no significant differences between patients and controls for all CSAs. Women had consistently smaller CSAs than men. The CSAs showed no significant correlation with the pain intensity of neck pain and headache but a consistent tendency of less neck pain and more headache with greater CSAs. CONCLUSION: This small study provides no evidence that subjects with smaller CSAs of cervical extensor muscles have a higher risk in developing symptoms after a whiplash injury and confirms smaller CSA in women.


Asunto(s)
Imagen por Resonancia Magnética/métodos , Lesiones por Latigazo Cervical/diagnóstico , Accidentes de Tránsito , Adulto , Índice de Masa Corporal , Estudios de Casos y Controles , Vértebras Cervicales/patología , Femenino , Humanos , Masculino , Modelos Estadísticos , Músculos/patología , Dolor , Estudios Prospectivos , Encuestas y Cuestionarios
6.
AJR Am J Roentgenol ; 197(4): 961-7, 2011 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21940586

RESUMEN

OBJECTIVE: The objective of our study was to evaluate whether there is injury to the transverse ligament of the atlas in patients with acute whiplash. MATERIALS AND METHODS: Ninety patients with an acute (< 48 hours) symptomatic whiplash-associated injury and 90 healthy age- and sex-matched asymptomatic control subjects (mean age of patients and control subjects, 36 years) were included. The maximal sagittal thickness of the transverse ligament was measured on midsagittal T1 volumetric interpolated breath-hold examination (VIBE) images and transverse reformatted VIBE images. The signal intensity of the transverse ligament was measured on transverse STIR images and on transverse reformatted T1 VIBE images before and after IV administration of gadoterate. Contrast between the transverse ligament and CSF and alterations of contrast after gadoterate injection were calculated. RESULTS: Patients had a minimally thicker transverse ligament (posttraumatic swelling) than control subjects, and the difference in thickness was significant in men only (p = 0.03). In patients, a significant signal alteration of the transverse ligament (p = 0.03) was seen on STIR (posttraumatic edema) and native VIBE sequences. The contrast between the transverse ligament and the CSF on VIBE images was significantly (p = 0.005) lower in patients than in control subjects. With the application of a contrast agent, the contrast difference between the transverse ligament and CSF in patients and control subjects was less pronounced (p = 0.038). There was no abnormal uptake of contrast agent by the transverse ligament or CSF. CONCLUSION: The results of our study indicate possible involvement of the transverse ligament in whiplash injury. Although MRI may be helpful to study injury-related changes of anatomic structures in cohorts, it is not suited for individual diagnosis because the alterations are too small.


Asunto(s)
Ligamentos/lesiones , Imagen por Resonancia Magnética/métodos , Lesiones por Latigazo Cervical/patología , Accidentes de Tránsito , Adulto , Estudios de Casos y Controles , Medios de Contraste , Femenino , Compuestos Heterocíclicos , Humanos , Interpretación de Imagen Asistida por Computador , Modelos Lineales , Masculino , Persona de Mediana Edad , Compuestos Organometálicos , Curva ROC
7.
J Peripher Nerv Syst ; 16(2): 92-7, 2011 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-21692906

RESUMEN

Intravenous immunoglobulin (IVIG) is the first-line therapy for multifocal motor neuropathy (MMN). This open-label multi-centre study (NCT00701662) assessed the efficacy, safety, and convenience of subcutaneous immunoglobulin (SCIG) in patients with MMN over 6 months, as an alternative to IVIG. Eight MMN patients (42-66 years), on stable IVIG dosing, received weekly SCIG at doses equivalent to previous IVIG using a "smooth transition protocol". Primary efficacy endpoint was the change from baseline to week 24 in muscle strength. Disability, motor function, and health-related quality of life (HRQL) endpoints were also assessed. One patient deteriorated despite dose increase and was withdrawn. Muscle strength, disability, motor function, and health status were unchanged in all seven study completers who rated home treatment as extremely good. Four experienced 18 adverse events, of which only two were moderate. This study suggests that MMN patients with stable clinical course on regular IVIG can be switched to SCIG at the same monthly dose without deterioration and with a sustained overall improvement in HRQL.


