RESUMEN
AIM: Migraine is a chronic neurovascular disease that affects the trigeminovascular system. The purpose of this study was to evaluate corneal subbasal nerve fibers, dendritic cells and to measure tear film parameters in migraine. PATIENTS AND METHODS: 87 eyes of 44 patients suffering from migraine with a mean age of 33.23 ± 11.41 years were included in our study. 25 age-matched controls (mean age of 30.16 ± 12.59 years; P = 0.162) were recruited. The corneal subbasal plexus and the dendritic cells (DC) were analyzed using in vivo confocal microscopy (Heidelberg Retina Tomograph II Rostock Cornea Module; Heidelberg Engineering GmbH), and the tear film was imaged using LacryDiag (Quantel Medical, France). RESULTS: Regarding the subbasal nerve fibers of the cornea, none of the examined parameters differed significantly in migraine patients from controls. We found a significant increase in the corneal DC density (P < 0.0001) and DC area (P < 0.0001) in migraine patients compared to healthy volunteers. DC density showed a positive correlation with the monthly attack frequency (r = 0.32, P = 0.041) and the DC area a negative correlation with corneal nerve branch density (r = -0.233, P = 0.039), nerve fiber length (r = -0.232, P = 0.04) and total branch density (r = -0.233, P = 0.039). Using LacryDiag a significant loss of Meibomian gland area could be detected on the superior eyelid (P = 0.005) in migraine. CONCLUSIONS: Our results suggest the presence of neuroinflammation in the cornea of migraine patients affecting the peripheral trigeminal system. Dendritic cells surrounding the subbasal plexus may be involved in the activation and modulation of pain in migraine.
RESUMEN
Background and purpose: Multifocal motor neuropathy (MMN) is a rare, immune-mediated illness attacking ex-clusively motor nerves. It is known that oxidative stress is present in peripheral neuropathies, but it has not been investigated MMN. Methods: We measured in our prospective study the L-arginine, symmetric and asymmetric dimethylarginine (SDMA, ADMA) serum concentrations of 10 patients and 10 controls before and after intravenous immunoglobulin treatment (IVIG), as markers of the L-arginine/NO pathway involved in chronic inflammation and oxidative stress. The functions of motor nerves were tested in all patients and the serum antiganglioside antibody levels were de-tec-ted, as well. Results: MMN patients showed significantly higher ADMA (p = 0.0048; 0.98 and 0.63, respectively) and SDMA le-vels (p = 0.001; 0.88 and 0.51, respectively) than healthy controls, while L-arginine was not different. Controlling for the covariant age, ADMA (B = -0.474; p = 0.041) or SDMA (B = -0.896; p < 0.0005) serum levels proved to be the significant predictors of the presence of MMN. IVIG therapy decreased significantly ADMA concentrations (p = 0.025; 0.98 and 0.84, respectively) and showed a trend to reduce SDMA levels (p = 0.1; 0.88 and 0.74, respectively). The dimethylamine levels did not correlate with the number of affected nerves, disease duration, or the presence of ganglioside antibodies. The conduction block-related peripheral motor dysfunction improved right after the IVIG treatment. Conclusion: Dimethylamine levels are elevated in the serum and are responsive to IVIG therapy in MMN. These findings support the presence of oxidative stress in MMN.
Asunto(s)
Enfermedades del Sistema Nervioso Periférico , Polineuropatías , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Estudios Prospectivos , Biomarcadores , Estrés Oxidativo , Polineuropatías/tratamiento farmacológicoRESUMEN
Diseases with peripheral motor symptoms are a rare, but important subgroup of the all peripheral neuropathies, radiculopathies and neuronopathies. In these mostly progressive neuropathies, the clinical features include pure motor symptoms with weakness and wasting of the striated muscles. The differentiation of these diseases is frequently a challenge for qualified clinical neurologists. A careful history taking, the disease time course, the findings of routine clinical physical examination and the electrophysiological studies are all necessary in the diagnostic procedure. The aim of this publication is to overview the clinical characteristics of the pure motor peripheral neuropathies, to consider the diagnostic steps and the differential diagnosis, and finally to summarize the treatment options.
