Background and Purpose—Injury-induced cortical reorganization is a widely recognized phenomenon. In contrast, there is almost no information on treatment-induced plastic changes in the human brain. The aim of the present study was to evaluate reorganization in the motor cortex of stroke patients that was induced with an efficacious rehabilitation treatment. Methods—We used focal transcranial magnetic stimulation to map the cortical motor output area of a hand muscle on both sides in 13 stroke patients in the chronic stage of their illness before and after a 12-day-period of constraint-induced movement therapy. Results—Before treatment, the cortical representation area of the affected hand muscle was significantly smaller than the contralateral side. After treatment, the muscle output area size in the affected hemisphere was significantly enlarged, corresponding to a greatly improved motor performance of the paretic limb. Shifts of the center of the output map in the affected hemisphere suggested the recru...

https://www.ahajournals.org/doi/pdf/10.1161/01.STR.31.6.1210

Treatment-Induced Cortical Reorganization After Stroke in Humans

We have performed a systematic review to summarize current knowledge concerning factors related to survival in ALS and to evaluate the implications of these data for clinical trials design. The median survival time from onset to death ranges from 20 to 48 months, but 10-20% of ALS patients have a survival longer than 10 years. Older age and bulbar onset are consistently reported to have a worse outcome. There are conflicting data on gender, diagnostic delay and El Escorial criteria. The rate of symptom progression was revealed to be an independent prognostic factor. Psychosocial factors, FTD, nutritional status, and respiratory function are also related to ALS outcome. The effect of enteral nutrition on survival is still unclear, while NIPPV has been found to improve survival. There are no well established biological markers of progression, although some are likely to emerge in the near future. These findings have relevant implications for the design of future trials. Randomization, besides the type of onset, should take into account age, respiratory status at entry, and a measure of disease progression pre-entry. Alternative trial designs can include the use of natural history controls, the so-called minimization method for treatment allocation, and the futility approach.

/pdf/prognostic-factors-in-als-a-critical-review-2qjr56a63n.pdf

Prognostic factors in ALS: A critical review

Motor cortex plasticity during constraint-induced movement therapy in stroke patients.

Heterogeneity of motor phenotypes is a clinically well-recognized fundamental aspect of amyotrophic lateral sclerosis (ALS) and is determined by variability of 3 independent primary attributes: body region of onset; relative mix of upper motor neuron (UMN) and lower motor neuron (LMN) deficits; and rate of progression. Motor phenotypes are determined by the anatomy of the underlying neuropathology and the common defining elements underlying their heterogeneity are that motor neuron degeneration is fundamentally a focal process and that it spreads contiguously through the 3-dimensional anatomy of the UMN and LMN levels, thus causing seemingly complex and varied clinical manifestations. This suggests motor neuron degeneration in ALS is in actuality a very orderly and actively propagating process and that fundamental molecular mechanisms may be uniform and their chief properties deduced. This also suggests opportunities for translational research to seek pathobiology directly in the less affected regions of the nervous system.

/pdf/als-motor-phenotype-heterogeneity-focality-and-spread-32k1a4fpxb.pdf

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5-10% of the cases are familial ALS. Both sporadic and familial ALS (FALS) are associated with degeneration of cortical and spinal motor neurons. The etiology of ALS remains unknown. However, mutations of superoxide dismutase 1 have been known as the most common cause of FALS. In this study, we provide a comprehensive review of ALS. We cover all aspects of the disease including epidemiology, comorbidities, environmental risk factor, molecular mechanism, genetic factors, symptoms, diagnostic, treatment, and even the available supplement and management of ALS. This will provide the reader with an advantage of receiving a broad range of information about the disease.

A comprehensive review of amyotrophic lateral sclerosis.

Motor neuron disease presenting with respiratory failure

A new method is presented for mapping the motor cortex by transcranial magnetic stimulation in which the position of the stimulation coil on the scalp is measured using a 3D digitizer. The reproducibility of the method was tested by mapping 3 times the left abductor digiti minimi of 6 right-handed subjects and calculating the position of the centre of gravity (CoG), the are and volume of the individual maps. For individual maps, the coordinates of the CoG were found to be reproducible within ±3mm and the map areas and normalized volumes to within ± 20%, when the induced current flows anteriorly. Six more subjects were mapped to estimate interindividual variability of the position of the CoG. The method's ability to differentiate the cortical representation of two close muscles was successfully tested by mapping the flexor carpi radialis and the biceps brachii in another subject. Coordinates are given in a Cartesian frame of reference defined by the two tragi and the nasion. This feature will facilitate the comparison of results and their superposition on MR images.

A new method for reproducible coil positioning in transcranial magnetic stimulation mapping

Reflex sympathetic dystrophy associated with amyotrophic lateral sclerosis

To assess the correlation between the severity of clinical symptoms and sensorymotor and autonomic function in familial amyloidotic polyneuropathy of the Portuguese type (FAP-PT), clinical grade (CG), electromyographic score (ES), sympathetic (SS) and parasympathetic score (PS) were compared in 47 patients. SS and PS were both abnormal in 72% of patients with ES = 0% and in 73% of patients with CG = 0. When SS and PS were compared with either CG and ES, PS progressed more rapidly and stabilized earlier than SS. As a whole, SS and PS were greater as CG and ES were higher. This pattern of autonomic involvement should be taken into account when assessing the course of the disease and evaluating new forms of therapy.

Correlation between clinical, electromyographic and dysautonomic evolution of familial amyloidotic polyneuropathy of the Portuguese type.

Spinal cord compression (SCC) often presents a similar clinical picture to amyotrophic lateral sclerosis (ALS). An early differential diagnosis is important because SCC is a potentially treatable clinical disorder. We carried out a longitudinal study of 43 patients with an initial diagnosis of ALS, in order to ascertain the percentage of patients with SCC, and to evaluate the usefulness of somatosensory evoked potentials (SEPs) in early diagnosis. Thirty-three patients had a final diagnosis of ALS and 8 of SCC. SEPs central conduction was abnormal in 3 ALS and 7 SCC patients, respectively (Fisher exact test, p < 0.05). We concluded that SEPs investigation is useful in the differential diagnosis between ALS and SCC patients with pure motor signs.

M.L. Sales Luís

Papers

Motor neuron disease presenting with respiratory failure

A new method for reproducible coil positioning in transcranial magnetic stimulation mapping

Reflex sympathetic dystrophy associated with amyotrophic lateral sclerosis

Correlation between clinical, electromyographic and dysautonomic evolution of familial amyloidotic polyneuropathy of the Portuguese type.

Somatosensory evoked potentials in the differential diagnosis between compression and amyotrophic srinal cord literal sclerosis