Asunto(s)
Enfermedades Desmielinizantes/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunoglobulinas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Polineuropatías/tratamiento farmacológico , Adulto , Anciano , Evaluación de la Discapacidad , Femenino , Humanos , Infusiones Subcutáneas , Masculino , Persona de Mediana Edad , Fuerza Muscular/efectos de los fármacos , Calidad de Vida
8.
Neurol Res Pract ; 2: 33, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33225223

RESUMEN

INTRODUCTION: In view of the importance of neurosyphilis and the difficulties encountered in diagnosing it, the S1 guideline "Neurosyphilis" has been published by the German Society for Neurology (DGN) in accordance with the stipulations of the Association of the Scientific Medical Societies in Germany (AWMF). The present article is an abridged translation of that German guideline. MAIN RECOMMENDATIONS: (a) Neurosyphilis can manifest as early neurosyphilis (meningitis, meningovascular neurosyphilis or syphilitic gummas) or late neurosyphilis (tabes dorsalis, general paresis). (b) The following diagnostic criteria help to establish the presence of probable neurosyphilis (always point iv, accompanied by any two of points i to iii): (i) subacute or chronic neuro-psychiatric symptoms; (ii) increased cerebrospinal fluid (CSF) cell count or signs of blood-CSF barrier disruption; (iii) positive effect of anti-neurosyphilis antibiotic therapy on clinical course and CSF findings; (iv) positive TPHA/TPPA or FTA test in serum. (c) The diagnosis of neurosyphilis is confirmed by the subsequent detection of intrathecal production of antibodies against Treponema pallidum. (d) In neurosyphilis, treatment with intravenous penicillin or ceftriaxone for 14 days is recommended. (e) The following parameters can be used to assess a therapeutic effect: clinical findings, serum VDRL, and CSF cell count. CONCLUSION: The German guideline on the diagnosis and treatment of neurosyphilis is a practical tool to support clinicians in diagnosing and treating patients with neurosyphilis. This article is an abridged translation of this guideline (Klein MW, J.; Angstwurm, K.; Esser, S.; Hahn, K.; Matschke, M.; Scheithauer, S.; Schoefer, H.; Sturzenegger, M.; Wildemann, B. Neurosyphilis, S1-Leitlinie. Deutsche Gesellschaft für Neurologie, Leitlinien für Diagnostik und Thearpie in der Neurologie 2020).

9.
Stroke ; 40(2): 499-504, 2009 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-19109549

RESUMEN

BACKGROUND AND PURPOSE: We set out to investigate the predictors and time course for recanalization of spontaneous dissection of the cervical internal carotid artery (SICAD). METHODS: We prospectively included 249 consecutive patients (mean age, 45+/-11 years) with 268 SICAD. Ultrasound examinations were performed at presentation, during the first month, and then at 3, 6, and 12 months, and clinical follow-ups after 3, 6, and 12 months. RESULTS: Of 268 SICADs, 20 (7.5%) presented with

Asunto(s)
Disección de la Arteria Carótida Interna/patología , Neovascularización Patológica/patología , Adulto , Anticoagulantes/uso terapéutico , Disección de la Arteria Carótida Interna/diagnóstico por imagen , Disección de la Arteria Carótida Interna/tratamiento farmacológico , Estenosis Carotídea/diagnóstico por imagen , Estenosis Carotídea/patología , Femenino , Fibrinolíticos/uso terapéutico , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Neovascularización Patológica/diagnóstico por imagen , Neovascularización Patológica/tratamiento farmacológico , Estudios Prospectivos , Ultrasonografía
10.
Stroke ; 39(1): 82-6, 2008 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-18006862