Asunto(s)
Enfermedad de la Neurona Motora/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Diagnóstico Diferencial , Progresión de la Enfermedad , Humanos , Enfermedad de la Neurona Motora/fisiopatología , Enfermedades del Sistema Nervioso Periférico/fisiopatologíaRESUMEN
BACKGROUND/AIM: Migraine-related intracerebral white matter lesions (WMLs) are likely to be microvascular in nature and can be found in all hemispheric lobes. The aim of this study was to investigate migraine patients with or without WMLs to see the effects of these tissue damages on cortical thickness and volume. The role of migraine characteristics (duration of headache, attack frequency, estimated lifetime attack number, aura) was also tested. METHODS: As study participants, 161 female migraine patients (63 with aura; 52 with WMLs) and 40 age-matched healthy female subjects were enrolled in the study. None of the included migraine patients' headache or aura (where present) was unilaterally side-locked. Patients and controls were all right handed. Except for migraine, patients were free of any medical comorbidity. Cortical reconstruction and segmentation were performed on the 3D T1-weighted images using Freesurfer 5.3 image analysis suite. The automatic cortical parcellation was based on Freesurfer's Desikan-Killiany-Tourville atlas, which has 31 cortical regions per hemisphere. The segmented regions were divided into five lobes (frontal, parietal, temporal, occipital, insula). Since the left and right differences in lobar and insular volumes/thicknesses were not different among our groups, volume and cortical thickness were calculated for corresponding bilateral lobes. RESULTS: There was no significant difference in age between the whole migraine and the control groups. Migraineurs with WMLs (L+ patients) were significantly older than lesion-free (L-) patients (P = 0.0003) and controls (P = 0.018). Disease duration (P = 0.003), the total number of migraine attacks (P = 0.022) and the rate of aura (P = 0.0003) were significantly higher in L+ patients than in L- patients. Cortical thickness and volume measurements of lobes were not statistically different between the three groups (L+, L-, control). Age showed a significant negative association with both thickness and volume in each examined lobe (P < 0.001). Intracranial volume (ICV) showed a significant positive association with all regional volumes (P < 0.001). There were no significant group*age, group*ICV, or age*ICV interactions. None of the migraine characteristics were selected by stepwise linear regression as significant predictors of cortical thickness or volume. Only age (for both thickness and volume) and ICV (for volume) were identified as significant predictors (P < 0.001). When the L + group was divided into two subgroups by median split of total and lobar lesion number and volume, the cortical measures did not show any significant difference between the groups with low vs. high lesion number/volume by stepwise linear regression. CONCLUSIONS: In a female migraine group, we found that the WMLs and clinical migraine characteristics have no effect on cortical thickness and volume of bilateral lobes. Lobar cortical thicknesses were equivalent within the range of ±0.1 mm. Only age and ICV proved to be significant predictors; the former for both cortical thickness and volume, while the latter for cortical volume.
Asunto(s)
Corteza Cerebral/patología , Trastornos Migrañosos/patología , Sustancia Blanca/patología , Adolescente , Adulto , Anciano , Corteza Cerebral/diagnóstico por imagen , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Persona de Mediana Edad , Trastornos Migrañosos/diagnóstico por imagen , Tamaño de los Órganos , Sustancia Blanca/diagnóstico por imagen , Adulto JovenRESUMEN
Background/Aim Migraine is a risk factor for the formation of silent brain white matter lesions (WMLs) that are possibly ischemic in nature. Although dysfunction of the L-arginine/nitric oxide (NO) pathway has been associated with oxidative stress and endothelial dysfunction in migraine, its role in WML development has not been specifically investigated. Thus, this prospective study aimed to measure the serum concentrations of the NO substrate L-arginine, the NO synthase inhibitor asymmetric dimethylarginine (ADMA), and the L-arginine transport regulator symmetric dimethylarginine (SDMA) in migraine patients in a headache-free period. Methods All participants underwent MR imaging to assess for the presence of WMLs on fluid-attenuated inversion recovery imaging. Altogether 109 migraine patients (43 with lesions, 66 without lesions) and 46 control individuals were studied. High-performance liquid chromatography was used to quantify L-arginine, ADMA and SDMA serum concentrations. Migraine characteristics were investigated, and participants were screened for risk factors that can lead to elevated serum ADMA levels independent of migraine. Results Migraine patients and controls did not differ in regard to vascular risk factors. Migraineurs with WMLs had a longer disease duration ( p < 0.001) and a higher number of lifetime headache attacks ( p = 0.005) than lesion-free patients. Higher L-arginine serum levels were found in both migraine subgroups compared to controls ( p < 0.001). Migraine patients with WMLs showed higher ADMA concentrations than lesion-free patients and controls ( p < 0.001, for both). In migraineurs, the presence of WMLs, aura and increasing age proved to be significant predictors of increased ADMA levels ( p = 0.008, 0.047 and 0.012, respectively). SDMA serum levels of lesional migraineurs were higher than in nonlesional patients ( p < 0.001). The presence of lesions and increasing age indicated an increased SDMA level ( p = 0.017 and 0.001, respectively). Binary logistic regression analysis showed that ADMA level ( p = 0.006), increasing age ( p = 0.017) and the total number of lifetime migraine attacks ( p = 0.026) were associated with an increased likelihood of exhibiting WMLs. There was no significant effect of age on ADMA and SDMA concentrations in controls. Conclusions Elevated ADMA levels may impact the pathogenesis of migraine-related WMLs by influencing cerebrovascular autoregulation and vasomotor reactivity. Higher SDMA concentrations may indirectly influence NO synthesis by reducing substrate availability. Elevated L-arginine serum levels might reflect an increased demand for NO synthesis.
