Correlation of Global and Regional Amyloid Burden by 18 F-Florbetaben PET/CT With Cognitive Impairment Profile and Severity.

PURPOSE: To assess the correlation between profile and severity deterioration in the neuropsychological assessment and the most affected regions in amyloid PET semiquantification. The influence of vascular risk and other potential confounding factors was also evaluated. METHODS: A retrospective, observational, and multicenter study including all patients referred for amyloid PET in daily practice was conducted. Patients underwent neuropsychological assessment, and cognitive decline severity and domain(s) affected were recorded. The patients were grouped according to cognitive impairment (CI) profile and severity: (A) no CI, single-domain amnestic CI, multiple-domain amnestic CI, and nonamnestic CI; and (B) mild CI, moderate and severe dementia. An adapted Framingham Stroke Risk Profile was calculated for each individual. Depression and parkinsonism were also recorded. Standardized quantitative analysis software was used to obtain standardized uptake value ratio (SUVR) values from PET/CT images. The corresponding associations were assessed with the most appropriate statistical tests. RESULTS: One hundred twenty-nine patients were included (62 men, 67 women; 64.67 ± 7.47 years old). Significant differences in global and regional amyloid load were exclusively found in women between non-CI and moderate dementia ( P = 0.006, for total-cerebellum SUVR). Posterior and anterior cingulates and prefrontal cortex best represented CI severity ( P = 0.003, 0.006, and 0.006, respectively). No relationship between the CI profile and the regional amyloid load was shown. A significantly high positive correlation was found between age and vascular risk and between these variables and amyloid load in nearly all regions, especially in women with moderate dementia. CONCLUSION: Semiquantitative analysis of amyloid PET by SUVR values revealed a significant correlation between amyloid burden and CI severity, although only in women.

Increasing the confidence of 18F-Florbetaben PET interpretations: Machine learning quantitative approximation.

AIM: To assess the added value of semiquantitative parameters on the visual assessment and to study the patterns of 18F-Florbetaben brain deposition. MATERIALS AND METHODS: Retrospective analysis of multicenter study performed in patients with mild cognitive impairment or dementia of uncertain origin. 18F-Florbetaben PET scans were visually interpreted by two experienced observers, analyzing target regions in order to calculate the interobserver agreement. Semiquantification of all cortical regions with respect to three reference regions was performed to obtain standardized uptake value ratios (SUVRs). The ability of SUVRs to predict the visual evaluation, the possibility of preferential radiotracer deposition in some target regions and interhemisphere differences were analyzed. RESULTS: 135 patients were evaluated. In the visual assessment, 72 were classified as positive. Interobserver agreement was excellent. All SUVRs were significantly higher in positive PET scans than in negative ones. Prefrontal area and posterior cingulate were the cortical regions with the best correlations with the visual evaluation, followed by the composite region. Using ROC analysis, the SUVRs obtained in same target locations showed the best diagnostic performance. CONCLUSIONS: The derived information from target regions seems to help the visual classification, based on a preferential amyloid ß deposit, allowing machine learning. The amyloid ß deposit, although diffuse in all cortical regions, seems not to be uniform and symmetric.

Epiteliopatía pigmentaria placoide multifocal posterior aguda. Una rara causa de ictus isquémico / Acute posterior multifocal placoid pigment epitheliopathy. A rare cause of ischaemic stroke

