Limbic encephalitis secondary to neuro-Behcet disease: an uncommon presentation. / Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita.

INTRODUCTION: Limbic encephalitis (LE) can have a wide range of etiologies, most frequently infectious (especially viral) or autoimmune. Behcet's disease (BD) can present with heterogeneous neurological manifestations. However, LE is not considered a typical presentation of neuro-Behcet's disease (NBD). CASE REPORT: A 40-years-old male presented with new-onset subacute headaches, memory problems and apathy. A review of systems revealed an unrecorded past history of recurrent oral sores for years, recent malaise and fever, as well as an episode of bilateral panuveitis four months before presentation. His general and neurologic examination revealed slight fever, an isolated oral aphtha, anterograde amnesia and signs of bilateral retinal vasculitis. Brain magnetic resonance imaging displayed a pattern of limbic meningoencephalitis, and his cerebrospinal fluid showed mononuclear inflammation. The patient met BD diagnostic criteria. Considering LE is a very rare presentation of NBD, alternative etiologies were thoroughly assessed and excluded, including infectious, autoimmune and paraneoplastic encephalitis. Therefore, he was diagnosed with NBD, and he recovered well after immunosuppression. DISCUSSION: Only two cases of NBD presenting with LE have been previously reported. We report a third case of this rare presentation and compare it with the previous two. We aim to highlight this association and contribute to enlarge the rich clinical spectrum of NBD.

TITLE: Encefalitis límbica secundaria a neuro-Behçet: una presentación insólita.Introducción. La encefalitis límbica (EL) puede tener un amplio abanico de etiologías, más frecuentemente la infecciosa (sobre todo viral) o autoinmune. La enfermedad de Behçet (EB) puede presentarse con manifestaciones neurológicas heterogéneas. Sin embargo, la EL no se considera una presentación típica del neuro-Behçet (NB). Caso clínico. Varón de 40 años que consultó por cefalea de novo subaguda, problemas de memoria y apatía. La anamnesis por sistemas reveló una historia no conocida previamente de aftas orales recurrentes durante años, fiebre y afectación general reciente, así como un episodio de panuveítis bilateral cuatro meses antes de la presentación. Su exploración general y neurológica reveló febrícula, una afta oral aislada, amnesia anterógrada y signos de vasculitis retiniana bilateral. La resonancia magnética mostró un patrón de afectación de meningoencefalitis límbica y su líquido cefalorraquídeo presentaba inflamación mononuclear. El paciente cumplía los criterios diagnósticos de la EB. Considerando que la EL es una presentación muy rara del NB, se buscaron exhaustivamente y se excluyeron otras etiologías alternativas, incluyendo las encefalitis infecciosas, autoinmunes y paraneoplásicas. En consecuencia, el paciente se diagnosticó de NB y mostró una buena recuperación con tratamiento inmunosupresor. Discusión. Sólo dos casos de NB con presentación en forma de EL se han publicado previamente. Comunicamos el tercer caso de esta rara manifestación clínica de la EB y lo comparamos con los dos anteriores, con el objetivo de destacar dicha asociación y contribuir a expandir el rico espectro clínico del NB.

Cerebral amyloid angiopathy-related inflammation: clinical features and treatment response in a case series.

INTRODUCTION: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an entity characterised by an inflammatory response to ß-amyloid deposition in the walls of cerebral microvessels. METHODS: We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria. RESULTS: The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed. CONCLUSIONS: Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.

[From in-person didactic sessions to videoconferencing during the COVID-19 pandemic: satisfaction survey among participants]. / Cambio de sesiones docentes presenciales a virtuales durante la pandemia de COVID-19 en un servicio de neurología: descripción del proceso y satisfacción de los usuarios.