Asunto(s)
Arginina/análogos & derivados , Arginina/sangre , Trastornos Migrañosos/sangre , Trastornos Migrañosos/diagnóstico por imagen , Sustancia Blanca/diagnóstico por imagen , Adulto , Anciano , Biomarcadores/sangre , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto JovenRESUMEN
PURPOSE: To compare the white matter lesions seen in multiple sclerosis and migraine using monoexponential and high b-value biexponential diffusion measurements. MATERIALS AND METHODS: Diffusion-weighted images were acquired on a 3.0-Tesla magnetic resonance imaging system. Diffusion parameters were estimated using monoexponential (0-1000 s/mm(2) ) and biexponential (0-5000 s/mm(2) ) approaches from 15 multiple sclerosis patients, 15 patients with migraine and 15 healthy control subjects. The study was performed in accordance with the approval of the Regional Research Ethics Committee. The apparent diffusion coefficient (ADC) values were measured in the lesions and the normal-appearing white matter of patients and in the white matter of controls. RESULTS: High lesional ADCmono values were detected in both patient groups without significant differences between the groups (10.72 and 9.86 × 10(-4) mm(2) /s for MS and migraine respectively, P = 0.2134). The biexponential measurements showed significantly higher ADCfast , ADCslow , and Pslow values in the migraine lesions than in the multiple sclerosis lesions (16.47 versus 14.29, 1.41 versus 0.76, and 20.34 versus 12.01 all values in 10(-4) mm(2) /s; P = 0.0344, P = 0.0019, P = 0.0021, respectively). CONCLUSION: Biexponential diffusion analysis may help to differentiate multiple sclerosis-related white matter lesions from migraine-related ones.
Asunto(s)
Imagen de Difusión por Resonancia Magnética , Trastornos Migrañosos/patología , Esclerosis Múltiple/patología , Sustancia Blanca/patología , Adulto , Femenino , Humanos , Imagenología Tridimensional , Masculino , Estudios Prospectivos , Reproducibilidad de los ResultadosRESUMEN
OBJECTIVE/BACKGROUND: The aim of this longitudinal study was to investigate changes of migraine-related brain white matter hyperintensities 3 years after an initial study. Baseline quantitative magnetic resonance imaging (MRI) studies of migraine patients with hemispheric white matter hyperintensities performed in 2009 demonstrated signs of tissue damage within the hyperintensities. The hyperintensities appeared most frequently in the deep white matter of the frontal lobe with a similar average hyperintensity size in all hemispheric lobes. Since in this patient group the repeated migraine attacks were the only known risk factors for the development of white matter hyperintensities, the remeasurements of migraineurs after a 3-year long follow-up may show changes in the status of these structural abnormalities as the effects of the repeated headaches. METHODS: The same patient group was reinvestigated in 2012 using the same MRI scanner and acquisition protocol. MR measurements were performed on a 3.0-Tesla clinical MRI scanner. Beyond the routine T1-, T2-weighted, and fluid-attenuated inversion recovery imaging, diffusion and perfusion-weighted imaging, proton magnetic resonance spectroscopy, and T1 and T2 relaxation time measurements were also performed. Findings of the baseline and follow-up studies were compared with each other. RESULTS: The follow-up proton magnetic resonance spectroscopy studies of white matter hyperintensities showed significantly decreased N-acetyl-aspartate (median values 8.133 vs 7.153 mmol/L, P=.009) and creatine/phosphocreatine (median values 4.970 vs 4.641 mmol/L, P=.015) concentrations compared to the baseline, indicating a more severe axonal loss and glial hypocellularity with decreased intracellular energy production. The diffusion values, the T1 and T2 relaxation times, and the cerebral blood flow and volume measurements presented only mild changes between the studies. The number (median values 21 vs 25, P<.001) and volume (median values 0.896 vs 1.140 mL, P<.001) of hyperintensities were significantly higher in the follow-up study. No changes were found in the hemispheric and lobar distribution of hyperintensities. An increase in the hyperintensity size of preexisting lesions was much more common than a decrease (median values 14 vs 5, P=.004). A higher number of newly developed hyperintensities were detected than disappeared ones (130 vs 22), and most of them were small (<.034 mL). Small white matter hyperintensities in patients with a low migraine attack frequency had a higher chance to disappear than large white matter hyperintensities or white matter hyperintensities in patients with a high attack frequency (coefficient: -0.517, P=.034). CONCLUSIONS: This longitudinal MRI study found clinically silent brain white matter hyperintensities to be predominantly progressive in nature. The absence of a control group precludes definitive conclusions about the nature of these changes or if their degree is beyond normal aging.