RESUMEN

BACKGROUND AND PURPOSE: Isolated Horner syndrome without associated cranial nerve palsies or ischemic symptoms is an important presentation of spontaneous internal carotid artery dissection (sICAD). Ultrasound is often used as a screening method in these patients because cervical MRI is not always available on an emergency basis. Current knowledge on ultrasound findings in patients with sICAD presenting with isolated Horner syndrome is limited. METHODS: Patients were recruited from prospective cervical artery dissection databases of 3 tertiary care centers. Diagnosis of sICAD was confirmed by cervical MRI and MR angiography or digital subtraction angiography in all patients. Data on Doppler sonography and color duplex sonography examinations performed within 30 days of symptom onset were analyzed. RESULTS: We identified 88 patients with Horner syndrome as the only sign of sICAD. Initial ultrasound examination was performed in 72 patients after a mean time interval from symptom onset to examination of 11 (SD 8) days. The overall frequency of false-negative ultrasound findings was 31% (22 of 72 patients). It showed stenosis >or=80% or occlusion in 34 (47%) patients, and stenosis

Asunto(s)
Disección de la Arteria Carótida Interna/complicaciones , Disección de la Arteria Carótida Interna/diagnóstico por imagen , Síndrome de Horner/etiología , Adulto , Encéfalo/irrigación sanguínea , Encéfalo/patología , Arterias Carótidas/diagnóstico por imagen , Arterias Carótidas/patología , Disección de la Arteria Carótida Interna/patología , Reacciones Falso Negativas , Femenino , Síndrome de Horner/diagnóstico , Síndrome de Horner/patología , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Ultrasonografía Doppler/métodos
11.
Ther Umsch ; 65(5): 259-63, 2008 May.
Artículo en Alemán | MEDLINE | ID: mdl-18622929

RESUMEN

Cerebral vasculitis is a rare disease with a potentially harmful or even fatal outcome that often affects young adults. Primary autoimmune mediated disease can be distinguished from secondary vasculitis associated to infectious disorders, connective tissue diseases, malignancies or toxic drug effects. Pathomechanisms lead to destruction of the vessel wall and consecutive hemorrhagic or ischemic brain lesions. Beyond these mechanisms direct autoimmune mediated neurotoxicity is postulated. Clinical presentation is highly variable with potentially fluctuating signs and symptoms. Besides multifocal deficits from disseminated CNS involvement, diffuse encephalopathy or psychosis may result from diffuse CNS affection. For systemic vasculitis with CNS involvement, affection of joints, skin and organs may facilitate the diagnostic evaluation. CNS affection in systemic diseases is highly variable and may even precede systemic manifestation. The diagnostic work-up includes clinical evaluation, analysis of autoantibodies, MRI, digital subtraction angiography and biopsy of the affected tissue in doubtful cases. Standard therapy are corticosteroids often combined with immunosuppressants such as azathioprine, methotrexate or mycophenolate mofetil in chronic disease or cyclophosphamid in acute disorder. When therapy can be initiated timely, prognosis of cerebral vasculitis is usually favourable.


Asunto(s)
Inmunosupresores/uso terapéutico , Insuficiencia Multiorgánica/diagnóstico , Insuficiencia Multiorgánica/terapia , Vasculitis del Sistema Nervioso Central/diagnóstico , Vasculitis del Sistema Nervioso Central/terapia , Humanos
12.
Stroke ; 37(10): 2499-503, 2006 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-16960096

RESUMEN

BACKGROUND AND PURPOSE: Few data exist about clinical, radiologic findings, clinical outcome, and its predictors in patients with spontaneous vertebral artery dissection (sVAD). METHODS: Clinical characteristics, imaging findings, 3-month outcomes, and its predictors were investigated in consecutive patients with sVAD. RESULTS: One hundred sixty-nine patients with 195 sVAD were identified. Brain ischemia occurred in 131 patients (77%; ischemic stroke, n=114, 67%; transient ischemic attack, n=17, 10%). Three patients with ischemic stroke showed also signs of subarachnoid hemorrhage (SAH); 3 (2%) had SAH without ischemia. The 134 patients with brain ischemia or SAH had head and/or neck pain in 118 (88%) and pulsatile tinnitus in seven (5%) patients. The remaining 35 patients (21%) had isolated head and/or neck pain in 21 (12%) cases, asymptomatic sVAD in 13 (8%), and cervical radiculopathy in one case (1%). Location of sVAD was more often in the pars transversaria (V2; 35%) or atlas loop (V3; 34%) than in the prevertebral (V1; 20%) or intracranial (V4; 11%) segment (P=0.0001). Outcome was favorable (modified Rankin scale score 0 or 1) in 88 (82%) of 107 ischemic stroke patients with follow up. Two (2%) patients died. Low baseline National Institutes of Health Stroke Scale score (P<0.0001) and younger age (P=0.007) were independent predictors of favorable outcome. CONCLUSIONS: sVAD is predominantly located in the pars transversaria (V2) or the atlas loop (V3). Most patients show posterior circulation ischemia. Favorable outcome is observed in most ischemic strokes and independently predicted by low National Institutes of Health Stroke Scale score and younger age.


