Changes in the motor cortical excitability in neurological disorders

Several condition-test paradigms using transcranial magnetic stimulation （TMS） have been recently developed to study motor cortical （M1） excitability changes in human. The short interval intra-cortical inhibition （SICI），interheispheric inhibition （IHI） and cerebellar inhibition （CBI） are widely used methods to evaluate M1 modulation through intrinsic GABAergic neurons, callosal fibers, and cerebellum-thalamus-M1 pathways, respectively. We studied these modulations in several neurological disorders. The SICI was abnormally reduced in cortical myoclonus, focal dystonia or Parkinson’s disease. In contrast, it was normal in chorea or essential tremor. The GABAergic dysfunction may be produced by GABA reduction within M1 or abnormal M1 regulation from the basal ganglia. The IHI was reduced in mild cognitive imparment patients and cortical myoclonus. This may be due to GABAergic dysfunction within the cortex. The CBI was decreased in patients with many kinds of cerebellar ataxia, progressive nuclear palsy （PSP），and essential tremor （ET）．Because it tests the function of cerebello-cerebellar connection, we first showed cerebellar dysfunction in PSP and ET who have no clinical cerebellar symptoms. TMS has enable us to study the human central nervous system function physiologically

Japanese version of the ALS-FTD-Questionnaire (ALS-FTD-Q-J)

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share common clinical, genetic and neuropathological features. Some ALS patients have behavioral/personality changes, which could result in significant obstacles in the care provided by family members and caregivers. An easy screening tool would contribute greatly to the evaluation of these symptoms. We translated the ALS-FTD-Questionnaire, developed in the Netherlands, into Japanese (ALS-FTD-Q-J) and examined the clinimetric properties (internal consistency, construct and clinical validity). Patients with ALS and/or behavioral variant FTD (bvFTD) were evaluated alongside healthy controls in this multicenter study. All ALS patients, regardless of bvFTD status, were further evaluated by the frontal behavioral inventory (FBI) and for frontal/executive function, cognition, anxiety/depression, and motor functions. Data from 146 subjects were analyzed: ALS (92), ALS-bvFTD (6), bvFTD (16), and healthy controls (32). The internal consistency of the ALS-FTD-Q-J was good (Cronbach α=0.92). The ALS-FTD-Q-J showed construct validity as it exhibited a high correlation with the FBI (r=0.79). However, correlations were moderate with anxiety/depression and low with cognitive scales, in contrast to the original report, i.e. a moderate correlation with cognition and a low correlation with anxiety/depression. The ALS-FTD-Q-J discriminated ALS patients from (ALS-)bvFTD patients and controls. Thus, the ALS-FTD-Q-J is useful for evaluating Japanese ALS/FTD patient