Asunto(s)
Leucoencefalopatías/etiología , Leucoencefalopatías/patología , Imagen por Resonancia Magnética , Trastornos Migrañosos/complicaciones , Adulto , Anciano , Ácido Aspártico/análogos & derivados , Ácido Aspártico/metabolismo , Creatina/metabolismo , Imagen de Difusión por Resonancia Magnética , Femenino , Lóbulo Frontal/metabolismo , Lóbulo Frontal/patología , Lateralidad Funcional , Humanos , Inositol/metabolismo , Estudios Longitudinales , Espectroscopía de Resonancia Magnética , Masculino , Persona de Mediana Edad , Protones , Adulto JovenRESUMEN
Background and aim: White matter hyperintensities (WMHs), presented on T2-weighted or fluid-attenuated inversion recovery magnetic resonance imaging (MRI) sequences, are lesions in the human brain that can be observed in both migraine and multiple sclerosis (MS). Methods: Seventeen migraine patients and 15 patients with relapsing-remitting multiple sclerosis with WMHs, and 17 healthy subjects age-and sex-matched to the migraine group were prospectively enrolled and underwent conventional and advanced MRI studies with diffusion-and perfusion-weighted imaging and single voxel proton magnetic resonance spectroscopy. Results: In both disease groups, elevated T2 relaxation time, apparent diffusion coefficient (ADC) values, and decreased N-acetyl-aspartate levels were found in the intralesional white matter compared to the contralateral normal-appearing white matter (NAWM), while there was no difference between the hemispheres of the control subjects. Migraine patients had the lowest intralesional creatine + phosphocreatine and myo-inositol (mI) values among the three groups, while patients with MS showed the highest intralesional T1 and T2 relaxation times, ADC, and mI values. In the contralateral NAWM, the same trend with mI changes was observed in migraineurs and MS patients. No differences in perfusion variables were observed in any groups. Conclusion: Our multimodal study showed that tissue damage is detectable in both diseases. Despite the differences in various advanced MRI measures, with more severe injury detected in MS lesions, we could not clearly differentiate the two white matter lesion types.
RESUMEN
BACKGROUND AND AIM: Brainstem descending modulatory circuits have been postulated to be involved in migraine. Differences in brainstem volume between migraineurs and healthy controls have been demonstrated in previous research, nevertheless, the effect of migraine aura on brainstem volume is still uncertain. The aim of this study was to investigate the brainstem volume in migraineurs and examine the effect of migraine aura on brainstem volume. METHODS: Our study included 90 female migraine patients without white matter lesions. (29 migraine patients with aura (MwA) and 61 migraine patients without aura (MwoA) and 32 age-matched female healthy controls (HC). Using the FreeSurfer image analysis suite, the volumes of the entire brainstem and its subfields (medulla, pons, and midbrain) were measured and compared between migraine subgroups (MwA vs. MwoA) and the healthy control group. The possible effects of migraine characteristics (i.e., disease duration and migraine attack frequency) on brainstem volume were also investigated. RESULTS: Migraineurs had greater medulla volume (MwoA 3552 ± 459 mm3, MwA 3424 ± 448 mm3) than healthy controls (3236 ± 411 mm3). Statistically, MwA vs. HC p = 0.040, MwoA vs. HC p = 0.002, MwA vs. MwoA p = 0.555. A significant positive correlation was found between disease duration and the volume of medulla in the whole migraine group (r = 0.334, p = 0.001). Neither the whole brainstem nor its subfields were significantly different in volume between migraine subgroups. CONCLUSION: Brainstem volume changes in migraine are mainly localized to the medulla and not specific to the presence of aura.