Introducción. La epiteliopatía pigmentaria placoide multifocal posterior aguda (EPPMPA) es una enfermedad inflamatoria rara, generalmente de etiología indeterminada, de la coriocapilar, el epitelio pigmentario y la retina externa. Afecta predominantemente a pacientes jóvenes y en algunos casos puede involucrar al sistema nervioso central en forma de ictus o de meningoencefalitis. Presentamos el caso clínico de una mujer joven con EPPMPA complicada con ictus e hipertensión intracraneal. Caso clínico. Mujer de 16 años que comienza con cefalea intensa sugestiva de hipertensión intracraneal, así como con un déficit agudo hemisférico izquierdo. La resonancia magnética craneal ponía de manifiesto lesiones embólicas o vasculíticas en diferentes territorios. No se evidenciaron datos de meningoencefalitis en el estudio del líquido cefalorraquídeo, pero sí de hipertensión intracraneal asociada. La presencia de lesiones muy específicas en el polo ocular posterior permitió el diagnóstico de EPPMPA complicada con ictus isquémico, probablemente por mecanismo vasculítico. Un amplio estudio etiológico fue negativo para identificar un factor desencadenante claro del proceso. Se inició tratamiento corticoideo con buena evolución clínica y radiológica. Conclusiones. La EPPMPA es una entidad rara que generalmente entraña buen pronóstico; sin embargo, en algunos casos puede complicarse con afectación del sistema nervioso central, y el ictus isquémico secundario a vasculitis es la complicación más grave. Ante un paciente joven con ictus que presente sintomatología visual y lesiones coriorretinianas, debe considerarse la EPPMPA en su diagnóstico etiológico (AU)

Introduction. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory disease, generally of unknown aetiology, affecting the choriocapillaris, the pigment epithelium and the outer retina. It predominantly affects young patients and in some cases may involve the central nervous system in the form of strokes or meningoencephalitis. We report the clinical case of a young female with APMPPE that was complicated by stroke and intracranial hypertension. Case report. Our patient was a 16-year-old female who began with intense headaches suggesting intracranial hypertension, as well as with an acute deficit in the left hemisphere. A magnetic resonance scan of the head revealed embolic or vasculitic lesions in different territories. No evidence of meningoencephalitis was found in the cerebrospinal fluid analysis, but signs of associated intracranial hypertension were observed. The presence of very specific lesions in the posterior pole of the eye led to a diagnosis of APMPPE complicated by ischaemic stroke, probably caused by a vasculitic mechanism. An extensive aetiological study failed to identify a clear precipitating factor underlying the process. Treatment with corticoids was established, with good clinical and radiological progression. Conclusions. APMPPE is an infrequent condition that generally has a good prognosis. In some cases, however, complications may arise owing to involvement of the central nervous system, and ischaemic stroke secondary to vasculitis is the most severe complication. In young patients with stroke who present visual symptoms and chorioretinital lesions, APMPPE must be considered in the aetiological diagnosis (AU)

[Acute posterior multifocal placoid pigment epitheliopathy. A rare cause of ischaemic stroke]. / Epiteliopatía pigmentaria placoide multifocal posterior aguda. Una rara causa de ictus isquémico.

INTRODUCTION: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory disease, generally of unknown aetiology, affecting the choriocapillaris, the pigment epithelium and the outer retina. It predominantly affects young patients and in some cases may involve the central nervous system in the form of strokes or meningoencephalitis. We report the clinical case of a young female with APMPPE that was complicated by stroke and intracranial hypertension. CASE REPORT: Our patient was a 16-year-old female who began with intense headaches suggesting intracranial hyper-tension, as well as with an acute deficit in the left hemisphere. A magnetic resonance scan of the head revealed embolic or vasculitic lesions in different territories. No evidence of meningoencephalitis was found in the cerebrospinal fluid analysis, but signs of associated intracranial hypertension were observed. The presence of very specific lesions in the posterior pole of the eye led to a diagnosis of APMPPE complicated by ischaemic stroke, probably caused by a vasculitic mechanism. An extensive aetiological study failed to identify a clear precipitating factor underlying the process. Treatment with corticoids was established, with good clinical and radiological progression. CONCLUSIONS: APMPPE is an infrequent condition that generally has a good prognosis. In some cases, however, complications may arise owing to involvement of the central nervous system, and ischaemic stroke secondary to vasculitis is the most severe complication. In young patients with stroke who present visual symptoms and chorioretinital lesions, APMPPE must be considered in the aetiological diagnosis.