Asunto(s)
Disección de la Arteria Vertebral/epidemiología , Isquemia Encefálica/epidemiología , Isquemia Encefálica/etiología , Comorbilidad , Bases de Datos Factuales , Diagnóstico por Imagen/métodos , Diagnóstico por Imagen/estadística & datos numéricos , Femenino , Fibrinolíticos/uso terapéutico , Estudios de Seguimiento , Humanos , Hipercolesterolemia/epidemiología , Hipertensión/epidemiología , Ataque Isquémico Transitorio/epidemiología , Ataque Isquémico Transitorio/etiología , Imagen por Resonancia Magnética , Masculino , Estudios Prospectivos , Estudios Retrospectivos , Factores de Riesgo , Rotura Espontánea , Índice de Severidad de la Enfermedad , Hemorragia Subaracnoidea/epidemiología , Hemorragia Subaracnoidea/etiología , Terapia Trombolítica , Acúfeno/epidemiología , Acúfeno/etiología , Resultado del Tratamiento , Disección de la Arteria Vertebral/diagnóstico , Disección de la Arteria Vertebral/tratamiento farmacológico
13.
Pathol Res Pract ; 202(3): 165-70, 2006.
Artículo en Inglés | MEDLINE | ID: mdl-16377100

RESUMEN

Rosai-Dorfman disease (RDD) is a non-neoplastic proliferative histiocytic disorder that primarily affects lymph nodes (sinus histiocytosis with massive lymphadenopathy). Primary RDD of the central nervous system is most uncommon. We report on a 35-year-old man with isolated RDD of the meninges overlying the left cerebral hemisphere. Presenting signs and symptoms included severe progressive ipsilateral headaches of 4 months duration, as well as laboratory evidence of mild non-specific systemic inflammatory reaction. On magnetic resonance imaging, the lesion was seen as a contrast-enhancing, plaque-like thickening of the dura mater over the left convexity,without impinging on adjacent bone or cerebral parenchyma. Meningeal biopsy revealed a mixed mononuclear infiltrate dominated by CD68(+), S100(+), CD1a(-) non-Langerhans type histiocytes on a background of fibrosis. Bacteria, in particular mycobacteria, and fungi were excluded with special stains. Extensive clinical workup, encompassing computed tomography of thoracal and abdominal organs, bone marrow biopsy, and bronchoalveolar lavage failed to reveal any extracranial involvement. Laboratory tests for autoimmunity, including C- and P-antineutrophil cytoplasmic antibodies, antinuclear antibody, and serum rheumatoid factor, were negative. Methylprednisolone therapy induced complete remission of symptoms, with the neuroradiologic status remaining unchanged on follow-up after 2 months. We discuss the complex clinicopathologic differential diagnosis and therapeutic issues of this rare condition. While the correct diagnosis of central nervous system RDD is unlikely to be established without invasive procedures (biopsy), a conservative therapeutic approach may be considered a legitimate option.


