Parkinson-like features in ALS with predominant upper motor neuron involvement

Abstract

Owing to the frequent observation of poverty of movements, facial hypomimia and balance impairment, amyotrophic lateral sclerosis (ALS) variant with predominance of upper motor neuron involvement (UMN-ALS) is prone to be diagnosed with Parkinsonism. A clinical assessment, including the velocity-dependent stretch response test to differentiate between pyramidal and extrapyramidal stiffness; the Unified Parkinson's Disease Rating Scale and the Berg Balance Scale to assess degree of bradykinesia and postural instability; and (123)I-FP-CIT scintigraphy evaluation to investigate the nigrostriatal circuit involvement, were carried out to characterize Parkinson-like features in UMN-ALS patients. Sixteen UMN-ALS patients were included in the study. The velocity-dependent stretch response indicated spasticity in all the muscles tested. The degree of stiffness was found to be related to bradykinesia and postural instability. Eleven patients (70%) showed a reduction in striatal (123)I-FP-CIT uptake found to be related to disease duration and patients' ages but not to scores of the functional scales. Slowness of movements and postural instability noted in our patients could be mostly attributed to spasticity. The lack of any correlation between UPDRS or BBS scores and the degree of nigrostriatal impairment on DaTSCAN seems to disprove nigrostriatal circuit involvement in these extrapyramidal-like features.

Female gender doubles executive dysfunction risk in ALS: a case-control study in 165 patients

“…Neuroprotective action of oestrogens has been recognised in Parkinson's disease as well,25 which suggests several analogies with ALS. ALS and Parkinson's disease may share a common genetic mutation in TARDBP, vulnerability for FTD and analogies in clinical features 26 27. This oestrogen action may explain, in terms of gonadal hormones neuroprotection, the predominance of ALS in males in younger age groups, as described in the literature, whereas the risk of ALS is reported to be the same for males and females after menopause.…”

Abnormal trunk control determines postural abnormalities in Amyotrophic Lateral Sclerosis

“…It was frequently assumed that balance problems are mainly related to muscle weakness of the lower limbs (Jette, Slavin, Andres, & Munsat, 1999; Kloos et al, 2004). However, some authors explained postural instability by other impairments (Montes et al, 2007; Nardone, Galante, Lucas, & Schieppati, 2001), such as spasticity, contribution of the extrapyramidal system, a decreased ability to use the vestibular system, or a preferred use of visual cues (Pradat et al, 2009; D’Ascenzo et al, 2012; Sanjak et al, 2014). Other evidence for an impaired vestibular system stems from electronystagmographic findings in ALS patients (Steinmetz, Lebo, & Norris, 1974; Ohki et al, 1994).…”

Neural Correlates of Motor Imagery of Gait in Amyotrophic Lateral Sclerosis

“…9 Extrapyramidal features have been consistently demonstrated in patients with UMN-predominant (UMNp) symptoms. 10,11 The neural underpinnings of gait and postural control have been previously studied by functional magnetic resonance imaging (fMRI) motor imagery and positron emission tomography (PET). 12,13 Motor imagery activates a dynamic state, analogous to a specific motor action, but activated "internally" without any motor output.…”