TITLE: Epiteliopatia pigmentaria placoide multifocal posterior aguda. Una rara causa de ictus isquemico.Introduccion. La epiteliopatia pigmentaria placoide multifocal posterior aguda (EPPMPA) es una enfermedad inflamatoria rara, generalmente de etiologia indeterminada, de la coriocapilar, el epitelio pigmentario y la retina externa. Afecta predominantemente a pacientes jovenes y en algunos casos puede involucrar al sistema nervioso central en forma de ictus o de meningoencefalitis. Presentamos el caso clinico de una mujer joven con EPPMPA complicada con ictus e hipertension intracraneal. Caso clinico. Mujer de 16 anos que comienza con cefalea intensa sugestiva de hipertension intracraneal, asi como con un deficit agudo hemisferico izquierdo. La resonancia magnetica craneal ponia de manifiesto lesiones embolicas o vasculiticas en diferentes territorios. No se evidenciaron datos de meningoencefalitis en el estudio del liquido cefalorraquideo, pero si de hipertension intracraneal asociada. La presencia de lesiones muy especificas en el polo ocular posterior permitio el diagnostico de EPPMPA complicada con ictus isquemico, probablemente por mecanismo vasculitico. Un amplio estudio etiologico fue negativo para identificar un factor desencadenante claro del proceso. Se inicio tratamiento corticoideo con buena evolucion clinica y radiologica. Conclusiones. La EPPMPA es una entidad rara que generalmente entrana buen pronostico; sin embargo, en algunos casos puede complicarse con afectacion del sistema nervioso central, y el ictus isquemico secundario a vasculitis es la complicacion mas grave. Ante un paciente joven con ictus que presente sintomatologia visual y lesiones coriorretinianas, debe considerarse la EPPMPA en su diagnostico etiologico.

Psoriasis during natalizumab treatment for multiple sclerosis.

Beta-interferon therapy is known to trigger psoriasis, but this effect has not been previously reported for other multiple sclerosis treatments, such as natalizumab. The following is a case report. A 31-year-old woman affected by psoriasis and relapsing-remitting multiple sclerosis suffered a severe worsening of psoriasis symptoms during natalizumab treatment and acquired a drug-resistant course. This case suggests that aggravation of psoriasis might be a rare side effect of natalizumab, and that clinicians should be aware of the possibility of paradoxical activation of autoimmune diseases during its treatment.

Oftalmoplejía dolorosa recurrente secundaria a displasia fibrosa poliostótica del seno maxilar con afectación de la hendidura esfenoidal / Recurrent painful ophthalmoplegia secondary to polyostotic fibrous dysplasia of the maxillary sinuses with involvement of the superior orbital fissure

Introducción. La displasia fibrosa es una enfermedad ósea benigna que suele cursar con lesiones asintomáticas pero que en ocasiones puede dar manifestaciones neurológicas por afectación de los huesos craneofaciales. Caso clínico. Mujer de 25 años, con antecedentes de migraña, que consultó a los 18 años por un cuadro de dolor en la región maxilar y retrocular derecha de características diferentes de las de su migraña habitual y que asocia oftalmoparesia ipsolateral; presenta un curso autolimitado con buena respuesta a corticoides, aunque recidivante. La exploración puso de manifiesto un intenso dolor a la palpación en el seno maxilar derecho y parálisis incompleta del III par craneal ipsolateral con ptosis palpebral. Los estudios complementarios fueron normales, salvo la resonancia magnética craneal y la tomografía axial computarizada facial, donde se evidenció una lesión expansiva con afectación del seno maxilar superior derecho y el ala mayor del esfenoides, con probable afectación de la hendidura esfenoidal, compatible con el diagnóstico de displasia fibrosa, que fue confirmado mediante estudio anatomopatológico. Conclusiones. La displasia fibrosa es un trastorno óseo benigno cuya etiología se desconoce y que consiste en la sustitución del tejido óseo normal por tejido conjuntivo amorfo. En ocasiones, existe afectación craneofacial y se forma una masa ósea hipertrófica que puede ocupar los senos paranasales y la órbita, y dar lugar a exoftalmos y alteraciones visuales. Hasta el momento, no se ha descrito en la bibliografía científica la manifestación de esta enfermedad en forma de brotes de oftalmoparesia dolorosa recurrente con respuesta a corticoides, como ocurre en nuestra paciente (AU)