Asunto(s)
Epilepsia , Migraña con Aura , Migraña sin Aura , Humanos , Femenino , Tronco Encefálico/diagnóstico por imagen , Tronco Encefálico/patología , Migraña con Aura/diagnóstico por imagen , Migraña sin Aura/diagnóstico por imagen , Mesencéfalo/patología , Imagen por Resonancia Magnética/métodosRESUMEN
OBJECTIVE: The aim of this study was to examine chronic brain white matter hyperintensities in migraine and to gain data on the characteristics of the lesions. BACKGROUND: Migraine associates with a higher incidence of magnetic resonance imaging (MRI)-visible white matter signal abnormalities. Several attack-related pathomechanisms have been proposed in the lesion development, including the effect of repeated intracerebral hemodynamic changes. METHODS: Supratentorial white matter hyperintensities of 17 migraine patients were investigated interictally with quantitative MRI, including quantitative single voxel spectroscopy, diffusion, and perfusion MRI at 3.0-Tesla. The findings were compared with data measured in the contralateral, normal-appearing white matter of migraineurs and in the white matter of 17 healthy subjects. RESULTS: Significantly higher apparent diffusion coefficient values, prolonged T2 relaxation times, and decreased N-acetyl-aspartate and creatine/phosphocreatine concentrations were found in the white matter hyperintensities. The cerebral blood flow and blood volume values were mildly decreased inside the hyperintensities. Differences were not present between the migraine patients' normal-appearing white matter and the white matter of healthy subjects. CONCLUSIONS: The MRI measurements denote tissue damage with axonal loss, low glial cell density, and an enlarged extracellular space with an increased extracellular water fraction. These radiological features might be the consequences of microvascular ischemic changes during migraine attacks.
Asunto(s)
Encéfalo/fisiopatología , Trastornos Migrañosos/fisiopatología , Fibras Nerviosas Mielínicas/patología , Adulto , Anciano , Enfermedad Crónica , Femenino , Humanos , Interpretación de Imagen Asistida por Computador , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Fibras Nerviosas Mielínicas/metabolismo , Adulto JovenRESUMEN
Background/aim: Migraine is a disabling headache with clinical and radiological complications. The aim of this study was to investigate the volume of the thalamus and hippocampus in migraineurs, the role of white matter lesions (WMLs), and the migraine characteristics in volume changes. Methods: Brain MRIs of 161 right-handed female episodic migraine patients and 40 right-handed, age-related, healthy women were performed. Left and right thalamus segmentation was performed on the 3D MPRAGE images using the Freesurfer 5.3 image analysis suite. Hippocampal subfield segmentation was based on a novel statistical atlas built primarily upon ultra-high-resolution ex vivo MRI data. Results: The left hippocampus had a smaller and the left thalamus had a larger total volume than the right one in both the control (p < 0.001) and migraine groups (p <0.001). Patients with white matter lesions (L+) showed smaller right thalamus and right hippocampal tail volumes than patients without lesions (L-) (p = 0.002 and p = 0.015, respectively) and controls (p = 0.039 and p = 0.025, respectively). For the right hippocampal body, we found significantly smaller volume in L+ patients when compared to L- patients (p = 0.018) and a similar trend when compared to the control group (p = 0.064). Patients without aura (A-) showed a larger right hippocampus (p = 0.029), right hippocampal body (p = 0.012), and tail volumes (p = 0.011) than patients with aura (A+). Inverse correlations were found between attack frequency and the volumes of the left and right hippocampal tails (p = 0.018 and p = 0.008, respectively). Conclusion: These findings indicate that WMLs may influence the volume of the right thalamus and hippocampus, while migraine aura and attack frequency may lead to volume changes in different parts of the hippocampi in migraine patients. These data support the necessity of effective migraine management to limit subcortical volume loss in migraineurs.
RESUMEN
BACKGROUND: Bilateral pallidal deep brain stimulation (DBS) is an established treatment option for primary generalized and segmental dystonia. In the present study we evaluated the results of our dystonia patients treated by DBS. METHODS: The surgical results of forty consecutive dystonia patients underwent DBS implantation were analyzed (age: 43.7 +/- 17.7 years; sex: 22 men; etiology: 24 primary and 16 secondary dystonia; topography: 24 generalized, 12 segmental and four hemidystonia; disease duration: 16.1 +/- 9.3 years). Severity of dystonia measured by Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and health-related quality of life measured by EQ-5D scale were obtained preoperatively and compared to the scores obtained at postoperative six months and subsequent yearly follow-ups. The average follow-up lasted 2.5 years (median, 0.5-8 years). In all cases the BFMDRS scores were re-evaluated by a rater blinded to the treatment. Treatment responsiveness was defined as an at least 25% improvement on the BFMDRS scores. Non-parametric Mann-Whitney, McNemar and Kruskal-Wallis tests were applied to test statistical significance. RESULTS: Severity of dystonia improved from 31 to 10 points (median, 68% improvement, p < 0.01) in the primary dystonia group, whereas in secondary dystonia these changes were statistically insignificant (improvement from 40 to 31.5 points, 21.2%, p > 0.05). However, the health-related quality of life significantly improved in both groups (primary dystonia: 0.378 vs. 0.788 and secondary dystonia: 0.110 vs. 0.388, p < 0.01). Significantly more patients in the primary dystonia group responded to DBS treatment than those in the secondary dystonia group (83.3% vs. 37.5%, p < 0.01). CONCLUSION: Our results are in accordance with previously published international findings demonstrating that DBS is a highly effective and long-lasting treatment option for primary dystonia. DBS is considerably less efficient in secondary dystonia; however, it still has a high impact on the quality of life presumably due to its pain-relieving effect.