INTRODUCTION AND AIM: COVID-19 pandemic has disturbed many hospital activities, including medical education. We describe the switch from in-person didactic sessions to videoconferencing in a Neurology department. We analyse the opinions and satisfaction of participants. MATERIAL AND METHODS: Narrative description of the adopted measures; Online survey among participants. RESULTS: One of the three weekly sessions was cancelled, and two switched to videoconferencing. There were more participants online than in the conference hall. 49 users answered the survey, 51% women, mean age 40.5 years (range 25-65). Satisfaction was higher for previous face-to-face meetings (8.68) than for videoconferencing (8.12) (p=0.006). There was a significant inverse correlation between age and satisfaction with virtual sessions (r=-0.37; p=0.01), that was not found for in-person attendance. Most users (75.5%) would prefer to continue with online sessions when the pandemic is over, and 87.8% support inter-hospital remote meetings, but the safety of web platforms is a concern (53.1%). CONCLUSIONS: The change from in-person to virtual sessions is an easy measure to implement in a neurology department, with a good degree of satisfaction among users. There are some unsolved problems with the use of commercial web platforms and inter-hospital connection. Most users recommend leadership and support from educational and health authorities.

TITLE: Cambio de sesiones docentes presenciales a virtuales durante la pandemia de COVID-19 en un servicio de neurología: descripción del proceso y satisfacción de los usuarios.Introducción y objetivo. La pandemia de COVID-19 ha trastornado la actividad hospitalaria, incluyendo la docente. Se describe el cambio de un sistema presencial a otro de sesiones en línea en un servicio de neurología, y se analizan la satisfacción y las opiniones de los usuarios. Material y métodos. Exposición de las medidas adoptadas para pasar a modalidad en línea y análisis de una encuesta entre los participantes. Resultados. Se pasó de tres a dos sesiones semanales, con restricción del público presencial. El público virtual superó al presencial. Contestaron la encuesta 49 participantes, un 51% mujeres, con una media de 40,5 años (rango: 25-65). La satisfacción de los asistentes fue mayor para las sesiones presenciales (8,68) que para las en línea (8,12) (p = 0,006). Existía una correlación inversa significativa entre la edad y la satisfacción con las sesiones en línea (r = ­0,37; p = 0,01) que no se daba para las sesiones presenciales. El 75,5% fue partidario de mantener las sesiones virtuales cuando se eliminaran las restricciones de aforo. Una mayoría (87,8%) apoyó sesiones interhospitalarias y recomienda que las autoridades sanitarias faciliten aplicaciones informáticas seguras (53,1%). Conclusiones. La introducción de sesiones virtuales es una medida fácil de implementar en un servicio de neurología, con un alto grado de satisfacción de los usuarios, aunque menor que con las sesiones presenciales. Existen problemas no resueltos respecto al uso de plataformas comerciales y conexión interhospitalaria. Sería recomendable que las autoridades sanitarias y educativas desarrollaran aplicaciones seguras y fomentaran la educación médica en línea.

Cerebral amyloid angiopathy-related inflammation: clinical features and treatment response in a case series. / Inflamación relacionada con angiopatía amiloide: características clínicas y respuesta al tratamiento en una serie de casos.

INTRODUCTION: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is an entity characterised by an inflammatory response to ß-amyloid deposition in the walls of cerebral microvessels. METHODS: We conducted a retrospective review of a series of patients with a diagnosis of CAA-ri according to histopathological study findings or clinical-radiological diagnostic criteria. RESULTS: The study included 7 patients (5 men) with a mean age of 79 years. Disease onset was acute or subacute in 6 patients. The most frequent symptoms were cognitive impairment (n = 6), behavioural alterations (n = 5), epileptic seizures (n = 5), focal neurological signs (n = 4), and headache (n = 2). Cerebrospinal fluid was abnormal in 3 patients (lymphocytic pleocytosis and high protein levels). The most frequent MRI findings were microbleeds (n = 7), subcortical white matter hyperintensities on T2-FLAIR sequences (n = 7), and leptomeningeal enhancement (n = 6). Lesions were bilateral in 3 patients and most frequently involved the parieto-occipital region (n = 5). Amyloid PET studies were performed in 2 patients, one of whom showed pathological findings. Two patients underwent brain biopsy, which confirmed diagnosis. All patients received immunosuppressive therapy. An initially favourable clinical-radiological response was observed in all cases, with 2 patients presenting radiological recurrence after treatment withdrawal, with a subsequent improvement after treatment was resumed. CONCLUSIONS: Early diagnosis of CAA-ri is essential: early treatment has been shown to improve prognosis and reduce the risk of recurrence. Although a histopathological study is needed to confirm diagnosis, clinical-radiological criteria enable diagnosis without biopsy.

