Lightning-related injuries most often involve impairment of the functions of the central and peripheral nervous systems, usually including cognitive dysfunctions. We evaluated the cognitive deficit of a patient who had survived a lightning strike and measured the improvement after her cognitive training. This therapeutic method appears to be a powerful tool in the neurorehabilitation treatment.
Amyotrophic lateral sclerosis (ALS), the most common adult-onset motor neuron disorder, is characterized by the progressive and selective loss of upper and lower motor neurons. Diagnosis of this disorder is based on clinical assessment, and the average survival time is less than 3 years. Injections of IgG from ALS patients into mice are known to specifically mark motor neurons. Moreover, IgG has been found in upper and lower motor neurons in ALS patients. These results led us to perform a case-control study using human protein microarrays to identify the antibody profiles of serum samples from 20 ALS patients and 20 healthy controls. We demonstrated high levels of 20 IgG antibodies that distinguished the patients from the controls. These findings suggest that a panel of antibodies may serve as a potential diagnostic biomarker for ALS.
The authors present a case report and review the literature on Hashimoto encephalopathy. The onset of the disease may be marked by focal and then progressively generalized seizures or other neurological symptoms, but a cognitive decline or various psychiatric symptoms may also emerge. High levels of anti-thyroid peroxidase antibodies and/or anti-thyroglobulin antibodies are present in the serum. Corticosteroid treatment usually results in an improvement of symptoms. The syndrome is frequently overlooked and, therefore, the authors strongly recommend testing serum thyroid autoantibodies in cases with encephalopathy of unknown origin independently on the presence of thyroid disease in the patient or family history. The importance of long-term immunosuppressive treatment should also be stressed.
We tested the efficacy of treatment with talampanel in a mutant SOD1 mouse model of ALS by measuring intracellular calcium levels and loss of spinal motor neurons. We intended to mimic the clinical study; hence, treatment was started when the clinical symptoms were already present. The data were compared with the results of similar treatment started at a presymptomatic stage. Transgenic and wild-type mice were treated either with talampanel or with vehicle, starting in presymptomatic or symptomatic stages. The density of motor neurons was determined by the physical disector, and their intracellular calcium level was assayed electron microscopically. Results showed that motor neurons in the SOD1 mice exhibited an elevated calcium level, which could be reduced, but not restored, with talampanel only when the treatment was started presymptomatically. Treatment in either presymptomatic or symptomatic stages failed to rescue the motor neurons. We conclude that talampanel reduces motoneuronal calcium in a mouse model of ALS, but its efficacy declines as the disease progresses, suggesting that medication initiation in the earlier stages of the disease might be more effective.
Motor neurons that exhibit differences in vulnerability to degeneration have been identified in motor neuron disease and in its animal models. The oculomotor and hypoglossal neurons are regarded as the prototypes of the resistant and susceptible cell types, respectively. Because an increase in the level of intracellular calcium has been proposed as a feature amplifying degenerative processes, we earlier studied the calcium increase in these motor neurons after axotomy in Balb/c mice and demonstrated a correlation between the susceptibility to degeneration and the intracellular calcium increase, with an inverse relation with the calcium buffering capacity, characterized by the parvalbumin or calbindin-D(28k) content. Because the differential susceptibility of the cells might also be attributed to their different cellular environments, in the present experiments, with the aim of verifying directly that a higher calcium buffering capacity is indeed responsible for the enhanced resistance, motor neurons were studied in their original milieu in mice with a genetically increased parvalbumin level. The changes in intracellular calcium level of the hypoglossal and oculomotor neurons after axotomy were studied electron microscopically at a 21-day interval after axotomy, during which time no significant calcium increase was detected in the hypoglossal motor neurons, the response being similar to that of the oculomotor neurons. The hypoglossal motor neurons of the parental mice, used as positive controls, exhibited a transient, significant elevation of calcium. These data provide more direct evidence of the protective role of parvalbumin against the degeneration mediated by a calcium increase in the acute injury of motor neurons.
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