Asunto(s)
Encefalopatías/diagnóstico , Histiocitosis Sinusal/diagnóstico , Meninges , Adulto , Antígenos CD/metabolismo , Antígenos de Diferenciación Mielomonocítica/metabolismo , Biomarcadores de Tumor/análisis , Encefalopatías/patología , Diagnóstico Diferencial , Histiocitosis Sinusal/tratamiento farmacológico , Histiocitosis Sinusal/patología , Humanos , Inmunohistoquímica , Imagen por Resonancia Magnética , Masculino , Meninges/patología , Metilprednisolona/uso terapéutico , Seudotumor Cerebral/diagnóstico , Proteínas S100/metabolismo , Resultado del Tratamiento
14.
Arch Neurol ; 62(2): 309-13, 2005 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-15710861

RESUMEN

BACKGROUND: Metachromatic leukodystrophy (MLD) is a lysosomal storage disease caused by the deficiency of arylsulfatase A (ARSA). Clinically, the disease is heterogeneous with respect to the age of onset, affection of peripheral and central nervous systems, and progression. OBJECTIVES: To analyze mutations in the ARSA gene of a patient with adult-onset MLD with no signs of peripheral polyneuropathy and to emphasize the clinical, neuroradiologic, neuropathologic, and genetic features of the disease. DESIGN: Case study of a patient clinically presenting with rapidly progressive dementia and behavioral abnormalities. We report the findings of clinical evaluation and neurophysiologic and neuropathologic studies of peripheral nerves; we also performed DNA sequence analysis, transfections, metabolic labeling, and immunoprecipitation of mutant ARSA polypeptides. SETTING: Genetic research and clinical unit, university hospital. RESULTS: Genetic analysis revealed homozygosity for a novel mutation in exon 3 of ARSA (F219V). This substitution leads to a misfolded unstable enzyme with a specific activity less than 1% of normal. There were no clinical or neurophysiologic signs of peripheral nervous system dysfunction. Typical neuropathologic signs for MLD were absent from nerve biopsy specimens. CONCLUSIONS: This novel mutation is associated with progressive psychocognitive impairment without clinical or electrophysiologic signs and only minor morphologic signs of peripheral nerve affection. The F219V substitution causes reduction in enzyme activity to an extent unexpected for an adult patient with MLD.


Asunto(s)
Cerebrósido Sulfatasa/genética , Leucodistrofia Metacromática/genética , Mutación , Nervios Periféricos/fisiopatología , Adulto , Animales , Animales Recién Nacidos , Encéfalo/patología , Células Cultivadas , Cerebrósido Sulfatasa/metabolismo , Ácido Cítrico/farmacología , Cricetinae , Análisis Mutacional de ADN/métodos , Electroforesis en Gel de Campo Pulsado/métodos , Retículo Endoplásmico/efectos de los fármacos , Retículo Endoplásmico/metabolismo , Potenciales Evocados/fisiología , Exones/genética , Femenino , Expresión Génica/fisiología , Humanos , Riñón/citología , Leucodistrofia Metacromática/patología , Leucodistrofia Metacromática/fisiopatología , Imagen por Resonancia Magnética/métodos , Microscopía Electrónica de Transmisión/métodos , Mutagénesis/fisiología , Nervios Periféricos/patología , Fenilalanina/genética , Compuestos de Amonio Cuaternario/farmacología , Nervio Sural/patología , Nervio Sural/fisiopatología , Nervio Sural/ultraestructura , Transfección/métodos , Valina/genética
15.
Front Neurol ; 6: 211, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-26528235

RESUMEN

AIM: To describe structural covariance networks of gray matter volume (GMV) change in 28 patients with first-ever stroke to the primary sensorimotor cortices, and to investigate their relationship to hand function recovery and local GMV change. METHODS: Tensor-based morphometry maps derived from high-resolution structural images were subject to principal component analyses to identify the networks. We calculated correlations between network expression and local GMV change, sensorimotor hand function and lesion volume. To verify which of the structural covariance networks of GMV change have a significant relationship to hand function, we performed an additional multivariate regression approach. RESULTS: Expression of the second network, explaining 9.1% of variance, correlated with GMV increase in the medio-dorsal (md) thalamus and hand motor skill. Patients with positive expression coefficients were distinguished by significantly higher GMV increase of this structure during stroke recovery. Significant nodes of this network were located in md thalamus, dorsolateral prefrontal cortex, and higher order sensorimotor cortices. Parameter of hand function had a unique relationship to the network and depended on an interaction between network expression and lesion volume. Inversely, network expression is limited in patients with large lesion volumes. CONCLUSION: Chronic phase of sensorimotor cortical stroke has been characterized by a large scale co-varying structural network in the ipsilesional hemisphere associated specifically with sensorimotor hand skill. Its expression is related to GMV increase of md thalamus, one constituent of the network, and correlated with the cortico-striato-thalamic loop involved in control of motor execution and higher order sensorimotor cortices. A close relation between expression of this network with degree of recovery might indicate reduced compensatory resources in the impaired subgroup.