Report by the Spanish Foundation of the Brain on the social impact of Alzheimer disease and other types of dementia. / Informe de la Fundación del Cerebro. Impacto social de la enfermedad de Alzheimer y otras demencias.

INTRODUCTION: Knowledge of the socioeconomic impact of dementia-related disorders is essential for appropriate management of healthcare resources and for raising social awareness. METHODS: We performed a literature review of the published evidence on the epidemiology, morbidity, mortality, associated disability and dependence, and economic impact of dementia and Alzheimer disease (AD) in Spain. CONCLUSIONS: Most population studies of patients older than 65 report prevalence rates ranging from 4% to 9%. Prevalence of dementia and AD is higher in women for nearly every age group. AD is the most common cause of dementia (50%-70% of all cases). Dementia is associated with increased morbidity, mortality, disability, and dependence, and results in a considerable decrease in quality of life and survival. Around 80% of all patients with dementia are cared for by their families, which cover a mean of 87% of the total economic cost, resulting in considerable economic and health burden on caregivers and loss of quality of life. The economic impact of dementia is huge and difficult to evaluate due to the combination of direct and indirect costs. More comprehensive programmes should be developed and resources dedicated to research, prevention, early diagnosis, multidimensional treatment, and multidisciplinary management of these patients in order to reduce the health, social, and economic burden of dementia.

[Trajan's decision: a neurological point of view]. / La decisión de Trajano: un punto de vista neurológico.

The emperor of Rome, Marco Ulpio Trajano, ruled the Roman Empire from 98 to 117 AD, being the first emperor of non-Italian origin and the man who took the Empire to its maximum geographical extent. Trajan's death is surrounded by mystery, given Adriano's controversial adoption as his successor just before his death, as well as rumors of poisoning by his wife, Plotina. In addition, despite the limited literary sources available, episodes of «paralysis¼, «stroke¼, «dropsy¼, diarrhea and nonspecific episodes of «illness¼ have been documented, related to the worsening of his health the months before his death. His special love of wine and life habits related to the personality of the emperor, could be associated with his delicate state of health during the last year of his government, although it is not possible to rule out other pathological processes with neurological involvement associated with the last years of life of the optimus princeps, which could interfere with his last decisions as ruler. In this article, the historical sources available are reviewed in order to analyze, from the neurological point of view, the last moments of the emperor with which Rome reached its maximum military splendor.

TITLE: La decisión de Trajano: un punto de vista neurológico.El emperador de Roma Marco Ulpio Trajano gobernó el Imperio romano desde el año 98 hasta el 117 d.C., y fue el primer emperador de origen no itálico y el hombre que llevó al Imperio a su máxima extensión geográfica. La muerte de Trajano está rodeada de misterio, dada la polémica adopción de Adriano como su sucesor justo antes del momento de su fallecimiento, así como los rumores de envenenamiento por parte de su mujer, Plotina. Además, a pesar de las escasas fuentes literarias disponibles, se han documentado episodios de «parálisis¼, «apoplejía¼, «hidropesía¼, diarrea y episodios inespecíficos de «enfermedad¼ relacionados con el empeoramiento de su salud los meses antes de su muerte. Su especial afición al vino y los hábitos de vida relacionados con la personalidad del emperador pudieron estar asociados con su delicado estado de salud durante el último año de su gobierno, si bien no es posible descartar otros procesos patológicos con afectación neurológica asociados a los últimos años de vida del optimus princeps, los cuales pudieron interferir con sus últimas decisiones como gobernante. En este artículo se revisan las fuentes históricas disponibles con el objeto de analizar, desde el punto de vista neurológico, los últimos momentos del emperador con el que Roma alcanzó su máximo esplendor militar.