Introduction. Fibrous dysplasia is a bone disease that is usually accompanied by asymptomatic lesions but which may sometimes display neurological manifestations due to the involvement of the craniofacial bones. Case report. A 25-year-old female, with a history of migraines, who visited at the age of 18 due to pain in the right retro-ocular and maxillary region, although with characteristics that were unlike those of her usual migraines, and which was associated with ipsilateral ophthalmoparesis. The condition had a self-limiting course and responded well to corticoids, although it was recurring. Examination revealed intense pain on palpation of the right-hand maxillary sinus and incomplete paralysis of the ipsilateral oculomotor nerve with palpebral ptosis. Results of complementary studies were normal, except for magnetic resonance imaging of the head and computerised axial tomography of the face, which revealed an expansive lesion with involvement of the right superior maxillary sinus and the greater wing of the sphenoid bone, with probable compromise of the superior orbital fissure, consistent with the diagnosis of fibrous dysplasia, which was confirmed by means of a pathology study. Conclusions. Fibrous dysplasia is a benign bone disorder, of unknown causation, in which normal bone tissue is replaced by amorphous conjunctive tissue. There is sometimes craniofacial involvement and a hypertrophic bone mass is formed which can fill the paranasal sinuses and the orbit, resulting in exophthalmus and visual disorders. To date the scientific literature does not include any reports of this disease manifesting as bouts of recurrent painful ophthalmoparesis which responds to corticoids, as happened in the case of our patient (AU)

[The use of oral lacosamide in a patient with refractory partial epileptic status]. / Uso de lacosamida oral en un paciente con estado epiléptico parcial refractario.

INTRODUCTION: Generalised epileptic status is a medical emergency with a well-defined therapeutic algorithm. Nevertheless, in the partial status, treatment must be tailored to each patient and the risk of intensive treatment has to be assessed. Unlike generalised epileptic status, partial status is not considered a medical emergency, which means that when it comes to choosing a type of treatment the risk-benefit ratio must be carefully evaluated bearing in mind the characteristics of each particular case. CASE REPORT: A 72-year-old male with partial status secondary to an ischaemic lesion which was refractory to conventional treatment; treatment was established with lacosamide and his condition was rapidly inverted. This is the third case that has been reported of epileptic status being treated with this drug and the second in which it is employed orally. CONCLUSIONS: Although further research is required, lacosamide could be a good therapeutic option with few interactions and side effects.

Uso de lacosamida oral en un paciente con estado epiléptico parcial refractario / The use of oral lacosamide in a patient with refractory partial epileptic status

Introducción. El estado epiléptico generalizado es una urgencia médica con un algoritmo terapéutico bien definido. Sin embargo, en el estado parcial, el tratamiento debe individualizarse y valorar el riesgo del tratamiento intensivo. A diferencia del estado epiléptico generalizado, el estado parcial no se considera una emergencia médica, por lo que en la elección de su tratamiento debe valorarse cuidadosamente y de modo individualizado la relación riesgo-beneficio.Caso clínico. Varón de 72 años con estado parcial secundario a lesión isquémica y refractario a tratamiento convencional, en el que se inicia tratamiento con lacosamida y se consigue su rápida reversión. Se trata del tercer caso de estado epiléptico publicado con este fármaco y del segundo en el que se emplea vía oral. Conclusión. Aunque se requieren más estudios la lacosamida podría representar una buena opción terapéutica con escasas interacciones y efectos secundarios (AU)

Introduction. Generalised epileptic status is a medical emergency with a well-defined therapeutic algorithm. Nevertheless, in the partial status, treatment must be tailored to each patient and the risk of intensive treatment has to be assessed. Unlike generalised epileptic status, partial status is not considered a medical emergency, which means that when it comes to choosing a type of treatment the risk-benefit ratio must be carefully evaluated bearing in mind the characteristics of each particular case. Case report. A 72-year-old male with partial status secondary to an ischaemic lesion which was refractory to conventional treatment; treatment was established with lacosamide and his condition was rapidly inverted. This is the third case that has been reported of epileptic status being treated with this drug and the second in which it is employed orally. Conclusions. Although further research is required, lacosamide could be a good therapeutic option with few interactions and side effects (AU)