Asunto(s)
Estimulación Encefálica Profunda , Distonía/terapia , Trastornos Distónicos/terapia , Calidad de Vida , Adulto , Anciano , Estimulación Encefálica Profunda/efectos adversos , Estimulación Encefálica Profunda/métodos , Distonía/etiología , Distonía/patología , Distonía/fisiopatología , Trastornos Distónicos/etiología , Trastornos Distónicos/patología , Trastornos Distónicos/fisiopatología , Electrodos Implantados , Femenino , Globo Pálido/cirugía , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Temblor/terapiaRESUMEN
Brain white matter hyperintensities are more prevalent in migraine patients than in the general population, but the pathogenesis and the risk factors of these hyperintensities are not fully elucidated. The authors analyzed the routine clinical data of 186 migraine patients who were referred to the Outpatient Headache Department of the Department of Neurology, Medical School, University of Pécs, Hungary between 2007 and 2009: 58 patients with white matter hyperintensities and 128 patients without white matter hyperintensities on 3 T MRI. Significant associations between the presence of white matter hyperintensities and longer disease duration (14.4 vs. 19.9 years, p = 0.004), higher headache frequency (4.1 vs. 5.5 attacks/month, p = 0.017), hyperhomocysteinemia (incidence of hyperintensity is 9/9 = 100%, p = 0.009) and thyroid gland dysfunction (incidence of hyperintensity is 8/14 = 57.1%, p = 0.038) were found. These data support the theory that both the disease duration and the attack frequency have a key role in the formation of migraine-related brain white matter hyperintensities, but the effects of comorbid diseases may also contribute to the development of the hyperintensities.
Asunto(s)
Cerebro/patología , Leucoencefalopatías/epidemiología , Leucoencefalopatías/patología , Trastornos Migrañosos/epidemiología , Trastornos Migrañosos/patología , Fibras Nerviosas Mielínicas/patología , Adolescente , Adulto , Comorbilidad/tendencias , Femenino , Humanos , Leucoencefalopatías/diagnóstico , Masculino , Persona de Mediana Edad , Trastornos Migrañosos/diagnóstico , Factores de Riesgo , Adulto JovenRESUMEN
Small fiber neuropathy develops due to the selective damage of the thin fibers of peripheral nerves. Many common diseases can cause this condition, including diabetes, infections, autoimmune and endocrine disorders, but it can occur due to genetic alterations, as well. Eighty-five skin biopsy-proven small-fiber neuropathy cases were analyzed. Forty-one (48%) cases were idiopathic; among secondary types, hypothyreosis (9.4%), diabetes mellitus (7%), cryoglobulinemia (7%), monoclonal gammopathy with unproved significance (4.7%), Sjögren's disease (3%), and paraneoplastic neuropathy (3%) were the most common causes. Two-thirds (68%) of the patients were female, and the secondary type started 8 years later than the idiopathic one. In a vast majority of the cases (85%), the distribution followed a length-dependent pattern. Intraepidermal fiber density was comparable in idiopathic and secondary forms. Of note, we found significantly more severe pathology in men and in diabetes. Weak correlation was found between patient-reported measures and pathology, as well as with neuropathic pain-related scores. Our study confirmed the significance of small fiber damage-caused neuropathic symptoms in many clinical conditions, the gender differences in clinical settings, and pathological alterations, as well as the presence of severe small fiber pathology in diabetes mellitus, one of the most common causes of peripheral neuropathy.