Syndrome and outcome of antibody-negative limbic encephalitis.

BACKGROUND AND PURPOSE: The aim was to report the clinical characteristics of 12 patients with limbic encephalitis (LE) who were antibody-negative after a comprehensive immunological study. METHODS: The clinical records of 163 patients with LE were reviewed. Immunohistochemistry on rat brain, cultured neurons and cell-based assays were used to identify neuronal autoantibodies. Patients were included if (i) there was adequate clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging information to classify the syndrome as LE, (ii) magnetic resonance images were accessible for central review and (iii) serum and CSF were available and were confirmed negative for neuronal antibodies. RESULTS: Twelve (7%) of 163 LE patients [median age 62 years; range 40-79; 9 (75%) male] without neuronal autoantibodies were identified. The most frequent initial complaints were deficits in short-term memory leading to hospital admission in a few weeks (median time 2 weeks; range 0.5-12). In four patients the short-term memory dysfunction remained as an isolated symptom during the entire course of the disease. Seizures, drowsiness and psychiatric problems were unusual. Four patients had solid tumors (one lung, one esophagus, two metastatic cervical adenopathies of unknown primary tumor) and one chronic lymphocytic leukemia. CSF showed pleocytosis in seven (58%) with a median of 13 white blood cells/mm3 (range 9-25). Immunotherapy included corticosteroids, intravenous immunoglobulins and combinations of both drugs or with rituximab. Clinical improvement occurred in six (54%) of 11 assessable patients. CONCLUSIONS: Despite the discovery of new antibodies, 7% of LE patients remain seronegative. Antibody-negative LE is more frequent in older males and usually develops with predominant or isolated short-term memory loss. Despite the absence of antibodies, patients may have an underlying cancer and respond to immunotherapy.

Slowly progressive behavioral frontotemporal dementia with C9orf72 mutation. Case report and review of the literature.

We present a 86-year-old woman without relevant medical history and two brothers who died by dementia, who started at 55 years with depression and personality changes with ongoing worsening (>30 years) and functional decline. Screening dementia blood test and brain magnetic resonance imaging did not show results that pointed to a secondary cause. The patient met the diagnostic criteria for possible behavioral frontotemporal dementia with a slow progression (bvFTD-SP), suggesting a benign variant. A genetic study confirmed a C9ORF72 hexanucleotide expansion, making this the sixth case mentioned in the literature. We review and discuss the other cases described previously.

TARDBP mutation associated with semantic variant primary progressive aphasia, case report and review of the literature.

Semantic variant primary progressive aphasia (svPPA) is a clinical syndrome included in the frontotemporal dementia (FTD) spectrum. Unlike other forms of FTD, it is sporadic in the majority of cases and not commonly associated with motor neuron disease (MND). We describe a case of svPPA associated with MND in the same family, due to a mutation of the transactive response DNA binding protein (TARDBP) gene, and review the literature.

Alzheimer's disease prevention: A way forward. / Prevención de la enfermedad de Alzheimer: un camino a seguir.

This review proposes a more optimistic view of Alzheimer's disease (AD), in contrast to that contributed by the ageing of the population and the failure of potentially curative therapies (vaccines and others). Treatment failure is likely due to the fact that AD gestates in the brain for decades but manifests in old age. This review updates the concept of AD and presents the results of recent studies that show that primary prevention can reduce the incidence and delay the onset of the disease. Half of all cases of AD are potentially preventable through education, the control of cardiovascular risk factors, the promotion of healthy lifestyles and specific drug treatments. These approaches could substantially reduce the future incidence rate of this disease.

