UNASSIGNED: Using transcranial magnetic stimulation (TMS) to delineate upper motor neuron (UMN) signs of two neurodegenerative disorders: amyotrophic lateral sclerosis (ALS) and multiple system atrophy (MSA).
UNASSIGNED: Medical records including clinical signs for UMN damage and TMS results were reviewed retrospectively. The UMN signs were classified into none, mild, and severe based on neurological examination of various reflexes. Then TMS-elicited motor evoked potentials (MEPs) were recorded from a hand and a leg muscle to calculate the central motor conduction time (CMCT), which represents fast, mono-synaptic conduction along the corticospinal tract. Relations between the UMN signs and CMCT were analysed for the two diseases.
UNASSIGNED: Prevalence and severity of the UMN signs for ALS and MSA were comparable for both upper and lower limbs. However, abnormality in CMCT was found more frequently in ALS: CMCT abnormalities were found in upper limbs for 44% in ALS patients but only for 7% in MSA patients; CMCT abnormalities in lower limbs were 55% in ALS and 20% in MSA. Some ALS patients showed abnormal CMCT in limbs without UMN signs, which was not true for most MSA patients.
UNASSIGNED: The abnormalities of CMCT were different in ALS and MSA, even for those who clinically had similar UMN signs. Sometimes, CMCT can reveal UMN damage in the absence of clinical UMN signs. Differences presumably derive from selective degeneration of different fibres in the motor descending pathways. Longitudinal studies must be conducted to accumulate neuroimaging and pathological findings.
UNASSIGNED: CMCT can be useful to differentiate ALS and MSA.

Spasticity causes an array of disabilities, which in turn may lead to the need for surgical intervention. Spasticity itself may also negatively affect surgical outcomes. This report reviews the potential benefit of perioperative (before, during, or after surgery) botulinum toxin (BoNT) injections for 3 patients with spasticity due to spinal cord injury, stroke, or multiple sclerosis. We discuss perioperative BoNT in 3 time periods: preoperatively, intraoperatively, and postoperatively. The cases demonstrate the use of perioperative BoNT in decreasing pain, improving wound healing, and improving surgical outcomes. We conclude by discussing the potential use of perioperative BoNT for surgical interventions in patients with spasticity and the need for further high-quality research in this field.

UNASSIGNED: To determine the reliability and validity of the Modified Heckmatt scale in assessing muscle echotexture in spasticity.
UNASSIGNED: Prospective, observational, 2-center study. Two residents and 2 ultrasound experienced staff physicians each rated 100 ultrasound images that were also analyzed using quantitative gray-scale.
UNASSIGNED: Academic ambulatory spasticity clinic.
UNASSIGNED: Participants (N=50) included 45 patients with upper or lower extremity spasticity and 5 healthy references.
UNASSIGNED: Not applicable.
UNASSIGNED: Modified Heckmatt scale ratings and quantitative gray-scale scores.
UNASSIGNED: Inter- and intra-rater intraclass correlation coefficients were 0.76 and 0.81, respectively (P<.001), indicating good to excellent reliability. A significant relationship was found between Modified Heckmatt scores and quantitative gray-scale scores (r=0.829; P<.001).
UNASSIGNED: The Modified Heckmatt scale demonstrated good reliability and validity to assess the pathologic muscle changes that occur in patients with spasticity.

Diagnosis of amyotrophic lateral sclerosis (ALS) depends on clinical evidence of combined upper motor neuron (UMN) and lower motor neuron (LMN) degeneration, although ALS patients can present with features predominantly of one or the other. Some UMN-predominant patients show hyperintense signal along the intracranial corticospinal tract (CST) on T2- and proton density (PD)-weighted images (ALS-CST +), and appear to have faster disease progression when compared to those without CST hyperintensity (ALS-CST -). The reason for this is unknown. We hypothesized that diffusion tensor tractography (DTT) would reveal differences in DTI abnormalities along the intracranial CST between these two patient subgroups. Clinical DTI scans were obtained at 1.5T in 14 neurologic controls and 45 ALS patients categorized into two UMN phenotypes based on clinical measures and MRI. DTT was used to quantitatively assess the CST in control and ALS groups. DTT revealed subcortical loss (\'truncation\') of virtual motor CST fibers (presumably) projecting from the precentral gyrus (PrG) in ALS patients but not in controls; in contrast, virtual fibers (presumably) projecting to the adjacent postcentral gyrus (PoG) were spared. No significant differences in virtual CST fiber length were observed between controls and ALS patients. However, the frequency of CST truncation was significantly higher in the ALS-CST + subgroup (9 of 21) than in the ALS-CST - subgroup (4 of 24; p = 0.049), suggesting this finding could differentiate these ALS subgroups. Also, because virtual CST truncation occurred only in the ALS patient group and not in the control group (p = 0.018), this DTT finding could prove to be a diagnostic biomarker of ALS. Significantly shorter disease duration and faster disease progression rate were observed in ALS patients with CST fiber truncation than in those without (p < 0.05). DTI metrics of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) were also determined in four regions of interest (ROIs) along the CST, namely: cerebral peduncle (CP), posterior limb of internal capsule (PLIC), centrum semiovale at top of lateral ventricle (CSoLV) and subcortical to primary motor cortex (subPMC). Of note, FA values along the left hemisphere virtual CST tract were significantly different between controls and ALS-CST + patients (p < 0.05) only at the PLIC level, but not at the CSoLV or subPMC level. Also, no significant differences in FA values were observed between ALS subgroups or between control and ALS-CST - groups (p > 0.05) in any of the ROIs. In addition, comparing FA values between ALS patients with CST truncation and those without in the aforementioned four ROIs, revealed no significant differences in either hemisphere. However, visual evaluation of DTT was able to identify UMN degeneration in patients with ALS, particularly in those with a more aggressive clinical disease course and possibly different pathologic processes.