16.
Int J Stroke ; 10(6): 887-92, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-26121371

RESUMEN

BACKGROUND: We prospectively investigated temporal and spatial evolution of intramural hematomas in patients with acute spontaneous internal carotid artery dissection using repeated magnetic resonance imaging over six-months. AIM: The aim of the present study was to assess dynamic changes of intramural hematoma in patients with acute spontaneous internal carotid artery dissection at multiple follow-up time-points with T1w, PD/T2w, and magnetic resonance angiography. METHODS: We performed serial multiparametric magnetic resonance imaging in 10 patients with spontaneous internal carotid artery dissection on admission, at days 1, 3, 7-14 and at months 1·5, 3, and 6. We calculated the volume and extension of the hyperintense intramural hematoma using T1w and PD/T2w fat suppressed sequences and assessed the degree of stenosis due to the hematoma using magnetic resonance angiography. RESULTS: Mean interval from symptom onset to first magnetic resonance imaging was two-days (SD 2·7). Two patients presented with ischemic stroke, three with transient ischemic attacks, and five with pain and local symptoms only. Nine patients had a transient increase of the intramural hematoma volume, mainly up to day 10 after symptom onset. Fifty percent had a transient increase in the degree of the internal carotid artery stenosis on MRA, one resulting in a temporary occlusion. Lesions older than one-week were predominantly characterized by a shift from iso- to hyperintese signal on T2w images. At three-month follow-up, intramural hematoma was no longer detectable in 80% of patients and had completely resolved in all patients after six-months. CONCLUSIONS: Spatial and temporal dynamics of intramural hematomas after spontaneous internal carotid artery dissection showed an early volume increase with concomitant progression of the internal carotid artery stenosis in 5 of 10 patients. Although spontaneous internal carotid artery dissection overall carries a good prognosis with spontaneous hematoma resorption in all our patients, early follow-up imaging may be considered, especially in case of new clinical symptoms.


Asunto(s)
Disección de la Arteria Carótida Interna/patología , Hematoma/patología , Isquemia Encefálica/patología , Isquemia Encefálica/fisiopatología , Disección de la Arteria Carótida Interna/fisiopatología , Estenosis Carotídea/patología , Estenosis Carotídea/fisiopatología , Angiografía Cerebral , Bases de Datos Factuales , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Hematoma/fisiopatología , Humanos , Angiografía por Resonancia Magnética , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Accidente Cerebrovascular/patología , Accidente Cerebrovascular/fisiopatología
17.
Stroke ; 35(2): 560-5, 2004 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-14726546

RESUMEN

BACKGROUND AND PURPOSE: Current knowledge of long-term outcome in patients with acute spinal cord ischemia syndrome (ASCIS) is based on few studies with small sample sizes and <2 years' follow-up. Therefore, we analyzed clinical features and outcome of all types of ASCIS to define predictors of recovery. METHODS: From January 1990 through October 2002, 57 patients with ASCIS were admitted to our center. Follow-up data were available for 54. Neurological syndrome and initial degree of impairment were defined according to American Spinal Injury Association (ASIA)/International Medical Society of Paraplegia criteria. Functional outcome was assessed by walking ability and bladder control. RESULTS: Mean age was 59.4 years; 29 were women; and mean follow-up was 4.5 years. The origin was atherosclerosis in 33.3%, aortic pathology in 15.8%, degenerative spine disease in 15.8%, cardiac embolism in 3.5%, systemic hypotension in 1.8%, epidural anesthesia in 1.8%, and cryptogenic in 28%. The initial motor deficit was severe in 30% (ASIA grades A and B), moderate in 28% (ASIA C), and mild in 42% (ASIA D). At follow-up, 41% had regained full walking ability, 30% were able to walk with aids, 20% were wheelchair bound, and 9% had died. Severe initial impairment (ASIA A and B) and female sex were independent predictors of unfavorable outcome (P=0.012 and P=0.043). CONCLUSIONS: Considering a broad spectrum of clinical presentations and origins, the outcome in our study was more favorable than in previous studies reporting on ASCIS subgroups with more severe initial deficits.