Practical application of brief cognitive tests.

INTRODUCTION: Brief cognitive tests (BCT) may help detect cognitive impairment (CI) in the clinical setting. Several BCT have been developed and/or validated in our country, but we lack specific recommendations for use. DEVELOPMENT: Review of studies on the diagnostic accuracy of BCT for CI, using studies conducted in Spain with BCT which take less than 20 min. We provide recommendations of use based on expert consensus and established on the basis of BCT characteristics and study results. CONCLUSION: The Fototest, the Memory Impairment Screen (MIS) and the Mini-Mental State Examination (MMSE) are the preferred options in primary care; other BCT (Clock Drawing Test [CDT], test of verbal fluency [TVF]) may also be administered in cases of negative results with persistent suspected CI or concern (stepwise approach). In the specialised care setting, a systematic assessment of the different cognitive domains should be conducted using the Montreal Cognitive Assessment, the MMSE, the Rowland Universal Dementia Assessment, the Addenbrooke's Cognitive Examination, or by means of a stepwise or combined approach involving more simple tests (CDT, TVF, Fototest, MIS, Memory Alteration Test, Eurotest). Associating an informant questionnaire (IQ) with the BCT is superior to the BCT alone for the detection of CI. The choice of instruments will depend on the patient's characteristics, the clinician's experience, and available time. The BCT and IQ must reinforce - but never substitute - clinical judgment, patient-doctor communication, and inter-professional dialogue.

[Numb chin syndrome caused by biphosphonates-induced osteonecrosis of the jaw]. / Hipoestesia del mentón secundaria a osteonecrosis mandibular inducida por bifosfonatos.

INTRODUCTION: Numb chin syndrome is caused by a mental or inferior alveolar nerve neuropathy. Traumatic and infectious injuries are the most frequent causes of the syndrome but, if an evident cause does not exist, a neoplastic etiology must be investigated. Other causes of the numb chin syndrome are rare. CASE REPORT: A 73-year-old woman had had a diagnosis of metastatic breast cancer and was been treated with zoledronic acid. She attended because of hypoesthesia and dysesthesia of the chin congruent with mental nerve distribution. A computed tomography of the jaw showed an osteolytic lesion with central bone sequestration, so biphosphonate-induced osteonecrosis of the jaw was diagnosed. After zoledronic acid was withdrawn, clinical neuropathy and imaging findings remained stable. CONCLUSIONS: Biphosphonates-induced osteonecrosis of the jaw is a recently described condition. It has been rarely reported as a cause for numb chin syndrome. In the future, osteonecrosis of the jaw must be considered in the differential diagnosis of this syndrome in cancer patients treated with biphosphonates.

[Post-traumatic supraorbital neuralgia: a benign condition]. / Neuralgia supraorbitaria postraumática: una entidad benigna.

INTRODUCTION: Supraorbital neuralgia has only recently been described. Most of the cases reported involve patients suffering from chronic idiopathic neuralgias that are difficult to treat and sometimes require surgery to release the nerve. We present our experience in patients with a variant of this neuralgia which has a known causation, is commonly seen and has a benign prognosis. CASE REPORTS: We studied five patients, four females and one male, with a mean age of 55 years (range: 29-69 years). They had all suffered direct banal traumatic injury to the frontal region due to different causes. Four of them developed continuous, piercing or burning-type pain; three of them had paroxysmal pain and one had itching. There were no autonomic manifestations. All of them were found to be abnormally sensitive in the affected area, with tactile hypaesthesia, hyperalgesia or allodynia and a positive Tinel's sign. Neuroimaging tests were normal. Two patients were treated with gabapentin and amitriptyline. One was treated with an anaesthetic blockade, which afforded temporary relief. Three of them received no treatment at all. After one year of follow-up, all of them had improved and three were no longer in pain, although sensory alterations persisted in all cases. CONCLUSIONS: Post-traumatic supraorbital neuralgia is a frequent condition, although it is probably underdiagnosed. It has its own characteristic clinical and developmental features that distinguish it from idiopathic supraorbital neuralgia. Progress is usually good and it responds favourably to symptomatic treatment, if needed.

