Electronystagmographic findings in motor neuron disease

Abstract

The electronystagmogram (ENG) was abnormal in 94 percent of 88 consecutive patients with motor neuron disease (MND). The most common ENG abnormalities were dysrhythmic caloric responses (64 percent), active random eye movements with eyes closed (25 percent), caloric directional preponderance (24 percent), and caloric vestibular preponderance (20 percent). Torsional preponderance, poor fixation suppression (of induced vestibular nystagmus), spontaneous nystagmus, square waves, tracking and optokinetic abnormalities were also frequent. Most of these ENG findings are considered to be of central nervous system origin. Although these findings are not considered specific for MND, they appear to have clinical significance.

Abnormal trunk control determines postural abnormalities in Amyotrophic Lateral Sclerosis

“…In our opinion, their findings are also in line with our interpretation of impaired axial motor control in ALS. In contrast to the vestibular deficit suggested by Sanjak et al, (2014) and other authors (Steinmetz et al, 1974; Ohki et al, 1994), we found a lower Gain in ALS with eyes closed as compared to healthy control subjects indicating a pronounced use of vestibular information (Engelhart et al, 2014; Peterka, 2002). In addition, larger and faster spontaneous body sway (RMS, MV, see above) support the hypothesis of more prominent vestibular information due to a higher internal sensory noise (Mergner, Nasios, Maurer, & Becker, 2001).…”

“…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). However, the contribution of the different sensorimotor systems and their central processing to postural instability has not been identifiedyet.…”

Vestibular Deficits in Neurodegenerative Disorders: Balance, Dizziness, and Spatial Disorientation

“…Vestibular-evoked myogenic potential measurement has also been performed on patients with MND, showing no abnormalities in patients in the early stages of the disease ( 65 ). Additional assessment of the vestibular system in MND has found abnormalities in visual suppression ( 66 ) and caloric testing ( 67 ). Ohki et al found abnormalities of visual suppression in two out of nine patients with early stages of MND ( 66 ), which is indicative of cerebellum pathology.…”