Bedside examination of the vestibular and ocular motor system in patients with acute vertigo or dizziness

In the initial assessment of the patient with acute vertigo or dizziness, both structured history-taking and a targeted bedside neuro-otological examination are essential for distinguishing potentially life-threatening central vestibular causes from those of benign, self-limited peripheral labyrinthine origin and thus for deciding on further diagnostic testing. In this article, the key elements of the vestibular and ocular motor examination, which should be obtained at the bedside in these acutely dizzy patients, will be discussed. Specifically, this will include the following five domains: ocular stability for (I) nystagmus and for (II) eye position (skew deviation), (III) the head-impulse test (HIT), (IV) postural stability, and (V) ocular motor deficits of saccades, smooth pursuit eye movements, and optokinetic nystagmus. We will also discuss the diagnostic accuracy of specific combinations of these bedside tests (i.e. HIT, testing for nystagmus and vertical divergence, referred to as the H.I.N.T.S. three-step examination), emphasizing that the targeted neuro-otological bedside examination is more sensitive for identifying central causes in acute prolonged vertigo and dizziness than early MRI of the brain.

Functional anatomy of human extraocular muscles during fusional divergence

We employed magnetic resonance imaging to quantify human extraocular muscle contractility during centered target fusion and fusional divergence repeated with each eye viewing monocularly at 20 cm through 8Δ and at 400 cm through 4Δ base in prism. Contractility, indicated by posterior partial volume (PPV) change, was analyzed in transverse rectus and in medial and lateral superior oblique (SO) muscle compartments and by cross-sectional area change in the inferior oblique (IO). At 20 cm, 3.1 ± 0.5° (SE) diverging eye abduction in 10 subjects was associated with 4.2 ± 1.5% whole lateral rectus (LR) PPV increase ( P < 0.05) and 1.7 ± 1.1% overall medial rectus (MR) PPV decrease attributable to 3.1 ± 1.8% reduction in the superior compartment ( P < 0.025), without change in its inferior compartment or in muscles of the aligned eye. At 400 cm, 2.2 ± 0.5° diverging eye abduction in nine subjects was associated with 6.1 ± 1.3% whole LR PPV increase ( P < 10−5) but no change in MR, with compartmentally similar relaxation in the LR and MR of the aligned eye. Unlike convergence, there were no IO or SO contractile changes for divergence to either target nor any change in rectus pulley positions. Results confirm and extend to proximal divergence the unique role of the superior MR compartment, yet no MR role for far divergence. Corelaxation of aligned eye LR and MR combined with failure of MR relaxation during divergence is consistent with the limited behavioral range of divergence. NEW & NOTEWORTHY Magnetic resonance imaging shows that the lateral rectus muscle must overcome continued contraction by its opponent the medial rectus when humans diverge their visual axes to achieve single, binocular vision. While the upper but not lower compartment of the medial rectus assists by relaxing for near targets, it does not do so when targets are far away. This behavior violates Sherrington’s law of reciprocal action of antagonists and conventional assumptions about the ocular motor system.

Protocadherin-mediated cell repulsion controls the central topography and efferent projections of the abducens nucleus

SummaryCranial motor nuclei in the brainstem innervate diverse types of head and neck muscles. Failure in establishing these neuromuscular connections causes congenital cranial dysinnervation disorders (CCDDs) characterized by abnormal craniofacial movements. However, mechanisms that link cranial motor nuclei to target muscles are poorly understood at the molecular level. Here, we report that protocadherin-mediated repulsion mediates neuromuscular connection in the ocular motor system in zebrafish. We identify pools of abducens motor neurons that are topographically arranged according to soma size and convergently innervate a single muscle. Disruptions of Duane retraction syndrome-associated transcription factors reveal that these neurons require Mafba/MAFB, but not Sall4/SALL4, for differentiation. Furthermore, genetic perturbations of Pcdh17/Protocadherin-17 result in defective axon growth and soma clumping, thereby abolishing neuromuscular connectivity. Our results suggest that protocadherin-mediated repulsion forms the central topography and efferent projection pattern of the abducens nucleus following Mafba-dependent specification, and imply potential involvement of protocadherins in CCDD etiology.

Abnormal vision

Neuro-ophthalmology is a discipline comprising a wide variety of disorders that overlap the fields of neurology, ophthalmology and general medicine. Diagnosis in this field requires a thorough knowledge of the anatomy and physiology of the visual pathways and ocular motor system, as well as the ability to carry out a thorough neuro-ophthalmological examination. The combination of the history and any abnormalities identified by the examination should enable a detailed differential diagnosis to be reached, leading to appropriate investigations if required, and a final diagnosis.