[Sarcoid myopathy. Report of two cases and review of the bibliography]. / Miopatía sarcoidea. Presentación de dos casos y revisión de la bibliografía.

INTRODUCTION: 5% of patients with sarcoidosis are affected by neurological complications, of which myopathy is one of the least frequent. We report the clinical course and therapeutic response of two patients with sarcoid myopathy. CASE REPORTS: We observed two females aged 63 and 55 who were previously diagnosed with sarcoidosis, which in one case was cutaneous and ophthalmic and in the other pulmonary. Both were asymptomatic from a systemic point of view when they visited the Neurology department. Both patients developed chronic myopathy (with a history of 9 months and 6 years), which was predominantly proximal, painful and both of them had normal or slightly high levels of creatine phosphokinase. The EMG was clearly myopathic in the two patients. A muscle biopsy showed a mononuclear-cell infiltrate with the formation of non-caseating granulomas. Both patients were treated with prednisone, although the therapeutic response was different in each case. One of the patients showed an important improvement in just a few days, while in the other case the disease remained stable, despite adding, first, azathioprine and later methotrexate to the treatment. CONCLUSIONS: Sarcoidosis can cause chronic, predominantly proximal, myopathy, and chiefly affects females over 50 years of age. The therapeutic response to steroids and immunosuppressants varies from case to case.

[Brachial presentation of spinal pseudochoreoathetosis. The result of proprioceptive information being processed in parallel]. / Seudocoreoatetosis espinal de presentación braquial. Resultado del procesamiento paralelo de la información propioceptiva.

INTRODUCTION: The term pseudochoreoathetosis is used to refer to the choreoathetoid movements that are produced by alterations in the proprioceptive sensitivity due to damage it has suffered at some point along its course. Proprioceptive sensitivity is considered to go up as far as the cortex along the posterior cords of the spinal cord, which means that if they are injured in the cervical region there should be a sensory deficit in both the upper (UL) and lower limbs (LL). CASE REPORTS: We describe five cases of transverse myelitis with localised cervical injury that selectively and mainly affected the posterior cords of the spinal cord. In the five patients there was selective involvement of the proprioceptive sensitivity in the UL respecting the LL and pseudochoreoathetoid movements of the limb that has been deafferented for proprioceptive sensitivity. The dissociation between the UL and the LL occurs because the spinocerebellar and spinocervical tracts take the proprioceptive information from the LL in parallel to the posterior cords, which receive the proprioceptive sensitivity from the UL. CONCLUSIONS: At present, the most widely accepted physiopathological mechanism explaining pseudochoreoathetosis consists in a failure in the integration of the sensory-motor afferences in the striatum, which causes faulty sensory-motor integration at this level and gives rise to pseudochoreoathetosis.

[Spinal epidural abscess in a patient with septicaemia]. / Absceso epidural vertebral en un paciente con septicemia.

INTRODUCTION: Spinal epidural abscess is a rare entity requiring early diagnosis and treatment. Sepsis is a factor with an unfavourable prognosis. CASE REPORT: We report the case of a 57 year old female with acute low back pain who was admitted to hospital suffering from a state of septic shock and multiple organ failure secondary to an infection disseminated by Staphylococcus aureus, which was treated early on with vancomycin. The probable source of infection was assumed to be necrotizing fasciitis of the left arm. Once the acute phase had been overcome, serious paraparesis became apparent and this led to magnetic resonance imaging of the spine being carried out, the results of which showed the existence of a lumbar spondylodiscitis with associated epidural abscess. CONCLUSION: In patients with sepsis and some previous symptom that arouses suspicion, it is important to consider this possible diagnosis, since treatment with antibiotics alone does not manage to prevent neurological complications in all cases.