RESUMEN
A 61-year-old man presented with slowly progressive cognitive decline. Brain MRI revealed left frontal lobe lesions with gadolinium enhancement along pia mater. Brain biopsy was performed and histopathological findings was compatible with the diagnosis of IgG4-related disease (IgG4-RD). Serum IgG4 level was within a normal range, and no other systemic organs were suggested to be involved by clinical symptoms or laboratory findings. Intravenous methylprednisolone therapy followed by oral prednisone induction markedly improved the cognitive functions and MRI findings detected at the initial diagnosis. Our case highlights the importance of including IgG4-RD as one of the differential diagnosis when encountering the patients suffering from isolated cranial lesions even in the absence of normal serum IgG4 level.
Asunto(s)
Enfermedad Relacionada con Inmunoglobulina G4 , Medios de Contraste , Gadolinio , Humanos , Inmunoglobulina G , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Masculino , Metilprednisolona , Persona de Mediana EdadRESUMEN
A 67-year-old man presented slowly progressive weakness of the extremities visited our hospital. Nerve conduction study showed axonal neuropathy and needle electromyography showed neurogenic changes with denervation findings in multiple limb muscles. While he was diagnosed as Probable amyotrophic lateral sclerosis (ALS), which is defined by the Awaji criteria for diagnosis of ALS, he did not develop either respiratory muscle paralysis or bulbar palsy, which are characteristic symptoms of sporadic ALS. Genetic testing revealed a novel gene variant in senataxin (SETX), the causative gene of ALS4. We could not make a definite diagnosis of ALS4 because he had no relatives who could perform genetic testing (segregation study). However, we considered the variant can be pathogenic because it was not previously reported and absent in at least 1,000 healthy control individuals, the variant site was highly conserved in mammals, and it may impair the function of senataxin protein (in silico analysis).
Asunto(s)
Esclerosis Amiotrófica Lateral , Parálisis Bulbar Progresiva , Anciano , Esclerosis Amiotrófica Lateral/diagnóstico , Esclerosis Amiotrófica Lateral/genética , ADN Helicasas/genética , Electromiografía , Humanos , Masculino , Enzimas Multifuncionales/genética , Músculo Esquelético/patología , ARN Helicasas/genéticaRESUMEN
BACKGROUND: Callithrix jacchus, generally known as the common marmoset, has recently garnered interest as an experimental primate model for better understanding the basis of human social behavior, architecture and function. Modelling human neurological and psychological diseases in marmosets can enhance the knowledge obtained from rodent research for future pre-clinical studies. Hence, comprehensive and quantitative assessments of marmoset behaviors are crucial. However, systems for monitoring and analyzing marmoset behaviors have yet to be established. NEW METHOD: In this paper, we present a novel multimodal system, MarmoDetector, for the automated 3D analysis of marmoset behavior under freely moving conditions. MarmoDetector allows the quantitative assessment of marmoset behaviors using computerised tracking analysis techniques that are based on a Kinect system equipped with video recordings, infrared images and depth analysis. RESULTS: Using MarmoDetector, we assessed behavioral circadian rhythms continuously over several days in home cages. In addition, MarmoDetector detected acute, transient complex behaviors of alcohol injected marmosets. COMPARISON TO EXISTING METHOD: Compared to 2D recording, MarmoDetector detects activities more precisely and is very sensitive as we could detect behavioral defects specifically induced by alcohol administration. CONCLUSION: MarmoDetector facilitates the rapid and accurate analysis of marmoset behavior and will enhance research on the neural basis of brain disorders.
Asunto(s)
Conducta Animal , Callithrix , Reconocimiento de Normas Patrones Automatizadas/métodos , Animales , Ritmo Circadiano , Femenino , Procesamiento de Imagen Asistido por Computador , Masculino , Actividad Motora , Grabación en VideoRESUMEN
Neural interfaces enabling light transmittance rely on optogenetics to control and monitor specific neural activity, thereby facilitating deeper understanding of intractable diseases. This study reports the material strategy underlying an optogenetic neural interface comprising stretchable and transparent conductive tracks and capable of demonstrating high biocompatibility after long-term (5-month) implantation. Ag/Au core-shell nanowires contribute toward improving track performance in terms of stretchability (<60% strain), transparency (<83%), and electrical resistance (15 Ω sq-1 ). The neural interface integrated with gel-coated exterior microelectrodes preserves low impedance (1.1-3.2 Ω cm2 ) in a saline solution over the evaluated 5-month period. Besides the use of efficient conductive materials, surface treatment using antithrombogenic polymer tends to prevent the growth of granulation tissue, thereby facilitating clear monitoring of electrocorticograms (ECoG) in a rodent during chronic implantation. The flexible and transparent neural interface pathologically exhibits noncytotoxicity and low inflammatory response while efficiently recording evoked ECoG in a nonhuman primate via optogenetic stimulation. The proposed highly reliable interface can be employed in multifaceted approaches for translational research based on chronic implants.
Asunto(s)
Corteza Cerebral/fisiología , Oro/química , Nanocables/química , Optogenética/métodos , Plata/química , Animales , Impedancia Eléctrica , Electrocorticografía , Electrodos Implantados , Potenciales Evocados Somatosensoriales/fisiología , Alcohol Polivinílico/química , RatasRESUMEN
We herein report the case of a 72-year-old man demonstrating myasthenia gravis (MG) with a dropped head and acute respiratory insufficiency. There was no ocular, bulbar, or limb involvement. The patient was seronegative for anti-acetylcholine receptor (AChR) antibodies and anti-muscle-specific tyrosine kinase (MuSK) antibodies. Subsequent tests showed seropositivity for anti-low-density lipoprotein receptor-related protein 4 (LRP4) antibodies. The addition of steroid pulse therapy resulted in a full remission of his respiratory symptoms. This presentation suggests that LRP4-positive MG should be considered in the differential diagnosis of patients presenting with acute respiratory insufficiency without either cranial or limb involvement.
Asunto(s)
Proteínas Relacionadas con Receptor de LDL/inmunología , Miastenia Gravis/complicaciones , Miastenia Gravis/diagnóstico , Insuficiencia Respiratoria/etiología , Enfermedad Aguda , Anciano , Diagnóstico Diferencial , Humanos , MasculinoRESUMEN
The land slug Limax performs both aversive and appetitive olfactory learning, and we investigated neurotransmitters involved in each type of learning. Slugs were conditioned by presenting a vegetable juice (appetitive conditioning) or a mixture of vegetable juice and quinidine (aversive conditioning), and the latency to reach the juice became shorter (appetitive conditioning) or longer (aversive conditioning) after conditioning. L-NAME injected either before conditioning or testing blocked the reduction in latency in appetitive conditioning but had no significant effects in aversive conditioning. 5,7-dihydroxytryptamine had no significant effects in appetitive conditioning. These results suggest different mechanisms for appetitive and aversive learning.