Asunto(s)
Neuralgia/etiología , Neuropatía de Fibras Pequeñas/complicaciones , Neuropatía de Fibras Pequeñas/patología , Adulto , Anciano , Biopsia/efectos adversos , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Piel/patología , Neuropatía de Fibras Pequeñas/diagnósticoRESUMEN
A 12-year-old girl developed acute erythromelalgia of distal extremities. Physical, imaging and laboratory examinations failed to find an infective, systemic autoimmune, metabolic, endocrine, and vascular origin. The severe pain and allodynia indicated small-fiber neuropathy but muscle weakness suggested an involvement of large myelinated nerve fibers. This was confirmed by electrophysiological testing. High-dose then slowly tapered methylprednisolone resulted in rapid remission of painful erythromelalgia and complete electrophysiological recovery. Our case may suggest an additional variant to recently described steroid-responsive erythromelalgia with small-fiber axonopathy and may denote a transitory variant to Guillain-Barré syndrome or chronic dysimmune neuropathies.
Asunto(s)
Enfermedades Autoinmunes del Sistema Nervioso/patología , Eritromelalgia/inmunología , Eritromelalgia/patología , Fibras Nerviosas Mielínicas/patología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedad Aguda , Edad de Inicio , Enfermedades Autoinmunes del Sistema Nervioso/fisiopatología , Axones/patología , Niño , Eritromelalgia/tratamiento farmacológico , Extremidades/inervación , Extremidades/fisiopatología , Femenino , Humanos , Neuronas Motoras/inmunología , Neuronas Motoras/patología , Debilidad Muscular/inmunología , Debilidad Muscular/patología , Debilidad Muscular/fisiopatología , Neuralgia/inmunología , Neuralgia/patología , Neuralgia/fisiopatología , Nervios Periféricos/patología , Nervios Periféricos/fisiopatología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Piel/inervación , Piel/patología , Piel/fisiopatología , Esteroides/farmacología , Esteroides/uso terapéutico , Resultado del Tratamiento , Degeneración Walleriana/inmunología , Degeneración Walleriana/patología , Degeneración Walleriana/fisiopatologíaRESUMEN
The authors report functional magnetic resonance imaging (fMRI) study data of a 60-year-old patient having short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Three consecutive pain attacks were detected during the imaging session and strong brainstem activation was found. It was concluded that the brainstem can be involved in the pain signal transmission in SUNCT syndrome.
Asunto(s)
Tronco Encefálico/fisiopatología , Síndrome SUNCT/diagnóstico , Síndrome SUNCT/fisiopatología , Vías Autónomas/anatomía & histología , Vías Autónomas/fisiopatología , Mapeo Encefálico/métodos , Tronco Encefálico/anatomía & histología , Tronco Encefálico/irrigación sanguínea , Circulación Cerebrovascular/fisiología , Humanos , Hipotálamo/anatomía & histología , Hipotálamo/fisiopatología , Imagen por Resonancia Magnética/métodos , Masculino , Persona de Mediana Edad , Vías Nerviosas/anatomía & histología , Vías Nerviosas/fisiopatología , Órbita/inervación , Órbita/fisiopatología , Sistema Nervioso Parasimpático/anatomía & histología , Sistema Nervioso Parasimpático/fisiopatología , Nervio Trigémino/anatomía & histología , Nervio Trigémino/fisiopatología , Núcleo Espinal del Trigémino/anatomía & histología , Núcleo Espinal del Trigémino/fisiopatologíaRESUMEN
Voxel-based morphometry and functional magnetic resonance imaging demonstrated severe atrophy and decreased activation of visual attention areas and occipital lobes in a patient with early posterior cortical atrophy compared with healthy controls and patients with early Alzheimer's disease. Our complex approach indicates that structures responsible for attention can be damaged early in posterior cortical atrophy and may contribute to the characteristic decline in higher visual functions.
Asunto(s)
Atrofia/complicaciones , Atrofia/patología , Atención/fisiología , Corteza Cerebral/patología , Lóbulo Frontal/patología , Lóbulo Parietal/patología , Trastornos de la Percepción/etiología , Percepción Visual/fisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos de la Percepción/diagnósticoRESUMEN
Drug-resistant partial epilepsy in children often has a major impact on cognitive development, and early surgical intervention has been advocated to prevent adverse neurobehavioral effects of seizures in such patients. We report a 5-year-old boy who had cryptogenic partial epilepsy of right parietal origin as documented by ictal electroencephalogram (EEG) and glucose metabolism positron emission tomographic (PET) scan. His atonic seizures could not be controlled by multiple antiepilepsy drugs; therefore, cortical resection was scheduled. However, his seizures remitted spontaneously after 1 year of failed medical treatment. The epileptiform abnormality disappeared on the follow-up EEGs, and a glucose PET scan also normalized. This boy has fully retained his cognitive and motor functions and has remained seizure free in the past 4z\x years off medications.