Asunto(s)
Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Isquemia de la Médula Espinal/diagnóstico , Isquemia de la Médula Espinal/fisiopatología , Enfermedad Aguda , Evaluación de la Discapacidad , Femenino , Estudios de Seguimiento , Trastornos Neurológicos de la Marcha/etiología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Examen Neurológico , Pronóstico , Recuperación de la Función , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores Sexuales , Isquemia de la Médula Espinal/complicaciones , Suiza , Síndrome , Tiempo , Vejiga Urinaria Neurogénica/etiología
18.
Stroke ; 33(7): 1828-33, 2002 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-12105361

RESUMEN

BACKGROUND AND PURPOSE: The purpose of this study was to evaluate the safety and efficacy of local intra-arterial thrombolysis (LIT) using urokinase in patients with acute stroke due to middle cerebral artery (MCA) occlusion. METHODS: We analyzed clinical and radiological findings and functional outcome 3 months after LIT with urokinase of 100 consecutive patients. To measure outcome, the modified Rankin scale (mRs) score was used. RESULTS: Angiography showed occlusion of the M1 segment of the MCA in 57 patients, of the M2 segment in 21, and of the M3 or M4 segment in 22. The median National Institutes of Health Stroke Scale (NIHSS) score at admission was 14, and, on average, 236 minutes elapsed from symptom onset to LIT. Forty-seven patients (47%) had an excellent outcome (mRs score 0 to 1), 21 (21%) a good outcome (mRs score 2), and 22 (22%) a poor outcome (mRs score 3 to 5). Ten patients (10%) died. Excellent or good outcome (mRs score < or =2) was seen in 59% of patients with M1 or M2 and 95% of those with M(3) or M(4) MCA occlusions. Recanalization as seen on angiography was complete (thrombolysis in myocardial infarction [TIMI] grade 3) in 20% of patients and partial (TIMI grade 2) in 56% of patients. Age <60 years (P<0.05), low NIHSS score at admission (P<0.00001), and vessel recanalization (P=0.0004) were independently associated with excellent or good outcome and diabetes with poor outcome (P=0.002). Symptomatic cerebral hemorrhage occurred in 7 patients (7%). CONCLUSIONS: LIT with urokinase that is administered by a single organized stroke team is safe and can be as efficacious as thrombolysis has been in large multicenter clinical trials.


Asunto(s)
Fibrinolíticos/administración & dosificación , Infarto de la Arteria Cerebral Media/tratamiento farmacológico , Accidente Cerebrovascular/tratamiento farmacológico , Activador de Plasminógeno de Tipo Uroquinasa/administración & dosificación , Adulto , Anciano , Anciano de 80 o más Años , Hemorragia Cerebral/etiología , Demografía , Femenino , Fibrinolíticos/efectos adversos , Hemorragia/etiología , Humanos , Infarto de la Arteria Cerebral Media/complicaciones , Infusiones Intraarteriales , Modelos Logísticos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Accidente Cerebrovascular/etiología , Terapia Trombolítica/efectos adversos , Resultado del Tratamiento , Activador de Plasminógeno de Tipo Uroquinasa/efectos adversos
19.
Arch Neurol ; 59(4): 549-53, 2002 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-11939889

