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Neuro-Ophthalmologic Emergencies in Movement Disorders

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

Eye movement abnormalities due to urgent or emergent etiologies in the field of movement disorders include acute/subacute onset supranuclear saccadic gaze palsies, ocular flutter and opsoclonus, various types of nystagmus, and oculogyric crisis. Supranuclear saccadic gaze palsies, from failure of brainstem supranuclear saccadic burst neurons, cause slowing of saccades (horizontally and/or vertically) with or without range of motion limitation of eye movements. Acute/subacute causes of saccadic gaze palsies include vascular, neoplastic/paraneoplastic, infectious, and inflammatory etiologies. Ocular flutter and opsoclonus are erratic bursts of high frequency, rapid saccades that oscillate about the midline of the visual axis with no intersaccadic interval. Flutter occurs only in the horizontal plane, while opsoclonus occurs in all trajectories. Ocular flutter and opsoclonus typically occur either in infectious/para-infectious brainstem encephalitis or paraneoplastic/autoimmune disease. Nystagmus is a rhythmic biphasic oscillation of the eyes, initiated by a slow drift of the eyes. Downbeat nystagmus may occur acutely or subacutely from ischemic or paraneoplastic cerebellar disorders. Upbeat nystagmus is common in Wernicke encephalopathy and may occur from a brainstem stroke. Pendular nystagmus from oculopalatal myoclonus is not an emergency, but may be mistaken for a recurrent brainstem stroke. Oculogyric crisis is an acute dystonic reaction of extraocular muscles with a conjugate deviation of the eyes, typically upward. Oculogyric crises are most commonly acute or tardive reactions to neuroleptics, although other etiologies exist.

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Abbreviations

ACTH:

Adrenocorticotropic hormone

CJD:

Creutzfeldt-Jakob disease

CNS:

Central nervous system

CSF:

Cerebrospinal fluid

DBN:

Downbeat nystagmus

EEG:

Electroencephalogram

GABAA:

γ-Aminobutyric acid A

GAD:

Glutamic acid decarboxylase

INC:

Interstitial nucleus of Cajal

IV:

Intravenous

IVIG:

Intravenous immunoglobulin

MRI:

Magnetic resonance imaging

NMDA:

N-methyl-D-aspartate

OKN:

Optokinetic nystagmus

PAS:

Periodic acid-Schiff

PCA:

Posterior cerebral arteries

PCR:

Polymerase chain reaction

PERM:

Progressive encephalomyelitis with rigidity and myoclonus

PET-CT:

Positron emission tomography-computed tomography

PPRF:

Paramedian pontine reticular formation

PSP:

Progressive supranuclear palsy

RIMLF:

Rostral interstitial medial longitudinal fasciculus

UBN:

Upbeat nystagmus

VOR:

Vestibulo-ocular reflexes

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Authors and Affiliations

  1. Department of Neurology, New York University Grossman School of Medicine, New York, NY, USA

    Jenna Conway & Janet C. Rucker

  2. University of Utah Moran Eye Center, Salt Lake City, UT, USA

    Meagan D. Seay

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  1. Department of Neurology, NYU Langone Health, New York, NY, USA

    Steven J. Frucht

Electronic Supplementary Material

Segment 1. Opsoclonus. A 42-year-old man with no past medical history presented to the emergency department with acute-onset dizziness, shakiness, and oscillopsia. Examination revealed opsoclonus, head titubation, and truncal myoclonus. Brain magnetic resonance imaging (MRI) with contrast, cerebrospinal fluid (CSF) analysis, paraneoplastic antibody panel, and co-registered body positron emission tomography-computed tomography (PET-CT) scan were unremarkable. Infectious and immune serologies were unremarkable. He was treated empirically with intravenous immunoglobulin (IVIG) and gradually recovered fully over 3 months. The working diagnosis was para-infectious brainstem encephalitis, although no specific infectious agent was identified. The next patient, developed opsoclonus-myoclonus-ataxia syndrome after a viral infection. PET-CT and paraneoplastic workup was unremarkable, and she recovered completely after treatment with IVIG and steroids. Segment 2. Impaired bilateral abduction and adduction with upbeat nystagmus. A 59-year-old woman presented to the emergency department with acute-onset vertical oscillopsia and double vision. She denied other neurological symptoms, though her family reported mild confusion. Medical history included diabetes and hypertension. She denied tobacco use, but reported daily alcohol intake. On examination, she was oriented to person and place, but not to year. Eye movement examination revealed incomplete bilateral abduction and adduction and upbeat nystagmus. Gait was narrow-based and steady, but she had mild difficulty with tandem gait. MRI revealed increased T2 signal around the third ventricle in bilateral thalami. She was diagnosed with Wernicke’s encephalopathy and treated with intravenous (IV) thiamine. Segment 3. Assessment of horizontal saccadic velocity,first showing normal saccade speed, then a patient with slow horizontal saccades with an abnormal trajectory. Regarding saccade speed, a general “rule-of-thumb” is that the examiner should not be able to see the eye move through the full trajectory of motion, but rather should see it start and then land on target. If the eye can follow the full trajectory of the saccade, then it is too slow. Segment 4. Downbeat nystagmus (DBN) is jerk nystagmus with slow upward drifts followed by downward corrective fast phases (MP4 101864 kb)

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Conway, J., Seay, M.D., Rucker, J.C. (2022). Neuro-Ophthalmologic Emergencies in Movement Disorders. In: Frucht, S.J. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana, Cham. https://doi.org/10.1007/978-3-030-75898-1_2

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