Asunto(s)
Epilepsias Parciales , Convulsiones , Preescolar , Electroencefalografía , Epilepsias Parciales/diagnóstico , Epilepsias Parciales/fisiopatología , Humanos , Masculino , Remisión Espontánea , Convulsiones/fisiopatología , Tomografía Computarizada de EmisiónRESUMEN
Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in children with alternating hemiplegia of childhood. Clinical and neurodevelopmental data, as well as standard uptake values in 25 brain regions and whole-brain serotonin synthesis capacity (unidirectional uptake rate constant or K-complex), were assessed in six patients with alternating hemiplegia of childhood (three girls and three boys; mean age = 7 6/12 years) using alpha[11C]methyl-L-tryptophan positron emission tomography (PET). The PET studies were performed interictally in three patients, during the ictal state in two patients, and postictally in one patient. The PET data were compared to those obtained interictally from six age-matched patients with focal epilepsy (two girls and four boys; mean age = 7 8/12 years) and six non-age-matched apparently normal siblings of autistic children (two girls and four boys; mean age = 9 11/12 years). Patients with alternating hemiplegia of childhood studied in the ictal or postictal state showed increased serotonin synthesis capacity in the frontoparietal cortex, lateral and medial temporal structures, striatum, and thalamus when compared to controls, and subjects with alternating hemiplegia of childhood studied interictally. The involvement of these brain regions was consistent with the semiology of the hemiplegic attacks. In patients with interictal studies and in the controls, the PET scans revealed similar and bilaterally symmetric regional patterns of serotonin synthesis capacity. Increased whole-brain serotonin synthesis capacity (reported in migraine subjects without aura) was not found in the alternating hemiplegia of childhood group. There was no correlation between the neurodevelopmental scores and regional standard uptake values; however, patients with a larger estimated lifetime attack number showed greater delay in communication (P = .005) and daily living skills (P = .042). These studies suggest increased regional serotonergic activity associated with attacks in alternating hemiplegia of childhood. Furthermore, the attack number may have an effect on neurodevelopmental delay, thus supporting the notion that alternating hemiplegia of childhood may be a progressive disorder.
Asunto(s)
Encéfalo/diagnóstico por imagen , Encéfalo/metabolismo , Hemiplejía/diagnóstico por imagen , Serotonina/biosíntesis , Triptófano/análogos & derivados , Adolescente , Adulto , Radioisótopos de Carbono , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Hemiplejía/metabolismo , Humanos , Procesamiento de Imagen Asistido por Computador , Lactante , Masculino , Tomografía Computarizada de EmisiónRESUMEN
The progressive nature of Sturge-Weber syndrome is well known, but the mechanisms of focal cortical and subcortical degeneration in this disorder are poorly understood. In the present study, we assessed the structural and functional integrity of gray and white matter in unihemispheric Sturge-Weber syndrome using quantitative magnetic resonance imaging (MRI) volumetry and MRI-based partial volume correction of [18F]fluorodeoxyglucose positron emission tomographic (PET) images. Gray- and white-matter volumes and glucose metabolism were measured in three brain regions (parieto-occipital underneath the angioma, temporal, and frontal) in six children with Sturge-Weber syndrome (two infants, ages 6 and 9 months; four older children, ages 4 to 14 years), all with unilateral parieto-occipital leptomeningeal angiomatosis. The gray-matter volumes ipsilateral to the angioma were smaller in all children, with the posterior regions underneath the angioma the most affected. In the infants, the white-matter volumes were increased in the region of the angioma, whereas in the regions remote from the angioma in the infants and in all regions of the older children, there were large decreases in white-matter volume. The decreases of frontal and temporal white-matter volume were more pronounced than the corresponding gray-matter volume decreases. The PET studies showed severe hypometabolism in the parieto-occipitalregion underneath the angioma in all of the children. However, the two infants showed glucose hypermetabolism in the frontal and temporal cortical gray matter, whereas these regions had relatively preserved metabolism in the older patients. These results demonstrate differential involvement of gray and white matter in Sturge-Weber syndrome. Both structural and functional abnormalities extend well beyond the angioma, indicating widespread abnormalities of growth and development of the affected hemisphere. Furthermore, whereas increased white-matter volume underlying the angioma may be seen in infants, ipsilateral white-matter regions outside the angioma show volume loss both in infants and in older patients. Extensive gray- and white-matter volume loss and hypometabolism ipsilateral to the angioma likely contribute to the frequently observed progressive cognitive dysfunction in these patients, regardless of the extent of the angioma.