RESUMEN

BACKGROUND: Results of recently published studies suggest that intravenous thrombolysis (IVT) and local intra-arterial thrombolysis (LIT) are feasible procedures in acute stroke after cervical artery dissection (CAD). OBJECTIVES: To describe 9 patients with acute stroke caused by CAD who were treated by LIT (n = 7) or IVT (n = 2) and to review the literature. METHODS: Retrospective analysis of clinical and neuroradiological findings; literature review from 1980 to present. MAIN OUTCOME MEASURE: Modified Rankin Scale (mRS) score. RESULTS: Of 7 patients treated with LIT, 3 had good outcomes (mRS score of 0-2) and 4 had bad outcomes (mRS score of 3-6) at 3 months. The 2 patients who had received IVT recovered to mRS scores of 0 and 3. Twenty-one patients were identified in the literature. Overall (N = 30), in the IVT group (n = 19), the outcome was good in 8 patients (42%) and bad in 11 (58%); in the LIT group (n = 11), 6 patients (55%) had a good outcome and 5 (45%) had a bad outcome. Overall, 47% (14/30) of the patients (IVT and LIT groups) had a good outcome. Total mortality was 13% (4/30). There were no secondary complications due to extension of wall hematoma or angiography. One symptomatic hemorrhage occurred. CONCLUSIONS: Thrombolysis is feasible in acute stroke caused by CAD. Local complications from extension of wall hematoma did not occur. Further prospective studies are needed to determine the safety and efficacy of thrombolysis in the special circumstance of acute stroke caused by CAD.


Asunto(s)
Vértebras Cervicales/irrigación sanguínea , Activadores Plasminogénicos/administración & dosificación , Accidente Cerebrovascular/tratamiento farmacológico , Accidente Cerebrovascular/etiología , Terapia Trombolítica , Disección de la Arteria Vertebral/complicaciones , Enfermedad Aguda , Adulto , Anciano , Angiografía Cerebral , Estudios de Factibilidad , Femenino , Humanos , Infarto de la Arteria Cerebral Media/tratamiento farmacológico , Infarto de la Arteria Cerebral Media/etiología , Inyecciones Intraarteriales , Inyecciones Intravenosas , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Accidente Cerebrovascular/diagnóstico por imagen , Terapia Trombolítica/métodos , Activador de Tejido Plasminógeno/administración & dosificación , Resultado del Tratamiento , Activador de Plasminógeno de Tipo Uroquinasa/administración & dosificación
20.
Pain ; 64(3): 429-434, 1996 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-8783306

RESUMEN

This study evaluated the course of psychological variables during a 2-year follow-up in patients after common whiplash of the cervical spine. From a sample of 117 non-selected patients with common whiplash (investigated on average 7.2 +/- 4.2 days after trauma) a total of 21 suffered trauma-related symptoms over 2 years following initial injury. These patients (symptomatic group) were compared with 21 age, gender and education pair-matched patients, who showed complete recovery from trauma-related symptoms during the 2-year follow-up (asymptomatic group). Both groups underwent standardised testing procedures (i.e., Freiburg Personality Inventory and Well-Being Scale) at referral, and at 3, 6 and 24 months. In the symptomatic group during follow-up no significant changes in rating of neck pain or headache were found. Significant differences between the groups and significant deviation of scores over time were found on the Well-Being and Nervousness Scales. There was a lack of significant difference between the groups on the Depression Scale, indicating a possible somatic basis for changes in psychological functioning in the investigated sample. With regard to scales of Extraversion or Neuroticism, there were neither significant differences between the groups nor significant deviation over time. These results highlight that patients' psychological problems are rather a consequence than a cause of somatic symptoms in whiplash.


Asunto(s)
Lesiones por Latigazo Cervical/psicología , Adulto , Factores de Edad , Educación , Femenino , Estudios de Seguimiento , Cefalea/etiología , Cefalea/psicología , Humanos , Masculino , Persona de Mediana Edad , Dolor de Cuello/etiología , Dolor de Cuello/psicología , Pruebas de Personalidad , Factores Sexuales , Conducta Social , Lesiones por Latigazo Cervical/complicaciones
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