Limb weakness in amyotrophic lateral sclerosis (ALS) is typically asymmetric. Previous studies have identified an effect of limb dominance on onset and spread of weakness, however relative atrophy of dominant and non-dominant brain regions has not been investigated. Our objective was to use voxel-based morphometry (VBM) to explore gray matter (GM) asymmetry in ALS, in the context of limb dominance. 30 ALS subjects were matched with 17 healthy controls. All subjects were right-handed. Each underwent a structural MRI sequence, from which GM segmentations were generated. Patterns of GM atrophy were assessed in ALS subjects with first weakness in a right-sided limb (n = 15) or left-sided limb (n = 15). Within each group, a voxelwise comparison was also performed between native and mirror GM images, to identify regions of hemispheric GM asymmetry. Subjects with ALS showed disproportionate atrophy of the dominant (left) motor cortex hand area, irrespective of the side of first limb weakness (p < 0.01). Asymmetric atrophy of the left somatosensory cortex and temporal gyri was only observed in ALS subjects with right-sided onset of limb weakness. Our VBM protocol, contrasting native and mirror images, was able to more sensitively detect asymmetric GM pathology in a small cohort, compared with standard methods. These findings indicate particular vulnerability of dominant upper limb representation in ALS, supporting previous clinical studies, and with implications for cortical organisation and selective vulnerability.

Magnetic resonance imaging measures have been proposed as objective markers to study upper motor neuron loss in motor neuron disorders. Cross-sectional studies have identified imaging differences between groups of healthy controls and patients with amyotrophic lateral sclerosis (ALS) or primary lateral sclerosis (PLS) that correlate with disease severity, but it is not known whether imaging measures change as disease progresses. Additionally, whether imaging measures change in a similar fashion with disease progression in PLS and ALS is unclear. To address these questions, clinical and imaging evaluations were first carried out in a prospective cross-sectional study of 23 ALS and 22 PLS patients with similar motor impairment and 19 age-matched healthy controls. Clinical evaluations consisted of a neurological examination, the ALS Functional rating scale-revised, and measures of finger tapping, gait, and timed speech. Age and ALSFRS score were not different, but PLS patients had longer duration of symptoms. Imaging measures examined were cortical thickness, regional brain volumes, and diffusion tensor imaging of the corticospinal tract and callosum. Imaging measures that differed from controls in a cross-sectional vertex-wise analysis were used as regions of interest for longitudinal analysis, which was carried out in 9 of the ALS patients (interval 1.26 ± 0.72 years) and 12 PLS patients (interval 2.08 ± 0.93 years). In the cross-sectional study both groups had areas of cortical thinning, which was more extensive in motor regions in PLS patients. At follow-up, clinical measures declined more in ALS than PLS patients. Cortical thinning and grey matter volume loss of the precentral gyri progressed over the follow-up interval. Fractional anisotropy of the corticospinal tracts remained stable, but the cross-sectional area declined in ALS patients. Changes in clinical measures correlated with changes in precentral cortical thickness and grey matter volume. The rate of cortical thinning was greater in ALS patients with shorter disease durations, suggesting that thickness decreases in a non-linear fashion. Thus, cortical thickness changes are a potential imaging marker for disease progression in individual patients, but the magnitude of change likely depends on disease duration and progression rate. Differences between PLS and ALS patients in the magnitude of thinning in cross-sectional studies are likely to reflect longer disease duration. We conclude that there is an evolution of structural imaging changes with disease progression in motor neuron disorders. Some changes, such as diffusion properties of the corticospinal tract, occur early while cortical thinning and volume loss occur later.